Abstract
Introduction: Physiologically corrected transposition of the great arteries [(S,L,L) TGA] is a rare congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Case Presentation: A 28-year-old man with (S,L,L) TGA, pulmonary valve (PV) stenosis, ventricular septal defect (VSD), and history of Kawasaki disease who presented for a gradual decline in exercise tolerance. Oxygen saturation was 96% at rest and dropped to 86% with ambulation. Hemoglobin was 17.7 g/dl, EKG showed no bradyarrhythmias. On stress test, peak heart rate was 159 beats/minute, and peak VO 2 22.6 ml/kg/min (83%, and 57% of predicted value respectively). Spirometry was normal. Transthoracic echocardiogram revealed biventricular hypertrophy with normal systolic function, a bicuspid PV with severe stenosis (peak and mean gradient of 155 and 90 mmHg respectively), large inlet VSD with bidirectional flow and mild systemic TV regurgitation. Coronary CTA showed no coronary artery aneurysms. Cardiac catheterization suggested hypoxia due to pulmonary-to-systemic shunting across the VSD with Q p /Q s = 0.86 (Figure). Consideration should be given to performing a high-risk surgical repair versus medical management. Conclusions: Natural history, symptoms, and timing of intervention are determined by the associated cardiac anomalies and the progressive dysfunction of the systemic right ventricle (SRV). Anatomic repair to restore the left ventricle as a systemic pump is a very high-risk procedure in adults. Functional repair maintains SRV, repair the VSD, and PV stenosis has a poor late outcome. Medical management ensures iron stores are repleted, provide empirical use of pharmacological heart failure therapy, and referral to heart transplantation when SRV systolic dysfunction ensues. Clinicians need to know the potential complications in both unoperated patients and following various surgical repairs to recommend appropriate treatment options.
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