339. ACHD: Electrophysiology in ACHD with Dr. Frank Fish
Oct 25, 2023
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Dr. Frank Fish, a Pediatric Electrophysiologist, discusses electrophysiology in adults with congenital heart disease, highlighting the challenges of managing arrhythmias and the importance of proactive management. The podcast covers case studies and treatment decision-making, along with techniques and challenges in accessing the atria. It also explores the risk of atrial arrhythmias in patients with congenital heart disease and emphasizes the complexity of arrhythmias in this population. In addition to cardiology, the guest shares his passion for extractions and guitar playing.
Assessing myocardial health is crucial in managing arrhythmias in adults with congenital heart disease (ACHD) due to the impact of acquired scar fibrosis.
Treatment of atrial arrhythmias in ACHD patients requires considerations of unique risk factors, such as residual shunts and scar tissue.
The selection of patients for cardiac resynchronization therapy (CRT) in ACHD population should take into account the individual's underlying anatomy and challenges associated with lead implantation.
Deep dives
Summary of Case 1: Arrhythmias in ACHD Patients
Arrhythmias in adults with congenital heart disease (ACHD) can be intrinsic to the defects themselves or a consequence of interventions. These patients often have scars, valve replacements, conduits, and surgical scars, making it challenging to manage their arrhythmias. Dr. Fish emphasizes the importance of assessing myocardial health, since acquired scar fibrosis can impact arrhythmia development. The complexity of defects requires a flexible approach, adjusting strategies based on unexpected problems. The management of atrial arrhythmias in Fontan patients involves evaluating substrate, looking for atrial thrombi, and addressing access and expertise considerations for catheter ablation.
Summary of Case 2: Electrophysiology Issues in Tetralogy of Fallot Patients
Patients with Tetralogy of Fallot (TOF) may be at risk of arrhythmias due to scar tissue, RV dilation, and RV systolic dysfunction. Dr. Clark highlights the importance of considering the anatomy, electrical patterns, and surgical approach when deciding on ICD implantation in TOF patients. They mention the significance of QRSD duration, fractionated QRS, and the location of scar tissue and focal aneurysms in the decision-making process. Additionally, understanding the risk factors and substrates prior to pulmonary valve replacement is crucial for optimal management.
Summary of Case 3: ICD Placement in Tetralogy of Fallot Patients
Determining the need for primary prevention ICD in Tetralogy of Fallot patients relies on assessing the status of the right and left ventricles, electrical patterns, and surgical approach. Dr. Clark mentions the risk of sudden cardiac death (SCD) in the presence of dilated, dysfunctional right ventricles and septal motion impairing left ventricular filling. Evaluating the electrical pattern on the ECG, including QRSD duration and fractionation, can also help identify SCD risks. Furthermore, understanding the patient's surgical history and presence of anatomic ismosis is essential to make an informed decision on ICD implantation.
The Challenge of Treating Atrial Arrhythmias in Adult Congenital Heart Disease Patients
One of the main challenges in treating atrial arrhythmias in adult congenital heart disease (ACHD) patients is the complexity of their anatomy and prior surgical repairs. These patients often have unique risk factors for arrhythmias, such as residual intracardiac shunts and scar tissue surrounding the heart. Treating these arrhythmias requires a thorough understanding of the patient's individual anatomy, surgical history, and surface electrocardiogram (ECG) findings. Special considerations need to be taken when performing catheter ablation or device therapy in ACHD patients, such as addressing intracardiac shunts before implanting transvenous leads and being prepared to address unforeseen complications during the procedure. Despite the challenges, the complexity and variability in ACHD patients' arrhythmia substrates make this field of EP particularly stimulating and rewarding.
Device Therapy in Adult Congenital Heart Disease: CRT and Considerations
Device therapy, specifically cardiac resynchronization therapy (CRT), can be beneficial for certain adult congenital heart disease (ACHD) patients. The selection of patients who can benefit from CRT in the ACHD population requires a detailed understanding of the patient's underlying anatomy, prior repairs, and the potential challenges associated with implanting leads. In patients with systemic left ventricles, CRT has shown positive outcomes; however, patients with systemic right ventricles present more challenges due to difficulties in achieving effective resynchronization. In some cases, targeting the intrinsic conduction system or performing multisite pacing may be more suitable than conventional biventricular pacing. Each ACHD patient must be approached individually, considering their specific anatomical and physiological characteristics to determine the optimal device therapy strategy.
CardioNerds (Amit Goyal and Daniel Ambider) ACHD series co-chair Dr. Daniel Clark (Vanderbilt University), cardiology FIT lead Dr. Stephanie Fuentes (Houston Methodist Hospital), and Dr. Frank Fish, a Pediatric Electrophysiologist and the Director of the Pediatric Electrophysiology (EP) Lab at Monroe Carrell Jr Children’s Hospital at Vanderbilt University. He is a board certified Adult Congenital Heart Disease (ACHD) physician and has a wealth of experience performing EP procedures in adults living with congenital heart disease. Audio editing was performed by student Dr. Shivani Reddy.
In this episode, we discuss key concepts and management of electrophysiologic issues that we can encounter when caring for adults with congenital heart disease. Arrythmias in adults with congenital heart disease can be intrinsic due to the defect itself or as a consequence of the interventions that they have undergone to palliate and/or repair these defects. The complex anatomy of these patients and the years of pressure and volume load make them not only exquisitely hemodynamically sensitive to arrhythmias (that may otherwise not be of much consequence to the general population) but they also make interventions (catheter ablation or device implant) complex. We therefore embark in a case-based discussion of patients with ACHD (Fontan circulation, Ebstein’s anomaly and Tetralogy of Fallot) in an effort to highlight the presentation of arrythmias and the management strategy in this very important group of patients.
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
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Pearls - Electrophysiology in ACHD
Patients with Fontan circulation have a high risk of developing atrial (and ventricular) arrhythmias and they are highly sensitive to the hemodynamic consequences that these arrythmias ensue. The goal of therapy then should be to achieve sinus or atrial paced rhythm. Rate control should NOT the goal.
Patients with Ebstein’s anomaly have high arrhythmic potential. They can have multiple accessory pathways (especially right sided) which can in turn be associated with sudden cardiac death. We should have low threshold for EPS +/- catheter ablation in patients with WPW pattern.
Patients with Tetralogy of Fallot have a unique risk for SCD that warrant ICD implant apart from the standard criteria (LVEF <=35% and NYHA II-III symptoms). This involves the pump (RV dilation/dysfunction), electricity (QRSd>180 ms) and surgical repair approach.
Patient’s anatomy is the major consideration when implanting devices (PPM/ICD). We ought to assess for residual intracardiac shunt at the atrial level and consider closing if feasible prior to placing a device. CRT has merit in systemic LV but less so in systemic RV.
Notes- Electrophysiology in ACHD
What should we know about atrial arrhythmias in a Fontan patient?
Intraatrial re-entrant tachycardia (IART) is slower than typical atrial flutter with atrial rates generally
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