PRIMARY PULMONARY HYPERTENSION

[摘要] A case is reported of a 4-month-old infant in which the outstanding clinical features were failure to gain weight, cough, and progressive dyspnea. There was marked accentuation of the second pulmonic sound and no significant murmur. The electrocardiogram was interpreted as showing right ventricular hypertrophy and roentgenographically, there was unusual clarity of peripheral lung fields and prominence of hilar vessels. Cardiac catheterization demonstrated pulmonary hypertension and revealed no left to right intracardiac shunt. Anatomically, there was right ventricular hypertrophy and prominence of the medial layer of the small pulmonary arteries.This vascular change is indistinguishable from that seen in the normal newborn and certain varieties of secondary pulmonary hypertension. Although the clinical diagnosis can be suspected, the definitive anomaly can be determined only at post-mortem examination.

[学科分类] 儿科学