What is the efficacy of surgical repair of tetralogy of Fallot (TOF) in infancy?

Updated: Nov 13, 2018
  • Author: Shabir Bhimji, MD, PhD; Chief Editor: Yasmine S Ali, MD, FACC, FACP, MSCI  more...
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Answer

It is important to understand that corrective surgery for tetralogy of Fallot performed in childhood is not curative surgery. Rather, this procedure only corrects the anatomic defects—without changing the progression of the disease or addressing the consequences of using a patch. As a result, these individuals develop new symptoms and eventually present to the cardiology clinic with diverse symptoms. Thus, most pediatric surgeons consider the initial surgery for tetralogy of Fallot to be long-term palliation, not definitive surgery.

With advancing age, children with tetralogy of Fallot who had corrective surgery are usually asymptomatic for the first decade after the initial procedure. Thereafter, they develop adverse myocardial problems, the majority of which involve varying degrees of pulmonary valve insufficiency, which, in turn, can lead to RV overload and RV distention and failure. [7]

RV enlargement also leads to the development of atrial and ventricular arrhythmias, which, if not recognized early, are a common cause of high morbidity and mortality. An estimated one third of adults with tetralogy of Fallot experience atrial and ventricular arrhythmias over a period of 2-3 decades. The incidence of sudden death ranges from 5% to 7% in patients who develop these arrhythmias.{ref53-INVALID REFERENCE}

Another concern regarding patients with tetralogy of Fallot is that many are lost to follow-up as they become adults, and thus the opportunity to manage complications before they become irreversible can be missed. Several surveys indicate that many children with repaired tetralogy of Fallot feel fine after surgery, believe that they are cured, and then stop seeing their cardiologist. In other cases, the primary care provider is unaware of the need to follow these patients, owing to the erroneous belief that surgery is curative.

See also Tetralogy of Fallot With Pulmonary Stenosis, Tetralogy of Fallot With Pulmonary Atresia, and Tetralogy of Fallot With Absent Pulmonary Valve.


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