Eisenmenger’s syndrome ACC/AHA guidelines for evaluation of patients

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief: Priyamavada Singh, MBBS [[2]] Assistant Editor-In-Chief: Kristin Feeney, B.S. [[3]]

ACC / AHA Guidelines- Recommendations for Evaluation of the Patient With Congenital Heart Disease– Pulmonary Arterial Hypertension (DO NOT EDIT)

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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)

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