Clinical Characteristics of Patients with a Right Ventricular Thrombus in Arrhythmogenic Right Ventricular CardiomyopathyFunding The Zurich ARVC Program is supported from funds from Schwyzer Foundation and Baugarten Foundation in Zurich, Wild Foundation in Zug, as well as from research grants from the Swiss National Foundation. The Beijing ARVC Program is supported by the Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences (2016-I2M-1-015) and the National Center for Cardiovascular Research.
26 November 2018
29 March 2019
10 June 2019 (online)
Background Thrombus formation within the left ventricle (LV) is a well-known clinical entity and is often associated with underlying myocardial disease, whereas right ventricular (RV) thrombi are rarely observed. This study aimed to investigate the clinical characteristics of patients with arrhythmogenic RV cardiomyopathy (ARVC) who developed an RV thrombus.
Methods and Results This study included patients with an RV thrombus from the ARVC databases of the University Heart Center in Zurich, Switzerland, and the Fuwai Hospital in Beijing, China. In total, there were 13 ARVC patients who had an RV thrombus detected. The mean age was 33 ± 15 (range: 11–51) years. Eight patients (62%) were male. The mean Task Force score was 6 ± 1. Nine of these patients (69%) had an RV thrombus only whereas four patients had biventricular thrombi. All 13 ARVC patients had a severely impaired RV function (RV fractional area change 16 ± 9% and RV ejection fraction 15 ± 4%); LV ejection fraction (LVEF) was 40 ± 15%. ARVC patients with an additional LV thrombus had a lower LVEF than the others (24 ± 11 vs. 47 ± 11, p = 0.02). Under therapeutic anticoagulation, complete thrombus resolution was observed in 9/13 patients (69%).
Conclusion RV thrombus formation is a potential complication of ARVC with impaired RV function. In patients with biventricular involvement, thrombi may also occur within the LV. Anticoagulation is generally effective to dissolve RV thrombi. This study highlights the need for awareness during cardiac imaging to detect this rare complication of ARVC.
* Deniz Akdis and Kai Chen are shared first authors.
** Firat Duru and Jiangping Song are senior co-authors.
- 1 Cameron J, Pohlner PG, Stafford EG, O'Brien MF, Bett JH, Murphy AL. Right heart thrombus: recognition, diagnosis and management. J Am Coll Cardiol 1985; 5 (05) 1239-1243
- 2 Otoupalova E, Dalal B, Renard B. Right heart thrombus in transit: a series of two cases. Crit Ultrasound J 2017; 9 (01) 14
- 3 Attenhofer Jost CH, Bombeli T, Schrimpf C, Oechslin E, Kiowski W, Jenni R. Extensive thrombus formation in the right ventricle due to a rare combination of arrhythmogenic right ventricular cardiomyopathy and heterozygous prothrombin gene mutation G20210 A. Cardiology 2000; 93 (1-2): 127-130
- 4 Wu L, Yao Y, Chen G. , et al. Intracardiac thrombosis in patients with arrhythmogenic right ventricular cardiomyopathy. J Cardiovasc Electrophysiol 2014; 25 (12) 1359-1362
- 5 Waller BF, Grider L, Rohr TM, McLaughlin T, Taliercio CP, Fetters J. Intracardiac thrombi: frequency, location, etiology, and complications: a morphologic review--part I. Clin Cardiol 1995; 18 (08) 477-479
- 6 Talle MA, Buba F, Anjorin CO. Prevalence and aetiology of left ventricular thrombus in patients undergoing transthoracic echocardiography at the University of Maiduguri Teaching Hospital. Adv Med 2014; 2014: 731936
- 7 Elliott P, Andersson B, Arbustini E. , et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008; 29 (02) 270-276
- 8 Felker GM, Shaw LK, O'Connor CM. A standardized definition of ischemic cardiomyopathy for use in clinical research. J Am Coll Cardiol 2002; 39 (02) 210-218
- 9 Lang RM, Badano LP, Mor-Avi V. , et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 2015; 16 (03) 233-270
- 10 Harjola VP, Mebazaa A, Čelutkienė J. , et al. Contemporary management of acute right ventricular failure: a statement from the Heart Failure Association and the Working Group on Pulmonary Circulation and Right Ventricular Function of the European Society of Cardiology. Eur J Heart Fail 2016; 18 (03) 226-241
- 11 Saguner AM, Duru F, Brunckhorst CB. Arrhythmogenic right ventricular cardiomyopathy: a challenging disease of the intercalated disc. Circulation 2013; 128 (12) 1381-1386
- 12 Saguner AM, Medeiros-Domingo A, Schwyzer MA. , et al. Usefulness of inducible ventricular tachycardia to predict long-term adverse outcomes in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 2013; 111 (02) 250-257
- 13 Akdis D, Brunckhorst C, Duru F, Saguner AM. Arrhythmogenic cardiomyopathy: electrical and structural phenotypes. Arrhythm Electrophysiol Rev 2016; 5 (02) 90-101
- 14 Wlodarska EK, Wozniak O, Konka M, Rydlewska-Sadowska W, Biederman A, Hoffman P. Thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Europace 2006; 8 (08) 596-600
- 15 Persson R, Earley A, Garlitski AC, Balk EM, Uhlig K. Adverse events following implantable cardioverter defibrillator implantation: a systematic review. J Interv Card Electrophysiol 2014; 40 (02) 191-205
- 16 Barrios D, Chavant J, Jiménez D. , et al; Registro Informatizado de la Enfermedad TromboEmbólica Investigators. Treatment of right heart thrombi associated with acute pulmonary embolism. Am J Med 2017; 130 (05) 588-595
- 17 Casazza F, Bongarzoni A, Centonze F, Morpurgo M. Prevalence and prognostic significance of right-sided cardiac mobile thrombi in acute massive pulmonary embolism. Am J Cardiol 1997; 79 (10) 1433-1435
- 18 Kajy M, Shokr M, Ramappa P. Use of direct oral anticoagulants in the treatment of left ventricular thrombus: systematic review of current literature. Am J Ther 2019; DOI: 10.1097/MJT.0000000000000937.