Dyspnea, edema, and syncope 35 years after "total correction" of tetralogy of Fallot
D. Luke Glancy, MD, Theresa A. Mills, MD, V. Bibb Saye, MD, and Jay L. Hollman, MD
A man had had an operation for tetralogy of Fallot at age 5. He was much improved for many years, but when he presented at age 40, he indicated that approximately 4 years earlier he had begun to note exertional dyspnea and ankle edema. Two and a half years earlier, he had had atrial flutter, but since then he had been in sinus rhythm. He had several syncopal episodes, but none in the previous 6 to 12 months.
Examination revealed elevated neck veins, ankle edema, a systolic ejection murmur in the second left intercostal space, and a short low-pitched diastolic murmur along the left sternal edge. The electrocardiogram (Figure 1) showed sinus rhythm, a P-R interval at the upper limit of normal (0.20 seconds), and right bundle branch block with an extremely long QRS duration of 0.21 seconds. Salient features of the echocardiogram were a huge right atrium with the atrial septum bulging into the much smaller left atrium and an enormous right ventricle that was moderately hypokinetic (Figure 2). The smaller left ventricle looked normal except for the abnormal septal motion associated with the volume-loaded right ventricle (Figure 3). Doppler examination revealed severe pulmonic regurgitation (Figure 4) and only trivial tricuspid regurgitation.
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