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Congenital Heart Disease in Association with Congenital Diaphragmatic Hernia. Impact on Outcome.

Usha Krishnan

E. Hahn

J. Wynn

G. Aspelund

W. Chung

M. S. Arkovitz

Erika Berman Rosenzweig


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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 06.22.2012

Presentation Type: Abstracts

U. Krishnan1, E. Hahn1, J. Wynn1, G. Aspelund1, W. Chung1, M.S. Arkovitz2, E. Berman Rosenzweig1

1. Columbia University Medical Center - New York, NY/US, 2. Rambam Medical Center - Haifa/IL DHREAMS study group

BACKGROUND: Congenital Diaphragmatic Hernia (CDH) occurs in 1:3000 live births and is associated with a post-operative survival of 60-70%. Congenital heart disease (CHD) has been reported in 11-15% of CDH, and is associated with a worse prognosis.

METHODS: We analyzed the influence of CHD in association with CDH on overall outcome, using data of patients with CDH from Columbia University Medical Center, who were enrolled in the DHREAMS (Diaphragmatic Hernia Research and Exploration, Advancing Medical Design)- RedCap database. Data analyzed included sampling (fetal Vs postnatal), echocardiographic and or autopsy diagnosis of CHD, type of CHD and outcome. Anomalies of cardiac position, fetal circulation remnants like patent foramen ovale and patent ductus arteriosus and physiologic valve incompetence were not included in the analysis. Patients with genetic syndromes were excluded. Outcomes analyzed were surgery for CDH, surgery for CHD and mortality.

RESULTS: 9/18 (50%) fetal and 29/189 (15.3%) postnatal patient data entries had a diagnosis of CHD. Complex anomalies included double outlet right ventricle (DORV) in 4, transposition of great arteries (TGA) in 3, aortic arch obstructions in 4. Other abnormalities seen were av canal defect(2), total anomalous veins, pulmonary atresia, tetralogy of Fallot (TOF), tricuspid atresia and hypoplastic left heart (HLHS) in 1 patient each. Simple defects included were secundum ASD (14) and VSD (18). There were 6 postnatal deaths, of whom, 4 (1 HLHS, 1 pulmonary atresia, 1 extreme prematurity with VSD and 1 avcanal defect) died in the neonatal period before CDH repair. Of the 2 who died post-operatively, 1 had severe coarctation with heart failure and the other had an unbalanced av canal with single ventricle. All the other 23 patients including 3 with TGA and 2 DORV/TOF had successful CDH and subsequent CHD repair and are alive at several years' follow-up.

CONCLUSION: Complex CHD with CDH is associated with a high rate of fetal demise. Postnatally, survival was excellent even for complex defects with CDH in this single institution cohort of patients.