The medical management of pulmonary arterial hypertension (PAH) is difficult, for several reasons:

• The disease is often relentlessly progressive

• Diagnosis is generally delayed until the condition is well advanced

• Many therapy options are difficult to use and/or are ineffective in many patients.

General medical therapy for pulmonary arterial hypertension (PAH) includes oral anticoagulants, oxygen, diuretics, and digoxin. In addition to vasodilator therapies (such as calcium channel blockers), agents are available that may possess anti-proliferative activity, including prostaglandins and, more recently, endothelin receptor antagonists (ERAs). Tracleer™  (bosentan), the first representative of this new class (ERA), is the only oral therapy licensed for the treatment of pulmonary arterial hypertension (PAH) to improve exercise capacity and symptoms in patients with grade III WHO Functional Class.

In patients with pulmonary hypertension with symptomatic progressive disease who, despite optimal medical treatment, remain severely ill (modified NYHA functional classes III or IV), a lung transplantation may be indicated.

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References

1. Gibbs JSR et al. Recommendations on the management of pulmonary hypertension in clinical practice. British Cardiac Society Guidelines and Medical Practice Committee. Heart (Sept 2001);86(1):i1-i13.