The man from the kingdom of the near-dead — modern treatment of pulmonary hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a rare and severe condition characterised by a progressive increase in pulmonary vascular resistance. Two decades ago patients with idiopathic PAH were defined as the people from the kingdom of near- -dead because of poor survival. The progress in treatment of PAH was made, however the disease is still severe and not curable. We present a 26 year-old male patient diagnosed with idiopathic PAH. The clinical course was complicated by progressive worsening, hemoptysis and thrombocytopenia. Treatment with treprostinil and bronchial artery embolisation was started, resulting in symptomatic and functional improvement.
Kardiol Pol 2011; 69, 11: 1171–1173