Ivan Varga, Milan Luknár, Peter Solík, Peter Lesný, Eva Goncalvesová
Pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD) represents the most frequent entity in the subgroup of associated PAH (APAH). Here we present an analysis approach and case report of a woman with 5 years history of CTD. She was admitted to hospital for right heart failure and pulmonary hypertension. One day after admission was the hospitalization complicated by soporous state and circulatory colapse caused by right ventricular failure due to pulmonary hypertension and superimposed cerebral vasculitis was suspected. Therapy was started immediately with puls of methylprednisolone and cyclophosphamide. Right heart catheterization revealed advanced PAH with severe right ventricle dysfunction. We decided to start specific PAH treatment with combination of sildenafil and bosentan. Clinical and hemodynamic stabilisation was achieved in a few days and sustained also after 8 months. Initial combination therapy could be an suitable method for patients with advanced PAH especially in instable cases with necessity of immediate action and unknown patient preference.