Cardionerds - A Cardiology Podcast:

Congenital Heart Disease Page

Welcome to the CardioNerds Adult Congenital Heart Disease Series Page! 

With landmark advances in the care of children with congenital heart disease, survival has improved, and more are living into adulthood. There are now more adults living with congenital heart disease than children. This is no longer a pediatric specialty alone. This growing population requires a specialized and personalized approach from multidisciplinary teams. While not every CardioNerd will specialized in ACHD, we will all have the opportunity to touch the lives of adults living with CHD, recognize their unique needs, and refer them to the appropriate centers if and when needed. 

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 SaefDr. 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. 

Episodes

Congenital heart disease is the most common birth defect, affecting 1 in 100 babies. Amongst these ventricular septal defects are very common with the majority of patients living into adulthood. In this episode we will be reviewing key features of VSDs including embryologic origin, anatomy, physiology, hemodynamic consequences, clinical presentation and management of VSDs. Dr. Tommy Das (CardioNerds Academy Program Director and FIT at Cleveland Clinic), Dr. Agnes Koczo (CardioNerds ACHD Series Co-Chair and FIT at UPMC), and Dr. Anu Dodeja (Associate Director for ACHD at Connecticut Children’s) discuss VSDs with expert faculty Dr. Keri Shafer. Dr. Shafer is an adult congenital heart disease specialist at Boston Children’s Hospital, and an assistant professor of pediatrics within Harvard Medical School. She is a medical educator and was an invited speaker for the inaugural CardioNerds Sanjay V Desai Lecture, on the topic of growth mindset. Script and notes were developed by Dr. Anu Dodeja. Audio editing by CardioNerds Academy InternShivani Reddy.

CardioNerds (Dan Ambinder), episode lead Dr. Sarah Fahnhorst (ACHD Cardiologist at Spectrum Health in Grand Rapids, Michigan), and series co-chair Dr. Agnes Koczo (fellow at UPMC) learn about ASD from Dr. Richard Krasuski (ACHD Cardiologist and Director of ACHD at Duke University). Audio editing by CardioNerds Academy Intern, student doctor Adriana Mares

An atrial septal defect (ASD) is a common congenital heart disease most often diagnosed in childhood, but initial presentation can be in adulthood. ASDs are abnormal communications between the left and the right atrium.  There are four types of ASDs with different embryologic origins. If the defects are large, they will require percutaneous or surgical closure. Unrepaired defects can lead to symptoms of shortness of breath, exercise intolerance, recurrent chest infections, or pulmonary hypertension. This episode of CardioNerds will review the natural history, embryologic origin, diagnostic modalities/findings, indication for closure and long term complications of repaired and unrepaired atrial septal defects. 

CardioNerds Dr. Josh SaefDan Ambinder, join Dr. Jim Kimber and interview experts Dr. Adrienne Kovacs, and Dr. Lauren Lastinger and discuss behavioral health needs and psychosocial wellbeing in the congenital heart disease population. In this episode, our experts tackle issues surrounding mental and behavioral health including anxiety/depression, ADHD, neurodevelopmental disabilities, psychosocial challenges, stressors unique to patients with ACHD and their families, and how the healthcare system can better optimize mental health care for the CHD patient population. Audio editing by CardioNerds Academy InternPace Wetstein.

CardioNerds (Daniel Ambinder) and ACHD series co-chair Dr. Dan Clark discuss advanced heart failure therapies including mechanical circulatory support (MCS) and heart transplantation (HT) in patients with adult congenital heart disease (ACHD) with Dr. Rafael Alonso-Gonzalez, cardiologist and director of Adult Congenital Heart Disease program at the University of Toronto and ACHD fellow Dr. Andy Pistner (University of Washington). They cover epidemiology of heart failure in ACHD, outcomes after HT, unique challenges of HT in this population, impact of allocation policies on access to transplantation, and regionalization of advanced heart failure care. They also discuss a practical approach to advanced heart failure therapy evaluation in ACHD. Audio editing by CardioNerds Academy Intern, student doctor Adriana Mares.

CardioNerd (Amit Goyal),  ACHD series co-chair Dr. Agnes Koczo (UPMC), and episode FIT lead, Dr. Logan Eberly (Emory University, incoming ACHD fellow at Boston Adult Congenital Heart) join Dr. Peter Ermis (Program Director of the Adult Congenital Heart Disease Program at Texas Children’s Heart Center), and Dr. Scott Cohen (Associate Professor and Director of the Adult Congenital Heart Disease Program at the Medical College of Wisconsin) for a discussion about transitions of care in congenital heart disease.

Congenital heart disease (CHD) is the most common clinically significant congenital defect, occurring in approximately 1 in 100 live births. With modern advances in pediatric cardiology and cardiac surgery, over 90% of children born in the developed world with CHD will now survive into adulthood, and there are currently more adults than children living with CHD in the United States1.

As these children become adults, they will need to transition their care from pediatric to adult-centered care. Unfortunately, during this transition period, there is often delayed or inappropriate care, improper timing of the transfer of care, and undue emotional and financial stress on the patients, their families, and the healthcare system. At its worst, patients are lost to appropriate follow-up. In this episode, we review the current climate in transitions of care for CHD patients from child-centered to adult-centered care, discuss the difficulties that can occur during the transitions process. We further discuss how to mitigate them, and highlight the key elements to the successful transitions of care.

CardioNerds (Daniel Ambinder), ACHD series co-chairs,  Dr. Josh Saef (ACHD fellow, University of Pennsylvania) Dr. Daniel Clark (ACHD fellow, Vanderbilt University), and ACHD FIT lead Dr. Jon Kochav (Columbia University) join Dr. Eric Krieger (Director of the Seattle Adult Congenital Heart Service and the ACHD Fellowship, University of Washington) to discuss multimodality imaging in congenital heart disease. Audio editing by CardioNerds Academy InternDr. Maryam Barkhordarian.

In this episode we discuss the strengths and weaknesses of the imaging modalities most commonly utilized in the diagnosis and surveillance of patients with ACHD.  Specifically, we discuss transthoracic and transesophageal echocardiography, cardiac MRI and cardiac CT. The principles learned are then applied to the evaluation of two patient cases – a patient status post tetralogy of Fallot repair with a transannular patch, and a patient presenting with right ventricular enlargement of undetermined etiology.

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 ACGMEadult congenital heart disease fellowship at Stanford. ), to discuss Tetrology of Fallot. Audio editing by CardioNerds Academy InternDr. 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.

CardioNerds (Amit Goyal and Daniel Ambinder), ACHD series co-chair Dr. Daniel Clark (Vanderbilt University), and ACHD FIT lead Dr. Danielle Massarella (Toronto University Health Network) join ACHD expert Dr. Yuli Kim (Associated Professor of Medicine & Pediatrics at the University of Pennsylvania), to discuss single ventricular heart disease and Fontan palliation. They cover the varied anatomical conditions that can require 3-step surgical palliation culminating in the Fontan circulation, which is characterized by passive pulmonary blood flow, high venous pressures, and low cardiac output. Audio editing by Dr. Gurleen Kaur (Director of the CardioNerds Internship and CardioNerds Academy Fellow). 

In this episode, CardioNerds (Amit Goyal), ACHD series co-chair,  Dr. Josh Saef (ACHD fellow at University of Pennsylvania) and episode lead fellow, Dr. Brynn Connor (Pediatric Cardiology fellow at Lucile Packard Children’s Hospital at Stanford) are joined by Dr. Maan Jokhadar (Advanced heart failure and adult congenital heart disease specialist at Emory University) to discuss transposition of the great arteries. Audio editing by CardioNerds Academy InternDr. Maryam Barkhordarian.

For a brief review of the basic anatomy and physiology of D-TGA, check-out this great video by Dr. Maan Jokhadar!

CardioNerds (Amit Goyal and Daniel Ambinder),  ACHD series co-chair Dr. Agnes Koczo (UPMC), and episode FIT lead, Dr. Natasha Wolfe (Washington University) join Dr. Ari Cedars   (Director of the Adult Congenital Heart Disease Program at Johns Hopkins) for a discussion about coarctation of the aorta.  

In this episode we discuss the presentation and management of unrepaired and repaired coarctation of the aorta in adults. We discuss the unique underlying congenital anatomy of coarctation and how that impacts physiology, clinical presentation, and diagnostic findings. We discuss the importance of long-term routine follow-up and screening of patients (including those who have been “repaired”) for common complications such as hypertension, re-coarctation, and aneurysm development. We end with a discussion of treatment options for coarctation and its complications.

Audio editing by CardioNerds Academy InternDr. Maryam Barkhordarian.

CardioNerds (Amit Goyal and Josh Saef) join ACHD fellow Dr. Prashanth Venkatesh and ACHD program director Dr. Jeannette Lin, both from the University of California, Los Angeles, for a deep dive into the complex disease entity that is Ebstein anomaly. They discuss the anatomic features of the dysplastic tricuspid valve as well as the right ventricle in patients with Ebstein anomaly, and how these structural features affect cardiovascular physiology and clinical presentation. This is followed by an in-depth discussion into associated entities including arrhythmias and atrial-level shunts as well as the appropriate multimodality evaluation. Finally, they tackle the difficult question of when and how to intervene, delving into the various interventional treatments and exploring their outcomes using illustrative case-based examples. Audio editing CardioNerds Academy Intern, Pace Wetstein.

CardioNerds (Amit Goyal and Daniel Ambinder), ACHD series co-chair Dr. Agnes Koczo (UPMC), and ACHD FIT lead Dr. Katia Bravo (UCLA) join ACHD expert Dr. Carole Warnes (Professor of Medicine and founder of the Adult Congenital Heart Disease Clinic at Mayo Clinic), to discuss adult congenial heart disease and pregnancy. They cover preconception counseling in women with congenital heart disease, appropriate risk stratification to estimate maternal and neonatal morbidity using existing tools and an individualized care approach and preparation for a multidisciplinary delivery plan. Audio editing by CardioNerds Academy InternDr. Leticia Helms.

CardioNerds ACHD Collection (Includes all ACHD episodes)

Meet Our Collaborators!

Adult Congenital Heart Association

Founded in 1998, the Adult Congenital Heart Association is an organization begun by and dedicated to supporting individuals and families living with congenital heart disease and advancing the care and treatment available to our community. Our mission is to empower the congenital heart disease community by advancing access to resources and specialized care that improve patient-centered outcomes. Visit their website (https://www.achaheart.org/) for information on their patient advocacy efforts, educational material, and membership for patients and providers

ChiP Network

CHiP Network

The CHiP network is a non-profit organization aiming to connect congenital heart professionals around the world. Visit their website (thechipnetwork.org) and become a member to access free high-quality educational material, upcoming news and events, and the fantastic monthly Journal Watch, keeping you up to date with congenital scientific releases. Visit their website (https://thechipnetwork.org/) for more information.

Heart University

Heart University

Heart University aims to be “the go-to online resource” for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of educational material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practicing provider. The site provides free content to a global audience in two broad domains: (1) A comprehensive curriculum of training modules and associated testing for trainees. (2) A curated library of conference and grand rounds recordings for continuing medical education. Learn more at www.heartuniversity.org/.

Adult Congenital Heart Disease Series Co-Chairs!

Dr. Josh Saef

Dr. Josh Saef

University of Pennsylvania

Josh is a first year ACHD Fellow at the University of Pennsylvania. He trained at Washington University in St Louis for med school and internal medicine residency, then at the Cleveland Clinic for cardiology. He’s is currently completing a master’s degree in clinical research, and is interested in data science applications in cardiology.
Dr. Agnes Koczo

Dr. Agnes Koczo

University of Pittsburgh Medical Center

Dr. Agnes Koczo is a third year cardiology fellow at the University of Pittsburgh Medical Center. She is interested in cardio-obstetrics and adult congenital heart disease. She plans to pursue a T32 postdoctoral research fellowship studying immune dysregulation in patients with cardiac complications in pregnancy.

 
 

Dr. Dan Clark

Vanderbilt University Medical Center

Dan Clark @DanClarkMD is a cardiologist with advanced imaging training who is currently in ACHD fellowship at Vanderbilt. He attended Northwestern University for his undergraduate education, then completed the combined MD/MPH at the University of Texas Health Science Center in San Antonio (UTHSCSA). He was a Med-Peds resident at Vanderbilt University and then stayed on to complete his adult cardiology training, where he served as chief fellow during his final year. During ACHD fellowship, he has a secondary faculty appointment as a Clinical Instructor in the Department of Medicine to read and teach Cardiac MRI. He is also currently finishing his 2nd year on a NIH T32 grant working on imaging-based research spanning topics from congenital heart disease, sports cardiology, and cardiovascular sequelae assessed by CMR among patients recovering from COVID-19. He is the husband to a wonderful Pediatrician who works in public health, dad to two playful kids, and an outdoors enthusiast. When not in the hospital, you can find him kayaking, hiking, or running with his dog.