Key result
Ex utero intrapartum treatment with transatrial stenting for left atrial decompression was performed in a fetus with HLHS, but the patient died from postoperative complications at 5 months of age.
Why the study?
Patients with hypoplastic left heart syndrome (HLHS) with intact atrial septum have an increased mortality rate.
Case Report (n=1)
Ex utero intrapartum treatment with transatrial stenting is a feasible approach for left atrial decompression in fetuses with HLHS and intact atrial septum, though overall mortality remains high.
May support EXIT stenting feasibility in HLHS with intact septum; leaves open questions on survival after staged palliation.
Patients with hypoplastic left heart syndrome (HLHS) with intact atrial septum have an increased mortality rate. This presentation occurs in 6% to 10% of cases. We present a patient with fetal diagnosis of HLHS with restrictive atrial septum. We performed a cesarean section at 37 weeks of gestation, and under ex utero intrapartum treatment proceeded with a median sternotomy and transatrial stenting for left atrial decompression due to findings of intact atrial septum on the fetal echocardiogram performed during the procedure. Subsequently, the patient underwent hybrid stage I palliation followed by a comprehensive stage II procedure at five months of age, but unfortunately died from postoperative complications.
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Escobar et al. (2023) conducted a case report in Hypoplastic left heart syndrome with restrictive atrial septum (n=1). Ex utero intrapartum treatment with median sternotomy and transatrial stenting was evaluated on Clinical outcome/survival. Ex utero intrapartum treatment with transatrial stenting for left atrial decompression was performed in a fetus with HLHS, but the patient died from postoperative complications at 5 months of age.
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