Mitral Atresia Associated With Pulmonary Venous Anomalies

Abstract

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...

Congenital mitral atresia.

“…Our Case 7 was born prematurely and this may have contributed to his early demise, especially as there was a large atrial septal defect and no evidence of pulmonary stenosis. Anomalous pulmonary venous drainage, a common associated abnormality in mitral atresia (Watson et al, 1960;Shone and Edwards, 1964), was not present in any of our cases.…”

Mitral atresia with normal aortic valve: a study of eighteen cases and a review of the literature.

“…One of our patients and seven from the literature6 28,34,38,45,47 survived the first year of life, with a maximum life span of 16 years.5 Of the patients who lived beyond one year, four had an atrial septal defect with pulmonary stenosis, two had an atrial septal defect, and one had pulmonary stenosis. Two of our patients are still alive; one at two years of age with a surgical atrial septostomy and the other at two and one-half years with a balloon atrial septostomy.…”

Intrapulmonary Segment in Anomalous Pulmonary Venous Connection

“…6 Roentgenographically, the large vein within the right lung in each of our cases may suggest the scimitar syndrome. In the scimitar syndrome the roentgenographically visible anomalous vein in the right lung is simply a vein which carries blood from all or part of the right lung to the inferior vena cava and does not represent a collateral channel from remote portions of the pulmonary venous bed.…”