A brief historical review of lprimary pulmonary arteriosclerosis and hypertension has been given. Ten additional cases with (letailed laboratory, roentgenographie, electrocardiographic, cardiac catheterization, and angiocardiographic studies are presented. The findings on 4 necropsied cases afie also included. Progressive exertional shortness of breath, syncope, left chest pain, right ventricul...

Primary pulmonary vascular sclerosis was first fully described by Brenner (1935) who stressed the presence of hypertrophy of the right, but not of the left ventricle; he concluded his description with the opinion that primary sclerosis is not a pathological entity but includes several conditions. Other authors, notably De Navasquez et al. (1940), preferred the term " primary or idiopathic right...

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 r...

Primary pulmonary hypertension has become a very treatable disease given the recent advances in medical therapy. Any patient with unexplained right ventricular enlargement or pulmonary hypertension needs a thorough workup to determine the cause. Patients with primary pulmonary hypertension should be referred to a specialized "Center of Excellence" to evaluate potential therapies on a case-by-ca...

The use of pharmacologic agents in the treatment of pulmonary hypertension has not proved to be uniformly successful or predictable. One possible reason for the vagaries in response is that the pulmonary vascular lesions are not consistent. We examined the relation between the structure of the pulmonary resistance vessels in unexplained (primary) pulmonary hypertension and the response to pulmo...

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 r...