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Guido Michielon
Duccio Di Carlo
Gianluca Brancaccio
Ennio Mazzera
Roberto Michele Di Donato
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Ann Thorac Surg 2003;76:581-588
© 2003 The Society of Thoracic Surgeons


Original article: cardiovascular

Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery

Guido Michielon, MDa*, Duccio Di Carlo, MDa, Gianluca Brancaccio, MDa, Paolo Guccione, MDb, Ennio Mazzera, MDa, Alessandra Toscano, MDb, Roberto Michele Di Donato, MDa

a Department of Medico-Chirurgico di Cardiochirurgia e, DMCCP, Ospedale Pediatrico Bambino Gesù, Roma, Italy
b Department of Cardiologia Pediatrica, DMCCP, Ospedale Pediatrico Bambino Gesù, Rome, Italy

Accepted for publication February 13, 2003.

* Address reprint requests to Dr Michielon, Dipartimento Medico-Chirurgico di Cardiologia Pediatrica, Ospedale Pediatrico Bambino Gesù, Piazza S. Onofrio 4, 00165 Rome, Italy
e-mail: guido.michielon{at}tin.it

BACKGROUND: This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery.

METHODS: Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months).

RESULTS: Fifteen-year actuarial survival was 92.9% ± 4.9% for coronary transfer, 40.0% ± 21.9% for subclavian interposition, and 89.9% ± 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% ± 7.4%). Left ventricular shortening fraction increased from 17.3% ± 6.3% before operation to 34.1% ± 4.6% at last follow-up (p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r2 = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% ± 5.2%) and best longitudinal shortening fraction recovery (36.4% ± 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors.

CONCLUSIONS: Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.




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