Cor triatriatum: A rare malformation of the heart, probably amenable to surgery: Report of a case, with review of literature

Abstract

A case report of a 29-year-old woman with increasing cardiac distress is reported. No murmur could be heard. The heart was not enlarged, but a prominent second left arch and pulmonary congestion were found on the roentgenogram. Moreover, the lower lobe of the left lung was markedly emphysematous. An electrocardiogram showed right ventricular hypertrophy. On heart catheterization, an excessive pulmonary hypertension was found, and the pulmonary “capillary pressure”, too, was very high. A mitral disease, therefore, was suggested, and the patient submitted to surgery. Thoracotomy revealed a normal mitral valve, and a normal pressure in the left atrium was measured directly. At post mortem the findings were fully explained, as a transverse septum with only one small perforation was separating the opening of the pulmonary veins from the rest of the atrium (so-called cor triatriatum). A review is made of the literature concerning anomalous, congenital structures within the left atrium. Thin cords or even broad bands across the lumen have been of no significance. The real cor triatriatum, however, was a serious disorder. Many of the patients died shortly after birth, and only a few reached the adult age. The clinical symptoms and signs were by no means characteristic, and no help was obtained from the usually employed investigations. An in-vivo diagnosis, therefore, must certainly be left for an explorative thoracotomy. The possibility of an effective surgical treatment is briefly discussed.