A Large Tumour of the Left Atrium – A 10-Year Follow-up

A Large Tumour of the Left Atrium – A 10 Year Follow up Accepted Manuscript A Large Tumour of the Left Atrium – A 10 Year Follow up Agnieszka M Łebek Szatańska, L Łebek Zbigniew, Marek J Dąbrowski, Pi[.]

Accepted Manuscript A Large Tumour of the Left Atrium – A 10-Year Follow-up Agnieszka M Łebek-Szatańska, L Łebek Zbigniew, Marek J Dąbrowski, Piotr S Kardaszewicz PII: S1109-9666(16)30289-5 DOI: 10.1016/j.hjc.2016.11.008 Reference: HJC 70 To appear in: Hellenic Journal of Cardiology Received Date: 21 July 2014 Accepted Date: 29 September 2015 Please cite this article as: Łebek-Szatańska AM, Łebek Zbigniew L, Dąbrowski MJ, Kardaszewicz PS, A Large Tumour of the Left Atrium – A 10-Year Follow-up, Hellenic Journal of Cardiology (2016), doi: 10.1016/j.hjc.2016.11.008 This is a PDF file of an unedited manuscript that has been accepted for publication As a service to our customers we are providing this early version of the manuscript The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain ACCEPTED MANUSCRIPT CASE REPORT: TITLE: A LARGE TUMOUR OF THE LEFT ATRIUM – A 10-YEAR FOLLOW-UP RI PT RUNNING TITLE: A LARGE TUMOUR OF THE LEFT ATRIUM SC AUTHORS: Łebek-Szatańska Agnieszka M.1, Łebek Zbigniew L.2, Dąbrowski Marek J.1, Kardaszewicz Piotr S.2 Cardiology Clinical of Physiotherapy Division of the 2nd Faculty of Medicine, Medical University of Warsaw, Bielański Hospital in Warsaw, Poland Department of Cardiology, Provincial St.Mary Hospital in Częstochowa, Poland M AN U CORRESPONDING AUTHOR: Agnieszka Łebek-Szatańska Ul.Zgrupowania Żmija 9/274, 01-875 Warszawa Tel +48694540351 Institution: Bielański Hospital, Cegłowska 80, 01-809 Warszawa, tel.+48225690292, fax +48228341820 AC C EP TE D KEY WORS: left atrial myxoma, echocardiography ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ABSTRACT: Benign myxomas are the most common among primary tumours in the left atrium of the heart and they usually require an urgent operative management However, in times of constant ageing of the population and dealing with patients with severe concomitant diseases, an interesting question appears about the conservative approach We present a case of 75-year-old woman, once diagnosed and operated on left atrium myxoma, with recurrent tumour in the site of resection found in control echocardiography 1,5 years later She has been observed for over 10 years as she has been repeatedly refusing the reoperation Regarding this example of relatively favourable clinical course of left atrium myxoma, we discuss the current knowledge about natural history of these tumours and the role of echocardiography in predicting their growth and complications ACCEPTED MANUSCRIPT MAIN TEXT: RI PT INTRODUCTION: Among primary tumours found in the left atrium, benign myxomas constitute a vast majority (80-90%).1 Over 20% of them are asymptomatic.2 In other cases, three main types of presentations are observed: embolic complications, obstruction to the intracardiac blood flow and systemic symptomes Considering the risk of lifethreatening complications, the recommended therapeutic method is a tumour removal surgery However, literature reports some infrequent situations requiring a conservative approach The paper describes a case of a 75-year-old woman with recurrence of left atrial myxoma diagnosed over 10 years before, who refused her consent on another surgery AC C EP TE D M AN U SC CASE STUDY: A 75-year old female patient with arterial hypertension, hypothyroidism treated substitutively, history of anxiety neurosis and insomnia, had been first referred to transthoracic echocardiography 13 years before, because of paroxysmal palpitations and a slight reduction of exercise tolerance The examination revealed a tumour of the left atrium associated with the interatrial septum, size of 33x57 mm in four-chamber (4C) view; no more abnormalities were found in the presentation of the heart Tumour was surgically excised Patomorphological examination confirmed the diagnosis of myxoma The procedure brought no significant clinical improvement, which was thought to be associated to the fact that symptoms were mostly of psychogenic character Control echography was performed after the surgery Another investigation, performed 1.5 years after the procedure, revealed a well-circumscribed formation demonstrating characteristic signs of myxoma, located at the resection site – most probably a recurrence, size 22x37 mm, without a visible pedicle or calcification, but with smooth surface, causing no disturbance to the mitral flow Since that time the patient has remained under supervision of the Cardiology Clinic (over 10 years now) She had been repeatedly informed about necessity of surgical removal and refused giving her informed consent on the repeated cardiological surgery Multiple echocardiographic investigations revealed a progression of the size of tumour, initially rapid (up to over 10 mm/year in both dimensions), then very slow In 10 years of follow-up the tumour has reached the size of 42x62 mm in 4C projection, with simultaneous echocardiographic features of pulmonary hypertension ( diastolic movement of the interventricular septum towards the left ventricle, transvalvular retrogradient through the tricuspid valve of approx 70 mmHg – compared with the retrogradient first defined of 35 mmHg years before) and development of moderate/severe tricuspid and pulmonary regurgitation During that time a slowly increasing signs of heart failure with preserved ejection fraction had been observed, mainly in form of paroxysmal nocturnal dyspnoea However, those symptoms cause only a slight impairment of the patient’s everyday function Only once the patient required hospitalisation because of exacerbation of circulatory failure presenting as pulmonary oedema, which was probably associated with intensive initial growth of the tumour Despite signs of the right ventricular insufficiency demonstrated by echocardiography, a clear clinical manifestation has been absent No episodes of atrial fibrillation have been registered, an electrocardiography (ECG) appeared normal Only the increased erythrocyte sedimentation rate (ESR) was remarkable in additional laboratory tests In course of the 10-year follow-up the patient has been irregularly using drugs: beta-blocker and diuretic at low doses, she has been also incompliant in ACCEPTED MANUSCRIPT terms of antithrombotic treatment For majority of the time she has failed to achieve therapeutic values of the international normalized ratio (INR) AC C EP TE D M AN U SC RI PT DISCUSSION: The case presented above is another example of natural course of cardiac myxoma, which in rare cases appears to be relatively benign and demonstrates no tendencies for clinically overt complications (including embolic events).3 As generally known, the diagnosis of left atrial myxoma constitutes an urgent indication for resection The periprocedural mortality rate is below 3% [4], and surgically treated myxomas are associated with favourable prognosis, both short- and long-term Rarely does the myxoma excision surgery associate with necessary implantation of an artificial valve or pacemaker.2 Recurrence following excision of sporadic tumours is observed in several percent of patients, most commonly during first years after the initial surgery During that time a yearly echocardiographic control is recommended in order to detect a possible re-growth of a tumour.2,5 The diagnosis of heart myxoma is an indication for coronarography before a considered cardiosurgical procedure In case of a co-existent advanced coronary disease the decision concerning the surgical procedure is additionally justified by a possibility of simultaneous coronary-aortal bypass grafting.6,7 In case of small and asymptomatic tumours and absence of other indications for surgery, the decision may be less obvious than expected.6 As majority of myxomas are excised shortly after making the diagnosis, little is known about the natural development of the condition Available scarce data regarding growth rate, often contradictory, indicate the growth rate ranging from absolute absence of growth to several millimetres per month.8 Recurrent myxomas are attributed a tendency for higher growth rate compared to primary ones.3 As indicated by this case, the myxoma growth rate is also variable in a single patient – initially a faster rate was observed (up to over 10 millimetres/year), followed by a later period when the growth was several times slower The calculated mean myxoma growth increase years after the diagnosis of recurrence (and for the next years) was only approx 0.15 millimetre/month Myxoma size ranges from several millimetres to as much as 15 cm in diameter Large tumours (diameter of over cm) are considered to be more commonly associated with cardiological symptoms In the presented case an initially more rapid growth of the tumour was correlated with development of symptoms of circulatory system decompensation (despite a relatively small size) Therefore, not only the size of myxoma [3], but also the current growth rate play an important role in development of clinical manifestation of the tumour’s presence in the left atrium The question of it is possible to predict behaviour of myxoma of the left atrium based on its features in follow-up echocardiographic investigations is an important tone In their study Pinede, Duhaut and Loire noted that in case of round myxoma with smooth surface, the incidence of embolic events is over twice less frequent in comparison to the situation when a tumour is described as villous, polypous and fragile.1,9 Similarly, in case of calcified tumours the risk of cardiogenic embolism may be lower Kay and Chow described a case of an 85-year-old patient with left atrial myxoma, who refused surgical excision of the tumour, just like our patient, and who was followed for another 15 years with a stable tumour.10 An important role in clinical manifestation is played also by presence of a pedicle and by mobility of the tumour The effect of, so called, “wrecking ball” is known – injury of the mitral valve resulting from repeated contact of the tumour with cusps.1 ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT Moreover, tumour prolapse through the mitral ostium hinders blood inflow into the left ventricle, causing clinical signs of mitral defect (stenosis or incompetence) It is believed that mobility of myxoma may in those cases lead to development of paroxysmal symptoms, sometimes posture-dependent.1 On the other hand, atrial fibrillation is not often observed in patients with atrial myxoma, because atrial enlargement is not common in those patients Atrial enlargement is described in approx 30% of patients [7,9], and atrial fibrillation in 20%.7 In the discussed patient the left atrium enlarged from 36 to 41 millimetres during the period of 10-years and was almost completely filled by the tumour (Fig.1-4) As it is generally known, many tumours occur more frequently in aged organisms, but myxomas are believed to be rather rare in the elderly.11 Although the senescent cells are considered more prone to oncogenesis, the senescent immune system may contribute to the phenomen of slower growth of some tumours and smaller aptitude for metastasis of malignant cells in that population However, the problem is much more complicated, depending on the kind of tumour as well as host humoral and local factors.12,13 Whether any of these observations can be also accurate for the growth of myxomas as benign neoplasms in aged patients is still a field to explore In face of common application of surgical treatment, there are currently no detailed guidelines concerning the conservative therapy Peripheral embolism with tumour fragments or myxoma cells-containing clots is a complication occurring in a half of patients with left atrial myxoma The complication is mainly associated with the central nervous system and limbs, but sometimes myxoma causes also embolism in coronary circulation or abdominal organs, or skin It is evident that with constant secretion of proinflammatory factors those tumours induce a chronic inflammatory response, favouring coagulation and formation of clots on tumour surface A chronic antithrombotic therapy is an option allowing reduction of risk of embolism However, difficulties associated with determination of an appropriate dose of coumarin derivative, and with monitoring of the therapy in elderly and sometimes noncooperative patients, often make a proper management of the therapy impossible Emergence of novel oral anticoagulants is an important alternative for those patients However, the increased risk of serious bleeding events, including haemorrhages to the central nervous system, in that group of patients has to be considered Summing up, there is currently no evidence that would justify departure from the adopted rule of immediate surgical therapy of left atrial myxoma, especially considering the unpredictability of their natural course, and hazards associated with their complications However, in some groups of patients, among others in elderly ones, with high surgical risk and short life expectancy, and especially when it is a conscious choice of a patient, the option is worth consideration REFERENCES: Sabatine MS, Colucci WS, Schoen FJ Pierwotne guzy serca In: Braunwald E, Bonow RO, Zipes PD et al Choroby serca Elsevier Urban & Partner, Wrocław 2007 Pruszczyk P, Styczyński G Nowotwory serca In: Szczeklik A, Tendera M et al Kardiologia Tom I Medycyna Praktyczna, Kraków 2009 Ullah W, McGovern R Natural history of an atrial myxoma, Age and Ageing, vol 34, 2005,p.186-188 Olivier WC Jr, Nuttall GA Uncommon Cardiac Diseases In: Kaplan J Essentials of Cardiac Anesthesia Philadelphia: Saunders Elesevier; 2008 ACCEPTED MANUSCRIPT M AN U SC RI PT Iskandar ME, Dimitrova K, Geller CM, Hoffman DM, Tranbaugh RF Complicated sporadic cardiac myxomas: a second recurrence and myxomatous cerebral aneurysms in one patient Case Rep Surg 2013;2013:642394 doi: 10.1155/2013/642394 Pasierski T Komentarz do: Kołacz J, Fedak A, Dziedzic P, Pasowicz M Śluzak lewego przedsionka Opis przypadku Choroby Serca i Naczyń 2005, tom 2, nr 4, 229–231 Ostrowski S, Wrona E, Kośmider A et al Jednoczesne usunięcie śluzaka lewego przedsionka i rewaskularyzacja mięśnia sercowego – opis przypadku Kardiochirurgia i Torakochirurgia Polska 2011; (2): 201–204 Vazir A, Douthwaite H Rapidly growing left atrial myxoma - a case report J Med Case Reports 2011;5:417 Pinede L, Duhaut P, Loire R Clinical presentation of left atrial cardiac myxoma A series of 112 consecutive cases Medicine (Baltimore) 2001; 80: 159–72 10 Kay JF, Chow WH Long-term survival of quiescent left atrial myxoma in an elderly patient Am J Geriatr Cardiol 2002; 11:165–8 11 Bire F, Roudaut R, Chevalier JM et al Cardiac myxoma in patients over 75 years of age Report of 19 cases Arch Mal Coeur Vaiss 1999 Mar;92(3):323-8 12 Anisimov VN Effect of host age on tumor growth rate in rodents Front Biosci 2006 Jan 1;11:412-22 13 Ershler WB Explanations for reduced tumor proliferative capacity with age Exp Gerontol 1992 Sep-Dec;27(5-6):551-8 EP TE D COMMENTS TO FIGURES: Fig.1 - TTE 2D Normal 4-chamber apical view Fig.2 - Normal long axis view Fig.3 - Normal 2-chamber apical view Fig.4 - Normal short axis view of ventricles Left atrium myxoma (arrow) LV-left ventricle, RV-right ventricle, LA-left atrium, RA-right atrium, Ao-aorta AC C STATEMENTS: We state that this manuscript has not been previously published and is not under consideration in the same or substantially similar form in any other peer-reviewed media All authors listed have contributed sufficiently to the manuscript To the best of our knowledge, no conflict of interest, financial or other, exists AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT ...ACCEPTED MANUSCRIPT CASE REPORT: TITLE: A LARGE TUMOUR OF THE LEFT ATRIUM – A 10-YEAR FOLLOW-UP RI PT RUNNING TITLE: A LARGE TUMOUR OF THE LEFT ATRIUM SC AUTHORS: Łebek-Szatańska Agnieszka... transthoracic echocardiography 13 years before, because of paroxysmal palpitations and a slight reduction of exercise tolerance The examination revealed a tumour of the left atrium associated with the. .. that would justify departure from the adopted rule of immediate surgical therapy of left atrial myxoma, especially considering the unpredictability of their natural course, and hazards associated