Related Experiment Video

Updated: May 30, 2026

Left Atrial Ligation in the Avian Embryo as a Model for Altered Hemodynamic Loading During Early Vascular Development
04:37

Left Atrial Ligation in the Avian Embryo as a Model for Altered Hemodynamic Loading During Early Vascular Development

Published on: June 16, 2023

Outcomes of hypoplastic left heart syndrome in low-birth-weight patients

Sarah Gelehrter1, Carlen G Fifer, Aimee Armstrong

  • 1Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan Health System, Ann Arbor, MI, USA. sgelehrt@umich.edu

Pediatric Cardiology
|July 26, 2011
PubMed

Insights

Outcomes for hypoplastic left heart syndrome (HLHS) neonates weighing ≤ 2.5 kg are poor, with only 36% surviving to Fontan completion. Key risks include being small for gestational age and noncardiac anomalies, particularly impacting early survival after Stage 1 palliation.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Surgery
  • Neonatal Intensive Care

Background:

  • Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect requiring complex surgical palliation.
  • Low-birth-weight neonates (≤ 2.5 kg) with HLHS present unique challenges in staged surgical management.
  • Outcomes for this high-risk population through complete palliation remain incompletely defined.

Purpose of the Study:

  • To evaluate the outcomes of neonates with HLHS weighing ≤ 2.5 kg undergoing staged palliation.
  • To identify risk factors associated with mortality throughout the palliation process, including the Fontan operation.
  • To assess survival rates at different stages of surgical palliation.

Main Methods:

  • Single-center retrospective review of HLHS patients weighing ≤ 2.5 kg.
  • Data abstraction included gestational age, birth weight, noncardiac anomalies, and survival to Fontan completion.
  • Statistical analysis to compare risk factors between survivors and nonsurvivors.

Main Results:

  • Fifty-two patients met inclusion criteria; 5 received comfort care. Of 47 initial surgical palliation patients, 51% survived to discharge.
  • Mortality was highest after Stage 1 palliation. Overall survival to Fontan was 36%.
  • Risk factors for early death included being small for gestational age (SGA), noncardiac anomalies, need for ECMO, and conversion to Sano shunt.

Conclusions:

  • Low-birth-weight HLHS neonates have significantly poor survival rates through staged palliation and the Fontan operation.
  • Being small for gestational age and the presence of noncardiac anomalies are critical preoperative risk factors for early mortality.
  • Interventions and management strategies should focus on mitigating risks associated with SGA and noncardiac anomalies in this vulnerable HLHS population.

More Related Videos

Fetal Mouse Cardiovascular Imaging Using a High-frequency Ultrasound (30/45MHZ) System07:34

Fetal Mouse Cardiovascular Imaging Using a High-frequency Ultrasound (30/45MHZ) System

Published on: May 5, 2018

Related Concept Videos

Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
Acute Respiratory Failure-II01:21

Acute Respiratory Failure-II

Type I Respiratory Failure, or hypoxemic respiratory failure, occurs when the partial pressure of oxygen (PaO2) in arterial blood falls below 60 mmHg while breathing room air without a corresponding increase in arterial carbon dioxide levels (PaCO2). This condition highlights a significant impairment in the lungs' capacity to oxygenate the blood.
The underlying physiological abnormalities that contribute to hypoxemic respiratory failure include:
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
Pathophysiology of Heart Failure01:17

Pathophysiology of Heart Failure

Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
Heart Failure III: Clinical Manifestations01:26

Heart Failure III: Clinical Manifestations

Heart failure (HF) manifests primarily as dyspnea, fatigue, and fluid retention, resulting in peripheral and pulmonary edema. Symptoms may vary depending on which ventricle is more affected, left or right.Left-Sided Heart FailureAlso known as left ventricular failure, this condition results from the left ventricle's inability to fill or eject sufficient blood into the systemic circulation. It leads to pulmonary congestion, which occurs when the left ventricle fails to eject blood effectively...