Related Experiment Video

Updated: Jun 6, 2026

Pediatric Animal Model of Extracorporeal Cardiopulmonary Resuscitation After Prolonged Circulatory Arrest
04:55

Pediatric Animal Model of Extracorporeal Cardiopulmonary Resuscitation After Prolonged Circulatory Arrest

Published on: May 26, 2023

Children with genetic disorders undergoing open-heart surgery: are they at increased risk for postoperative

Carsten Doell1, Vera Bernet, Luciano Molinari

  • 1Pediatric Intensive Care and Neonatology, University Children's Hospital, Zurich, Switzerland.

Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
|November 9, 2010
PubMed

Insights

Infants with congenital heart disease and genetic disorders face similar mortality rates but increased risks for renal issues and reintubation. Trisomy 21 specifically elevates risks for chylothorax and sepsis post-surgery.

Area of Science:

  • Pediatric Cardiology
  • Clinical Genetics
  • Critical Care Medicine

Background:

  • Congenital heart disease (CHD) combined with genetic disorders may increase postoperative risks.
  • Understanding these risks is crucial for managing pediatric patients undergoing cardiac surgery.

Purpose of the Study:

  • To compare postcardiopulmonary bypass outcomes in infants with CHD and genetic disorders versus those with CHD alone.
  • Identify specific risks associated with genetic conditions in this population.

Main Methods:

  • Prospective cohort study involving 211 infants (<1 year) undergoing bypass surgery for CHD.
  • Comparison of perioperative courses between infants with and without genetic disorders.
  • Univariate and regression analyses to control for confounding factors.

Main Results:

  • No significant difference in mortality was observed between groups.
  • Genetic disorders independently predicted renal insufficiency and reintubation.
  • Trisomy 21 was linked to higher rates of chylothorax and sepsis.
  • Longer hospital stays were noted for infants with genetic disorders other than trisomy 21.

Conclusions:

  • Infants with CHD and genetic disorders do not have increased mortality risk post-surgery.
  • Genetic conditions are risk factors for renal insufficiency and reintubation.
  • Trisomy 21 presents unique risks for chylothorax and sepsis, requiring tailored management strategies.
Abstract

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

Related Concept Videos

Cardiomyopathy VII: Pre and Post Operative Nursing Management01:28

Cardiomyopathy VII: Pre and Post Operative Nursing Management

Patients with hypertrophic cardiomyopathy (HCM) and left ventricular outflow tract (LVOT) obstruction who remain symptomatic despite optimal medical therapy may undergo a septal myectomy (Morrow procedure). This procedure involves excising a portion of the hypertrophied septum below the aortic valve using a heart-lung machine to improve blood flow through the LVOT. Effective preoperative and postoperative nursing management ensures successful patient outcomes, minimizes complications, and...
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...
Cardiac Catheterization I: Pre-Procedure Overview01:28

Cardiac Catheterization I: Pre-Procedure Overview

Cardiac catheterization is an invasive diagnostic technique used to identify and evaluate structural and functional diseases of the heart and major blood vessels. This technique diagnoses congenital heart disease, coronary artery disease, valvular heart disease, and coronary spasms and assesses ventricular function. It helps guide treatment decisions, including the need for revascularization procedures like percutaneous coronary intervention (PCI) or coronary artery bypass grafting (CABG) and...
Cardiac Catheterization II: Right Heart Catheterization01:21

Cardiac Catheterization II: Right Heart Catheterization

Right Heart Catheterization: An OverviewRight heart catheterization is an invasive diagnostic procedure that measures right-sided cardiac and pulmonary artery pressures, calculates cardiac output, and identifies intracardiac shunts. It provides detailed hemodynamic data essential for diagnosing and managing various cardiovascular conditions, such as pulmonary hypertension.Access SitesCommon access sites for right heart catheterization include the internal jugular vein in the neck region, the...
Aneurysm IV: Nursing Management01:22

Aneurysm IV: Nursing Management

Vigilant monitoring for aneurysm rupture is essential for patients undergoing aortic surgery.Preoperative Nursing ManagementContinuously monitor the patient for manifestations of aneurysm rupture, such as pallor, weakness, tachycardia, hypotension, abdominal, back, groin, or periumbilical pain, changes in consciousness, and a pulsating abdominal mass. Regularly assess the patient's peripheral pulses.Instruct the patient to consume a clear liquid diet the day before surgery and administer...
Peripheral Artery Disease V: Postoperative Nursing Management01:23

Peripheral Artery Disease V: Postoperative Nursing Management

During the postoperative period, it is crucial to focus on maintaining circulation, identifying and managing potential complications, and planning for discharge.Nursing AssessmentVital signs monitoring: Regularly monitor vital signs, including blood pressure, heart rate, respiratory rate, and temperature, to detect early signs of complications such as bleeding and infection.Circulation assessment: Monitor pulses, perform Doppler assessments, and check capillary refill, color, temperature, and...