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Tetralogy of Fallot with Pulmonary Atresia

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Critical Care of Children with Heart Disease

Abstract

Tetralogy of Fallot with pulmonary atresia (TOF-PA) accounts for 2% of all forms of congenital heart disease and for 20% of all forms of TOF. TOF-PA is slightly more prevalent in males than in females and is frequently associated with the 22q11 syndrome. The intra-cardiac anatomy of TOF-PA has all the features of classic Tetralogy of Fallot: ventricular septal defect, overriding of the aorta, right ventricular outflow obstruction, and right ventricular hypertrophy. The difference is in the membranous or complete atresia of the pulmonary valve and extreme variability of the architecture of the main and distal pulmonary arteries. The central pulmonary arteries can be of good size, variably hypoplastic, discontinuous, or even absent. A persistent ductus arteriosus (PDA), major aorto-pulmonary collaterals (MAPCAs), or both may provide blood flow to the pulmonary vasculature.

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Author information

Authors and Affiliations

  1. Pediatrics and Critical Care Medicine, University of Pittsburgh, Cardiac Intensive Care Unit, Pittsburgh, PA, USA

    Yuliya A. Domnina

  2. University of Pittsburgh, Cardiac Intensive Care Unit, Pittsburgh, PA, USA

    Wonshill Koh

  3. Pediatric Cardiology, Division of Cardiac Critical Care Medicine/Telemedicine, Children’s National Health System, Washington, DC, USA

    Alejandro Lopez-Magallon

  4. Department of Pediatric Cardiothoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Victor O. Morell

  5. Department of Pediatric Cardiothoracic Surgery, UPMC – Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Jose Pedro da Silva

  6. Division of Adolescent and Young Adult Medicine, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Traci M. Kazmerski

  7. Children’s Heart Institute, Memorial Care Miller Children’s and Women’s Hospital Long Beach, Long Beach, CA, USA

    Constantinos Chrysostomou

Authors
  1. Yuliya A. Domnina
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  2. Wonshill Koh
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  3. Alejandro Lopez-Magallon
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  4. Victor O. Morell
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  5. Jose Pedro da Silva
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  6. Traci M. Kazmerski
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  7. Constantinos Chrysostomou
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Corresponding author

Correspondence to Yuliya A. Domnina .

Editor information

Editors and Affiliations

  1. Chief, Division of Cardiac Critical Care Medicine Executive Director, Telemedicine Co-director, Children’s National Heart Institute, Professor of Pediatrics, George Washington University School of Medicine, Children’s National Health System, Washington, DC, USA

    Ricardo A. Munoz

  2. Department of Pediatric Cardiothoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Victor O. Morell

  3. Associate Medical Director, CHCO Heart Institute Head, Pediatric Cardiac Critical Care Program & Inpatient Services Director, Cardiac Intensive Care Unit Children’s Hospital Colorado, Tenured Professor of Pediatrics, Pediatric Cardiology and Intensive Care University of Colorado Denver, School of Medicine, Aurora, CO, USA

    Eduardo M. da Cruz

  4. Department of Pharmacy, Pediatric Critical Care, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Carol G. Vetterly

  5. Department of Pediatric Cardiothoracic Surgery, UPMC – Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Jose Pedro da Silva

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Domnina, Y.A. et al. (2020). Tetralogy of Fallot with Pulmonary Atresia. In: Munoz, R., Morell, V., da Cruz, E., Vetterly, C., da Silva, J. (eds) Critical Care of Children with Heart Disease . Springer, Cham. https://doi.org/10.1007/978-3-030-21870-6_20

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  • DOI: https://doi.org/10.1007/978-3-030-21870-6_20

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-21869-0

  • Online ISBN: 978-3-030-21870-6

  • eBook Packages: MedicineMedicine (R0)

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