Fetal Hypoplastic Left Heart Syndrome with Intact Atrial Septum: From Successful in utero Stenting to Subtotal Stent Occlusion - A Case Report.

Introduction: Fetuses with hypoplastic left heart syndrome (HLHS) and intact atrial septum (IAS) have an overall poor prognosis and a high risk of neonatal death due to severe secondary lung damage. Intrauterine stenting of the atrial septum was introduced in these patients to enable survival.

Case Presentation: We present a case of a HLHS fetus with IAS and signs of incipient nutmeg lung, in whom at 30 weeks gestation an atrial stent was successfully placed but continuously developed subtotal stent-obstruction over the next weeks.

The anatomic repair of recurrent aortic arch obstruction in children and adolescents.

Objective: Surgery for recurrent aortic arch obstruction is highly challenging and publications are rare. The aim of this retrospective, single-center study was to evaluate mortality, complications, and reintervention rate after an anatomic repair.

Methods: Between 1999 and 2022, in total 946 operations on the aortic arch were performed at the Children's Heart Center Linz.

Myocardial biomechanical effects of fetal aortic valvuloplasty.

Fetal critical aortic stenosis with evolving hypoplastic left heart syndrome (CAS-eHLHS) can progress to a univentricular (UV) birth malformation. Catheter-based fetal aortic valvuloplasty (FAV) can resolve stenosis and reduce the likelihood of malformation progression. However, we have limited understanding of the biomechanical impact of FAV and subsequent LV responses.

Outcome after extracorporeal membrane oxygenation therapy in Norwood patients before the bidirectional Glenn operation.

Objectives: Patients after the Norwood procedure are prone to postoperative instability. Extracorporeal membrane oxygenation (ECMO) can help to overcome short-term organ failure. This retrospective single-centre study examines ECMO weaning, hospital discharge and long-term survival after ECMO therapy between Norwood and bidirectional Glenn palliation as well as risk factors for mortality.

Pre-intervention myocardial stress is a good predictor of aortic valvoluplasty outcome for fetal critical aortic stenosis and evolving HLHS.

Fetal critical aortic stenosis with evolving hypoplastic left heart syndrome (CAS-eHLHS) causes biomechanical and functional aberrations, leading to a high risk of progression to hypoplastic left heart syndrome (HLHS) at birth. Fetal aortic valvuloplasty (FAV) can resolve outflow obstruction and may reduce progression risk. However, it is currently difficult to accurately predict which patients will respond to the intervention and become functionally biventricular (BV) at birth, as opposed to becoming functionally univentricular (UV).