Left circumflex coronary artery from the pulmonary artery in scimitar syndrome

Abstract

BackgroundScimitar syndrome is a rare combination of cardiopulmonary abnormalities found in 1–3 per 1000 live births. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is only found in 1 in 250–400 congenital heart disease patients.ObjectiveWe aimed to investigate the incidence of left circumflex ALCAPA within our referral center’s cohort of scimitar syndrome patients.Materials and methodsA review of medical records, cardiac imaging and operative notes from all patients diagnosed with scimitar syndrome at our center between 1992 and 2016 was undertaken and all imaging reviewed.ResultsFifty-four patients with scimitar syndrome and imaging were identified. Of these, 3 patients (1 male and 2 female) with ALCAPA were identified, representing an incidence of 5.5% (95% confidence interval [CI] 0–11.67%). In all three cases, the anomalous coronary arising from the pulmonary artery was the left circumflex coronary artery (LCx) and the point of origin was close to the pulmonary arterial bifurcation.ConclusionWe hypothesize that the prevalence of LCx-ALCAPA, in the setting of scimitar syndrome, may be greater than previously thought. We suggest that any patient with scimitar syndrome, especially with evidence of ischaemia, should be investigated for ALCAPA. Given its noninvasive nature and simultaneous imaging of the lungs, we suggest that cardiovascular CT is the most appropriate first-line investigation for these patients.

Diagnostic detection with cardiac tomography and resonance of extremely rare coronary anomaly: A case report and review of literature

“…To the best of our knowledge, the presented case is the first one in scientific literature that describes this anomaly association. Moreover, of 12 similar cases of CAAs reported in literature, only 2 were detected and confirmed with C-MRI or C-CT, the others were diagnosed with CX-A or during surgical treatment[26-37]. Our patient was a healthy child with no previous serious or recurrent or unusual cardiac symptoms.…”

Anomalous Origin of Left Coronary Artery From Right Pulmonary Artery in Association With Scimitar Syndrome

“…To the best of the present authors’ knowledge, this is the first case to describe an anomalous LMCA origin from the RPA in association with scimitar syndrome. Few other reports have described isolated cases of scimitar syndrome and anomalous left circumflex coronary artery from the MPA ( 3 , 4 ).…”