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Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect where the pulmonary veins abnormally connect to the systemic venous circulation, rather than the left atrium (LA). This study retrospectively analyzed 48 children diagnosed with TAPVC from January 2014 to January 2024 to investigate how specific echocardiographic and morphological characteristics impact prognosis. Perioperative records, including echocardiography, computed tomography angiography (CTA) images, surgical details, and patient outcomes, were reviewed. Data were statistically analyzed using tools such as t-tests, Mann-Whitney U tests, χ² tests, and logistic regression. Key factors examined included age at surgery, anatomical subtype of TAPVC, presence of preoperative obstruction, surgical techniques used, and postoperative complications. By identifying the impact of these variables on survival rates and hospital recovery, the study aims to improve risk stratification and management strategies for better outcomes in children with TAPVC.
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http://dx.doi.org/10.1038/s41598-025-94619-5 | DOI Listing |
J Am Soc Echocardiogr
September 2025
Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA.
Introduction: Repaired total anomalous pulmonary venous connection (TAPVC) patients with preoperative pulmonary venous obstruction (PVO) have reductions in echocardiographic metrics, such as left atrial reservoir function and pulmonary venous variability index (PVVI). We hypothesized reduced preoperative left atrial strain mechanics in isolated TAPVC patients serve as risk factors for postoperative PVO. We also evaluated echocardiographic metrics and clinical characteristics associated with preoperative and postoperative PVO, as well as compared these to healthy controls.
View Article and Find Full Text PDFKaohsiung J Med Sci
September 2025
Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.
Anomalous pulmonary venous connection (APVC), including total (TAPVC) and partial (PAPVC) forms, is a congenital heart defect with abnormal pulmonary vein drainage; and while surgical repair has improved survival, its long-term impact on cardiopulmonary function remains unclear. This retrospective study evaluated exercise capacity and pulmonary function in 26 pediatric APVC patients (17 TAPVC, 9 PAPVC) using cardiopulmonary exercise testing (CPET) and compared them with 63 age-matched healthy controls. Patients with complex defects or significant comorbidities were excluded.
View Article and Find Full Text PDFJ Assoc Physicians India
July 2025
Professor, Department of Cardiology, Government Medical College and Hospital, Chandigarh, India.
Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease with abnormal drainage of pulmonary veins (PVs) into a systemic vein or right atrium (RA). It is divided into four types on the basis of anatomical pattern of drainage: supracardiac, cardiac, infracardiac, and mixed type. The mixed variant is further divided into the "3 + 1" and "2 + 2" patterns.
View Article and Find Full Text PDFSci Rep
August 2025
Mary Elizabeth's Hospital, Copenhagen University Hospital Rigshospitalet, Edel Sauntes Allé 8, Copenhagen, DK-2100, Denmark.
Total anomalous pulmonary venous connection (TAPVC) is a rare and life-threatening condition, and long-term patient outcomes remain to be fully explored. This registry-based national cohort study including all Danish children born with TAPVC between 2000 and 2018 aimed to evaluate the survival rates of Danish children receiving a TAPVC repair procedure. Additionally, the prevalence of early morbidity, severe chronic diseases, and mental and/or behavioral disorders in children diagnosed with TAPVC was investigated using Danish health registries.
View Article and Find Full Text PDFIndian J Thorac Cardiovasc Surg
August 2025
Department of Radiodiagnosis, PGIMER, Chandigarh, -160012 India.
Total anomalous pulmonary venous drainage (TAPVC) is a rare congenital anomaly with drainage of all the pulmonary veins into the systemic circulation instead of the left atrium. The commonest anomalous pattern of supracardiac TAPVC includes formation of the left-sided vertical vein draining into the left brachiocephalic vein. All the pulmonary veins forming a right-sided vertical vein and draining into proximal superior vena cava (SVC) are a highly uncommon variant, with distinct anatomical course.
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