Cardiovascular
Medicine
Bland-Garland-White Syndrome ALCAPA Cardiovascular CT Anomalous Coronary Artery

Chest pain after surgical repair of Bland-Garland-White Syndrome

Images in Cardiovascular Medicine
Bland-Garland-White Syndrome ALCAPA Cardiovascular CT Anomalous Coronary Artery
André Azul Freitas
Additional authors
Rui Baptista, Valdirene Gonçalves, Nuno Campos, Lino Gonçalves
Issue
2022/01
DOI:
https://doi.org/10.4414/cvm.2022.02191
Cardiovasc Med. 2022;25:w02191

Published on 01.01.2022

Exceptional clinical scenario highlighting the increasing importance of noninvasive imaging in the evaluation of patients after surgical correction of coronary abnormalities.

Case description

A 19-year-old female with a past history of a surgical correction of a Bland-Garland-White syndrome was admitted to the emergency department complaining of chest pain precipitated by exercise. One year before, the patient underwent a left main closure by a pulmonary artery patch and a left internal mammary artery (LIMA) bypass to the left anterior descending (LAD) artery, with a substantial clinical improvement at the time. However, over the previous 3 months, the patient suffered symptom recurrence with a progressive worsening of the chest pain on physical exertion. The physical examination and the laboratory analysis (including serial cardiac troponin levels) were unremarkable. Electrocardiography showed sinus rhythm and a left anterior fascicular block. An echocardiogram revealed a preserved left ventricle ejection fraction, anterior hypokinesia and a systolic jet in right ventricular outflow tract that was suggestive of a left-to-right shunt. (video 1: Echocardiogram showing a left ventricle with preserved ejection fraction, an anterior hypokinesia and a systolic jet in right ventricular outflow tract).
Coronary computed tomography (CT) angiography revealed a patent LIMA to LAD bypass, ectasia of right coronary artery and a suture dehiscence with a recanalisation of the left coronary artery ostium with signs of arterial flow from the left main to the pulmonary trunk (fig. 1) The patient underwent new cardiac surgery, and an ostium closure was performed with a polypropylene suture in an autologous pericardium patch. The patient was discharged from the hospital and after a 6-month follow-up remained asymptomatic.
Figure 1.
Axial and sagittal coronary computed tomography angiography images showing an anomalous left coronary artery from the pulmonary artery with signs of contrast flowing to the pulmonary artery suggestive of a left-to-right shunt (A and B). A patent left internal mammary artery to left anterior descending artery bypass can be observed in multiplanar reconstructed (C and E) and volume rendered (D) images. Coronary ectasia is also evident (F) as a result of a compensatory mechanism and a high-flow state originated by a coronary steal phenomenon. In this situation, blood is diverted from the right coronary artery and collateral circulation to the left coronary artery and the pulmonary trunk. In order to avoid myocardial ischaemia, the coronary arteries dilate to allow an increase of blood flow.
Bland-Garland-White syndrome rarely presents in adults and data regarding the surgical strategy is limited and controversial [1–3]. In this patient, the heart team decided to perform ostium closure and LIMA bypass rather than reimplantation, as coronary mobilisation would not be adequately performed. Chest pain after this surgical correction may have several causes. Cardiovascular CT has high spatial resolution and rapid acquisition time, being the preferred technique for the evaluation of coronary artery anomalies and allowing the assessment of high-risk anatomy features [4]. In this particular case, cardiovascular CT allowed the identification of an ostium recanalisation and excluded other important causes of thoracic pain, including bypass failure.
This case describes an exceptional clinical scenario and highlights the increasing importance of noninvasive imaging in the evaluation of patients after surgical correction of coronary abnormalities when a complication or technique failure is suspected.
You will find the video file in the multimedia collection of ­«Cardiovascular Medicine»:https://cardiovascmed.ch/online-only-content
No financial support and no other potential conflict of interest relevant to this article was reported.
André Azul Freitas, MD
Serviço de Cardiologia
Hospitais da Universidade de Coimbra
Praceta Mota Pinto 
3000 Coimbra
Portugal
andre.azulfreitas[at]gmail.com
1.  Surgical Outcome in Adolescents and Adults With Anomalous Left Coronary Artery From Pulmonary Artery. Ann Thorac Surg. 2018 Dec;106(6):1860–7. http://dx.doi.org/10.1016/j.athoracsur.2018.05.051 PubMed 1552-6259
2.  Repair of anomalous coronary artery from the pulmonary artery: a-signal center 20-year experience. Int J Cardiol. 2016 Nov;223:625–9. http://dx.doi.org/10.1016/j.ijcard.2016.08.221 PubMed 1874-1754
3. . Surgical strategies for anomalous origin of coronary artery from pulmonary artery in adults. J Thorac Cardiovasc Surg. 2014 Jul;148(1):220–4. http://dx.doi.org/10.1016/j.jtcvs.2013.08.026 PubMed 1097-685X
4.  ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2020; http://dx.doi.org/10.1093/eurheartj/ehaa554   http://dx.doi.org/10.1093/eurheartj/ehaa702 PubMed 0195-668XEdifix has not updated this reference because the article title in PubMed significantly differs from the author's original. The PubMed reference is Baumgartner H, De Backer J. The ESC Clinical Practice Guidelines for the Management of Adult Congenital Heart Disease 2020. Eur Heart J. 2020 Nov;41(43):4153–4. (Ref. 4 "Baumgartner, De Backer, Babu-Narayan, et al., 2020")

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