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Tetralogy of Fallot

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Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

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Abstract

Tetralogy of Fallot is one of the most common congenital cardiac malformations and consists of (1) ventricular septal defect, (2) right ventricular outflow tract obstruction, (3) aortic override, and (4) right ventricular hypertrophy. The initial presentation of the patient with tetralogy of Fallot is variable and dependent on the degree of right ventricular outflow tract obstruction. Initial medical management of tetralogy of Fallot is aimed at monitoring and managing hypoxemia and preventing hypercyanotic spells. A small percentage of patients will present with significant cyanosis in the neonatal period. The majority, however, will have stable pulmonary artery blood flow and require no immediate treatment. Cyanosis gradually progresses as pulmonary blood flow is limited by increasing right ventricular outflow tract obstruction. Diagnosis is usually established with transthoracic echocardiography. Further anatomic detail and functional data may also be garnered with cardiac MRI or cardiac catheterization, which may help define and characterize additional sources of pulmonary blood flow or abnormalities of systemic venous return.

Indications for surgical intervention are symptoms of hypercyanotic episodes or the presence of oxygen saturations that are persistently below 75–80%. In most children with tetralogy of Fallot, elective complete repair is recommended by 1 year of age with most centers recommending elective repair in the first 3–6 months of life. While palliative procedures can be performed to delay the need for definitive complete repair, most centers favor early complete repair, reserving palliative procedures for those patients that have contraindications to open cardiac surgery. In the current era, outcomes for complete repair are excellent, even when definitive repair is required in the neonatal period.

Morphology and associated defects in tetralogy of Fallot may include tetralogy of Fallot with absent pulmonary valve, tetralogy of Fallot with complete atrioventricular septal defect, and tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals. These entities may behave differently, and strategy and timing for surgical repair are often more complicated than for simple tetralogy of Fallot. Following repair of tetralogy of Fallot, lifelong follow-up is usually indicated in order to monitor for recurrent or persistent pathology of the right ventricular outflow tract and for appropriate intervention if indicated.

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Author information

Authors and Affiliations

  1. Department of Cardiovascular Services, Nemours Children’s Hospital, Orlando, FL, USA

    Jennifer S. Nelson

  2. Department of Cardiac Surgery, University of Michigan Medical School, Ann Arbor, MI, USA

    Edward L. Bove & Jennifer C. Hirsch-Romano

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  1. Jennifer S. Nelson
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  2. Edward L. Bove
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  3. Jennifer C. Hirsch-Romano
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Correspondence to Jennifer S. Nelson .

Editor information

Editors and Affiliations

  1. Children’s Hospital Colorado University of Colorado Denver, School of, Aurora, CO, USA

    Eduardo M. da Cruz

  2. Children's Hospital Colorado University of Colorado School of Medicin, Aurora, CO, USA

    Dunbar Ivy

  3. Department of Cardiac Surgery, German Pediatric Heart Center, Sankt Augustin, Germany

    Viktor Hraska

  4. Pediatric Cardiac Surgery, Children's Hospital Colorado University of Colorado, Aurora, CO, USA

    James Jaggers

Section Editor information

  1. The Heart Institute, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA

    Eduardo M. da Cruz

  2. The Heart Institute, Pediatric Cardiac Surgery, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA

    James Jaggers

  3. Cardiothoracic Surgery, University of Colorado School of Medicine The Children’s Hospital Pediatrics, Aurora, CO, USA

    James Jaggers

  4. Dept. Pediatric Cardiology, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    Dunbar Ivy

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Nelson, J.S., Bove, E.L., Hirsch-Romano, J.C. (2021). Tetralogy of Fallot. In: da Cruz, E.M., Ivy, D., Hraska, V., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4999-6_18-2

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  • DOI: https://doi.org/10.1007/978-1-4471-4999-6_18-2

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  • Print ISBN: 978-1-4471-4999-6

  • Online ISBN: 978-1-4471-4999-6

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