Severe Pulmonary Hypertension Secondary to Concomitant Mitral Stenosis with Veno-occlusive Disease in the Context of Systemic Sclerosis: Importance of Careful and Comprehensive Assessment

Mina Boutgourine

Cardiology Department, Mohammed VI University Hospital, Marrakech, Morocco.

Hind Nabawi *

Cardiology Department, Mohammed VI University Hospital, Marrakech, Morocco.

Bouchra Maatof

Cardiology Department, Mohammed VI University Hospital, Marrakech, Morocco.

Mohammed El-Jamili

Cardiology Department, Mohammed VI University Hospital, Marrakech, Morocco.

Saloua El-Karimi

Cardiology Department, Mohammed VI University Hospital, Marrakech, Morocco.

Mustapha El Hattaoui

Cardiology Department, Mohammed VI University Hospital, Marrakech, Morocco.

*Author to whom correspondence should be addressed.


Abstract

Pulmonary Arterial Hypertension (PAH) is a clinical syndrome consisting of physiologic/ hemodynamic criteria that are a consequence of several etiologies. Confirmation of pulmonary hypertension is based on right heart catheterization.

Pulmonary hypertension is a devastating condition that can lead to considerable morbidity and premature mortality. In the last few decades, significant advancement in the pharmacotherapy of pulmonary hypertension has resulted from better understanding of the complex pathogenesis and pathophysiology of this dreaded disease. Despite these accomplishments, pharmacotherapy of pulmonary hypertension is still far from perfect, and the mortality in this modern treatment era is still unacceptably high.

We report a complex clinical presentation characterized by severe pulmonary hypertension secondary to concomitant mitral stenosis with veno-occlusive disease in the context of systemic sclerosis.

Our case highlights the importance of a systematic and comprehensive diagnostic approach to avoid missing an underlying pathology.

Keywords: Pulmonary arterial hypertension, pulmonary veno-occlusive disease, systemic sclerosis, mitral stenosis, etiological diagnosis, right heart catheterization


How to Cite

Boutgourine , Mina, Hind Nabawi, Bouchra Maatof, Mohammed El-Jamili, Saloua El-Karimi, and Mustapha El Hattaoui. 2023. “Severe Pulmonary Hypertension Secondary to Concomitant Mitral Stenosis With Veno-Occlusive Disease in the Context of Systemic Sclerosis: Importance of Careful and Comprehensive Assessment”. Cardiology and Angiology: An International Journal 12 (4):162-70. https://doi.org/10.9734/ca/2023/v12i4355.

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References

Hoeper M, Bogaard H, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25):D42–D50.

Lefèvre G, Dauchet L, Hachulla E, et al. Survival and prognostic factors in systemic sclerosis-associated pul- monary hypertension: A systematic review and meta-analysis. ArthritisRheum. 2013;65(9):2412–2423.

Boucly A, Girerd B, Bourlier D, Nemlaghi S, Caliez J, Savale L et al. Pulmonaryveno-occlusive disease. Journal of RespiratoryDiseases. 2018;35:160-170.

Galiè N, Hoeper M, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009; 34:1219-63.

Galiè N, Hoeper M, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The 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 the international society of heart and lung transplantation (ISHLT). Eur Heart J. 2009;30:2493- 537.

Montani D, Price LC, Dorfmuller P, et al. Pulmonary veno-occlusive disease. EurRespir J. 2009;33:189—200.

Montani D, Lau EM, Dorfmüller P, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2016; 47:1518—34.

Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. EurRespir J. 2019;53(1):1801913. DOI : 10.1183/13993003.01913-2018 .

Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25):D34–D41. DOI: 10.1016/j.jacc.2013.10.029 .

Chennakesavulu PV, Uppaluri S, Koyi J, et al. Pulmonary Hypertension in Scleroderma– Evaluation and Management. Disease-a-Month. 2022; 101468. DOI: 10.1016/j.disamonth.2022.101468

Humbert M, Gabor Kovacs G, Hoeper M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension developed by the 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 the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). European Heart Journal. 2022;43:3618–3731.

Mukerjee D, George DS, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis-associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62:1088–93.

Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: A French nationwide prospective multicenter study. Arthritis Rheum. 2005;52:3792-800.

Lechartier B, Humbert M. Pulmonary arterial hypertension in systemic sclerosis.Presse Med. 2021 Apr;50(1):104062

Hora J. Zurhistologie der klinischen "primarenpulmonalsklerose". Frankfurt Z Pathol, 1934;47:100-108.

O'Callaghan D, Dorfmuller P,Jaïs X, Mouthon L, Sitbon O, Simonneau G, et al . Pulmonary veno-occlusive disease: the bête noire of pulmonary hypertension in connective tissue diseases?. Presse Med. 2011;40:e87–e100.

Montani D, Achouh L, Dorfmuller P et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore). 2008;87:220-33.

Lau EMT, Manes A, Celermajer DS, et al. Early detection ofpulmonary vascular disease in pulmonary arterial hypertension:Time to move forward. Eur Heart J. 2011;32:2489-98.

Best DH, Sumner KL, Austin ED, et al. EIF2AK4 mutations inpulmonary capillary hemangiomatosis. Chest. 2014;145: 231-6.

Resten A, Maitre S, Humbert M, et al. Pulmonary hypertension:CT of the chest in pulmonary venoocclusive disease. AJR Am JRoentgenol. 2004;183:65—70.

Laveneziana P, Montani D, Dorfmüller P, et al. Mechanisms ofexertionaldyspnoea in pulmonary veno-occlusive disease withEIF2AK4 mutations. EurRespir J. 2014;44:1069-72.

Montani D, Girerd B, Jaïs X, et al. Clinical phenotypesand outcomes of heritable and sporadic pulmonary veno-occlusive disease: A population-based study. Lancet Respir Med. 2017;5:125-34.

Montani D, Sitbon O, Jaïs X, et al. Treatment of pulmonaryarterial hypertension. Presse Med. 2005;34:1445-55.

Humbert M, Maître S, Capron F, et al. Pulmonary edemacomplicating continuous intravenous prostacyclin in pulmo-nary capillary hemangiomatosis. Am J Respir Crit Care Med. 1998;157: 1681—5.