Abstract
Pulmonary arterial hypertension (PAH) has a multifactorial pathobiology. Vasoconstriction, remodeling of the pulmonary vessel wall, and thrombosis contribute to increased pulmonary vascular resistance in PAH.
Prostanoids have played a prominent role in the treatment of PAH. They improves exercise capacity, hemodynamics, and survival in patients with idiopathic pulmonary arterial hypertension (IPAH).
Several interventional and surgical approaches are available for treatment of PAH - atrial septostomy, lung or heart-lung transplantation. Pulmonary endarterectomy is the accepted method of treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH).
Establishing the diagnosis and the classification of the type of lesions by pulmonary angiography is crucial for optimal patients selection. Fewer then 3,000 PEA operations have been performed worldwide, although it is potentially curative treatment option patients with CTEPH.
Pulmonary endarterectomy have been performed successfully in the Czech Republic in the Clinic of Cardiovascular Surgery at the General Teaching Hospital in Prague in September 2004.