Cor Triatriatum as a Problem of Adult Heart Disease

The occurrence of severe pulmonary venous obstruction due to the congenital anomaly known as cor triatriatum in a 19-year-old furniture mover is described. Relentless and massive hemoptysis led to pulmonary edema, and an attempt at surgical correction under unfavorable conditions was not successful. Seven other cases of cor triatriatum in adults are reviewed. The hemodynamic abnormalities closely resemble mitral stenosis, except that pressures in the true left atrium were normal in the presence of elevated pulmonary capillary pressures. The availability of flow across the obstructing membrane during systole as well as diastole is probably the major element in the surprisingly good tolerance of severe degrees of anatomic obstruction for long periods of time. Slow increase in the degree of obstruction could also be a factor. The clinical picture in these patients very closely resembles mitral stenosis. Hemoptysis appeared to be more frequent in cor triatriatum. Features suggestive of the correct diagnosis would include the absence of an opening snap, the absence of a typical murmur of mitral stenosis, regular rhythm, and lesser evidence of left atrial enlargement in the presence of obvious pulmonary hypertension. Five of these patients have undergone successful correction of their disease by operation. Representative films from the first known angiograms in this condition in an adult are reproduced and discussed.