NPC-QIC Registry data showed that angiotensin-converting enzyme inhibitors were described in almost 38% for patients with single ventricle physiology after stage-I Norwood palliation. However, mortality and ventricular dysfunction or atrioventricular valve insufficiency seems to be not improved by oral application of angiotensin-converting enzyme inhibitors. The final conclusion was that despite limited evidence of benefit for patients with hypoplastic left heart syndrome, prescription of angiotensin-converting enzyme inhibitors during interstage is still common. Taking into account of the predominant cardiovascular regulation in newborns and young infants by circulating catecholamines, no real improvement is to be expected from angiotensin-converting enzyme inhibitor monotherapy. The goals of drug therapy after stage-I Norwood palliation in hypoplastic left heart syndrome are prevention of systemic right ventricle failure, balancing pulmonary and systemic blood flow, and reduction of oxygen consumption with regard to limitations of oxygen supply by the single ventricle, furthermore, avoiding harmful effects of endogenous catecholamine production in the long term on somatic and cognitive development. In this light of knowledge, we want to recommend the use of a long-acting and highly specific ß1-adrenoreceptor blocker for almost all patients after stage-I Norwood palliation and a combination with angiotensin-converting enzyme inhibitors only by indication after exclusion of potential side effects.

中文翻译：

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左心发育不良综合征Ⅰ期后血管紧张素转化酶抑制剂治疗的网络化

NPC-QIC 注册数据显示，在 I 期 Norwood 姑息治疗后，近 38% 的单心室生理患者使用血管紧张素转换酶抑制剂。然而，口服血管紧张素转换酶抑制剂似乎并未改善死亡率和心室功能障碍或房室瓣功能不全。最后的结论是，尽管对左心发育不良综合征患者有益的证据有限，但在期间开具血管紧张素转换酶抑制剂的处方仍然很常见。考虑到循环儿茶酚胺对新生儿和小婴儿的主要心血管调节作用，预计血管紧张素转换酶抑制剂单一疗法不会有真正的改善。左心发育不全综合征 I 期 Norwood 姑息治疗后药物治疗的目标是预防全身性右心室衰竭，平衡肺和全身血流，以及在单心室供氧受限的情况下减少耗氧量，此外，避免长期内源性儿茶酚胺产生对躯体和认知发育的有害影响。鉴于此，我们建议在 I 期 Norwood 姑息治疗后的几乎所有患者中使用长效和高度特异性的 ß1-肾上腺素受体阻滞剂，并仅在排除潜在的适应症后与血管紧张素转换酶抑制剂联合使用。副作用。减少单心室供氧的耗氧量，进一步避免内源性儿茶酚胺产生对躯体和认知发育的长期有害影响。鉴于此，我们建议在 I 期 Norwood 姑息治疗后的几乎所有患者中使用长效和高度特异性的 ß1-肾上腺素受体阻滞剂，并仅在排除潜在的适应症后与血管紧张素转换酶抑制剂联合使用。副作用。减少单心室供氧的耗氧量，进一步避免内源性儿茶酚胺产生对躯体和认知发育的长期有害影响。鉴于此，我们建议在 I 期 Norwood 姑息治疗后的几乎所有患者中使用长效和高度特异性的 ß1-肾上腺素受体阻滞剂，并仅在排除潜在的适应症后与血管紧张素转换酶抑制剂联合使用。副作用。