Hypoplastic Left Heart Syndrome With a Hypoplastic Lung in Congenital Diaphragmatic Hernia: What Should Be Done First?

摘要

Congenital diaphragmatic hernia (CDH) is a severe congenital defect, with the degree of lung hypoplasia correlated with unfavorable outcomes. Norwood stage 1 palliation (Norwood SIP) for hypoplastic left heart syndrome (HLHS) is one of the riskiest congenital heart surgical procedures. In cases where both CDH and HLHS are present, poor outcomes generally resu thus, a strategic approach is required to improve patient outcomes. Dr Balduf et al report on 5 cases and propose an algorithm for treating patients with CDH and HLHS. Their results suggest that successful palliation of neonates with HLHS and associated CDH is possible. Although CDH and HLHS are fatal without appropriate management, both conditions can be improved through surgical procedures. Lung hypoplasia can recover through surgical reduction of CDH, and the hemodynamic status of HLHS can be improved through Norwood SIP However, determining which procedure should be performed first is crucial. Balduf et al suggest that the decision should be based on the "adequacy" of lung function, which is assessed by clinical evaluation, including echocardiography for the "degree of lung hypoplasia." If lung function is adequate and the CDH is less severe, a Norwood SIP should be the initial procedure; otherwise, CDH repair should be performed first.