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ALCAPAAnomalous Origin of the Left Coronary Artery from the Pulmonary Artery
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In infants and children with ALCAPA, a false initial diagnosis at different ages occurs frequently owing to the lack of specificity in the clinical manifestations, which range from asymptomatic to severe myocardial ischemia, heart failure, and sudden death.
Transthoracic echocardiography (TTE) showed left ventricular ejection fraction (LVEF) of 40%, moderate mitral valve regurgitation (MR), and suspicion of ALCAPA that was confirmed by cardiac catheterization and coronary computed tomographic angiography (CTA) (figure 1).
2] ALCAPA is associated with high mortality in infants and is an uncommon diagnosis in adults.
ALCAPA is a rare congenital coronary abnormality also known as Bland-White-Garland syndrome.
Sell, who has repaired more than a dozen cases of ALCAPA in children and several in adults, is one of the most experienced surgeons in the world treating the rare and complex heart defect.
Catheterization-angiography was decided to be performed considering ALCAPA as a result of echocardiographic examination.
There are two types of ALCAPA syndrome: the adult and the infant type.
About 10% of ALCAPA patients can survive to adulthood by well-developed intercoronary and extra coronary systemic collaterals (mainly from the bronchial arteries) (2, 4).
Keywords: ALCAPA, Congestive cardiac failure, Angina pectoris, Syncope, Mitral regurgitation.
The acronym ALCAPA stands for 'anomalous left coronary artery originating from the pulmonary artery.
ALCAPA syndrome which is defined as anomalous origin of the left coronary artery from the pulmonary artery constitutes 0.