Exploring the Passion and Complexity of Adult Congenital Heart Disease

CardioNerds (Daniel Ambinder), ACHD series co-chair,  Dr. Josh Saef (ACHD fellow at University of Pennsylvania), and ACHD FIT lead Dr. Charlie Jain (Mayo Clinic) join ACHD expert Dr. George Lui (Medical Director of The Adult Congenital Heart Program at Stanford and Program Director for the ACGME adult congenital heart disease fellowship at Stanford) to discuss Tetrology of Fallot. Audio editing by CardioNerds Academy Intern, Dr. Leticia Helms. Tetralogy of Fallot (ToF) is the most common cyanotic heart disease and one of the most common congenital heart diseases that we see in adults overall. The anatomy includes a ventricular septal defect (VSD), an overriding aorta, and infundibular hypertrophy with subpulmonic +/- pulmonic valvular +/- supravalvular stenosis, which causes severe RV outflow obstruction and subsequent RV hypertrophy. Patients require surgery during childhood, which includes patching the VSD and relieving RV outflow obstruction. This results in pulmonic regurgitation (usually severe) and patients can live with this for decades. Adults with ToF commonly will require pulmonic valve replacement, potential relief of subvalvular or supravalvular stenoses, and tricuspid valve repair (for functional tricuspid regurgitation caused by RV dilation). These patients are at increased risk of atrial and ventricular arrhythmias and may warrant prophylactic ICDs. The CardioNerds Adult Congenital Heart Disease (ACHD) series provides a comprehensive curriculum to dive deep into the labyrinthine world of congenital heart disease with the aim of empowering every CardioNerd to help improve the lives of people living with congenital heart disease. This series is multi-institutional collaborative project made possible by contributions of stellar fellow leads and expert faculty from several programs, led by series co-chairs, Dr. Josh Saef, Dr. Agnes Koczo, and Dr. Dan Clark. The CardioNerds Adult Congenital Heart Disease Series is developed in collaboration with the Adult Congenital Heart Association, The CHiP Network, and Heart University. See more Disclosures: None Pearls • Notes • References • Guest Profiles • Production Team CardioNerds Adult Congenital Heart Disease PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Pearls - Tetralogy of Fallot Tetralogy of Fallot is the most common cyanotic heart disease and the 4 anatomic features are: VSD, infundibular hypertrophy (with RVOT obstruction), overriding aorta, and RV hypertrophy. The most common lesion you will see in adults with repaired Tetralogy of Fallot is pulmonic regurgitation.Pulmonic regurgitation (PR) can be easy to miss on exam as the murmur is brief and even shorter when the PR is severe. In patients with PR and aortic regurgitation, remember PR is clearest when laying supine, in comparison to aortic regurgitation which is loudest while leaning forward.Patients with ToF may also have coronary anomalies (e.g. LAD off RCA), right-sided aortic arches, and also left-sided heart disease (LV diastolic or systolic dysfunction).Patients with ToF are at risk for atrial and ventricular arrhythmias, and clinicians should consider prophylactic ICD for those with multiple risk factors for sudden death (e.g. QRS >180ms, scar on MRI).In all patients with congenital heart disease, inspection is a key part of the physical exam (e.g. right thoracotomy could clue you into a prior BTT shunt) and in patients with prior BTT shunts and/or prior brachial cut-downs (look in the antecubital fossa for scars), radial arterial access is discouraged. Show notes - Tetralogy of Fallot LesionTTETEECardiac MRICardiac CTTetralogy of Fallot(1) Routine assessment of RV and LV size and function (2) Routine semiquantitative assessment of pulmonic valve regurgitation (3) Evaluation of PVR/conduit gradients,