https://doi.org/10.4081/ecj.2020.8310
Pulmonary hypertension: when the acute event leads to diagnosis. Case report in a patient with Ehlers-Danlos syndrome.
Submitted: 27 May 2019
Accepted: 17 January 2020
Published: 17 March 2020
Accepted: 17 January 2020
Abstract Views: 1788
PDF: 491
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a pulmonary vascular pathology caused by the chronic obstruction of the major pulmonary arteries, usually being the consequence of recurrent episodes of pulmonary embolism. Such events are usually unknown; the delay in such a diagnosis can therefore lead to a deterioration of the clinical picture, worsening the overall prognosis. This is a case of a 55-year-old man who came to the Emergency Room (ER) because of an acute exacerbation of chronic dyspnea, that he experienced for several years, in the absence of an explanatory diagnosis. Acute pulmonary embolism was diagnosed in the setting of the ER, with bedside echography resulting to be crucial to the work up. A multidisciplinary approach allowed proper treatment, management and a favourable outcome.
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
J. Pepke-Zaba, M. Delcroix, I. Lang, E. Mayer, P. Jansa, D. Ambroz, C. Treacy, A.M. D'Armini, M. Morsolini, R. Snijder, et al., Chronic thromboembolic pulmonary hypertension (cteph): results from an international prospective registry, Circulation; 124 (18) (2011) 1973–1981.
Lang IM. Chronic thromboembolic pulmonary hypertension: not so rare after all. N Engl J Med 2004;350:2236-8.
DOI: https://doi.org/10.1056/NEJMp048088
L. Guérin, F. Couturaud, F. Parent, M.P. Revel, F. Gillaizeau, B. Planquette, D. Pontal, M. Guégan, G. Simonneau, G. Meyer, et al., Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of cteph after pulmonary embolism, Thromb. Haemost. 2014; 112 (3):598–605.
DOI: https://doi.org/10.1160/TH13-07-0538
Andrea Maria D’Armini, Giorgio Zanotti et al.; Terapia chirurgica dell’ipertensione polmonare cronica tromboembolica mediante endoarteriectomia polmonare; G Ital Cardiol 2006; 7 (7): 454-463
Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, Albanese P, Biasiolo A, Pegoraro C, Iliceto S, Prandoni P. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004;350: 2257–2264.
DOI: https://doi.org/10.1056/NEJMoa032274
Escribano-Subias P, Blanco I, Lopez-Meseguer M, Lopez-Guarch CJ, Roman A, Morales P, Castillo-Palma MJ, Segovia J, Gomez-Sanchez MA, Barbera JA. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J 2012;40:596–603
DOI: https://doi.org/10.1183/09031936.00101211
Delcroix M, Vonk Noordegraaf A, Fadel E, et al. Vascular and right ventricular remodelling in chronic thromboembolic pulmonary hypertension. Eur Respir J 2013; 41: 224–232.
DOI: https://doi.org/10.1183/09031936.00047712
Van de Veerdonk MC, Bogaard HJ, Voelkel NF. The right ventricle and pulmonary hypertension. Heart Fail Rev 2016; 21: 259–271.
DOI: https://doi.org/10.1007/s10741-016-9526-y
Torbicki A, Galie N, Covezzoli A, Rossi E, De Rosa M, Goldhaber SZ. Right heart thrombi in pulmonary embolism: results from the International Cooperative Pulmonary Embolism Registry. J Am Coll Cardiol. 2003;41:2245e2251.
DOI: https://doi.org/10.1016/S0735-1097(03)00479-0
A. Z. Schwartzbard, P. A. Tunick, B. P. Rosenzweig, and I. Kronzon, “The role of transesophageal echocardiography in the diagnosis and treatment of right atrial thrombi,” Journal of the American Society of Echocardiography, 1999; 12(1):64–69,
DOI: https://doi.org/10.1016/S0894-7317(99)70174-4
P. Pruszczyk, A. Torbicki, R. Pacho et al., “Noninvasive diagnosis of suspected severe pulmonary embolism: TTE versus spiral CT,” Chest 1997; 112, (3):722–728,
DOI: https://doi.org/10.1378/chest.112.3.722
Finlayson GN. Right heart thrombi: consider the cause. Can J Cardiol. 2008;24:888
DOI: https://doi.org/10.1016/S0828-282X(08)70713-X
Life-Threatening Acute on Chronic Pulmonary Thromboembolism Requiring Extracorporeal Membrane Oxygenation, Heemoon Lee MD, Korean J Thorac Cardiovasc Surg 2018;51:205-208
DOI: https://doi.org/10.5090/kjtcs.2018.51.3.205
Connective Tissue and Its Heritable Disorders: Molecular, Genetic, and Medical Aspects (5th edition), Edited by Peter M. Royce and Beat Steinmann. New York: Wiley-Liss, 2002, pp. 431-523
Asherson RA, Bosman C, Tikly M, et al. Ehlers-Danlos syndrome type IV in a young man. J Rheumatol 2006;33:2091–6.
Recurrent venous thrombosis in Ehlers-Danlos syndrome type III: an atypical manifestation; Jiménez- Encarnación E, Vilá LM. BMJ Case Rep doi:10.1136/bcr-2013-008922
DOI: https://doi.org/10.1136/bcr-2013-008922
How to Cite
Secco, G., Falchi, A. G., Salinaro, F., Blatti, C., Giacomuzzi Moore, B., & Perlini, S. (2020). Pulmonary hypertension: when the acute event leads to diagnosis. Case report in a patient with Ehlers-Danlos syndrome. Emergency Care Journal, 16(1). https://doi.org/10.4081/ecj.2020.8310
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
