On the Cause of Syncope and Sudden Death in Primary Pulmonary Hypertension

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Abstract
Syncope and sudden death are common clinical features of primary pulmonary hypertension but their mechanism has not been satisfactorily explained. Most explanations have failed because they are unable to account for the infrequency of syncope and sudden death in patients with secondary pulmonary hypertension. The diagnosis of "primary pulmonary hypertension" is much abused, and the selection of satisfactory cases is limited by its rare occurrence. After careful search 3 satisfactory cases were studied, all occurring in young women who either had syncope or died suddenly or both. In each case there was extensive pathology of both the sinus node and A-V node, particularly involving their nutrient arteries. A similar examination of the sinus node and A-V node of 6 patients with severe secondary pulmonary hypertension and 7 patients with disseminated lupus erythematosis failed to demonstrate these particular findings. It is postulated that syncope and sudden death in patients with primary pulmonary hypertension may be related to focal disease involving the conduction centers of their hearts. Of perhaps greater significance is the fact that the nodal arteries are in the systemic circulation, and that their pathology resembles that involving the pulmonary arteries, suggesting that so-called "primary pulmonary hypertension" may be a generalized disease.