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Pulmonary hypertension refers to increased blood pressure in the pulmonary circulation. It is defined as blood pressure in the pulmonary artery greater than 25mmHg at rest or 30mmHg during physical activity. During diseased state, patients may experience symptoms e.g. dyspnea, fatigue, exertional chest pain, exertional syncope and weight gain from edema. Heart, lung and thromboembolic diseases are possible causes of pulmonary hypertension. It is defined as pulmonary artery hypertension (PAH) if narrowing of pulmonary arteries is the direct cause. Drugs used in hypertension e.g. calcium channel blockers have a limited role in PAH, as novel vasodilators have been shown to be more effective against PAH.

Endothelin receptor antagonists (ERA)
ERA exerts its therapeutic action by binding to endothelin receptors on the pulmonary blood vessels selectively, relaxing pulmonary blood vessels and lowering pulmonary blood pressure. ERA must not be initiated in women of childbearing potential unless contraception other than pills has been adopted. Blood tests should be performed regularly to monitor liver function and blood count during the treatment.

Bosentan (Pulmonat) is indicated for the treatment of pulmonary arterial hypertension to improve exercise ability and to decrease clinical worsening. It is the only oral medication approved in patients with WHO functional class IV symptoms. It is contraindicated to be used concomitantly with Cyclosporin A in view of significant drug interaction with CYP3A4.