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The case is reported of an infant with congenital stenosis of each of the four individual pulmonary veins near the left atrium. The characteristic features were failure to thrive, increasing cyanosis and dyspnea, and death in congestive cardiac failure at 7 months of age. A precordial systolic murmur and an early systolic ejection click were heard; the sounds over the base of the heart were diminished in intensity. Roentgenograms of the thorax revealed a heart of normal size with fullness of the main pulmonary artery segment and increased pulmonary vascular markings that had the reticulated appearance associated with pulmonary venous engorgement. Electrocardiography showed right axis deviation, right atrial enlargement, and a pattern of right ventricular systolic overload. Cardiac catheterization demonstrated increased right atrial pressure and markedly elevated right ventricular and pulmonary arterial pressures. Angiocardiographic studies revealed a right-to-left shunt at atrial level, enlargement of the right ventricle, and enlarged and tortuous pulmonary arteries. In the late films the left atrium appeared normal in size, but the pulmonary veins were tortuous and there was evidence of constriction of the pulmonary veins at the veno-atrial junctions, associated with delay in the passage of opaque material from the pulmonary veins to the left atrium.It is suggested that the definitive diagnosis most likely is to be made by a selective angiocardiogram performed from the main pulmonary artery.It would appear feasible, at least in theory, to obtain complete surgical correction of this anomaly.

In mitral atresia with a closed or narrowly patent foramen ovale, anomalous pulmonary venous connexions may occur that function as collateral pathways for the return of pulmonary venous blood to the heart. In 2 cases (Edwards and DuShane, 1950;Lucas et al., 1962), although all the pulmonary veins' connected normally, an anomalous channel-termed a levo-atriocardinal veinformed the collateral pathway connecting the left atrium to the left innominate vein.This report describes coexisting pulmonary venous anomalies in 5 cases among a group of 29 cases of mitral atresia studied by us. In 4 of these 5, the anomalous pulmonary veins took the form of anomalous pulmonary venous connexion: in the remaining 1 the pulmonary venous anomaly was represented by cor triatriatum. In 3 of the 5 cases (Group I) mitral atresia and premature closure of the foramen ovale coexisted. The anomalous pulmonary venous connexions in this group provided collateral routes for the flow of pulmonary venous blood, and can be understood as developing in response to obstruction at the foramen ovale when the mitral valve is atretic.In the remaining 2 cases (Group II) no such causative factor could be invoked for the presence of the pulmonary venous anomalies, since in each an interatrial communication was present. In one of the latter group total anomalous pulmonary venous connexion coexisted with a common atrium. In the other, cor triatriatum coexisted with a patent foramen ovale-the four individual pulmonary veins connecting to the upper (accessory) left atrial chamber.In all 5 cases, regardless of the specific anatomical arrangement, obstruction to pulmonary venous flow occurred. Grossly, in the 4 cases where the lungs were available for study, this organ appeared much congested and cedematous and the pleural surfaces showed a reticulated pattern of distended lymphatic channels. Histologically, there was medial hypertrophy of muscular arteries with very prominent elastic membranes, medial hypertrophy of the arterioles, congestion of alveolar walls, and dilatation of lymphatics (Fig. 1).The purpose of this report is to define the pulmonary venous anomalies in 4 of the 5t cases. Two of them (Cases 1 and 3) indicate the complexity of the pulmonary venous system that may develop when obstruction to flow is present during the stages when the lungs and their respective vascular beds are developing. The material also will indicate how normal mediastinal structures may contribute to obstruction of anomalous pulmonary veins.Health Service.t One of the 5 cases, an example with a levo-atriocardinal vein, has been reported by Lucas et al. (1962). In this case-that of an 1 1-week-old female infant-mitral atresia coexisted with partial premature closure of the foramen ovale. Total anomalous pulmonary venous drainage occurred through a levo-atriocardinal vein to the left innominate vein. Also present were a ventricular septal defect, hypoplastic left ventricle, tubular hypoplasia of the aortic arch, and a patent ductus arteriosus. 241 group.bmj.com on May 29, 20...

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