IJCA-18135; No of Pages 2 International Journal of Cardiology xxx (2014) xxx–xxx

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International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard

Letter to the Editor

Spontaneous contrast in all cardiac chambers in a patient with a normal heart Case report with literature review Ritesh Kanyal, Johanna Brugger, Anil Ramoutar, Waleed Arshad, Arvinder S. Kurbaan, Han B. Xiao ⁎ Department of Cardiology, Homerton University Hospital, London, United Kingdom

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Article history: Received 19 April 2014 Accepted 22 April 2014 Available online xxxx Keywords: Spontaneous echo contrast (SEC) Fibrinogen mean corpuscular volume Thrombosis Immune thrombocytopenic purpura

Spontaneous contrast on echocardiography (SEC) is frequently present in the left atrium (LA) in patients with cardiac conditions causing LA dilatation and stasis, such as mitral stenosis, mitral valve prosthesis and chronic atrial arrhythmias. It is also commonly seen in the left ventricle (LV) in those with dilated and dyskinetic LV due to ischemic or idiopathic dilated cardiomyopathy. Right atrial SEC has been documented in those with dilated right atrium associated with atrial arrhythmias or right ventricle systolic dysfunction [2]. SEC is a risk factor for thromboembolic events and even death regardless of the existing pathology and therefore serves as a definite indication for anticoagulation [1]. We report a case of spontaneous contrast in all cardiac chambers in the absence of dilatation or ventricular dysfunction or valvular lesion, along with a mini literature review. A 46-year-old male of Bosnian origin was seen in the Rapid Access Chest Pain Clinic with chest pain and palpitations on exertion. He is a current smoker (50–60-pack year) and has a family history of premature coronary artery disease. He was diagnosed to have immune thrombocytopenic purpura (ITP) in 2004 and cold hemagglutinin disease. He underwent splenectomy in 2006 for ITP, supra-umbilical hernia repair in 2012 and active psychotherapy for post-trauma stress disorder.

⁎ Corresponding author at: Cardiology Department, Homerton University Hospital NHS Foundation Trust, Homerton Row, London E9 6SR, United Kingdom. E-mail address: [email protected] (H.B. Xiao).

His current medications were lansoprazole 30 mg once a day, 15 mg prednisolone once a day and thrombopoietin injections (romiplostim, 250 μg) once every week. On examination, his pulse was 71 beats/min and blood pressure was 155/92 mm Hg. His heart sounds were normal and no murmurs were heard. His chest was clear and there were no peripheral markers of cold agglutinin disease such as acrocyanosis (purple discoloration of tip of nose, ear lobes and digits). The likelihood for him to have coronary artery disease was estimated at 70% according to NICE Guidance no 95. His 12 lead ECG was normal. A 24-hour ECG showed sinus rhythm throughout, a few episodes of nocturnal sinus bradycardia (58 beats per min), a few atrial ectopic beats and 2 episodes of nocturnal Mobitz type I second degree heart block; there were no significant arrhythmias either at rest or on exertion. He had a myocardial perfusion scan (adenosine) which showed normal left ventricular function and no inducible ischemia. A transthoracic echocardiogram showed spontaneous contrast in all 4 cardiac chambers despite normal cavity size, good ventricular function of both sides and no evidence of valvular lesions (Fig. 1 and Video 1). The spontaneous contrast was investigated with designated software for myocardial contrast echocardiography (Philips IE 33 Q Lab XP) without using any contrast agent; cavity opacification of soft echo contrast was clearly visible in all four chambers (Fig. 2 and Video 2). Such echocardiographic images can only be possible with intravenous echo contrast agent, such as SonoVue in normal practice [8]. The extraordinary echo findings led us to review this case systematically, apart from the past medical history summarized earlier. We found that his recent laboratory investigations showed a significant increase in the size of red blood cells (RBC). The mean corpuscular volume (MCV) was 104.3 fl (normal range 80–98), packed cell volume (PCV) was 0.282 (normal range, 0.40–0.54), slightly reduced platelet counts at 123 × 109/l (normal range 150–400 × 109/l), nearly normal erythrocyte sedimentation rate (ESR) (13 mm/h, normal range 1–10), and normal fibrinogen at 3.1 g/l (normal range 1.5–4.5). In addition, the blood film showed agglutination of red blood cells and large platelets. His myocardial perfusion scan was normal suggesting that his chest pain was unlikely to be cardiac in origin; he was therefore discharged from cardiology clinic but followed up in hematology clinic. Our patient with a normal heart in both structure and function, which was confirmed with an echocardiogram and a myocardial perfusion scan, is an unlikely candidate for spontaneous cardiac contrast in any cardiac chamber let alone all four of them. Alternative causes

http://dx.doi.org/10.1016/j.ijcard.2014.04.234 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.

Please cite this article as: Kanyal R, et al, Spontaneous contrast in all cardiac chambers in a patient with a normal heart, Int J Cardiol (2014), http:// dx.doi.org/10.1016/j.ijcard.2014.04.234

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R. Kanyal et al. / International Journal of Cardiology xxx (2014) xxx–xxx

Fig. 1. Apical 4 chamber view showing spontaneous contract in all 4 chambers. Left ventricle (LV), right ventricle (RV).

Fig. 2. Opacification seen in all 4 chambers without contrast agent. Left ventricle (LV), right ventricle (RV).

were explored in particular hematological disorders. Indeed, he had not only increased size of red blood cells but also agglutination of red blood cells and large platelets on blood film. Structurally normal heart is not thought to be at risk of SEC. RBC and plasma proteins are considered to be responsible for the formation of SEC. More recent studies have suggested fibrinogen to be a principal plasma protein for SEC generation. A stoichiometric relationship between fibrinogen (Fg) and RBC in SEC formation has been established in a recent study [3,5]. At lower hematocrit (Hct) ranges, a higher Fg concentration is required, while the reverse is true at higher Hct ranges for SEC formation. SEC density is determined by flow rate and on the relative concentration of fibrinogen and red blood cell (RBC). Our patient was also diagnosed to have immunoglobulin G (Ig G) cold hemagglutinin disease. In cold hemagglutinin disease cold agglutinins bind to erythrocyte surface antigens at a temperature of optimum 0–4 °C in vitro. Clinical manifestations of primary cold hemagglutinin disease are hemolytic anemia and cold induced circulatory symptoms ranging from moderate acrocyanosis to severe Raynaud phenomenon [4]. It is rare to see red cell agglutination in vivo at a temperature of 37 °C in the absence of peripheral signs of the disease [6]. In our case the risk of thromboembolism has been considered to be very low from the hematological point of view. Independent studies have demonstrated that stroke or other embolic event rates are higher in patients with SEC compared to patients without SEC in structurally abnormal hearts and chronic atrial arrhythmias [7]. So the concern in our case is if there is any future risk of thromboembolism. Unfortunately, there has been no data available to guide us. It is known that SEC is a marker of hyper-coagulable state, and hence a risk factor of thromboembolism [6]. For this case, it is possible that the increased mean corpuscular volume and autoimmune hematological disorders are independent predictors of spontaneous contrast and future thromboembolism. If so, one must wonder if further research

needs to be undertaken to assess whether echocardiography should be performed in all such patients and anticoagulation be offered to all those with spontaneous contrast even if the heart is structurally normal. In summary we present a case of SEC in all cardiac chambers in a patient with a normal heart but with macrocytosis and autoimmune hematological disorder. This article raises questions if echocardiography should be carried out in patients with autoimmune hematological condition to detect SEC, if anticoagulation be offered to positive SEC and if MCV is a predictor of SEC. Further studies are needed to address any of these questions. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2014.04.234. References [1] Black IW. Spontaneous echo contrast: where there's smoke there's fire. Echocardiography May 2000;17(4):373–82. [2] Bashir M, Asher CR, Garcia MJ, et al. Right atrial spontaneous echo contrast and thrombus in atrial fibrillation: a transesophageal study. J Am Soc Echocardiogr Feb 2001;14(2):122–7. [3] Rastegar R, Harmick DJ, Weidemann P, et al. Spontaneous echo contrast video density is flow related and is dependent on the relative concentration of fibrinogen and red blood cells. J Am Coll Cardiol Feb 19 2003;41(4):603–10. [4] Berensten S, Beiske K, Tjonnfjord GE. Primary chronic cold agglutinin disease: an update on pathogenesis, clinical features and therapy. Hematology Oct 2007;12(5):361–70. [5] Kwaan HC, Sakurai S, Wang J. Rheological abnormalities and thromboembolic complications in heart disease: spontaneous contrast and red cell aggregation. Semin Thromb Hemost Oct 2003;29(5):529–34. [6] Frank MM, Schreiber AD, Atkinson JP, Jaffe CJ. Pathophysiology of immune hemolytic anemia. Ann Intern Med Aug 1 1997;vol. 87(No. 2). [7] Handke M, Harloff A, Hefzel A, Olschewski M, Bode C, Geibel A. Predictors of left atrial spontaneous echocardiographic contrast or thrombus formation in stroke patients with sinus rhythm and reduced left ventricular function. Am J Cardiol Nov 1 2005;96(9):1342–4. [8] Senior R, Andersson O, Caidahl K, et al. Enhanced left ventricular endocardial border delineation with an intravenous injection of SonoVue, a new echocardiographic contrast agent: a European multicenter study. Echocardiography 2000;17:705–11.

Please cite this article as: Kanyal R, et al, Spontaneous contrast in all cardiac chambers in a patient with a normal heart, Int J Cardiol (2014), http:// dx.doi.org/10.1016/j.ijcard.2014.04.234

Spontaneous contrast in all cardiac chambers in a patient with a normal heart: case report with literature review.

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