CardioNerds (Amit Goyal and Daniel Ambinder), ACHD series co-chairs Dr. Dan Clark and Dr. Josh Saef, and ACHD FIT lead Dr. J.D. Serfas (Duke University) and Cardiology Fellow Dr. Victoria Thomas (Vanderbilt University) join ACHD experts Dr. Jamil Aboulhosn (Professor of Medicine at UCLA and the director of the Ahmanson/UCLA Adult Congenital Heart Disease Center) and Dr. Joanna Ghobrial, Medical and Interventional Director of the Adult Congenital Heart Disease Center at Cleveland Clinic. They discuss common ACHD pathologies that benefit from interventional procedures such as transcatheter pulmonic valve replacement (TPVR) and share new advancements in transcatheter approaches to correct sinus venosus defects. They end with a brief discussion on how to become an adult cardiology interventionalist that performs ACHD interventions. Episode notes were drafted by Dr. Victoria Thomas. Audio editing by CardioNerds Academy Intern, student doctor Akiva Rosenzveig.
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
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Pearls - ACHD: Interventional Cardiology
The ductus arteriosus, which is formed from the distal portion of the left sixth arch, is key to fetal circulation because it allows blood to bypass
Transcatheter pulmonic valve replacement (TPVR) is a treatment for many ACHD patients that can spare them repeat sternotomies. This is important as many ACHD patients hava already undergone multiple surgeries in their childhood.
Before any ACHD cardiology intervention, appropriate imaging (TEE, TTE, Cardiac MRI, Cardiac CTA, and/or 3D printing) is imperative to understanding the relevant anatomy and hemodynamics to guide procedural indication and planning.
As with other structural interventions, consider a SENTINEL device (cerebral embolic protection system) to provide embolic protection in procedures that could lead to debris/embolic dislodgement when appropriate.
Sinus venosus defects can be repaired via a transcatheter approach with a covered stent in the superior vena cava (SVC).
Consider using 3D printing or 3D digital imaging when preparing for complex ACHD interventions.
Notes- ACHD: Interventional Cardiology
1. When considering a patient for TPVR there are 3 types of landing zones for pulmonic valves in ACHD patients:
Pulmonary conduits or homografts. These are typically seen in patients with TOF or prior Ross or Rastelli procedure. These may be calcified and stenotic and so pre-dilatation is often needed before valve replacement.
Bioprosthetic Valves. (Valve in Valve TPVR)
Native outflow tract
2. What are some of the more severe complications to consider when talking to an ACHD patient about a TPVR?
Coronary artery compression
Conduit rupture
Vessel injury (including the pulmonary bed)
Valve embolization
Endocarditis
3. What are some of the hemodynamic measurements one would want to pay attention to in a patient with a Fontan heart?
You will see higher CVPs in patients with a Fontan palliation.
