Chronic Thromboembolic Pulmonary Hypertension related to Splenectomy: A case report
Hipertensión pulmonar tromboembólica crónica relacionada con esplenectomía: Reporte de un caso
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Over the last decades, an association between splenectomy and the development of prothrombotic states has been suggested. These patients may be at increased risk for venous thromboembolic events, including pulmonary embolism. Nevertheless, most reported cases are from patients with hematologic conditions, which may be confounding factors because of these patients’ increased prothrombotic risk. Additionally, surgery after a traumatic abdominal injury may be relevant with thromboembolism as an early medical complication.
A 48-year-old man with a medical history of traumatic splenectomy 22 years ago presented to the emergency department with a one-year history of shortness of breath during exercise, fatigue, and presyncope. For the last two months, he experienced worsening dyspnea and dry cough. Hours prior to admission, he presented a syncopal event. A contrast-enhanced computed tomography showed extensive acute pulmonary embolism, and transesophageal echocardiography demonstrated a dilated right ventricle with a high probability of pulmonary arterial hypertension. He was discharged with warfarin and home-oxygen therapy. Four months later, an echocardiogram was performed, showing persistence of indirect signs of pulmonary hypertension. Perfusion scintigraphy demonstrated persistence of pulmonary embolism in the posterior apical segment of the left upper lobe and apical segment of the right upper lobe. The patient underwent right heart catheterization that showed chronic thromboembolic pulmonary hypertension. In the follow-up, he presented improvement of referred symptoms. Anticoagulation continued indefinitely.
Splenectomized patients have an increased incidence of venous thrombotic events and could potentially be at greater risk of developing chronic thromboembolic pulmonary hypertension.
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- Kimmig LM, Palevsky HI. Review of the association between splenectomy and chronic thromboembolic pulmonary hypertension. Ann Am Thorac Soc. 2016;13(6):945–54. doi: 10.1513/AnnalsATS.201512-826FR
- Long B, Koyfman A, Gottlieb M. Complications in the adult asplenic patient: A review for the emergency clinician. Am J Emerg Med. 2021;44:452-457. doi: 10.1016/j.ajem.2020.03.049
- Lin JN, Chen HJ, Lin MC, Lai CH, Lin HH, Yang CH, Kao CH. Risk of venous thromboembolism in patients with splenic injury and splenectomy. A nationwide cohort study. Thromb Haemost. 2016;115(1):176-83. doi: 10.1160/TH15-05-0381
- Palkar AV, Agrawal A, Verma S, Iftikhar A, Miller EJ, Talwar A. Post splenectomy related pulmonary hypertension. World J Respirol. 2015;5(2):69-77. doi: 10.5320/wjr.v5.i2.69
- Rottenstreich A, Kleinstern G, Spectre G, Da N, Ziv E, Kalish Y. Thromboembolic Events Following Splenectomy: Risk Factors, Prevention, Management and Outcomes. World J Surg. 2017;42(3):675-681. doi: 10.1007/s00268-017-4185-2
- Stamou K, Toutouzas K, Kekis P, Nakos S, Gafou A, Manouras A, Krespis E, Katsaragakis S, Bramis J. Prospective Study of the Incidence and Risk Factors of Postsplenectomy Thrombosis of the Portal, Mesenteric, and Splenic Veins. Arch Surg. 2006;141(7):663-669. doi: 10.1001/archsurg.141.7.663
- Jais X, Ioos V, Jardim C, Sitbon O, Parent F, Hamid A, et al. Splenectomy and chronic thromboembolic pulmonary hypertension. Thorax. 2005;60(12):1031–4. doi: 10.1136/thx.2004.038083
- Kristinsson SY, Gridley G, Hoover RN, Check D, Landgren O. Long-term risks after splenectomy among 8,149 cancer-free American veterans: a cohort study with up to 27 years follow-up. Haematologica. 2014; 99(2): 392-98. doi: 10.3324/haematol.2013.092460
- Thomsen RW, Schoonen WM, Farkas DK, Riis A, Fryzek JP, Sørensen HT. Risk of venous thromboembolism in splenectomized patients compared with the general population and appendectomized patients: a 10-year nationwide cohort study Thromb Haemost. 2010;8(6):1413-6. doi: 10.1111/j.1538-7836.2010.03849.x
- Kim NH, Delcroix M, Jais X, Madani MM, Matsubara H, Mayer E, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019; 53: 1801915. doi: 10.1111/j.1538-7836.2010.03849.x
- Moser KM, Bloor CM. Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest. 1993;103(3):685-92. doi: 10.1378/chest.103.3.685
- Zhang L, Yan P, Yang K, et al. Association between splenectomy and chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis. BMJ Open. 2021;11:e038385. doi: 10.1136/bmjopen-2020-038385
- Reiter CD, Wang X, Tanus-Santos JE, Hogg N, Cannon RO, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med. 2002;8(12):1383-9. doi: 10.1038/nm1202-799
- Amabile N, Guignabert C, Montani D, Yeghiazarians Y, Boulanger CM, Humbert M. Cellular microparticles in the pathogenesis of pulmonary hypertension. Eur Respir J. 2013;42(1):272-9. doi: 10.1183/09031936.00087212
- Rautou P, Mackman N. Deletion of microvesicles from the circulation. Circulation. 2012;125(13):1601-1604. doi: 10.1161/CIRCULATIONAHA.112.094920
- Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treat- ment 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 Respir J. 2016;37(1): 67-119. doi: 10.1093/eurheartj/ehv317
- Madani MM. Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: state-of-the-art 2020. Pulm Circ. 2021;11(2):20458940211007372. doi: 10.1177/20458940211007372
- Madani MM. Surgical treatment of chronic thromboembolic pulmonary hypertension: pulmonary thromboendarterectomy. Methodist Debakey Cardiovasc J. 2016;12(4):213-218. doi: 10.14797/mdcj-12-4-213
- Mayer E, Jenkins D, Lindner J, D’Armini A, Kloek J, Meyns B, Ilkjaer LB, Klepetko W, Delcroix M, Lang I, Pepke-Zaba J, Simonneau G, Dartevelle P. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011;141(3):702-10. doi: 10.1016/j.jtcvs.2010.11.024
- Madani MM, Auger WR, Pretorius V, Sakakibara N, Kerr KM, Kim NH, Fedullo PF,Jamieson SW. Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2,700 patients. Ann Thorac Surg. 2012;94(1):97-103. doi: 10.1016/j.athoracsur.2012.04.004
- Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011;124(18): 1973-1981. doi: 10.1161/CIRCULATIONAHA.110.015008
- Araszkiewicz A, Darocha S, Pietrasik A, et al. Balloon pulmonary angioplasty for the treatment of residual or recurrent pulmonary hypertension after pulmonary endarterectomy. Int J Cardiol. 2019; 278: 232-237. doi: 10.1016/j.ijcard.2018.10.066
- Ghofrani HA, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013; 369: 319-329. doi: 10.1056/NEJMoa1209657
- Konstantinides SV, Meyer G, Becattini C, et al. 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS). Eur Respir J. 2019; 54(3):1901647. doi: 10.1183/13993003.01647-2019
- Gabriel L, Delavenne X, Bedouch P et. al. Risk of Direct Oral Anticoagulant Bioaccumulation in Patients with Pulmonary Hypertension. Respiration. 2016;91(4):307-315. doi: 10.1159/000445122
- Bertoletti L, Mismetti V, Giannakoulas G. Use of anticoagulants in patients with pulmonary hypertension. Hamostaseologie 2020;40(3):348-355. doi: 10.1055/a-1171-3995
- Sena S, Bulent M, Derya K et. al. Real-life data of direct anticoagulant use, bleeding risk and venous thromboembolism recurrence in chronic thromboembolic pulmonary hypertension patients: an observational retrospective study. Pulm Circ. 2020;10(1):1-10. doi: 10.1177/2045894019873545
- Gavilanes F, Alves J, Fernandes C, et al. Use of direct oral anticoagulants for chronic thromboembolic pulmonary hypertension. Clinics (Sao Paulo). 2018;73:e216. doi: 10.6061/clinics/2018/e216
- Bunclark K, Newnham M, Chiu YD, et. al. A multicenter study of anticoagulation inoperable chronic thromboembolic pulmonary hypertension. J Thromb Haemost. 2020;18(1):114-122. doi: 10.1111/jth.14649
- Sedhom R, Megaly M, Gupta E et. al. Use of direct oral anticoagulants in chronic thromboembolic pulmonary hypertension: a systematic review. J Thromb Thrombolysis. 2021. doi: 10.1007/s11239-021-02501-8