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      Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study

      research-article
      Author(s): , MD 1 , , , MD 2 , , MD 3 , , MD 4 , , MD 5 , , MD 6 , , MD 7 , , MD 8 , , MD 9 , , MD 1 , , MD 10 , , MD 11 , , MD 12 , , MD 13 , , MD 14 , , MD 2 , , MD 14 , , MD, MPH 15 , , MD 16 , , MD 17 , , MD 18 , , MD 19 , , MD 20 , , MD 21 , , MD 22 , , MD 23 , , MD 24 , , MD 2 , 12 , , MD 25
      Publication date (Electronic):
      Journal: Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
      Publisher: John Wiley and Sons Inc.
      Keywords: congenital heart disease, Ebstein anomaly, mortality, neonate, outcome, palliation, right ventricle, tricuspid regurgitation, Clinical Studies, Congenital Heart Disease, Valvular Heart Disease, Echocardiography, Mortality/Survival

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          Abstract

          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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          The incidence of congenital heart disease.

          Julien Hoffman, Samuel Kaplan (2002)
          This study was designed to determine the reasons for the variability of the incidence of congenital heart disease (CHD), estimate its true value and provide data about the incidence of specific major forms of CHD. The incidence of CHD in different studies varies from about 4/1,000 to 50/1,000 live births. The relative frequency of different major forms of CHD also differs greatly from study to study. In addition, another 20/1,000 live births have bicuspid aortic valves, isolated anomalous lobar pulmonary veins or a silent patent ductus arteriosus. The incidences reported in 62 studies published after 1955 were examined. Attention was paid to the ways in which the studies were conducted, with special reference to the increased use of echocardiography in the neonatal nursery. The total incidence of CHD was related to the relative frequency of ventricular septal defects (VSDs), the most common type of CHD. The incidences of individual major forms of CHD were determined from 44 studies. The incidence of CHD depends primarily on the number of small VSDs included in the series, and this number in turn depends upon how early the diagnosis is made. If major forms of CHD are stratified into trivial, moderate and severe categories, the variation in incidence depends mainly on the number of trivial lesions included. The incidence of moderate and severe forms of CHD is about 6/1,000 live births (19/1,000 live births if the potentially serious bicuspid aortic valve is included), and of all forms increases to 75/1,000 live births if tiny muscular VSDs present at birth and other trivial lesions are included. Given the causes of variation, there is no evidence for differences in incidence in different countries or times.
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            Prevalence of congenital heart disease.

            Richard R. Liberthson, Matthew G Hoffman, S. Kaplan (2004)
            Today most patients with congenital heart disease survive childhood to be cared for by adult cardiologists. The number of physicians that should be trained to manage these lesions is unknown because we do not know the number of patients. To answer this question, the expected numbers of infants with each major type of congenital heart defect born in each 5-year period since 1940 were estimated from birth rates and incidence. The numbers expected to survive with or without treatment were estimated from data on natural history and the results of treatment. Finally, lesions were categorized as simple, moderate, or complex, based on the amount of expertise in management needed for optimal patient care. From 1940 to 2002, about 1 million patients with simple lesions, and half that number each with moderate and complex lesions, were born in the United States. If all were treated, there would be 750,000 survivors with simple lesions, 400,000 with moderate lesions, and 180,000 with complex lesions; in addition, there would be 3,000,000 subjects alive with bicuspid aortic valves. Without treatment, the survival in each group would be 400,000, 220,000, and 30,000, respectively. The actual numbers surviving will be between these 2 sets of estimates. Survival of patients with congenital heart disease, treated or untreated, is expected to produce large numbers of adults with congenital disease, and it is likely that many more adult cardiologists will need to be trained to manage moderate and complex congenital lesions.
            Article 0 comments Cited 145 times – based on 0 reviews     Review now
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              Theoretical and empirical derivation of cardiovascular allometric relationships in children.

              Thierry Sluysmans, Steven D Colan (2005)
              Basic fluid dynamic principles were used to derive a theoretical model of optimum cardiovascular allometry, the relationship between somatic and cardiovascular growth. The validity of the predicted models was then tested against the size of 22 cardiovascular structures measured echocardiographically in 496 normal children aged 1 day to 20 yr, including valves, pulmonary arteries, aorta and aortic branches, pulmonary veins, and left ventricular volume. Body surface area (BSA) was found to be a more important determinant of the size of each of the cardiovascular structures than age, height, or weight alone. The observed vascular and valvar dimensions were in agreement with values predicted from the theoretical models. Vascular and valve diameters related linearly to the square root of BSA, whereas valve and vascular areas related to BSA. The relationship between left ventricular volume and body size fit a complex model predicted by the nonlinear decrease of heart rate with growth. Overall, the relationship between cardiac output and body size is the fundamental driving factor in cardiovascular allometry.
              Article 0 comments Cited 76 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Lindsay R. Freud:
                ORCID: https://orcid.org/0000-0002-1221-081X
                lindsay.freud@sickkids.ca
                Journal
                Journal ID (nlm-ta): J Am Heart Assoc
                Journal ID (iso-abbrev): J Am Heart Assoc
                Journal ID (doi): 10.1002/(ISSN)2047-9980
                Journal ID (publisher-id): JAH3
                Journal ID (hwp): ahaoa
                Title: Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Electronic): 2047-9980
                Publication date (Electronic): 20 October 2020
                Publication date Collection: 03 November 2020
                Volume: 9
                Issue: 21 ( doiID: 10.1002/jah3.v9.21 )
                Electronic Location Identifier: e016684
                Affiliations
                [ 1 ] Division of Cardiology Department of Pediatrics NewYork‐Presbyterian Morgan Stanley Children’s Hospital Columbia University Medical Center New York NY
                [ 2 ] Division of Cardiology Department of Pediatrics Lucile Packard Children’s Hospital Stanford School of Medicine Palo Alto CA
                [ 3 ] Division of Newborn Medicine Department of Pediatrics Boston Children’s Hospital Harvard Medical School Boston MA
                [ 4 ] Department of Pediatric Cardiology Hospital Sant Joan de Déu Universitat de Barcelona Spain
                [ 5 ] Division of Pediatric Cardiology Department of Pediatrics Cleveland Clinic Children’s Hospital Lerner College of Medicine at Case Western Reserve University Cleveland OH
                [ 6 ] Division of Cardiology Department of Pediatrics Primary Children’s Hospital University of Utah School of Medicine Salt Lake City UT
                [ 7 ] Division of Cardiology Department of Paediatrics Hospital for Sick Children University of Toronto Toronto Ontario Canada
                [ 8 ] Division of Cardiology Department of Pediatrics Children’s Hospital of Philadelphia Perelman School of Medicine at the University of Pennsylvania Philadelphia PA
                [ 9 ] Division of Cardiology Department of Pediatrics Johns Hopkins All Children’s Hospital St. Petersburg FL
                [ 10 ] Division of Cardiology Department of Pediatrics Monroe Carell Jr. Children’s Hospital Vanderbilt University School of Medicine Nashville TN
                [ 11 ] Division of Cardiology Department of Pediatrics Children’s Healthcare of Atlanta Sibley Heart Center Emory University School of Medicine Atlanta GA
                [ 12 ] Division of Cardiology Department of Pediatrics UCSF Benioff Children’s Hospital University of California‐San Francisco School of Medicine San Francisco CA
                [ 13 ] Division of Cardiology Department of Pediatrics Children’s National Medical Center George Washington University School of Medicine and Health Sciences Washington DC
                [ 14 ] Division of Cardiology Department of Pediatrics Children’s Hospital Colorado University of Colorado School of Medicine Aurora CO
                [ 15 ] Division of Cardiology Department of Pediatrics Texas Children’s Hospital Baylor College of Medicine Houston TX
                [ 16 ] Division of Cardiology Department of Pediatrics Children’s Hospital Los Angeles University of Southern California Keck School of Medicine Los Angeles CA
                [ 17 ] 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
                [ 18 ] Division of Cardiology Department of Pediatrics Nationwide Children’s Hospital Ohio State University College of Medicine Columbus OH
                [ 19 ] Division of Cardiology Department of Pediatrics Children’s Medical Center University of Texas Southwestern Medical School Dallas TX
                [ 20 ] Division of Cardiology Department of Pediatrics Seattle Children’s Hospital University of Washington School of Medicine Seattle WA
                [ 21 ] Division of Cardiology Department of Pediatrics Children’s Healthcare of Atlanta Emory University School of Medicine Atlanta GA
                [ 22 ] Division of Cardiology Department of Pediatrics Mattel Children’s Hospital University of California‐Los Angeles David Geffen School of Medicine Los Angeles CA
                [ 23 ] Division of Cardiology Department of Pediatrics Ann & Robert H. Lurie Children’s Hospital of Chicago Northwestern University Feinberg School of Medicine Chicago IL
                [ 24 ] Division of Cardiology Department of Pediatrics Hassenfeld Children’s Hospital at NYU Langone New York University School of Medicine New York NY
                [ 25 ] Department of Cardiology Boston Children’s Hospital Harvard Medical School Boston MA
                Author notes
                [*] [* ]Correspondence to: Lindsay R. Freud, MD, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada. E‐mail: lindsay.freud@ 123456sickkids.ca
                Author information
                https://orcid.org/0000-0002-1221-081X
                https://orcid.org/0000-0001-9085-0882
                https://orcid.org/0000-0003-0956-071X
                Article
                Publisher ID: JAH35602
                DOI: 10.1161/JAHA.120.016684
                PMC ID: 7763426
                PubMed ID: 33076749
                SO-VID: 769239b1-375a-4ae9-9aa1-dcb856d4d780
                Copyright © © 2020 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.
                License:

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                Date received : 19 March 2020
                Date accepted : 06 September 2020
                Page count
                Figures: 4, Tables: 4, Pages: 13, Words: 8982
                Categories
                Subject: Original Research
                Subject: Original Research
                Subject: Pediatric Cardiology
                Custom metadata
                source-schema-version-number 2.0
                cover-date 03 November 2020
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:5.9.3 mode:remove_FC converted:03.11.2020

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: congenital heart disease,ebstein anomaly,mortality,neonate,outcome,palliation,right ventricle,tricuspid regurgitation,clinical studies,valvular heart disease,echocardiography,mortality/survival
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: congenital heart disease, ebstein anomaly, mortality, neonate, outcome, palliation, right ventricle, tricuspid regurgitation, clinical studies, valvular heart disease, echocardiography, mortality/survival

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