What is the prognosis of tetralogy of Fallot (TOF)?

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

In the present era of cardiac surgery, children with simple forms of tetralogy of Fallot (TOF) enjoy good long-term survival with an excellent quality of life. Late outcome data suggest that most survivors are in New York Heart Association (NYHA) classification I, although maximal exercise capability is reduced in some.

About 75% infants who undergo repair during infancy will survive to reach their second to third decade of life without major consequences. However, after the first two decades of life, symptoms start to appear due to pulmonary valve regurgitation. By the fourth decade of life, most survivors are symptomatic.

Adult patients with tetralogy of Fallot who undergo surgery again are usually symptom free for 10-15 years, but by the time they reach their fifth decade, even these patients begin to have symptoms. [16] Although the second surgery reduces the rate of death, the majority of these individuals have a shorter lifespan than age-matched control subjects without a history of congenital heart disease. Adults with recalcitrant arrhythmias and right heart failure have the worst prognosis.

Sudden death from ventricular arrhythmias has been reported in 1-5% of patients at a later stage in life, and the cause remains unknown. It has been suspected that ventricular dysfunction may be the cause. One study found left ventricular longitudinal dysfunction to be associated with a greater risk of developing life-threatening arrhythmias. [19] Continued cardiac monitoring into adult life is necessary. For some time, it has been suspected that certain children may have inherited a predispostion to developing long QT syndrome. A 2012 study by Chiu confirmed this suspicion. [20]

Most individuals who survive to age 30 years develop congestive heart failure (CHF), although individuals whose shunts produce minimal hemodynamic compromise have been noted, albeit rarely, and these individuals achieve a normal life span. Survival of patients into their 80s have been reported. Due to advanced surgical techniques, a 40% reduction in deaths associated with tetralogy of Fallot was noted from 1979 to 2005. [18]


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