Waqas Ahmed, Sabeen Razaque, Ayesha Khalid.
Primary pulmonary hypertension.
Pak Heart J Jan ;39(3-4):51-8.

Primary pulmonary hypertension (PPH) is a rare disease characterized by elevated pulmonary artery pressure without a demonstrable cause. PPH is also termed pre-capillary pulmonary hypertension or, more recently, idiopathic pulmonary arterial hypertension (IPAH). The diagnosis is usually made after excluding other etiologies that may cause secondary pulmonary hypertension. The recent emergence of prostacyclin analogues, endothelin receptor antagonists, and other novel drug therapies has greatly improved the outlook for patients with PPH and PPH-like diseases. PPH occurs at a femaleto-male ratio ranging from 2-9:1, depending on the treatment center sampled. No racial predilection is recognized. PPH typically develops in younger women of childbearing age. However, it can also affect women in their fifth and sixth decades of life or older. The mean age at presentation for PPH is fourth decade. This is a review article.

PakMediNet -Pakistan's largest Database of Pakistani Medical Journals - http://www.pakmedinet.com