Angiogenesis and pulmonary hypertension: a unique process in a unique disease

Severe pulmonary hypertension is a fatal condition associated with marked alterations of the cellular components of pulmonary arteries. In this review, we discuss the component of endothelial cell proliferation present in pulmonary arteries in patients with severe pulmonary hypertension. Because these proliferated endothelial cells exhibit markers of angiogenesis, we have named this process as "disordered or misguided angiogenesis." We also discuss the recent evidence that germline or somatic inactivating mutations in tumor suppressor or proapoptotic genes may play a significant role in the abnormal proliferation of pulmonary endothelial cells. The unraveling of the pathobiology of severe pulmonary hypertension may lead us to novel therapies and approaches to better diagnose the disease.