Eisenmenger’s syndrome diagnostic study of choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Abdelrahman Ibrahim Abushouk, MD[2], Priyamavada Singh, MBBS [3], Kristin Feeney, B.S. [4]

2018 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)

Class I

"1. Care of adult patients with CHD-related PAH should be performed in centers that have shared expertise and training in both ACHD and PAH. (Level of Evidence: C)"

"2. The evaluation of all ACHD patients with suspected PAH should include noninvasive assessment of cardiovascular anatomy and potential shunting, as detailed below:"

"a. Pulse oximetry, with and without administration of supplemental oxygen, as appropriate. (Level of Evidence: C)"

"b. Chest x-ray. (Level of Evidence: C)"

"c. ECG. (Level of Evidence: C)"

"d. Diagnostic cardiovascular imaging via TTE, TEE, MRI, or CT as appropriate. (Level of Evidence: C)"

"e. Complete blood count and nuclear lung scintigraphy. (Level of Evidence: C)"

"3. If PAH is identified but its causes are not fully recognized, additional testing should include the following:"

"a. Pulmonary function tests with volumes and diffusion capacity (diffusing capacity of the lung for carbon monoxide). (Level of Evidence: C)"

"b. Pulmonary embolism-protocol CT with parenchymal lung windows. (Level of Evidence: C)"

"c. Additional testing as appropriate to rule out contributing causes of PAH. (Level of Evidence: C)"

"d. Cardiac catheterization at least once, with potential for vasodilator testing or anatomic intervention, at a center with expertise in catheterization, PAH, and management of CHD-PAH. (Level of Evidence: C)"

Class IIa

"1. It is reasonable to include a 6-minute walk test or similar nonmaximal cardiopulmonary exercise test as part of the functional assessment of patients with CHD-PAH. (Level of Evidence: C)"