Serino Giorgio, Guazzi Marco, Micheletti Angelo, Lombardi Carlo, Danesi Rossella, Negura Diana, Carminati Mario, Chessa Massimo
Department of Internal and Vascular Medicine, IRCCS-Policlinico, San Donato, Italy.
Clin Med Insights Cardiol. 2013;7:29-34. doi: 10.4137/CMC.S10237. Epub 2013 Feb 6.
This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.
这项单中心回顾性分析评估了波生坦对7例患有唐氏综合征和艾森曼格综合征（DS-ES）的成年患者的长期治疗效果。在基线期和2年随访期间，对实验室检查、6分钟步行距离（6MWD）、心功能分级和多普勒超声心动图进行了评估。开始使用波生坦后，观察到6MWD有所改善或维持稳定（第12个月时较基线提高了68米）。6MWD一直维持到第2年。总体而言，6例患者在2年治疗期间心功能分级有显著改善（P = 0.01）。多普勒超声心动图测量的参数无显著变化。没有患者因肺动脉高压（PAH）进展而需要住院治疗或接受额外的PAH治疗。波生坦治疗总体耐受性良好；未发现肝功能异常或严重药物不良反应。在这个DS-ES队列中，波生坦似乎耐受性良好且临床有效。
