Neil Patel1, Pamela A Lally2, Florian Kipfmueller3, Anna Claudia Massolo4, Matias Luco5, Krisa P Van Meurs6, Kevin P Lally2, Matthew T Harting2. 1. Department of Neonatology, Royal Hospital for Children, Glasgow, United Kingdom. 2. Department of Pediatric Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston and Children's Memorial Hermann Hospital, Houston, Texas. 3. Department of Neonatology and Pediatric Critical Care Medicine, University of Bonn, Bonn, Germany. 4. Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, Scientific Institute for Research, Hospitalization and Healthcare, Rome, Italy. 5. Department of Neonatology, Pontifical Catholic University of Chile, Santiago, Chile; and. 6. Division of Neonatal and Developmental Medicine, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California.
Abstract
Rationale: 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. Objectives: Our aim was to investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH. Methods: 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 (RVdys), left ventricular dysfunction only (LVdys), and combined RV and LV dysfunction (RV&LVdys). Univariate, multivariate, and Cox proportional hazards regression analyses were performed.Measurements and Main Results: Cardiac function data from early echocardiograms were available for 1,173 (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%; and RV&LVdys, 51% (P < 0.001). The adjusted risk of death (hazard ratio) for cases with LVdys was 1.96 (95% confidence interval [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 extracorporeal membrane oxygenation (P < 0.005).Conclusions: Early ventricular dysfunction occurs frequently in CDH and is an independent determinant of severity and clinical outcome.
Rationale: 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. Objectives: Our aim was to investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH. Methods: 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 (RVdys), left ventricular dysfunction only (LVdys), and combined RV and LV dysfunction (RV&LVdys). Univariate, multivariate, and Cox proportional hazards regression analyses were performed.Measurements and Main Results: Cardiac function data from early echocardiograms were available for 1,173 (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%; and RV&LVdys, 51% (P < 0.001). The adjusted risk of death (hazard ratio) for cases with LVdys was 1.96 (95% confidence interval [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 extracorporeal membrane oxygenation (P < 0.005).Conclusions: Early ventricular dysfunction occurs frequently in CDH and is an independent determinant of severity and clinical outcome.
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