A case of “late adult type” of ALCAPA syndrome in a 76-year-old woman

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare condition; late adult presentation is extremely rare but, with modern diagnostics, more asymptomatic or mildly symptomatic elderly patients with ALCAPA are found. A 76-year-old woman was admitted to our emergency department in December 2021 with fever, fatigue, cognitive-motor slowing, and hyposthenia of left hemisoma. Cranium computed tomography (CT) was performed, with no signs of stroke. Contrast-enhanced CT, performed with electrocardiogram-gated technique, showed a dilatated left coronary artery arising directly from the main pulmonary artery; right coronary artery was markedly dilated and tortuous and characterized by multiple inter-coronary collateral arteries with left coronary artery, in particular in the retro-aortic side and along the cone artery. ALCAPA syndrome represents one of the most common causes of myocardial ischemia in children and, if left untreated, results in a mortality rate of up to 80–90 % within the first year of life. In our case, no previous history of cardiological disease was found that could have suggested this type of condition. This is extremely rare, especially if we consider that our patient reached the 8th decade of life.

Learning objectives

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  ALCAPA syndrome can present in adulthood.

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  Collaterals between coronary arteries are essential to guarantee the survival into adulthood.

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  Cardiac computed tomography imaging is essential for the diagnosis.