Caso 5/2010 - Criança de 7 anos, do sexo feminino, com tetralogia de fallot e agenesia da valva pulmonar

Abstract

Fallot tetralogy, absent pulmonary valve. main pulmonary arteries. Usually, this anomaly accompanies tetralogy of Fallot with annular pulmonary stenosis and interventricular septal defect. Differential diagnosisOther heart diseases associated with pulmonary valve regurgitation may also have manifestations similar to those previously described. Among them, we can mention pulmonary valve stenosis, heart diseases with pulmonary artery dilatation due to increased pulmonary blood flow, and also heart diseases accompanied by pulmonary arterial hypertension, all in the long term. Diagnostic confirmationThe clinical findings were decisive for the diagnosis of tetralogy of Fallot with absent pulmonary valve, particularly the systolic-diastolic murmur with mild hypoxemia (bidirectional shunt through the VSD) and, above all, the characteristic radiographic image.Echocardiography confirmed this diagnosis, showing significantly dilated pulmonary arteries with diameters of 24 mm on the right and 14 mm on the left. The pulmonary annulus was narrow, measuring 8 mm; there was also infundibular pulmonary stenosis with anterior deviation of the infundibular septum and a large subaortic VSD, with 15 mm of diameter. Pulmonary valve regurgitation and tricuspid valve regurgitation were also present. The ventricles were dilated (Figure 2).Cardiac catheterization showed similar pressures in both ventricles (80/10 mmHg), and the pressure in the main pulmonary artery (30/5 mmHg) evidenced severe pulmonary valve regurgitation, in addition to pulmonary valve stenosis. ManagementDuring surgery, ECC time was 160 min and anoxia time was 70 min. The infundibular stenosis was resected via the right atrium; the absent pulmonary valve was visualized via the pulmonary trunk, as remaining thick and retracted buds in a narrow pulmonary ring. The RV outflow tract was enlarged to the pulmonary trunk and the VSD was closed with bovine pericardium. Number-18 decellularized homograft was implanted in the pulmonary position, and the tricuspid regurgitation was repaired using annuloplasty.The patient had a favorable outcome and was discharged seven days later. Clinical dataThe patient presented with hypoxia in the neonatal period, and for this reason an early systemic-to-pulmonary shunt was indicated at 13 days of life. Shortness of breath since birth did not prevent her from performing full physical activity. She continued to present with mild cyanosis on exertion, although the Blalock-Taussig shunt had not been patent since she was one year old. The patient was receiving digoxin sinceas of three years of age.On physical examination, she was in good general state of health, with no respiratory distress or cyanosis and her pulses were normal. Wake arterial oxygen saturation was 88.0% and the aorta was not palpable at the suprasternal notch.The cardiovascular examination revealed slightly forceful precordial impulses at the left sternal border, and the apex impulse was palpable at the 4th left intercostal space. The heart sounds were accentuated, and a grade...