Case 3/2014 - 26-year-old man with pulmonary atresia and ventricular septal defect in late postoperative evolution.

Abstract

Clinical data: Surgical correction of pulmonary atresia with ventricular septal defect and aortopulmonary collateral arteries was performed in three steps. The first one, with 19 months, with the unification of major collateral artery in the right upper lobe (8 mm) to the right pulmonary artery (RPA), which nurtured the right lower lobe, and Blalock-Taussig anastomosis from the right subclavian artery with its own collateral artery. The second one, with 27 months, with unification of the collateral artery to the left lower lobe with the left pulmonary artery (LPA) of 4 mm and Blalock-Taussig to the left with 6 mm tube in the pulmonary hilum. The third one, with nine years, with no. 18 valved corrugated pericardium tube placement and expansion of LPA and RPA anastomosis with right upper lobe artery. Immediate evolution was considered good, with pulmonary systolic pressure of 22 mmHg. Late evolution was good until the age of 26, remaining without symptoms and without medication. Physical examination: Good general condition, eupneic, acyanotic, normal pulses. Weight: 74 kg; height: 183 cm; BP = 105/70 mmHg; HR: 86 bpm. Aorta discreetly palpated with mild systolic murmur at the suprasternal notch. In the precordium, apical impulse was not palpable and there were no systolic impulses. The heart sounds were normal and moderate systolic murmur of ++ intensity was harsh across the left sternal border (LSB), not accompanied by fremitus. Diastolic murmur was mild, +/++, at the LSB. The liver was not palpable and the lungs were clear.