| Autor: |
Freud LR; Division of Cardiology Department of Pediatrics NewYork-Presbyterian Morgan Stanley Children's Hospital Columbia University Medical Center New York NY., McElhinney DB; Division of Cardiology Department of Pediatrics Lucile Packard Children's Hospital Stanford School of Medicine Palo Alto CA., Kalish BT; Division of Newborn Medicine Department of Pediatrics Boston Children's Hospital Harvard Medical School Boston MA., Escobar-Diaz MC; Department of Pediatric Cardiology Hospital Sant Joan de Déu Universitat de Barcelona Spain., Komarlu R; Division of Pediatric Cardiology Department of Pediatrics Cleveland Clinic Children's Hospital Lerner College of Medicine at Case Western Reserve University Cleveland OH., Puchalski MD; Division of Cardiology Department of Pediatrics Primary Children's Hospital University of Utah School of Medicine Salt Lake City UT., Jaeggi ET; Division of Cardiology Department of Paediatrics Hospital for Sick Children University of Toronto Toronto Ontario Canada., Szwast AL; Division of Cardiology Department of Pediatrics Children's Hospital of Philadelphia Perelman School of Medicine at the University of Pennsylvania Philadelphia PA., Freire G; Division of Cardiology Department of Pediatrics Johns Hopkins All Children's Hospital St. Petersburg FL., Levasseur SM; Division of Cardiology Department of Pediatrics NewYork-Presbyterian Morgan Stanley Children's Hospital Columbia University Medical Center New York NY., Kavanaugh-McHugh A; Division of Cardiology Department of Pediatrics Monroe Carell Jr. Children's Hospital Vanderbilt University School of Medicine Nashville TN., Michelfelder EC; Division of Cardiology Department of Pediatrics Children's Healthcare of Atlanta Sibley Heart Center Emory University School of Medicine Atlanta GA., Moon-Grady AJ; Division of Cardiology Department of Pediatrics UCSF Benioff Children's Hospital University of California-San Francisco School of Medicine San Francisco CA., Donofrio MT; Division of Cardiology Department of Pediatrics Children's National Medical Center George Washington University School of Medicine and Health Sciences Washington DC., Howley LW; Division of Cardiology Department of Pediatrics Children's Hospital Colorado University of Colorado School of Medicine Aurora CO., Selamet Tierney ES; Division of Cardiology Department of Pediatrics Lucile Packard Children's Hospital Stanford School of Medicine Palo Alto CA., Cuneo BF; Division of Cardiology Department of Pediatrics Children's Hospital Colorado University of Colorado School of Medicine Aurora CO., Morris SA; Division of Cardiology Department of Pediatrics Texas Children's Hospital Baylor College of Medicine Houston TX., Pruetz JD; Division of Cardiology Department of Pediatrics Children's Hospital Los Angeles University of Southern California Keck School of Medicine Los Angeles CA., van der Velde ME; Division of Cardiology Department of Pediatrics University of Michigan Congenital Heart Center C.S. Mott Children's Hospital University of Michigan Medical School Ann Arbor MI., Kovalchin JP; Division of Cardiology Department of Pediatrics Nationwide Children's Hospital Ohio State University College of Medicine Columbus OH., Ikemba CM; Division of Cardiology Department of Pediatrics Children's Medical Center University of Texas Southwestern Medical School Dallas TX., Vernon MM; Division of Cardiology Department of Pediatrics Seattle Children's Hospital University of Washington School of Medicine Seattle WA., Samai C; Division of Cardiology Department of Pediatrics Children's Healthcare of Atlanta Emory University School of Medicine Atlanta GA., Satou GM; Division of Cardiology Department of Pediatrics Mattel Children's Hospital University of California-Los Angeles David Geffen School of Medicine Los Angeles CA., Gotteiner NL; Division of Cardiology Department of Pediatrics Ann & Robert H. Lurie Children's Hospital of Chicago Northwestern University Feinberg School of Medicine Chicago IL., Phoon CK; Division of Cardiology Department of Pediatrics Hassenfeld Children's Hospital at NYU Langone New York University School of Medicine New York NY., Silverman NH; Division of Cardiology Department of Pediatrics Lucile Packard Children's Hospital Stanford School of Medicine Palo Alto CA.; Division of Cardiology Department of Pediatrics UCSF Benioff Children's Hospital University of California-San Francisco School of Medicine San Francisco CA., Tworetzky W; Department of Cardiology Boston Children's Hospital Harvard Medical School Boston MA. |
| Abstrakt: |
Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1-5.0], 95% CI, per m/s; P =0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3-14.2]; P =0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. Conclusions Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival. |
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