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Chapter 56: Congenital Heart Disease in Adolescents and Adults

A 56-year-old man with a nonrestrictive VSD develops worsening exercise intolerance, pulmonary hypertension, and cyanosis. Which of the following is not true of the Eisenmenger syndrome?

A. Survival in patients with Eisenmenger physiology is reduced compared to healthy control subjects

B. Patients with Eisenmenger physiology are susceptible to in situ pulmonary arterial thrombosis

C. The risk for hyperviscosity is low, with a hemoglobin < 20 g/dL

D. A pulmonary vasodilator may be indicated

E. Closure of the VSD is indicated

The answer is E. (Hurst’s The Heart, 14th Edition, Chap. 56) This patient has the Eisenmenger complex (a reversed shunt in the presence of a nonrestrictive VSD). Survival in patients with Eisenmenger syndrome is reduced by approximately 20 years compared with healthy control subjects (option A).1,2 Shunt correction is contraindicated once shunt reversal occurs (option E). Cyanosis is associated with multiple complications, including in situ pulmonary arterial thrombosis, hyperviscosity (rarely with a hemoglobin < 20 g/dL), pulmonary arterial atherosclerosis, increased susceptibility to gout, and a heightened risk for infective endocarditis (options B and C).3-5 Promising advances have occurred over the past decade in the treatment of patients with pulmonary hypertension with pulmonary vasodilators. In the Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) trial, patients with Eisenmenger syndrome who were treated with Bosentan had a significant decrease in pulmonary vascular resistance and systemic vascular resistance, and a significant increase in 6-minute walk distance (option D).6,7

Which of the following patients does not require prophylactic antibiotics prior to bacteremic procedures, such as dental surgery?

A. A 54-year-old man with a mechanical mitral valve prosthesis

B. A 43-year-old woman with repaired patent ductus arteriosus

C. A 43-year-old woman with Eisenmenger complex

D. A 32-year-old man with tricuspid atresia treated with a Fontan conduit

E. A 31-year-old man with a history of infective endocarditis.

The answer is B. (Hurst’s The Heart, 14th Edition, Chap. 56) Certain subgroups are considered at higher risk for infective endocarditis. Guidelines from the European Society of Cardiology and the AHA/ACC on the prevention of infective endocarditis place patients with prosthetic valves, cyanosis, and systemic or pulmonary artery conduits, as well as patients with previous endocarditis, into a high-risk subgroup (options A, C, D, and E).8,9 Most other congenital cardiac conditions are in a moderate-risk category, except for patients who have undergone surgical or transcatheter repair of ASD, VSD, or PDA (without residua beyond 6 months), who are considered low risk provided there are no sequelae ...

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