Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) diagnosed in children and adolescents

Abstract

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death. By the development or lack of coronary collateral, it can be classified as infantile or adult type. However, even with the compensatory mechanism in adult patients, there is an estimated 80 to 90% incidence of sudden death at the mean age of 35 years. Methods: We enrolled 9 patients with ALCAPA within the age group 5 to 16 years. Results: Only one patient developed symptoms (apsychia), whereas other patients were asymptomatic, and there was no evident left ventricular dysfunction found in any of the cases. Conclusion: With the development of imaging techniques, asymptomatic adult-type ALCAPA patients could be identified and diagnosed in childhood or adolescence. As a potential cause of sudden death, ALCAPA should be surgically repaired soon after the diagnosis.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in adults: Collateral circulation does not preclude direct reimplantation

“…The clinical presentation and classification will vary according to the presence of collateral circulation, generally divided into infantile and adult types. The infantile type does not have enough collateral circulation, leading to myocardial ischemia early in infancy 2–4 . Children are prone to death without early surgical repair within a few months after birth 1,2,5,6 .…”

“…Late‐diagnosed cases happen to be due to symptoms such as angina, palpitations, dyspnea,or fatigue 3,9 . Risk of sudden cardiac death can be as high as 80%–90% at the fourth decade of life 2,4,6 …”

Surgical treatment for anomalous origin of the right coronary artery from the pulmonary artery: a case report with five-year follow-up

“…There is consensus about early intervention in patients with ARCAPA to prevent later complications such as cardiac dysfunction and myocardial ischemia. The aims of treatment are to eliminate the “coronary steal” and establish dual coronary system originating from the aorta [ 8 ]. Three surgical strategies are commonly applied which are simple RCA ligation, RCA ligation with CABG and RCA reimplantation onto aorta.…”

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

“…Several studies reported that simultaneous MV repair had no effect on the normalization of LV function or other surgical outcomes. They thought that with coronary revascularization, the recovery of LV dilatation and papillary dyskinesia could improve valve function [ 12 - 13 , 15 - 16 , 20 - 23 ]. Some recommended that simultaneous mitral annuloplasty should be performed at the time of ALCAPA repair in all patients with mild to severe MR [ 17 , 24 ].…”

“…Surgical repair was performed in a form of coronary reimplantation in 26 (90%) patients with MV repair in one of them and Takeuchi repair in three patients (10%).ICU stay was eight (6-17) days and hospital stay was 15(12)(13)(14)(15)(16)(17)(18)(19)(20)(21) days. Duration of positive pressure ventilation (PPV) was two (1-4) days.…”