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Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience.

[congenital diaphragmatic hernia]

To evaluate the impact of congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH).In the present retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and November 2012 in a single center were reviewed. Cases were classified into two groups: those with "isolated" CDH and those with both CDH and CHA. Patients with CHA were further sub-classified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery 1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery score (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies (including 'syndromic cases') were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of life and need for Extracorporeal Membrane Oxygenation (ECMO), respectively.Out of 180 infants with CDH, 41 patients were excluded because of the presence of non-cardiac associated anomalies. 118 infants had isolated CDH and 21 had CDH+CHA (16 with minor CHA and 5 with major CHA). Receiver operating characteristic (ROC) analyses demonstrated the best cut-off for survival was when the scoring value wasthan 2 for both RACHS-1 (area under the curve: 0.74; p=0.04; sensitivity=80.0%; specificity=87.5%) and STS-EACTS (area under the curve: 0.83; p=0.03; sensitivity=100%; specificity=87.5%). The survival rate at 1 year of life was significantly lower in the group of major CHA+CHD (40.0%; p=0.04) in contrast with 'isolated CDH' (77.1%) and 'CDH + minor CHA' (81.3%). We found no significant differences among the groups concerning the need for ECMO.In general, mild forms of CHA do not appear to impact negatively on survival for infants with CDH. However, mortality appears significantly higher in infants with major forms of CHA in infants with CDH. The scoring systems appear to be useful predictors to classify the effects of CHA in this population of patients.