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

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.

Current surgical techniques in the management of the symptomatic neonate with severe Ebstein anomaly: Too much, too little, or just enough?

“… 34 For those neonates critically unstable with a circular shunt, immediate Starnes' palliation or (emergency) ligation of the main pulmonary artery, or ligating the ductus and placing a more peripheral aortopulmonary shunt, may be lifesaving. 35 Definitive palliation can be deferred for 7 to 10 days once the patient has stabilized. Our suggested algorithmic approach to symptomatic neonates is summarized in Table 1 and Figure 6 .…”

Simple, reproducible, and consistent physiology: The argument for single-ventricle repair in critically ill neonates with Ebstein anomaly

“…Approximately half (46%) of newborn infants diagnosed with Ebstein anomaly ultimately require some form of neonatal intervention. 2 We seek to offer an opinion regarding the best method to manage these children with an emphasis on single-ventricle palliation.…”

Commentary: The heart of symptomatic neonatal Ebstein anomaly: Negative interventricular interaction and ventricular myopathy

“…The number of newborn infants in each category is relatively equal. 8 It has been our experience that children who are able to transition and go home have a reasonable-sized true right ventricle (RV) and/or little to no tricuspid regurgitation. They do not usually develop symptoms or require surgical intervention within their first year of life.…”