Case Report: ALCAPA syndrome: successful repair with an anatomical and physiological alternative surgical technique

Abstract

Anomalous left coronary artery from the pulmonary artery, or ALCAPA syndrome, is a rare congenital cardiac disease that can cause myocardial infarction, heart failure and even death in paediatric patients. Only few untreated patients survive until adult age. Here we present the case of a 33-year-old female patient with paroxysmal tachycardia, syncope and mild exertional dyspnoea. She was diagnosed with ALCAPA syndrome and underwent surgical correction with an alternative technique of left main coronary artery extension to the aorta.

Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy

“…Without early and accurate diagnosis and treatment, the natural course of the disease is devastating and 90% untreated children die in the first year of life because of massive myocardial injury and following end-stage heart failure. [ 3 , 4 ] With appropriate diagnosis and emergency cardiac surgery, they have a much better chance to survive. However, owing to the lack of specificity in the clinical manifestations, ALCAPA has a higher rate of false initial diagnosis.…”

Complex Repair of Anomalous Left Coronary Artery From the Pulmonary Artery in a 55-Year-Old Patient

“…The gap between the pulmonary artery and aorta can be bridged by a Cabrol-type reconstruction using an interposition graft, or by harvesting a strip of pulmonary artery along with the coronary and adding an anterior patch. 5 We chose the former, as the native tissues were friable. The interposition coronary conduit made direct reanastomosis of the pulmonary artery difficult, necessitating an additional interposition graft for the pulmonary artery reconstruction.…”

Surgical Outcome in Adolescents and Adults With Anomalous Left Coronary Artery From Pulmonary Artery

“…Various approaches have been adopted to treat adult patients, and short-term effects are usually satisfactory [2][3][4][5][6]. However, due to lack of evidence, surgical strategies for older patients have not yet been standardized.…”

“…Only 10% to 15% survive to adolescence or even adulthood due to formation of extensive collateral circulations [1]. Nowadays, with improvement in health care, the number of reported adult patients is growing [2][3][4][5]. However, the prognosis of ALCAPA, if left untreated, is still very poor, and sudden death frequently occurs within the first 3 decades of life [2].…”

“…However, there are few reports comparing ALCAPA surgical treatments in adolescent and adult patients, expect for some case reports and sporadic studies analyzing adult patients mixed with pediatric patients [2][3][4][5][6]. Furthermore, long-term outcome analysis on surgical treatments for adult patients is still lacking.…”