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Unrepaired tetralogy of fallot with right hemitruncus in an adult: a rare case.

2007; National Institutes of Health; Volume: 34; Issue: 2 Linguagem: Inglês

Rajiv Patel, Ramzan M. Zakir, Virender Sethi, Jayant N. Patel, John Apovian, John C. Alexander, Marc Klapholz, Muhamed Sarić,

Tracheal and airway disorders

A 36-year-old immigrant from El Salvador, who had had a heart murmur since childhood, presented after recent-onset dyspnea on exertion and several episodes of “turning blue.” A physical examination revealed a resting oxygen saturation of 98% on room air, blood pressure of 114/70 mmHg, and a regular heart rate of 76 beats/min. There was a normal S1, an absent P2, and a grade 4/6 systolic ejection murmur with a palpable thrill along the left mid-sternal border. There was no evidence of peripheral or central cyanosis, but clubbing was present. The patient's hemoglobin concentration was 20 g/dL (hematocrit, 58%). An electrocardiogram showed sinus rhythm, right-axis deviation, right atrial enlargement, and the Katz-Wachtel phenomenon (a large, equiphasic voltage in midprecordial leads), indicating biventricular hypertrophy (Fig. 1). Fig. 1 An electrocardiogram illustrates the Katz-Wachtel phenomenon (a large, equiphasic QRS voltage in midprecordial leads), indicating biventricular hypertrophy. Transthoracic echocardiography was consistent with a diagnosis of tetralogy of Fallot: there was an overriding aorta, right ventricular hypertrophy, pulmonic stenosis (peak gradient of at least 72 mmHg), and a perimembranous ventricular septal defect (Fig. 2) with a minimal bidirectional shunt on color-flow Doppler. Cardiac catheterization revealed a 90-mmHg gradient in the infundibulum below the pulmonic valve. Aortography showed a right-sided aortic arch and the anomalous origin of the right pulmonary artery from the ascending aorta (right hemitruncus; Fig. 3). The left pulmonary artery was the sole branch of the main pulmonary trunk (Fig. 4). Fig. 2 Transthoracic echocardiography shows tetralogy of Fallot: a perimembranous ventricular septal defect (arrow), an overriding aorta (Ao), and right ventricular (RV) hypertrophy. Fig. 3 Iodine contrast injection into the aortic root (Ao) reveals the anomalous origin of the right pulmonary artery (arrows) from the proximal ascending aorta. Fig. 4 Iodine contrast injection into the main pulmonary artery (MPA) shows only the left pulmonary artery (LPA) with its branches. The right pulmonary artery (asterisk) is not opacified. The patient underwent repair of the ventricular septal defect and resection of the infundibular obstruction; the right hemitruncus was left in place. Six months later, he was acyanotic, with normal right ventricular pressures and markedly improved overall functional capacity.