Ventricular Dysfunction Is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and underrecognized contributor to CDH pathophysiology and determinant of disease severity. Our aim was to investigate the association between early, postnatal ventricular...

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Bibliographic Details
Published in:American journal of respiratory and critical care medicine 2019-12, Vol.200 (12), p.1522-1530
Main Authors: Patel, Neil, Lally, Pamela A, Kipfmueller, Florian, Massolo, Anna Claudia, Luco, Matias, Van Meurs, Krisa P, Lally, Kevin P, Harting, Matthew T
Format: Article
Language:English
Subjects:
Cardiac arrhythmia
Congenital diseases
Databases, Factual
Echocardiography
Female
Hernias
Hernias, Diaphragmatic, Congenital - complications
Hernias, Diaphragmatic, Congenital - diagnosis
Hernias, Diaphragmatic, Congenital - mortality
Humans
Infant
Infant, Newborn
Male
Physiology
Retrospective Studies
Risk Factors
Survival Rate
Ventricular Dysfunction, Left - complications
Ventricular Dysfunction, Left - diagnosis
Ventricular Dysfunction, Left - mortality
Ventricular Dysfunction, Right - complications
Ventricular Dysfunction, Right - diagnosis
Ventricular Dysfunction, Right - mortality
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Summary:Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and underrecognized contributor to CDH pathophysiology and determinant of disease severity. Our aim was to investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH. Multicenter, prospectively collected data in the CDH Study Group (CDHSG) registry, abstracted between 2015 and 2018, were evaluated. Ventricular function on early echocardiograms, defined as obtained within the first 48 hours of life, was categorized into four hierarchical groups: normal function, right ventricular dysfunction only (RV ), left ventricular dysfunction only (LV ), and combined RV and LV dysfunction (RV&LV ). Univariate, multivariate, and Cox proportional hazards regression analyses were performed. Cardiac function data from early echocardiograms were available for 1,173 (71%) cases and categorized as normal in 711 (61%), RV in 182 (15%), LV in 61 (5%), and combined RV&LV in 219 (19%) cases. Ventricular dysfunction was significantly associated with prenatal diagnosis, CDHSG stage, intrathoracic liver, and patch repair (all  
ISSN:1073-449X
1535-4970
DOI:10.1164/rccm.201904-0731OC