A Controversial Clinical Case of a Child With HypoplasticLeftHeartSyndrome. We present the case of a child born with hypoplasticleftheartsyndrome. 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
Evaluating Hospital Resource Utilization for HypoplasticLeftHeartSyndrome Through Linkage of a Clinical Registry and an Administrative Database. Resource utilization estimates for hypoplasticleftheartsyndrome (HLHS) in early childhood are lacking. We linked the National Pediatric Cardiology Quality Improvement (NPC-QIC) clinical registry to the Pediatric Health Information Systems (PHIS
Mid-Term Outcomes of Patients with HypoplasticLeftHeartSyndrome and Left Ventricle-Coronary Artery Fistula. Infants with hypoplasticleftheartsyndrome (HLHS) with mitral stenosis/aortic atresia (MS/AA) have worse outcomes compared to other anatomic variants; this may be related to left ventricle-coronary artery (LV-CA) fistula. We reviewed patients with HLHS (MS/AA) referred to Boston
Fetal Echocardiographic Evaluation of Tricuspid Valve and Right Ventricular Function Including Global Longitudinal Strain in HypoplasticLeftHeartSyndrome and Association with Postnatal Outcomes. Despite significant advancements in the care of patients with hypoplasticleftheartsyndrome (HLHS) morbidity and mortality remain high. Postnatal right ventricular dysfunction and tricuspid
Prognostic Value of RV Function Analysis During the Interstage Period in Patients with HypoplasticLeftHeartSyndrome. Cardiac dysfunction is associated with mortality in children with hypoplasticleftheartsyndrome (HLHS). We evaluated the ability of qualitative and quantitative RV functional parameters to predict outcomes in HLHS patients. In this retrospective, single-center study
Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in HypoplasticLeftHeartSyndrome. Controlling pulmonary blood flow in patients who have undergone Norwood palliation, especially early postoperatively, is challenging due to a change in the balance of systemic and pulmonary vascular resistance. We applied a combination therapy of clipping and balloon angioplasty for right ventricle-pulmonary artery (RV-PA) shunt to control pulmonary blood flow, but the influence of the combination therapy on the PA condition is uncertain. Retrospectively analysis was conducted of all infants with hypoplasticleftheartsyndrome who had undergone Norwood palliation with RV-PA shunt at Okayama University Hospital from January
Short and Mid-term Outcomes of Ductal Graft with Bilateral Pulmonary Banding in HypoplasticLeftHeartSyndrome and Variants. This study evaluates a novel surgical technique inspired by the hybrid Norwood procedure, which involves ductal graft placement and bilateral pulmonary artery banding. This technique is designed to avoid the high-risk classical Norwood stage 1 operation and to avoid the need for first stage invasive catheter interventions and interstage catheter interventions when ductal stent placement is not feasible in developing countries with limited resources. Between June 2015 and December 2022, 14 patients with hypoplasticleftheartsyndrome and variants were treated using this procedure at our center. Polytetrafluoroethylene graft was used for ductal graft procedure
Update of How Pediatric Cardiologists Noninvasively Evaluate Patients with HypoplasticLeftHeartSyndrome: 2013 vs. 2023. Noninvasive functional assessment of systemic right ventricles (RV) in hypoplasticleftheartsyndrome (HLHS) is challenging. This study aimed to compare pediatric cardiologists' current noninvasive imaging practices to those reported in 2013. A web-based survey
Reprint of: How He Did It - The James S. Tweddell Approach to Single Ventricle Palliation in Patients With HypoplasticLeftHeartSyndrome. This article will detail Dr. James Tweddell's operative technique and considerations for 26 all three stages of SVP in patients with HLHS. This will include the Norwood Procedure with 27 Right-Ventricle-to-Pulmonary-Artery Conduit (Sano Modification
Long-Term Enlargement of the Neo-Aortic Root and Aortic Arch Following Arch Reconstruction in HypoplasticLeftHeartSyndrome. Long-term enlargement of the aortic arch after aortic arch reconstruction (AAR) in hypoplasticleftheartsyndrome (HLHS) is not well described. Aortic arch measurements for 50 patients with HLHS who achieved Fontan completion were converted to Pediatric Heart Network z
Assessment of B-Natriuretic Peptide Levels After Stage 1 Palliation in HypoplasticLeftHeartSyndrome Patients. We describe the perioperative BNP trends in patients with HLHS from S1P until interstage discharge to home, death, or S2P. This prospective cohort study includes all newborns with hypoplasticleftheartsyndrome (HLHS) who underwent Norwood procedures (S1P) at Texas Children's Hospital
Response to "Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in HypoplasticLeftHeartSyndrome". The article "Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in HypoplasticLeftHeartSyndrome" offers valuable insights into Right Ventricular-Pulmonary Artery Shunt in HypoplasticLeftHeartSyndrome. This letter commends the study for its relevance and potential to improve patient care but highlights several limitations. Notably, the study overlooks the impact of Balloon angioplasty is a useful treatment for recoarctation in individuals with HLHS, but it frequently necessitates further operations. Following
HypoplasticLeftHeartSyndrome with Low Birth Weight or Prematurity: What is the Optimal Approach? Hypoplasticleftheartsyndrome with low birth weight or prematurity comprises a high-risk population with no optimal treatment pathway. Using the Pediatric Health Information System, we compared management approaches across the United States. We analyzed neonates (≤30 days) with birth weight
Associations of Home Monitoring Data to Interventional Catheterization for Infants with Recurrent Coarctation of the Aorta and HypoplasticLeftHeartSyndrome. The post-Norwood interstage period for infants with hypoplasticleftheartsyndrome is a high-risk time with 10-20% of infants having a complication of recurrent coarctation of the aorta (RCoA). Many interstage programs utilize mobile
Mitochondrial MICOS complex genes, implicated in hypoplasticleftheartsyndrome, maintain cardiac contractility and actomyosin integrity. Hypoplasticleftheartsyndrome (HLHS) is a severe congenital heart disease (CHD) with a likely oligogenic etiology, but our understanding of the genetic complexities and pathogenic mechanisms leading to HLHS is limited. We therefore performed whole genome
The Prevalence of Congenital Anomalies of the Airway or Lung in Infants with HypoplasticLeftHeartSyndrome and Differences in Midterm Outcomes: A National Pediatric Cardiology Quality Improvement Collaborative Registry Analysis. Infants with single ventricle physiology and congenital anomalies of the airway and/or lung are potentially poor candidates for staged palliation. The prevalence and midterm outcomes for patients with anomalies of the airway or lung with hypoplasticleftheartsyndrome are unknown. We performed an analysis of data in infants with hypoplasticleftheartsyndrome from the National Pediatric Cardiology Quality Improvement Collaborative registry. The prevalence of congenital anomalies of the airway or lung in the registry was determined. Clinical characteristics
Modeling of the Tricuspid Valve and Right Ventricle in HypoplasticLeftHeartSyndrome With a Fontan Circulation. In hypoplasticleftheartsyndrome, tricuspid regurgitation (TR) is associated with circulatory failure and death. We hypothesized that the tricuspid valve (TV) structure of patients with hypoplasticleftheartsyndrome with a Fontan circulation and moderate or greater TR differs from those with mild or less TR, and that right ventricle volume is associated with TV structure and dysfunction. TV of 100 patients with hypoplasticleftheartsyndrome and a Fontan circulation were modeled using transthoracic 3-dimensional echocardiograms and custom software in SlicerHeart. Associations of TV structure to TR grade and right ventricle function and volume were investigated. Shape
Prenatal diagnosis of recurrent hypoplasticleftheartsyndrome associated with MYH6 variants: a case report. Hypoplasticleftheartsyndrome (HLHS) is a rare but genetically complex and clinically and anatomically severe form of congenital heart disease (CHD). Here, we report on the use of rapid prenatal whole-exome sequencing for the prenatal diagnosis of a severe case of neonatal recurrent
Attributable mortality benefit of digoxin treatment in hypoplasticleftheartsyndrome after the Norwood operation: An instrumental variable-based analysis using data from the Pediatric Health Information Systems Database. Observational studies have demonstrated an association between use of digoxin and reduced interstage mortality after Norwood operation for hypoplasticleftheartsyndrome
Echocardiographic Strain to Predict Need for Transplant or Mortality in Fontan Patients with HypoplasticLeftHeartSyndrome. Despite recent advances, hypoplasticleftheartsyndrome (HLHS) patients subsequent to the Fontan still have significant morbidity and mortality. Some require heart transplant due to systemic ventricular dysfunction. Limited data exist on timing for transplant referral -free survival in patients with hypoplasticleftheartsyndrome having undergone Fontan palliation. Higher strain values (closer to zero) may be a helpful tool in determining when transplant evaluation is warranted in these patients.