Department of Pediatric Cardiology, Deutsches Herzzentrum München, Munich, Germany.
Ann Thorac Surg. 2011 Nov;92(5):1761-5. doi: 10.1016/j.athoracsur.2011.06.021. Epub 2011 Oct 31.
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.
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.
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).
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.
动脉起源于肺动脉的左冠状动脉异常（ALCAPA）是一种非常罕见的疾病。因此，成功手术治疗后，患者长期随访中左心室心肌瘢痕的发生率和分布情况尚不清楚。因此，本研究旨在通过钆延迟增强磁共振（LGE-MRI）描述此类患者左心室（LV）心肌瘢痕的分布情况。
14 名年龄超过 12 岁的 ALCAPA 患者，无其他心脏异常，在我院行矫正手术后，前瞻性入组本研究。所有患者均行心脏磁共振检查，包括 LV 功能分析和 LGE-MRI。其中 12 名患者还进行了心肺运动试验。
4 名患者无心肌瘢痕证据。所有患者的 LGE-MRI 心肌瘢痕程度中位数（范围，0%至 11%）仅为 2%，表示为 LV 心肌总体积的百分比。LV 射血分数中位数为 0.55（范围，0.31 至 0.73）。LV 舒张末期容积指数中位数为 84 mL/m²（范围，39 至 120 mL/m²）。心肺运动试验时的峰值摄氧量中位数为 24 mL·kg⁻¹·min⁻¹（范围，19 至 51 mL/kg/min）。
尽管 ALCAPA 患者矫正手术前 LV 功能通常严重受损且存在瘢痕证据，但在长期随访中，瘢痕组织相对较少。
