Togănel Rodica, Benedek I, Suteu Carmen, Blesneac Cristina
Institute of Cardiovasculare Diseases and Transplantation, Department of Pediatric Cardiology, Târgu-Mureş, Romania.
Rom J Intern Med. 2007;45(3):229-34.
Congenital heart diseases are the most common congenital malformations and account for about eight cases per 1000 births and are often associated with pulmonary arterial hypertension. Increased shear stress and the excess flow through the pulmonary vascular bed due to a systemic-to-pulmonary shunt lead to the development of pulmonary vascular disease and an increase in pulmonary vascular resistance. Without surgical repair approximately 30% of patients develop pulmonary vascular disease. Eisenmenger syndrome represents the extreme end of pulmonary arterial hypertension with congenital heart disease. We summarized the current therapeutic options for pulmonary arterial hypertension; conventional treatments including calcium channel blockers, anticoagulation, digitalis, diuretics, and new treatment: prostacyclin, bosentan, sildenafil, ambrisentan. Preliminary data of new therapies are encouraging with disease significantly improved natural history, but there is need for more evidence-based data.
先天性心脏病是最常见的先天性畸形，每1000例出生中约有8例，且常与肺动脉高压相关。由于体循环至肺循环分流导致的剪切应力增加和通过肺血管床的血流过多，会引发肺血管疾病并导致肺血管阻力增加。未经手术修复的患者中约30%会发展为肺血管疾病。艾森曼格综合征代表先天性心脏病伴肺动脉高压的极端情况。我们总结了目前肺动脉高压的治疗选择；传统治疗包括钙通道阻滞剂、抗凝治疗、洋地黄、利尿剂，以及新的治疗方法：前列环素、波生坦、西地那非、安立生坦。新疗法的初步数据令人鼓舞，疾病的自然史有显著改善，但仍需要更多基于证据的数据。
