- A rare usually fatal disease most commonly seen in women that is best managed in centres with expertise in pulmonary hypertension.
- Presenting symptoms and signs are non-specific and include dyspnoea on exertion, fatigue, and a loud pulmonary component of the second heart sound.
- Diagnosis of exclusion. Transthoracic Doppler echocardiography and right heart catheterisation can diagnose pulmonary hypertension. Laboratory tests and imaging studies can rule out known causes of pulmonary hypertension.
- General supportive therapy includes oral anticoagulants, diuretics, supplemental oxygen, and digoxin.
- Targeted treatment options include parenteral and inhaled prostanoids, oral endothelin receptor antagonists, and oral phosphodiesterase type-5 inhibitors.
- Prognosis is not good, as most patients eventually develop right ventricular failure.
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Last updated: Jan 16, 2013