Severe Ebstein’s Anomaly of the Neonate with Pulmonary Atresia and Hypoplastic Pulmonary Arteries: A Staged Biventricular Approach Combining Starnes and Cone Procedure in a Preterm with Low Birth Weight

Background: Severe Ebstein’s anomaly (EA) of the neonate carries a high risk of mortality. The Starnes procedure can be applied to stabilize critically ill neonates with severe EA. However, it leads patients down the single ventricle pathway with all its well-known complications. Biventricular physiology can be preserved by means of Cone repair, but not all neonates tolerate tricuspid valve repair in the neonatal period. Here, we describe a staged approach combining both surgical techniques to achieve biventricular circulation.

Methods: Case Report: Echocardiography of a male preterm (36 weeks of gestation) with low birth weight (2,400 g) demonstrated severe EA classified with Carpentier type C, membranous pulmonary atresia, and hypoplastic pulmonary arteries (PA; right PA [RPA]: 3.2 mm; z = −1.7; left PA [LPA]: 2.7 mm; z = −2.3). At 10 days of age, the patient underwent Starnes procedure. Subsequently, numerous dilatations of the AP shunt and the Starnes fenestration were performed to induce right ventricle (RV) rehabilitation and growth of the PAs. At the age of 15 months, Cone repair along with implantation of a 12-mm conduit in pulmonary position was performed. Subsequently, surgical revision was required to address insufficiency of the tricuspid and pulmonary valve as well as bifurcation stenosis of the PAs. At the age of 33 months, severe RPA branch stenosis (3.5 mm; z = −5.1) along with severely impaired RV function (ejection fraction of 11%) and dilatation of the RV (end diastolic volume of 219 mL/m²) required implantation of a 6 × 12 stent. The LPA measured 4.9 mm (z = −2.2) without branch or peripheral pulmonary stenosis. Cardiac index was at 4.6 L/min/m² and central venous pressures dropped from 18 to 5 mm Hg. Within a couple of weeks after the procedure, RV function and dilatation improved significantly. At the last follow-up, the patient was 3 years of age and was doing well with no obvious signs of decreased exercise tolerance.

Conclusion: Conversion from Starnes to Cone may combine the advantages of Starnes in stabilizing critically ill newborns with the long-term superiority of biventricular physiology, representing a paradigm shift. It is technically feasible, even in cases of severe EA, prematurity, low birth weight, and additional cardiac comorbidities and provides promising initial results. However, more data are needed to define candidacy and timing of conversion to biventricular circulation and to determine long-term outcomes.

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Artikel online veröffentlicht:
11. Februar 2025