title: Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia.
abstract: In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.
authors: Division of Cardiology, Department of Pediatrics, Stanford University, Palo Alto, California. Electronic address: tierneys@stanford.edu.
authors: Elif Seda
authors: ES
authors: Selamet Tierney
authors: Division of Cardiology, Department of Pediatrics, Stanford University, Palo Alto, California.
authors: Doff B
authors: DB
authors: McElhinney
authors: Division of Cardiology, Department of Pediatrics, Columbia University Medical Center, New York, New York.
authors: Lindsay R
authors: LR
authors: Freud
authors: Department of Pediatric Cardiology, Harvard Medical School, Boston, Massachusetts.
authors: Wayne
authors: W
authors: Tworetzky
authors: Division of Cardiology, Department of Pediatrics, Children's Hospital Colorado, Denver, Colorado.
authors: Bettina F
authors: BF
authors: Cuneo
authors: Departamento Del Cardiología Pediátrica, University Pontificia Bolivariana, Medellín, Colombia.
authors: Maria C
authors: MC
authors: Escobar-Diaz
authors: Division of Cardiology, Department of Pediatrics, University of Texas, Southwestern Medical Center, Austin, Texas.
authors: Catherine
authors: C
authors: Ikemba
authors: Department of Pediatric Cardiology, Harvard Medical School, Boston, Massachusetts.
authors: Brian T
authors: BT
authors: Kalish
authors: Department of Pediatric Cardiology, Harvard Medical School, Boston, Massachusetts.
authors: Rukmini
authors: R
authors: Komarlu
authors: Division of Cardiology, Department of Pediatrics, Columbia University Medical Center, New York, New York.
authors: Stéphanie M
authors: SM
authors: Levasseur
authors: Division of Cardiology, Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.
authors: Michael D
authors: MD
authors: Puchalski
authors: Division of Cardiology, Department of Pediatrics, University of California, Medical Center, Los Angeles, Los Angeles.
authors: Gary M
authors: GM
authors: Satou
authors: Division of Cardiology, Department of Pediatrics, Stanford University, Palo Alto, California.
authors: Norman H
authors: NH
authors: Silverman
authors: Division of Cardiology, Department of Pediatrics, University of California, San Francisco, San Francisco, California.
authors: Anita J
authors: AJ
authors: Moon-Grady
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D018450:Disease Progression; D004437:Ebstein Anomaly / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D004452:Echocardiography; D005260:Female; D005865:Gestational Age; D006801:Humans; D011247:Pregnancy; D011256:Pregnancy Outcome; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D016216:Ultrasonography, Prenatal