Related Experiment Video

Updated: Nov 11, 2025

Author Spotlight: Establishment and Confirmation of a Postnatal Right Ventricular Volume Overload Mouse Model
06:04

Author Spotlight: Establishment and Confirmation of a Postnatal Right Ventricular Volume Overload Mouse Model

Published on: June 9, 2023

1.3K

Outcomes of single ventricle palliation in infants with heterotaxy syndrome

Janez Vodiskar1,2, Takashi Kido1,2, Martina Strbad1,2

  • 1Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technical University of Munich, School of Medicine, Munich, Germany.

European Journal of Cardio-Thoracic Surgery : Official Journal of the European Association for Cardio-Thoracic Surgery
|March 30, 2021
PubMed

Insights

Heterotaxy increases risks in single ventricle palliation. Early intervention for restrictive pulmonary blood flow and moderate atrioventricular valve regurgitation is crucial for improving survival in these complex patients.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Heterotaxy is associated with increased morbidity and mortality in patients undergoing single ventricle palliation.
  • Understanding outcomes in this specific patient population is critical for optimizing surgical strategies.

Purpose of the Study:

  • To evaluate the outcomes of staged single ventricle palliation in patients with heterotaxy.
  • To identify risk factors associated with mortality in this cohort.

Main Methods:

  • Retrospective analysis of patients with heterotaxy undergoing staged single ventricle palliation between 2001 and 2019.
  • Inclusion criteria: patients born within the study period requiring staged palliation for single ventricle physiology and heterotaxy.
  • Statistical analysis included multivariate COX regression to identify independent risk factors for mortality.

Main Results:

  • Fifty-three patients were included, with common findings including right ventricular dominance (66%) and atrioventricular septal defects (70%).
  • Overall survival was 92% at 1 month, 74% at 1 year, and 68% at 10 years.
  • Independent risk factors for mortality were restrictive pulmonary blood flow (HR 3.23) and atrioventricular valve regurgitation greater than mild (HR 3.57).

Conclusions:

  • Patients with single ventricle and heterotaxy face significant mortality and morbidity.
  • Restrictive pulmonary blood flow and significant atrioventricular valve regurgitation are key predictors of mortality.
  • Total anomalous pulmonary venous connection was not a significant risk factor in the current era.
Abstract

Keywords:
HeterotaxySingle ventricle palliation

More Related Videos

Implantation of Total Artificial Heart in Congenital Heart Disease07:27

Implantation of Total Artificial Heart in Congenital Heart Disease

Published on: July 18, 2014

24.9K
A Rodent Model of The Ross Operation: Syngeneic Pulmonary Artery Graft Implantation in A Systemic Position11:20

A Rodent Model of The Ross Operation: Syngeneic Pulmonary Artery Graft Implantation in A Systemic Position

Published on: April 1, 2022

3.3K

Related Concept Videos

Mitral Stenosis III: Medical Management01:26

Mitral Stenosis III: Medical Management

Mitral stenosis, a condition marked by the narrowing of the mitral valve, necessitates an integrated approach for effective management. This approach includes preventative measures, medical therapy, and surgical interventions to reduce symptoms and prevent complications.PreventionPrevention of mitral stenosis primarily focuses on reducing the incidence of bacterial infections, particularly streptococcal infections, which can lead to rheumatic fever and subsequent valvular damage. Timely...
93
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...
106
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...
125