Long-term myocardial scarring after operation for anomalous left coronary artery from the pulmonary artery

Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a very rare disease. Accordingly, the incidence and distribution of myocardial scarring in long-term follow-up of patients after successful surgery of ALCAPA is unknown. Therefore, the aim of this study was to describe the distribution of left ventricular (LV) myocardial scarring by late gadolinium enhancement magnetic resonance in these patients.

Methods: Fourteen consecutive patients over 12 years of age with ALCAPA without any further cardiac abnormalities, and after corrective surgery at our center, participated prospectively in this study. All patients underwent cardiac magnetic resonance including LV function analysis and late gadolinium enhancement magnetic resonance. Of these 14 patients, 12 patients also underwent cardiopulmonary exercise testing.

Results: Four patients had no evidence of myocardial scarring. The median extent of the myocardial scar of all patients by late gadolinium enhancement magnetic resonance, expressed as a percentage of the total LV myocardial volume, was only 2% (range, 0% to 11%). Median LV ejection fraction was 0.55 (range, 0.31 to 0.73). Median LV end-diastolic volume indexed to body surface area was 84 mL/m(2) (39 to 120 mL/m(2)). Median peak oxygen uptake during cardiopulmonary exercise was 24 mL · kg(-1) · minute(-1) (range, 19 to 51 mL/kg/minute).

Conclusions: We conclude that despite often severely compromised LV function and evidence of scarring before corrective surgery of patients with ALCAPA, in long-term follow-up scar tissue is relatively scarce.