Hypoplastic left heart syndrome with premature closure of foramen ovale: report of an unusual type of totally anomalous pulmonary venous return.

We report on a case of hypoplastic left heart syndrome (HLHS), associated with premature closure of the foramen ovale and an unusual type of totally anomalous pulmonary venous return. The existence of an anomalous connection of the right upper pulmonary vein to the superior vena cava-right atrial (SVC-RA) junction and the existence of the anomalous intrapulmonary venous channel between right upper and lower pulmonary vein allowed all the pulmonary blood to drain into the SVC-RA junction, whereas she had only partially anomalous pulmonary venous connection. The several intrapulmonary venous channels helped to delay the progression of pulmonary venous obstruction. Chromosomal analysis revealed that the patient had XO Turner syndrome. We conclude that all infants with HLHS should be carefully evaluated for the existence of anomalous pulmonary venous return. Two-dimensional Doppler echocardiography is one of the most useful techniques for evaluating such anomalies.