Long-Term Outcome of the Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery (ALCAPA) in Children After Cardiac Surgery: A Single-Center Experience

Abstract

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.

The utility of speckle-tracking echocardiography in early and midterm follow-up after anomalous origin of the left coronary artery from the pulmonary artery repair

“…Improvement in LV function was apparent by 6 weeks and normalized by 1 year of age. [ 4 ] The largest and most contemporary cohort of ALCAPA was reported by Radman et al There were 170 patients who survived to discharge and the LV function normalized in 79% by 1 year and 97% by 3 years. [ 9 ] However, these studies used the motion mode (M-mode) echocardiography to assess LV function, and the trajectory of LV function improvement in the 1 st year after surgery was not reported.…”

“…[ 3 ] Published data suggest that the LV function returns to normal by 1 year after the surgical repair. [ 4 ] It is believed that chronic insufficiency results in a myocardium that is hypocontractile but whose viability is preserved – a so-called “hibernating myocardium” in those children whose LV function recovers after surgery. [ 5 ] Speckle-tracking echocardiography (STE) has the potential to predict improvement or deterioration of LV function earlier than global markers such as ejection fraction (EF).…”

Anomalous left coronary artery from pulmonary artery (ALCAPA) as a silent cause of mitral regurgitation in children

“…The current diagnostic method of choice is echocardiography [6]. The characteristic findings that can be seen are a dilated right coronary artery (RCA), retrograde Doppler flow from the left coronary artery (LCA) to pulmonary artery (PA), and a septal flow due to collaterals [9].…”

“…Due to the rarity of this disease, it often goes unnoticed. The most common diagnostic tool is echocardiography [6]. However, some cases may go undiagnosed, leading to complications later in life or during surgical correction of the mitral valve [7].…”