[Association of severe bronchial disease (bronchial casts, bronchiectasis) and partial abnormal pulmonary venous drainage in 2 children with Turner's syndrome].

Abstract

The prevalence of cardiovascular malformation is high in Turner's syndrome. We report two cases of partial anomalous pulmonary venous drainage associated with a severe bronchial disease. CASE REPORTS: Case 1: a 5-year-old girl with monosomy X presented with acute respiratory failure. The chest X-ray showed an atelectasis of the left lower lobe. The patient improved following bronchoscopic removal of a bronchial cast. During hospitalization, an anomalous pulmonary venous drainage of the right lung was discovered. In spite of complete surgical repair of the cardiac malformation, several episodes of respiratory failure occurred several years after the operation. Case 2: a 9-year-old girl with monosomy X and coarctation of the aorta underwent pre-operative cardiac catheterization. She had a history of bronchiectasis, leading to a lobectomy. Her respiratory condition improved following surgery. A partial anomalous pulmonary venous return was discovered during the cardiac angiography. The coarctation was repaired surgically. The correction of the left-to-right shunt was not necessary. CONCLUSION: The partial anomalous pulmonary venous return is frequently reported in Turner's syndrome. The possible respiratory disease associated with this cardiac malformation is usually described as a consequence of the increase in pulmonary blood flow. Our two cases did not match this hypothesis.