Tetralogy of fallot natural history complications and prognosis
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Tetralogy of fallot Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [3]
Overview
The prognosis of patients with repaired Tetralogy of Fallot has improved over the years and these patients now have the potential to lead normal lives.
Natural History
- If left untreated, the natural history of Tetralogy of Fallot results in progressive right ventricular hypertrophy and right ventricular dilatation due to the increased resistance to flow into the pulmonary circuit.
- The dilated cardiomyopathy progresses to right heart failure, usually with accompanying left heart failure.
Complications
- Delayed growth and development
- Irregular heart rhythms (arrhythmias)
- Seizures as a result of hypoxia
- Death
Prognosis
Unrepaired Tetralogy of Fallot:
The survival for untreated Tetralogy of Fallot is:
- 75% after the first year of life
- 60% by four years
- 30% by ten years
- 5% by forty years
Repaired Tetralogy of Fallot
- Patients with repaired Tetralogy of Fallot have the potential to lead normal lives with continued excellent cardiac function, with some considerations:
- Current techniques for total surgical repair greatly improve the hemodynamic function of the heart with Tetralogy of Fallot but do not provide a lifetime correction of the defect.
- Ninety percent of patients with total repair as infants develop a progressively leaky pulmonary valve as the heart grows to its adult size. Patients also may have some degree of residual right outflow stenosis and damage to the electrical system of the heart from surgical incisions, causing abnormalities as detected by EKG and/or arrhythmias.
- Long-term follow up studies show that this patient population is at risk for sudden cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult congenital cardiologist is recommended to monitor these risks and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary. Risk factors for sudden death include older age at repair, male sex, advanced NY heart association class, repair via atriotomy. Two major electrocardiographic risk factors include complete heart block beyond the third post operative day and QRS duration > 18 milliseconds, and rapid development of QRS prolongation in the first 6 months after repair. There is limited data regarding defibrillator implantation.
- Antibiotic prophylaxis is indicated during dental treatment in order to prevent infective endocarditis.