Anomalous origin of coronary artery from the pulmonary artery

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Synonyms: ACAPA

A rare coronary artery congenital malformation characterized by an anomalous origin of the left (ALCAPA) or right (ARCAPA) coronary artery from the pulmonary artery with variable clinical presentation ranging from asymptomatic to early heart failure and death depending on the degree of development of collateral circulation between the left and right coronary artery systems as well as the pressure level of the pulmonary artery. Infants typically present with feeding difficulties failure to thrive dyspnea irritability hyperhidrosis heart murmurs tachypnea tachycardia and/or chest pain while adults usually associate dyspnea chest pain syncope and intolerance to physical exercise. Sudden death may occur due to congestive heart failure myocardial infarction valvular insufficiencies or ventricular arrhythmias. The majority of cases reported are of an ALCAPA while ARCAPA is rarely observed.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version July 2024

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