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Corrected Tetralogy of Fallot in an Adult: A Case of Long-term ComplicationsStephanie Muir, OMS-III; Navin Kedia, DO; William Peppo, DOMidwestern University Arizona College of Osteopathic Medicine; Carondelet Heart and Vascular Institute Physicians, Cardiology East

ABSTRACTIntroduction: Tetralogy of Fallot is a congenital heart defect consisting of a ventricular septal defect,overriding aorta, pulmonary artery stenosis and right ventricular hypertrophy. It is the most commoncause of cyanotic congenital heart defect occurring in approximately 3-6 infants per 10,000 births andpresent in roughly 10% of congenital heart defect cases. Historically, more than 90-95% of patients withtetralogy of Fallot died before the age of 20 without surgery. However, with the advent of improvedcardiovascular surgical techniques, more than 85% of these patients survive to adulthood, posing newchallenges for the management of adult patients with tetralogy of Fallot.

Clinical Presentation: Patient AH was a 26-year-old male with a history of three corrective surgeries fortetralogy of Fallot who was seen due to complaint of random episodes of lightheadedness and syncope,accompanied by substernal chest pain. Imaging revealed a stenotic conduit and runs of non-sustainedventricular tachycardia.

Conclusion: The presentation and management of tetralogy of Fallot is historically discussed in childrenborn with the congenital cardiac defect. However, as surgical interventions have become moresuccessful in treating these cases, the focus needs to shift to include long-term management of thesequelae arising in adults following corrective surgery.

CASE PRESENTATION• AH was a 26-year-old male with a history of corrected tetralogy of Fallot who was seen due to

complaints of random episodes of lightheadedness and syncope, accompanied by substernal chestpain. He denied any known provocations. He had a history of three corrective surgeries: the first at 2days old, then 2 months old and the last at 16 years old. Despite the corrective surgeries, herequired supplemental oxygen over the last several years at variable times throughout the day andwhile sleeping at night due to occasional oxygen desaturation per his pulse oximeter anddevelopment of peripheral cyanosis.

• On physical exam, AH was a thin-appearing young man in no apparent distress. Cardiac examrevealed a holosystolic murmur loudest at the left sternal border. Lungs were clear to auscultation.There were no signs of peripheral or central cyanosis or clubbing of his digits.

• An EKG was performed in office to establish a baseline while a 30-day event monitor was ordered tobe worn at home. He was also to follow up for a chest x-ray and a trans-thoracic echocardiogram.

DISCUSSIONTetralogy of Fallot (TOF) is a congenital heart defect consisting of a ventricular septal defect (VSD),overriding aorta, pulmonary artery stenosis and right ventricular hypertrophy. It occurs when there isanterior and cephalad deviation of the infundibular septum resulting in a misaligned ventricular septaldefect with an overriding aortic root that causes a right ventricular outflow tract (RVOT) obstruction.The degree of right ventricular outflow tract obstruction and thus adequacy of pulmonic flow is themost important determinant of prognosis. As obstruction increases, there is increased resistance ofpulmonic blood flow, which causes a right-to-left shunt of blood flow, and an increase in the amount ofdeoxygenated blood bypassing pulmonic circulation to move directly into systemic circulation.

TOF surgery may include repair of the VSD and/or creating a conduit to increase the blood flowing intothe pulmonary artery and thereby decreasing the rate of deoxygenated blood entering the aorta. Therate of TOF children surviving into adulthood has increased from less then 10% to close to 85% with theadvent of the cardiovascular repair surgery.

Postoperative TOF repair requires long-term follow up due to risk of developing chronic complications.In addition to creating a right bundle branch block, complications include pulmonary regurgitation,residual RVOT obstruction, right ventricular dysfunction, cardiac arrhythmias such as aortic valveinsufficiency, and sudden cardiac death. Right ventricular dysfunction can lead to exercise intolerance,right heart failure, and other arrhythmias such as ventricular tachycardia, atrial flutter, and atrialfibrillation.

As this population of repaired TOF patients grow older, more sequelae will be discovered that needs tobe monitored and managed.

IMAGING

Initial EKG: Normal sinus rhythm with

right bundle branch block and right

ventricular hypertrophy

30-Day event monitor: Runs of

non-sustained ventricular

tachycardia (VT)

Chest X-ray: Mild nonspecific

cardiomegaly without pulmonary edema or

effusions. The pulmonary vasculature

appear normal

LAB RESULTS

CONCLUSION

REFERENCES/ACKNOWLEDGEMENT• Bhimji S. Tetralogy of Fallot. Medscape. http://emedicine.medscape.com/article/2035949-overview#a7. Published

September 27, 2016. Accessed March 26, 2017.

• Doyle, Thomas MD; Kavanaugh-McHugh, Ann MD. Pathophysiology, clinical features, and diagnosis of tetralogy ofFallot. Uptodate. https://www.uptodate.com/contents/pathophysiology-clinical-features-and-diagnosis-of-tetralogy-of-fallot?source=search_result&search=tetralogy%20of%20fallot%20adult&selectedTitle=1~139. Updated May 6, 2015.Accessed March 26, 2017.

• Doyle Thomas MD, Kavanaugh-McHugh Ann MD, Fish Frank A MD. Management and Outcome of Tetralogy of Fallot.Uptodate. https://www.uptodate.com/contents/management-and-outcome-of-tetralogy-of-fallot?source=see_link.Updated June 21, 2016. Accessed March 26, 2017.

• Gerber, Rudolph. Congenital Heart Disease. Jour Amer Osteo Assoc. 1953; 53(2):107-110.

• Huehnergarth KV, Gurvitz M, Stout KK, et al. Repaired tetralogy of Fallot in the adult: monitoring and management.Heart 2008;94(12):1663-1669. http://heart.bmj.com/content/94/12/1663.long. Accessed March 26, 2017.

• Special thanks to Dr. Kedia and Dr. Peppo for their continued education and mentorship.

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• With the advent of corrective surgery, patients born with tetralogy ofFallot have an increased rate of survival into adulthood.

• With this increased survival comes an increased amount of long-termsequelae, some of which is still being discovered. Thus, physiciansneed to be vigilant to catch these complications.

• Patient AH had several long-term post-operative complications,including conduit stenosis and regurgitation, syncope, right bundlebranch block, non-sustained ventricular tachycardia, and risk ofsudden cardiac death. Metoprolol tartrate 25 mg twice a day wasprescribed to control his heart rate and decrease the risk ofdeveloping sustained ventricular tachycardia and sudden cardiacdeath.

• All TOF patients should have an EKG to establish baseline so anychanges can be monitored. Additional testing for cardiac relatedsymptoms include an echocardiogram, chest X-ray, MRI, andangiogram. This patient is scheduled for an outpatient cardiac MRI tofurther evaluate his cardiac function.

• These patients must be monitored and treated before they suffereven further complications, such as sudden cardiac death.

• Ca2+: 9.3

• Albumin: 4.7

• Total bilirubin: 1.0

• Alkaline phosphatase: 40

• AST: 28

• ALT: 29

• Troponin: <0.03

Echocardiogram (not shown): TOF repair with stenotic right ventricle-pulmonary artery conduit with severe conduit

regurgitation, right ventricular enlargement, and moderate tricuspid regurgitation. No residual ventricular defect or aortic regurgitation.

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