Cardiac-type total anomalous pulmonary venous return is not benign

Area of Science:

• Cardiology
• Pediatric Surgery
• Medical Imaging

Background:

• Total anomalous pulmonary venous connection (TAPVC) is a complex congenital heart defect requiring surgical repair.
• Postsurgical pulmonary vein stenosis (PPVS) is a significant complication following TAPVC repair, impacting patient outcomes.
• Understanding the morphological factors associated with PPVS is crucial for improving surgical strategies and patient management.

Purpose of the Study:

• To investigate the association between morphological variations and the development of postsurgical pulmonary vein stenosis (PPVS) in pediatric patients with cardiac total anomalous pulmonary venous connection (TAPVC).
• To identify specific anatomical features that predict the risk of PPVS after TAPVC surgical repair.

Main Methods:

• Retrospective analysis of 168 pediatric patients who underwent surgical repair for cardiac TAPVC.
• Utilized three-dimensional computed tomography (3D CT) modeling and geometric analysis to assess morphological features.
• Examined the correlation between identified morphological characteristics and the incidence of PPVS.

Main Results:

• A single pulmonary vein orifice was significantly associated with a higher incidence of PPVS compared to multiple orifices (P < .001).
• The confluence-to-total PV area ratio (HR, 4.78; P = .001) and length of drainage route (HR, 1.22; P < .001) were independent predictors of PPVS in the coronary sinus type.
• Anomalous pulmonary vein return to the right atrial roof was a significant predictor of PPVS in the right atrial type (P < .001).

Conclusions:

• The number of pulmonary vein orifices and specific anatomical configurations are critical factors in the development of PPVS after TAPVC repair.
• Morphological features, including the confluence-to-total PV ratio, drainage route length, and location of anomalous venous return, are important predictors of PPVS.
• Detailed morphological assessment and subcategorization can aid in surgical decision-making and risk stratification for PPVS in TAPVC patients.