A 66-year-old male presented with progressive dyspnoea at 39 years of age and was diagnosed with an anomalous left coronary artery from the pulmonary artery (ALCAPA). He underwent coronary artery bypass graft surgery with the left internal mammary artery to the left anterior descending artery and a reversed saphenous venous graft to the obtuse marginal artery in view of the difficulty in the left main coronary artery translocation. He underwent a dual chamber pacemaker for sick sinus syndrome at 54 years of age. Now he presented with progressive functional class IV dyspnoea. Chest X-ray revealed a suspicious area of patchy calcification in the cardiac silhouette (Figure 1A). Echocardiography showed moderate systolic left ventricular dysfunction with grade III diastolic dysfunction, severe right ventricular dysfunction, and moderate mitral and tricuspid regurgitation with severe pulmonary hypertension. Interestingly mid and basal portions of the anterior and anterolateral walls as well as both of the papillary muscles were severely calcified which showed acoustic shadowing in echocardiography (Figure 1B-D, Supplementary Videos 1, 2, 3, 4). Cardiac computed tomography revealed significant calcification of corresponding segments with extensive calcification of both the papillary muscles (Figures 1E-G, 2, and 3). Grafts were normal. Chronic ischemia led to significant calcification of myocardium in the left coronary artery circulation territory due to chronic coronary steal.
Figure 1. (A) Chest X-ray showing opacity in cardiac silhouette raising suspicion of calcification of mediastinal structure; 2-dimensional-echocardiogram in parasternal long axis (B), apical 4-chamber (C) and parasternal short axis (D) views showing extensive anterolateral (arrow) and posteromedial (arrowhead) papillary muscle with severe endocardial (D) calcification having acoustic shadow; Cardiac computed tomogram in coronal (E), sagittal (F) and axial (G) view showing extensive anterolateral (arrow) and posteromedial (arrowhead) papillary muscle with severe endocardial (D) calcification.
Figure 2. Volume rendering image showing extensive myocardial calcification (note that calcification is restricted to left coronary territory without affecting right coronary territory). Note the stump of left main coronary artery which was ligated during surgery (arrowhead). Asterix: myocardial calcification with likely both papillary muscles calcification.
LAD = left anterior descending artery; LIMA = left internal mammary artery; RCA = right coronary artery.
Figure 3. Cinematic rendering of the heart with window level adjusted for high attenuation structures showing dense calcifications in the left ventricle. White colour: calcification.
Myocardial calcification can be dystrophic or metastatic. Major causes of myocardial dystrophic calcification are ischemia, healed myocarditis (infective or inflammatory), endomyocardial fibrosis, and neoplasm.1) Whereas, important causes of metastatic myocardial calcification are renal failure, hyperparathyroidism, vitamin D deficiency, and idiopathic.2) Specific vascular territory involvement and extensive calcification supported the aetiology as ALCAPA rather than other etiologies. Extensive myocardial dystrophic calcification can lead to systolic or diastolic dysfunction, complete heart block or ventricular tachycardia and predispose patients to high risk of major cardiovascular adverse events.1),3) Rare survivors of ALCAPA may present with severe myocardial calcification and its complications and left coronary territory myocardial calcification in adulthood should always raise the suspicion of ALCAPA.4)
Patient consent for publication was obtained.
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: Mondal S, Kumar SG.
- Data curation: Mondal S, Suresh S.
- Formal analysis: Mondal S, Suresh S.
- Investigation: Mondal S, Suresh S, Kumar SG, Radhakrishnan J.
- Supervision: Kumar SG.
- Validation: Kumar SG.
- Writing - original draft: Mondal S.
- Writing - review & editing: Mondal S, Radhakrishnan J.
SUPPLEMENTARY MATERIALS
Two-dimensional echocardiogram in parasternal long axis view.
Two-dimensional echocardiogram in apical 4-chamber view.
Two-dimensional colour mode echocardiogram in apical 4-chamber view.
Two-dimensional echocardiogram in mid-ventricular short-axis view.
References
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Associated Data
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Supplementary Materials
Two-dimensional echocardiogram in parasternal long axis view.
Two-dimensional echocardiogram in apical 4-chamber view.
Two-dimensional colour mode echocardiogram in apical 4-chamber view.
Two-dimensional echocardiogram in mid-ventricular short-axis view.