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Management after childhood repair of tetralogy of Fallot

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Opinion statement

The four characteristic findings in tetralogy of Fallot (TOF) include the following: 1) a malaligned ventricular septal defect; 2) right ventricular outflow and/or pulmonary valve/artery stenosis or atresia; 3) a dextraposed overriding aorta; and 4) right ventricular hypertrophy. This article focuses solely on the outcomes and treatment of surgically corrected patients born with TOF. In the modern era, early surgical repair consisting of ventricular septal defect closure and alleviation of right ventricular outflow obstruction have gained favor over early palliation with an aortopulmonary shunt followed by intracardiac repair. Surgical outcomes are excellent and dramatically improve prognosis. However, these patients are not "cured" and are at significant risk of developing subsequent electrical and hemodynamic problems. An operated patient with TOF should be evaluated at regular intervals by a cardiologist trained in congenital heart disease; any symptoms suggestive of hemodynamic or electrical compromise should spur further investigation. Advances in imaging, medical therapy, electrophysiology, device/resynchronization therapy, and percutaneous intervention provide the clinician with a number of therapeutic options. Surgical techniques have changed significantly since the early intracardiac repairs of the 1960s and 1970s. The deleterious hemodynamic and electrical effects of pulmonary regurgitation and ventriculotomy scars have spurred efforts to ensure pulmonary valvular competence and minimize the extent of ventricular incisions. Since Blalock and Taussig’s first palliative shunt in 1945, the survival and quality of life for patients with TOF has improved dramatically; this is one of the great accomplishments for cardiovascular medicine in the 20th century. The 21st century promises further success with the advent of a myriad of technologic advancements.

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Authors and Affiliations

  1. Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, David Geffen School of Medicine at UCLA, 650 Charles E. Young Drive South, BH-307, 90095-1679, Los Angeles, CA, USA

    John S. Child MD

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  1. Jamil AboulHosn MD
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AboulHosn, J., Child, J.S. Management after childhood repair of tetralogy of Fallot. Curr Treat Options Cardio Med 8, 474–483 (2006). https://doi.org/10.1007/s11936-006-0036-4

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