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

Congenital Diaphragmatic Hernia Study Group

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
JournalAmerican Journal of Respiratory and Critical Care Medicine
DOIs
Publication statusE-pub ahead of print - Aug 13 2019

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Ventricular Dysfunction
Left Ventricular Dysfunction
Mortality
Right Ventricular Dysfunction
Ventricular Function
Congenital Diaphragmatic Hernias
Prenatal Diagnosis
Registries
Regression Analysis
Morbidity
Membranes
Liver

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

In: American Journal of Respiratory and Critical Care Medicine, 13.08.2019.

Research output: Contribution to journalArticle

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title = "Ventricular Dysfunction is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia",
abstract = "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.",
author = "{Congenital Diaphragmatic Hernia Study Group} and Neil Patel and Lally, {Pamela A} and Florian Kipfmueller and Massolo, {Anna Claudia} and Matias Luco and {Van Meurs}, {Krisa P} and Lally, {Kevin P} and Harting, {Matthew T}",
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journal = "American Journal of Respiratory and Critical Care Medicine",
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T1 - Ventricular Dysfunction is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia

AU - Congenital Diaphragmatic Hernia Study Group

AU - Patel, Neil

AU - Lally, Pamela A

AU - Kipfmueller, Florian

AU - Massolo, Anna Claudia

AU - Luco, Matias

AU - Van Meurs, Krisa P

AU - Lally, Kevin P

AU - Harting, Matthew T

PY - 2019/8/13

Y1 - 2019/8/13

N2 - 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.

AB - 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.

U2 - 10.1164/rccm.201904-0731OC

DO - 10.1164/rccm.201904-0731OC

M3 - Article

C2 - 31409095

JO - American Journal of Respiratory and Critical Care Medicine

JF - American Journal of Respiratory and Critical Care Medicine

SN - 1073-449X

ER -