A 47-year-old man presents to the clinic for a follow-up visit after a recent ablation for atrial flutter (AFL). He has tetralogy of Fallot (TOF) that was repaired in infancy and included a ventriculotomy with a transannular patch. He subsequently had a surgical pulmonary valve (PV) repair at 15 years of age, followed by a transcatheter pulmonary valve replacement (TPVR) with a 22 mm Melody Transcatheter Pulmonary Valve (Medtronic, Minneapolis, Minnesota) in 2008.
At 42 years of age, he had nonsustained ventricular tachycardia (NSVT) and received a single-chamber implantable cardioverter-defibrillator (ICD) for primary prevention. At 43 years of age, he underwent a ventricular tachycardia (VT) ablation; since then, he has not had recurrent VT. At 46 years of age, he developed AFL, which was diagnosed after an inappropriate shock attributed to AFL conducting 1:1 at his set VT rate threshold.
During discussion about the management of his arrhythmias, he states that he would also like to discuss competitive exercise. He does not endorse any recent cardiorespiratory symptoms and is training for an upcoming Tough Mudder (Spartan Race Inc., Boston, Massachusetts) event, a 5 km race that includes rough terrain and obstacles.
An echocardiogram during his clinic visit reveals normal biventricular systolic function, moderate pulmonary stenosis, moderate PV regurgitation, and no residual shunting defects. His last cardiac magnetic resonance imaging, obtained 1 year before his presentation to the clinic, revealed right ventricular (RV) ejection fraction 58%, RV end-diastolic volume indexed 117 mL/m2, and left ventricular (LV) ejection fraction 48%. Interrogation of his ICD reveals a normally functioning device without any detected arrhythmias or delivered therapies following his ablation. An exercise stress test with standard Bruce protocol does not demonstrate any arrhythmias or signs of ischemia. His maximum heart rate is 137 bpm (76% predicted), his peak blood pressure is 140/60 mm Hg, and he does not have any desaturations. The resting electrocardiogram before exertion demonstrated stable normal sinus rhythm with a right bundle branch block (QRS 120 msec). His peak oxygen consumption (VO2) is 43.2 mL/kg/min (88% predicted), respiratory exchange ratio is 1.14, minute ventilation/carbon dioxide production is 24.6, and peak oxygen pulse is 23 mL/beat.
He asks to be cleared for this event and is confident that he can participate.
In this patient with repaired tetralogy of Fallot (rTOF), subsequent TPVR, and arrhythmia history with an ICD, which one of the following is the most appropriate guidance in addressing his risk of participating in a high-endurance obstacle race?
Show Answer
The correct answer is: D. Discuss his case with the event athletic trainer, ensure an automated external defibrillator (AED) is present, and ensure event leaders are aware of and agree to his participation.
Discussing this patient's case with the event athletic trainer, ensuring an AED is present, and ensuring event leaders are aware of and agree to his participation would be the most appropriate guidance in this case. TOF is the most common cyanotic congenital heart disease (CHD), with prevalence estimated to be 3.85 of every 10,000 births, and is the most common lesion for which infants undergo palliative repair.1 The predominant defects constituting TOF include obstruction of flow through the right ventricular outflow tract (RVOT), a ventricular septal defect, and an overriding aorta. Patients with TOF have varying degrees of cyanosis depending on the severity of RVOT stenosis and pulmonary artery anatomy. Most infants undergo a complete repair between 4 and 6 months of age on cardiopulmonary bypass. This repair has excellent short-term and long-term survival rates, especially in the most recent surgical eras, with 30-year survival rates estimated up to 90%.2 Despite the expectancy that these patients will live into adulthood, they often experience multiple complications over time, including pulmonary regurgitation, severe RV enlargement and dysfunction, aortic root enlargement, and the occurrence of arrhythmias that can lead to sudden cardiac death (SCD). Known risks of SCD in rTOF include LV systolic or diastolic dysfunction, NSVT, QRS duration ≥180 msec, extensive RV scarring, and inducible sustained VT on electrophysiology study.3 There are known coronary abnormalities that occur in TOF in approximately 5-7% of cases, although most coronary arrangements are normal and do not carry an inherently increased risk of coronary artery disease that may be detected by exercise stress testing.4
For young adults with CHD, exercise capacity and participation in competitive sports are important considerations. Sports might contribute to improved quality of life and life expectancy. Poor exercise capacity in adults with congenital heart disease (ACHD) increases both morbidity and mortality risks; VO2 <65% predicted in patients with rTOF indicates an increased risk of death or sustained VT.5,6 Exercise is important to mitigate SCD risk and to cultivate a healthy lifestyle in patients with ACHD. In general, activity restrictions should be individualized to each patient. For patients with rTOF, those with a good initial repair without residual RV outflow obstruction or shunting defects can participate in various activities without restriction.
The most recent Bethesda guidelines from 2015 suggest that athletes with rTOF without significant ventricular dysfunction or outflow tract obstructions may be considered for participation in moderate-intensity to high-intensity sports, especially if exercise testing is without evidence of exercise-induced arrhythmias, hypotension, ischemia, or other concerning clinical symptoms.7 These guidelines do, however, suggest that athletes with rTOF who have a history of recurrent or uncontrolled ventricular arrhythmias be restricted from all competitive sports, with the possible exception of low-intensity sports. In addition, historical expert opinion suggests that those with moderate-to-severe hemodynamic abnormalities such as severe pulmonary insufficiency and/or RV systolic pressure from at least one-half to two-thirds the systemic pressure with normal biventricular function are generally recommended not to do more than light or low-intensity exercise (e.g., hiking, golfing, or bowling), although this is opinion without a large amount of supporting literature.
Overall, although he had an ICD, his biventricular function was normal, he did not have frequent arrhythmia recurrences, and his VO2 on exercise testing (88% predicted) was reassuring. This case highlights that many clinical circumstances patients with ACHD find themselves in regarding participation in competitive athletics or moderate-intensity to high-intensity exertion pose a need for a comprehensive approach to guidance. In this case, there was not enough evidence to recommend full unrestricted participation or restriction. Thus, a focus should be placed on the concept of shared decision making, in which key stakeholders in the patient's success are involved and able to share their expertise to ensure proper patient outcomes.
References
Centers for Disease Control and Prevention (CDC). Improved national prevalence estimates for 18 selected major birth defects--United States, 1999-2001. MMWR Morb Mortal Wkly Rep 2006;54:1301-5.
Cuypers JA, Menting ME, Konings EE, et al. Unnatural history of tetralogy of Fallot: prospective follow-up of 40 years after surgical correction. Circulation 2014;130:1944-53.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019;73:e81-e192.
Gupta D, Saxena A, Kothari SS, et al. Detection of coronary artery anomalies in tetralogy of Fallot using a specific angiographic protocol. Am J Cardiol 2001;87:241-4, A9.
Müller J, Hager A, Diller GP, et al. Peak oxygen uptake, ventilatory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol 2015;196:158-64.
Diller GP, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005;112:828-35.
Van Hare GF, Ackerman MJ, Evangelista JK, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015;66:2372-84.
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