Cardionerds: A Cardiology Podcast

CardioNerds (Amit Goyal & Daniel Ambinder) join join Mayo Clinic cardiology fellows (Mays Ali, Charlie Jain, Korosh Sharain) for a scenic walk through gorgeous Rochester, Minnesota! They discuss a fascinating case of constrictive pericarditis and severe mitral regurgitation. Dr. Rick Nishimura provides the E-CPR and program director Dr. Frank Brozovich provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Bibin Varghese with mentorship from University of Maryland cardiology fellow Karan Desai.   Jump to: Patient summary – Case figures & media – Case teaching – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Constrictive Pericarditis & Severe Mitral Regurgitation – Patient Summary A woman in her late 40s with a history of lupus and hypertension presented with worsening dyspnea on exertion and orthopnea over a year. She reported intermittent pleuritic chest discomfort that had persisted since an episode of acute pericarditis years prior. A TTE suggested severe mitral regurgitation, and she was referred to the Mayo Clinic for mitral valve intervention.    The official TTE report from the OSH suggested non-dilated LV, EF 55-60%, normal RV function, severe MR with thickened leaflets and sub-valvular apparatus, moderate to severe TR and a dilated IVC. Furthermore, the CXR showed pericardial calcifications. Upon evaluation by the Mayo Clinic fellows, the JVP was elevated to about 10-12 cm with rapid x and y descents, a positive Kussmaul’s sign, and the murmurs of MR and TR. Her lungs were clear to auscultation and extremities did not demonstrate edema. Re-review of the TTE images revealed posterior pericardial thickening, no septal shift on respiration, but suggestion of annulus reversus where medial mitral annulus tissue doppler (9 cm/s) was greater than lateral (8 cm/s). Further, there was evidence of expiratory hepatic vein diastolic flow reversal.  For the team, there was discordance between the apparent severity of her MR reported by echocardiogram and her clinical symptoms. In addition, the echocardiogram was suggestive of specific signs of constrictive pericarditis. Thus, simultaneous RHC/LHC was obtained. There was equalization of RV/LV pressures during diastole, demonstration of a “square root sign” and importantly discordance between LV and RV pressures with respiration. Thus, discordant clinical findings led to a suspicion for constrictive pericarditis and was corroborated by discordance on invasive hemodynamics! Further, the V-waves were not prominent on wedge pressure tracing and to investigate the mitral regurgitation further, an LV ventriculogram was done. This demonstrated 3+ to 4+ MR.  Based on all the findings, the patient was diagnosed with constrictive pericarditis, severe MR and moderate to severe TR. She underwent pericardiectomy, mitral valve replacement (given that repair was not feasible due to the sub-valvular thickening) and given that TR has been shown to worsen after pericardiectomy and is a poor prognostic factor, she additionally underwent tricuspid valve repair. Her symptoms improved markedly following intervention.  Case Media A B C D E F G H Click to Enlarge A. CXR: Heart size was borderline enlarged with biatrial enlargement.  LV does not appear very enlarged. B. Mitral Regurgitation by CW Doppler C. Tricuspid regurgitation by CW Doppler. TR Max 2.43D. Tissue Doppler of the mitral valve annulus: Medial e’ = 9 cm/s, Lateral e’ 8 cm/sE. Hepatic Vein PW Doppler F. Right atrial pressure tracingG. RV and LV simultaneous pressure tracings H. Wedge pressure and LV simultaneous pressure tracings Neck Vein Exam TTE TTE: Color Doppler across mitral valve TTE: Short Axis TTE: Short Axis, TV TTE: Hepatic Vein Doppler Left ventriculogram with severe mitral regurgitation Episode Schematics & Teaching Click to enlarge! The CardioNerds 5! – 5 major takeaways from the #CNCR case- Constrictive Pericarditis & Severe Mitral Regurgitation One of the early clues to the etiology of this patient’s dyspnea was a positive Kussmaul’s sign. What is the mechanism and differential of Kussmaul’s Sign?   Typically the JVP decreases with inspiration as the negative intrathoracic pressure “sucks in” blood from the vena cavae. When there is a lack of decrease or an increase in JVP with inspiration, this finding is called Kussmaul’s sign. Kussmaul’s sign reflects conditions where there is right ventricular dysfunction, impaired RV filling and elevated right atrial pressure    Kussmaul’s sign is classically associated with constrictive pericarditis. Remember, during normal inspiration, the diaphragm contracts and increases abdominal pressure with variable effect on venous return. However, in constriction, the rise in abdominal pressure increases venous return from the congested hepato-splanchnic vasculature. And since RV filling is constrained by a non-compliant pericardium, there is a rise in JVP during inspiration.    Other conditions where we may see a positive Kussmaul’s sign include restrictive cardiomyopathy, RV predominant infarction, massive pulmonary embolism, tricuspid stenosis, and severe tricuspid regurgitation.  2. A decision was made to pursue invasive hemodynamic evaluation to differentiate between restrictive and constrictive physiology. How do we differentiate between the two on echocardiogram?   Differentiating between the two diagnoses requires an understanding of the pathophysiologic differences between constriction and restriction.  Enjoy Ep #58 and CN5 for understanding constrictive physiology and echocardiographic findings! In restriction we do not have intrathoracic-intracardiac disassociation as intrathoracic pressures are transmitted normally to the cardiac chambers during respiration. Further, there is no exaggerated interventricular dependence.    We may see increased myocardial thickness in restriction (secondary to infiltrative or storage disorders – enjoy Ep #50 – restrictive cardiomyopathy) and increased pericardial thickness/calcification in constriction.    Unlike constrictive pericarditis, restrictive cardiomyopathies should not demonstrate respirophasic septal shift or variation across the atrioventricular valves. Both conditions will demonstrate hepatic vein diastolic flow reversal. However, in constriction, the exaggerated ventricular interdependence and greater LV filling during expiration causes septal bowing into the RV and results in greater hepatic vein diastolic flow reversal during expiration. In restriction, hepatic vein flow reversal is more prominent in inspiration as the stiff myocardium is unable to tolerate the increased RV preload that occurs during inspiration.    The estimated pulmonary artery systolic pressures tend to be elevated (>55-60 mmHg) in restrictive cardiomyopathy and are often normal in constriction. In constriction, the annular tissue velocity is preserved except at the lateral annulus, where tethering of the lateral wall results in decreased velocities and annulus reversus (See Ep#58 and CN5). Meanwhile, annular tissue velocity (e’) is significantly reduced in restrictive cardiomyopathy.    Enjoy Ep #58 and CN5 for differences on invasive hemodynamics!  3. Given that the patient had subvavlular thickening, the patient was not a candidate for mitral valve repair and the patient underwent mitral valve replacement. What are the indications for mitral valve surgery in patients with severe chronic MR? Why is mitral valve repair preferred for primary MR?   Class I indications for mitral valve surgery include (1) symptomatic patients in the absence of severe LV dysfunction (defined as EF <30%; Stage D MR); (2) asymptomatic patients with chronic severe MR and mild to moderate LV dysfunction (EF 30% to 60% and/or LV end-systolic dimension ≥ 40 mm; Stage C); (3) patients with chronic severe primary MR undergoing cardiac surgery for other indications.  Class IIa indications include (1) asymptomatic patients with LVEF > 60% and LVESD < 40 mm (Stage C1) in whom the likelihood of successful repair is >95% and expected mortality is <1%; (2) asymptomatic patients with new-onset atrial fibrillation and preserved LVEF (Stage C1); (3) asymptomatic patients with pulmonary hypertension (defined as PASP > 50 mmHg) and preserved LVEF.  For primary mitral regurgitation involving an abnormality of the valve apparatus (e.g., leaflets, chordae, annulus, papillary muscles) – MV repair is preferred over MV replacement in most patients as it is associated with lower operative mortality and improved long term survival. Feasibility of valve repair will generally depend on valve anatomy and surgical experience. Class I indications for MV repair over mitral valve replacement (MVR) include chronic severe primary MR limited to the posterior leaflet and chronic severe primary MR involving the anterior leaflet or both leaflets when a successful and durable repair appears feasible.  4. The patient underwent TV repair. What is the reason to potentially pursue TV intervention in patients undergoing pericardiectomy?   Tricuspid regurgitation (TR) is often a co-morbid condition in patients with constrictive pericarditis. After pericardiectomy, TR can worsen after a thickened, calcified pericardium is removed allowing RV dilation and subsequent dilation of the TV annulus leading to worsening functional TR. Tricuspid regurgitation is associated with increased short and long-term mortality following pericardiectomy, and the Mayo Clinic in particular has been integral in demonstrating that even mild TR is a marker of increased operative and long-term mortality and worsening symptoms. Thus, in patients with moderate to severe TR at the time of pericardiectomy, consideration should be given for tricuspid valve repair (if feasible). Of note, most data to date suggests that this approach provides symptomatic improvement but no clear mortality benefit has been demonstrated.  5. Finally, the Mayo CardioNerds taught us to think about our thinking! What are some cognitive errors to be aware of when evaluating patients?   The team at the Mayo Clinic masterfully demonstrated how to avoid the common cognitive errors of the anchoring heuristic and premature closure while diagnosing a case of constrictive pericarditis and severe mitral regurgitation.  Anchoring heuristic is the tendency to rely on selected signs or simple investigations, often fixating on early data and not incorporating new information. In other words, one “anchors” onto the initial data and impressions. Ways to avoid anchoring include: attention to base rates of disease, knowledge of test characteristics of diagnostics, deliberately seeking more information to support or refute the initial impression, and following data-driven diagnostic algorithms.   Premature closure is the acceptance of a diagnosis before it has been fully verified. To avoid this, consider alternative diagnoses and search for data that challenge the provisional diagnosis.  References for episode 59: Constrictive Pericarditis & Severe Mitral Regurgitation Geske, J. B., Anavekar, N. S., Nishimura, R. A., Oh, J. K. & Gersh, B. J. Differentiation of Constriction and Restriction: Complex Cardiovascular Hemodynamics. J. Am. Coll. Cardiol. 68, 2329–2347 (2016).  Garcia, M. J. Constrictive Pericarditis Versus Restrictive Cardiomyopathy? J. Am. Coll. Cardiol. 67, 2061–2076 (2016).  Chiabrando, J. G. et al. Management of Acute and Recurrent Pericarditis: JACC State-of-the-Art Review. J. Am. Coll. Cardiol. 75, 76–92 (2020).  Scott, I. A. Errors in clinical reasoning: causes and remedial strategies. BMJ 338, (2009).  Nishimura, R. A. et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J. Am. Coll. Cardiol. 70, 252–289 (2017).  Bilchick, K. C. & Wise, R. A. Paradoxical physical findings described by Kussmaul: pulsus paradoxus and Kussmaul’s sign. Lancet Lond. Engl. 359, 1940–1942 (2002).  Tariq, M. U., Aman, W., Karwa, A., Benatti, R. & Klein, A. Right Ventricular Dilatation Post Pericardiectomy Causes Tricuspid Regurgitation. J. Am. Coll. Cardiol. 65, A1315 (2015).  Lazam, S. et al. Twenty-Year Outcome After Mitral Repair Versus Replacement for Severe Degenerative Mitral Regurgitation: Analysis of a Large, Prospective, Multicenter, International Registry. Circulation 135, 410–422 (2017).  CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder) join join University of Tennessee cardiology fellows (Rachel Goodwin, Emmanuel Isang, and William Black) for some chocolate cake and hikes in the Smoky Mountains! They discuss a fascinating case of constrictive pericarditis. Dr. Tjuan Overly provides the E-CPR and a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.   Jump to: Patient summary – Case figures & media – Case teaching – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A man in his late 40s with a history of renal failure secondary to IgA nephropathy and now status post a kidney transplant 10-15 years ago was referred by hepatology for evaluation of recurrent ascites and LE edema. He appeared grossly volume overloaded on exam with JVP elevated past the mandible, RV heave, and 2+ pitting edema. TTE demonstrated LVEF of 55-60%, RVSP 40mmHg, abnormal septal motion with respiration, and respirophasic variation in mitral inflow across the mitral valve raising the suspicion for constrictive pericarditis. RHC pressures demonstrated a mean RA pressure of 20mmHg, RV 40/25mmHg, PA 38/30mmHg (mean 32 mmHg) and PCWP mean of 26 with V-waves at 28 mmHg. Simultaneous LV and RV pressure tracings showed ventricular discordance with respirophasic variation, consistent with constrictive physiology. Patient underwent pericardiectomy with markedly improved heart failure symptoms. Repeat TTE showed no evidence of constriction.  Case Media A B C Click to Enlarge! A. ECGB. Pulsed-wave Doppler spectrum of tricuspid inflow velocities demonstrates a marked respiratory variation (In irregular rhythms, such as the atrial fibrillation seen here, respirophasic changes may still be seen but are confounded by the varying R-R interval)C. Simultaneous LV and RV pressure tracings showing discordance with respirophasic variation Apical 4-chamber view demonstrating abnormal septal motion due to interventricular dependence – dissociation of thoracic and cardiac chamber pressures leads to increased RV filling during inspiration Short axis view of the LV demonstrating a D-shaped interventricular septum during inspiration.  Note the presence of a pericardial effusion as well. Episode Schematics & Teaching Click to enlarge! The CardioNerds 5! – 5 major takeaways from the #CNCR case The initial presentation clinically seemed to be right greater than left heart failure. What are the signs and common causes of right heart failure?    The signs and symptoms of RHF are often similar to left-sided CHF, but may describe more severe dyspnea on exertion, significant abdominal distension, and early satiety due to ascites or gut edema. Symptoms of pulmonary edema from elevated left-sided filling pressures (orthopnea, paroxysmal nocturnal dyspnea) may be absent. On examination, there will be elevated JVP with likely prominent v-waves, possibly Kussmaul’s sign (inspiratory rise in JVP rather than fall) depending on the pathology, abdominal ascites, pulsatile hepatomegaly, and lower extremity edema. An RV heave may be discernible along with a loud P2 component and murmur of TR. Broadly, RV failure may be caused by pressure overload (ex: pulmonary hypertension, pulmonic stenosis), volume overload (ex: intracardiac shunt, tricuspid regurgitation), or myocardial disease (ex: cardiomyopathies, ischemia/infarct). The most common cause of chronic right heart failure is LV failure (causing post-capillary pulmonary hypertension). Other causes of RV failure include pre-capillary pulmonary hypertension, congenital heart disease (e.g., ASD, residual RVOT obstruction in Tetralogy of Fallot patients), ARVC, RV ischemia, myocarditis, right sided valvular disease, constrictive pericarditis, and restrictive cardiomyopathy. The patient in this case was diagnosed with constrictive pericarditis (CP). What are the causes of CP?   Remember that the etiology of CP can vary considerably depending on the patient’s demographics. In developed countries, the majority of cases are idiopathic or viral, post-operative, or post-radiation therapy. In developing countries, infectious etiologies are more common, with tuberculosis the most common cause.  Amongst the causes, remember that acute bacterial and tuberculosis pericarditis have the highest chances of progressing into constriction. With post-radiation constrictive pericarditis, remember there can be significant delay (even up to 20 years) between radiation therapy and development of constriction and often accompanies concomitant myocardial fibrosis with restrictive physiology as well.  Other etiologies include immunologic disorders (e.g., rheumatoid arthritis, lupus, sarcoidosis), malignancy (e.g., breast and lung cancers, lymphoma, mesothelioma), and myocardial infarction.   3.  What are the TTE findings suggestive of constrictive pericarditis?   To understand the basic TTE findings, we need a basic understanding of the pathophysiology. Constriction leads to a noncompliant pericardium that encases the heart. Heart failure occurs because there is impaired diastolic ventricular filling.    The ventricles fill almost entirely in early diastole, because once they can no longer expand because of the non-distensible pericardium, diastolic filling abruptly stops. This pathophysiology reflects one of the key findings in CP: equalization of the end-diastolic pressures.  At the same time, the thickened/fibrotic/calcified pericardium prevents the normal transmission of intrathoracic pressures to the cardiac chambers. However, structures “outside” the pericardium – such as the pulmonary vasculature – still “see” the normal changes in intrathoracic pressure. Normally, when we take a breath in: the intrathoracic pressure falls and this is transmitted equally to the pulmonary capillaries (e.g., the wedge pressure) and the cardiac chambers. The gradient for mitral valve inflow reflects the difference in wedge pressure and intra-cardiac chamber (LV) pressure.  In CP, the drop in intrathoracic pressure with inspiration is transmitted to the pulmonary capillaries but not the cardiac chambers. Thus, there is now a decreased gradient for mitral valve inflow during inspiration. This is called intrathoracic-intracardiac pressure disassociation.  At the same time, with inspiration right heart preload increases and to accommodate this volume the RV expands. However, RV expansion is limited by the encasing noncompliant pericardium, and thus to accommodate the volume the interventricular septum shifts to the left. This, further decreases the gradient for mitral valve inflow and the physiology is termed enhanced ventricular interdependence. The opposite occurs on expiration.    Thus, on echocardiogram we may see abnormal respirophasic septal shift, reflecting enhanced ventricular interdependence. The septum moves to the left in early diastole with inspiration and then back to the right on expiration. This is one of the most sensitive echocardiographic findings for CP.  Reflecting compromised diastolic filling, markedly elevated filling pressures, and equalization of end-diastolic pressures, there will be a high E-wave velocity with a decreased A-wave velocity (E/A > 1) across the mitral valve. Due to pericardial restraint, this early rapid diastolic filling (high velocity with a tall E wave) stops abruptly and so the E wave has a short deceleration time. The latter is analogous to the pericardial knock heard on physical exam and the ventricular early diastolic “square root” sign (dip and plateau) on the RHC.   Reflecting intrathoracic-intracardiac pressure disassociation and enhanced ventricular interdependence, there will be increased respirophasic variation in mitral and tricuspid valve inflow. This typically is best demonstrated with the first few beats of inspiration and expiration. Specific cut-offs include a decrease in peak mitral E-wave velocity > 25% and increase in peak tricuspid E-wave velocity > 40% during inspiration (opposite during expiration). In other words, as you inspire there is increased filling of the RV with decreased filling of the LV.   Expiratory hepatic vein diastolic flow reversal is one of the most specific findings of CP. Reflecting intrathoracic-intracardiac pressure disassociation and enhanced ventricular interdependence, during expiration RV filling is compromised (as the LV is filling with septal shift to the right) and thus there is “back-flow” from the right heart and we see more prominent flow reversal in the hepatic veins during expiration. In contrast, hepatic vein diastolic flow reversal occurs predominantly during inspiration with restrictive physiology.   Normally, the lateral e’ (tissue doppler) velocity > medial e’ velocity. In CP, we see annulus reversus, where the peak e’ at the medial annulus > lateral annulus because in constriction the lateral free wall may be tethered to the fibrotic/calcified pericardium and restricted in movement. In addition, when considering constriction vs restriction, normal or elevated annular e’ velocities are more consistent with constriction (normal myocardial relaxation) whereas reduced annular e’ velocities are more indicative of restriction (impaired myocardial relaxation).   4. What are the characteristics of CP on invasive hemodynamics?   All the findings on invasive hemodynamics are reflecting reliance on early diastolic filling and equalization of diastolic pressures.  The end-diastolic pressures in the ventricles are usually within 5 mmHg of each other.  We may see the square root sign on ventricular pressure tracings. The upward deflection in early diastole reflects rapid early diastolic filling and the subsequent plateau represents the abrupt halt in filling once the non-compliant pericardium can no longer expand. This is analogous to a pericardial knock on physical exam and a tall E wave with a short deceleration time on echocardiogram.   The right atrial pressure waveform may have a “W-shape” reflecting rapid x and y descents. Conversely restrictive physiology may have rapid y descents but typically not with the x descent.   Finally, simultaneous RV and LV pressures tracings will show discordance in pressures with respiration – with inspiration RV pressure increases and LV pressure decreases. This is analogous to respirophasic septal shift and changes in mitral and tricuspid inflow velocities seen on echocardiogram. This should not be present in patients with restriction.   Note, if patients are hypovolemic, typical features of CP may not be seen, and thus a fluid bolus may be required to unmask the findings of CP.   5. What’s the general approach to management of CP?   If active inflammation is present on labs or imaging, trial a course of anti-inflammatory medications such as colchicine and NSAIDs is recommended before surgery. Similarly, patients with effusive-constrictive pericarditis a pericardiocentesis and a trial of medical therapy initially is recommended. Refractory cases may warrant anti-inflammatory escalation including steroids, steroid-sparing agents, and biologics including anti-IL1 agents. Prolonged courses may be required with therapy tailored to symptoms, inflammatory markers (ESR, CRP), and cardiac MRI. Enjoy Ep #33 – CMR with Dr. Kwon!  If patient has persistent NYHA III or IV symptoms refractory to medical therapy without severe co-morbid illnesses, pericardiectomy may be indicated. Patients with idiopathic or viral pericarditis tend to have better outcomes with pericardiectomy than those with radiation therapy.  References Geske, J. B., Anavekar, N. S., Nishimura, R. A., Oh, J. K., & Gersh, B. J. (2016). Differentiation of Constriction and Restriction: Complex Cardiovascular Hemodynamics. Journal of the American College of Cardiology, 68(21), 2329–2347.  Garcia M. J. (2016). Constrictive Pericarditis Versus Restrictive Cardiomyopathy?. Journal of the American College of Cardiology, 67(17), 2061–2076.  Chiabrando, J. G., Bonaventura, A., Vecchié, A., Wohlford, G. F., Mauro, A. G., Jordan, J. H., Grizzard, J. D., Montecucco, F., Berrocal, D. H., Brucato, A., Imazio, M., & Abbate, A. (2020). Management of Acute and Recurrent Pericarditis: JACC State-of-the-Art Review. Journal of the American College of Cardiology, 75(1), 76–92.  CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder) join Penn cardiology fellows (Brian McCauley, Norrisa Haynes, and Mahesh Vidula) for a rooftop picnic in sunny Philadelphia! They discuss an informative case of peripartum cardiomyopathy with cardiogenic shock. Program director Dr. Frank Silvestry provides the E-CPR segment and a message to applicants. Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.  Jump to: Patient summary – Case figures & media – Case teaching – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary Two weeks postpartum, a woman in her mid 20s, G1P1, with no past medical history presented following a tonic-clonic seizure. Prior to this, she had been experiencing 1 week of worsening dyspnea and lower extremity edema. Initial work-up revealed a left MCA stroke and she underwent thrombectomy. Limited TTE found LVEF <20% and a LV apical thrombus; she was started on milrinone due to concern for cardiogenic shock and transferred to the University of Pennsylvania.  Upon arrival, she was found to be hypotensive and tachycardic. Exam was notable for elevated JVP, +S3, LE edema and R sided hemiparesis. Labs showed multiorgan injury, elevated NT-proBNP and elevated lactate. EKG demonstrated sinus tachycardia with no ST-T changes. Formal TTE showed severely dilated LV with EF 10%, diffuse LV hypokinesis, and confirmed a large LV apical thrombus. A pulmonary artery catheter was placed for tailored therapy and found elevated L-sided > R-side filling pressures with low cardiac index despite inotropes. Cardiac power output (CPO) was severely decreased with borderline pulmonary artery pulsatility index (PAPI), corroborating left > right heart failure. Patient ultimately required a durable left ventricular assist device (LVAD). Over the course of 9 months her guideline directed medical therapy (GDMT) was titrated and her intrinsic cardiac function and symptoms improved. Her EF improved to 35-40% and she tolerated an LVAD weaning protocol, so her LVAD was ultimately explanted! She is currently doing well on GDMT alone!   Case Media CXR: Mild interstitial edema, +ET tube ST (131), LAD, nonspec T wave flattening, nl intervals Click to Enlarge TTE 1 TTE 2 Episode Schematics & Teaching Click to enlarge! The CardioNerds 5! – 5 major takeaways from the #CNCR case 1. How do we define Peripartum Cardiomyopathy?   Diagnosis is made by the development of heart failure towards the end of pregnancy or in the months following delivery (~5 months postpartum), no other identifiable cause of HF, and demonstration of LV systolic dysfunction with LVEF typically less than 45% with or without dilation.  Risk factors include history of pre-eclampsia, hypertension, cocaine use, multifetal pregnancies, older maternal age, and African descent.     Keep a broad differential diagnosis for new onset heart failure in the peripartum period. The differential includes pre-existing cardiomyopathy, valvular disease or congenital cardiomyopathy unmasked by the hemodynamic changes of pregnancy (see CNCR episode 48 for more on the hemodynamic changes of pregnancy!). Other differentials should include ischemia/spontaneous coronary artery dissection (SCAD), stress-induced cardiomyopathy, CM due to systemic disease (e.g. sepsis, rheumatologic disease), myocarditis and tachycardia-induced CM.  2. The team used invasive hemodynamics to guide shock management. Why is a PAC helpful?   Despite data from the ESCAPE trial, a pulmonary artery catheter (PAC) can be very useful in the diagnosis and management of cardiogenic shock. Remember, the ESCAPE trial included chronic HF patients in whom there was clinical uncertainty on whether a PAC may be useful. Further, any patient on milrinone was excluded. Cardiac power output (CPO) and pulmonary artery pulsatility index (PAPi) are some of the parameters obtained from a PAC that can guide cardiogenic shock therapy and need for mechanical circulatory support (MCS).  Resting CPO = CO x MAP / 451. CPO measures the “pumping power” of the LV and correlates with end-organ perfusion. In the SHOCK registry, CPO <0.53 watts highly correlated with ↑ in-hospital mortality. CPO can also help identify patients ready for LVAD weaning trials and/or explantation. As with most cardiac parameters, the trends matter as much as the absolute values!     PAPi: PA(Systolic) – PA(diastolic) / RA(mean). PAPi was initially developed as a marker severe RV dysfunction in acute inferior wall myocardial infarction and those undergoing LVAD placement. It measures the ratio of the PA pulse pressure to a given preload (RA pressure). It is hard to apply PAPi thresholds across different clinical situations, but we can generally use less than 1-1.75 as a predictor of worsening right-sided function.    3. How should we approach “typical HF” management in peripartum cardiomyopathy?   Though many aspects of the acute management of peripartum cardiomyopathy are similar to typical heart failure and/or cardiogenic shock management, there are a few key differences.   Remember, ACEI/ARBs/ARNI and spironolactone are contraindicated during pregnancy. ACE-I have demonstrated safety data while breastfeeding, but ARNIs and ARBs have not been studied robustly.    The decision to pursue an ICD in patients with PPCM needs special attention to the natural history of PPCM. Many of these patients will have recovery of LV function in 3-12 months, thus deferring primary prevention ICD while titrating GDMT for ~6 months may be appropriate. There may also be a role for a wearable cardioverter/defibrillators in those with severe LV dysfunction as a bridge to recovery or until an implantable ICD is indicated.   Consider early mechanical support in patients deteriorating on medical therapy (including inotropes) alone.   4. What are some considerations unique to peripartum cardiomyopathy management?   The prothrombotic state of pregnancy and stasis within a weak ventricle both increase the risk of LV thrombus and a subsequent cardio-embolic event. Therefore, anticoagulation should be considered in PPCM patients with a severely reduced LVEF (EF < 30% by AHA vs ≤35% by ESC) during late pregnancy and 6 to 8 weeks postpartum. LMWH does not cross the placenta and is preferred during pregnancy. Both warfarin and LMWH may be used during lactation, while the direct oral anticoagulants have not been studied either during pregnancy or lactation and should be avoided.   Bromocriptine, a dopamine agonist, reduces prolactin levels (a possible culprit in the development of PPCM). However, data is conflicting on benefit, and thus this therapy should be considered investigative. It is pro-thrombotic, and so it is recommended for patients to be on anticoagulation if bromocriptine is used.   Prior to delivery, multidisciplinary discussions should occur around timing and method of delivery for patients diagnosed with PPCM during pregnancy. During delivery, remember that there are several hemodynamic changes (See Ep #48 – AS Complicating Pregnancy!). These include increased preload from placental auto-transfusion and relief of IVC compression.    The 2018 ESC guidelines suggest avoiding breastfeeding in patients with severe HF because of the high metabolic demands of lactation and the induced prolactin may contribute to worsening HF. However, some small studies suggest in clinically stable patients, breastfeeding does not worsen HF symptoms or LVEF. Considerations regarding possible risk must be counterbalanced with the known benefits of breastfeeding for infants and mothers.    5. How do we approach long-term management in patients with peripartum cardiomyopathy?   Providers should engage in shared decision-making about future pregnancies in patients with recovered EF (>50%) as they are at higher risk of future recurrence. The 2018 ESC guidelines advise against pregnancy if EF has not recovered to >50-55%.    Contraception counseling should be done early: before discharge or at the time of diagnosis. Patients should avoid estrogen-containing methods especially in the early postpartum period as thromboembolism risk is high.   If a patient plans on another pregnancy, teratogenic HF medications, like ACE/ARB/ARNI and spironolactone, must be stopped prior to stopping contraception. Following cessation of these GDMT medication, follow-up echocardiography to ensure LV functional stability should be performed after at least 3 months off therapy. The patient should be monitored very closely during a subsequent pregnancy for changes in symptoms and followed with serial echocardiograms and NT-proBNP levels during pregnancy and thereafter.    References Davis MB, Arany Z, McNamara DM et al. Peripartum Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol. 2020;75(2):207-221. doi:10.1016/j.jacc.2019.11.014  Korabathina R, Heffernan KS, Paruchuri V, et al. The pulmonary artery pulsatility index identifies severe right ventricular dysfunction in acute inferior myocardial infarction. Catheter Cardiovasc Interv. 2012;80(4):593-600. doi:10.1002/ccd.23309  Kang G, Ha R, Banerjee D. Pulmonary artery pulsatility index predicts right ventricular failure after left ventricular assist device implantation [published correction appears in J Heart Lung Transplant. 2017 Nov;36(11):1272]. J Heart Lung Transplant. 2016;35(1):67-73. doi:10.1016/j.healun.2015.06.009  Kochav SM, Flores RJ, Truby LK, Topkara VK. Prognostic Impact of Pulmonary Artery Pulsatility Index (PAPi) in Patients With Advanced Heart Failure: Insights From the ESCAPE Trial. J Card Fail. 2018;24(7):453-459. doi:10.1016/j.cardfail.2018.03.008  Lim HS, Gustafsson F. Pulmonary artery pulsatility index: physiological basis and clinical application. Eur J Heart Fail. 2020;22(1):32-38. doi:10.1002/ejhf.1679  Fincke R, Hochman JS, Lowe AM, et al. Cardiac power is the strongest hemodynamic correlate of mortality in cardiogenic shock: a report from the SHOCK trial registry. J Am Coll Cardiol. 2004;44(2):340-348. doi:10.1016/j.jacc.2004.03.060  Mendoza DD, Cooper HA, Panza JA. Cardiac power output predicts mortality across a broad spectrum of patients with acute cardiac disease. Am Heart J. 2007;153(3):366-370. doi:10.1016/j.ahj.2006.11.014  CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

Lisa Wilsbacher and Benjamin Freed join Cardionerds to discuss a fascinating case of arrhythmogenic desmoplakin cardiomyopathy. They cover topics such as the psychological impact of ICD shocks, genetic cardiomyopathy workup, diagnostic challenges in cardiac sarcoidosis, and the importance of genetic testing for non-ischemic cardiomyopathy. An engaging and educational podcast with expert perspectives.

CardioNerds (Amit Goyal & Daniel Ambinder) join UT-Austin cardiology fellows (Priya Kothapali, Sergio Montano, Travis Benzing, and Michael Grzeskowiak) for a speedboat adventure on Lake Travis! They discuss a fascinating case of Suicide LV post-TAVR. Dr. Mark Pirwitz provides the E-CPR and program director Dr. Clay Cauthen provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Evelyn Song with mentorship from University of Maryland cardiology fellow Karan Desai.  Jump to: Patient summary – Case figures & media – Case teaching – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A woman in her early 70s, with a history of CAD s/p PCI to LAD & RCA with DES six months prior, to presentation, paroxysmal atrial fibrillation s/p ablation, type 2 diabetes mellitus, hypertension, prior TIA, and severe symptomatic AS was admitted for elective TAVR. She underwent successful implantation of a 29mm Medtronic Evolut Pro valve via left common femoral artery access. Post-valve deployment and following protamine administration for heparin reversal, course was complicated by hypotension with PEA arrest requiring CPR for 4 minutes. Intra-op TEE and angiogram showed a well-seated prosthetic valve with trace paravalvular leak and no evidence of acute aortic regurgitation, significant paravalvular leak, pericardial effusion, coronary obstruction, aortic dissection, or access site complications. She was treated for suspected Protamine reaction with high dose steroids & epinephrine. However, she remained hypotensive with MAP in the 50s on high dose Epinephrine, Norepinephrine, and Vasopressin. Hemodynamics by pulmonary artery catheter demonstrated CVP 7, mPA 26, PCWP 18 mmHg and CO/CI 2.8 L/min and 1.3 L/min/m2. Her lactate was elevated at 5.92 mmol/L and EKG demonstrated normal sinus rhythm. Bedside TTE in the ICU showed hyperdynamic LV function with LVEF 70% and near-complete mid to distal cavity obliteration with significant intracavitary gradient. She was diagnosed with post-TAVR suicide LV and managed with aggressive volume resuscitation and rapid wean of Epinephrine/Norepinephrine with improvement in her hemodynamics. She was eventually extubated and discharged on beta-blocker therapy. Case Media A. ECG B. CXR Click to Enlarge A. ECG: Normal sinus rhythm, no evidence of AV block, no ST segment elevation or depression.B. CXR: Pulmonary vascular congestion, no pneumothorax, ETT at level of carina, PAC in appropriate position Pre-Aortogram Implantation Post-Dilation Post-Aortogram TEE: Mid-Esophageal Three-Chamber View TEE: Mid-Esophageal Short Axis View Abdominal aortography showed no evidence of vascular access site complication or contrast extravasation. Aortic root angiography showed no evidence of AI, coronary obstruction, or dissection. TTE: PLAX and A4C Episode Schematics & Teaching Created by Dr. Karan Desai Graphic by Dr. Carine Hamo Click to enlarge! The CardioNerds 5! – 5 major takeaways from the #CNCR case Remind all CardioNerds – how do we define Severe Aortic Stenosis?  By TTE, severe AS is defined as a mean gradient >40 mmHg, a peak velocity >4 m/s, an aortic valve area <1 cm2 and dimensionless index < 0.25. But remember, in HF patients decreased trans-aortic valve flow secondary to LV disease can make the diagnosis challenging!  In HFrEF, patients with severe AS by aortic valve area (AVA) may not meet velocity or gradient criteria in one of four contexts: AVA measurement error, pseudo-severe AS, poor contractile reserve, or true severe Low Flow, Low Gradient AS (LFLG AS).  Similarly, in HFpEF patients, disproportionately low flow or gradient AS may occur due to AVA measurement error or from true severe “paradoxical” LFLG due to decrease stroke volume (stroke volume index <35 mL/m2).  Low gradient AS is a critical subset of patients with generally with a comparatively worse clinical trajectory marked by increased heart failure hospitalizations and mortality compared with high gradient AS.  Enjoy the aortic stenosis series (Ep #1-2) and related figure for a deeper dive!  2.     This patient received a TAVR for her severe AS. What are the major complications?  Generally there are five relatively important post-TAVR complications – termed the “Big 5” – that contribute to long-term morbidity. These include disabling stroke, acute kidney injury, moderate/severe paravalvular leak, vascular and bleeding complications, and conduction abnormalities with possible need for PPM.  Modern TAVR valves include mechanisms to reduce paravalvular leak (i.e., an outer skirt or a porcine pericardial tissue wrap or cuff), but may lead to more conduction abnormalities  3.     This case covered Suicide LV. What is the pathophysiology behind this?  Severe AS leads to LV hypertrophy and chronic pressure overload. When the fixed obstruction is relieved, the increased contractility of the LV is “unmasked” and can lead to dynamic LV cavity obstruction or obliteration.   When this compromises cardiac output, these labile hemodynamics are termed “suicide LV”.    4.     What are risk factors for developing Suicide LV post-AVR?  The major risk factors for Suicide LV with dynamic intracavitary gradient include small LVOT and LV size, hyperdynamic and elevated LVEF, asymmetric septal hypertrophy with an elevated interventricular septal to posterior wall thickness ratio, high valve gradients prior to AVR and small LV wall mass.  5.     What should be my basic approach to Suicide LV management?  The management of suicide LV is similar to that of Hypertrophic Obstructive Cardiomyopathy (see Ep#3) and related figure for a refresher!  The basics include increasing preload with intravenous fluids, increasing afterload to reduce intra-cavitary gradients (phenylephrine tends to be the vasoactive of choice), avoiding inotropes (e.g., digoxin or beta-agonist infusions) while considering negative inotropes (e.g., beta blockers), and maintaining AV synchrony to ensure adequate LV preload  References Evaluation of Shock Following TAVR – ACC   Big 5 Complications post-TAVR – JACC IC 2019  Suh 2010 – Suicide LV following TAVI  CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerd (Amit Goyal) join Washington University in St. Louis cardiology fellows (Adam Lick, Manny Rivera Maza, and Sam Lindner) for some amazing local St. Louis craft brews! They discuss a fascinating case of  wild-type aTTR cardiac amyloid. Prior to meeting up with the group, Amit bumps into Rachita Navara: a Wash U #FIT, aspiring electrophysiologist, & a rock star of the band “The Pacemakers” (be sure to check out their performance at the end of the episode!) who shares thoughts about the program and her cutting edge contributions to the field of EP. Dr. Katie Zhang provides the E-CPR and program director Dr. Andy Kates provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.   Jump to: Patient summary – Case figures & media – Case teaching – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A man in his early 70s, with a history of hypertension, a bicuspid aortic valve, chronic kidney disease and carpal tunnel syndrome presents with two weeks of worsening dyspnea on exertion. At baseline, he is an avid cyclist and noticed he can now only bike ½ mile when before he could bike extended distances. In addition, he noted abdominal swelling and palpitations. Vitals signs showed mild tachycardia, irregularly irregular rhythm, and no clear evidence of volume overload. Labs demonstrated acute on chronic kidney disease, an elevated NT-proBNP and elevated troponin. ECG demonstrated atrial flutter with variable conduction block. TTE demonstrated marked concentric left ventricular hypertrophy with preserved ejection fraction, biatrial enlargement, reduced global longitudinal strain with apical sparing, and bicuspid aortic valve with moderate aortic stenosis. Further diagnostics revealed normal serum kappa/lambda light chains and PYP scan was positive. Patient underwent EMB which demonstrated ATTR amyloid deposition and genetic screening did not show mutations commonly associated with hereditary ATTR.  Case Media A B C D A. CXR: Stable mild enlargement of the cardiac silhouette; mildly tortuous aorta. Lung fields are clear, no pneumonia, pleural effusions, or pneumothorax.B. AV continuous wave DopplerC. Strain ImageD. Tc-99 PYP Scan TTE 1 TTE 4 TTE 2 TTE 5 TTE 3 TC-99 PYP Scan Strain video 1 Strain video 2 Strain video 3 Episode Schematics & Teaching Created by Dr. Karan Desai Created by Dr. Carine Hamo (updated 9.2020) Click to enlarge! The CardioNerds 5! – 5 major takeaways from the #CNCR case Cardiac amyloidosis can have a range of cardiac and extra-cardiac findings as amyloid fibrils can deposit in many different tissues, depending on the amyloid protein involved. The first step in diagnosis is maintaining a high index of suspicion.  The presence of prominent right-sided HF symptoms, low voltage on ECG, biatrial enlargement, ventricular hypertrophy, conduction disease, and pericardial effusion should prompt a high suspicion for cardiac amyloid. These are typical features of infiltrative or storage restrictive cardiomyopathies. PEARL: ATTR CM can cause asymmetric LVH and is an important HCM phenocopy!  Remember, amyloidosis is a systemic disease and extra-cardiac findings are common. ATTR amyloid has a predilection for the musculoskeletal system (including bilateral carpal tunnel syndrome, lumbar spinal stenosis, and biceps tendon rupture) and peripheral nerves. Conversely, AL amyloid is widely deposited outside the CNS an can result in protean manifestations, including periorbital ecchymoses from vascular fragility, macroglossia, and visceral organ involvement (including nephrotic syndrome, hepatic infiltration, and gut amyloid).  When considering cardiac amyloid, there are four primary etiologies:  Wild-Type (non-hereditary) ATTR amyloidosis is caused by deposition of misfolded transthyretin proteins. It is an under-recognized cause of HFpEF, with incidence increasing with age.  Hereditary ATTR is caused by a genetic mutation that leads to instability of the transthyretin tetramer. The most common mutation is the Val30Met variant. Specific mutations tend to have templated organ manifestations, natural history, and prognosis.   AL amyloidosis is caused by deposition of light-chains from a clonal plasma cell dyscrasia.  AA amyloidosis is a rare form of cardiac amyloid caused by deposition of the acute phase reactant serum amyloid A protein due to a chronic inflammatory process.  Evaluating for AL amyloid with appropriate lab workup is crucial. “Missing AL amyloid is like missing a STEMI” – Dr. Paul Cremer (Episode #8)! Workup should include SPEP, UPEP, serum/urine immunofixation, and serum kappa/lambda free light chains to maximize sensitivity. Note: SPEP and UPEP alone are insensitive and inadequate!  Like many cardiac diseases, multimodal diagnostics are key in the diagnosis of cardiac amyloid  ECG: Low voltage, pseudoinfarct pattern, variable conduction disease, atrial arrhythmias. PEARL: 10% of patients with cardiac amyloid may have high voltages on ECG. LVH on imaging out of proportion to EKG voltages is a red flag!  TTE: We have discussed typical features of an infiltrative or storage restrictive cardiomyopathy previously , including marked LVH with normal LV volumes and bi-atrial enlargement. Other features include: RV hypertrophy, thickened valve leaflets, thick interatrial septum, speckled appearance of the myocardium, and small pericardial effusion. Bi-atrial enlargement may lead to functional MR and TR as well as atrial arrhythmias. Mitral inflow pattern and tissue doppler will show varying degrees of diastolic dysfunction depending on stage. Systolic function may be borderline and progressively decline in “burned-out” disease. Reduced global longitudinal strain with apical sparing may help differentiate cardiac amyloid from hypertensive heart disease. There is an overlap with aortic stenosis which will frequently manifest as a low flow and/or low gradient phenotype due to reduce stroke volumes.  Cardiac MRI: There are characteristic findings of early subendocardial late gadolinium enhancement (LGE) and later transmural LGE with abnormal blood-pool-to-myocardial nulling (an MRI technique to accentuate pathology).   PYP scan: >99% sensitivity for cardiac ATTR amyloid. If monoclonal gammopathy is excluded, the positive predictive value is 100%.  RHC +/- EMBx: the hemodynamic profile is that of restrictive cardiomyopathy with elevated filling pressures, blunted x descent, and steep y descent. There may be diastolic pressure equalization. If EMBx is pursued, pathology will reveal amyloid protein as a salmon-pink color when congo red stain is applied and when placed under polarized light the amyloid proteins have an apple-green birefringence. Amyloid fibrils are seen with electron microscopic study.  Mass spec for protein identification.  Cardiac amyloid can be very difficult to treat with typical HF regimens, as beta blockers and ACE-I/ARB can lead to excessive hypotension in the setting of autonomic neuropathy and restrictive hemodynamics.  For TTR amyloid, there are treatment options like tafamidis (a transthyretin protein stabilizer) which can reduce mortality considerably in selected patients.  Investigational RNA-targeted therapies (i.e., patisiran) are increasing the options for treating TTR, but cost remains a major barrier to care. For AL amyloid, a multidisciplinary cardio-oncology team is vital to coordinate chemotherapy and cardiovascular care. Heart transplant +/- bone marrow transplant may be options in advanced stages of disease. The challenges of managing advanced disease highlight the importance of early recognition.   References TTR Ca Review – JACC 2019  Amyloid CM Case – JACC Case Reports 2019  CardioNerds Amyloid Page  Strain Imaging Echocardiography: What Imaging Cardiologists Should Know  Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy  CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal & Daniel Ambinder) join Temple Cardiology Fellows (Anika Vaidy and Anne- Sophie LaCharite-Roberge) in Philadelphia, PA! They discuss a fascinating case of pulmonary hypertension secondary to Chronic Thromboembolic Pulmonary Hypertension (CTEPH) associated with May Thurner syndrome and large uterine fibroids. Dr. Vaidya provides the E-CPR and message to applicants. Episode notes were developed by Johns Hopkins internal medicine resident, Eunice Dugan, with mentorship from University of Maryland cardiology fellow Karan Desai.  “To study the phenomena of disease without books is to sail an uncharted sea, while to study books without patients is not to go to sea at all.” – Sir William Osler. CardioNerds thank the patients and their loved ones whose stories teach us the Art of Medicine and support our Mission to Democratize Cardiovascular Medicine. Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values. Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A woman in her late 40s with history of iron deficiency anemia, uterine fibroids, and recurrent DVTs/PEs for the past 6 years despite different anticoagulation regimens, presented with syncope and progressive dyspnea on exertion. Family history was negative for DVT/PE or hypercoagulable states. On exam, she was euvolemic. Labs were significant for anemia, a normal pro-BNP, and a negative extensive hypercoagulable workup. TTE showed interventricular systolic septal flattening, right ventricular outflow tract pulse wave doppler with mid-systolic notch, and shortened acceleration time consistent with elevated pulmonary artery pressure. A VQ scan showed bilateral right greater than left mis-matched perfusion defects. CT angiogram showed right greater than left chronic mural thrombus, correlating with pulmonary angiogram which showed severe proximal and mid-vessel disease on the right and distal disease on the left. RHC corroborated the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) with mean pulmonary artery pressure of 41 and PVR of 5.2 Woods Units (WU).   To determine the etiology of her recurrent clots, a lower extremity venogram was performed and showed 80% stenosis of her left common iliac vein by the overlying right common iliac artery confirming May-Thurner syndrome. Lower extremity venogram also showed severe proximal stenosis of right iliac vein thought to be due to large uterine fibroids. Given her severe proximal and mid-vessel clot burden, she underwent pulmonary thromboendarterectomy with a subsequent drop in pulmonary vascular resistance to 1 WU.  The etiology of DVTs and CTEPH was determined to be external compression related to both May-Thurner syndrome and uterine fibroids. To prevent future thromboembolic events, she underwent stenting of her left common iliac vein and hysterectomy. With these interventions, RV function returned to normal, and her symptoms completely resolved!  Case Media A B C D E F G H I JClick to enlarge ??? Figue Legend: A. CXRB. ECGC. TTE: Interventricular systolic septal flattening, RVOT pulse wave Doppler with mid-systolic notch and shortened acceleration time, consistent with elevated PVR, RV:LV ratio 1.3, consistent with moderate RV enlargement, The RV is apex-sharing with an open apical angleD. VQ Scan: Multiple b/l perfusion defects, R > L, V scan normalE. CT Angiogram: 1) Enlarged R main PA2) large proximal chronic mural thrombus with minimal vessel count throughout R side, 3) Segmental LLL lining thrombusF. Pulmonary angiogram significant for severe proximal and mid-vessel disease in the right segmental arteries. Patient also had severe distal disease in the left sub segmental disease (not shown).G. Pulmonary Thromboendarterectomy (PTE)H. LE Venogram: LEFT CIV  > 80% stenosis as a result of compression from an overlying right common iliac artery. This is consistent with May-Thurner syndrome. RIGHT EIV (not shown)– Significant proximal stenosisI. Status Post left iliac vein stentJ. ECG: New typical atrial flutter Episode Schematics & Teaching Click to enlarge ??? The CardioNerds 5! – 5 major takeaways from the #CNCR case In patients with progressive dyspnea, exercise intolerance, and persistence of symptoms of PE despite adequate anticoagulation, there should be an evaluation for Chronic Thromboembolic Pulmonary Hypertension (CTEPH).  CTEPH is a form of pre-capillary pulmonary hypertension (PH) from incomplete resolution of pulmonary thromboemboli causing chronic, fibrotic, flow limiting changes to the pulmonary vascular bed. Risk factors include recurrent PE, hypercoagulable states, and medical conditions such as splenectomy, ventriculo-atrial shunts, infected intravenous catheters/devices, malignancy, and external venous compression. NOTE: many patients will not have a documented history of DVT/PE and so a high index of suspicion is important.  The following echocardiographic findings suggest pulmonary hypertension: interventricular systolic septal flattening indicating RV pressure overload, RV outflow tract (RVOT) pulse wave doppler with mid-systolic notch, decreased RVOT acceleration time, right atrial enlargement, right ventricular dilation and hypertrophy +/- functional tricuspid regurgitation, and an elevated estimated RV systolic pressure (RVSP).  CTEPH is rare and carries a high rate of morbidity and mortality; therefore, a high index of suspicion is necessary. There are two components to diagnosis: Diagnose CTEPH: Multiple imaging modalities are involved to confirm and assess severity. V/Q scan is highly sensitive and is the initial screening modality to detect perfusion abnormalities. Chest CT with IV contrast may identify parenchymal and mediastinal pathology not otherwise seen; although not sensitive, CT may show: PA dilation, eccentric filling defects with variable degrees of occlusion, vascular webs or bands, mosaic perfusion pattern of the lung parenchyma, and RV enlargement. RHC can confirm the diagnosis of pulmonary hypertension, differentiate pre-capillary from post-capillary PH, quantify vascular resistance and cardiac output, and can be a comparative marker post-intervention. Conventional PA angiography is useful for pre-operative planning.  Diagnose the underlying predisposition/etiology: Think about Virchow’s Triad: Hypercoagulability, Stasis, and Endothelial injury. Apart from ruling out hypercoagulable states, evaluating for lower extremity vascular compression can be important in select patients with imaging such as venography. May-Thurner syndrome is a condition of extrinsic venous compression of the left common iliac vein by the common iliac artery. Evidence of any compression should be addressed to prevent future venous thrombi and subsequent emboli.   Treatment for CTEPH starts with lifelong anticoagulation. Pulmonary angiography aids in surgical planning as proximal disease is more likely to be operable than distal disease. Based on severity and other comorbidities, pulmonary endarterectomy (PEA) can be a favorable option. PEA improves symptoms, survival, hemodynamics parameters, and RV remodeling. Other treatment strategies include medical management, balloon pulmonary angioplasty, and lung transplant. Pulmonary artery denervation is being considered as an experimental modality.   Supraventricular tachycardia (SVT) is common in patients with pulmonary artery hypertension and CTEPH and often indicate progression of right-sided dysfunction. PH patients rely more on active than passive RV filling, and are highly sensitive to changes in RV afterload. Supraventricular tachycardias may further precipitate decompensation given (1) the loss of atrial kick which compromises diastolic filling, and (2) tachycardias which increase RV wall tension increasing RV afterload. Therefore, restoration of sinus rhythm is strongly encouraged.  Educational Video Produced by Dr. Karan Desai References JACC 2018 – CTEPH Review  ACC Expert Analysis 2019 – Rx of CTEPH  May-Thurner Updodate  Pulmonary Circulation 2019 – Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in PAH and CTEPH  Pulmonary Circulation 2012 – Evaluation of patients with CTEPH for PTE  ESC/ERC Dx and Eval PH  CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal & Carine Hamo) discuss the past, present, and future of Women’s Heart Health & Women in Cardiology with Dr. Nanette Wenger, Professor of Medicine in the Division of Cardiology at the Emory University School of Medicine. Dr. Wenger is a true leader in the field of women’s heart health and a strong proponent for women in cardiology and medicine. Her passion, dedication, and advocacy have inspired countless trainees to carry this torch and continue to build on her truly impactful work. Special introduction by Dr. Martha Gulati and birthday wishes to Dr. Wenger by the entire CardioNerds Team! Special thanks to Dr. Kimberly Manning for her invaluable mentorship. Episode graphic by Dr. Carine Hamo The Cardionerds CV prevention series  includes in-depth deep dives on so many prevention topics including the ABCs of prevention, approach to obesity, hypertension, diabetes mellitus and anti-diabetes agents, personalized risk and genetic risk assessments, hyperlipidemia, women’s cardiovascular prevention, coronary calcium scoring and so much more! CardioNerds Episode PageCardioNerds Prevention PageCardioNerds Women’s Cardiovascular Health PageCardioNerds Academy – Apply now! Subscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron! We are truly honored to be producing the Cardionerds CVD Prevention Series in collaboration with the American Society for Preventive Cardiology! The ASPC is an incredible resource for learning, networking, and promoting the ideals of cardiovascular prevention! This series is kicked off by a message from Dr. Amit Khera, President of the American Society for Preventive Cardiology and President of the SouthWest Affiliate of the American Heart Association. Cardionerds Cardiovascular Prevention Series References and Links 1. Wenger NK (2005) Women in cardiology: The US experience. Heart. 2. Douglas PS, Rzeszut AK, Noel Bairey Merz C, Duvernoy CS, Lewis SJ, Walsh MN, Gillam L (2018) Career preferences and perceptions of cardiology among us internal medicine trainees factors influencing cardiology career choice. JAMA Cardiol. 3. Wenger NK, Speroff L, Packard B (1993) Cardiovascular Health and Disease in Women. N Engl J Med. 4. Burgess S, Shaw E, Zaman S (2019) Women in Cardiology. Circulation. Meet Dr. Wenger! Dr. Nanette Wenger is Professor of Medicine in the Division of Cardiology at the Emory University School of Medicine. Dr. Wenger received her medical degree from Harvard Medical School in 1954 as one of their first female graduates followed by training at Mount Sinai Hospital where she was the first female to be chief resident in the cardiology department. She is among the first physicians to focus on heart disease in women with an expertise in cardiac rehabilitation and geriatric medicine. Dr. Wenger has received numerous awards including the Distinguished Achievement Award from the Scientific Councils of the American Heart Association and its Women in Cardiology Mentoring Award, the James D. Bruce Memorial Award of the American College of Physicians for distinguished contributions in preventive medicine, the Gold Heart Award, the highest award of the American Heart Association, a Lifetime Achievement Award in 2009 and the Inaugural Bernadine Healy Leadership in Women’s CV Disease Distinguished Award, American College of Cardiology. She chaired the U.S. National Heart, Lung, and Blood Institute Conference on Cardiovascular Health and Disease in Women, is a Past President of the Society of Geriatric Cardiology and is past Chair, Board of Directors of the Society for Women’s Health Research. Dr. Wenger serves on the editorial boards of numerous professional journals and is a sought-after lecturer for issues related to heart disease in women, heart disease in the elderly, cardiac rehabilitation, coronary prevention, and contemporary cardiac care. She is listed in Best Doctors in America. Carine Hamo, MD Amit Goyal, MD

CardioNerds (Amit Goyal & Dan Ambinder) join Lankenau Medical Center cardiology fellows (Gwen McNeill and Shaung Ooi) for some Philly cheesesteaks! They discuss a fascinating case of Embolic Acute Coronary Syndrome from PFO and Pulmonary Hypertension. Dr. John Clark provides the E-CPR and program director Dr. Jeanine Romanelli provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai.  Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A woman in her early 40s with history of tobacco and prior methamphetamine use presented with acute onset chest pain. She was found to have an elevated troponin, anterior T wave inversions, and apical akinesis on TTE. Coronary angiography showed a coronary embolism in the mid-distal LAD. Attempts to wire the lesion led to distal embolization of the clot, and IVUS showed no underlying arteriosclerosis.   To investigate the etiology of the coronary embolus, a repeat TTE with agitated saline was done that suggested a PFO with right to left flow, as well as decreased RV function with an estimated RVSP of 70 mmHg. The clinical picture was that of a paradoxical coronary embolus, arising from the right (venous) side traveling to the left (arterial) side via a PFO. PFO closure was discussed but not performed given severe pulmonary hypertension with Right to Left shunt. In this circumstance, the PFO functions as a “pop-off valve” for the overloaded RV; closing it risks precipitating acute RV overload and failure. A RHC showed a PA pressure of 70/24 mmHg with mPAP of 40 mmHg, PCWP 5 mmHg, and PVR of 11 woods units. Given concern for idiopathic PH, a vasodilator challenge was done which did not show reactivity, and she was started on ambrisentan and sildenafil. Ultimately, the etiology of her pulmonary hypertension was felt to be due to PAH from prior methamphetamine use vs. idiopathic PAH. On follow-up, her PA pressures and RV function had greatly approved, allowing for safe and successful PFO closure in an attempt to prevent future emboli.  Final diagnosis: Embolic Acute Coronary Syndrome from PFO & Pulmonary Hypertension. Case Media Before treatment: short axis Before treatment: apical 4 Before treatment: RVSP After treatment: Short axis After treatment: Apical 4 After treatment: RVSP Click to enlarge ??? https://youtu.be/XpnJKZuNVrM 1. Coronary angio of embolism2. PFO on TEE with R to L shunt by Doppler3. TTE bubble after PFO closure Episode Schematics & Teaching Click to enlarge ??? The CardioNerds 5! – 5 major takeaways from the #CNCR case Coronary emboli are an uncommon cause of myocardial infarction. We can think of the etiology of coronary emboli in three major categories: Direct, Paradoxical, or Iatrogenic.  Direct emboli originate from the left side of the heart: sources include clot (from atrial appendage, apical thrombus), valvular lesion (vegetation, thrombus, fibroelastoma), or left sided cardiac mass (atrial myxoma, rhabdomyosarcoma).  Paradoxical emboli originate from the right side or systemic venous circulation, and pass from right to left through an atrial septal defect, patent foramen ovale, or pulmonary arteriovenous malformation.  Iatrogenic emboli occur following procedures such as valve replacement or PCI. Note that iatrogenic is the most common etiology!  PFOs are present in up to 25% of adults, but are usually clinically insignificant. However, there is increasing evidence that PFO closure is moderately beneficial compared to antiplatelet therapy alone in patients less than 60 years old with cryptogenic, non-lacunar ischemic stroke. PFO closure may particularly benefit those with a large right-to-left shunt or an associated atrial septal aneurysm. Remember closing an ASD in the setting of significant pulmonary hypertension can lead to decompensation, as the interatrial connection may be serving as a “pop-off” valve to decompress the RV and maintain cardiac output in the setting of high PA pressures! Specifically, ASD closure is generally contraindicated if the PA systolic pressure is > 2/3 systolic blood pressure, pulmonary vascular resistance > 2/3 systemic vascular resistance, or if  a net right-to-left shunt is present.  Recall that the hemodynamic definitions of pulmonary hypertension changed in 2019. A mean pulmonary artery pressure of 20 or greater confirms a diagnosis of pulmonary hypertension. A PCWP ≤15 mmHg with pulmonary vascular resistance ≥3 woods units suggests pre-capillary pulmonary hypertension, while a PCWP >15 with pulmonary vascular resistance <3 woods units suggests isolated post-capillary PH.   During a right heart catheterization, a pulmonary vasodilator challenge can be done to identify vasoreactive patients who may respond to calcium channel blockers. This is typically recommended for idiopathic and hereditary PAH. A positive response is defined as a drop in mean PAP to <40 mmHg, with a decrease of at least > 10 mmHg, with unchanged or increased cardiac output. These patients can be started on amlodipine or nifedipine, but should be followed closely as they may not always remain vasoreactive and clinical deterioration is possible!  Educational Video https://youtu.be/IidG4gUijm8 Produced by Dr. Karan Desai References P Raphael, C. E., Heit, J. A., Reeder, G. S. et al. (2018). Coronary Embolus: An Underappreciated Cause of Acute Coronary Syndromes. JACC. Cardiovascular interventions, 11(2), 172–180.   Mojadidi, M. K., Zaman, M. O., Elgendy, I. Y. et al. (2018). Cryptogenic Stroke and Patent Foramen Ovale. Journal of the American College of Cardiology, 71(9), 1035–1043.   Silvestry, F. E., Cohen, M. S., Armsby, L. B. et al. (2015). Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale: From the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, 28(8), 910–958.  Simonneau, G., Montani, D., Celermajer, D. S. et al. (2019). Haemodynamic definitions and updated clinical classification of pulmonary hypertension. The European respiratory journal, 53(1), 1801913.  Stout, K. K., Daniels, C. J., Aboulhosn, J. A. et al. (2019). 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation, 139(14), e637–e697.   CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD

CardioNerds (Amit Goyal & Dan Ambinder) discuss a case of hereditary restrictive cardiomyopathy with Duke University cardiology fellows Navid Nafissi and Sipa Yankey, and Marat Fudim, an advanced heart failure attending. E-CPR is provided by Dr. Richa Agarwal, fellowship program director of advanced heart failure and cardiac transplantation at Duke University with a final message by fellowship director Dr. Anna Lisa Crowley. Episode notes were developed by Johns Hopkins internal medicine resident Colin Blumenthal with mentorship from University of Maryland cardiology fellow Karan Desai.   Jump to: Patient summary – Case figures & media – Case teaching – Educational video – References – Production team Episode graphic by Dr. Carine Hamo The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus. We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director. CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademySubscribe to our newsletter- The HeartbeatSupport our educational mission by becoming a Patron!Cardiology Programs Twitter Group created by Dr. Nosheen Reza Patient Summary A 69 yo M with history of atrial fibrillation presents with 5 months of progressive HF symptoms, now NYHA class IV. He was found to be grossly volume overloaded, tachycardic in atrial fibrillation, and hypoxic. CXR demonstrated significant cardiomegaly, and labs indicated new normocytic anemia with evidence of hepatic dysfunction and an elevated NT proBNP. TTE demonstrated massive bi-atrial enlargement, preserved ejection fraction, filling pattern consistent with grade III diastolic dysfunction, and torrential TR. The echocardiogram did not have evidence of constrictive pericarditis and agitated saline study showed Right to Left shunt through a likely PFO. MRI to evaluate for infiltrative cardiomyopathy did not show late gadolinium enhancement (LGE). RHC demonstrated findings consistent with restrictive cardiomyopathy including equalization of diastolic pressures, square root sign, and concordance of RV and LV pressures. PYP scan evaluating for TTR amyloid was negative and laboratory workup did not suggest AL amyloid, Fabry’s, Hemochromatosis, or storage disease. Patient’s symptoms remained refractory and thus eventually underwent successful OHT. Genetic testing eventually revealed missense mutation in MYBPC3 – revealing an inherited cause of restrictive CM for the patient!  Case Media Chest X-ray -Rate-controlled A fib with frequent PVCs, LPFB, non-specific ST changes https://youtu.be/LTUaLd2R7js Episode Schematics & Teaching Click to enlarge ??? The CardioNerds 5! – 5 major takeaways from the #CNCR case When thinking about the etiology of a restrictive cardiomyopathy, we can organize the causes into four major categories:  Infiltrative (e.g., amyloidosis, sarcoidosis)  Storage diseases (e.g., hemochromatosis, Fabry’s)  Non-infiltrative (e.g., idiopathic, diabetic cardiomyopathy, genetic causes)  Endomyocardial (e.g., endomyocardial fibrosis, hypereosinophilic syndrome)  On examination, patients with restrictive cardiomyopathy may have prominent right-sided symptoms, including hepatomegaly, ascites, and significant peripheral edema. On exam, neck veins may demonstrate a steep y descent, and cardiac auscultation may have a +S4, and murmurs of tricuspid and mitral regurgitation. A multimodal imaging workup is recommended for evaluating RCM.   TTE: Typically demonstrates normal LVEF, normal chamber volumes, biatrial enlargement, and restrictive diastolic filling patterns (.e.g, E/A > 2, E/e’ > 14, decreased mitral deceleration time < 150 ms). We can see increased wall thickness with infiltrative processes and storage diseases. cMRI: specific patterns of Late Gadolinium Enhancement may indicate certain pathology (e.g., amyloid may demonstrate diffuse subendocardial, heterogeneous, or transmural signal). T2 signal can identify inflammation and quantify iron.  PYP scan: It has a >99% sensitivity for cardiac ATTR amyloid. If monoclonal gammopathy excluded, PPV 100%.  Invasive hemodynamics can show physiology consistent with RCM. This includes square root sign, equalization of diastolic pressures, and concordance during respiration of LV/RV pressure changes. In contrast, constrictive physiology will yield discordant respirophasic LV/RV pressure changes. Endomyocardial biopsy may be necessary to identify etiology but the yield for patchy diseases, like sarcoid, can be low.  Patients with end-stage RCM can be difficult to manage medically, especially as they become refractory to diuretics. They poorly tolerate antihypertensive agents (due to inability to augment limited stroke volume), heart rate lowering medication (due to low SV, they are dependent on HR to maintain CO which is HR x SV), and have a very limited optimal preload window (need high filling pressures to fill the stiff ventricles but also have symptoms of fluid overload). Due to anatomic considerations, patients are rarely candidates for durable left ventricular assist devices and often require orthotopic heart transplant. However, patients with RCM have higher waitlist mortality and longer wait times, in part due to lower utilization of MCS.    Educational Video https://youtu.be/LDgP_3Cv49w Produced by Dr. Karan Desai https://twitter.com/CBlumenthal2/status/1299288171981598722?s=20 References Spectrum of Restrictive & Infiltrative CM – Part 1 article  Spectrum of Restrictive & Infiltrative CM – Part 2 article  Spectrum of Restrictive & Infiltrative CM – Part 1 notes  Spectrum of Restrictive & Infiltrative CM – Part 2 notes  Restrictive CM – Circ Res 2017  Echocardiography Diagnostic Criteria for Constriction  Differentiation of Constriction and Restriction: Complex Cardiovascular Hemodynamics  Constrictive Pericarditis Versus Restrictive Cardiomyopathy?  Does Survival on the Heart Transplant Waiting List Depend on the Underlying Heart Disease?  Genetic Counseling in Inherited Cardiomyopathies  2016 ASE Echo Guidelines  CardioNerds Case Reports: Recruitment Edition Series Production Team Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD