CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, The Netherlands.
Department of Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands.
Heart. 2021 Nov;107(21):1725-1730. doi: 10.1136/heartjnl-2020-318074. Epub 2021 Jan 15.
Pharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients.
Data on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported. Patients with a systemic RV ejection fraction of ≤35% who were symptomatic despite treatment with β-blocker and ACE-inhibitor/angiotensin II receptor-blockers were started on sacubitril/valsartan. This cohort underwent structural follow-up including echocardiography, exercise testing, laboratory investigations and quality of life (QOL) assessment.
Six-month follow-up data were available in 18 out of 20 patients, including 12 (67%) patients with TGA after atrial switch and 6 (33%) patients with ccTGA. N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) decreased significantly (950-358 ng/L, p<0.001). Echocardiographic systemic RV fractional area change and global longitudinal strain showed small improvements (19%-22%, p<0.001 and -11% to -13%, p=0.014, respectively). The 6 min walking distance improved significantly from an average of 564 to 600 m (p=0.011). The QOL domains of cognitive function, sleep and vitality improved (p=0.015, p=0.007 and p=0.037, respectively).
We describe the first patient cohort with systemic RV failure treated with sacubitril/valsartan. Treatment appears feasible with improvements in NT-pro-BNP and echocardiographic function. Our positive results show the potential of sacubitril/valsartan for this patient population.
大动脉转位（TGA）后经心房调转或矫正性大动脉转位（ccTGA）患者的右心系统衰竭（RV），其药物治疗选择并不明确。本研究旨在调查我们中心的单中心队列患者中沙库巴曲缬沙坦治疗的可行性和效果。
报告了我们中心所有接受沙库巴曲缬沙坦治疗的、在双心室循环中右心系统衰竭的、连续成人患者（n=20，平均年龄 46 岁，50%为女性）的数据。在β受体阻滞剂和血管紧张素转换酶抑制剂/血管紧张素 II 受体阻滞剂治疗后仍有症状、且右心系统射血分数≤35%的患者，开始接受沙库巴曲缬沙坦治疗。该队列接受了结构随访，包括超声心动图、运动测试、实验室检查和生活质量（QOL）评估。
20 例患者中，18 例（90%）有 6 个月的随访数据，包括 12 例（67%） TGA 后经心房调转和 6 例（33%） ccTGA。N 端脑利钠肽前体（NT-pro-BNP）显著下降（950-358ng/L，p<0.001）。超声心动图右心系统射血分数和整体纵向应变显示略有改善（19%-22%，p<0.001 和-11%至-13%，p=0.014）。6 分钟步行距离从平均 564 米显著增加至 600 米（p=0.011）。认知功能、睡眠和活力的 QOL 域得到改善（p=0.015，p=0.007 和 p=0.037）。
我们描述了首个接受沙库巴曲缬沙坦治疗的右心系统衰竭患者队列。该治疗方法似乎可行，NT-pro-BNP 和超声心动图功能均有改善。我们的积极结果表明沙库巴曲缬沙坦对该患者人群具有潜在疗效。
