title: Resolution of pathologic Q wave, left ventricular dysfunction and mitral regurgitation after dual coronary repair of the anomalous origin of the left coronary artery from the pulmonary artery.
abstract: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect that usually presents as dilated cardiomyopathy in infancy. From 1984 to 2005, 13 (five males and eight females, 0.13%) out of 9,950 patients with congenital heart disease were identified as ALCAPA at our institute. Corrective surgery was performed at a median age of 9 months (range: 2 months to 5 years). Eleven patients underwent direct reimplantation of the left coronary artery (LCA) to the aorta, while two received extrapulmonary baffling. The overall survival rate was 92%. Only one patient died 5 months after reimplantation of the LCA due to acute myocardial ischaemia. Left ventricular ejection fraction (LVEF) was significantly lower in the eight (62%) patients operated during infancy than in those operated beyond 5 months (median: 35% vs. 75%). Left ventricle function was normalised in 11 patients (85%). Among the eight patients without concomitant mitral annuloplasty, mitral regurgitation (MR) improved to a mild or trivial degree in six patients and remained at the pre-operative level in two patients. Pathologic Q wave was noted in 11 patients, which eventually regressed in all except two cases. The median interval of recovery was 16 days, 6 months and 24 months for MR, LVEF and electrocardiogram (ECG) changes, respectively. In conclusion, ALCAPA is also a rare disease in Asian countries, such as Taiwan. The subsequent recovery of MR, left ventricular (LV) function and even pathologic Q wave can be expected after dual coronary repair, regardless of the age at repair.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Hsin-Hui
authors.initials: HH
authors.lastname: Chiu
authors.forename: Jou-Kou
authors.initials: JK
authors.lastname: Wang
authors.forename: Chun-An
authors.initials: CA
authors.lastname: Chen
authors.forename: Sheunn-Nan
authors.initials: SN
authors.lastname: Chiu
authors.forename: Ming-Tai
authors.initials: MT
authors.lastname: Lin
authors.forename: Hung-Chi
authors.initials: HC
authors.lastname: Lue
authors.forename: Chung-I
authors.initials: CI
authors.lastname: Chang
authors.forename: Ing-Su
authors.initials: IS
authors.lastname: Chiu
authors.forename: Mei-Hwan
authors.initials: MH
authors.lastname: Wu
mesh_terms: D002648:Child; D002675:Child, Preschool; D003331:Coronary Vessels / Q000033:anatomy & histology* / Q000601:surgery*; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008133:Long QT Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D008297:Male; D008944:Mitral Valve Insufficiency / Q000209:etiology*; D011183:Postoperative Complications; D011300:Preoperative Care; D011651:Pulmonary Artery / Q000002:abnormalities*; D012720:Severity of Illness Index; D018487:Ventricular Dysfunction, Left / Q000209:etiology / Q000503:physiopathology / Q000601:surgery