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A. Eisenmenger Syndrome
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Large congenital systemic-to-pulmonary shunt leading to pulmonary vasculopathy, increased PVR and reversal of shunt. Cyanosis and erythrocytosis present.
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B. PAH associated with a predominant systemic-to-pulmonary shunt
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Moderate-large shunt with mild-moderately increased pulmonary vascular resistance. Patient is not cyanotic at rest. Defect may be correctable or non-correctable.
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C. PAH associated with a small defect
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Significantly elevated pulmonary vascular resistance in the presence of a small defect (ASD < 2 cm diameter, VSD < 1 cm diameter). Often behaves similarly to idiopathic pulmonary arterial hypertension.
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D. PAH associated with a repaired defect
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PAH persists after closure or develops/recurs following closure.
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