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Background 

Hypoplastic left heart syndrome (HLHS) represents a constellation of defects affecting the left side of the heart, resulting in an inability to support systemic circulation. The standard management involves a three-stage palliation, starting with the Norwood procedure or one of its variants as a neonate, followed by bidirectional cavopulmonary anastomosis at around four to six months of age, and ends with the completion Fontan procedure two to three years later. 

In six percent of these patients, an intact or restrictive atrial septum is present, which is associated with increased mortality due to the lack of adequate atrial communication. Once diagnosed, careful monitoring is required, with the possibility of fetal or immediate postnatal interventions. 

The current management options include transcatheter intervention during pregnancy in the form of ultrasound-guided balloon septostomy or immediate post-delivery left atrial decompression using a transcatheter, surgical, or hybrid approach. The success of the transcatheter options is limited in cases of hypoplastic left heart syndrome with an intact atrial septum (HLHS/IAS), and leaving the interventions for the postnatal period creates an emergency scenario where the baby becomes hemodynamically unstable and must be rushed to the operating room or the hybrid laboratory. 

Therefore, the authors propose an innovative solution that allows the creation of adequate atrial communication under the most stable circumstances by combining the ex utero intrapartum treatment (EXIT) and open atrial septectomy techniques. 

Case 

A 29-year-old pregnant woman who had a fetal echocardiogram at 28 weeks gestation presented showing HLHS/IAS. All management options were discussed with the parents, and the decision was made to proceed with EXIT-to-open atrial septectomy at 38-weeks’ gestation. 

EXIT 

An elective cesarean section was performed, and the baby was partially delivered while maintaining the placenta-fetal circulation. This was followed by a transthoracic echocardiogram that confirmed the lack of atrial communication. 

Operation 

Median sternotomy was then performed, and a purse string suture was placed in the free wall of the right atrium. Using bicaval inflow occlusion, the heart was allowed to empty for one to two, beats followed by an incision in the right atrium within the purse string suture. The atrial septum was grasped and resected. The caval clamps were removed to restore circulation and allow deairing of the heart, followed by pulling the purse string suture to control bleeding. The total caval occlusion time was 90 seconds. An epicardial echocardiogram confirmed the presence of adequate atrial communication. The neonate was then intubated, and delivery was completed with clamping and division of the umbilical cord. The neonate was subsequently transferred to the second operating room where he was intubated, adequately resuscitated, and prostaglandin infusion was initiated, followed by temporary chest closure. Less than 24 hours later, modified Norwood/Sano stage I palliation was successfully completed. 

Postoperative Course 

There were no major events during the postoperative course, and the baby underwent chest closure two days later and was extubated on the fourth postoperative day. However, he required reintubation for fluid overload and was subsequently extubated. He was discharged two weeks after his Norwood procedure. 

Comment 

This innovative solution represents a valuable option for managing neonates with HLHS/IAS. Performing the atrial septectomy under the most stable conditions, which can only be present when the neonate remains connected to the placenta, was the most logical option. It avoids the creation of an unnecessary emergency situation after delivery. Several factors have contributed to the success of this case. These include learning from prior experiences, multidisciplinary teamwork, avoidance of cardiopulmonary bypass, in addition to the execution of a well-organized plan, and excellent time management. 

References

1. Said SM, Essa Y, Mashadi AH, Rajendran G, Silber A, Mittnacht A, Jambhekar BN, Kumaraswami S, Linder A, Glover K, Leong M, Pinto M, Singh AR, Chan KM, Mehta B, Jain S, Fethke E, Rosenzweig EB. Open Atrial Septectomy on Placental Support: A Novel and Logistic Approach for Hypoplastic Left Heart Syndrome with Intact Atrial Septum. Pediatr Cardiol. 2025 Jun 10. doi: 10.1007/s00246-025-03922-5. Epub ahead of print. PMID: 40495010.

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