HEART DOCTORS IN CHIKKAJALA, BANGALORE;
Pulmonary hypertension is an uncommon but important cause of dyspnoea. Many patients with
this chronic and often severe illness will have raised pulmonary artery pressures as a result of a
cardiac or respiratory illness. Other patients may present with increasing dyspnoea without an
obvious cardiac or respiratory problem. Idiopathic (primary) pulmonary hypertension (IPH)
is diagnosed only when other causes of pulmonary hypertension have been excluded.
By definition, pulmonary hypertension is present when the mean pulmonary artery pressure
(PAP) exceeds 25 mmHg at rest or 30 mmHg during exercise. The classification of pulmonary
hypertension has been revised. The Venice classification was released in 2003. The term ‘primary pulmonary
hypertension’ has been replaced with ‘idiopathic pulmonary hypertension’ .
Patients may have used fenfluramine or phenermine (appetite-suppressing drugs), or both.
Use of these drugs for long periods has been associated with the greatest risk of developing
In cases of IPH there may be a family history (6%; autosomal dominant condition with
incomplete penetrance, 20–80%). The majority of familial cases are associates with a mutation
on the BMPR2 gene.
There may be associated symptoms including fatigue, chest pain, syncope and oedema.
Cough and haemoptysis can be present.