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Tabitha Moe, MD | HonorHealth Heart Care

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease and impacts approximately 1:3,000 live births in the United States today. It accounts for approximately 10% of all cases of complex congenital heart disease. Morbidity and mortality from TOF have steadily declined in the modern era of surgical repair and nearly 97% of patients are thriving into adulthood.

TOF is a constellation of defects, including a ventricular septal defect (VSD), pulmonary stenosis, overriding aorta and right ventricular hypertrophy. The defect is palliated with VSD closure and relieving the pulmonary outflow stenosis. Long-term complications include pulmonary valve stenosis and insufficiency, aortic dilation, and atrial and ventricular arrhythmias. Ventricular arrhythmias can be predicted by an increase in QRSd to >150ms, or an increase in QRS by >30ms/year.

• EKG’s should be performed annually
• Ambulatory telemetry monitoring should be performed every 12-24 months
• Congenital transthoracic echocardiography performed every six to 12 months
• Cardiopulmonary exercise testing performed every three to five years
• Cardiac MRI or cardiac CTA performed every two to four years

About the author

Tabitha Moe, MD, is a board-certified cardiologist offering the highest quality of care for adult patients born with congenital heart disease, including full-spectrum care with pregnancy, pulmonary hypertension and multi-organ involvement following complex palliations for congenital heart disease. To refer a patient for congenital heart disease, please call 602-386-1100.

Sources cited

1. Attenhofer Jost CH, Connolly HM, Burkhart HM, et al. Tetralogy of fallot repair in patients 40 years or older. Mayo Clin Proc 2010; 85:1090.
2. Presbitero P, Demarie D, Aruta E, et al. Results of total correction of tetralogy of Fallot performed in adults. Ann Thorac Surg 1988; 46:297.
3. Dittrich S, Vogel M, Dähnert I, et al. Surgical repair of tetralogy of Fallot in adults today. Clin Cardiol 1999; 22:460.
4. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2019; 139:e698.

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