Related Experiment Video

Updated: Jun 19, 2026

Implantation of Left Ventricular Assist Device (LVAD) in Juvenile Landrace Swine: A LVAD Implantation Model of Pediatric Heart Failure
05:18

Implantation of Left Ventricular Assist Device (LVAD) in Juvenile Landrace Swine: A LVAD Implantation Model of Pediatric Heart Failure

Published on: January 16, 2026

Left ventricular aneurysm in a child with severe combined immunodeficiency syndrome

Ramin Jamshidi1, Lisa K Hornberger, Tom R Karl

  • 1Department of Surgery, UCSF Medical Center, San Francisco, California, USA.

The Annals of Thoracic Surgery
|October 27, 2009
PubMed

Insights

A young girl with combined immunodeficiency syndrome underwent successful surgical repair for a left ventricular aneurysm. The procedure restored normal heart function and shape after bone marrow transplant engraftment.

Area of Science:

  • Pediatric Cardiology
  • Immunology
  • Cardiovascular Surgery

Background:

  • Combined immunodeficiency syndrome (CIS) can present with complex multisystemic manifestations.
  • Left ventricular aneurysms are rare but serious cardiac complications, potentially leading to heart failure and thromboembolic events.

Observation:

  • A 23-month-old girl with CIS developed a significant left ventricular aneurysm with an intramural thrombus.
  • The patient's condition necessitated surgical intervention due to the aneurysm's size and thrombotic burden.

Findings:

  • Surgical repair of the left ventricular aneurysm was successfully performed using endoventricular circular patch plasty (Dor procedure).
  • The intervention was carried out post-bone marrow transplant engraftment, ensuring a stable immunological state.
  • Postoperative assessment revealed excellent outcomes, with normalization of ventricular geometry and cardiac function.

Implications:

  • This case highlights the feasibility and effectiveness of surgical correction for left ventricular aneurysms in pediatric patients with complex congenital conditions.
  • The successful Dor procedure underscores the importance of a multidisciplinary approach in managing rare cardiovascular complications in immunocompromised children.
  • Early detection and timely intervention can significantly improve long-term prognosis for pediatric patients with combined immunodeficiency syndrome and cardiac anomalies.

More Related Videos

Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery (ALCAPA)13:10

Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery (ALCAPA)

Published on: April 24, 2017

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

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...
Immunodeficiency Diseases01:25

Immunodeficiency Diseases

Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency disorders...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
Aneurysm I: Introduction01:30

Aneurysm I: Introduction

An aortic aneurysm is a localized outpouching or dilation at a weak point in the artery wall. It may involve different parts of the aorta, such as the abdominal aorta, aortic arch, or thoracic aorta.Etiological factorsSeveral disorders are associated with aortic aneurysms.Congenital causes, such as primary connective tissue disorders like Marfan syndrome, impact the integrity and strength of connective tissues, notably affecting the aorta. Marfan syndrome is a genetic disorder that specifically...
Aortic Regurgitation III: Medical Management01:25

Aortic Regurgitation III: Medical Management

Aortic regurgitation (AR) is when the aortic valve does not close or seal properly, leading to backward blood circulation from the aorta into the left ventricle during diastole. Common causes of AR include rheumatic heart disease, congenital valve defects, and aortic root dilation. Managing AR requires a multifaceted approach to alleviate symptoms, preserve left ventricular function, and address the underlying cause of the regurgitation. Patients with symptomatic AR or significant left...
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...