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. 2020 Nov 30;4(6):1-5.
doi: 10.1093/ehjcr/ytaa318. eCollection 2020 Dec.

Late adult presentation of ALCAPA syndrome: need for a new clinical classification? A case report and literature overview

Eno-Martin Lotman  1 , Külliki Karu  1 , Mehis Mikkel  1 , Märt Elmet  1
Affiliations

Late adult presentation of ALCAPA syndrome: need for a new clinical classification? A case report and literature overview

Eno-Martin Lotman et al. Eur Heart J Case Rep. .

Abstract

Background: Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is a very uncommon congenital coronary artery anomaly, most commonly presenting in early infancy. Late adult presentation of ALCAPA syndrome is extremely rare.

Case summary: We present a case of a 76-year-old patient with first presentation of ALCAPA. The coronary anomaly was first diagnosed during elective coronary angiography. The case was discussed at the Heart Team meeting and as the patient was asymptomatic, had good coronary collateral circulation, a medical treatment strategy was chosen and the patient was discharged in a good physical condition. During 3 years of follow-up, the patient has experienced no cardiovascular complications.

Discussion: We hereby also discuss briefly the clinical presentation, epidemiology, diagnostics and treatment options for adults with newly diagnosed ALCAPA and discuss the need for a new clinical classification. Only a few cases have been published of septuagenarians or octogenarians with first presentation of ALCAPA. To our knowledge, the patient presented in our case was one of the least symptomatic patients during her eight decades of life.

Keywords: ALCAPA; Bland–White–Garland syndrome; Case report; Coronary artery anomaly.

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Figures

Figure 1
Figure 1
Aortography with a single coronary artery (right coronary artery) from the aorta.
Figure 2
Figure 2
Coronarography with a severely dilated right coronary artery, collateral flow, and anomalous left coronary artery with retrograde flow into the pulmonary artery.
Figure 4
Figure 4
Parasternal long axis echocardiography. Dilated right coronary artery clearly visible.
Figure 5
Figure 5
Coronary computed tomography three-dimensional reconstruction of posterior collaterals from the right coronary artery to the circumflex branch of left coronary artery.
Figure 3
Figure 3
Coronary computed tomography two-dimensional views of the proximal right coronary artery from the aorta and proximal and anomalous left coronary artery flowing into the pulmonary artery.
See this image and copyright information in PMC

References

    1. Bland EF, White PD, Garland J.. Congenital anomalies of coronary arteries. Am Heart J 1933;8:787–801.
    1. Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J 1959;21:149–161. - PMC - PubMed
    1. 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;34:204–210. - PMC - PubMed
    1. Kayalar N, Burkhart HM, Dearani JA, Cetta F, Schaff HV.. Congenital coronary anomalies and surgical treatment. Congenit Heart Dis 2009;4:239–251. - PubMed
    1. Sinha SK, Verma CM, Krishna V, Thakur R, Kumar B, Goel A. et al. ALCAPA in an octogenarian woman: an enigma. Cardiol Res 2015;6:289–291. - PMC - PubMed

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