ABSTRACT
Three cases of unilateral right-sided pulmonary venous atresia were evaluated over an 18-year period. These bring the total number of cases to 25 in the literature. The clinical presentation of all these patients was similar and consisted of recurrent pulmonary infections, asthma-like symptoms, and exercise intolerance. The patients presented in 1982 (patient 1, a 12-year-old boy), 1994 (patient 2, a 9-year-old girl), and 1999 (patient 3, a 13-year-old boy). All patients were evaluated with a chest roentgenogram, and patients 1 and 2 had a ventilation and perfusion scan. Patients 1 and 3 also had cardiac catheterization and pulmonary angiography. Patient 2 had a magnetic resonance imaging study of the chest. Only patient 3 had wedge pulmonary angiography. Although a rare congenital defect, this diagnosis should be strongly suspected based on the typical clinical presentation and the preliminary studies, such as the chest roentgenogram and ventilation and perfusion scan. However, for definitive diagnosis, cardiac catheterization with wedge pulmonary angiography is necessary. Anastomosis of the atretic pulmonary veins to the left atrium is a theoretical consideration. However, this may not be feasible due to pulmonary venous anatomy or significant pulmonary dysfunction with pulmonary vascular changes. In these circumstances, we recommend performing pneumonectomy to remove the nidus for repeated bouts of pulmonary infections, to eliminate the left-to-right shunt, and to eliminate the dead space contributing to exercise intolerance.
Subject(s)
Pulmonary Atresia/diagnosis , Pulmonary Veins/abnormalities , Pulmonary Veno-Occlusive Disease/diagnosis , Adolescent , Angiography , Child , Diagnosis, Differential , Female , Humans , Magnetic Resonance Imaging , Male , Pulmonary Atresia/surgery , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgery , Pulmonary Veno-Occlusive Disease/surgery , Surgical Procedures, OperativeABSTRACT
We report the case of a child with hypoplastic left heart syndrome who developed pulmonary arteriovenous (AV) malformations after superior cavopulmonary anastomoses. Resolution of the pulmonary AV malformations occurred following a completion Fontan procedure. This phenomenon has been reported previously, but only in patients with heterotaxy and polysplenia.
