Discussion

The Starnes procedure, reported in 1991, ^{Reference Starnes, Pitlick, Bernstein, Griffin, Choy and Shumway2} is a useful palliative procedure in the single ventricle strategy for Ebstein’s anomaly, but it is known to have a high operative mortality rate. Because the lungs are hypoplastic due to marked cardiac dilatation, an early neonatal systemic-to-pulmonary shunt may fail due to neonatal pulmonary hypertension or worsen cardiac function due to diastolic coronary blood flow steal. Additionally, compression of the dilated right ventricle restricts left ventricular function, making maintaining circulation difficult. ^{Reference Lang, Oberhoffer and Cook3}

We hypothesised that the three components of the Starnes procedure, including right ventricle exclusion, atrial septal defect creation, and systemic-to-pulmonary shunt, could be organised and reconstructed in a consecutive two-stage strategy to control postoperative circulatory fluctuations (Figure 2a–c). The strategy of performing systemic-to-pulmonary shunt initially, followed by right ventricle exclusion, may avoid the use of cardiopulmonary bypass during the initial surgery. However, the former operation only changes the pulmonary blood flow source from the ductus to a shunt graft and does not improve haemodynamics; therefore, a second-stage operation is required immediately afterwards. In addition to that, early shunt surgery during the neonatal period may lead to diastolic hypotension, impaired coronary perfusion, and acute deterioration of myocardial function. Therefore, we prefer a standby approach to delay shunt placement until after the neonatal phase when pulmonary vascular resistance has stabilised.

Figure 2.

Haemodynamic schematic representations. ( a ) At birth, the patient exhibits a circular shunt. ( b ) After the initial surgery, the main pulmonary artery is ligated, and the tricuspid valve is closed using an autologous pericardial patch with fenestration. Pulmonary circulation is maintained via ductus-dependent circulation. ( c ) Following the second stage, pulmonary circulation is maintained via a shunt instead of the ductus. RA = right atrium; RV = right ventricle; PA = pulmonary artery; DA = ductus arteriosus; AO = aorta; LA = left atrium; LV = left ventricle.

Conversely, right ventricle exclusion and atrial septal defect creation can be expected to improve haemodynamics. Although open heart surgery with cardiopulmonary bypass in neonates remains challenging, it is performed to improve haemodynamics with acceptable risk. Therefore, we believe that right ventricle exclusion should be performed first to improve left ventricular function. Subsequently, a systemic-to-pulmonary shunt is performed around 1 month when pulmonary vascular resistance has stabilised. This approach requires precise monitoring of ductal formation and size, along with careful regulation of medication, inspired oxygen concentration, and ventilator settings to balance pulmonary circulation. Although this strategy is not universally applicable to all cases, we believe it provides a safer initial pathway for patients with extreme circulatory fragility.

We have experienced three other cases of neonatal Ebstein’s anomaly managed using a similar strategy in our institution, and all reached total cavopulmonary connection in satisfactory condition.