Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular
Guido Michielon1, Duccio Di Carlo, Gianluca Brancaccio
1Department of Medico-Chirurgico di Cardiochirurgia e, DMCCP, Ospedale Pediatrico Bambino Gesù, Rome, Italy. guido.michielon@tin.it
Insights
Early surgical repair of anomalous coronary artery origin from the pulmonary artery in infants improves left ventricular function and survival. Coronary transfer offers the best long-term outcomes, with most patients recovering normal mitral valve function.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiovascular Research
Background:
- Anomalous coronary artery origin from the pulmonary artery (ACoPA) is a rare congenital heart defect.
- Early diagnosis and surgical intervention are crucial for improving outcomes.
Purpose of the Study:
- To investigate the 15-year longitudinal correlation between surgical timing and left ventricular (LV) and mitral valve function after ACoPA repair.
- To evaluate the long-term survival and reoperation rates associated with different surgical techniques for ACoPA.
Main Methods:
- Retrospective analysis of 31 patients who underwent ACoPA repair between 1987 and 2002.
- Surgical techniques included subclavian interposition, intrapulmonary tunnel, and direct aortic reimplantation (coronary transfer).
- Longitudinal assessment of LV function (shortening fraction) and mitral valve function, with a mean follow-up of 77.2 months.
Main Results:
- Fifteen-year actuarial survival rates varied significantly by technique: coronary transfer (92.9%), intrapulmonary tunnel (89.9%), and subclavian interposition (40.0%).
- Coronary transfer demonstrated the best freedom from reoperation (92.3%).
- LV shortening fraction significantly improved post-surgery (17.3% to 34.1%), with younger infants (<6 months) showing the best recovery.
- 90.4% of survivors experienced major mitral valve function improvement within 1 year.
Conclusions:
- Surgical repair of ACoPA in younger, symptomatic infants leads to optimal LV function recovery.
- Coronary transfer is the preferred technique, offering superior long-term survival and reduced reoperation rates.
- Simultaneous mitral valve surgery is generally not warranted as most patients achieve normal function post-coronary repair.
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.
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