Interventions and neonatal outcomes of fetuses with hypoplastic left heart syndrome and congenital diaphragmatic hernia: a systematic review.

OBJECTIVE: To evaluate the current interventions and outcomes in neonates born with hypoplastic left heart syndrome (HLHS) and congenital diaphragmatic hernias (CDHs).

METHODS: A comprehensive search of the literature using relevant search words was conducted from the date of each database inception to August 2018 to identify interventions and outcomes in patients with CDH and HLHS. Conference papers, review articles, case reports, and animal studies were not eligible for this review. Articles were also excluded if left heart hypoplasia, rather than HLHS, was studied. Neither language nor sample size was used for exclusion. A preliminary screening of article titles was performed to rule out irrelevant articles and article types by a single reviewer. A second screening circle was performed by two independent reviewers to assess remaining abstracts. Data were presented as a systematic review and were descriptively summarized.

RESULTS: Of the 162 retrieved articles, five studies were eligible for inclusion. The total number of neonates included in this systematic review was 5063. One hundred and eighteen (2.3%) presented specifically with CDH and HLHS. Overall, a total of 62 of the 118 patients underwent a surgical procedure (51%) with 36 (59%) surviving to discharge.

CONCLUSIONS: Available data suggest current surgical interventions still result in a high degree of mortality. Age at which surgery occurred, pulmonary function and the use of extra-corporeal membrane oxygenation (ECMO) are potential variables that may determine and improve outcomes in these patients in the future. However, long-term outcomes and analyses of more variables are needed to determine effective treatment for this rare but often fatal comorbid congenital diagnosis.