Congenital Stenosis of Individual Pulmonary Veins

Abstract

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.

Congenital and Acquired Pulmonary Vein Stenosis

“…Stenosis of the pulmonary veins may appear as a relatively discrete shelf, as a longer segment of narrowing at the junction of the pulmonary vein to the left atrium that extends slightly into the pulmonary vein, or as diffuse hypoplasia of the pulmonary veins. 4,7 Pulmonary vein stenosis in children and even adults with no apparent preceding or concomitant cause of stenosis has been termed "congenital". However, except in the small group of patients with diffusely hypoplastic pulmonary veins, we prefer the term "primary" pulmonary vein stenosis as the designation.…”

Familial pulmonary veno-occlusive disease: a case report.

“…In this respect the patient resembles more those characterised by extrapulmonary venous stenosis, atresia, and thrombosis (Bernstein et al, 1959;Shone et al, 1962;Calderon and Burdine, 1974;Mortensson and Lundstr6m, 1974;Sade et al, 1974).…”

“…There is one recent publication describing occlusion of both intra-and extrapulmonary veins by a process presumably of thrombotic origin (Calderon and Burdine, 1974). This observation suggests that some examples of occlusion restricted to extrapulmonary veins (Bernstein et al, 1959;Shone et al, 1962;Mortensson and Lundstr6m, 1974;Sade et al, 1974) may also have been expressions of pulmonary veno-occlusive disease.…”

Recipient Twin Circular Shunt Physiology Before Fetal Laser Surgery: Survival and Risks for Postnatal Right Ventricular Outflow Tract Obstruction

“…Circular shunt physiology has been previously described in children with severe forms of CHD, including Ebstein's anomaly and pulmonary atresia with left ventricle to right atrial communication. 13,14 The authors defined a "circular" shunt as one in which blood originating in one cardiac chamber is shunted through the heart to return to the original chamber without ever crossing a capillary bed. Typically, the finding of CSP portends a poor prognosis both in utero and postnatally.…”