Adult patients with congenital heart disease and pulmonary arterial hypertension: First open prospective multicenter study of bosentan therapy

• Published in: The American heart journal Vol. 150; no. 4; pp. 716.e7 - 716.e12
• Main Authors: Schulze-Neick, Ingram, Gilbert, Nadine, Ewert, Ralf, Witt, Christian, Gruenig, Ekkehard, Enke, Beate, Borst, Mathias M., Lange, Peter E., Hoeper, Marius M.
• Format: Journal Article
• Language: English
• Published: United States Mosby, Inc 01-10-2005; Elsevier Limited
• Subjects: Adolescent; Adult; Antihypertensive Agents - therapeutic use; Blood pressure; Cardiovascular disease; Defects; Drug therapy; Enzymes; Female; Heart; Heart Diseases - complications; Heart Diseases - congenital; Heart rate; Humans; Hypertension; Hypertension, Pulmonary - complications; Hypertension, Pulmonary - drug therapy; Male; Middle Aged; Prospective Studies; Pulmonary arteries; Quality of life; Sulfonamides - therapeutic use
• ISSN: 0002-8703; 1097-6744
• DOI: 10.1016/j.ahj.2005.07.005

Endothelin receptor antagonism has been introduced as an effective oral therapy of patients with idiopathic pulmonary arterial hypertension. In view of the pathophysiologic and histologic similarities between idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), there is a rationale for treating these patients with the oral dual (ET
A/ET
B) endothelin receptor antagonist bosentan.
Thirty-three patients with PAH-CHD (43 ± 14 years, 23 with Eisenmenger syndrome) were treated with bosentan for a mean of 2.1 ± 0.5 years. Efficacy was assessed by a panel of tests, including New York Heart Association functional class, 6-minute walking distance, and echocardiographic and hemodynamic parameters.
Mean 6-minute walking distance increased from 362 ± 105 to 434 ± 68 m (
P = .001). New York Heart Association class also improved significantly (3.1 to 2.4,
P = .0001). This was associated with slight trends in improvements of transcutaneous oxygen saturation (86% ± 7% to 88% ± 7%,
P = .13) and maximum oxygen uptake (13.2 ± 4.0 to 14.9 ± 2.5,
P = .18). Right ventricular systolic pressure measured by echocardiographic decreased from 111 ± 32 to 106 ± 22 mm Hg (
P = .001). Bosentan treatment was well tolerated by all patients.
Long-term bosentan treatment in adult patients with PAH-CHD was well tolerated and improved functional status as well as exercise capacity. These findings have to be corroborated by controlled studies that are presently ongoing.