Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.
Freud LR, McElhinney DB, Kalish BT, Escobar-Diaz MC, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Selamet Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, Tworetzky W.
J Am Heart Assoc. 2020 Nov 3;9(21):e016684. doi: 10.1161/JAHA.120.016684. Epub 2020 Oct 20. PMID: 33076749 Free article.
Take Home Points:
- In this large multicenter study, the neonatal mortality in Ebstein anomaly and tricuspid valve dysplasia patients is high and risk factors include earlier gestational age at birth and lower birth weight.
- Echocardiographic risk factors for neonatal mortality include tricuspid valve jet velocity and a lack of antegrade flow across the pulmonary valve.
- Among those patients who underwent right ventricular exclusion, none died.
Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric/Fetal Cardiology Journal Watch: The authors present outcome and management data from a large retrospective multicenter cohort study in fetuses with Ebstein anomaly (EA) or tricuspid valve dysplasia (TVD). From 2005 to 2011 243 fetuses with EA/TVD were included. Pre- and postnatal clinical as well as echocardiographic data were assessed in live-borns.
Out of the 176 live-borns, eleven patients died within 24 hours, 7 received comfort care and 4 were excluded due to incomplete data. In the group of the remaining 154 patients, 38 patients died before hospital discharge. Figure 2 shows the management and outcome at the time of hospital discharge.
The authors could show that nonsurvivors were born at an earlier gestational age and had a lower birth weight compared to survivors (please see Figure 1). In addition, they received more intense medical management. 18 patients were treated with extracorporeal membrane oxygenation and among them only 3 survived.
The surgical procedures performed together with the neonatal survival, are shown in Figure 3. Patients who had more than 1 surgery in the neonatal period were more likely to die. Interestingly, all 15 patients who underwent right ventricular exclusion survived.
Multilogistic regression analysis of echocardiographic data revealed that a lower tricuspid jet velocity and a lack of antegrade flow across the pulmonary valve was associated with neonatal mortality. In a multivariable analysis with echocardiographic indexes, gestational age at birth or birth weight, lower birth weight, and lower TR jet velocity were significantly associated with neonatal mortality.
Overall, this study describes in detail the medical, surgical and interventional management and the outcome of this large cohort of EA and TVD patients. The presented data are helpful for clinical practice and might support to guide therapy in this complex and heterogenous group of patients with EA/TVD.