Related Experiment Video

Updated: Jan 18, 2026

Creation of Patient-Specific Silicone Cardiac Models with Applications in Pre-surgical Plans and Hands-on Training
09:15

Creation of Patient-Specific Silicone Cardiac Models with Applications in Pre-surgical Plans and Hands-on Training

Published on: February 10, 2022

4.1K

The complex and hazardous course for heterotaxy-associated congenital heart disease

Anna Olds1,2, James M Meza1,2, Nithya Rajeev1

  • 1Division of Cardiothoracic Surgery, Department of Surgery, University of Southern California, Los Angeles, Calif.

JTCVS Open
|September 9, 2025
PubMed

Insights

Heterotaxy-associated congenital heart disease (CH D) surgery carries high risks. Key predictors of death/transplant include coarctation, total anomalous pulmonary venous return repair, tricuspid atresia, and Fontan or atrioventricular valve reintervention.

Area of Science:

  • Cardiology
  • Pediatric Cardiac Surgery
  • Congenital Heart Disease

Background:

  • Heterotaxy-associated congenital heart disease (CH D) often necessitates complex, multi-stage surgical interventions.
  • Understanding longitudinal risk factors is crucial for improving outcomes in these high-risk patients.

Purpose of the Study:

  • To define the cardiac surgical course in a large cohort of patients with heterotaxy-associated CH D.
  • To identify longitudinal risk factors associated with death or the need for heart transplantation.

Main Methods:

  • Retrospective review of 217 patients with heterotaxy-associated CH D undergoing cardiac surgery (2005-2022).
  • Analysis of demographics, cardiac anatomy, and operative course.
  • Parametric hazard analysis, treating operations as time-varying covariates, to identify risk factors for death/transplant.

Main Results:

  • Transplant-free survival rates at 1, 5, and 10 years were 79.4%, 71.0%, and 63.9%, respectively.
  • Early phase risk factors included coarctation and total anomalous pulmonary venous return (TAPVR) repair with pulmonary blood flow control.
  • Later phase risk factors included tricuspid atresia, Fontan revision, and atrioventricular valve reintervention. Glenn procedure reduced risk.

Conclusions:

  • Surgical management of heterotaxy-associated CH D is associated with significant risk, particularly with TAPVR repair.
  • Post-infancy risk is largely dictated by the need for Fontan palliation or atrioventricular valve reoperation.
Abstract

Keywords:
congenital heart diseaseheterotaxypediatric cardiac surgery

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

25.1K
A Pipeline to Characterize Structural Heart Defects in the Fetal Mouse08:19

A Pipeline to Characterize Structural Heart Defects in the Fetal Mouse

Published on: December 16, 2022

2.4K

Related Concept Videos

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...
416
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
472
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...
485
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
480
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...
343
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
514