Case report of asymptomatic very late presentation of ALCAPA syndrome: review of the literature since pathophysiology until treatment

Abstract

Background: Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is an unusual congenital heart defect which affects approximately 1 in 300 000 live births and accounts for 0.5% of all congenital heart disease. Without surgical intervention, most patients die in infancy (nearly 90%).

Case summary: We present a rare case of an asymptomatic 67-year-old female. Transthoracic echocardiography demonstrated a dilated right coronary artery (RCA) and multiple collaterals. ALCAPA was confirmed by multidetector computed tomography. The left main artery was seen originating from the pulmonary artery and well-developed collaterals were visualized between the RCA and LCA. No areas of myocardial infarction were identified on cardiac magnetic resonance. Stress studies showed no inducible ischaemia.

Discussion: Our clinical case of an ALCAPA patient who survived and remained asymptomatic to their late 60's, highlights the importance of well-collateralized and pressurized coronary system to maintain adequate myocardial perfusion. Physicians should be aware of this congenital anomaly as appropriate early diagnosis is crucial to prevent irreversible myocardial damage, acute ischaemia, and arrhythmias, and can improve patient outcomes. Surgical treatment is suggested irrespective of symptomatology or the presence of inducible myocardial ischaemia.

Keywords: Adult-type ALCAPA; Cardiac multidetector computed tomography; Case report; Congenital coronary artery anomaly; Epicardial collaterals; No inducible myocardial ischaemia.

Figure 1

(A–C) Cardiac magnetic resonance. (A) Sagittal short axis showing normal origin of the right coronary artery. (B) Axial view characterized the origin of the left main artery in the pulmonary artery. (C) Axial view displaying prominent coronary arteries, collaterals, and dilated left ventricle. Ao, aorta; LA, left atrium; LAD, left anterior descending; LCx, left circumflex; LMA, left main artery; LPA, left pulmonary artery; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RCA, right coronary artery; RV, right ventricle.

Figure 2:

(A–F): 64-multislice cardiac multidetector computed tomography. (A–C) 3D volume-rendered reconstruction of multidetector computed tomography images showing left coronary artery and right coronary artery systems connecting with well-developed collaterals. (A) Anterior view. (B) Antero-lateral volume-rendered. (C) Lateral volume-rendered. (D) Sagittal oblique confirming the connection of the left main artery with the pulmonary artery. (E) Sagittal view showing the origin of the right coronary artery. (F) Sagittal oblique showing huge epicardial collaterals. Ao, aorta; Co, collaterals; IVC, inferior vena cava; LAD, left anterior descending; LMA, left main artery; LPA, Left pulmonary artery; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RCA, right coronary artery; SVC, superior vein cava.