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<p>Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect in which all pulmonary veins drain into systemic venous channels instead of the left atrium. We report a fatal case of obstructed supracardiac TAPVC in a term neonate who presented with early-onset cyanosis and refractory hypoxemia. Imaging suggested drainage via a narrow retroaortic vertical vein into the superior vena cava. Postmortem dissection revealed a fibrotic, atretic common pulmonary vein, a horizontal pulmonary venous confluence, and two distinct vertical veins entering the superior vena cava, a rare, unusual dual-channel variant. No pulmonary venous openings were identified in the left atrium, confirming total anomalous drainage. This case underscores the diagnostic and embryologic complexities of TAPVC and highlights the value of detailed cadaveric dissection in validating imaging findings, clarifying vascular anatomy, and deepening understanding of rare congenital variants.</p>

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Obstructed supracardiac TAPVC with atretic common pulmonary vein and dual vertical veins: Clinical and postmortem correlation in a term neonate

  • 期刊论文

  • 发表日期：

    2026年2月28日

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  • Sarthak Gupta,
  • Surajit Ghatak,
  • Arun Kumarendu Singh,
  • G Prabhakaran,
  • Rengarajan Rajagopal,
  • Shreya Gupta,
  • Choudri Muzafar Paswal,
  • Shilpi Gupta Dixit

摘要

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect in which all pulmonary veins drain into systemic venous channels instead of the left atrium. We report a fatal case of obstructed supracardiac TAPVC in a term neonate who presented with early-onset cyanosis and refractory hypoxemia. Imaging suggested drainage via a narrow retroaortic vertical vein into the superior vena cava. Postmortem dissection revealed a fibrotic, atretic common pulmonary vein, a horizontal pulmonary venous confluence, and two distinct vertical veins entering the superior vena cava, a rare, unusual dual-channel variant. No pulmonary venous openings were identified in the left atrium, confirming total anomalous drainage. This case underscores the diagnostic and embryologic complexities of TAPVC and highlights the value of detailed cadaveric dissection in validating imaging findings, clarifying vascular anatomy, and deepening understanding of rare congenital variants.