Ismail Muna, Jijeh Abdulraouf, Alhuwaymil Rathath M, Alahmari Raneem, Alshahrani Rawan, Almutairi Reem, Habshan Fahad, Shaath Ghassan A
Cardiac Sciences Department, Pediatric Cardiology, King Abdulaziz Cardiac Center, Ministry of the National Guard - Health Affairs, Riyadh, SAU.
Cardiac Sciences Department, Pediatric Cardiac Intensive Care Unit, Ministry of National Guard - Health Affairs, Riyadh, SAU.
Cureus. 2020 Dec 1;12(12):e11829. doi: 10.7759/cureus.11829.
Background The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery anomaly. It induces left ventricular (LV) dysfunction and mitral valve regurgitation (MR). If untreated, survival beyond infancy is rare. The aim of our study was to analyze the outcome in children with ALCAPA after cardiac surgery. Methods We retrospectively reviewed all patients who were diagnosed at our institution with ALCAPA and underwent surgical repair from 1999 to the end of 2018 (for 20 years). We followed LV dimensions, function, the progress of MV regurgitation, and the somatic growth of children after surgical repair. Results Twenty-nine patients underwent ALCAPA repair while 15 (52%) patients were male. The median age at surgical repair was 5.3 (IQR: 3.8-7.4) months and the mean weight was 5.5±2 kg. Surgical repair was performed in form of coronary reimplantation in 26 (90%) patients and Takeuchi repair in three (10%) patients. Intensive care unit (ICU) stay was eight (IQR: 6-17) days and hospital stay was 15 (IQR: 12-21) days. The follow-up duration was 5±3.6 years. Echocardiographic parameters started to improve by six weeks after the repair, and they normalized by one year. At the time of surgery ejection fraction (EF) was 34±17%, fractional shortening (FS) was 15±10%, and LV inner diameter in diastole (LVIDD) z score was 5.7±2.8. These parameters improved by one year after surgery to 66±7%, 34±6%, and 0±1.3, respectively. However, somatic growth started to improve six months after surgical repair. MR was moderate to severe in seven (24%) patients at the time of surgery and regressed to no more moderate nor severe MR at the last follow-up. None of the 29 patients died. Conclusions LV systolic function and dimensions start to improve by six weeks after surgery and reach normal values by one year. MR regresses without intervention in correspondence with the regression of LV dimensional parameters. The somatic growth of children improves six months after repair.
背景 左冠状动脉起源于肺动脉（ALCAPA）是一种罕见的先天性冠状动脉异常。它会导致左心室（LV）功能障碍和二尖瓣反流（MR）。若不治疗，婴儿期后的存活率很低。我们研究的目的是分析接受心脏手术后患有ALCAPA的儿童的治疗结果。方法 我们回顾性分析了1999年至2018年底（共20年）在我们机构被诊断为ALCAPA并接受手术修复的所有患者。我们跟踪了手术修复后儿童的左心室尺寸、功能、二尖瓣反流的进展以及身体生长情况。结果 29例患者接受了ALCAPA修复，其中15例（52%）为男性。手术修复时的中位年龄为5.3（四分位间距：3.8 - 7.4）个月，平均体重为5.5±2千克。26例（90%）患者以冠状动脉再植入的形式进行手术修复，3例（10%）患者进行了竹内修复。重症监护病房（ICU）住院时间为8（四分位间距：6 - 17）天，住院时间为15（四分位间距：12 - 21）天。随访时间为5±3.6年。超声心动图参数在修复后六周开始改善，并在一年后恢复正常。手术时射血分数（EF）为34±17%，缩短分数（FS）为15±10%，舒张期左心室内径（LVIDD）z评分为5.7±2.8。这些参数在手术后一年分别改善到66±7%、34±6%和0±1.3。然而，身体生长在手术修复后六个月开始改善。手术时7例（24%）患者的二尖瓣反流为中度至重度，在最后一次随访时退化为不再是中度或重度二尖瓣反流。29例患者均未死亡。结论 左心室收缩功能和尺寸在手术后六周开始改善，并在一年后达到正常值。二尖瓣反流在无干预的情况下随着左心室尺寸参数的消退而消退。儿童的身体生长在修复后六个月改善。
