Chronic Thromboembolic Pulmonary Hypertension (CTEPH) presenting as a right atrial myxoma- A case report and review of the literature

Document Type : Case Report


1 Ross University School of Medicine, Barbados

2 College of Biomedical Sciences, Larkin University, Miami, Florida, USA JAS Medical Management, Miramar, Florida, USA

3 Dr. Kiran C. Patel College of Osteopathic Medicine, Nova Southeastern University, Fort Lauderdale, Florida, USA

4 Biological Sciences Department, Broward College, Davie, Florida, USA

5 College of Biomedical Sciences, Larkin University, Miami, Florida, USA


Background: Pulmonary hypertension arise from an increase in resistance within the pulmonary vasculature, leading to consequences such as hypoxia, hypoxemia, and right heart failure. The underlying causes of this increased resistance are diverse and classified using the World Health Organization (WHO) classification system. One specific etiology is chronic thromboembolic pulmonary hypertension or simply CTEPH.
Case presentation: This case report focuses on a 42-year-old African American cisgender female who presented with a deep vein thrombosis in her left leg and shortness of breath. Further investigation revealed a large mass in her right atrium during a bedside echocardiogram, suggestive of a possible right atrial myxoma. This patient subsequently underwent median sternotomy with thrombectomy.
Discussion: As a sequela to this clinical presentation, our patient developed pulmonary hypertension. This report delves into the challenges and complexities involved in diagnosing and treating pulmonary hypertension, while also highlighting the unique presentation of this patient.


Main Subjects

  1. Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25):D42-50. doi:10.1016/j.jacc.2013.10.032 PMid:24355641
  2. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. doi:10.1183/13993003.01913-2018 PMid:30545968 PMCid:PMC6351336
  3. Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J. 2019;53(1):1802148. doi:10.1183/13993003.02148-2018 PMid:30552088 PMCid:PMC6351332
  4. Hoeper MM, Humbert M, Souza R, Idrees M, Kawut SM, Sliwa-Hahnle K, et al. A global view of pulmonary hypertension. Lancet Respir Med. 2016;4(4):306-22.
  5. Sumimoto K, Tanaka H, Mukai J, Yamashita K, Tanaka Y, Shono A, et al. Optimal Cut-Off of tricuspid regurgitation velocity according to the new definition of pulmonary hypertension - its use in predicting pulmonary hypertension. Circ Rep. 2020;2(10): 625-9. doi:10.1253/circrep.CR-20-0094 PMid:33693186 PMCid:PMC7932846
  6. Zangiabadi A, De Pasquale CG, Sajkov D. Pulmonary hypertension and right heart dysfunction in chronic lung disease. Biomed Res Int. 2014;2014:1-13. doi:10.1155/2014/739674
    PMid:25165714 PMCid:PMC4140123
  7. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43 (38): 3618-731. doi:10.1093/eurheartj/ehac237
  8. Wijeratne DT, Lajkosz K, Brogly SB, Lougheed MD, Jiang L, Housin A, et al. Increasing incidence and prevalence of World Health Organization groups 1 to 4 pulmonary hypertension: A population-based cohort study in Ontario, Canada. Circ Cardiovasc Qual Outcomes. 2018;11(2):e003973. doi:10.1161/CIRCOUTCOMES.117.003973 PMid:29444925 PMCid:PMC5819352
  9. Azzo L, Ali YS, Vaqar S. Strategies For Individualizing Pulmonary Hypertension Treatment to Ensure Optimal Patient Outcomes. InStatPearls [Internet] 2022 Nov 7. StatPearls Publishing.
  10. Galiè N, Humbert M, Vachiery J-L, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: The joint task force for the diagnosis and treatment of pulmonary hypertension of the European society of cardiology (ESC) and the European respiratory society (ERS) endorsed by: Association for European paediatric and congenital cardiology (AEPC), international society for heart and lung transplantation (ISHLT). Eur Heart J. 2016;37(1):67-119. doi:10.1093/eurheartj/ehv317 PMid:26320113
  11. Sabbula BR, Akella J. Chronic Thromboembolic Pulmonary Hypertension. StatPearls Publishing; 2022.
  12. Cabrol S, Souza R, Jais X, Fadel E, Ali RHS, Humbert M, et al. Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2007;26(4): 357-62. doi:10.1016/j.healun.2006.12.014 PMid:17403477
  13. Islam AKMM. Cardiac myxomas: A narrative review. World J Cardiol. 2022;14(4):206-19. doi:10.4330/wjc.v14.i4.206 PMid:35582466 PMCid:PMC9048271
  14. Nguyen T, Vaidya Y. Atrial Myxoma. StatPearls Publishing; 2023.
  15. Medrek S, Safdar Z. Epidemiology and pathophysiology of chronic thromboembolic pulmonary hypertension: Risk factors and mechanisms. Methodist Debakey Cardiovasc J. 2016;12(4):195. doi:10.14797/mdcj-12-4-195 PMid:28289493 PMCid:PMC5344468