Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology

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• 作者：Michele D'Alto ; ^{mic.dalto@tin.it} ; Emanuele Romeo ; Paola Argiento ; Berardo Sarubbi ; Giuseppe Santoro ; Nicola Grimaldi ; Anna Correra ; Giancarlo Scognamiglio ; Maria Giovanna Russo ; Raffaele Calabrò
• 关键词：Pulmonary arterial hypertension ; Congenital heart disease ; Association therapy
• 刊名：International Journal of Cardiology
• 出版年：2012
• 期刊代码：128_01675273
• 类别：med
• 出版时间：22 March, 2012
• 卷：155
• 期：3
• 页码：378-382
• 文件大小：157 K

摘要

Objectives

The aim of the present study was to evaluate the safety, tolerability, clinical and haemodynamic impact of add-on sildenafil in patients with congenital heart disease (CHD)-related pulmonary arterial hypertension (PAH) and Eisenmenger physiology after failure of oral bosentan therapy.

Methods

Thirty-two patients with CHD-related PAH (14 male, mean age 37.1 卤 13.7 years) treated with oral bosentan underwent right heart catheterization (RHC) for clinical worsening. After RHC, all patients received oral sildenafil 20 mg thrice daily in addition to bosentan. Clinical status, resting transcutaneous oxygen saturation (SpO₂), 6-minute walk test (6MWT), serology and RHC were assessed at baseline (before add-on sildenafil) and after 6 months of combination therapy.

Results

Twelve patients had ventricular septal defect, 8 atrio-ventricular canal, 6 single ventricle, and 6 atrial septal defect. Twenty-eight/32 had Eisenmenger physiology and 4 (all with atrial septal defect) did not. All patients well tolerated combination therapy. After 6 months of therapy, an improvement in clinical status (WHO functional class 2.1 卤 0.4 vs 2.9 卤 0.3; P = 0.042), 6-minute walk distance (360 卤 51 vs 293 卤 68 m; P = 0.005), SpO₂ at the end of the 6MWT (72 卤 10 vs 63 卤 15%; P = 0.047), Borg score (2.9 卤 1.5 vs 4.4 卤 2.3; P = 0.036), serology (pro-brain natriuretic peptide 303 卤 366 vs 760 卤 943 pg/ml; P = 0.008) and haemodynamics (pulmonary blood flow 3.4 卤 1.0 vs 3.1 卤 1.2 l/min/m², P = 0.002; pulmonary vascular resistances index 19 卤 9 vs 24 卤 16 WU/m², P = 0.003) was observed.

Conclusions

Addition of sildenafil in adult patients with CHD-related PAH and Eisenmenger syndrome after oral bosentan therapy failure is safe and well tolerated at 6-month follow-up, resulting in a significant improvement in clinical status, effort SpO₂, exercise tolerance and haemodynamics.