Zusammenfassung

• 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.