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Management of Adults With Congenital Heart Disease

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7 Table 5. Physiological Variables as Used in ACHD AP Classification Variable Description NYHA functional classification system Functional Capacity I. Patients with cardiac disease but resulting in no limitation of physical activity: Ordinary physical activity does not cause undue fatigue, palpitation, dyspnea, or anginal pain. II. Patients with cardiac disease resulting in slight limitation of physical activity: ey are comfortable at rest. Ordinary physical activity results in fatigue, palpitations, dyspnea, or anginal pain. III. Patients with cardiac disease resulting in marked limitation of physical activity: ey are comfortable at rest. Less than ordinary activity causes fatigue, palpitations, dyspnea, or anginal pain. IV. Patients with cardiac disease resulting in inability to carry on any physical activity without discomfort: Symptoms of heart failure or the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort increases. Pulmonary arterial hypertension Pulmonary arterial hypertension is defined as: • Mean pulmonary artery pressure by right heart catheterization ≥20 mm Hg and a pulmonary capillary wedge pressure ≤15 mm Hg ; and pulmonary vascular resistance ≥2 Wood units. Pulmonary arterial hypertension risk categories are measured using the 3-strata risk score calculator. Shunt (hemodynamically significant shunt) An intracardiac shunt is considered hemodynamically significant if there is evidence of chamber enlargement distal to the shunt and/or evidence of sustained Qp:Qs ≥1.5. An intracardiac shunt not meeting these criteria would be described as small or trivial. Modified with permission from Stout et al. Copyright © 2018 American Heart Association, Inc. and American College of Cardiolog y Foundation. ACHD indicates adult congenital heart disease; AP, anatomic and physiological; ICD, implantable cardioverter-defibrillator; NYHA, New York Heart Association; and Qp:Qs, pulmonary-to-systemic blood flow ratio. (cont'd) 3.1. Care Access and Delivery in the ACHD Population COR LOE Recommendation 1 B-NR 1. ACHD programs and clinicians who care for adults with congenital heart disease should follow policies and procedures that reduce barriers, reduce attrition, and improve access to lifelong ACHD care.

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