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Review Article| Volume 19, ISSUE 1, P25-33, January 2023

Unusual Forms of Pulmonary Hypertension

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      References

        • Simonneau G.
        • Montani D.
        • Celermajer D.S.
        • et al.
        Haemodynamic definitions and updated clinical classification of pulmonary hypertension.
        Eur Respir J. 2019; 53https://doi.org/10.1183/13993003.01913-2018
        • Gavilanes F.
        • Fernandes C.J.
        • Souza R.
        Pulmonary arterial hypertension in schistosomiasis.
        Curr Opin Pulm Med. 2016; 22: 408-414
        • Gryseels B.
        • Polman K.
        • Clerinx J.
        • et al.
        Human schistosomiasis.
        Lancet. 2006; 368: 1106-1118
        • Colley D.G.
        • Bustinduy A.L.
        • Secor W.E.
        • et al.
        Human schistosomiasis.
        Lancet. 2014; 383: 2253-2264
        • Lapa M.
        • Dias B.
        • Jardim C.
        • et al.
        Cardiopulmonary Manifestations of Hepatosplenic Schistosomiasis.
        Circulation. 2009; 119: 1518-1523
        • Hatz C.
        • Jenkins J.M.
        • Ali Q.M.
        • et al.
        A review of the literature on the use of ultrasonography in schistosomiasis with special reference to its use in field studies. 2. Schistosoma mansoni.
        Acta Trop. 1992; 51: 15-28
        • Sibomana J.P.
        • Campeche A.
        • Carvalho-Filho R.J.
        • et al.
        Schistosomiasis Pulmonary Arterial Hypertension.
        Front Immunol. 2020; 11: 608883
        • Hoette S.
        • Figueiredo C.
        • Dias B.
        • et al.
        Pulmonary artery enlargement in schistosomiasis associated pulmonary arterial hypertension.
        BMC Pulm Med. 2015; 15: 118
        • Mauad T.
        • Pozzan G.
        • Lancas T.
        • et al.
        Immunopathological aspects of schistosomiasis-associated pulmonary arterial hypertension.
        J Infect. 2014; 68: 90-98
        • dos Santos Fernandes C.J.
        • Jardim C.V.
        • Hovnanian A.
        • et al.
        Survival in schistosomiasis-associated pulmonary arterial hypertension.
        J Am Coll Cardiol. 2010; 56: 715-720
        • Fernandes C.
        • Dias B.A.
        • Jardim C.V.P.
        • et al.
        The role of target therapies in schistosomiasis-associated pulmonary arterial hypertension.
        Chest Apr. 2012; 141: 923-928
        • Fernandes C.J.C.
        • Piloto B.
        • Castro M.
        • et al.
        Survival of patients with schistosomiasis-associated pulmonary arterial hypertension in the modern management era.
        Eur Respir J. 2018; 51https://doi.org/10.1183/13993003.00307-2018
        • Fernandes C.J.
        • Calderaro D.
        • Assad A.P.L.
        • et al.
        Update on the Treatment of Pulmonary Arterial Hypertension.
        Arq Bras Cardiol. 2021; 117 (Atualizacao no Tratamento da Hipertensao Arterial Pulmonar): 750-764
        • Baughman R.P.
        • Field S.
        • Costabel U.
        • et al.
        Sarcoidosis in America. Analysis Based on Health Care Use.
        Ann Am Thorac Soc. 2016; 13: 1244-1252
        • Belperio J.A.
        • Shaikh F.
        • Abtin F.G.
        • et al.
        Diagnosis and Treatment of Pulmonary Sarcoidosis: A Review.
        JAMA. 1 2022; 327: 856-867
        • Kirkil G.
        • Lower E.E.
        • Baughman R.P.
        Predictors of Mortality in Pulmonary Sarcoidosis.
        Chest. 2018; 153: 105-113
        • Jeny F.
        • Uzunhan Y.
        • Lacroix M.
        • et al.
        Predictors of mortality in fibrosing pulmonary sarcoidosis. Respiratory medicine.
        Aug. 2020; 169: 105997https://doi.org/10.1016/j.rmed.2020.105997
        • Bourbonnais J.M.
        • Samavati L.
        Clinical predictors of pulmonary hypertension in sarcoidosis.
        Eur Respir J Aug. 2008; 32: 296-302
        • Shlobin O.A.
        • Kouranos V.
        • Barnett S.D.
        • et al.
        Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry.
        Eur Respir J. 2020; 55https://doi.org/10.1183/13993003.01747-2019
        • Shorr A.F.
        • Helman D.L.
        • Davies D.B.
        • et al.
        Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics.
        Eur Respir J. 2005; 25: 783-788
        • Huitema M.P.
        • Bakker A.L.M.
        • Mager J.J.
        • et al.
        Prevalence of pulmonary hypertension in pulmonary sarcoidosis: the first large European prospective study.
        Eur Respir J. 2019; 54https://doi.org/10.1183/13993003.00897-2019
        • Pabst S.
        • Hammerstingl C.
        • Grau N.
        • et al.
        Pulmonary arterial hypertension in patients with sarcoidosis: the Pulsar single center experience.
        Adv Exp Med Biol. 2013; 755: 299-305
        • Bandyopadhyay D.
        • Humbert M.
        An update on sarcoidosis-associated pulmonary hypertension.
        Curr Opin Pulm Med. 2020; 26: 582-590
        • Nunes H.
        • Humbert M.
        • Capron F.
        • et al.
        Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis.
        Thorax. 2006; 61: 68-74
        • Baughman R.P.
        • Shlobin O.A.
        • Wells A.U.
        • et al.
        Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry.
        Respir Med. 2018; 139: 72-78
        • Ozyilmaz E.
        • Akilli R.
        • Berk I.
        • et al.
        The frequency of diastolic dysfunction in patients with sarcoidosis and it's relationship with HLA DRB1∗ alleles.
        Sarcoidosis Vasculitis Diffuse Lung Dis. 2019; 36: 285-293
        • Ungprasert P.
        • Crowson C.S.
        • Matteson E.L.
        Association of Sarcoidosis With Increased Risk of VTE: A Population-Based Study, 1976 to 2013.
        Chest. 2017; 151: 425-430
        • Savale L.
        • Huitema M.
        • Shlobin O.
        • et al.
        WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension.
        Eur Respir Rev. 2022; : 31https://doi.org/10.1183/16000617.0165-2021
        • Perlman D.M.
        • Sudheendra M.T.
        • Furuya Y.
        • et al.
        Clinical Presentation and Treatment of High-Risk Sarcoidosis.
        Ann Am Thorac Soc. 2021; 18: 1935-1947
        • Keir G.J.
        • Walsh S.L.
        • Gatzoulis M.A.
        • et al.
        Treatment of sarcoidosis-associated pulmonary hypertension: A single centre retrospective experience using targeted therapies.
        Sarcoidosis Vasculitis Diffuse Lung Dis. 2014; 31: 82-90
        • Boucly A.
        • Cottin V.
        • Nunes H.
        • et al.
        Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension.
        Eur Respir J. 2017; 50https://doi.org/10.1183/13993003.00465-2017
        • Baughman R.P.
        • Shlobin O.A.
        • Gupta R.
        • et al.
        Riociguat for Sarcoidosis-Associated Pulmonary Hypertension: Results of a 1-Year Double-Blind, Placebo-Controlled Trial.
        Chest. 2022; 161: 448-457
        • Waxman A.
        • Restrepo-Jaramillo R.
        • Thenappan T.
        • et al.
        Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease.
        N Engl J Med. 2021; 384: 325-334
        • Gunther S.
        • Perros F.
        • Rautou P.E.
        • et al.
        Understanding the Similarities and Differences between Hepatic and Pulmonary Veno-Occlusive Disease.
        Am J Pathol. 2019; 189: 1159-1175
        • 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: 220-233
        • Montani D.
        • Lau E.M.
        • Descatha A.
        • et al.
        Occupational exposure to organic solvents: a risk factor for pulmonary veno-occlusive disease.
        Eur Respir J. 2015; 46: 1721-1731
        • Laveneziana P.
        • Montani D.
        • Dorfmuller P.
        • et al.
        Mechanisms of exertional dyspnoea in pulmonary veno-occlusive disease with EIF2AK4 mutations.
        Eur Respir J. 2014; 44: 1069-1072
        • Weatherald J.
        • Dorfmuller P.
        • Perros F.
        • et al.
        Pulmonary capillary haemangiomatosis: a distinct entity?.
        Eur Respir Rev : official J Eur Respir Soc. 2020; : 29https://doi.org/10.1183/16000617.0168-2019
        • Ogawa A.
        • Sakao S.
        • Tanabe N.
        • et al.
        Use of vasodilators for the treatment of pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: A systematic review.
        Respir Investig. 2019; 57: 183-190
        • Montani D.
        • Girerd B.
        • Jais X.
        • et al.
        Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study.
        Lancet Respir Med. 2017; 5: 125-134
        • Emile J.F.
        • Cohen-Aubart F.
        • Collin M.
        • et al.
        Histiocytosis.
        Lancet. 2021; 398: 157-170
        • Radzikowska E.
        Update on Pulmonary Langerhans Cell Histiocytosis.
        Front Med (Lausanne). 2020; 7: 582581
        • Heiden G.I.
        • Sobral J.B.
        • Freitas C.S.G.
        • et al.
        Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis.
        Chest. 2020; 158: 2440-2448
        • Dauriat G.
        • Mal H.
        • Thabut G.
        • et al.
        Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis.
        Transplantation. 2006; 81: 746-750
        • Fartoukh M.
        • Humbert M.
        • Capron F.
        • et al.
        Severe pulmonary hypertension in histiocytosis X.
        Am J Respir Crit Care Med. 2000; 161: 216-223
        • Le Pavec J.
        • Lorillon G.
        • Jais X.
        • et al.
        Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies.
        Chest. 2012; 142: 1150-1157
        • Kavanagh P.L.
        • Fasipe T.A.
        • Wun T.
        Sickle Cell Disease: A Review.
        JAMA. 2022; 328: 57-68
        • Piel F.B.
        • Hay S.I.
        • Gupta S.
        • et al.
        Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions.
        Plos Med. 2013; 10: e1001484
        • Mehari A.
        • Klings E.S.
        Chronic Pulmonary Complications of Sickle Cell Disease.
        Chest. 2016; 149: 1313-1324
        • Alves J.L.
        • Oleas F.G.
        • Souza R.
        Pulmonary Hypertension: Definition, Classification, and Diagnosis.
        Semin Respir Crit Care Med. 2017; 38: 561-570
        • Parent F.
        • Bachir D.
        • Inamo J.
        • et al.
        A hemodynamic study of pulmonary hypertension in sickle cell disease.
        N Engl J Med. 2011; 365: 44-53
        • Fonseca G.H.
        • Souza R.
        • Salemi V.M.
        • et al.
        Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease.
        Eur Respir J. 2012; 39: 112-118
        • Mehari A.
        • Gladwin M.T.
        • Tian X.
        • et al.
        Mortality in adults with sickle cell disease and pulmonary hypertension.
        JAMA. 2012; 307: 1254-1256
        • Gladwin M.T.
        • Sachdev V.
        • Jison M.L.
        • et al.
        Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.
        N Engl J Med. 2004; 350: 886-895
        • Carstens G.R.
        • Paulino B.B.A.
        • Katayama E.H.
        • et al.
        Clinical relevance of pulmonary vasculature involvement in sickle cell disease.
        Br J Haematol. 2019; 185: 317-326
        • Ribeiro de Campos P.T.
        • Lopes A.A.
        • Issa V.S.
        • et al.
        Morphologic and immunohistochemical features of pulmonary vasculopathy in end-stage left ventricular systolic failure.
        J Heart Lung Transplant. 2018; 37: 422-425
        • Wood K.C.
        • Gladwin M.T.
        • Straub A.C.
        Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure.
        Heart. 2020; 106: 562-568
        • Klings E.S.
        • Machado R.F.
        • Barst R.J.
        • et al.
        An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.
        Am J Respir Crit Care Med. 2014; 189: 727-740
        • Machado R.F.
        • Barst R.J.
        • Yovetich N.A.
        • et al.
        Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity.
        Blood. 2011; 118: 855-864
        • Cramer-Bour C.
        • Ruhl A.P.
        • Nouraie S.M.
        • et al.
        Long-term tolerability of phosphodiesterase-5 inhibitors in pulmonary hypertension of sickle cell disease.
        Eur J Haematol. 2021; 107: 54-62
        • Barst R.J.
        • Mubarak K.K.
        • Machado R.F.
        • et al.
        Exercise capacity and haemodynamics in patients with sickle cell disease with pulmonary hypertension treated with bosentan: results of the ASSET studies.
        Br J Haematol. 2010; 149: 426-435