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Year : 2018  |  Volume : 11  |  Issue : 6  |  Page : 571-572  

Commentary on: Anomalous origin of the left coronary artery from the main pulmonary artery – A case report

Department of Pediatric Cardiology, Puerta del Mar Universitary Hospital, Cadiz, Spain

Date of Web Publication15-Nov-2018

Correspondence Address:
Moises Rodriguez-Gonzalez
Department of Pediatric Cardiology, Puerta del Mar Universitary Hospital, 21 of Ana de Viya Avenue, Cadiz
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_51_18

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How to cite this article:
Rodriguez-Gonzalez M. Commentary on: Anomalous origin of the left coronary artery from the main pulmonary artery – A case report. Med J DY Patil Vidyapeeth 2018;11:571-2

How to cite this URL:
Rodriguez-Gonzalez M. Commentary on: Anomalous origin of the left coronary artery from the main pulmonary artery – A case report. Med J DY Patil Vidyapeeth [serial online] 2018 [cited 2019 Aug 25];11:571-2. Available from: http://www.mjdrdypv.org/text.asp?2018/11/6/571/245431

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare form of congenital heart disease (CHD) occurring in about 1:300,000 live births.[1],[2] The importance of ALCAPA lies in three facts:first, it is the most prevalent coronary artery anomaly described in children (0.25%–0.5% of all CHD);[1],[2],[3] second, it is associated with high mortality (90%) if left uncorrected;[4] and third, excellent early (mortality 0%–16%) and late (survival rates 80%–100% at 10 years) outcomes can be obtained with the contemporary repair of ALCAPA in the majority of patients.[1],[2],[3],[4] Therefore, the early diagnosis and treatment of ALCAPA can overcome its otherwise poor prognosis, and all physicians that take care of children must acquire skills to identify this CHD.

All the clinical and complementary test features of ALCAPA are secondary to the development of myocardial ischemia at the subsidiary territory of the left main coronary artery, the anterolateral aspect of the left ventricle.[1],[2],[3],[4],[5] The extent of myocardial necrosis of the left ventricle is determined by the balance between timing of closure of the ductus arteriosus, decrease in pulmonary vascular resistance after the early neonatal period, and most important, the development of intercoronary collaterals between the right and left coronary arteries large enough to maintain adequate perfusion of the left myocardium. Few weeks after birth pulmonary arterial pressure fall below diastolic pressure in the aorta and coronary system. In the absence of adequate collateralization, a left to right shunting occurs leading to a coronary steal phenomenon (the blood in the anomalous coronary artery reverses its flow to the lung instead of the myocardium), that results in severe myocardial infarction and left ventricular dilation/dysfunction and severe mitral regurgitation. When there is an adequate degree of collateral circulation development, the myocardial perfusion is enough to avoid ischemic episodes out of stress settings, and left ventricle function is conserved. However, chronic hypoperfusion causes subendocardial ischemia and later fibrosis/scar, the substrate for chronic mitral regurgitation and ventricular arrhythmias.

Clinical and electrocardiogram (ECG) features of ALCAPA falls in a bimodal presentation based on the development of an adequate inter coronary collateral net. The infant pattern (poor collateral development), is the presentation in up to 75%–85% of cases. The chief symptom is intermittent episodes of irritability (angina pain) elicited by only slight effort, such as feeding, followed by signs of poor peripheral perfusion. These features started within 2 months of birth and progressed until the development of cardiogenic shock. Necrotic Q waves and ST elevation in D1, aVL, and left precordial leads on an ECG are a highly characteristic finding that must lead us to make a prompt diagnosis.[1],[4] The older children/adult pattern (well-collateral development) is the presentation in up to 15%–25% of cases. Patients usually remained asymptomatic until adolescence or young adulthood. Such patients can have angina on exertion and are at risk of sudden death.[6] Furthermore, they can be incidentally discovered in the echocardiographic study of mitral regurgitation or dilated cardiomyopathy. Alternatively, they can present with signs of congestive heart failure similar to the infant pattern, caused by decompensation of already borderline cardiac function, secondary to acute infectious illness. Their baseline ECG usually shows ischemic changes only in cases of decompensation or during a stress test.[1],[3]

In experienced hands and with clinical and ECG suspicion of ALCAPA, color-flow Doppler echocardiography is currently the diagnostic method of choice.[1],[7],[8] The diagnosis of ALCAPA should be considered from different echocardiography signs: absence of a left coronary in the left aortic sinus, dilatation of the right coronary, continuous abnormal flow within the ventricular septum (intercoronary collateral circulation and differential diagnosis with multiple ventricular septal defects), retrograde flow into the left anterior descending artery, diastolic flow into the pulmonary artery (differential diagnosis with ductus arteriosus), globally dilated hypokinetic cardiomyopathy, hyperechogenic papillary muscles, and mitral regurgitation. Coronary angiography is the classical gold standard for confirming the diagnosis of ALCAPA, but advances in computed tomography might make this method a better alternative when the diagnosis is in doubt, moreover in critically ill patients.[1],[7],[8] Cardiac MRI is particularly useful in the postoperative follow-up. The combination of myocardial viability, stress perfusion, wall motion, and ventricular volume analysis makes it a powerful tool for predicting freedom from cardiac adverse events in patients at risk for coronary stenosis and myocardial ischemia.[1],[3],[7],[8]

The standard treatment options for establishing a two-coronary anatomy and physiology in ALCAPA cases are direct aortic reimplantation or creation of intrapulmonary baffle (Takeuchi procedure), when coronary translocation is not feasible.[1],[2],[5] Mitral valve regurgitation management is controversial and depends on the exact cause; the functional mitral regurgitation due to mitral annular dilation resolves completely after surgery and improvement of the left ventricular function. Nevertheless, the structural mitral regurgitation due to papillary muscle infarction is unlikely to resolve after surgery and these patients might need mitral valve repair at the time of the ALCAPA repair.[2],[5] Despite excellent left ventricle recovery and long-term survival rates after ALCAPA repair, follow-up complications such as persistent mitral regurgitation, late-onset congestive heart failure, and coronary arterial stenosis may necessitate reintervention.[3],[4] Therefore, long-term follow-up for these patients is warranted.

  References Top

Rodriguez-Gonzalez M, Tirado AM, Hosseinpour R, de Soto JS. Anomalous origin of the left coronary artery from the pulmonary artery: Diagnoses and surgical results in 12 pediatric patients. Tex Heart Inst J 2015;42:350-6.  Back to cited text no. 1
Neumann A, Sarikouch S, Bobylev D, Meschenmoser L, Breymann T, Westhoff-Bleck M, et al. Long-term results after repair of anomalous origin of left coronary artery from the pulmonary artery: Takeuchi repair versus coronary transfer. Eur J Cardiothorac Surg 2017;51:308-15.  Back to cited text no. 2
Schmitt B, Bauer S, Kutty S, Nordmeyer S, Nasseri B, Berger F, et al. Myocardial perfusion, scarring, and function in anomalous left coronary artery from the pulmonary artery syndrome: A long-term analysis using magnetic resonance imaging. Ann Thorac Surg 2014;98:1425-36.  Back to cited text no. 3
Cabrera AG, Chen DW, Pignatelli RH, Khan MS, Jeewa A, Mery CM, et al. Outcomes of anomalous left coronary artery from pulmonary artery repair: Beyond normal function. Ann Thorac Surg 2015;99:1342-7.  Back to cited text no. 4
Naimo PS, Fricke TA, d'Udekem Y, Cochrane AD, Bullock A, Robertson T, et al. Surgical intervention for anomalous origin of left coronary artery from the pulmonary artery in children: A Long-term follow-up. Ann Thorac Surg 2016;101:1842-8.  Back to cited text no. 5
Hill SF, Sheppard MN. A silent cause of sudden cardiac death especially in sport: Congenital coronary artery anomalies. Br J Sports Med 2014;48:1151-6.  Back to cited text no. 6
Goo HW. Coronary artery imaging in children. Korean J Radiol 2015;16:239-50.  Back to cited text no. 7
Attili A, Hensley AK, Jones FD, Grabham J, DiSessa TG. Echocardiography and coronary CT angiography imaging of variations in coronary anatomy and coronary abnormalities in athletic children: Detection of coronary abnormalities that create a risk for sudden death. Echocardiography 2013;30:225-33.  Back to cited text no. 8


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