Echocardiography derived pulmonary artery capacitance and right ventricular outflow velocity time integral on first day of life can predict survival in congenital diaphragmatic hernia

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Highlights

  • Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome despite many advances in care.

  • Liver herniation, ECMO duration and diaphragmatic hernia type can differentiate survivors versus non-survivors CDH infants.

  • Pulmonary artery capacitance and RVOT velocity time integral can differentiate survivors versus non-survivors CDH infants.

  • PAC and RVOT velocity time integral can be obtained in every patient regardless of the severity of TR (even mild TR jet).

Abstract

Correlation between right ventricle systolic pressure (derived from moderate/severe tricuspid regurgitation) and survival of congenital diaphragmatic hernia (CDH) infants was established. We hypothesize that other non-tricuspid valve regurgitation (TR) dependent Echo parameters can predict CDH mortality. Our retrospective study included 20 CDH infants from January 2008 to September 2015. Inclusion criteria included: all CDH patients admitted to our neonatal intensive care unit. Exclusion criteria were: hereditary malformation of air ways, congenital heart disease other than patent ductus arteriosus (PDA) and/or PFO (patent foramen ovale) or atrial septal defect (ASD), sepsis, genetic syndromes and high frequency ventilation usage. Relevant non-Echo data was collected. The following Echo parameters were evaluated: severity of TR, ratio between systolic and diastolic duration of right ventricle (RV), pulmonary artery capacitance (PAC), RV outflow tract velocity time integral (RVOT VTI), and others.

CDH survivors showed higher RVOT VTI (12.3 ± 3 ml vs 9 ± 3.1 ml), and higher PAC (0.3 ± 0,2 ml3 × mm Hg 1 versus 0.18 ± 0.07 m3 × mm Hg 1). Cronbach's alpha for intra-rater reliability was 0.82 for PAC and 0.98 for RVOT VTI and for inter-rater reliability was 0.74 and 0.89 consecutively. RVOT VTI of value > 10.5 ml and PAC of value > 0.24 ml3 × mm Hg 1 differentiated CDH survivors with area under curve (AUC) 0.78(p = 0.02) and AUC 0.89(p = 0.002) consecutively with sensitivity and specificity for both > 70%. Proportional Hazard analysis showed PAC < 0.24 has a mortality risk ratio of 25.8 versus 4.36 for RVOT VTI < 10.5. First 24 h echo derived (PAC) and (RVOT VTI) can predict survivors in congenital diaphragmatic hernia patients.

Section snippets

Abbreviations used

CDHcongenital diaphragmatic hernia
PACpulmonary artery capacitance
PVRpulmonary vascular resistance
RVOT VTIright ventricular outflow velocity time integral
ATacceleration time
RVETright ventricular ejection time

Methods

After institutional review board approval, we reviewed the records of 28 consecutive congenital diaphragmatic hernia patients treated at our institution from January 2008 through December 2015. Inclusion criteria included: all congenital diaphragmatic hernia patients admitted to Virginia Commonwealth University neonatal intensive care unit. Exclusion criteria were: genetic syndrome (e.g. Down syndrome), hereditary malformation of upper or lower air way disease, pulmonary vein stenosis, aorto

Results

We reviewed all 28 CDH infants presenting over the 7-year period. Eight infants were excluded due to meconium aspiration (n = 2), perinatal hypoxia (n = 2), positive for group B streptococci (n = 20, hypothermia (n = 1) and inadequate echo data (n = 1). The remaining 20 patients represent the study group.

Frequency of liver herniation (p = 0.02), ECMO duration (p = 0.003) and type of repair (p = 0.0001) differentiated survivors from non-survivors in CDH patients. While, gestational age (p = 0.05), gender (p = 0.6),

Discussion

Congenital diaphragmatic hernia is a condition with a highly variable outcome despite many advances in care. Identifying high-risk infants prenatally as well as postnatally may allow for targeted therapy [15] and help physicians reduce resource use by directing expensive treatment (ECMO) towards patients who may best benefit from it [16].

We found three non-echo parameters that can differentiate survivors versus non-survivors: liver herniation, ECMO duration and type of diaphragmatic hernia

Conclusion

Pulmonary artery capacitance and RVOT velocity time integral measured in first 24 h of life can predict survivors versus non-survivors CDH infants. Furthermore, these echocardiographic parameters can be obtained in every patient regardless of the severity of TR. Pulmonary artery capacitance and RV velocity time integral may better detect the total RV-pulmonary vascular interaction in CDH patients and may be helpful to direct the clinical management of these patients and perhaps others.

Recommendation

Larger prospective studies are needed that incorporate pulmonary artery capacitance and RVOT velocity time integral in each echo study for CDH infants to validate or reject our results. Our results may help in parents counseling and ECMO support patient selection.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not –for-profit services.

Acknowledgment

We thank Dr. Zack Goode at Virginia Commonwealth University for assistance with methodology that greatly improved the manuscript.

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Authors declare there is no conflict of interest.

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