Abstract

Among adult patients with congenital heart disease, symptoms and clinical trajectory can vary dramatically based on the type and severity of the underlying condition. Due to improved imaging techniques and widely available screening tests, most life-threatening congenital heart disease is diagnosed in utero in the United States. In patients who present with congenital heart disease in adulthood, many have been appropriately treated (often surgically corrected), followed by a pediatric/congenital cardiologist, and remain on a stable trajectory. For patients who present for the first time with congenital heart disease in adulthood, there is typically a reason that the presentation was delayed. Among patients with severe congenital heart disease (cyanotic heart disease, large ventricular septal defect, or atrial septal defect), this is often due to lack of proper medical care and surveillance, but many patients can also present with progression of a clinical condition that was not previously detected despite adequate medical attention. Many patients also present after being lost to follow-up years after initial surgical treatment. It is helpful to group these patients based on underlying pathophysiology and the presence or absence of previous surgical correction.

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