Objective: Ventricular dysfunction may be found in 40% of newborns with CDH. Ventricular performance is not only a predictor of disease severity but also mortality and need for ECMO. We studied the utility of serial echocardiography in the management of newborns with CDH and on survival outcomes.Study Design: This is a retrospective study wherein the demographic, clinical and echocardiographic data were retrieved from the local CDH registry and hospital clinical database. We studied the correlation of timed echocardiographic findings with mortality and other outcomes.Results: Fourty-two newborns with CDH were admitted during the study period (M/F:19/23), with median gestation of 38 weeks (IQR:36-39) and birth weight of 2.83 kg (IQR 2.45-3.17). Thirty-one were left-sided, seven right, one central, and three bilateral hernias. Twelve infants (28%) died in early infancy. Three infants were excluded from our analysis due to either palliation at birth or significant cardiac anomaly, resulting in a total of 137 echos from 39 infants being included in the analysis. Seventy percent of newborns who died and had an echo in first 72 hours, suffered from moderate to severe PH. Birth weight <2.8Kg, RVSP> 45.5 in the first 72 hours and postoperative VIS >23.5 and RSS >4.3 were good predictors of mortality in our cohort. Markers of elevated pulmonary pressures and cardiac function were useful in guiding therapy.Conclusions: Serial timed functional echocardiography (f-Echo) monitoring allows targeted therapy of patients with CDH. Birth weight, initial severity of pulmonary hypertension and postop RSS and VIS may be useful in predicting mortality.

Purpose: To assess the effect of inhaled nitric oxide (iNO) on oxygenation in the management of pulmonary hypertension (PH) secondary to arteriovenous malformations (AVMs) in neonates.Method: A matched retrospective cohort study from January 1, 2013, to December 31, 2017. European inhaled nitric oxide registry from 43 neonatal and pediatric ICUs in 13 countries across Europe was used to extract data. The target population was neonates treated with iNO for the management of PH. The cases (PH secondary to AVMs treated with iNO) were matched (1:4 ratio) to controls (PH without AVMs treated with iNO). The main outcome measure was the absolute change of oxygenation index (OI) from baseline to 60 minutes after starting iNO in cases and control.Results: A total of 45 infants out of 1830 (9 cases and 36 controls) were identified, who received inhaled nitric oxide during ICU stay. The mean baseline value of OI (%) (cases: 27.5, controls: 34), the mean value of OI (%) after 60 minutes of iNO use (cases: 16.8, controls: 28.5), and the mean absolute change in OI value (%) from baseline to 60 minutes of iNO (cases: 10.7 and controls: 6) was not statistically different between groups.Conclusion: Infants with PH secondary to AVMs treated with iNO did not respond differently compared to babies presented with PH without AVMs treated with iNO. Right ventricle dysfunction on echocardiography was higher in cases (cases: 66.7% and controls: 28.6%) but not statistically significant. The death rate before discharge was significantly higher among cases (cases: 55.6% and controls: 8.3%).