Acute Hemodynamic Effects of Single Oral Dose of Bosentan in Patients with Pulmonary Arterial Hypertension Related to Congenital Heart Disease

作者：Ajami Gholamhossein; Ahmadipour Maryam; Amoozgar Hamid^*; Bourzoee Mohammad; Cheriki Sirous; Shakiba Ali Mohammad; Edraki Mohammad Reza

来源：Congenital Heart Disease, 2014, 9(4): 343-348.

DOI：10.1111/chd.12147

• Congenital Heart Disease
• Pulmonary Arterial Hypertension
• Pulmonary Vasodilation
• Bosentan

Background. Endothelin-1 is a potent endogenous vasoconstrictor and an important remodeling factor in the pathogenesis of pulmonary arterial hypertension (PAH). Bosentan, a nonselective and active dual endothelin receptor antagonist, is used as a vasodilator in treatment of such patients. This study aimed to evaluate acute response to a single oral dose of bosentan as a vasodilator agent in comparison with nasal oxygen (O-2) in patients with PAH related to congenital heart disease (CHD). %26lt;br%26gt;Materials and Methods. We enrolled 20 patients with PAH-CHD, with a mean age of 5.45 +/- 4.5 years. Hemodynamic variables were measured at baseline state, after administration of nasal O-2 (5 L/min) for 20 minutes, and then when hemodynamic variables returned to the baseline, the measurements were repeated for the third time 3 hours after administration of a single oral dose of bosentan (2 mg/kg). %26lt;br%26gt;Results. Mean pulmonary vascular resistance was 9.92 +/- 2.97 Wood units m(2) at baseline and was lowered by O-2 to 6.17 +/- 2.71 Wood units m(2) (P = .001) and by bosentan to 5.90 +/- 2.69 Wood units m2 (P = .0001). Mean pulmonary artery pressure was 71.2 +/- 15.4 mm Hg at baseline and was reduced to 62.6 +/- 15.2 mm Hg (P = .001) by O-2 and to 61.6 +/- 14.8 mm Hg (P = .0003) by bosentan. %26lt;br%26gt;Conclusion. A single oral dose of bosentan has the same acute vasodilatory effect on the pulmonary vascular bed as nasal O-2 in patients with PAH related to CHD. Such patients may benefit from long-term therapy with this novel medication.

• 出版日期2014-8