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Tetralogy of Fallot: management of residual hemodynamic and electrophysiological abnormalities
  1. Ali N Zaidi
  1. Mount Sinai Adult Congenital Heart Disease Center, Mount Sinai Heart, Icahn School of Medicine at Mount Sinai, New York, New York, USA
  1. Correspondence to Dr Ali N Zaidi, Cardiology, Mount Sinai School of Medicine, New York, New York, USA; Ali.Zaidi{at}mountsinai.org

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Learning objectives

  • Describe the basic anatomical and physiological abnormalities and variations associated with tetralogy of Fallot.

  • Identify the long-term hemodynamic consequences of repaired tetralogy of Fallot.

  • Identity the long-term electrophysiological consequences of repaired tetralogy of Fallot.

  • Understand the parameters at which patients with repaired tetralogy of Fallot with severe pulmonary regurgitation undergo pulmonary valve replacement.

Background

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD). Approximately 3.5% of all infants born with CHD have TOF which corresponds to 0.28 out of every 1000 live births. TOF affects males and females approximately equally; however, most cases are sporadic. The risk of recurrence in siblings is approximately 2%–3%, and the risk of the offspring of a patient having TOF (in the absence of 22q.11.2 deletion) is 3%–4%.1 With improvements in surgical and medical expertise, it is postulated that there are now more adults living with TOF than children in the USA.

Pathophysiology

TOF is the result of the four anatomical features: a ventricular septal defect (VSD), an overriding (rightward deviating) aorta, right ventricular outflow tract obstruction (RVOTO) and right ventricular hypertrophy (RVH) (figure 1). The anterocephalad deviation of the outlet septum, which is the muscular structure that separates the subaortic from the subpulmonary outlets, is the primary pathological event. The VSD is usually perimembranous, often large and unrestrictive, except when its right ventricular margin is shielded by accessory tricuspid valve tissue or if septal hypertrophy narrows the defect.2 The aorta may override the VSD, along with multilevel obstruction to pulmonary blood flow. Infundibular stenosis is present in almost all TOF patients which is likely due to the narrowed diameter of the infundibular region.2 The pulmonary valve is usually thickened, bicuspid and may cause valvular stenosis.3 Rarely, stenosis or hypoplasia of the pulmonary arteries has also been …

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Footnotes

  • Contributors ANZ is the sole author to this manuscript. Ms Mikaela Stiver is responsible for two illustrations: Figure 1 and Figure 2.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Provenance and peer review Commissioned; internally peer reviewed.

  • Author note References which include a * are considered to be key references.

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