How to Image Repaired Tetralogy of Fallot
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A 45-year-old woman with repaired tetralogy of Fallot (rTOF) was admitted for evaluation of palpitations and lightheadedness. She underwent a Blalock–Taussig shunt in infancy followed by patch closure of the ventricular septal defect (VSD) and a transannualar right ventricular outflow tract (RVOT) patch at age 7 years. She was asymptomatic and without exercise limitations during the next 3 decades. A pediatric cardiologist followed her annually until college, but she did not establish cardiac care afterward. She presented at age 38 years with a nonproductive cough and fatigue. On review of systems, she noted gradual decline in her exercise capacity. On physical examination, her vital signs were normal, and she appeared comfortable. The pulse in the right arm was diminished and was normal in the left arm. Well-healed right thoracotomy and median sternotomy scars were noted. The jugular venous pressure was normal, and her lungs were clear. Cardiovascular examination revealed a right ventricular (RV) heave, normal S1, single S2, right-sided S3, and a grade 2 decrescendo diastolic murmur at the left lower sternal border. The liver was not enlarged and she did not have ascites.
An ECG revealed normal sinus rhythm with complete right bundle branch block and a QRS duration of 178 ms (Figure 1). A 24-hour Holter monitor noted sinus rhythm with occasional isolated premature ventricular beats. A chest radiograph showed cardiomegaly with enlargement of the right heart structures (Figure 2). An exercise stress test showed moderately depressed peak workload (136 W, 62% predicted) and peak oxygen consumption (20.3 mL kg−1 min−1, 65% predicted) with low-grade ventricular ectopy. An echocardiogram revealed a dilated RV, severe pulmonary regurgitation (PR; Figure 3), mild tricuspid regurgitation (TR) with an estimated RV systolic pressure of 30 mm Hg above right atrial pressure, and a maximum instantaneous Doppler gradient across the RVOT …