• Users Online: 178
  • Print this page
  • Email this page

Table of Contents
Year : 2019  |  Volume : 8  |  Issue : 3  |  Page : 84-86

Anomalous origin of the left coronary artery from the pulmonary artery-adult type with heart failure and ventricular arrhythmia presentation

Rajaie Cardiovascular Medical and Research Center, Rajaei Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran

Date of Submission23-Jul-2019
Date of Acceptance07-Aug-2019
Date of Web Publication08-Nov-2019

Correspondence Address:
Dr. Nasrin Panahifar
Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/rcm.rcm_18_19

Rights and Permissions

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital anomaly occurring in 1 of 300,000 births. As it has high mortality rate during the 1st year of life, adult type is very rare. Arrhythmias, heart failure, myocardial infarction, and sudden cardiac death are common presentations of ALCAPA syndrome in adults. Here, we report a 28-year-old male with malignant ventricular arrhythmia and sudden cardiac death. ALCAPA syndrome was diagnosed by coronary angiography and echocardiography; finally, cardiac surgery was performed with a good result.

Keywords: Anomalous origin of the left coronary artery from the pulmonary artery, arrhythmia, cardiac surgery, congenital anomaly, heart failure, sudden cardiac death

How to cite this article:
Saedi S, Ghavidel AA, Panahifar N. Anomalous origin of the left coronary artery from the pulmonary artery-adult type with heart failure and ventricular arrhythmia presentation. Res Cardiovasc Med 2019;8:84-6

How to cite this URL:
Saedi S, Ghavidel AA, Panahifar N. Anomalous origin of the left coronary artery from the pulmonary artery-adult type with heart failure and ventricular arrhythmia presentation. Res Cardiovasc Med [serial online] 2019 [cited 2022 Oct 4];8:84-6. Available from: https://www.rcvmonline.com/text.asp?2019/8/3/84/270587

  Introduction Top

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly occurring in 1 of 300,000 births.[1] There are two types of ALCAPA syndrome: the infant type and the adult type. Infants experience myocardial infarction and congestive heart failure, and approximately 90% die within the 1st year of life.[2] In a minority of cases (approximately 20%), sufficient myocardial collaterals develop from the normally arising right coronary artery (RCA), and survival into adulthood may occur;[3] in this situation, a left to right shunt into the pulmonary artery (PA) is created, leading to a coronary steal phenomenon that results in myocardial ischemia. Few patients survive past childhood without surgical repair, and up to 90% die suddenly at a mean of age of 35 years.[4] Among the reported adult cases, most of them, if not all, presented with evidence of irreversible impairment of cardiac function such as severe dilated cardiomyopathy, sudden cardiac death, acute myocardial infarction, malignant arrhythmias secondary to myocardial scar tissue, impaired left ventricle (LV) contractile function, and development of significant mitral regurgitation.[5] Here, we present a case of adult-type ALCAPA syndrome.

  Case Report Top

A 28-year-old male was referred to our center for further evaluation of dyspnea and retrosternal chest pain. Although he reported a history of increased dyspnea on exertion and fatigue since childhood, he remained without definite diagnosis until recent admission in other heart centers. He presented to another hospital's emergency unit with acute-onset palpitation and subsequent unconsciousness. The electrocardiogram revealed ventricular fibrillation. He was treated immediately with direct current cardioversion and amiodarone administration. After the successful termination of arrhythmia, cardiac evaluation including transthoracic echocardiography and coronary angiography was performed. Findings were strongly in favor of ALCAPA syndrome. Finally, he was referred to our department for complementary imaging and therapeutic surgery. He was not a smoker, diabetic, or hypertensive. He did not drink alcohol or use drugs. He was on furosemide and amiodarone for 3 months ago. On physical examination, he had cachectic appearance. His blood pressure was 105/60 mmHg and pulse rate was 102 beats/min. Cardiac auscultation showed S3 gallop at the site of LV apex with a Grade IV/VI continuous murmur at the left upper sternal border with radiation to back. The lungs were clear. The electrocardiography revealed sinus tachycardia, deep T wave inversion and also pathologic Q wave in v1 to v6, D1 and AVL. There was also Poor R wave progression (no obvious R wave in precordial until V4) [Figure 1].
Figure 1: Twelve leads ECG: Sinus tachycardia, poor R wave progression, Q and Twave Inversion in D1, AVL and V1 to V6

Click here to view

The chest radiograph showed severe cardiomegaly. Transthoracic echocardiography demonstrated severe LV enlargement with severe systolic dysfunction, no left ventricular hypertrophy (LVH), anterior wall dyskinesia, and moderate mitral regurgitation. There was dilated origin of the RCA from the right aortic sinus. The left circumflex artery (LCX) had common ostium with the RCA [Figure 2].
Figure 2: Transthoracic echocardiography: Right coronary artery originated from the right aortic sinus

Click here to view

No vessel originated from the left aortic sinus, and left anterior descending (LAD) artery was arising from the main PA (MPA) [Figure 3].
Figure 3: Transthoracic echocardiography: Left anterior descending artery originated from the pulmonary artery

Click here to view

Left heart catheterization indicated dilated LV with reduced systolic function. It also showed dilated and tortuous RCA originating from the right aortic sinus with a large number of collaterals to the LV. The LCX had common ostium with RCA, originating from the right aortic sinus [Figure 4].
Figure 4: (a) Aortic root injection in coronary angiography: Dilated and tortuous right coronary artery with great collaterals to the left coronary artery. (b) Left circumflex artery had common ostium with right coronary artery, originating from the right aortic sinus

Click here to view

LAD had retrograde filling via collaterals from the RCA and terminating into the PA. In cardiac computed tomography (CT), LAD was arising from the inferior surface of MPA; RCA and LCX were originating from the right aortic sinus with common ostium. LCX had moderate ostium stenosis with retroaortic course toward the LV. Finally, our patient underwent cardiac surgery by means of coronary artery bypass graft (left internal mammary artery [LIMA] to LAD graft) with a successful result.

  Discussion Top

ALCAPA occurs approximately in 0.25%–0.5% of children with congenital heart disease.[6] It is usually well tolerated at birth due to the elevated pressure in newborn pulmonary arteries, which provides antegrade perfusion of myocardial through the left coronary artery. As pulmonary vascular resistance falls, which results in a progressive retrograde filling of the left main coronary and pulmonary arteries through the collateral vessels from the RCA. This results in a steal of blood flow from the myocardium, leading to myocardial ischemia, heart failure, and mitral regurgitation.[2] While adult patients can be completely asymptomatic, they may present with angina, dyspnea, syncope, myocardial infarction, arrhythmia, or sudden cardiac death.[6] Although the diagnosis of ALCAPA is possible with noninvasive modalities, coronary angiography remains the gold standard method. The diagnosis is usually made when a dilated and tortuous RCA with collateral filling of the LCA system and variable degrees of shunting to the PA is shown; the sensitivity of an angiography may be limited in the diagnosis of an anomalous coronary artery due to its invasive nature.[7]

When coronary artery anomalies such as ALCAPA are diagnosed, urgent surgery is often indicated to prevent myocardial ischemia, malignant ventricular arrhythmias, and sudden cardiac death.[8] Today, surgical procedures are aimed at creating a two-coronary system via (1) a bypass graft mammary artery or saphenous vein in combination with ligation of the anomalous artery, (2) the Takeuchi-procedure where an intrapulmonary tunnel from the aortopulmonary window to the coronary artery is created, or (3) translocation of the left coronary artery from the pulmonary trunk to the aortic sinus.[9],[10] In our patient, bypass of the LAD with LIMA graft was done.

  Conclusion Top

ALCAPA is a rare congenital anomaly with high mortality in infants. Survival into adulthood may occur in a minority of cases. ALCAPA syndrome should be considered in young adults with cardiac symptom such as exertion intolerance, dyspnea, arrhythmia, and sudden cardiac death. Surgical repair which should be done immediately after the diagnosis of ALCAPA is confirmed. Timely diagnosis and surgical treatment confer good long-term prognosis.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Erdinc M, Hosgor K, Karahan O. Repair of anomalous origin of the left coronary artery arising from right pulmonary artery with rolled-conduit-extended reimplantation in an adult. J Card Surg 2011;26:604-7.  Back to cited text no. 1
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. 2
Taylor AJ, Virmani R. Coronary artery anomalies. In: Crawford MH, Di Marco JP, Paulus WJ, Aurigemma G, Bakris GL, Deanfield J, et al., editors. Cardiology. 3rd ed. Philadelphia: Elsevier; 2010. p. 231-41.  Back to cited text no. 3
Brooks HS. Two cases of an abnormal coronary artery of the heart arising from the pulmonary artery: With some remarks upon the effect of this anomaly in producing cirsoid dilatation of the vessels. J Anat Physiol 1885;20:26-9.  Back to cited text no. 4
Nielsen HB, Perko M, Aldershvile J, Saunamäki K. Cardiac arrest during exercise: Anomalous left coronary artery from the pulmonary trunk. Scand Cardiovasc J 1999;33:369-71.  Back to cited text no. 5
Fierens C, Budts W, Denef B, Van De Werf F. A 72 year old woman with ALCAPA. Heart 2000;83:E2.  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
Lange R, Vogt M, Hörer J, Cleuziou J, Menzel A, Holper K, et al. Long-term results of repair of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 2007;83:1463-71.  Back to cited text no. 8
Dahle G, Fiane AE, Lindberg HL. ALCAPA, a possible reason for mitral insufficiency and heart failure in young patients. Scandinavian Cardiovascular Journal 2007;41:51-8.  Back to cited text no. 9
Michielon G, Di Carlo D, Brancaccio G, Guccione P, Mazzera E, Toscano A, et al. Anomalous coronary artery origin from the pulmonary artery: Correlation between surgical timing and left ventricular function recovery. Ann Thorac Surg 2003;76:581-8.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded160    
    Comments [Add]    

Recommend this journal