Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.
Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S57-69. doi: 10.1097/PCC.0b013e3181d10cce.
Preoperative pulmonary vascular disease remains an important risk factor for death or right-heart failure in selected children undergoing two-ventricle repair, single-ventricle palliation, or heart transplantation. Preoperative criteria for poor outcome after operation remain unclear. The purpose of this review is to critically assess both the historic and current data and make recommendations where appropriate. An extensive literature search was undertaken in October 2009. Data were analyzed by an expert multidisciplinary team and recommendations were made by consensus. PubMed was searched in October 2009. Data were analyzed and recommendations were made by consensus of a multidisciplinary team. In patients with suspected pulmonary vascular disease anticipating a two-ventricle repair, although preoperative testing via cardiac catheterization with vasodilators is reasonable, the preoperative parameters and the precise values of these parameters that best correlate with early and late outcome remain unclear. Further investigation is warranted in selected populations, such as the growing group of children with congenital heart disease complicated by chronic lung disease of prematurity, and in the developing world where patients may be more likely to present late with advanced pulmonary vascular disease. In patients with a functional single ventricle, there is growing evidence that mean pulmonary artery pressure of >15 mm Hg may be associated with both early and late mortality after the Fontan operation. The relationship of preoperative pulmonary hemodynamics to early and late morbidity remains to be defined. There most likely is a level of preoperative pulmonary vascular disease that puts an individual patient at increased risk for death or severe cyanosis after a bidirectional cavopulmonary anastomosis. It remains unclear, however, how to best assess this risk preoperatively. The limitations in obtaining an accurate assessment of pulmonary vascular disease in the complex single ventricle are discussed. In children awaiting cardiac transplantation with elevated pulmonary vascular disease of >6 U.m and/or transpulmonary gradient of >15 mm Hg, heart transplantation is deemed feasible in most transplant centers if the administration of inotropes or vasodilators can decrease the pulmonary vascular disease to <6 U.m or transpulmonary gradient to <15 mm Hg. In patients with preoperative pulmonary vascular disease, there may be contributing factors to the pulmonary vascular disease, such as the specifics of the cardiac lesion (atrioventricular valve regurgitation, low cardiac output), parenchymal and/or airway issues, and/or individual genetic predisposition. Amelioration of any reversible factors before operation and optimization of their management in the preoperative and postoperative period are recommended.
术前肺血管疾病仍然是选择行双心室修复、单心室姑息术或心脏移植术的患儿术后死亡或右心衰竭的重要危险因素。术后预后不良的术前标准仍不明确。本文的目的是批判性地评估既往和目前的数据,并在适当的情况下提出建议。我们于 2009 年 10 月进行了广泛的文献检索。多学科专家团队对数据进行了分析,并达成共识提出建议。我们于 2009 年 10 月在 PubMed 上进行了检索。多学科团队对数据进行了分析并达成共识提出建议。对于术前怀疑有肺血管疾病、拟行双心室修复的患儿,虽然术前应用血管扩张剂行心导管检查是合理的,但目前仍不清楚哪些术前参数及其确切值与早期和晚期结果相关性最好。进一步的研究是必要的,尤其是在一些特殊人群中,例如患有先天性心脏病合并早产儿慢性肺部疾病且正在不断增多的患儿,以及在发展中国家,这些患儿可能会因为晚期出现严重的肺血管疾病而就诊。对于功能性单心室的患儿,越来越多的证据表明,Fontan 术后平均肺动脉压(mean pulmonary artery pressure,mPAP)>15mmHg 可能与早期和晚期死亡率相关。术前肺血流动力学与早期和晚期发病率的关系仍有待明确。很可能存在一定水平的术前肺血管疾病,使个体患者在行双向腔肺吻合术后死亡或严重发绀的风险增加。但是,目前尚不清楚如何在术前最好地评估这种风险。我们讨论了在复杂的单心室中获得准确的肺血管疾病评估的局限性。在等待心脏移植的患儿中,如果应用正性肌力药或血管扩张剂能将 mPAP 降至<6U.m 或跨肺梯度(transpulmonary gradient,TPG)降至<15mmHg,大多数移植中心认为肺动脉高压>6U.m 且/或 TPG>15mmHg 的患儿行心脏移植是可行的。对于术前有肺血管疾病的患儿,肺血管疾病可能有一些促成因素,如心脏病变的具体情况(房室瓣反流、心输出量低)、实质和/或气道问题和/或个体遗传易感性。建议在术前改善任何可逆转的因素,并优化术前和术后的管理。