How should I treat an adult with unoperated complex cyanotic heart disease with severe pulmonary stenosis?

BACKGROUND: A 33-year-old adult with complex congenital heart disease and severe cyanosis presented to us with increasing symptoms of hyperviscosity, decreasing exercise tolerance and a thromboembolic complication. INVESTIGATION: Physical examination, laboratory investigations, ECG, transthoracic echocardiogram, cardiopulmonary exercise testing, angiography. DIAGNOSIS: Subvalvular and valvular pulmonary stenosis due to a calcified pulmonary valve. TREATMENT: Amiodarone, angiotensin-converting enzyme inhibitor, beta-blockers, percutaneous pulmonary balloon valvuloplasty, warfarin.

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