Cardionerds: A Cardiology Podcast

CardioNerds (Amit Goyal and Daniel Ambinder), Dr. Pablo Sanchez (CardioNerds Ambassador, Stanford University Medical Center), Dr. Maria Pabon (CardioNerds Ambassador, Brigham and Women’s Hospital), and Karen Malacon (Student doctor and LMSA co-chair at Stanford University Medical Center) join Dean for Students at Harvard Medical School, Dr. Fidencio Saldana, for an important discussion about Latinx representation in cardiology. We established the multifaceted benefits of diversity in healthcare, including improving access, cultural competency, and quality of care delivered. We also talked about the need to increase the number of underrepresented minority students in medicine in addition to the importance of removing barriers to improve education. By providing appropriate resources as well as early mentorship and exposure to the medical field, we can address the “leaky pipeline,” or as Dr. Saldana reframed it, “the clogged pipeline.”  Then, we dove into Dr. Saldana’s experiences in medical school, the barriers he overcame, and how his parents’ hard work and generosity motivated him to become the cardiologist he is now. This event hosted the ACC Massachusetts Chapter. Stay tuned for a message by chapter Governor Dr. Malissa Wood. The PA-ACC & CardioNerds Narratives in Cardiology is a multimedia educational series jointly developed by the Pennsylvania Chapter ACC, the ACC Fellows in Training Section, and the CardioNerds Platform with the goal to promote diversity, equity, and inclusion in cardiology. In this series, we host inspiring faculty and fellows from various ACC chapters to discuss their areas of expertise and their individual narratives. Join us for these captivating conversations as we celebrate our differences and share our joy for practicing cardiovascular medicine. We thank our project mentors Dr. Katie Berlacher and Dr. Nosheen Reza. Audio editing by CardioNerds Academy Intern, Dr. Gurleen Kaur. Video Version • Quotables • Notes • References • Production Team The PA-ACC & CardioNerds Narratives in Cardiology PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Video version https://www.youtube.com/watch?v=cpq2wVgG8mA Quotable We strive to ensure that we have a diverse set of college students and a diverse set of medical students and residents and fellows. But I think it’s also just as important to ensure that we have the resources to ensure that those individuals that we’ve recruited and have done so hard to recruit, continue to succeed. I’ve realized that you can teach mentoring, you can teach advising. And I think it’s important to be able to create that culture and expectation. Some people may be a little bit better at it than others, but I think it’s important to place an emphasis on that at each level of training, so that you can train to be a better mentor and a better advisor. Show Notes 1. How is the LatinX representation in medicine compared to in the general population? Based on the most recent data from the US census Bureau, as of 2019, the Hispanic proportion of the US population is about 18.5%. A recent report by the AAMC showed that for the academic year 2020-21, of around 22,000 medical school matriculants, only 11% were from LatinX background, although this number was higher compared to 2017 where only 9.8% of the matriculants were of LatinX origin.   2. How does increasing workforce diversity improve quality of care? Cultural competency forms an important cornerstone of high-quality and equitable care for a diverse population, and it is learned not by lecture but by exposure, experience, and atmosphere. Medicine involves not only knowledge but meaningful connection and having a physician with a common background enhances the patient-doctor interaction by a spectrum of constructive effects. Inasmuch as research questions are guided by interests of investigators, the inclusion of underrepresented minorities among the pool of investigators stands to better delineate problems and articulate solutions. Actuating the gears of change at state/local governments and health systems to solve issues of access and quality of care will be only benefited by inclusion of medical-trained underrepresented minorities at the table.  3. How do can we promote diversity in fellowship programs? Participate in and support efforts to promote recruitment of underrepresented minorities for residency and fellowship programs as well as retention at the faculty level. Participate in initiatives focused on mentoring underrepresented minorities early on (high school or college students). Educate ourselves and others about the barriers that underrepresented minorities must overcome such as inadequate housing, food insecurity, and/or financial support. 4. What is the role of mentorship in diversity and inclusion? Mentorship is one of the pillars to diversify our health care workforce. Mentoring one single medical student will translate into exponential beneficial effects for patients. Mentors can help to overcome internal and systemic barriers that students may encounter during their path as early as during high school and college education. Mentorship does not stop after training is complete and should continue at the faculty level. Mentorship and advising can be taught and should be an expectation during training.  References Weinstein DF, Saldana F. DACA and the Dream of Becoming a Physician. N Engl J Med. 2017 Nov 16;377(20):1913-1915. doi: 10.1056/NEJMp1713102. Epub 2017 Oct 25. PMID: 29068757. Cohen JJ, Gabriel BA, Terrell C. The case for diversity in the health care workforce. Health Aff (Millwood). 2002 Sep-Oct;21(5):90-102. doi: 10.1377/hlthaff.21.5.90. PMID: 12224912. AAMC, Matriculants to US medical schools by race. 10/26/2020.    https://www.aamc.org/media/6031/download Production Team Daniel Ambinder, MD Amit Goyal, MD Gurleen Kaur, MD

CardioNerds (Amit Goyal and Daniel Ambinder), Cardio-OB series co-chair and University of Texas Southwestern Cardiology Fellow, Dr. Sonia Shah, and episode lead and Columbia University Cardiology Fellow Dr. Ersilia DeFilippis discuss hypertensive disorders of pregnancy (HDP) with Dr. Jennifer Lewey from the University of Pennsylvania. In this episode, we cover chronic hypertension, gestational hypertension, and pre-eclampsia—all of which encompass HDP and complicate approximately 5-10% of all pregnancies. We also review risk factors for HDP, diagnostic criteria, peripartum and postpartum management, and much more! Be sure to tune in to hear Dr. Lewey discuss the future impact of HDP on cardiovascular disease for women later in life and strategies that can help improve care. Finally Dr. DeFilippis shares her perspectives from her ACC.org FIT Section article titled “Shattering the Glass” including strategies to shattering the glass and tackling imposter syndrome in improving the representation of women in medicine. Audio editing by CardioNerds Academy Intern, Leticia Helms. Claim free CME for enjoying this episode! Pearls • Quotables • Notes • References • Guest Profiles • Production Team CardioNerds Cardio-Obstetrics Series PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Pearls To make a diagnosis of HDP, blood pressure should be measured on at least 2 occasions at least 4 hours apart. Hypertension diagnosed before 20 weeks gestation is considered chronic hypertension whereas hypertension after 20 weeks is categorized as gestational hypertension. Always check for warning signs for pre-eclampsia! One of the key treatments for pre-eclampsia is delivery but pre-eclampsia can occur still in the postpartum period. Aspirin use in pregnancy during second and third trimesters can reduce the risk of pre-eclampsia among women at risk. Quotables “Ideally a lot of these conditions come down to prevention and addressing potential cardio-obstetrics issues before they reach the cardio-obstetrics specialist.” “Blood pressure can continue to be elevated even after delivery.” “Pregnancy complications not only increase cardiovascular risk in the middle age population but also in the postmenopausal population.” “Women feel so relieved when they know that their care team is dedicated to taking care of women with cardiovascular disease when they are pregnant.” Show notes Why should CardionNrds learn and know about hypertensive disorders of pregnancy? Hypertensive disorders of pregnancy (HDP) complicate about 5-10% of all pregnancies. HDP is a term that encompasses chronic hypertension, gestational hypertension, pre-eclampsia, and pre-eclampsia superimposed on chronic hypertension. Women with history of HDP are at increased risk for stroke, ischemic heart disease, and cardiovascular death later in life. It is important to include obstetric history as part of our assessment of cardiovascular risk. This obstetric history should include the number of pregnancies, history of gestational diabetes, gestational hypertension, history of pre-eclampsia as well as history of pre-term birth. Following delivery, these women need close monitoring for development of chronic hypertension as well as primary prevention of cardiovascular disease. 2. How is gestational hypertension distinguished from chronic hypertension? The key factor in distinguishing gestational hypertension from chronic hypertension is the timing of diagnosis. Before 20 weeks gestation, elevated blood pressure (defined as a reading > 140/90 on two occasions at least 4 hours apart) is considered chronic hypertension. After 20 weeks gestation, elevated blood pressures signify a pregnancy-induced process, namely gestational hypertension or pre-eclampsia if blood pressure elevation is severe enough or there is evidence of end-organ damage. Blood pressure is often lower in the 1st and 2nd trimesters due to lower systemic vascular resistance. We think the process of gestational hypertension and pre-eclampsia result from how spiral arteries implant in the uterus. There are a whole host of local factors that lead to an increase in blood pressure later on in pregnancy. 3. What are warning signs of pre-eclampsia? Pre-eclampsia is diagnosed in the setting of high blood pressure, usually after 20 weeks, in the setting of proteinuria or end-organ dysfunction. End-organ dysfunction most commonly includes thrombocytopenia, transaminitis, right upper quadrant pain, headaches, pulmonary edema, and symptoms and signs that are not explained by other causes. Notably, blood pressure readings greater than > 160/110 mm Hg are sufficient for the diagnosis in the absence of other findings. 4. What are risk factors for pre-eclampsia or pre-eclampsia superimposed on chronic hypertension? Risk factors for pre-eclampsia include a personal or family history of pre-eclampsia in prior pregnancies, chronic hypertension, older age, multiple gestation, obesity, and Black race. 5. When do we start treatment for hypertension during pregnancy? For women who are newly diagnosed with hypertension during pregnancy, the threshold to begin treatment is 160 mm systolic and 110 mm diastolic. The caveat to this is for women with known cardiovascular disease or other comorbidities, a lower blood pressure goal should be targeted closer to 120-130 mm Hg systolic over 80 mm diastolic. Typically, a higher blood pressure is tolerated in order to avoid uteroplacental hypoperfusion which can impact the growth of the baby. The CHIPS randomized trial was published in the NEJM in 2015 and compared less tight control (targeting diastolic blood pressure of 100 mm Hg) or tight control (targeting diastolic blood pressure of 85 mm Hg). Although tight control decreased the rate of severe maternal HTN, there was no significant difference in the risk of pregnancy loss or overall maternal complications. How can we modify risk in women with hypertension during pregnancy? Low dose aspirin use (81 mg daily) in pregnancy during second and third trimesters can reduce the risk of pre-eclampsia among women at risk. The USPTF recommends the use of aspirin as preventative medication after 12 weeks of gestation in women at high risk for pre-eclampsia (Grade B). Risk factors that qualify someone as high risk include history of pre-eclampsia, multifetal gestation, chronic hypertension, type 1 or 2 diabetes, renal disease, and/or autoimmune disease. ACOG also recommends aspirin prophylaxis initiated between 12 and 28 weeks of gestation, optimally before 16 weeks and continued daily until delivery. 7. What is the best approach to anti-hypertensive medication use in pregnancy? First line agents used during pregnancy for treatment of hypertension include labetalol and nifedipine. Hydralazine is a second-line agent that may be used. Although methyldopa classically appears on board exams, it is less effective and has side effects including depression and fatigue. For women with a history of hypertension prior to pregnancy that is well-controlled on agents such as amlodipine or hydrochlorothiazide, these can be continued since limited evidence suggests they are safe although there is just less available evidence. For women on angiotensin-converting enzyme inhibitors, angiotensin-receptor blockers, or mineralocorticoid antagonists, these must be stopped given their teratogenic effects. This table from a recent JACC review by Halpern et al summarizes nicely safety of antihypertensive medications in pregnancy and during breastfeeding. 8. How do we treat pre-eclampsia? Treatment of pre-eclampsia depends on severity and gestational age. Management is primarily two-fold: 1) using intravenous medications such as labetalol and hydralazine to lower the blood pressure; 2) magnesium sulfate for seizure prophlaxis. Delivery may help resolve the symptoms of pre-eclampsia and may be considered following 37 weeks gestational age. References Halpern et al. Use of Medication for Cardiovascular Disease During Pregnancy: JACC State-of-the-Art Review. JACC 2019;73(4):457-476. ACOG Committee Opinion. Low Dose Aspirin-Use During Pregnancy. July 2018. Magee et al. Less-Tight versus Tight Control of Hypertension in Pregnancy. NEJM 2015;372:407-417. Wang Y-X, Arvizu M, Rich-Edwards JW, et al. Hypertensive disorders of pregnancy and subsequent risk of premature mortality. J Am Coll Cardiol. 2021;77(10):1302-1312. doi.org/10.1016/j.jacc.2021.01.018. Guest Profiles Dr. Jennifer Lewey Dr. Jennifer Lewey is an Assistant Professor at the  University of Pennsylvania, Director of Penn Women’s Cardiovascular Health Program, and Co-Director of the Pregnancy and Heart Disease Program. Dr. Silia DeFilippis Ersilia DeFilippis is a third-year general cardiology fellow at Columbia University Irving Medical Center in New York City. She will be beginning her fellowship in advanced heart failure and transplant cardiology next academic year at Columbia. Her research and clinical interest are in sex differences in advanced heart failure management and outcomes, racial disparities in heart failure outcomes, and cardio-obstetrics. CardioNerds Cardioobstetrics Production Team Natalie Stokes, MD Sonia Shah, MD Amit Goyal, MD Daniel Ambinder, MD

In the last episode, episode 126, we discussed pregnancy and aortic disorders as part of The CardioNerds Cardio-obstetrics Series. This episode brought to mind episode 76, where our colleagues from the Cleveland Clinic taught us about a woman named Lizzie Gasser, who at the young age of 27 tragically presented with postpartum pulmonary edema, found to have papillary muscle rupture, and was ultimately diagnosed with Vascular Ehlers Danlos Syndrome (VEDS) at autopsy. This case has been published in JACC Case Reports: CardioNerds Corner. Now, in this very special episode, we meet Lizzie Gasser, beyond her heart disease through the eyes of her loving husband, Todd Gasser. Her legacy underscores the importance of seeing our patients as people beyond their illness, in the context of their lives, values, and loved ones. This powerful discussion is led by Dr. Erika Hutt (cardiology fellow at the Cleveland Clinic) and Dr. Eunice Dugan,  (internal medicine resident at Johns Hopkins Hospital). Dr. Harry “Hal” Dietz (professor of pediatrics, an associate professor of medicine and an assistant professor of neurological surgery at the Johns Hopkins University School of Medicine and a leading authority on genetic aortopathies (including Loeys-Dietz Syndrome which carries his name). Audio editing by CardioNerds Academy Intern, Dr. Maryam Barkhordarian. This episode is brought to you in collaboration with the VEDS Movement. The VEDS Movement’s mission is to save lives and improve the quality of life of individuals with Vascular Ehlers-Danlos Syndrome (VEDS). By pursuing the most innovative research, educating the medical community, general public and affected individuals, and providing support to patients, families, and caregivers, The VEDS Movement, which is a division of The Marfan Foundation, charges forward and improves the outcomes for those living with VEDS. Individuals affected with VEDS can access medical webinars presented by the experts, join support groups, get involved in events and research, and donate by visiting TheVEDSMovement.org. Providers can also find resources, including CME opportunities, at TheVEDSMovement.org. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Photos & Youtube videos featuring Lizzie Gasser and her family https://www.youtube.com/watch?v=W4sVuctrUZs&t=1s Video from the VEDS movement that Lizzie’s family is interviewed in. Their interview is from 7m30s – 17m16s  https://www.youtube.com/watch?v=6CeXYnZHhbg Video of Lizzy’s life life that a friend put together. Episode Guests Dr. Erika Hutt Dr. Eunice Dugan Dr. Harry “Hal” Dietz CardioNerds Case Report Production Team Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerd Amit Goyal, Cardio-OB series co-chair and University of Texas Southwestern Cardiology Fellow, Dr. Sonia Shah, and episode lead and Johns Hopkins University Cardiology Fellow, Dr. Anum Minhas, discuss pregnancy and aortic disorders with Dr. Nupoor Narula of Weill Cornell Medical College. Special introduction by Sukrit Narula. In this episode we discuss the presentation and management of aortopathies during pregnancy. We begin by examining the pathophysiology of aortic disease during pregnancy, followed by a review of the heritable aortopathies and their risk of dissection. We then discuss preconception evaluation and antepartum care of women with aortopathies. We end with addressing management at the time of labor and delivery. Claim free CME for enjoying this episode! Abstract • Pearls • Quotables • Notes • References • Guest Profiles • Production Team CardioNerds Cardio-Obstetrics Series PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Episode Abstract – Pregnancy and Aortic Disorders In this episode we discuss the presentation and management of aortopathies during pregnancy. We begin by examining the pathophysiology of aortic disease during pregnancy, followed by a review of the heritable aortopathies and their risk of dissection. We then discuss preconception evaluation and antepartum care of women with aortopathies. We end with addressing management at the time of labor and delivery. Pearls – Pregnancy and Aortic Disorders 1. Assessment of aortic root and ascending aortic measurements should be performed prior to conception in women with known aortopathies, connective tissue diseases with high risk for aortopathies, bicuspid aortic valve or familial thoracic aortic syndromes. Dimensions should always be verified with multi-modality imaging prior to decision-making. 2. It is important to recognize that the immediate postpartum period is a high risk period for aortic dissection in women with aortopathies. 3. Goal systolic blood pressure is < 120 mmHg and diastolic blood pressure is < 80 mmHg in women with aortopathies. This goal should be discussed with the patient’s obstetric provider during pregnancy, however, to ensure a blood pressure target is chosen that is appropriate for optimal maternal and fetal outcomes. Beta-blockers followed by calcium channel blockers should be used for these targets. Aldosterone receptor antagonists are contraindicated during pregnancy. 4. Per the 2018 ESC guidelines, during pregnancy, women with aortic pathology should have an echocardiogram performed every 12 weeks if low risk, and every month (4 weeks) if high risk. 5. Women with type A dissection during pregnancy should be evaluated for urgent Caesarean section and aortic surgery if the fetus is viable. Women with a stable type B dissection may be managed medically if stable. All decisions should be made in a multidisciplinary fashion with consultation with Maternal Fetal Medicine and Cardiothoracic Surgery. Quotables – Pregnancy and Aortic Disorders 1. “First and foremost, we must verify our dimensions. I’ll say that again. We must verify our dimensions because nothing could have greater impact.” 2. “You know, our most important role is to provide transparency in pre-pregnancy risk counseling. We have to present the data that are available. We need to present the gaps in literature.  We need to present the possibility that in the right individual, we can proceed through pregnancy safely with close clinical and imaging monitoring and follow up in a multidisciplinary construct. Show notes – Pregnancy and Aortic Disorders What is the risk of aortic dissection during pregnancy in Marfan syndrome? Women who undergo pregnancy with prepartum counseling and close clinical and imaging follow-up usually maintain stability of aortic dimensions over time and risk of type A dissection appears low. Type B dissection remains unpredictable. Overall, the risk of aortic complications surrounding pregnancy remains eight-fold higher than in the non-pregnant state. The risk for dissection is higher with greater aortic root size, but there still remains a risk for dissection at diameters less than 40 mm. Note, in the GenTAC registry (which included 184 women with Marfan’s Syndrome in whom pregnancy information was available), lack of knowledge of the underlying diagnosis of Marfan’s was not uncommon. What is the underlying pathophysiology behind increased risk for aortic dissection during pregnancy? Pregnancy is associated with both hemodynamic changes and alterations in tissue architecture which may both increase the risk of exacerbating an underlying aortopathy syndrome. Physiologic changes during pregnancy include increased stroke volume and heart rate, resulting in increased cardiac output, with concomitant decrease in the peripheral resistance, particularly in the second and third trimesters and immediate postpartum period (Review our Initial Episode in the Cardio-Ob Series for more details). Pregnancy-associated morphologic and histochemical changes of the aortic wall also occur. In women with Marfan syndrome, at baseline aortic tissue tends to be weaker and more prone to dilatation and dissection. Animal studies also implicate oxytocin in increasing dissection risk, with a suggestion that withholding lactation or administration of an oxytocin receptor antagonist may reduce dissection risk. For patients with suspected or confirmed aortopathy or familial aortopathy syndromes, what evaluation is recommended prior to and during pregnancy? Complete physical exam, including cardiac and vascular exams, should be performed, as well as review of personal and family medical/surgical history. Echocardiogram should be performed to inform the safety of pregnancy.  Echocardiogram can be used to obtain aortic dimensions and identify any co-existing disease prior to conception. Per the 2018 ESC guidelines, during pregnancy, women with aortic pathology should have echocardiogram performed every 12 weeks if low risk, and every month (4 weeks) if high risk. Beta-blockers throughout pregnancy should be considered, along with fetal growth monitoring.  While aldosterone receptor antagonists may normally be advised, these are contraindicated during pregnancy due to their teratogenic effects and should be withheld. Collaboration with colleagues in Maternal Fetal Medicine for management decisions is critical. What are some specific syndromes related to aortic pathology and the risks of each of these conditions? Marfan syndrome AHA/ACC guidelines recommend consideration of elective aortic surgery prior to pregnancy if aortic root is > 4.0 cm. The 2018 ESC guidelines advise against pregnancy for aortic root diameter > 4.5 cm. The decision to intervene prophylactically during pregnancy is highly individualized and complex, and we must weight patient factors including gestational age and rapidity of growth while balancing risk/benefits to the fetus and mother.  Ehlers-Danlos syndrome (EDS) Vascular EDS patients are at high risk of arterial and uterine rupture, and the 2018 ESC Guidelines recommend against pregnancy. If after shared-decision making a patient decides to proceed with pregnancy, the patient should be cared for in a specialized center with a multi-disciplinary Cardio-Ob team. Loeys-Dietz syndrome There is limited data is available, and it is insufficient to make any clear recommendations regarding pregnancy. Most centers will generally approach prophylactic aortic root surgery in Loeys-Dietz syndrome similar to Marfan disease and consider elective repair at 4.0 to 4.5 cm. More frequent intrapartum echocardiograms may be considered given some suggestion of increased risk of dilatation during pregnancy. Turner Syndrome There is a strong association with bicuspid aortic valve (present in 15-30% of patients). Generally, we consider elective surgery in patients with an aortic size index (ASI) (ascending aortic root size indexed to body surface area) of > 2.5 cm/m2 and that pregnancy should be avoided at this ASI cutoff. During pregnancy, prophylactic aortic surgery can be considered with a dilated aorta (> 2.5 cm/m2 or rapid enlargement > 3mm) Many women with Turner syndrome require assisted reproductive therapy (ART) and assisted reproductive therapy may increase the risk of cardiovascular complications. Imaging of the thoracic aorta and heart are recommended within two years prior to pregnancy or assisted reproductive therapy. Imaging should be performed at least once per 20 weeks if there is no increased ASI or other cardiovascular risk factors (e.g., family history of dissection or sudden death, bicuspid aortic valve, coarctation, elongation of the transverse arch). Imaging should be performed every 4-8 weeks for those with ASI > 2.0 cm/m2 or additional risk factors during pregnancy, and once during the first month after delivery. Subsequent imaging intervals depend on the severity of the aortic enlargement. Bicuspid aortic valve (BAV) Up to 50% of patients with BAV also have ascending aortic dilatation ESC guidelines recommend elective aortic surgery if the ascending aorta is > 5.0 cm prior to pregnancy. Familial Thoracic aortic aneurysms Family history is present in 20% of patients. A series in 53 women with ACTA2 mutations and total of 137 pregnancies showed 8 pregnancy-associated dissections (6 were type A) . Evidence is limited, however, and no clear recommendations for pregnancy are available. What are some delivery considerations for women with aortopathies? The overall goal is to decrease the cardiovascular stress of the delivery process, and risk for both the mother and baby need to be considered jointly with the Maternal Fetal Medicine team. For Marfan Syndrome and aortic root between 4-4.5 cm, vaginal delivery with an expedited second stage and regional anesthesia is typically the preferred mode of delivery to avoid spikes in blood pressure. For patients with vascular EDS, consider C-section at 35-37 weeks’ gestation. There is a similar recommendation for patients with Marfan syndrome and aortic root > 4.5 cm and Turner syndrome patients with ASI > 2.5 cm/m2. For those with a type A dissection during pregnancy the approach depends on fetal viability. If viable, then urgent C-section and surgical aortic repair should be performed. If the fetus is currently non-viable, aortic repair is performed without delivery, acknowledging the high fetal risk in this context. The approach to unstable type B dissection is as for type A dissections as above with C-section and aortic surgery if the fetus is viable or aortic surgery without deliver if the fetus is not viable at the time.  For stable type B dissection, medical therapy can be pursued. References Regitz-Zagrosek V et al. 2018 ESC Guidelines for the Management of Cardiovascular Diseases during Pregnancy: The Task Force for the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology. European Heart Journal. 2018;39(34):3165-3241. https://academic.oup.com/eurheartj/article/39/34/3165/5078465#186438440 Mehta et al. Cardiovascular Considerations in Caring for Pregnant Patients: A Scientific Statement from the American Heart Association. Circulation. 2020;141:e884–e903. https://www.ahajournals.org/doi/suppl/10.1161/CIR.0000000000000772 Guest Profiles Dr. Nupoor Narula Dr. Nupoor Narula. Dr. Narula earned her medical degree from the Icahn School of Medicine at Mount Sinai, and graduated with Distinction in Research. She then went to the esteemed Mayo Clinic for Internal Medicine residency, followed by a Vascular Medicine fellowship at Mount Sinai Hospital and Cardiology Fellowship at New York Presbyterian Hospital – Weill Cornell Medical College. She has pursued basic and clinical research training in cardiovascular genetics and is currently completing her Masters in Clinical Epidemiology. Dr. Narula is also a superstar researcher and received grant funding for her research during her Fellowship by the Michael Wolk Heart Foundation and is currently a Fund for the Future Grant Kellen Scholar at Weill Cornell Medicine. She is studying aortopathies, such as Marfan syndrome, with special emphasis on pregnancy and dissection risk. Dr. Anum Minhas Dr. Anum Minhas went to medical school at Duke University. She had an interest in imaging and spent a year studying radiation dose estimation and radiation safety. She also developed a passion for clinical cardiology and subsequently went to the University of Michigan for internal medicine residency. Here she studied CT reconstruction for transcatheter aortic valve replacement, as well as the incidence of Takotsubo cardiomyopathy. Anum is a fourth year cardiology fellow at Johns Hopkins interested in cardio-OB. In fact, she has designed and is the inaugural Cardio-Obstetrics fellow at Hopkins. In her free time she enjoys drawing, reading, traveling, and spending time with friends and family. CardioNerds Cardioobstetrics Production Team Natalie Stokes, MD Sonia Shah, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal & Karan Desai) join University of Minnesota fellows, Dr. Julie Power, Dr. Sasha Prisco, and Dr. Abdisamad Ibrahim for a riveting discussion in which they were pressured to diagnose a young woman with syncope. The fellows expertly take us through the next steps in the differential diagnosis, and management of pulmonary hypertension in this young patient! University of Minnesota faculty and expert in right ventricular (RV) failure in pulmonary arterial hypertension (PAH) Dr. Kurt Prins provides the E-CPR for this episode. With this episode, the CardioNerds family warmly welcomes The University of Minnesota to the CardioNerds Healy Honor Roll. The CardioNerds Healy Honor Roll programs support and foster the the CardioNerds spirit and mission of democratizing cardiovascular education. Healy Honor Roll programs nominate fellows from their program who are highly motivated and are passionate about medical education. The University of Minnesota fellowship program director, Dr. Jane Chen has nominated Dr. Julie Power for this position. In addition to being a CardioNerds Ambassador, Julie has already done amazing CardioNerds work as part of the CardioNerds Academy fellowship. Claim free CME just for enjoying this episode! Disclosures: None Jump to: Patient summary – Case media – Case teaching – References CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Patient Summary- Syncope and Pulmonary Hypertension A Somali woman in her mid-30s with no significant past medical history presented with shortness of breath and exertional syncope. EKG revealed evidence of RV strain. CTA-PE protocol did not show PE. However, there was RV dilation and subsequent echocardiogram demonstrated normal LV, but moderately reduced RV function with evidence of RV pressure and volume overload. RVSP was estimated to be 188 mmHg! Case Media A B C D E F G H Click to Enlarge A. CXR, B. ECG, C. PA measurements: Main PA measures 2.4 cm, right PA measures 2.3 cm, left PA measures 1.9 cm, D. Tricuspid valve Doppler, E. RA tracing, F. RV tracing, G. PA tracing, H. Wedge tracing CTA PE: No PE, markedly dilated pulmonary trunk at 4.7 cm. Right main pulmonary artery measures 3.1 cm. TTE: Parasternal long axis: Moderate right ventricular dilation compressing left ventricle. Global right ventricular function is moderately reduced. TTE: Parasternal long axis- RV view: Right ventricular dilation with mild pulmonary regurgitation TTE: Mild pulmonary regurgitation with dilation of main PA TTE: Paradoxical septal motion consistent with right ventricular pressure and volume overload. TTE: Apical 4 chamberParadoxical septal motion consistent with right ventricular pressure and volume overload. Moderate right ventricular dilation.Global right ventricular function is moderately reduced.Severe right atrial enlargement. Paradoxical septal motion consistent with right ventricular pressure and volume overload.Moderate right ventricular dilation.Global right ventricular function is moderately reduced.Severe right atrial enlargement.Moderate to severe tricuspid regurgitation. TTE: Positive bubble study Episode Teaching Pearls Pulmonary hypertension (PH) can generally be categorized as pre-, post-, or combined pre- and post-capillary PH. Isolated pre-capillary pulmonary hypertension is characterized by: mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, a pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) ≥ 3 Woods units (WU). Pulmonary arterial hypertension (PAH) (WHO Group 1) falls under pre-capillary pulmonary hypertension. Schistosomiasis is the most common cause of PAH (WHO Group I) worldwide. Approximately 7% of patient with hepatosplenic schistosomiasis have PAH. Some studies suggest that treatment of with praziquantel reverses vascular remodeling; however, there is point of no return, beyond which, anthelmintic therapies are ineffective to prevent progression. Exertional syncope and pericardial effusion are both risk factors for higher mortality in PAH. Women with severe PAH have extremely high risk of maternal morbidity and mortality. Endothelin receptor antagonists are contraindicated in pregnancy due to teratogenicity. Therefore, a pregnancy test must be obtained monthly while on this therapy. Patients with a lower socioeconomic status, based on median household income, have more advanced PAH at the time of diagnosis. Notes 1. How do you approach syncope? Syncope is a sudden transient loss of consciousness associated with absence of postural tone followed by complete and usually rapid recovery. There should be not be clinical evidence of “non-syncope” conditions including seizures, hypoglycemia, drug or alcohol intoxication, concussion due to head trauma and so forth. One approach to determining the etiology of the syncope is to consider 4 major categories: orthostatic, reflex-mediated, cardiac-obstructive, or cardiac-electrical. Reflex-mediated (neurocardiogenic) syncope typically has a prodrome and encompasses vasovagal syncope, situational syncope, and carotid hypersensitivity. Orthostatic syncope is syncope occurring when rising from recumbency. It is generally associated with an orthostatic SBP drop by more than 20 mmHg or DBP drop by more than 10 mmHg with a compensatory rise in heart rate. We most commonly think of dehydration or hypovolemia causing orthostasis. Being post prandial can cause orthostasis as well. Neurogenic orthostatic hypotension (OH) involves excessive pooling of blood volume in the splanchnic and/or leg circulation. Upon standing, there is decreased venous return to the heart with a subsequent decrease in cardiac output and cerebral perfusion. The autonomic nervous system can typically increase vascular tone, inotropy and chronotropy; however, in neurogenic OH these mechanisms are inadequate. Conditions where neurogenic OH is relatively common include multiple system atrophy, Parkinson’s disease, Huntington’s disease, peripheral neuropathies (e.g., diabetes, amyloidosis), and spinal cord injury, amongst other etiologies. Finally, common medications associated with orthostatic syncope include diuretics, alpha blockers, and tricyclic antidepressants. Cardiac-obstructive syncope may occur from structural obstruction (i.e., aortic stenosis, HCM, mitral stenosis, pulmonary embolism) or other lesions which limit the stroke volume (i.e., pericardial tamponade, pulmonary hypertension. Cardiac-electrical syncope include both tachyarrhythmias and bradyarrhythmias, often without a prodrome. 2. What are the different types of pulmonary hypertension (PH)? What are the hemodynamic definitions of pulmonary hypertension? The WHO separates PH into 5 groups: Group 1: Pulmonary arterial hypertension (e.g., idiopathic, heritable [BMPR2], anorexigen associated, drug or toxin-associated, HIV, connective tissue disease associated, schistosomiasis, portal hypertension, congenital heart disease, amongst other causes) Group 2: Pulmonary hypertension due to left sided heart disease (e.g., HFrEF, HFpEF, left-side valvular heart disease) Group 3: Pulmonary hypertension due to lung disease or hypoxia: (e.g.,COPD, ILD, OSA, hypoxia without lung disease such as high altitude, developmental lung disorders) Group 4: PH due to pulmonary artery obstructions most commonly Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Group 5: Multifactorial causes such as hematologic disorders (chronic hemolytic anemia, as with myeloproliferative disorders), metabolic disorders (e.g., Gaucher disease, glycogen storage diseases, CKD), and systemic disorders (e.g., pulmonary Langerhans cell histiocytosis, neurofibromatosis, sarcoidosis) When we consider the hemodynamics of pulmonary hypertension, we break down PH into isolated pre-capillary, isolated post-capillary, or combined pre-and post-capillary pulmonary hypertension. Mean Pulmonary Artery Pressure (mmHg) Wedge Pressure (mmHg) Pulmonary Vascular Resistance (Woods Units) WHO Groups Pre-capillary > 20 ≤ 15 ≥ 3 1, 3, 4, 5 Post-capillary > 20 > 15 < 3 2, 5 Combined pre- and post-capillary > 20 > 15 ≥ 3 2, 5, multifactorial PAH falls under pre-capillary pulmonary hypertension, which is defined as mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, a pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) ≥ 3 Woods units (WU). 3. How do you work up suspected PAH? To start investigating for PAH, as always, we start with a thorough history and physical. The most common presenting symptom of pulmonary hypertension in general is exertional dyspnea/reduced exercise tolerance. Symptoms of PH can be nonspecific, especially early in its course, and thus there can be a delay in diagnosis. Remember, some patients have increased risk of developing PH and should be screened. These are patients with known risk factors for developing PAH, including relatives of patients with BMPR2 mutations, HIV, connective tissue disease (especially systemic sclerosis), portal hypertension, etc. Other accompanying symptoms may include chest pain, fatigue, and lightheadedness. Manifestations of more advanced disease include syncope, abdominal distension, and significant lower extremity edema attributable to right ventricular (RV) failure. Physical exam may reveal a loud P2, murmur of tricuspid regurgitation, an RV S3, jugular venous distension with or without Kussmaul’s sign, liver pulsatility, ascites, and/or peripheral edema. Review the CXR and EKG for findings consistent with pulmonary hypertension. Common EKG findings include right atrial enlargement, right axis deviation, RBBB, and an RV strain pattern in the right precordial leads. Findings on chest X-ray may include enlarged main and hilar pulmonary arteries (with loss of the peripheral blood vessels) and RV enlargement. Basic lab work should include a CBC with differential, biochemistry, TFTs, and HIV. The patient’s clinical presentation could lead you to screen for serologic evidence of connective tissue disorders or hepatitis. Cardiac biomarkers, including NT-proBNP, may have a role in prognosis and treatment response. Echocardiogram is essential in the evaluation of PH. In addition to estimating the pulmonary artery systolic pressure (PASP) by measuring the tricuspid regurgitant jet, we can characterize RV and RA size, RV function, and RV wall thickness, which may help both support the diagnosis and gauge prognosis for PH. Further, we can evaluate for left-sided heart disease contributing to PH. The presence of a pericardial effusion is a poor prognostic sign.  PFTs, overnight oximetry with blood gases, and CT chest can help delineate the relative contribution of pulmonary disease to the patient’s PH disease (WHO Group 3). VQ scan can rule out a diagnosis of CTEPH with high sensitivity (WHO Group 4). Right heart catheterization is necessary to confirm the diagnosis of PH. Furthermore, we can determine if a patient is “vasodilator responsive.” In the catheterization lab, a positive vasodilator response is defined as a decrease in mPAP ≥ 10 mmHg to an absolute value of ≤ 40 mmHg (without a decrease in cardiac output) with the use of inhaled nitric oxide or IV epoprostenol. If a patient has positive vasodilator test, calcium channel blockers can be initiated, however not all patients will be long term responders. We tend to do vasoreactivity testing in patients with PAH and not for other forms of PH (e.g., Pulmonary Veno-Occlusive Disease or Groups 2, 3, 4, or 5). 4. What are PAH specific pharmacologic treatments? Remember that PAH is fundamentally a disease of increased pulmonary vascular resistance (PVR) causing elevated pulmonary pressures. The consequence of increased PVR includes increased RV afterload and hypoxemia and the subsequent clinical manifestations of PAH. Normally, the pulmonary vascular bed has a balance between vasodilators and vasoconstrictors that can maintain a low-resistance, high-compliance state. This balance is disturbed in PAH and the goal of therapy is to “restore” balance between vasodilation and vasoconstriction. The management of PAH has 3 medication groups: Nitric oxide pathway: PDE5 inhibitors: Sildenafil and tadalafil. These medications prevent the breakdown of cGMP which mediates the potent vasodilator and inhibitor of platelet aggregation, nitric oxide. The most common side effect for these medications is headache. Remember these medications should not be taken with nitrates! Soluble guanylate cyclase (sGC) stimulators: riociguat. These medications stimulate sGC and thus increase sensitivity to NO. Riociguat is primarily used in CTEPH, but can also be used in PAH (PATENT-1 and -2 Trials) including PAH associated with sickle cell disease Endothelin-1 (ET-1) pathway: Endothelin receptor antagonists (ERAs): macitentan, bosentan, ambrisentan. Endothelin-1 is produced by endothelial cells and acts on two receptors, endothelin receptor A (ET-A) and endothelin receptor B (ET-B). ET-A is expressed on vascular smooth muscle cells and ET-B on both smooth muscle cells and endothelial cells. Stimulation of both receptors tends to lead to vasoconstriction, while stimulation of ET-B leads to vasodilation. ERAs antagonize these receptors to shift the balance towards vasodilation (e.g., bosentan is a dual ET-A/ET-B antagonist and ambrisentan is a more selective ET-A antagonist). Common side effects are lower extremity edema and hepatotoxicity. NOTE: patient should not get pregnant on ERAs because of teratogenicity! Prostacyclin Pathway Prostacyclin analogs: epoprostenol, iloprost, treprostinil. Prostacyclin is a potent endogenous vasodilator and inhibits platelet aggregation. This class of medications have PO, SQ, IV, and inhaled formulations. Common side effects include headache, diarrhea, nausea, and jaw pain. PCA-receptor agonist: selexipag (oral). Also remember CCB in vasodilator responsive patients with PAH! Other aspects of pharmacologic PAH treatment not discussed here include diuretics, digoxin, and oral anticoagulation, especially for patients with more advanced disease and on continuous parenteral prostacyclin therapy due to microthrombi in pulmonary arterioles. 5.How do we risk stratify PAH patients and response to treatment? The REVEAL 2.0 risk score helps determine 1 year mortality based on WHO group, renal function, functional class, age, heart rate, 6 minute walk test, BNP, presence of pericardial effusion of echocardiogram, PFTs, and RHC values. Patients should also undergo routine 6 minute walk test and/or cardiopulmonary exercise test to assess their functional status and response to medications. Biomarkers may be helpful to assess treatment response. Echocardiography and RHC may be used every 6 to 12 months in patients with unstable or deteriorating symptoms to guide therapy and then considered on a case-by-case basis in stable patients. References Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009; 54(suppl 1):S55-S66. Barst RJ, Gibbs JS, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2009; 54(suppl 1):S78-S84. Benza RL, Gomberg-Maitland M, Miller DP, et al. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012; 141(2):354-362. Delcroix M. and Naeije R.: “Optimising the management of pulmonary arterial hypertension patients: emergency treatments”. Eur Respir Rev. 2010; 19: 204. Knafl D, Gerges D, King CH, Humbert M, Bustinduy AL. Schistosomiasis-associated pulmonary arterial hypertension: a systematic review. European Respiratory Review. 2020; 29 (155). McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009; 119:2250-2294. Nazzareno G, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013; 62(sup 25):D60-D72. Sherman, Stephanie (Host). (2019, January 31). Syncope (Episode 12) [Audio podcast episode]. In The Clinical Problem Solvers. https://clinicalproblemsolving.com/2019/01/31/episode-12-syncope-with-dr-stephanie-sherman/. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019: 53(1). Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009; 54(suppl 1):S43-S54. Sitbon O, Gaine S. Beyond a single pathway: combination therapy in pulmonary arterial hypertension. European Respiratory Review. 2016; 25 (142) 408-417. Talwar A, Sahni S, Talwar A, Kohn N, Klinger JR. Socioeconomic status affects pulmonary hypertension disease severity at time of first evaluation. Pulm Circ. 2016; 6(2):191-195. doi:10.1086/686489. CardioNerds Case Report Production Team Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerd (Amit Goyal), cardioobstetrics series co-chair Dr. Sonia Shah (FIT, UT Southwestern) and episode lead Dr. Kayle Shapero (FIT, UPMC) discuss pregnancy in patients with pulmonary hypertension with Dr. Candice Silversides, Associate Professor of Medicine and the Director of the Pregnancy and Heart Disease program and head of the Obstetric Medicine program at the University of Toronto. Disclosures: None Claim free CME for enjoying this episode! Abstract • Pearls • Quotables • Notes • References • Guest Profiles • Production Team CardioNerds Cardio-Obstetrics Series PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Episode Abstract In this episode we discuss the important and challenging topic of pulmonary hypertension in pregnancy. We’ll start by discussing the prevalence of pulmonary hypertension in pregnancy, as well as the associated maternal morbidity and mortality associated with each WHO class. We will use a case to help us illustrate the appropriate workup for pulmonary hypertension patients and to help us broach the challenging topic of pregnancy termination. In this case we will further explore advanced management options including pulmonary vasodilators, anti-coagulation, and the use of mechanical support. Don’t miss this opportunity to hear Dr. Silversides’ share her wisdom on the importance of a multidisciplinary care team to plan both the delivery as well as post-partum care to help prevent adverse outcomes for both the mother and baby. Pearls Pregnancy in pulmonary hypertension, regardless of the class, is considered high risk. Even women who appear hemodynamically stable at baseline can easily decompensate in pregnancy, and thus the overall mortality and morbidity are very high. Due to the high risk of maternal morbidity and mortality during pregnancy for women with pulmonary arterial hypertension, the option of termination of pregnancy should be discussed.  Multidisciplinary care teams are the key to achieving optimal pregnancy outcomes in these patients. It is critical to create a team of experts with experience in pulmonary hypertension and plan for constant communication over the course of pregnancy. Pulmonary vasodilators including CCBs, phosphodiesterase inhibitors, and prostacyclin analogues should be initiated early to mitigate adverse outcomes. The majority of the complications in pulmonary hypertension patients occur after delivery, and so having a clear and safe postpartum plan is critical to a positive outcome. Quotables “We will someday identify the women who maternal morbidity and mortality is perhaps lower and we’ll be able to give a better, risk assessment. But we’re not quite there yet. And so currently, any woman who has pulmonary hypertension, true pulmonary hypertension in particular, pulmonary arterial hypertension, should be advised to avoid pregnancy.“ – Dr. Silversides “Women with PH can be falsely reassuring because they can walk in and look pretty good. And they’re young, you know, they’re not like the normal 70-year-old you might see on the ward. And so, you think they’re going to be okay, but they can spiral downward very quickly. So I do think you also have to have a very high, um, uh, level of. Uh, caution in these patients.“- Dr. Silversides on assessing PH patients in pregnancy  “I would tell you that I still think honesty is the best policy. I think you should offer women as much information as we currently know, so they can make informed decisions that are right for them. I think you also do have to really be sensitive to how you’re delivering this information, because remember (for) some women it will have never occurred to them that they can’t have a pregnancy. They may have been planning on having a kids and family and this information can really derail them. So you do have to use sensitivity, but I think you have to do it to accommodate to the patient that you’re seeing. I don’t think there can be a one size fits all approach.”- Dr. Silversides on the challenging topic of how to approach pregnancy termination conversations “… continue to optimize your care, the better shape the woman is going into delivery. The better outcomes you’ll have at the time of labor and delivery.”- Dr. Silversides Show notes 1. How do we define pulmonary hypertension (PH) and why is it such a big deal in pregnancy? According to recent guidelines pulmonary hypertension is a mean pulmonary artery pressure ≥ 20 mmHg. The WHO separates PH into 5 groups: Group 1: Pulmonary arterial hypertension (e.g., idiopathic, heritable [BMPR2], anorexigen associated, drug or toxin-associated, HIV, connective tissue disease associated, schistosomiasis, portal hypertension, congenital heart disease, amongst other causes) Group 2: Pulmonary hypertension due to left sided heart disease (e.g., HFrEF, HFpEF, left-side valvular heart disease) Group 3: Pulmonary hypertension due to lung disease or hypoxia: (e.g.,COPD, ILD, OSA, hypoxia without lung disease such as high altitude, developmental lung disorders) Group 4: PH due to pulmonary artery obstructions most commonly Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Group 5: Multifactorial causes such as hematologic disorders (chronic hemolytic anemia, as with myeloproliferative disorders), metabolic disorders (e.g., Gaucher disease, glycogen storage diseases, CKD), and systemic disorders (e.g., pulmonary Langerhans cell histiocytosis, neurofibromatosis, sarcoidosis) The prevalence of pulmonary hypertension and pregnancy is somewhere between 0.011 and 0.02%2. Although rare, this number has been rising due to the number of congenital patients living to childbearing age, as well as the emergence of effective pulmonary vascular therapy. Complications of PH during pregnancy include: The normal physiologic changes of pregnancy (increased plasma volume, increased stroke volume, increased cardiac output, decreased systemic vascular resistance), are poorly tolerated in PH due to an inability to decrease pulmonary vascular resistance and accommodate this increased plasma volume. This can lead to increased right ventricular overload. Decrease in systemic vascular resistance and associated drop in blood pressure can also lead to decreased RV perfusion, contributing to RV failure and making it increasingly difficult to accommodate the extra afterload demand. Pregnancy is both a prothrombotic and a pro-arrhythmic state, and maternal morbidity and mortality may also be related to complications from DVT/PE/arrhythmias, all of which are poorly tolerated by a failing RV with the increased afterload of PH and possible decreased perfusion from lower SVR. Abnormal maternal hemodynamics in PH also contribute to increased fetal and neonatal complications including preterm birth, fetal and neonatal death. 2. How does the severity of PH or the patient’s WHO group impact maternal outcomes? Mortality Overall mortality for PH patients during pregnancy is quite high: Two major systematic reviews covering a time span of 30 years in nearly 200 pregnancies cited total mortality to range between 25-38%. The majority of patients died within the first month after delivery and major causes of death were heart failure, sudden cardiac death and pulmonary embolism4,5   Morbidity A comprehensive study looking at approximately 1500 pregnant women with PH from the national inpatient sample (spanning from 2003-2012) found that the rate of major adverse cardiovascular events was around 24.8%.6 Karen et al assessed outcomes in ~150 pregnancies from the ROPAK study according to PH etiology (idiopathic PAH, PH due to congenital heart disease, PH due to left sided disease). Morbidity and mortality were highest in women with idiopathic PAH, and lowest in women with PH due to left sided heart disease. Complications were also higher in patient with severe PH (RVSP >70mmHg).7 Meng et al assessed 49 pregnancies from four large centers and found mortality to vary according to PH subgroup:  23% mortality in Group 1 patients, as compared to a 5% mortality in all other WHO groups. Similarly, patients with severe PH (RVSP >50mmHg) had a higher need for advanced therapies as compared to women with mild PH.1 Several variables determine risk during pregnancy related to PH, including the WHO group, etiology of PH, functional class, need for PAH medications at baseline, as well as cardiac size and function.  3. What is the recommended initial workup to help identify and risk stratify patients with PH? Helpful baseline information includes BNP, prior echocardiograms, as well as hemodynamics from prior right heart catheterizations. For those patients previously treated for PH, it is important to identify which medications were used in the past or are currently being prescribed. This particularly important to identify teratogenic medications like endothelin antagonists. One of the most important factors is to identify the patient’s functional status including assessing six-minute walk tests, including O2 saturation, desaturation, and distance walked. Of note, while a RHC helps define the hemodynamic profile, the entire vascular bed is more fragile during pregnancy than in a non-pregnant state, and there have been reports of pulmonary artery rupture with interventions during pregnancy.8 This must be kept in mind when assessing the need for RHC. 4. How do you approach management of patients with PH during pregnancy? Frequent follow-ups and communication with the entire multidisciplinary team are vital. This team should include cardiology, a PH specialist and/or team, high risk obstetrics, and OB anesthesia. Patients should be monitored serially throughout pregnancy with the use of BNP, echocardiography, and assessment of functional status/symptoms. While every patient situation will be different, it is important to provide the patient with as much information as possible to make an informed decision. Given the high-risk nature of pregnancy in these patients, as well as the increased maternal morbidity and mortality, it is important to discuss the option of termination of pregnancy. 5. What is the role of pulmonary vasodilators, and which medications are considered safe in pregnancy? Pulmonary vasodilators should be implemented early to mitigate risk of adverse outcomes as outlined above. Options include: Calcium channel blockers, phosphodiesterase inhibitors (sildenafil, tadalafil), and prostacyclin analogs (epoprostenol, treprostinil). NOTE: endothelin receptor antagonists (such as bosentan, ambrisentan) are teratogenic(Category X) and are contraindicated during pregnancy and in women of childbearing age who are not using reliable contraception. Pregnancy must be excluded before initiation, serially during treatment, and for a period after treatment has been discontinued. CCB/PDE5 inhibitors have been the most commonly reported medications in the series of PH patients during pregnancy. Prostacyclin analogs are considered safe during pregnancy, but their IV formulation may cause challenges in medication administration given the need for continuous administration. 6. How do you approach anticoagulation in PH patients during pregnancy? Pulmonary embolisms are one of the biggest contributors to morbidity and mortality in pulmonary hypertension patients as PE can acutely raise pulmonary artery, and therefore right sided pressures. Currently there are no clear recommendations for full dose anticoagulation in PH patients without risk factors for DVT/PE. Heparin is a large molecule that does not cross the placenta, and therefore considered safe in pregnancy. Typically, LMWH is used as it is considered superior to unfractionated heparin, especially in pregnancy. Other options such as warfarin are considered teratogenic (especially at does >5mg daily), and the class of DOACs have limited data on their safety in pregnancy and should be avoided. Initiation of anticoagulation should be coordinated in conjunction with obstetric hematologists. 7. What are some of the most important delivery considerations to keep in mind for these patients? Delivery timing and location: Consider delivering most patients by 37-weeks gestation (if not earlier) depending on the stability of the patient and fetus. While some women may follow at obstetric hospitals, delivery should take place in a location with backup of a cardiology, ICU, and advanced heart failure teams. Mode of delivery: IF women are stable, vaginal delivery- with an assisted second stage- is preferred as it is associated with fewer complications. Monitoring: In conjunction with OB, consider the use of arterial lines to assess blood pressure, central access for IV medication administration etc. Fetal monitoring is essential. Anesthesia: An epidural with good pain management is preferred, with avoidance of general anesthesia if possible (as GA is associated with greater complications in patients with PH). ECMO: If concerns for patient destabilization arise, VA-ECMO should be immediately available. Post-partum: The majority of complications occur after delivery, with the first post-partum week posing the highest risk period. Close monitoring must be initiated, preferably in a CCU/ICU for several days post-delivery. Early diuresis is paramount, as fluid mobilization after delivery can lead to fluid overload and right heart failure.  References 1. Meng ML, Landau R, Viktorsdottir O, et al. Pulmonary hypertension in pregnancy a report of 49 cases at four tertiary north American sites. Obstet Gynecol. Published online 2017. doi:10.1097/AOG.0000000000001896 2. Lima F V., Yang J, Xu J, Stergiopoulos K. National Trends and In-Hospital Outcomes in Pregnant Women With Heart Disease in the United States. Am J Cardiol. Published online 2017. doi:10.1016/j.amjcard.2017.02.003 3. Hemnes AR, Kiely DG, Cockrill BA, et al. Statement on pregnancy in pulmonary hypertension from the pulmonary vascular research institute. Pulm Circ. Published online 2015. doi:10.1086/682230 4. Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J. Published online 2009. doi:10.1093/eurheartj/ehn597 5. Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: A systematic overview from 1978 through 1996. J Am Coll Cardiol. Published online 1998. doi:10.1016/S0735-1097(98)00162-4 6. Thomas E, Yang J, Xu J, Lima F V., Stergiopoulos K. Pulmonary hypertension and pregnancy outcomes: Insights from the national inpatient sample. J Am Heart Assoc. Published online 2017. doi:10.1161/JAHA.117.006144 7. Sliwa K, van Hagen IM, Budts W, et al. Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology. Eur J Heart Fail. Published online 2016. doi:10.1002/ejhf.594 8. Barash PG, Nardi D, Hammond G, et al. Catheter-induced pulmonary artery perforation. Mechanisms, management, and modifications. J Thorac Cardiovasc Surg. Published online 1981. doi:10.1016/s0022-5223(19)39380-8 9. Marc Humbert, Olivier Sitbon GS. Hypertension, Treatment of Pulmonary Arterial. N Engl J Med. 2004;351:1425-1436. Two additional excellent sources regarding Pregnancy in PH Patients: Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease. J Am Coll Cardiol. Published online 2019. Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy. Eur Heart J. Published online 2011. doi:10.1093/eurheartj/ehr218 Guest Profiles Dr. Candice Silversides Dr. Candice Silversides is an Associate Professor of Medicine at the University of Toronto.  She serves as the Director of the Pregnancy and Heart Disease program and head of the Obstetric Medicine program. Along with her >180 publications and numerous book chapters, she has contributed to a number of international practice guidelines and consensus statements, and she also helped develop the CARPREG II cardiovascular risk prediction score. Dr. Kayle Shapero Dr. Kayle Shapero is a first year cardiology fellow at the University of Pittsburgh Medical Center. She began her undergraduate training in chemical engineering and completed a PhD in biomedical engineering. She spent her PhD studying tissue engineered heart valves which ultimately piqued her interest in medicine. She went on to complete her medical degree at Tufts Medical School followed by internal medicine residency at Yale. She is currently in her first year of fellowship at UPMC. She is interested cardio-obstetrics and sports cardiology, and is always looking for ways to combine these two clinical and research interests. Outside of the hospital she attempts to keep in shape by training for triathlons and marathons. CardioNerds Cardioobstetrics Production Team Natalie Stokes, MD Sonia Shah, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerd (Amit Goyal), cardioobstetrics series co-chair Dr. Natalie Stokes, Cardionerds Duke University CardioNerds Ambassador and episode lead fellow, Dr. Kelly Arps, join Dr. Andrea Russo, Director of Electrophysiology and Arrhythmia Services at Cooper Medical School of Rowan University and immediate past president Heart Rhythm Society, for a discussion about pregnancy and arrhythmia. Stay tuned for a message from Dr. Sharonne Hayes about WomenHeart. Audio editing by Gurleen Kaur. Claim free CME for enjoying this episode! Dr. Russo’s disclosures: Johnson and Johnson, Medtronic, Inc., Boston Scientific Corporation, Kestra, Medilynx, Up-to-Date, and ABIM. Abstract • Pearls Notes • References • Guest Profiles • Production Team CardioNerds Cardio-Obstetrics Series PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Episode Abstract Pregnant patients may have exacerbation of underlying arrhythmic syndromes or unmasking of previously undiagnosed arrhythmic syndromes. Management of atrial and ventricular tachyarrhythmias should proceed with increased urgency in pregnant patients due to risk of adverse hemodynamic events in the mother and fetus. Cardioversion of atrial and ventricular arrhythmias is safe in pregnancy. Preferred antiarrhythmic agents in pregnant patients include metoprolol, propranolol, verapamil, flecainide, propafenone, sotalol, procainamide, and lidocaine. Management of arrhythmias in pregnancy should include collaboration with obstetrics and maternal-fetal medicine teams. Pearls Pre-conception counseling is a shared decision making process; include obstetrics and maternal-fetal medicine colleagues in challenging cases. Have a high sense of urgency for acute arrhythmias in pregnancy due to risk of impaired fetal perfusion. Goals of acute arrhythmic management should include rapid treatment while avoiding hypotension. In scenarios when beta blockers are indicated, metoprolol and propranolol are first choice. Avoid atenolol as this drug has the highest risk of fetal bradycardia and intra-uterine growth retardation in the class. Lidocaine or procainamide should be first line for ventricular arrhythmias in pregnancy. Amiodarone is potentially teratogenic and should not be used in pregnant patients unless all other options have been exhausted. Show notes 1. What are the expected electrophysiologic changes associated with pregnancy? Increase in resting heart rate which peaks in third trimester PR shortening ECG axis shift leftward and upward Non-specific ST and T wave changes These changes, along with increased cardiac output and volume with increased stretch in all chambers, increase the risk of re-entrant arrhythmias in those who are predisposed. ↑ atrial volume -> ↑ stretch -> ↑ ectopy -> ↑ risk for re-entrant arrhythmias 2. What is the approach to pre-conception counseling for patients with known arrhythmias or arrhythmic syndromes? Anticipate frequency and potential severity of adverse arrhythmic outcomes during pregnancy and post-partum period Consider available options for rhythm control and anticoagulation therapy, as appropriate, during the pre-conception, pregnancy, and post-partum periods Consider catheter ablation prior to pregnancy, particularly for curable arrhythmias such as Wolff-Parkinson-White (WPW) and AVNRT    Offer genetic counseling about hereditary risk to fetus for inherited arrhythmias such as Brugada syndrome and Long QT syndrome 3. What is the management of SVT in pregnancy? Consider the increased risk of tachyarrhythmias in pregnancy: Typically benign arrhythmias can lead to more rapid decompensation in mother due to increased baseline cardiac output. Typically benign arrhythmias can lead to rapid danger to the fetus due to maternal hypotension and shortened diastolic filling time, both of which contribute to impaired fetal perfusion.  Treatment algorithm is identical to that of non-pregnant patients Attempt vagal maneuvers Adenosine is safe Cardioversion is safe: monitor the fetus during and after cardioversion In stable arrhythmias, choose nodal blocking agents with the best safety profile: metoprolol, propranolol, and verapamil. Evaluation of the pregnant patient with new onset SVT Have a high index of suspicion for underlying structural heart disease such as peripartum cardiomyopathy in a pregnant women with new diagnosis of SVT – presence of structural heart disease significantly increases the risk of maternal morbidity and mortality. Pregnancy can be the first presentation of inherited arrhythmia syndromes that commonly present in young adults such as WPW, Brugada Syndrome, Catecholaminergic Polymorphic VT (CPVT), Long QT Syndrome (LQTS), Arrhythmogenic Right Ventricular Cardiomyopathy / Dysplasia (ARVC/D), and Hypertrophic Cardiomyopathy (HCM).       4. What are some special considerations for acute management of VT in pregnancy? Cardioversion is safe. First line pharmacologic therapy: lidocaine or procainamide Lidocaine has been associated fetal bradycardia but has been used safely without reported teratogenic effect Brugada syndrome: consider quinidine in Brugada syndrome Fascicular VT: use verapamil Only use amiodarone if absolutely necessary, and after the first trimester 5. What is the approach to chronic arrhythmia management in pregnancy? Preferred rate control agents: Metoprolol Propranolol Digoxin Verapamil AVOID: atenolol (increased risk of fetal bradycardia and intrauterine growth restriction; note that this risk is present with all beta blockers*) Preferred rhythm control agents: Flecainide (if no structural heart disease) Propafenone (if no structural heart disease) Sotalol Lidocaine Procainamide Quinidine AVOID: amiodarone; use only in a patient with refractory unstable arrhythmias after the first trimester (due to fetal thyroid and neurodevelopmental issues) AVOID: dronedarone; Category X in pregnancy Catheter ablation in the pregnant patient Best delayed until late in pregnancy or after delivery Maternal-fetal medicine colleagues should be involved in procedural planning Minimize fluoroscopic time Shield the pelvis during fluoroscopy and use electroanatomic mapping *Surveillance for pregnant patients on beta blockers: Serial growth ultrasounds in the third trimester Antenatal testing of for bradycardia and hypoglycemia Postnatal monitoring for:  Bradycardia Apnea Growth retardation 6. What is the approach to antiarrhythmic therapy in the breastfeeding patient? All antiarrhythmic drugs are passed into breast milk Preferred rate control agents: metoprolol, propranolol (watch for fetal bradycardia) Rhythm control agents: weigh risks and benefits; read dosing adjustments on prescribing instructions carefully AVOID: atenololAVOID amiodaroneAVOID: dronedarone 7. What is the approach to anticoagulation in pregnancy and breastfeeding? Use the CHAD2S2-VASc score to estimate stroke risk for pregnant patients with AF and AL Risk of stroke with AF and AFL in pregnancy are uncertain, as women of childbearing age were minimally represented in large studies evaluating prophylactic antithrombotic drug treatment. Pregnancy Low molecular weight heparin is preferred in the first trimester and around the time of delivery. Warfarin should be avoided during the first trimester (especially at doses >5 mg daily), but may be used in the second and beginning of the third trimester. Avoid DOACs Breastfeeding Use warfarin or LMWH AVOID: DOACs may be excreted in breast milk and should not be used during breast feeding. 8. What is the approach to specific arrhythmic syndromes? AVNRT: recommend catheter ablation prior to conception if prior diagnosis. Manage acute events if they occur during pregnancy. WPW: recommend catheter ablation prior to conception if prior diagnosis. Use procainamide for acute arrhythmic events and avoid nodal blocking agents. LQTS:recommend beta blockers (metoprolol or propranolol) through pregnancy and at least through the post -partum period CPVT: recommend beta blockers (metoprolol or propranolol) through pregnancy and at least through the post-partum period 9. What is the approach to management of cardiac arrest in the pregnant patient? ACLS should be performed per ACLS guidelines, including chest compressions and defibrillation. Positioning: aim to avoid IVC compression and impaired venous return to the heart in the supine pregnant patient. Patients with a pulse: left lateral decubitus No pulse: Manually displace the uterus to the left All patients: place IVs above the diaphragm Be prepared for difficult airway in mother due to airway edema Call OB and neonatal teams immediately to determine need for emergency C-section if no ROSC within the first several minutes. References Lindley KJ, Judge N. Arrhythmias in Pregnancy. Clin Obstet Gynecol. 2020;63(4):878-892. doi:10.1097/GRF.0000000000000567 Vaidya VR, Arora S, Patel N, et al. Burden of Arrhythmia in Pregnancy. Circulation. 2017;135(6):619-621. doi:10.1161/CIRCULATIONAHA.116.026681 Seth R, Moss AJ, McNitt S, et al. Long QT syndrome and pregnancy. J Am Coll Cardiol. 2007;49(10):1092-1098. doi:10.1016/j.jacc.2006.09.054 Hodes AR, Tichnell C, Te Riele AS, et al. Pregnancy course and outcomes in women with arrhythmogenic right ventricular cardiomyopathy. Heart. 2016;102(4):303-312. doi:10.1136/heartjnl-2015-308624 European Society of Gynecology (ESG); Association for European Paediatric Cardiology (AEPC); German Society for Gender Medicine (DGesGM), et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart J. 2011;32(24):3147-3197. doi:10.1093/eurheartj/ehr218 Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society [published correction appears in J Am Coll Cardiol. 2018 Oct 2;72(14):1760]. J Am Coll Cardiol. 2018;72(14):e91-e220. doi:10.1016/j.jacc.2017.10.054 Page RL, Joglar JA, Caldwell MA, et al. 2015 ACC/AHA/HRS Guideline for the Management of Adult Patients With Supraventricular Tachycardia: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society [published correction appears in Circulation. 2016 Sep 13;134(11):e232-3]. Circulation. 2016;133(14):e471-e505. doi:10.1161/CIR.0000000000000310 January CT, Wann LS, Alpert JS, et al. 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society [published correction appears in Circulation. 2014 Dec 2;130(23):e272-4]. Circulation. 2014;130(23):e199-e267. doi:10.1161/CIR.0000000000000041 Guest Profiles Dr. Andrea Russo Dr. Andrea Russo is Professor of Medicine at Cooper Medical School of Rowan University, where she is also Director of the Clinical Cardiac Electrophysiology Fellowship Program, as well as Director of Electrophysiology and Arrhythmia Services at Cooper University Hospital and Director of Research at Cooper Heart Institute. She is the immediate past president of the Heart Rhythm Society. She’s also had numerous leadership positions in HRS, the American College of Cardiology, American Heart Association, American Board of Internal Medicine, National Quality Forum, and the American Board of Medical Specialties. Dr. Russo is currently serving as a member of the ACC Cardiovascular Disease in Women Committee and the ABIM Cardiovascular Board. She’s written extensively in the field of electrophysiology, with a focus on sex differences in arrhythmias, ICDs and subcutaneous cardiac devices, atrial fibrillation, digital health, and performance improvement activities. She has been on the steering committee for trials including UNTOUCHED and the Apple Heart Study and contributed to multiple guideline and consensus statements including as first author of the 2013 multisociety Appropriate Use Criteria for implantable cardioverter-defibrillators and cardiac resynchronization therapy and senior author of the ACC / AHA Guidance for Cardiac Electrophysiology During the COVID-19 Pandemic. In addition to her academic achievements, Dr. Russo has been recognized many times as a “Top Doctor” in Philadelphia and Southern New Jersey. Dr. Agnes Koczo Kelly completed medical school at Emory University and residency at Johns Hopkins Hospital, where she served as editor-in-chief for the 2018 Osler Medicine Survival Guide. She is currently is a clinical cardiology fellow at Duke University and served on the 2019-2020 ACC FIT website editorial board in the education section. Kelly is planning to pursue a career in cardiac electrophysiology with a focus on ventricular arrhythmias in heart failure and infiltrative cardiomyopathies. CardioNerds Cardioobstetrics Production Team Natalie Stokes, MD Sonia Shah, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerd (Amit Goyal), Narratives in Cardiology FIT representative Dr. Zarina Sharalaya and Cleveland Clinic fellow Dr. Gregory Ogunnowo join Dr. Quinn Capers IV, UTSW as Professor of Medicine, Associate Dean of Faculty Diversity, and the inaugural Vice Chair of Diversity, Equity, and Inclusion in the Department of Internal Medicine, for an important and moving discussion about diversity, implicit bias, and #BlackMenInMedicine. Special thanks to Dr. Kimberly Manning for her introductory remarks for Dr. Capers. Audio editing by CardioNerds Academy Intern, Dr. Maryam Barkhordarian. Claim free CME just for enjoying this episode! Click here to see Dr. Caper’s tweet regarding his daughter’s original peice Cardionerds Narratives in Cardiology PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll Subscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! https://twitter.com/DrQuinnCapers4/status/1277715623246733317?s=20 CardioNerds Narratives in Cardiology The CardioNerds Narratives in Cardiology series features cardiovascular faculty representing diverse backgrounds, subspecialties, career stages, and career paths. Discussing why these faculty chose careers in cardiology and their passion for their work are essential components to inspiring interest in the field. Each talk will feature a cardiology faculty from an underrepresented group, within at least one of several domains: gender, race, ethnicity, religion, national origin, international graduate status, disadvantaged backgrounds, etc. Featured faculty will also represent a variety of practice settings, academic ranks, subspecialties (e.g. clinical cardiology, interventional cardiology, electrophysiology, etc), and career paths (e.g. division chief, journal editor, society leadership, industry consultant, etc). Faculty will be interviewed by fellows-in-training for a two-part discussion that will focus on: 1) Faculty’s content area of expertise2) Faculty’s personal and professional narrative As part of their narrative, faculty  will discuss their unique path to cardiology and their current professional role with particular attention to challenges, successes, and advice for junior trainees. Specific topics will be guided by values relevant to trainees, including issues related to mentorship, work-life integration, and family planning. To help guide this important initiative, the CardioNerds Narratives Council was founded to provide mentorship and guidance in producing the Narratives series with regards to guests and content. The CardioNerds Narratives Council members include: Dr. Pamela Douglas, Dr. Nosheen Reza, Dr. Martha Gulati, Dr. Quinn Capers, IV, Dr. Ann Marie Navar, Dr. Ki Park, Dr. Bob Harrington, Dr. Sharonne Hayes, and Dr. Michelle Albert. The Narratives Council includes three FIT advisors who will lead the CardioNerds’ diversity and inclusion efforts, including the current project: Dr. Zarina Sharalaya, Dr. Norrisa Haynes, and Dr. Pablo Sanchez. Guest Profiles – Physician Scientists Women Electrophysiology Dr. Quinn Capers, IV Dr. Quinn Capers, IV grew up in Dayton Ohio and left his hometown to do his undergraduate training at Howard University. He began his journey in medicine at The Ohio State University and went on to do residency, cardiology fellowship, and interventional cardiology training at Emory University. After graduation he worked for 8 years in private practice, and made the switch back to academics and came back to Ohio State to continue his career.  In 2009, he was named associate dean of admissions and in 10 years, the College of Medicine went from 13%  underrepresented minorities to 26% of the 2019 entering class, and in the last 6 years women have outnumbered men in the incoming classes. In 2019 he was promoted to Vice Dean for Faculty Affairs, received the award for professor of the year, and the Diversity Champion Award from the institution. Most recently, the state of Texas gained a gem as Dr. Capers has moved to join the UTSW as Professor of Medicine, Associate Dean of Faculty Diversity, and the inaugural Vice Chair of Diversity, Equity, and Inclusion in the Department of Internal Medicine. He has an expansive list of accolades and awards. Dr. Capers was awarded the AHA Laennec Clinician Educator Award in 2018. He was recognized as the 2020 recipient of the Exemplary Leadership Award of the Group on Diversity and Inclusion from the Association of American Medical Colleges (AAMC). He is an inaugural member of the American College of Cardiology’s Diversity and Inclusion Task Force. In 2021 he received the Pamela S. Douglas Distinguished Award for Leadership in Diversity and Inclusion. He has had an impactful presence on social media where he created the hashtags #BlackMenInMedicine and #TakeAWomanToTheCathLab. Dr. Capers is a passionate advocate for enhancing diversity and inclusion, a champion for improving health equity, and a devoted mentor to countless trainees. Dr. Zarina Sharalaya Dr. Zarina Sharalaya is an interventional cardiology fellow at the Cleveland Clinic. She completed medical school at The Ohio State University and then completed her residency at The University of North Carolina Chapel Hill. She moved back to her home state of Ohio to do general cardiology fellowship at The Cleveland Clinic. Zarina has been very involved with the Ohio ACC and this year has served as co-chair of the FIT Council. She is passionate about the Women in Cardiology initiative has been able to help formulate the first WIC chapter for Ohio ACC. She enjoys traveling, music, and spending time with her husband and new puppy Zuma. Dr. Greg Ogunnowo Dr. Gregory Ogunnowo (Dr_GregoryO) is a cardiology fellow at the Cleveland Clinic. He completed medical school at the University of South Carolina School of Medicine in Columbia, South Carolina. He went on to complete internal medicine residency at Washington University School of Medicine in St. Louis where he stayed on as faculty in the Department of Hospital Medicine for a year prior to pursing fellowship. His interests include outcomes research in interventional cardiology and medical education In his spare time, Greg enjoys traveling, exercising, and experiencing new cultures through their food. When he’s not in the hospital, you can find Greg planning a trip with close friends and family. References Ellis J, Otugo O, Landry A, Landry A. Interviewed while Black. N Engl J Med. 2020 Dec 17;383(25):2401-2404. doi: 10.1056/NEJMp2023999. Epub 2020 Nov 11. PMID: 33176078. Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder) join Dr. Rayan Jo Rachwan, Dr. Anupama Joseph, and Dr. Mohammed Merchant from the University of Wisconsin-Madison for a classic Madison dinner cruise! They discuss the following case: Mixed shock secondary to severe right ventricular outflow tract obstruction with Gemella Haemolysans prosthetic pulmonary valve endocarditis in a young patient with Shone Complex (syndrome). Dr. Ford Ballantyne III provides the E-CPR segment for this episode. Special introductory music composed by Dr. Rayan Jo Rachwan. We are excited to welcome University of Wisconsin- Madison to the CardioNerds Healy Honor Roll and Dr. Rayan Jo Rachwan as the CardioNerds Ambassador. Claim free CME just for enjoying this episode! Jump to: Patient summary – Case media – Case teaching – References CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Patient Summary A 26-year-old male with history of bicuspid aortic valve and Shone Complex (syndrome)—status post coarctation repair, subaortic resection and Ross-Konno operation—presenting with 3 months of constitutional and respiratory symptoms. Initial evaluation demonstrated that patient was in a state of mixed shock due to a large pulmonary Melody valve thrombus with superimposed Gemella Haemolysans prosthetic valve endocarditis. He required treatment with inotropes, pressors, followed by intubation and extracorporeal membrane oxygenation (ECMO). Patient was treated initially via right heart catheterization with balloon dilation and stent placement to his right ventricle-to-pulmonary artery conduit, which lead to significant improvement in his hemodynamics. Patient was then decannulated from ECMO, extubated, weaned off pressor support and later underwent a successful surgical resection of the infected pulmonary homograft and Melody valve/stents and replacement with pulmonary-valved conduit. He was also discharged on a prolonged course of antibiotics. Case Media – Shone Complex A B C DClick to Enlarge A. CXR, B. ECG, C. TV Doppler, D. PV Doppler CTA Chest •Melody pulmonic valve repair with large thrombus arising from the mid-distal valve extending into the main pulmonary artery and proximal left pulmonary artery. Evaluation for distal subsegmental pulmonary emboli is limited. •Tiny focus of air in the thrombus may be related to contrast injection. Infection is less likely. •Enlarged right heart chambers, may be in part chronic right heart enlargement and/or new right heart strain. No pulmonary infarct. CT chest abdomen and pelvis with contrast •Findings suggestive of acute hepatitis and acute pancreatitis. No pancreatic hypoenhancement or peripancreatic fluid collection. •No abscess within the abdomen or pelvis. •Small caliber of the infrarenal abdominal aorta and bilateral iliac arteries, probably congenital.  TTE 1 TTE 2 TTE 3 TTE 4 TTE 5 TTE 6 RHC with balloon dilation of the RV-PA conduit and evidence of multiple levels of stent fracture. Pulmonary angiogram showing no evidence of distal embolization or significant pulmonary embolism and no evidence of perforation. There is evidence of moderate pulmonary insufficiency into a dilated right ventricle. Episode Teaching – Shone Complex Pearls 1. Patients with congenital heart disease are more predisposed to infective endocarditis (IE). Therefore, there should be a low-threshold for infectious workup in the case of unexplained fever or malaise without associated symptoms for >72 hours. Every routine visit should screen for symptoms and signs of IE. 2. Treatment of right ventricular (RV) outflow tract obstruction with balloon dilation +/- stenting can be considered as a bridge to valve replacement in the case of severe hemodynamic compromise; thus, restoring RV and pulmonary artery coupling. As with many complex decisions this should be made in consultation with an experience heart team and shared decision making with the patient or proxy. 3. Patients with RV volume and/or pressure overload from right-sided valve etiology should be assessed serially (i.e., yearly) with transthoracic echocardiography. 4. When precise quantitative data about the RV is required to make important clinical decisions (e.g., when to recommend pulmonary valve replacement), cardiac magnetic resonance imaging (CMR) remains the diagnostic modality of choice. 5. Repairing or replacing the pulmonary valve should be considered when RV end-diastolic volume >160 mL/m2 and/or RV end-systolic volume >80 mL/m2 on CMR. Notes What is Shone syndrome? Shone syndrome (a.k.a. Shone anomaly, Shone complex) is a rare congenital abnormality that accounts for 0.6% of all congenital abnormalities. It is characterized by a series of left-sided obstructive lesions. The diagnosis is made with the presence of at least 3 of 8 described lesions. The 8 described lesions are: Cor Triatriatum Supramitral ring Parachute mitral valve Subaortic stenosis Bicuspid aortic valve and small aortic valve annulus Coarctation of the aorta Hypoplastic (stiff) left ventricle. Small aortic arch Supravalvular mitral ring, parachute mitral valve, subaortic stenosis, and coarctation of the aorta were the first four described lesions and constitute the classic constellation of Shone complex. Incomplete forms involve an LV inflow lesion plus at least one LV outflow lesion. Frequently, coarctation of the aorta is recognized before the other defects are detected. The coarctation may mask the effects of the other lesions and some patients with Shone syndrome are only diagnosed when symptoms persist after coarctation repair. How does Shone syndrome manifest? The symptoms associated with Shone syndrome are mostly symptoms of congestive heart failure (which can occur in the first week of life), potentially presenting in early childhood as fatigue, rapid breathing and wheezing, faster than normal heart rate, poor oral intake, poor weight gain, fluid retention (edema) in the legs, pallor (anemia), and frequent pneumonias. In a series of 28 adult patients with Shone syndrome followed for a median of 8 years (Aslam et al., CJC 2016), nearly half had cardiovascular hospitalizations during adulthood, mostly for arrhythmias or heart failure. The severity and prognosis depend on the individual lesions involved and the degree of obstruction to flow they cause. How is Shone syndrome diagnosed? Diagnosis involved multimodality imaging predominantly with TTE, as well as TEE, CMR, and/or cardiac CTA as useful adjuncts depending on the lesions and patient age. Invasive hemodynamics and angiography are important adjuncts, particularly while planning repair. How is Shone syndrome treated? It is treated by addressing each of the constituent defects. For example: Coarctation of the aorta:Treated surgically with excision with end-to-end anastomosis or subclavian flap angioplasty. Alternatively, it can be treated with transcatheter balloon angioplasty, particularly in the case of re-coarctation after surgical repair. Subaortic stenosis:Treated surgically by excising the excess tissue below the aortic valve. If other forms of aortic stenosis are present, surgical repair may involve the replacement of the aortic valve. Mitral stenosis (caused by “parachute” mitral valve and by supravalvular mitral membrane):Treated by surgery (valve replacement vs. valve repair). What is the prognosis of patients with Shone syndrome? The long-term prognosis for patients with Shone syndrome is difficult to predict and is extremely variable depending on the lesions involved and degree of obstruction. It depends on the extent of mitral valve disease, the degree to which the left ventricle is hypoplastic, and the cumulative effects of surgical treatments. Moreover, patients who develop pulmonary arterial hypertension (PAH) have a poorer prognosis. Early surgical intervention is important to prevent the adverse consequences of long-standing obstruction and ensuing PAH. How are patients with Shone syndrome followed-up? With regard to right ventricular (RV) assessment, echocardiography (more widely available) provides useful diagnostic information in many clinical circumstances that affect the right heart. However, when precise quantitative data is required to make important clinical decisions (e.g., when to recommend pulmonary valve replacement), cardiac magnetic resonance imaging (CMR) remains the diagnostic modality of choice. Repairing or replacing a dysfunctional pulmonary valve should be considered when RV end-diastolic volume (RVEDV) >160 mL/m2 and/or RV end-systolic volume (RVESV) >80 mL/m2 on CMR. RV normalization could be achieved with pulmonary valve replacement when preoperative RVEDV is ≤160 mL/m2 or RVESV is ≤80 mL/m2 on CMR. What is the Melody valve and what is it used for? The Melody valve consists of bovine jugular vein sewn within a platinum-iridium stent. Transcatheter pulmonary valve placement with the Melody valve is effective in the short term for relief of right ventricular outflow tract (RVOT) obstruction and pulmonary regurgitation in patients with surgically implanted right ventricle–to–pulmonary artery conduits. Melody stent fracture (MSF) with valve dysfunction is the most common indication for reintervention after Melody valve placement. MSF is more likely in patients with severely obstructed RVOT conduits and when the Melody valve is directly behind the anterior chest wall and/or clearly compressed. Pre-stenting of the conduit before valve implantation improves the durability of the implanted valve. Congenital heart disease (CHD) and infective endocarditis (IE)? The risk of infective endocarditis (IE) remains a major concern in patients with congenital heart disease (CHD), whether unrepaired, palliated, or corrected. The overall incidence of endocarditis in adults with CHD is 11 per 100 000 person-years (vs. 1.5 to 6.0 per 100 000 patient-years in the general population). The increased survival of children with CHD and the use of conduits and prostheses in corrective surgery may have contributed to an increasing incidence of IE. What is Gemella haemolysans bacteria? Gemella haemolysans is a Gram-positive coccoid, catalase-negative, facultative anaerobic microorganism of the mucus membranes in humans. It is able to cause severe and generalized infection as opportunistic pathogens, and it has become an emerging bacterial etiology in IE. Generally, Gemella endocarditis is associated with previous valvular damage or a poor dental state. References Nicholson, George T., et al. “Late outcomes in children with Shone’s complex: a single-centre, 20-year experience.” Cardiology in the Young 27.4 (2017): 697. Delaney, Jeffrey W., et al. “Covered CP stent for treatment of right ventricular conduit injury during melody transcatheter pulmonary valve replacement: results from the PARCS study.” Circulation: Cardiovascular Interventions 11.10 (2018): e006598. McElhinney, Doff B., et al. “Stent fracture, valve dysfunction, and right ventricular outflow tract reintervention after transcatheter pulmonary valve implantation: patient-related and procedural risk factors in the US Melody Valve Trial.” Circulation: Cardiovascular Interventions 4.6 (2011): 602-614. Schneider, Adriaan W., et al. “Twenty-year experience with the Ross–Konno procedure.” European Journal of Cardio-Thoracic Surgery 49.6 (2016): 1564-1570. Brown, John W., et al. “The Ross-Konno procedure in children: outcomes, autograft and allograft function, and reoperations.” The Annals of thoracic surgery 82.4 (2006): 1301-1306. Mulder, Barbara JM. “Endocarditis in congenital heart disease: who is at highest risk?.” (2013): 1396-1397. Bokma, Jouke P., et al. “Preoperative thresholds for mid-to-late haemodynamic and clinical outcomes after pulmonary valve replacement in tetralogy of Fallot.” European heart journal 37.10 (2016): 829-835. Aslam S, Khairy P, Shohoudi A, Mercier LA, Dore A, Marcotte F, Miró J, Avila-Alonso P, Ibrahim R, Asgar A, Poirier N, Mongeon FP. Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood. Can J Cardiol. 2017 Feb;33(2):253-259. doi: 10.1016/j.cjca.2016.09.005. Epub 2016 Sep 29. PMID: 27956040. CardioNerds Case Report Production Team Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal, Daniel Ambinder, Carine Hamo, and Karan Desai) are honored to bring to you the Braunwald Chronicles. These are stories of discovery, innovation, accidents, perseverance, and more…truly these are the stories of cardiology, directly from a father of modern cardiology himself, Dr. Eugene Braunwald. Dr. Braunwald’s life and stories together are the saga which have brought us to this day in modern cardiology. So please join us for this wonderful series, as we journey through the history of cardiology, across 6 extraordinary chapters. We complete The CardioNerds Braunwald Chronicles with Chapter 6 where Dr. Braunwald discusses triple threats, randomized controlled trials, textbooks & digital education. He reflects on the impact he has had through education through text books and how being an educator has been just as gratifying to him as being a scientist. We thank Dr. Karan Desai, Editorial APD with the CardioNerds Academy, and fellow at University of Maryland, for all the work he put into designing the Braunwald Chronicles. Audio editing by Pace Wetstein. CardioNerds Braunwald Chronicles Series PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron!