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Risk Factors for Persistent Pulmonary Hypertension and Mortality in Congenital Diaphragmatic Hernia

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 at a frequency of about 1:3000 live births and constitutes about 8% of birth defects. Historically, neonatal mortality was reported ~ 50%, largely from pulmonary hypoplasia and pulmonary arterial hypertension (PAH), although with improved surgical techniques and neonatal management, survival is now reported ~ 60-70%. We analyzed the prevalence of pulmonary hypertension (PH) by echocardiogram after CDH repair at 1 and 3 months of age, and correlated the same with prenatal factors including timing of diagnosis, side of CDH, use of ECMO and use of inhaled nitric oxide (iNO). In patients diagnosed with PH at 1 month, we analyzed factors associated with persistence of PH at 3 months of age.

METHODSThe DHREAMS (Diaphragmatic Hernia Research and Exploration, Advancing Molecular Design) RedCap database was used to obtain data of patients diagnosed with CDH after 2007 at Columbia University Medical Center. Echocardiograms were read by a cardiologist blinded to patient data, and the degree of PH was ranked as A none, B mild, C moderate, D severe, based on tricuspid regurgitant gradient, septal position, RVH, PDA gradient and RV function.

RESULTS: Overall mortality was 17/123, with 7 pre-op and 10/116 post-op (8.6%) deaths. One month echocardiograms were available in 67 patients, and patients were classified into group A- 17, B- 12; C- 23; D- 15 based on degree of PH. At 3 months follow up, all in groups A and B improved or remained the same and there was no mortality. Group C and D patients received targeted PH therapy. In Group C, PH improved in 9/12; 2/12 -same; 1/12 died. Group D- 3/15 no PH; 4/15 mild-moderate; 3/15 severe; 3/15 died. ECMO (n=16) pre-op did not predict PH at 1 month, and there were 4 deaths (3 post-OP). iNO (n=24) use peri-operatively was associated with 3 pre-op and 2 post-op deaths. 3/12 who received iNO+ECMO, died post-OP. The use of iNO or ECMO did not correlate with outcome at 3 months. The only significant correlation with mortality was presence of severe PH at 1 month (p<0.05).

CONCLUSIONS. Improved surgical, intensive care and PH management techniques have resulted in improved survival (91.4%) after CDH repair. Presence of severe PH at 1 month is a risk factor for persistence of PH and mortality. Use of ECMO or iNO did not correlate with post-operative mortality in this single institution cohort of patients.