Primary Pulmonary Hypertension in a Child

Clinical, pharmacologic, and pathologic findings presented are those in a 12-year-old boy with primary pulmonary hypertension associated with medial hypertrophy of the pulmonary muscular arteries and arterioles. Intrapulmonary arterial administration of acetylcholine, tolazoline (Priscoline), or isoproterenol resulted in a fall in pulmonary vascular resistance and pressure. Perforation of the right ventricle by an indwelling catheter terminated studies on the results of long-term intrapulmonary arterial administration of drugs shown to be effective.