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Objective: The objective of this study was to investigate the association between morphological variation and postsurgical pulmonary vein (PV) stenosis (PPVS) in patients with cardiac total anomalous pulmonary venous connection (TAPVC).
Methods: This single-center, retrospective study included 168 pediatric patients who underwent surgical repair of cardiac TAPVC from 2013 to 2019 (connection to the coronary sinus [CS], n = 136; connection directly to the right atrium [RA], n = 32). Three-dimensional computed tomography modeling and geometric analysis were performed to investigate the morphological features; their relevance to the PPVS was examined.
Results: The connection type had no association with PPVS (CS type: 18% vs right atrial type: 19%; P = .89) but there was a higher incidence of PPVS in patients with a single PV orifice than > 1 orifice (P < .001). Confluence-to-total PV area ratio (hazard ratio, 4.78, 95% CI, 1.86-12.32; P = .001) and length of drainage route (hazard ratio, 1.22; 95% CI, 1.14-1.31; P < .001) had a 4- and 1-fold increase in the risk for PPVS in the CS type after adjustment for age and preoperative pulmonary venous obstruction. In the right atrial type, those with anomalous PV return to the RA roof were more likely to develop PPVS than to the posterior wall of the RA (P < .001).
Conclusions: The number of inter-junction PV orifice correlated with PPVS development in cardiac TAPVC. The confluence-to-total PV ratio, length of drainage route, and anomalous PV return to the RA roof are important predictors for PPVS. Morphological subcategorization in this clinical setting can potentially assist in surgical decision-making.
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http://dx.doi.org/10.1016/j.jtcvs.2022.03.036 | DOI Listing |
JAMA Cardiol
September 2025
Department of Cardiology, Inselspital University Hospital of Bern, University of Bern, Bern, Switzerland.
Importance: Right anomalous aortic origin of a coronary artery (R-AAOCA) is a rare congenital condition increasingly diagnosed with the growing use of cardiac imaging. Due to dynamic compression of the anomalous vessel, invasive fractional flow reserve (FFR) during a dobutamine-atropine volume challenge (FFR-dobutamine) is considered the reference standard. A reliable alternative method is needed to reduce extensive invasive testing, but it remains uncertain whether noninvasive imaging can accurately assess the hemodynamic relevance of R-AAOCA.
View Article and Find Full Text PDFEClinicalMedicine
October 2025
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, China.
Background: Paediatric patients who underwent surgery for mitral regurgitation (MR) have a high risk of recurrence or death; however, no prediction tool has been developed to risk-stratify this challenging subpopulation.
Methods: In this multicentre cohort study, paediatric patients undergoing surgery for congenital MR in Shanghai Children's Medical Center in January 1st, 2009-December 31st, 2022 were included for analysis while those had a combination with infective endocarditis, anomalous left coronary artery from the pulmonary artery, rheumatic valvular disease, connective tissue disease, or single ventricle were excluded. A Cox regression model predictive of the primary outcome (a composite of mortality or mitral valve [MV] re-operation) was derived and converted to a point-based risk score.
Retina
September 2025
Ulucanlar Eye Training and Research Hospital, Retina Clinic of Ophthalmology Department, Ankara, Turkey.
Purpose: To compare the clinical features, multimodal imaging characteristics, and treatment outcomes of primary and secondary large retinal capillary aneurysms (LRCA).
Methods: A total of 34 eyes were included: seven with primary LRCA and 27 with secondary LRCA. All patients underwent fundus photography, optical coherence tomography (OCT), and fundus fluorescein angiography.
JTCVS Open
August 2025
Division of Cardiothoracic Surgery, Department of Surgery, University of Southern California, Los Angeles, Calif.
Objective: Patients with heterotaxy-associated congenital heart disease often require multiple operations, which may have a cumulative effect on their outcomes. This study aimed to define the cardiac surgical course in a large cohort and identify longitudinal risk factors for death/transplant.
Methods: All patients with heterotaxy-associated congenital heart disease who underwent cardiac surgery at one institution from 2005 to 2022 were retrospectively reviewed.
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.
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