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Survival into the Sixth Decade after a Pott’s Palliation for Tetralogy of Fallot, complicated by shunt infective endocarditis and massive pulmonary embolism
EP30456
Poster Title: Survival into the Sixth Decade after a Pott’s Palliation for Tetralogy of Fallot, complicated by shunt infective endocarditis and massive pulmonary embolism
Submitted on 08 Nov 2019
Author(s): Lucy Cosbey, Tamara Naneishvili, Adrian Morley-Davies, Hefin Jones
Affiliations: None
This poster was presented at BIR Annual Congress 2019
Poster Views: 120
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Poster Information
Abstract: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease1. This case describes one of the oldest documented patients with an uncorrected TOF and is accompanied by CT images demonstrating TOF with Potts shunt in a patient of this age and of the rare complications that can occur.

A 56-year-old female with TOF was admitted with unexplained weight loss. The patient underwent a Pott’s shunt procedure aged two, however never had the corrective surgery. Blood cultures were positive for streptococcus mitis/oralis. Echocardiography showed a patent Potts shunt and no clinical evidence of endocarditis. The patient was treated for shunt infective endocarditis (IE). Subsequently she developed shortness of breath and CTPA demonstrated an extensive pulmonary embolus from the right ventricular outflow tract to pulmonary root with large right pulmonary infarct.

Survival into late adulthood is rare in patients with unrepaired TOF, with only one reported case of a patient who lived into the seventh decade2. The average life expectancy is low (35 to 40 years).3 For patients with an unrepaired TOF, the incidence of IE is as high as 8% .4 A retrospective study showed 44% incidence of echocardiogram-negative IE.5

This case highlights the rarity of survival into the sixth decade with unrepaired TOF and the rare complications of shunt IE and pulmonary embolism. Congenital heart disease is significantly correlated with an increased incidence of IE. High incidence of echo-negative IE highlights the need to embrace the term: “echo-negative endocarditis” in patients with compatible clinical picture. 

Summary: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease1. This case describes one of the oldest surviving patients with an uncorrected TOF and highlights not only the features of TOF with Potts shunt in a patient of this age but also the rare complications that can occur.
References: -Bertranou EG, Blackstone EH, Hazelrig JB, Turner ME, Kirklin JW. “Life expectancy without surgery in tetralogy of Fallot.” The American Journal of Cardiology. 1978 Sep;42(3):458-66.

-Oosterhof T, Jacobs M, Cramer MJ, and Mulder B. "Survival into Seventh Decade after a Potts Palliation for Tetralogy of Fallot." Congenital Heart Disease (2007): 55-57.

-Viswanathan N. ‘A rare case of tetralogy of fallot (TOF) with Potts shunt – 4th Decade of survival.’ University journal of medicine and medical sciences. Volume 2 Issue 5 2016

-Kaplan EL, Rich H, Gersony W, Manning J. "A collaborative study of infective endocarditis in the 1970s. Emphasis on infections in patients who have undergone cardiovascular surgery." Circulation (1979): 327-35.

-Mirit Hershman-Sarafov, Alona Paz, Israel Potasman. “Echo-Negative Endocarditis: Analysis of 538 Consecutive Transesophageal Echocardiographies”, Open Forum Infectious Diseases, Volume 3, 1 December 2016, 1105, https://d
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