Incidence and clinical relevance of primary congenital anomalies of the coronary arteries in children and adults

2012 ◽  
Vol 23 (3) ◽  
pp. 381-386 ◽  
Author(s):  
Giulia Tuo ◽  
Maurizio Marasini ◽  
Claudio Brunelli ◽  
Lucio Zannini ◽  
Manrico Balbi

AbstractObjectivesTo describe our experience in the management of coronary artery anomalies both in an adult and in a paediatric population and to compare the two groups for finding out differences in terms of angiographic incidence and treatment.Patients and methodsDatabases at the Department of Cardiology of San Martino Adult's Hospital and of Gaslini Children's Hospital were searched for all patients with a diagnosis of coronary artery anomaly who underwent coronary angiography between 1994 and 2006.ResultsCoronary anomalies were diagnosed in 76 (1%) adult patients. Anomalous left circumflex artery was the commonest coronary anomaly (25%). Anomalous left coronary artery from pulmonary artery and myocardial bridges were the only anomalies responsible for angina-like symptoms. No patients except the one with anomalous left coronary artery from pulmonary artery needed surgical intervention. In the paediatric population, we found 28 (0.9%) patients with coronary anomalies. Anomalous left coronary artery from pulmonary artery was the most common anomaly (48%) and always required emergency surgical treatment; in addition there were two patients with stenosis of the left main coronary artery.ConclusionCoronary artery anomalies may be associated with very acute, even life-threatening symptoms in children, whereas they are usually clinically silent and detected by accident on coronary angiography in adults. Recognition of coronary artery anomalies enables early treatment or close follow-up in children, whereas it could be useful in case of cardiac surgery in adults.

2019 ◽  
Vol 3 (4) ◽  
pp. 1-4
Author(s):  
Martin Hölscher ◽  
Walter Knirsch ◽  
Hitendu Dave ◽  
Barbara E U Burkhardt

Abstract Background Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare coronary abnormality. Although it exists usually as an isolated abnormality, ALCAPA has been described with aortic pathologies like coarctation or aortopulmonary window. Case summary An 18-day-old female was admitted to the paediatric intensive care unit because of a heart murmur and weak femoral pulses. A transthoracic two-dimensional echocardiography was performed and confirmed suspected diagnosis of aortic coarctation. In addition, a total retrograde perfusion of the left circumflex coronary artery (LCX) was found, without visible flow through the ostium of the left coronary artery (LCA) into the aorta. A coronary angiography was performed, showing a single right coronary artery with a normal right posterior descending artery (RPD). Supplied by collaterals from the RPD, the LCX was perfused retrogradely, passing by the lateral wall of the ascending aorta without flowing into it, but into the right pulmonary artery. At 23 days of age, surgery was performed with resection of the aortic coarctation and reimplantation of the LCA into the posterior aortic wall. Discussion This case demonstrates that coronary artery anomalies like ALCAPA may occur together with other cardiac malformations. Despite concomitant cardiac lesions, careful assessment of the coronary arteries is mandatory, including cardiac catheterization in case of doubt.


2013 ◽  
Vol 16 (4) ◽  
pp. 210 ◽  
Author(s):  
Sachin Talwar ◽  
Aandrei Jivendra Jha ◽  
Shiv Kumar Choudhary ◽  
Saurabh Kumar Gupta ◽  
Balram Airan

Between January 2002 and December 2012, five patients (4 female) underwent corrective surgery for anomalous left coronary artery from pulmonary artery (ALCAPA). They were older than 1 year (range, 3-56 years). One of the 2 patients younger than 10 years had presented with congestive heart failure, and the other had experienced repeated episodes of lower respiratory tract infection since childhood. Of the remaining 3 adult patients, 2 had experienced angina with effort, and 1 patient had had repeated respiratory tract infections since childhood, with mild dyspnea on effort of New York Heart Association (NYHA) class II. Three patients had the anomalous left coronary artery implanted directly into the ascending aorta via coronary-button transfer, and 2 patients underwent coronary artery bypass with obliteration of the left main ostium. Two patients underwent concomitant mitral valve repair procedures, and 1 patient underwent direct closure of a perimembranous ventricular septal defect. Four patients survived the surgery, and 1 patient died because of a persistently low cardiac output. Follow-up times ranged from 3 months to 4 years. All survivors are in NYHA class I and have left ventricular ejection fractions of 45% to 60%, with moderate (n = 1), mild (n = 1), or no (n = 2) mitral insufficiency. We conclude that a few naturally selected patients with ALCAPA do survive beyond infancy and can undergo establishment of 2 coronary systems with satisfactory results.


2020 ◽  
Vol 4 (3) ◽  
pp. 1-5
Author(s):  
Madelien V Regeer ◽  
Olga Bondarenko ◽  
Katja Zeppenfeld ◽  
Anastasia D Egorova

Abstract Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital disorder resulting in ischaemia and myocardial infarction which can act as a potential substrate for life-threatening arrhythmias and sudden cardiac death. Case summary A 19-year-old man was admitted to the hospital after successful resuscitation from an out-of-hospital cardiac arrest (OHCA) due to ventricular fibrillation occurring during jogging. In the diagnostic work-up of the OHCA, computed tomography identified an ALCAPA. The patient was referred to our tertiary hospital for surgical correction. Direct reimplantation of the left coronary artery in the aorta was performed. During follow-up, 24-h electrocardiogram revealed short episodes of non-sustained ventricular tachycardia (VT). The magnetic resonance imaging at initial admission showed focal wall thinning and transmural late gadolinium enhancement consistent with a previous anterolateral myocardial infarction. Therefore, the aetiology of the OHCA could be due to a scar-related mechanism and not necessarily due to a reversible cause and an implantable cardioverter-defibrillator (ICD) was considered indicated. Given the young age and the lower complication rates, a subcutaneous device was preferred over a transvenous ICD. However, as a subcutaneous ICD (S-ICD) lacks the possibility of anti-tachycardia pacing, programmed electrical stimulation (PES) was performed to test for inducibility of monomorphic, re-entrant VT. After a negative PES, an S-ICD was implanted. Discussion ALCAPA is a potential cause of OHCA in young patients. Some of these patients keep an irreversible substrate for ventricular arrhythmias despite full surgical revascularization and might be candidates for (subcutaneous) ICD implantation.


2012 ◽  
Vol 16 (3) ◽  
pp. 100-101 ◽  
Author(s):  
Farzanah Ismail

Anomalous left coronary artery originating from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly that presents with myocardial ischaemia or infarction and/or cardiac failure in infants. It is associated with a mortality rate of 90% within the first year of life. Surgical correction to re-establish a two-coronary artery perfusion system is the treatment of choice, once patients are medically stable.


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