Decision-making for biventricular repair (BVR) or univentricular palliation is challenging in neonates with hypoplastic left heart complex (HLHC). Hybrid strategy can be used successfully to achieve BVR in some of these patients. Between June 1998 and January 2022, 342 patients with a diagnosis of HLHS/variants, ductal-dependent lower body circulation with two ventricles, or HLHC with borderline left ventricle (LV) underwent initial bilateral pulmonary artery banding and ductal stenting in our institution. Among these 342 patients, 224 patients were defined as HLHS/variants and underwent univentricular palliation. 118 patients were determined to have borderline LV and hypoplastic left ventricular structures (HLHC, n = 48) or ductal-dependent lower body circulation with two ventricles (n = 70) considered suitable for BVR. 48 patients had multilevel obstructions including both aortic and mitral valve hypoplasia, aortic arch hypoplasia, and borderline LV. These were considered to have HLHC. These 48 HLHC patients are the subject of this report. Neonatal hybrid palliation at a median age of seven days was performed in 48 patients and 46 patients underwent BVR at a median age of 156 days. In 46 patients who underwent BVR, hospital mortality was not observed. The median follow-up was 66 months, with no late mortality. Heart transplant-free survival at 5, 10, and 15 years was 95.7%. 12 patients (26%) required reoperation and 9 (19.5%) required catheter reinterventions. Hybrid palliation may increase the chances for biventricular circulation in patients with borderline LV and small left-sided structures. Giessen hybrid approach as a left ventricular recruitment strategy achieves excellent early and long-term results.
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http://dx.doi.org/10.1177/21501351221116274 | DOI Listing |
Pediatrics
January 2025
Cincinnati Children's Hospital Medical Center and Department of Pediatrics, College of Medicine, University of Cincinnati, Cincinnati, Ohio.
We present the case of a child born with hypoplastic left heart syndrome. After developing multiple complications following the first surgical stage (Norwood procedure), her parents decide not to proceed with the second stage (Glenn operation). Cardiac surgeons, pediatric intensivists, a psychologist, and a bioethicist analyze whether further surgical intervention is ethically obligatory.
View Article and Find Full Text PDFJ Med Ultrasound
February 2024
Department of Obstetrics and Gynecology, Faculty of Medicine, Karadeniz Technical University, Ortahisar, Trabzon, Turkey.
Background: The effect of congenital cardiac malformation on fetal cerebral circulation has not been well known. This study aimed to compare the cerebral blood circulation of fetuses with congenital heart disease (CHD) with healthy fetuses.
Methods: This prospective cohort study included 37 pregnant women who presented to the gynecology and obstetrics department of department of Farabi Hospital, Faculty of Medicine, Karadeniz Technical University for anomaly screening in the second trimester.
Ann Thorac Surg Short Rep
September 2024
Biostatistics Unit, Department of Data Science, National Center for Child Health and Development, Tokyo, Japan.
Background: The primary treatment for hypoplastic left heart syndrome (HLHS) is the Fontan pathway, which entails performing the Glenn procedure. We hypothesized that the superior vena cava in patients with HLHS was short. As the length of the superior vena cava influences the Glenn procedure, we compared its length between patients with HLHS and those with other congenital heart diseases.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Section of Pediatric Cardiac Surgery, Morgan Stanley Children's Hospital, New York, New York.
Among repairs for ventriculoarterial discordance, ventricular septal defect, and pulmonary stenosis, aortic root translocation (Nikaidoh operation) offers the most anatomic result. With a diminutive pulmonary annulus or hypoplastic left ventricular outflow tract, the distance gained posteriorly with aortic translocation is negligible. We developed the "hemi-Nikaidoh" procedure as an alternative.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan.
Undetected coronary anomalies at Norwood procedure are associated with poor prognosis due to inadequate myocardial protection. We report a case of anomalous origin of the right coronary artery from the main pulmonary artery trunk with hypoplastic left heart syndrome and aortic atresia. Although, during bilateral pulmonary artery banding as initial palliation, the proximity between the right coronary artery origin and the aortic root made a visual diagnosis difficult, it was diagnosed using computed tomography before the Norwood procedure.
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