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CASE REPORT
Year : 2018  |  Volume : 11  |  Issue : 6  |  Page : 568-570  

Anomalous origin of the left coronary artery from the main pulmonary artery


1 Department of Paediatrics, Federal Medical Centre, Nguru, Yobe State, Nigeria
2 Department of Surgery, Cardiothoracic Unit, Aminu Kano Teaching Hospital, Kano, Nigeria
3 Department of Family Medicine, Aminu Kano Teaching Hospital, Kano, Nigeria
4 Department of Paediatrics, Aminu Kano Teaching Hospital, Kano, Nigeria
5 Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria

Date of Submission06-Feb-2018
Date of Acceptance15-May-2018
Date of Web Publication15-Nov-2018

Correspondence Address:
Ismail Inuwa Mohammed
Department of Surgery, Aminu Kano Teaching Hospital, Kano
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_28_18

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  Abstract 


The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart anomaly; this occurs in 1/300,000 live births. ALCAPA syndrome was first described in 1933 by Bland and co-authors in autopsy specimens; however, further description of its clinical manifestations resulted in the naming of Bland–White–Garland syndrome. The case of a 2-year-old boy who was referred for echocardiographic investigation due to recurrent cough, catarrh, and occasional noisy breathing is reported in this communication; his chest X-ray was normal, while electrocardiogram showed Q-waves on limb leads I and aVL and the echocardiographic study showed ALCAPA.

Keywords: Anomalous origin of the left coronary artery from the pulmonary artery, chest-X-ray, echocardiography, electrocardiography


How to cite this article:
Garba N, Mohammed II, Michael GC, Peter ID, Shakur AM, Asani MO, Aliyu I. Anomalous origin of the left coronary artery from the main pulmonary artery. Med J DY Patil Vidyapeeth 2018;11:568-70

How to cite this URL:
Garba N, Mohammed II, Michael GC, Peter ID, Shakur AM, Asani MO, Aliyu I. Anomalous origin of the left coronary artery from the main pulmonary artery. Med J DY Patil Vidyapeeth [serial online] 2018 [cited 2018 Dec 19];11:568-70. Available from: http://www.mjdrdypv.org/text.asp?2018/11/6/568/245424




  Introduction Top


The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart anomaly; this occurs in 1/300,000 live births.[1],[2],[3] ALCAPA syndrome was first described in 1933 by Bland and co-authors in autopsy specimens; however, further description of its clinical manifestations resulted in the naming of Bland–White–Garland syndrome.[1],[3] It is an isolated congenital heart disease, but it is rarely associated with other congenital anomalies such as ventricular septal defect, transposition of great arteries, tetralogy of Fallot, and truncus arteriosus.[1],[2] The syndrome may be developed as a result of conotruncal division defect, or an abnormality of the persistence of pulmonary artery buds or aortic bud involution during fetal heart development.[1] There are two clinical patterns that have been described in relation to age and the time of presentation (infantile and adult types).[1],[2],[3],[4] Perry and Scott[5] also attempted classifying the ALCAPA further, based on clinical presentations into Group I, infant syndrome onset at 2–4 months with angina-like symptoms and cardiomegaly; Group II, consisting of mitral insufficiency of unknown etiology; Group III, presenting with continuous murmur syndrome; and Group IV, adults with sudden death. ALCAPA usually presents within few days to months of life in infants compared to adults in whom ALCAPA shows late presentation.[4] The early presentation in infant type is as a result of absence of collateral formation between the right and left coronary arteries that ensures adequate blood flow in the left coronary arterial zones.[4] Left ventricular dysfunction and mitral regurgitation could be the resulting complications of steal phenomenon.[4],[6]


  Case Report Top


We present the case of a 2-year-old boy who was referred for echocardiographic investigation due to recurrent cough, catarrh, and occasional noisy breathing. He was born of a nonconsanguineous marriage; there was no family history of a cardiac disorder. He had normal growth, development, and general physical examination findings. Cardiovascular examination revealed pulse rate of 120 beats/min, apical impulse at the 4th left intercostal space, and there were 1st and 2nd heart sounds only. Chest radiography was normal [Figure 1]. Echocardiographic examination revealed retrograde diastolic blood flow from main pulmonary artery to the left coronary artery by color Doppler study [Figure 2] and there was mitral regurgitation [Figure 3]. The right coronary artery was larger than the left coronary artery. Systolic function was normal with ejection fraction of 56% and shortening fraction of 29%. The cardiac chambers were normal and the left atrial/aortic root ratio was 1.2. The mitral valve was normal and other valves were structurally normal. Electrocardiographic (ECG) findings revealed sinus tachycardia and the presence of prominent Q-waves in leads I and aVL with inverted P-wave in lead III [Figure 4]. Other diagnostic tests such as cardiothoracic angiography and cardiac catheterization were not done because they are not available in our center. Furthermore, the patient was referred for surgical intervention abroad because of lack of facility for cardiac surgery in our setting; meanwhile, the child is still being followed up in our cardiology clinic with his parents being the source of funding.
Figure 1: Chest radiography of the patient showing normal-sized heart

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Figure 2: Color Doppler showing retrograde flow in red color from the main pulmonary artery into the left coronary artery

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Figure 3: Parasternal long-axis view showing mitral regurgitation

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Figure 4: Electrocardiogram showing deep Q-waves in the limb leads

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  Discussion Top


ALCAPA is one of the rare congenital heart anomalies, with occurrence of 1/300,000 live births.[1],[3] Collaterals are often formed between the right and left coronary arteries to enable perfusion of the zones of the left coronary artery.[1],[2],[3],[4] Myocardial steal phenomenon develops as a result of collateral formation leading to right-to-left shunt from the right coronary artery to the pulmonary artery.[1],[2],[3],[4] Although collateral circulation enhances left ventricular perfusion, this may not prevent the development of symptoms except stenosis of the steal osteum which may increase intercoronary perfusion pressure resulting in increased coronary blood flow and delayed presentation, especially in adults.[6] We report an incidental finding of ALCAPA in a 2-year-old boy whose echocardiographic examination and ECG findings are consistent with ALCAPA. The age of this patient is consistent with infantile type.[2],[3] Presence of Q-waves in limb leads I and aVL was noted in the index case; this was also reported by Perry and Scott.[5] Furthermore, color Doppler echocardiographic assessment may show retrograde diastolic blood flow from pulmonary trunk to the left coronary artery which was demonstrated in this patient, with exception of those that developed pulmonary hypertension in the absence of collateral formation that may need cardiac catheterization for further evaluation.[7] Magnetic resonance imaging, computed tomography, and cardiac angiography are important diagnostic investigations to determine the origin of coronary artery. Management of patients with diuretics, inotropes, and other medications is important in congestive heart failure before surgical intervention. Surgery is the mainstay management by detachment of coronary artery from pulmonary artery and re-implantation of coronary artery into the aortic root.[8] Other surgical methods include Takeuchi procedure.[8] Cardiac transplantation has been recommended in patients with cardiomyopathy not amenable to arterial switch surgery.[8]


  Conclusion Top


ALCAPA syndrome is a rare congenital heart disease that has variable clinical presentation. A high index of suspicion is important for early identification and prompt treatment to prevent the development of left ventricular dysfunction and late cardiomyopathy. Echocardiographic examination with color Doppler is essential in diagnosing this disease in a suspected child.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Aliasghar M, Mohammed AT, Hassan MM, Nahid Z. The anomalous of the left coronary artery from the pulmonary artery (ALCAPA): A case series and brief review. Int J Pediatr 2016;4:1397-405.  Back to cited text no. 1
    
2.
Daniel B. Anomalous of left coronary artery from the pulmonary artery. In: Robert MK, Bonital FS, Joseph W, editors. Nelson Textbook of Paediatrics. 20th ed. Elsevier, Philadelphia, USA; 2015. p. 2236-7.  Back to cited text no. 2
    
3.
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. 3
    
4.
Kazmierczak PA, Ostrowska K, Dryzek P, Moll JA, Moll JJ. Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants. Interact Cardiovasc Thorac Surg 2013;16:797-801.  Back to cited text no. 4
    
5.
Perry LW, Scott LP. Anomalous of left coronary artery from pulmonary artery; Repair of 11 cases, review and indication for and result of surgery. Circulation 1970;41:1043-52.  Back to cited text no. 5
    
6.
Kreutzer C, Schlichter AJ, Roman MI, Kreutzer GO. Emergency ligation of anomalous left coronary artery arising from the pulmonary artery. Ann Thorac Surg 2000;69:1591-2.  Back to cited text no. 6
    
7.
Markus S, Omid S, Trace E, Naeem M. Anomalous origin of the left coronary artery from the main pulmonary artery. Circulation 2004;110:e511-3.  Back to cited text no. 7
    
8.
Malakan Rad E. Anomalous origin of left coronary artery from pulmonary artery associated with pulmonary hypertension. Indian Heart J 2014;66:700-3.  Back to cited text no. 8
    


    Figures

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



 

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