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Anomalous origin of the coronary artery from the pulmonary artery in a 33-year-old man
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  1. Dominika M Zoltowska1,
  2. Yashwant Agrawal2,
  3. Guramrinder Singh Thind1,
  4. Jagadeesh K Kalavakunta3
  1. 1Department of Internal Medicine, Western Michigan University School of Medicine, Kalamazoo, Michigan, USA
  2. 2Western Michigan University Homer Stryker School of Medicine, Kalamazoo, Michigan, USA
  3. 3Cardiology, Borgess Medical Center, Kalamazoo, Michigan, USA
  1. Correspondence to Dr Dominika M Zoltowska, dominika.zoltowska{at}med.wmich.edu

https://doi.org/10.1136/bcr-2018-225310

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    Description 

    A 33-year-old man, without prior history of coronary artery disease, was referred to a catheterisation laboratory with a diagnosis of non-ST elevation myocardial infarction. Coronary angiogram showed a dilated, dominant right coronary artery of normal origin with multiple collaterals draining to the left coronary system (figure 1A,B) and a coronary ‘steal’ phenomenon into the pulmonary artery (PA). Left main coronary artery (LMA) was not visualised on contrast injection into the aortic root (figure 1B). Ventriculogram showed ejection fraction of 40%–45% and anterior wall hypokinesis. Subsequent three-dimensional heart computer tomogram revealed anomalous origin of the LMA from PA, (figure 1C), confirming the diagnosis of anomalous origin of the coronary artery from the pulmonary artery (ALCAPA).

    Figure 1
    Figure 1

    (A) Left anterior oblique (LAO) view. Large dominating RCA with extensive collaterals to the left coronary system. (B) Failure to visualise left coronary artery on contrast injection to the left coronary cusp. (C) Three-dimensional computer reconstruction of coronary arteries showing anomalous origin of the left coronary artery system from the PA. Ao, aorta; LAD, left anterior descending artery; LCx, left circumflex artery; PA, pulmonary artery; RCA, right coronary artery.

    ALCAPA, also known as Bland-White-Garland syndrome, is a rare anomaly with estimated prevalence of 1:300 000 and high mortality rate in early childhood if left untreated. Very rarely it can be associated with other cardiac anomalies such as septal defects, coarctation of aorta, patent ductus arteriosus and tetralogy of Fallot.1 In our case, we did not see any other cardiac anomalies except redundant mitral leaflets with minimal prolapse (video 1). There are only a few survivors diagnosed in adulthood, presenting with various clinical symptoms, most commonly dyspnoea and angina.2 Surgery is the mainstream therapy. Our patient underwent left coronary artery (LCA) reimplantation to the aorta without complications. Successful treatment with coronary artery bypass grafting combined with LCA ligation from the PA was also reported.3 

    Video 1

    Transthoracic echocardiogram with Doppler studies revealing trace mitral prolapse.

    Learning points

    • Anomalous coronary arteries as important differential diagnosis of chest pain in young population.

    • The importance of diagnosis and treatment of anomalous origin of the coronary artery from the pulmonary artery to prevent mortality and mortality.

    • The utilisation of three-dimensional heart computer tomography to establish the anatomy of anomalous coronary arteries.

    References

    1. ↵
      1. Khanna A,
      2. Torigian DA,
      3. Ferrari VA, et al
      . Anomalous origin of the left coronary artery from the pulmonary artery in adulthood on CT and MRI. AJR Am J Roentgenol 2005;185:326–9.doi:10.2214/ajr.185.2.01850326
      OpenUrlPubMed
    2. ↵
      1. Boutsikou M,
      2. Shore D,
      3. Li W, 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.doi:10.1016/j.ijcard.2018.02.082
      OpenUrl
    3. ↵
      1. Alsara O,
      2. Kalavakunta JK,
      3. Hajjar V, et al
      . Surviving sudden cardiac death secondary to anomalous left coronary artery from the pulmonary artery: a case report and literature review. Heart Lung 2014;43:476–80.doi:10.1016/j.hrtlng.2014.06.048
      OpenUrlCrossRefPubMed

    Footnotes

    • Contributors All authors were the physicians in charge of the patient throughout hospitalisation and follow-up. DMZ and JK were responsible for performing, diagnosing and discussing the imaging studies of the patient. DMZ prepared the manuscript draft, which was critically revised by YA, GST and JK, and approved by all authors.

    • Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.