Long-Term Follow-Up After Repair of Tetralogy of Fallot

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Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Successful surgical palliation was developed 50 years ago, and, for the past 40 Years. complete intracardiac repair has been possible. Surgical techniques have improved, and expected adult survival rates are now between 76 and 95% Exercise tolerance and quality of life are generally excellent into early adulthood. However, a number of residua and sequelae have resulted in late cardiac mortality from 5 to 9%. Long-term problems include rhythm disturbances, residual pulmonary regurgitation, and sudden cardiac death. The incidence of ventricular arrhythmias is as high as 50% late after surgery for TOF, and might be accompanied by hemodynamic abnormality, requiring surgical intervention. Although symptomatic ventricular arrhythmias require treatment, differences of opinion exist regarding the need to treat asymptomatic patients with incidental ventricular arrhythmias There are fewer studies of atrial arrhythmias in the postoperative TOF patients; however, sinus node dysfunction and atrial arrhythmias are present in 30% and can be responsible for symptoms in such patients. Pulmonary regurgitation is common and results in a variety of findings, including right ventricular dilation, right ventricular dysfunction, tricuspid regurgitation, and exercise intolerance. Such findings may indicate a need for pulmonary valve placement late during the follow up of TOF patients. Late sudden cardiac death is responsible for the majority of late mortality and may be related to or heralded by ventricular arrhythmias. It is possible that newer surgical techniques, using a transatrial approach, will reduce the incidence of late sudden cardiac death. Because patients after complete repair of TOF are at increased risk of cardiac morbidity and mortality, they require close, serial follow-up by-physicians knowledgeable in the long-term history of congenital cardiac defects. An aggressive surveillance and treatment approach is recommended to identify and prevent adverse long term consequences. For most adults after repair of TOF, quality of life is excellent, including opportunities for employment, education, physical activity, and pregnancy.

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