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Related Experiment Video

Updated: Apr 6, 2026

Computed Tomography (CT) Guided Implantation of a Totally Implantable Venous Access Port (TIVAP) through Subclavian Vein
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[Total Anomalous Pulmonary Venous Connection].

Yukihiro Kaneko1

  • 1Division of Cardiovascular Surgery, National Medical Center for Children and Mothers, Tokyo, Japan.

Kyobu Geka. the Japanese Journal of Thoracic Surgery
|July 23, 2015
PubMed
Summary

Total anomalous pulmonary venous connection (TAPVC) is a common congenital heart defect. Early surgical intervention is crucial, but prenatal diagnosis is often missed, impacting infant outcomes.

Area of Science:

  • Congenital Heart Disease
  • Pediatric Cardiology
  • Neonatal Surgery

Context:

  • Total anomalous pulmonary venous connection (TAPVC) is a frequent congenital heart defect necessitating urgent neonatal surgery.
  • Pulmonary venous obstruction in TAPVC can rapidly lead to severe cyanosis, respiratory failure, and shock, requiring immediate intervention.
  • Prenatal diagnosis of TAPVC aids in seamless transition from delivery to surgical management, yet isolated forms are often missed on prenatal ultrasound.

Purpose:

  • To review the diagnostic challenges and management strategies for total anomalous pulmonary venous connection (TAPVC) in neonates.
  • To outline surgical and interventional approaches based on clinical presentation and patient factors.
  • To discuss prognostic indicators for neonates with TAPVC, particularly those with associated anomalies.

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Summary:

  • Surgical repair via common pulmonary venous chamber-to-left atrial anastomosis is the primary intervention for isolated obstructive TAPVC in neonates.
  • Exceptions to immediate surgery include severe intraventricular hemorrhage, extremely low birth weight, or gestational age ≤30 weeks.
  • For neonates with right isomerism, functional single ventricle, and severe TAPVC, catheter intervention may be preferred to delay open-heart surgery due to poor prognosis.

Impact:

  • Improved understanding of TAPVC management strategies can optimize surgical timing and approach.
  • Highlighting limitations in prenatal diagnosis emphasizes the need for heightened vigilance in neonatal screening.
  • Identifying high-risk neonates guides therapeutic decisions, potentially improving outcomes in complex cases.