Background: In this study, we aimed to analyze the predictors and risk factors of mortality in patients who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome.
Methods: Between January 2009 and December 2020, a total of 139 patients (95 males, 44 females) who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome in our center were retrospectively analyzed.
Results: The median birth weight was 3,200 (range, 3,000 to 3,350) g and the median age at the time of operation was seven (range, 5 to 10) days. Pulmonary flow was achieved with a Sano shunt in the majority (72%) of patients. Survival rate was 41% after the first stage. Reoperation for bleeding (p=0.017), reoperation for residual lesion (p=0.011), and postoperative peak lactate level (p=0.029), were associated with in-hospital mortality. Nineteen (33%) of 57 patients died before the second stage. Thirty-three (58%) patients underwent second stage, and survival after the second stage was 94%. Thirteen patients underwent third stage, and survival after the third stage was 85%. Estimated probability of survival at six months, and one, two, three, and four years were 33%, 33%, 25%, 25%, and 22% respectively.
Conclusion: Hospital and inter-stage mortality rates are still high and this seems to be the most challenging period in term of survival efforts of the patients with hypoplastic left heart syndrome. Early recognition and reintervention of anatomical residual defects, close follow-up in the inter-stage period, and the accumulation of multidisciplinary experience may help to improve the results to acceptable limits.
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http://dx.doi.org/10.5606/tgkdc.dergisi.2022.22397 | DOI Listing |
Eur J Cardiothorac Surg
January 2025
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Munich Germany, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität München, Munich, Germany, Europäisches Kinderherzzentrum München, Munich, Germany.
Objectives: This study investigated the longitudinal changes in ventricular function and atrioventricular valve function to clarify the timing and the mechanisms of failing Fontan.
Methods: Patients who underwent total cavopulmonary connection between 1994 and 2023 were reviewed, and longitudinal echocardiographic examinations of ventricular function and atrioventricular valve regurgitation were analyzed.
Results: A total of 650 patients were included.
World J Pediatr Congenit Heart Surg
January 2025
The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Background: With an increasing number of Fontan patients surviving into adulthood, the burden of end-stage heart failure is increasing. Prior studies have reported suboptimal heart transplantation (HTx) outcomes. Therefore, the authors describe their institutional experience of HTx in patients with Fontan circulation failure, including heart-liver transplantation (HLTx) and pretransplant systemic ventricular assist device (SVAD) therapies.
View Article and Find Full Text PDFPediatrics
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.
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