A rare hepatic-venous anomaly with portal drainage of the pulmonary veins.

Major congenital defects of the hepatic veins are readily detectable at autopsy but they have been described rarely. In the case reported here an anomalous disposition of these veins brought venous blood from the left half of the liver into the left atrium and blood from the right half of the liver into the right atrium. We have traced only two other similar cases (Arnold, 1868; Epstein, 1886). Pulmonary venous anomalies are better documented. They may divert the whole or a part of the pulmonary venous return from the left atrium into the right atrium or its tributaries. We shall neglect anomalies of parts of the pulmonary veins, for the present case is one of total anomalous venous drainage. Brody (1942) first subdivided this condition into an isolated form accompanied only by a patent foramen ovale or ductus arteriosus which makes the circulation possible, and into a complicated form in which the lesion is associated with major congenital cardiac defects, as in our case. Keith et al. (1958) recorded the insertions of the common pulmonary vein in 103 cases of the isolated form of the disease: in 11 of these the insertion was into the portal vein or the ductus venosus. Edwards (1960) stated that 66 cases of the complicated form of total anomalous pulmonary venous drainage were to be found, mostly reported cases but some in the material of the Mayo Clinic. The associated cardiac defects have included cor biloculare and truncus arteriosus: persistent atrio-ventricular canal, gross systemic venous malformations, and agenesis of the spleen, which were found in the present case, have also been recorded. Darling et al. (1957) registered infra-diaphragmatic insertions of the anomalous pulmonary veins in 10 out of 40 descriptions of the complicated form that they studied. Butler (1952) has divided cases of both forms of portal drainage of the pulmonary veins into group A in which the ductus venosus is the recipient vessel, as in our case, and group B in which the common pulmonary vein enters the left gastric vein. CASE REPORT