Adult ALCAPA: from histological picture to clinical features

Abstract

Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity. Case presentation: We were involved in the assessment, treatment, and pathological evaluation of two adult ALCAPA patients who were rescued from ventricular fibrillation and then surgically treated to establish a dual coronary artery system. Histological studies indicated various chronic ischemic changes in the myocardium, patchy fibrosis, and severely thickened arteriolar walls in both ventricles. The first patient is alive and well 11.5 years after surgical correction without any implantable cardioverter defibrillator (ICD) activations. The second patient required redo surgery 9 months after the initial operation but subsequently died. Histologically, chronic ischemic alteration of the myocardium and thickened arteriolar walls persisted even after surgical correction, and coronary angiography (CAG) showed an extremely slow flow phenomenon even after surgical correction in both patients. The average postoperative opacification rate in the first case was 7.36 + 1.12 (n = 2) in the RCA, 3.81 + 0.51 (n = 3) in the left anterior descending (LAD) artery, and 4.08 + 0.27 (n = 4) in the left circumflex (LCx) artery. The slow flow phenomenon may represent persistent high arteriolar resistance in both ventricles. Conclusions: Seldom reported or new findings in adult ALCAPA were identified in two cases. More frequent diagnosis of adult ALCAPA can be expected because of the widespread availability of resuscitation and more advanced diagnostic modalities. Accumulation of pathological and clinical findings and confirmation of the longterm follow-up results after treatment may contribute to expanding our knowledge of this rare entity and establishing optimal treatment.

Intraoperative Diagnosis of ALCAPA Complicating an Aortopulmonary Window Repair

“…Surgical management is mandatory because the mortality without intervention goes up to 90% during first year of life. 4…”

“…Both techniques offer good surgical results with long-term survival from 78% to 100% and low operative mortality, but a risk of reoperation as high as 30% can be seen in the Takeuchi technique. 4,5,6…”

Anomalous origin of left main coronary artery from pulmonary artery: Patient characteristics and imaging associations on multidetector computed tomography angiography

“…As per literature, 80%-90% of the patients with adult ALCAPA may experience sudden cardiac death by 35-40 years of age, more common in male athletes due to physical exertion. 1,[16][17][18] However, the mean age in our series is quite high (highest 54 years), which could be attributable to the right dominant system and extensive collateral network.…”

Transcatheter occlusion of an anomalous origin of left coronary artery from pulmonary artery in an adult as an alternative to surgery

“…3 Histological ischaemic changes indicating coronary steal persist despite coronary bypass due to slow-flow in the venous grafts. 5 Saphenous venous grafts are unlikely to retain adequate patency beyond few years. 3 Even though patient refusal for surgery forced this intervention, the resolution of ischaemia after left coronary occlusion showed the utility of this strategy as a surgical alternative.…”