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Tetralogy of Fallot with pulmonary stenosis

Last edited: 4/10/2026

Overview

Tetralogy of Fallot (TOF) with pulmonary stenosis is a complex congenital heart defect characterized by four anatomical abnormalities: ventricular septal defect, overriding aorta, right ventricular outflow tract obstruction (pulmonary stenosis), and right ventricular hypertrophy 1. Management of infants and neonates with TOF remains a subject of ongoing discussion and evolving treatment strategies 1.

Diagnosis

  • Diagnosis is typically made prenatally or in the neonatal period via echocardiography 1.
  • Echocardiography is essential for assessing the severity of pulmonary stenosis and other anatomical features 1.
  • Management

  • Surgical repair is the definitive treatment for TOF 1.
  • The timing and type of surgical intervention are critical considerations 1.
  • Management strategies aim to address the right ventricular outflow tract obstruction 1.
  • Key Recommendations

  • Practitioners must consider newer, evolving treatment strategies for TOF with limited data to guide decision-making 1. (Evidence: Expert opinion)
  • A multidisciplinary approach involving cardiologists and surgeons with expertise in TOF is recommended for management 1. (Evidence: Expert opinion)
  • The framework for management should focus on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for outcome assessment 1. (Evidence: Expert opinion)
  • References

    1 Miller JR, Stephens EH, Goldstone AB, Glatz AC, Kane L, Van Arsdell GS et al.. The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot. The Journal of thoracic and cardiovascular surgery 2023. link

    Original source

    1. [1]
      The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot.Miller JR, Stephens EH, Goldstone AB, Glatz AC, Kane L, Van Arsdell GS et al. The Journal of thoracic and cardiovascular surgery (2023)

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