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Research: Ventricular Dysfunction is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia


Research: Ventricular Dysfunction is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia

Am J Respir Crit Care Med. 2019 Aug 13. doi: 10.1164/rccm.201904-0731OC. [Epub ahead of print]


Ventricular Dysfunction is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia.

Patel N1Lally PA2Kipfmueller F3Massolo AC4Luco M5Van Meurs KP6Lally KP2Harting MT7Congenital Diaphragmatic Hernia Study Group.

Author information

1Royal Hospital for Children Glasgow, 59842, Department of Neonatology, Glasgow, Glasgow, United Kingdom of Great Britain and Northern Ireland; neilpatel1@nhs.net.2University of Texas Health Science Center at Houston, 12340, Department of Pediatric Surgery, Houston, Texas, United States.3University of Bonn, Department of Neonatology, Bonn, Germany.4Bambino Gesù Children’s Hospital, Department of Medical and Surgical Neonatology, Rome, Italy.5Pontificia Universidad Católica de Chile, 28033, Department of Neonatology, Santiago, Chile.6Stanford University School of Medicine, 10624, Department of Pediatrics, Division of Neonatal and Developmental Medicine, Stanford, California, United States.7University of Texas Health Science Center at Houston, 12340, Pediatric Surgery, Houston, Texas, United States.


RATIONALE Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and under-recognized contributor to CDH pathophysiology and determinant of disease severity. OBJECTIVES To investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH. METHODS Multicenter, prospectively-collected data in the Congenital Diaphragmatic Hernia Study Group (CDHSG) registry, abstracted between 2015 and 2018, were evaluated. Ventricular function on early echocardiograms, obtained within the first 48 hours of life, was categorized into four hierarchical groups: normal function, right ventricular dysfunction only (RVdys), left ventricular dysfunction only (LVdys), and combined RV and LV dysfunction (RV&LVdys). Univariate, multivariate, and Cox proportional hazards regression analyses were performed. MEASUREMENT AND MAIN RESULTS Cardiac function data from early echocardiograms were available for 1173 (71%) cases and categorized as normal in 711 (61%), RVdys in 182 (15%), LVdys in 61 (5%), and combined RV&LVdys in 219 (19%) cases. Ventricular dysfunction was significantly associated with prenatal diagnosis, CDHSG stage, intrathoracic liver, and patch repair (all p<0.001). Survival varied by category: normal function 80%, RVdys 74%, LVdys 57%, RV&LVdys 51%, p<0.001. The adjusted risk of death (hazard ratio) for cases with LVdys was 1.96 (95% CI 1.29 – 2.98, p=0.020) and for cases with RV&LVdys was 2.27 (95% CI 1.77 – 2.92, p=0.011). All cardiac dysfunction categories were associated with use of extra-corporeal membrane oxygenation (ECMO) (p<0.005). CONCLUSIONS Early ventricular dysfunction occurs frequently in CDH and is an independent determinant of severity and clinical outcome.


heart; pulmonary hypertensionPMID: 31409095 DOI: 10.1164/rccm.201904-0731OC

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