title: Management of pulmonary arterial hypertension associated with congenital heart disease.
abstract: Congenital heart diseases are the most common congenital malformations and account for about eight cases per 1000 births and are often associated with pulmonary arterial hypertension. Increased shear stress and the excess flow through the pulmonary vascular bed due to a systemic-to-pulmonary shunt lead to the development of pulmonary vascular disease and an increase in pulmonary vascular resistance. Without surgical repair approximately 30% of patients develop pulmonary vascular disease. Eisenmenger syndrome represents the extreme end of pulmonary arterial hypertension with congenital heart disease. We summarized the current therapeutic options for pulmonary arterial hypertension; conventional treatments including calcium channel blockers, anticoagulation, digitalis, diuretics, and new treatment: prostacyclin, bosentan, sildenafil, ambrisentan. Preliminary data of new therapies are encouraging with disease significantly improved natural history, but there is need for more evidence-based data.
authors.affiliation: Institute of Cardiovasculare Diseases and Transplantation, Department of Pediatric Cardiology, Târgu-Mureş, Romania. roditog@yahoo.com
authors.forename: Rodica
authors.initials: R
authors.lastname: Togănel
authors.forename: I
authors.initials: I
authors.lastname: Benedek
authors.forename: Carmen
authors.initials: C
authors.lastname: Suteu
authors.forename: Cristina
authors.initials: C
authors.lastname: Blesneac
mesh_terms: D002121:Calcium Channel Blockers / Q000627:therapeutic use; D003326:Coronary Circulation; D004359:Drug Therapy, Combination; D016232:Endothelins / Q000494:pharmacology / Q000627:therapeutic use; D011464:Epoprostenol / Q000494:pharmacology; D006330:Heart Defects, Congenital / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology*; D016040:Lung Transplantation; D009569:Nitric Oxide / Q000627:therapeutic use; D058986:Phosphodiesterase 5 Inhibitors; D014665:Vasodilator Agents / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D002121:Calcium Channel Blockers; D016232:Endothelins; D058986:Phosphodiesterase 5 Inhibitors; D014665:Vasodilator Agents; D009569:Nitric Oxide; D011464:Epoprostenol