Outcomes in Late Presenting Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection-Single Centre
Kartik Patel1, Deepti Kakkar1, Himani Pandya2
1Department of Cardio Vascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Center (affiliated to BJ Medical College, Ahmedabad), Ahmedabad, Gujarat, India.
World Journal for Pediatric & Congenital Heart Surgery
|January 27, 2025
Summary
Survival beyond one month is rare for infants with obstructed infracardiac total anomalous pulmonary venous connection (TAPVC). This study identified key risk factors for surgical outcomes in this rare pediatric cardiac condition.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Obstructed infracardiac total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect.
- Infants with this condition rarely survive beyond one month of age.
- Limited data exist on surgical outcomes for this specific patient subset.
Purpose of the Study:
- To identify risk factors associated with surgical outcomes in infants with obstructed infracardiac TAPVC surviving beyond one month.
- To evaluate the efficacy of surgical intervention in this rare pediatric cardiac population.
Main Methods:
- Retrospective study of 14 patients with obstructed infracardiac TAPVC surviving beyond one month.
- Data included demographics, preoperative labs, echocardiograms, and CT scans.
- Intraoperative, postoperative, and follow-up clinical and echocardiographic data were collected.
Main Results:
- Low weight, elevated preoperative bilirubin, use of deep hypothermic circulatory arrest, tricuspid regurgitation, and pulmonary artery hypertension were significant mortality risk factors.
- The mean follow-up was over 6 years, with most patients exhibiting excellent clinical and echocardiographic outcomes.
- Only one patient had mild, asymptomatic flow acceleration across the anastomosis.
Conclusions:
- Infants with obstructed infracardiac TAPVC surviving beyond one month represent a rare but treatable cohort.
- Timely surgical intervention can lead to acceptable early and excellent long-term outcomes.
- Identifying and managing risk factors is crucial for improving surgical results in this population.
Related Concept Videos
01:21Cardiac 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...
01:22Aortic Regurgitation II: Clinical Features and Diagnostic Tests
Aortic valve regurgitation (AR) occurs when the aortic valve fails to close properly, allowing blood to flow backward from the aorta into the left ventricle. This backflow can result in two distinct clinical presentations: acute and chronic AR, each characterized by its own set of symptoms and physical findings.Acute Aortic RegurgitationAcute AR presents with a sudden onset of severe symptoms. Patients typically experience profound dyspnea (shortness of breath), chest pain, and signs of left...
01:30Coronary Artery Disease III: Clinical Manifestations
Coronary Artery Disease (CAD) is a primary health risk worldwide, leading to significant morbidity and mortality. The condition arises from the buildup of atherosclerotic plaques within the coronary arteries, resulting in diminished blood supply to the heart muscle.The clinical manifestations of CAD vary widely, from asymptomatic stages to severe, life-threatening conditions. Understanding these manifestations is crucial for early diagnosis and effective management.Angina Pectoris: The Warning...
01:19Acute Coronary Syndrome II: Pathophysiology and Clinical Manifestations
The pathophysiology of Acute Coronary Syndrome [ACD] involves several key processes:The main underlying cause of ACD is atherosclerosis, a chronic inflammatory disease characterized by the buildup of lipid-laden plaques within the coronary arteries.As the atherosclerotic plaque grows in the coronary artery, it may become unstable due to the formation of a lipid-rich core and a thin fibrous cap. Inflammatory cells within the plaque, such as macrophages, secrete enzymes that degrade the...
01:26Heart 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...
01:29Cardiomyopathy 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...