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Table of Contents
Year : 2022  |  Volume : 12  |  Issue : 3  |  Page : 136-138

Ostial stenosis of anomalous origin of the left coronary artery from the pulmonary artery in an adult: Lives from constraints and dies from freedom

Department of Cardiology, Yashoda Hospitals, Hyderabad, Telangana, India

Date of Submission29-Jun-2021
Date of Decision02-Sep-2021
Date of Acceptance06-Sep-2021
Date of Web Publication14-Sep-2022

Correspondence Address:
Dr. Pankaj Jariwala
Department of Cardiology, Yashoda Hospitals, Somajiguda, Raj Bhavan Road, Hyderabad - 500 082, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jicc.jicc_40_21

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An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital anomaly, the enlarged right coronary artery supplies the left ventricle through retrograde collaterals before preferentially directing into the lower-pressure pulmonary artery system, resulting in coronary steal. The infant type is characterized by the absence of collateral vessels, and global myocardial ischemia is a major cause of death in infancy. If not treated, up to 90% of them will die during the 1st year of life. Myocardial ischemia or infarction left ventricular (LV) dysfunction with or without mitral regurgitation, life-threatening arrhythmias, or sudden cardiac death may all be manifestations of symptomatic adult-type ALCAPA. In those patients who have survived to adulthood without surgery, pathophysiological mechanisms enabling adequate LV perfusion, such as a large number of well-formed functioning collaterals, and in a few cases, the presence of ostial stenosis of the left coronary artery (LCA), provide selective survival advantage. We review the correlation between clinical presentation, pathophysiological findings, and angiographic features of ostial stenosis of LCA in the setting of adult-type ALCAPA.

Keywords: Anomalous origin of the coronary artery from a pulmonary artery, cardiac pathology, congenital heart disease, coronary angiography

How to cite this article:
Jariwala P, Jadhav KP. Ostial stenosis of anomalous origin of the left coronary artery from the pulmonary artery in an adult: Lives from constraints and dies from freedom. J Indian coll cardiol 2022;12:136-8

How to cite this URL:
Jariwala P, Jadhav KP. Ostial stenosis of anomalous origin of the left coronary artery from the pulmonary artery in an adult: Lives from constraints and dies from freedom. J Indian coll cardiol [serial online] 2022 [cited 2022 Oct 1];12:136-8. Available from: https://www.joicc.org/text.asp?2022/12/3/136/356065

  Introduction Top

Art lives from constraints and dies from freedom.

-Leonardo Da Vinci, c. 1480

Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery anomaly that usually manifests in early childhood. Most of patients with ALCAPA syndrome succumb during the 1st year of life of ischemic cardiomyopathy and endocardial fibrosis caused by decreased oxygen supply in the left coronary artery (LCA) territory if they are not operated on. It has been proposed that even despite intervention, 15% of patients may live to adulthood, but 90% of these die unexpectedly at the average age of 35 years.[1]

The clinical presentation of the ALCAPA syndrome in late adulthood is highly uncommon. When compared to the infant-type ALCAPA, adult-type ALCAPA has distinct features, such as milder symptoms and various compensatory anatomic alterations.[2] However, little is known about the pathological changes, the clinical course of ALCAPA in adults, and sudden death. Before terminating into the main pulmonary artery (PA), we identify a case of adult ALCAPA with ostial stenosis of the LCA, a less described finding that may postpone the clinical manifestation of the disease.

  Case Report Top

A 28-years-old male presented with complaints of chest pain on exertion, Canadian Cardiovascular Society Class II for 2-months. A soft continuous murmur was discovered during examination in the parasternal region. Electrocardiography demonstrated left ventricular (LV) hypertrophy with inferolateral ST-segment depression. Echocardiography revealed global hypokinesia, mild LV dysfunction with mild mitral regurgitation.

During coronary angiography, LCA could not engage but to our surprise and because of the poor angiographic visualization and the suspicion of ALCAPA transfemoral aortic root angiography displayed a dilated, and dominant right coronary artery (RCA) that visualized the entire LCA retrogradely through multiple dense extensive networks of collaterals. The delayed phase demonstrated an ectatic left anterior descending artery with significant stenosis of the ostial segment, just before draining into the main PA and its branches, right and left pulmonary arteries [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. As the diagnosis of ALCAPA was confirmed by conventional angiography, he was referred to the department of cardiothoracic surgery for the establishment of a dual coronary artery system.
Figure 1: (a-d) Aortic root angiography indicating the absence of origin of the LCA from the left coronary sinus of the aorta (AO) (Panel A, B). The enlarged and tortuous RCA (Panel A, B, C) in the delayed phase revealed the opacification of the main PA and its branches, RPA and LPA (Panel D) through the dilated and tortuous LCA (solid white arrows; Panel A, B, C). The diagnostic feature involves a large network of collaterals emerging from the RCA (black arrow; Panel B), connecting the LCA to the main PA, a steal phenomenon. The peculiar feature of the case was the presence of discrete significant stenosis of the ostial segment of the LCA before its connection to the main PA (Dashed black arrow; Panel B). RCA: Right coronary artery, PA: Pulmonary artery, RPA: Right PA, LPA: Left PA, LCA: Left coronary artery

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  Discussion Top

ALCAPA, also called as Bland-White-Garland syndrome occurs once per 300,000 live births usually as an isolated cardiac lesion. ALCAPA is often reported in childhood as early as infancy as they present with congestive heart failure. Up to 90% of children with ALCAPA will die before surgical intervention during the 1st year of life.[3],[4]

The diagnosis of ALCAPA in living adults against autopsy series is thought to be exceedingly rare, with only a few cases published in the literature.[1],[5],[6] Yau et al. reviewed 151 adult cases of ALCAPA published in the literature and the mean observed age was 41 years. 66% of those who presented had symptoms of angina, dyspnoea, palpitations, or fatigue. 17% had ventricular arrhythmia, syncope, or died suddenly. A small percentage (14%) were asymptomatic. The onset of clinical features is linked to coronary reverse flow to the PA, which is dependent on the postnatal gradual decline in the pulmonary vascular resistance and closure of the ductus arteriosus after 2-months.[7]

The development of ischemia, on the other hand, promotes the creation of further collaterals between the RCA and the LCA. It may be due to major collaterals between both the right and left coronary arteries supplying enough oxygenated blood to the myocardium, but pulmonary hypertension played a role as well.[8] Because blood is diverted from the RCA to the LCA through collateral circulation, and then into the PA, pulmonary hypertension would develop as a result of volume overload caused by the left-to-right shunt. Pulmonary hypertension can help improve sufficient myocardial perfusion by reducing the shunt from LCA to PA.[9]

Extensive interarterial collateral vessels between both the RCA and the LCA, RCA dominance, limited coronary steal from the PA like the development of ostial stenosis of LCA, and establishment of systemic blood supply to the LCA are variables in infancy that facilitate survival to adulthood as described in [Table 1].[4]
Table 1: Characteristics of adult-type anomalous origin of the left coronary artery from the pulmonary artery

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As illustrated in our case, adult-type have a narrowing of the LCA ostium, which acts as a protective mechanism against myocardial ischemia by reducing the steal effect and enhancing myocardial perfusion pressure similar to the development of PA hypertension as discussed.[10] Stenosis likely provided a protective effect on reducing the coronary-PA shunt through infancy.[11] Adults have been diagnosed with ALCAPA, however, occasionally the development of ostial LCA stenosis, which limits coronary steal and raises the pressure of myocardial perfusion, contributing to the delayed clinical presentation, as in our case.[12] The exact etiology of the ostial stenosis is unknown. However, Hong et al. observed interstitial, perivascular patchy fibrosis that was more prevalent in the subendocardial area in pig models.[13] Exaggerated subendocardial fibrosis can contribute to the development of ostial stenosis at the site of increased shear stress.

With advanced diagnostic imaging, several asymptomatic or slightly symptomatic adult ALCAPA patients may be discovered. Despite the surgical correction in this demographic, the risk of sudden death tends to decrease after the age of 50 years in adult patients due to the development of ostial stenosis as in our case. In these rare patients, who often have no or mild symptoms, the risk of surgical correction outweighs the potential advantage, but surgical intervention is recommended in the vast majority of cases of anomalous origin of coronary arteries from the PA to mitigate worsening ischemia, arrhythmias, or sudden death.[2],[14]

To make a final decision, patients with inducible ischemia should be given surgical repair, which is associated with symptomatic and likely prognostic advantage.[1]

  Conclusion Top

The current case demonstrates that ALCAPA syndrome can go largely unnoticed. The asymptomatic course and the patient's survival to adulthood can be due to a systemic collateral blood supply to the coronaries, although this is seldom due to the existence of ostial stenosis of the LCA. Since no randomized clinical trials are yet present that would make it possible to determine the long-term results of these pathological observations in the management of adult ALCAPA, collecting systematic explanations of the evidence in each case may expose the real significance of this unusual entity and its optimal care.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We thank Mr. Naveen Rao, Mr. Srinivas Rao, Mr. Bhaskar, and Mr. Ramulu for their technical assistance.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Boutsikou M, Shore D, Li W, Rubens M, Pijuan A, Gatzoulis MA, et al. Anomalous left coronary artery from the pulmonary artery (ALCAPA) diagnosed in adulthood: Varied clinical presentation, therapeutic approach and outcome. Int J Cardiol 2018;261:49-53.  Back to cited text no. 1
Lotman EM, Karu K, Mikkel M, Elmet M. Late adult presentation of ALCAPA syndrome: Need for a new clinical classification? A case report and literature overview. Eur Heart J Case Rep 2020;4:1-5.  Back to cited text no. 2
Kubota H, Endo H, Ishii H, Tsuchiya H, Inaba Y, Terakawa K, et al. Adult ALCAPA: From histological picture to clinical features. J Cardiothorac Surg 2020;15:1-9.  Back to cited text no. 3
Peña E, Nguyen ET, Merchant N, Dennie C. ALCAPA syndrome: Not just a pediatric disease. Radiographics 2009;29:553-65.  Back to cited text no. 4
Zhuang T, Li-Gang F, Yong-Tai L, Shu-Yang Z. Anomalous origin of the left coronary artery from the pulmonary artery detected by echocardiography in an asymptomatic adult. Intern Med 2013;52:233-6.  Back to cited text no. 5
Kristensen T, Kofoed KF, Helqvist S, Helvind M, Søndergaard L. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: A case report. J Cardiothorac Surg 2008;3:33.  Back to cited text no. 6
Yau JM, Singh R, Halpern EJ, Fischman D. Anomalous origin of the left coronary artery from the pulmonary artery in adults: A comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman. Clin Cardiol 2011;34:204-10.  Back to cited text no. 7
Cambronero-Cortinas E, Moratalla-Haro P, González-García AE, Oliver-Ruiz JM. Case report of asymptomatic very late presentation of ALCAPA syndrome: Review of the literature since pathophysiology until treatment. Eur Heart J Case Rep 2020;4:1-5.  Back to cited text no. 8
Barbetakis N, Efstathiou A, Efstathiou N, Papagiannopoulou P, Soulountsi V, Fessatidis I. A long-term survivor of Bland-White-Garland syndrome with systemic collateral supply: A case report and review of the literature. BMC Surg 2005;5:23.  Back to cited text no. 9
Chattranukulchai P, Namchaisiri J, Tumkosit M, Puwanant S, Vorasettakarnkij Y, Srimahachota S, et al. Very late presentation of anomalous origin of the left coronary artery from the pulmonary artery: Case report. J Cardiothorac Surg 2018;13:70.  Back to cited text no. 10
Legendre A, Ou P, Bonnet D. Ostial stenosis of an anomalous left coronary artery from the pulmonary artery in a teenager. Pediatr Cardiol 2009;30:1194-5.  Back to cited text no. 11
Schwerzmann M, Salehian O, Elliot T, Merchant N, Siu SC, Webb GD. Images in cardiovascular medicine. Anomalous origin of the left coronary artery from the main pulmonary artery in adults: Coronary collateralization at its best. Circulation 2004;110:e511-3.  Back to cited text no. 12
Hong H, Aksenov S, Guan X, Fallon JT, Waters D, Chen C. Remodeling of small intramyocardial coronary arteries distal to a severe epicardial coronary artery stenosis. Arterioscler Thromb Vasc Biol 2002;22:2059-65.  Back to cited text no. 13
Vergara-Uzcategui CE, das Neves B, Salinas P, Fernández-Ortiz A, Núñez-Gil IJ. Anomalous origin of coronary arteries from pulmonary artery in adults: A case series. Eur Heart J Case Rep 2020;4:1-5.  Back to cited text no. 14


  [Figure 1]

  [Table 1]


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