Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery.
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 (r(2) = 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.
Michielon
Guido
G
Department of Medico-Chirurgico di Cardiochirurgia e, DMCCP, Ospedale Pediatrico Bambino Gesù, Rome, Italy. guido.michielon@tin.it
Di Carlo
Duccio
D
Brancaccio
Gianluca
G
Guccione
Paolo
P
Mazzera
Ennio
E
Toscano
Alessandra
A
Di Donato
Roberto Michele
RM
D000206:Actuarial Analysis; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality; D002675:Child, Preschool; D015331:Cohort Studies; D003330:Coronary Vessel Anomalies / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016014:Linear Models; D008297:Male; D011336:Probability; D011651:Pulmonary Artery / Q000002:abnormalities*; D020127:Recovery of Function; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D016277:Ventricular Function, Left / Q000502:physiology*