Interní Med. 2006; 8(6): 266-269

Pulmonary arterial hypertension in congenital heart disease

MUDr. Pavel Jansa

Centrum pro plicní hypertenzi, II. interní klinika kardiologie a angiologie, VFN a 1. LF UK, Praha

Pulmonary arterial hypertension (PAH) is a heterogeneous group of diseases that share the prekapilary pulmonary hypertension, similar pathophysiology (with vasoconstriction, remodeling of pulmonary arteries and thrombosis) and options of specific pharmacotherapy. In the group of PAH, especially PAH in systemic connective tissue diseases, congenital left-to right shunts, portal hypertension, HIV infection and abuse of certain anorectics are together with idiopathic and familial PAH included. Pulmonary hypertension is present in about 10% of patients with congenital heart disease in adults. Most frequently it is a consequence of recirculation across left to right shunt. Pulmonary hypertension gradually advances, patients become symptomatic, dying in the 3rd to 5th decade of life. Opinions on conventionally used noninvasive treatment strategies (anticoagulation, oxygen therapy, hemodilution) are mostly controversial. Parenteral therapy with prostanoids improves physical endurance and hemodynamics, but has many complications. Heart and lung transplantation or lung transplantation with correction of congenital heart disorder is a solution for strictly selected group of patients after exhaustion of conservative therapy. An option of a specific oral therapy is a great progress of recent years. It was successfully tested mainly in idiopathic PAH and PAH in systemic connective tissue diseases. Last year results of first large randomized clinical trial in patients with Eisenmenger´s syndrome were published. Bosentan treatment (dual antagonist of endothelin receptors) did not worsen the desaturation and lead to improvement of physical endurance and hemodynamics.

Keywords: Key words: pulmonary arterial hypertension, left to right shunt, Eisenmenger´s syndrome, prostanoids, bosentan.

Published: June 1, 2006  Show citation