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Review Article| Volume 19, ISSUE 1, P1-9, January 2023

Mildly Elevated Pulmonary Hypertension

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      References

        • Galiè N.
        • Humbert M.
        • Vachiery J.L.
        • 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: 67-119
        • Hatano S.
        • Strasser T.
        • Organization W.H.
        Primary pulmonary hypertension: Report on a WHO meeting.
        World Health Organization, Geneva1975: 15
        • Simonneau G.
        • Montani D.
        • Celermajer D.S.
        • et al.
        Haemodynamic definitions and updated clinical classification of pulmonary hypertension.
        Eur Respir J. 2019; 53: 1801913
        • Mercurio V.
        • Bianco A.
        • Campi G.
        • et al.
        New Drugs, Therapeutic Strategies, and Future Direction for the Treatment of Pulmonary Arterial Hypertension.
        Curr Med Chem. 2019; 26: 2844-2864
        • Valerio C.J.
        • Schreiber B.E.
        • Handler C.E.
        • et al.
        Borderline mean pulmonary artery pressure in patients with systemic sclerosis: Transpulmonary gradient predicts risk of developing pulmonary hypertension.
        Arthritis Rheum. 2013; 65: 1074-1084
        • Kovacs G.
        • Maier R.
        • Aberer E.
        • et al.
        Borderline Pulmonary Arterial Pressure Is Associated with Decreased Exercise Capacity in Scleroderma.
        Am J Respir Crit Care Med. 2009; 180: 881-886
        • Bae S.
        • Saggar R.
        • Bolster M.B.
        • et al.
        Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry.
        Ann Rheum Dis. 2012; 71: 1335-1342
        • Stamm A.
        • Saxer S.
        • Lichtblau M.
        • et al.
        Exercise pulmonary haemodynamics predict outcome in patients with systemic sclerosis.
        Eur Respir J. 2016; 48: 1658-1667
        • Kovacs G.
        • Berghold A.
        • Scheidl S.
        • et al.
        Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review.
        Eur Respir J. 2009; 34: 888-894
        • Kovacs G.
        • Olschewski H.
        Debating the new haemodynamic definition of pulmonary hypertension: Much ado about nothing?.
        Eur Respir J. 2019; 54: 10-12
        • Brusca S.B.
        • Zou Y.
        • Elinoff J.M.
        How low should we go? Potential benefits and ramifications of the pulmonary hypertension hemodynamic definitions proposed by the 6th World Symposium.
        Curr Opin Pulm Med. 2020; 26: 384-390
        • Gibbs J.S.R.
        • Torbicki A.
        Proposed new pulmonary hypertension definition: is 4 mm(Hg) worth re-writing medical textbooks?.
        Eur Respir J. 2019; 53: 1900197
        • Hoeper M.M.
        • Humbert M.
        The new haemodynamic definition of pulmonary hypertension: Evidence prevails, finally.
        Eur Respir J. 2019; 53: 3-6
        • Kolte D.
        • Lakshmanan S.
        • Jankowich M.D.
        • et al.
        Mild pulmonary hypertension is associated with increased mortality: A systematic review and meta-analysis.
        J Am Heart Assoc. 2018; 7: 1-13
        • Xue L.
        • Yang Y.
        • Sun B.
        • et al.
        Mildly Elevated Pulmonary Arterial Pressure Is Associated With a High Risk of Progression to Pulmonary Hypertension and Increased Mortality: A Systematic Review and Meta-Analysis.
        J Am Heart Assoc. 2021; 10: e018374
        • Jaafar S.
        • Visovatti S.
        • Young A.
        • et al.
        Impact of the revised haemodynamic definition on the diagnosis of pulmonary hypertension in patients with systemic sclerosis.
        Eur Respir J. 2019; 54: 1900586
        • Vachiéry J.L.
        • Tedford R.J.
        • Rosenkranz S.
        • et al.
        Pulmonary hypertension due to left heart disease.
        Eur Respir J. 2019; 53: 1801897
        • Kovacs G.
        • Olschewski A.
        • Berghold A.
        • et al.
        Pulmonary vascular resistances during exercise in normal subjects: a systematic review.
        Eur Respir J. 2012; 39: 319-328
        • Hoeper M.M.
        • Bogaard H.J.
        • Condliffe R.
        • et al.
        Definitions and Diagnosis of Pulmonary Hypertension.
        J Am Coll Cardiol. 2013; 62: D42-D50
        • Kovacs G.
        • Zeder K.
        • Rosenstock P.
        • et al.
        Clinical Impact of the New Definition of Precapillary Pulmonary Hypertension.
        Chest. 2021; 159: 1995-1997
        • Nagel C.
        • Marra A.M.
        • Benjamin N.
        • et al.
        Reduced Right Ventricular Output Reserve in Patients With Systemic Sclerosis and Mildly Elevated Pulmonary Artery Pressure.
        Arthritis Rheum. 2019; 71: 805-816
        • Torbicki A.
        Definition of pulmonary hypertension challenged?.
        Nat Rev Cardiol. 2016; 13: 250-251
        • Tedford R.J.
        • Beaty C.A.
        • Mathai S.C.
        • et al.
        Prognostic value of the pre-transplant diastolic pulmonary artery pressure–to–pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension.
        J Hear Lung Transpl. 2014; 33: 289-297
        • Maron B.A.
        • Hess E.
        • Maddox T.M.
        • et al.
        Association of Borderline Pulmonary Hypertension With Mortality and Hospitalization in a Large Patient Cohort: Insights From the Veterans Affairs Clinical Assessment, Reporting, and Tracking Program.
        Circulation. 2016; 133: 1240-1248
        • Douschan P.
        • Kovacs G.
        • Avian A.
        • et al.
        Mild elevation of pulmonary arterial pressure as a predictor of mortality.
        Am J Respir Crit Care Med. 2018; 197: 509-516
        • Coghlan J.G.
        • Wolf M.
        • Distler O.
        • et al.
        Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis.
        Eur Respir J. 2018; 51: 1701197
        • Assad T.R.
        • Maron B.A.
        • Robbins I.M.
        • et al.
        Prognostic effect and longitudinal hemodynamic assessment of borderline pulmonary hypertension.
        JAMA Cardiol. 2017; 2: 1361-1368
        • Kovacs G.
        • Avian A.
        • Tscherner M.
        • et al.
        Characterization of Patients With Borderline Pulmonary Arterial Pressure.
        Chest. 2014; 146: 1486-1493
        • Xanthouli P.
        • Jordan S.
        • Milde N.
        • et al.
        Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension.
        Ann Rheum Dis. 2020; 79: 370-378
        • Heresi G.A.
        • Aytekin M.
        • Hammel J.P.
        • et al.
        Plasma interleukin-6 adds prognostic information in pulmonary arterial hypertension.
        Eur Respir J. 2014; 43: 912-914
        • Nemoto K.
        • Oh-Ishi S.
        • Akiyama T.
        • et al.
        Borderline pulmonary hypertension is associated with exercise intolerance and increased risk for acute exacerbation in patients with interstitial lung disease.
        BMC Pulm Med. 2019; 19: 1-7
        • Simonneau G.
        • Hoeper M.M.
        The revised definition of pulmonary hypertension: Exploring the impact on patient management.
        Eur Hear J Suppl. 2019; 21: K4-K8
        • Swietlik E.M.
        • Ruggiero A.
        • Fletcher A.J.
        • et al.
        Limitations of resting haemodynamics in chronic thromboembolic disease without pulmonary hypertension.
        Eur Respir J. 2019; 53: 1801787
        • Taboada D.
        • Pepke-Zaba J.
        • Jenkins D.P.
        • et al.
        Outcome of pulmonary endarterectomy in symptomatic chronic thromboembolic disease.
        Eur Respir J. 2014; 44: 1635-1645
        • Kiely D.G.
        • Lawrie A.
        • Humbert M.
        Screening strategies for pulmonary arterial hypertension.
        Eur Heart J Suppl. 2019; 21: K9-K20
        • Attanasio U.
        • Cuomo A.
        • Pirozzi F.
        • et al.
        Pulmonary Hypertension Phenotypes in Systemic Sclerosis: The Right Diagnosis for the Right Treatment.
        Int J Mol Sci. 2020; 21: 4430
        • Visovatti S.H.
        • Distler O.
        • Coghlan J.G.
        • et al.
        Borderline pulmonary arterial pressure in systemic sclerosis patients: a post-hoc analysis of the DETECT study.
        Arthritis Res Ther. 2014; 16: 493
        • Sarı A.
        • Şener Y.Z.
        • Armağan B.
        • et al.
        How did the updated hemodynamic definitions affect the frequency of pulmonary hypertension in patients with systemic sclerosis?.
        Anatol J Cardiol. 2021; 25: 30-35
        • Pan Z.
        • Marra A.M.
        • Benjamin N.
        • et al.
        Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group study (EDITA study).
        Arthritis Res Ther. 2019; 21: 217
        • Saggar R.
        • Khanna D.
        • Shapiro S.
        • et al.
        Brief Report: Effect of ambrisentan treatment on exercise-induced pulmonary hypertension in systemic sclerosis: A prospective single-center, open-label pilot study.
        Arthritis Rheum. 2012; 64: 4072-4077
        • Kovacs G.
        • Maier R.
        • Aberer E.
        • et al.
        Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure.
        Arthritis Rheum. 2012; 64: 1257-1262