Open Atrial Septectomy on Placental Support: A Novel and Logistic Approach for Hypoplastic Left Heart Syndrome with
Sameh M Said1, Yasin Essa2, Ali H Mashadi2
1Division of Pediatric and Adult Congenital Cardiac Surgery, Maria Fareri Children's Hospital and Westchester Medical Center, Department of Surgery, New York Medical College, 100 Woods Road, Valhalla, NY, 10595, USA. Sameh.Said@wmchealth.org.
Insights
Hypoplastic left heart syndrome with an intact atrial septum presents management challenges. A novel Ex utero, Intrapartum Treatment (EXIT)-to-open atrial septectomy approach offers a promising solution for these complex congenital heart cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Fetal Surgery
Background:
- Hypoplastic left heart syndrome (HLHS) with an intact inter-atrial septum is a rare but severe condition.
- High mortality rates are associated with HLHS and intact atrial septum due to limited left atrial outflow.
- Current management strategies are varied and lack standardization, highlighting the need for innovative approaches.
Purpose of the Study:
- To report the first successful application of a novel Ex utero, Intrapartum Treatment (EXIT)-to-open atrial septectomy followed by rapid Stage I Norwood palliation.
- To evaluate the feasibility and potential benefits of this combined approach in neonates with HLHS and intact atrial septum.
Main Methods:
- An open atrial septectomy was performed at 38 weeks gestation under placental support via EXIT procedure before completion of delivery.
- The neonate underwent Stage I Norwood palliation 24 hours after the septectomy.
- Cardiopulmonary bypass was avoided during the atrial septectomy procedure.
Main Results:
- Successful creation of an unrestrictive atrial communication (open atrial septectomy) was achieved.
- The neonate tolerated subsequent Stage I Norwood palliation without immediate complications.
- This rapid sequence palliation was completed successfully, demonstrating the viability of the approach.
Conclusions:
- Ex utero, Intrapartum Treatment (EXIT)-to-open atrial septectomy under placental support is a feasible and logistically sound strategy for HLHS with intact atrial septum.
- This approach provides a controlled environment for critical left atrial decompression.
- Swiftly followed by Stage I palliation, this rapid sequence intervention holds potential for improved outcomes in this challenging HLHS subgroup.
Abstract:
Hypoplastic left heart syndrome with intact inter-atrial septum continues to be a challenge with high mortality, despite representing only 6% of those with hypoplastic left heart syndrome. No standard management exists for these patients, and centers vary in their approach depending on expertise and available resources. Interventions range from fetal transcatheter balloon atrial septostomy with or without stent placement-to-postnatal transcatheter, surgical, and/or hybrid strategies. In the current report, we present the first successful Ex utero, Intrapartum Treatment (EXIT)-to-open atrial septectomy-to-rapid stage I Norwood palliation in a neonate with hypoplastic left heart syndrome and intact inter-atrial septum. Creation of unrestrictive atrial communication (open atrial septectomy) was performed at 38 weeks gestation with partial delivery of the fetus who was kept under placental support till the septectomy was completed and without the use of cardiopulmonary bypass. 24-h later, stage I Norwood palliation was completed successfully. In conclusion, Open atrial septectomy under placental support (EXIT) is a logistic approach for hypoplastic left heart syndrome with intact atrial septum. It provides a controlled environment for left atrial decompression and can be followed swiftly with stage I palliation. This rapid sequence first stage palliation may have the potential for producing better results in this challenging subgroup of patients.
