Inai Kei
Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University.
Int Heart J. 2015;56 Suppl:S12-6. doi: 10.1536/ihj.14-396.
The operability of congenital heart disease with left to right shunt depends on the severity of the pulmonary vascular disease induced by the increased pulmonary blood flow. Although some recommendations exist regarding operative indications according to pathological, hemodynamic, and epidemiological factors, the evidence underlying these recommendations is not conclusive. Recently, oral pulmonary vasodilator therapy has been reported to improve outcomes in patients with idiopathic pulmonary arterial hypertension, and this therapy also appears to be effective in patients with congenital heart disease and pulmonary arterial hypertension, including those with postoperative pulmonary hypertension and Eisenmenger syndrome. It is expected that the availability of novel pulmonary vasodilator therapy will expand the operative indications in patients with congenital heart disease with left to right shunt, but there is currently insufficient evidence to definitively determine this. A multicenter double-blind study should be conducted to further examine this issue.
左向右分流型先天性心脏病的可手术性取决于肺血流量增加所导致的肺血管疾病的严重程度。尽管根据病理、血流动力学和流行病学因素存在一些关于手术指征的建议,但这些建议所依据的证据并不确凿。最近,有报道称口服肺血管扩张剂疗法可改善特发性肺动脉高压患者的预后,并且该疗法在患有先天性心脏病和肺动脉高压的患者中似乎也有效,包括术后肺动脉高压和艾森曼格综合征患者。预计新型肺血管扩张剂疗法的应用将扩大左向右分流型先天性心脏病患者的手术指征,但目前尚无足够证据明确确定这一点。应开展一项多中心双盲研究以进一步探讨这一问题。