A 34-year-old woman with chronic coronary syndrome and heart failure with preserved ejection fraction (New York Heart Association [NYHA] Class II) was admitted to the hospital for continuation of diagnostics and treatment. She experienced acute coronary syndrome one month prior to the hospitalization. Coronary angiography did not show the left coronary artery (LCA) originating from the aortic bulb. The corkscrew-like right coronary artery (RCA) was widened (Supplementary Video 1), collateral circulation was visualized (Figure 1A and B). Transthoracic echocardiography showed an enlarged left ventricle with preserved left ventricular (LV) ejection fraction and severe functional mitral regurgitation. Computed tomography (CT) confirmed the diagnosis of Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) (Figure 1C-E). Steal syndrome from LCA to main pulmonary artery (MPA) was observed (Figure 2). Magnetic resonance (Figure 3) revealed a subendocardial scar (involving 25–33% of the myocardial wall thickness) affecting the apex, anterior and anteroseptal wall of the left ventricle as well as the anterolateral papillary muscle (Figure 4, Supplementary Videos 2, 3, 4, 5). The patient was qualified for surgical treatment. The abnormal shunt between the LCA and pulmonary artery (PA) was closed using an access through the MPA and an arterial bypass was implanted. Mitral annuloplasty using a Carpentier Edwards Lifesciences Annuloplasty Ring was performed. ALCAPA is a rare anomaly (1/300,000 live births).1) This defect, usually first diagnosed in infancy, may cause potentially fatal congestive heart failure. Adults can be asymptomatic, but the chronic, subendocardial ischaemia is considered to be a risk factor for sudden cardiac death due to ventricular arrhythmias.2) Multimodality imaging is a diagnostic method of choice. Echocardiography is the first-line method allowing to assess the myocardial function and potential valvular lesions. CT allows to confirm the diagnosis and provides detailed information regarding the course of vessels, which is crucial when planning the surgical procedure. Cardiac magnetic resonance (CMR) on the other hand can be used to measure the LV ejection fraction and visualize scarring of the LV myocardium, which as mentioned before is helpful in the clinical assessment of the patient and evaluating the extent of ischemia caused by the anomaly.
Figure 1. CCT of ALCAPA. (A, B) CCT VRT reconstruction showing the corkscrew-like widened RCA.(C-E)VRT reconstruction: LCA originating from the MPA different imaging planes.
ALCAPA = Anomalous Left Coronary Artery from the Pulmonary Artery; Ao = aorta; CCT = cardiac computed tomography; LCA = left coronary artery; MPA = main pulmonary artery; RCA = right coronary artery; VRT = volume rendering technique.
Figure 2. Steal syndrome from LCA to the MPA visualised in multiplanar reconstruction.
Ao = aorta; LCA = left coronary artery; MPA = main pulmonary artery.
Figure 3. Magnetic resonance with visible ALCAPA in the coronal plane.
ALCAPA = Anomalous Left Coronary Artery from the Pulmonary Artery.
Figure 4. Late gadolinium enhancement-cardiac magnetic resonance images. Images demonstrate subendocardial scar affecting anterior, anteroseptal wall and the apex of the left ventricle (A) horizontal axis view, (B) mid-ventricular short axis view, as well as (C) the anterolateral papillary muscle.
Written informed consent was obtained from the patient.
Footnotes
Funding: The authors received no financial support for the research, authorship, and/or publication of this article.
Conflict of Interest: The authors have no financial conflicts of interest.
Data Sharing Statement: The data generated in this study is available from the corresponding author upon reasonable request.
- Conceptualization: Głowacki J.
- Data curation: Danel A, Suchodolski A, Szulik M.
- Formal analysis: Danel A, Suchodolski A, Szulik M.
- Methodology: Głowacki J.
- Software: Danel A, Głowacki J.
- Supervision: Szulik M, Głowacki J.
- Validation: Szulik M.
- Visualization: Danel A.
- Writing - original draft: Danel A, Suchodolski A, Szulik M, Głowacki J.
- Writing - review & editing: Danel A, Suchodolski A, Szulik M.
SUPPLEMENTARY MATERIALS
Coronary angiography of the lesion, a corkscrew like right coronary artery is visualised.
Cine sequences of the cardiac magnetic resonance image, showing 2-chamber view.
Cine sequences of the cardiac magnetic resonance image, showing 3-chamber view.
Cine sequences of the cardiac magnetic resonance image, showing the short axis view.
Cardiac magnetic resonance image showing the anomalous origin of the left coronary artery from the pulmonary trunks.
References
- 1.Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg. 2002;74:946–955. doi: 10.1016/s0003-4975(02)03633-0. [DOI] [PubMed] [Google Scholar]
- 2.Peña E, Nguyen ET, Merchant N, Dennie C. ALCAPA syndrome: not just a pediatric disease. Radiographics. 2009;29:553–565. doi: 10.1148/rg.292085059. [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Coronary angiography of the lesion, a corkscrew like right coronary artery is visualised.
Cine sequences of the cardiac magnetic resonance image, showing 2-chamber view.
Cine sequences of the cardiac magnetic resonance image, showing 3-chamber view.
Cine sequences of the cardiac magnetic resonance image, showing the short axis view.
Cardiac magnetic resonance image showing the anomalous origin of the left coronary artery from the pulmonary trunks.