Outcomes of anomalous left coronary artery from pulmonary artery repair: beyond normal function
Antonio G Cabrera1, Diane W Chen2, Ricardo H Pignatelli1
1Section of Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Insights
Surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) yields excellent long-term outcomes. Standard measures like ejection fraction may not fully capture persistent myocardial dysfunction after ALCAPA repair.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare congenital heart defect with high mortality if untreated.
- Surgical correction is essential for survival and long-term health in affected infants and children.
Purpose of the Study:
- To evaluate the long-term clinical outcomes and ventricular function following surgical repair of ALCAPA.
- To assess changes in myocardial function using standard echocardiography and speckle tracking in patients post-ALCAPA repair.
Main Methods:
- Retrospective review of 34 patients undergoing ALCAPA repair between 1996 and 2011.
- Assessment of ventricular function included ejection fraction, shortening fraction, LV end-diastolic dimension, and mitral regurgitation severity.
- Speckle tracking echocardiography was used to detect subclinical myocardial dysfunction.
Main Results:
- No early mortality; 5 reoperations (heart transplant, mitral valve replacement, CABG, VSD closure) and 1 late death occurred in 34 patients over median 6-year follow-up.
- Significant improvements in ejection fraction (21% to 60%), shortening fraction (25% to 38%), LV dimensions, and mitral regurgitation were observed.
- Despite normal ejection fraction and shortening fraction in all patients at follow-up, 79% showed impaired longitudinal and circumferential strain on speckle tracking.
Conclusions:
- Surgical repair of ALCAPA achieves excellent long-term outcomes with low reintervention rates and no need for mechanical support.
- Standard echocardiographic parameters (EF, SF) may not fully reflect myocardial recovery, highlighting the utility of advanced techniques like speckle tracking for assessing subclinical dysfunction.
Background:
Anomalous left coronary artery from pulmonary artery (ALCAPA) is associated with high mortality if left uncorrected. We describe long-term outcomes after surgical repair and ventricular function changes on long-term follow-up.
Methods:
A retrospective review of patients who had ALCAPA repair from January 1996 to December 2011 was completed. Ventricular function was assessed by shortening fraction and ejection fraction, left ventricular end-diastolic dimension, and severity of mitral regurgitation. Speckle tracking echocardiography served as a marker of early myocardial dysfunction.
Results:
In total, 34 patients underwent ALCAPA repair at median age of 5 months (range, 3 days to 39 years). Surgical interventions included coronary translocation in 31 patients (91%), Takeuchi repair in 2 (6%), and coronary ligation in 1 patient (3%). Concomitant mitral valve repair was performed in 5 patients (15%). No patient required mechanical circulatory support postoperatively. There was no early mortality. At median follow-up of 6 years (range, 1 month to 14 years), there were 5 reoperations: 2 heart transplants (6%), 1 mitral valve replacement (3%), 1 coronary artery bypass graft (3%), and 1 ventricular septal defect closure (3%); there was 1 death 2 years after surgery. Ejection fraction improved from 21% ± 6% to 60% ± 7% (p = 0.008), mean shortening fraction from 25% ± 14% to 38% ± 5% (p = 0.01), mean left ventricular end-diastolic dimension Z-score from 6.0 ± 3.8 to 0.9 ± 0.7 (p < 0.001), and number of patients with moderate-severe mitral regurgitation from 44% (15 of 34) to 5% (1 of 21). All patients had normal ejection fraction and shortening fraction at last follow-up. Speckle tracking echocardiography showed decreased global longitudinal (p = 0.01) and circumferential strain (p = 0.03) for 11 of 14 patients (79%).
Conclusions:
Excellent outcomes are achieved with repair of ALCAPA without mechanical circulatory support and with low reintervention rates. Normal ejection fraction and shortening fraction do not accurately convey myocardial dysfunction in these patients.
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