Decreased levels of serum Angiotensin-(1-7) in patients with pulmonary arterial hypertension due to congenital heart disease

摘要

Pulmonary arterial hypertension (PAH) is a progressive disease with poor survival outcome. The presence of PAH increases morbidity and reduces survival in patients with congenital heart disease (CHD) [1]. The field of CHD-PAH has seen dramatic progress over the last two decades with improved survival and quality of life for patients, however, this remains a deadly and debilitating disease [2]. The exact pathogenesis of CHD-PAH is poorly understood, therapeutic options for those patients are limited. The angiotensin (Ang) converting enzyme 2 (ACE2)-Ang-(l-7)-Mas receptor axis is an important component of the renin-angiotensin system, ACE2 coverts Ang II into Ang-(1-7), which exerts both vasodilatory and anti-proliferative effects [3,4]. Recent studies have demonstrated the therapeutic effects of Ang-(1-7) in monocrotaline-induced PAH model [5,6]. Our previous study showed that serum ACE2 levels and activity were decreased in the patients with CHD-PAH [7,8]. In this study, we explored the hypothesis that serum Ang-(l-7) levels was also decreased in the patients with CHD-PAH.