Cor Vasa 2009, 51(7-8):470-477 | DOI: 10.33678/cor.2009.120

Pulmonary hypertension in congenital heart disease

Jana Popelová^*, Štěpán Černý

Kardiocentrum, Oddělení kardiochirurgie, Nemocnice Na Homolce, Praha, Česká republika

In our series of 2,300 adult patients with congenital heart disease (CHD), pulmonary hypertension (PH) was diagnosed in 5.8%. Ventricular septal defect was diagnosed in 36% of our PH patients, atrial septal defect in 26%, complex CHD in 12%, atrioventricular septal defect in 11%, patent ductus arteriosus in 6%, and coarctation of the aorta in 3%. Eisenmenger's syndrome was present in 28% in PH patients, with 10-year mortality from Eisenmenger's syndrome being 18%. To date, we have operated on 52 patients with CHD and PH (39% of all PH patients), with a mortality rate of 3.8%. Conservative measures are very important for inoperable patients with PH. Venepuncture is generally not indicated in patients with Eisenmenger's syndrome. In some patients, symptoms can be improved by specific vasodilator. Our experience is compared with that reported in the relevant literature.

Keywords: Congenital heart disease; Pulmonary hypertension; Eisenmenger's syndrome; Pulmonary vasodilator therapy; Surgery in pulmonary hypertension

Published: July 1, 2009  Show citation