Skip to main content
Erschienen in: Heart Failure Reviews 3/2016

05.10.2015

Definition, epidemiology and registries of pulmonary hypertension

verfasst von: R. Awdish, H. Cajigas

Erschienen in: Heart Failure Reviews | Ausgabe 3/2016

Einloggen, um Zugang zu erhalten

Abstract

Pulmonary arterial hypertension (PAH) is a subcategory of pulmonary hypertension (PH) that comprises a group of disorders with similar pulmonary vascular pathology. Though PH is common, the estimated incidence of IPAH is 1–3 cases per million, making it a rare disease. The hemodynamic definition of PAH is a mean pulmonary artery pressure at rest >OR = 25 mm Hg in the presence of a pulmonary capillary wedge pressure <OR = 15 mmHg with pulmonary vascular resistance (PVR) greater than 3 WU. Specific maneuvers during right heart catheterization can be utilized to disclose vasoreactivity and heart failure with preserved ejection fraction, which have implications for management. The inherent complexity in studying a rare disease that exhibits clinical overlap with a common syndrome necessitated the creation of registries. These registries have been indispensable in the characterization and mapping of the natural history of the disease. Equations and risk calculators derived from registries have given clinicians a basis for risk stratification and prognostication. The sequential accumulation of data since the registries began in the 1980s allows for comparisons to be made. Patients who are differentiated by treatment eras and environments can be contrasted. Variability among inclusion criteria similarly allows for comparisons of these subpopulations. This article provides an overview of available registries, highlights insights provided by each and discusses key issues around the interpretation and extrapolation of data from PAH registries. Registries have allowed us to appreciate the improvement in survival afforded by modern therapy and enhanced detection of this disease. Moving forward, a more global approach to registries is needed, as is enhanced collaboration and centralization.
Literatur
1.
Zurück zum Zitat Badesch DB et al (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54(1 Suppl):S55–S66CrossRefPubMed Badesch DB et al (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54(1 Suppl):S55–S66CrossRefPubMed
2.
Zurück zum Zitat Hoeper MM et al (2013) Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D42–D50CrossRefPubMed Hoeper MM et al (2013) Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D42–D50CrossRefPubMed
3.
Zurück zum Zitat Kovacs G et al (2009) Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 34(4):888–894CrossRefPubMed Kovacs G et al (2009) Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 34(4):888–894CrossRefPubMed
4.
Zurück zum Zitat Bae S et al (2012) 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 71(8):1335–1342CrossRefPubMedPubMedCentral Bae S et al (2012) 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 71(8):1335–1342CrossRefPubMedPubMedCentral
5.
Zurück zum Zitat Heresi GA et al (2013) Clinical characterization and survival of patients with borderline elevation in pulmonary artery pressure. Pulm Circ 3(4):916–925CrossRefPubMedPubMedCentral Heresi GA et al (2013) Clinical characterization and survival of patients with borderline elevation in pulmonary artery pressure. Pulm Circ 3(4):916–925CrossRefPubMedPubMedCentral
6.
Zurück zum Zitat McLaughlin VV et al (2009) ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Associati. Circulation 119(16):2250–2294CrossRefPubMed McLaughlin VV et al (2009) ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Associati. Circulation 119(16):2250–2294CrossRefPubMed
7.
Zurück zum Zitat Halpern SD, Taichman DB (2009) Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest 136(1):37–43CrossRefPubMed Halpern SD, Taichman DB (2009) Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest 136(1):37–43CrossRefPubMed
8.
9.
Zurück zum Zitat Hoeper MM et al (2006) Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 48(12):2546–2552CrossRefPubMed Hoeper MM et al (2006) Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 48(12):2546–2552CrossRefPubMed
10.
Zurück zum Zitat Abenheim L et al (1994) The International Primary Pulmonary Hypertension Study (IPPHS). Chest 105(2 Suppl):37S–41S Abenheim L et al (1994) The International Primary Pulmonary Hypertension Study (IPPHS). Chest 105(2 Suppl):37S–41S
11.
Zurück zum Zitat Peacock AJ et al (2007) An epidemiological study of pulmonary arterial hypertension. Eur Respir J 30(1):104–109CrossRefPubMed Peacock AJ et al (2007) An epidemiological study of pulmonary arterial hypertension. Eur Respir J 30(1):104–109CrossRefPubMed
12.
Zurück zum Zitat Humbert M et al (2006) Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 173(9):1023–1030CrossRefPubMed Humbert M et al (2006) Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 173(9):1023–1030CrossRefPubMed
13.
Zurück zum Zitat Rich S et al (1987) Primary pulmonary hypertension. A national prospective study. Ann Intern Med 107(2):216–223CrossRefPubMed Rich S et al (1987) Primary pulmonary hypertension. A national prospective study. Ann Intern Med 107(2):216–223CrossRefPubMed
14.
Zurück zum Zitat McGoon MD et al (2013) Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 62(25 Suppl):D51–D59CrossRefPubMed McGoon MD et al (2013) Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 62(25 Suppl):D51–D59CrossRefPubMed
15.
Zurück zum Zitat Lee WT et al (2012) Predicting survival in pulmonary arterial hypertension in the UK. Eur Respir J 40(3):604–611CrossRefPubMed Lee WT et al (2012) Predicting survival in pulmonary arterial hypertension in the UK. Eur Respir J 40(3):604–611CrossRefPubMed
16.
Zurück zum Zitat Humbert M et al (2010) Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122(2):156–163CrossRefPubMed Humbert M et al (2010) Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122(2):156–163CrossRefPubMed
17.
Zurück zum Zitat Thenappan T et al (2010) Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J 35(5):1079–1087CrossRefPubMed Thenappan T et al (2010) Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J 35(5):1079–1087CrossRefPubMed
18.
Zurück zum Zitat Benza RL et al (2012) An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 142(2):448–456CrossRefPubMed Benza RL et al (2012) An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 142(2):448–456CrossRefPubMed
19.
Zurück zum Zitat Jing ZC et al (2007) Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest 132(2):373–379CrossRefPubMed Jing ZC et al (2007) Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest 132(2):373–379CrossRefPubMed
20.
Zurück zum Zitat Alves JL Jr et al (2015) Pulmonary arterial hypertension in the southern hemisphere: results from a registry of incident Brazilian cases. Chest 147(2):495–501CrossRefPubMed Alves JL Jr et al (2015) Pulmonary arterial hypertension in the southern hemisphere: results from a registry of incident Brazilian cases. Chest 147(2):495–501CrossRefPubMed
21.
Zurück zum Zitat Hovnanian A et al (2010) Schistosomiasis associated pulmonary hypertension. Int J Clin Pract Suppl 165:25–28CrossRefPubMed Hovnanian A et al (2010) Schistosomiasis associated pulmonary hypertension. Int J Clin Pract Suppl 165:25–28CrossRefPubMed
22.
Zurück zum Zitat McGoon MD et al (2008) Design of the REVEAL registry for US patients with pulmonary arterial hypertension. Mayo Clin Proc 83(8):923–931CrossRefPubMed McGoon MD et al (2008) Design of the REVEAL registry for US patients with pulmonary arterial hypertension. Mayo Clin Proc 83(8):923–931CrossRefPubMed
23.
Zurück zum Zitat Benza RL et al (2012) The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 141(2):354–362CrossRefPubMed Benza RL et al (2012) The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 141(2):354–362CrossRefPubMed
24.
Zurück zum Zitat Frost AE et al (2013) Demographics and outcomes of patients diagnosed with pulmonary hypertension with pulmonary capillary wedge pressures 16–18 mmHg: insights from the REVEAL Registry. Chest 143(1):185–195CrossRefPubMed Frost AE et al (2013) Demographics and outcomes of patients diagnosed with pulmonary hypertension with pulmonary capillary wedge pressures 16–18 mmHg: insights from the REVEAL Registry. Chest 143(1):185–195CrossRefPubMed
25.
Zurück zum Zitat Hoeper MM et al (2013) Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol 168(2):871–880CrossRefPubMed Hoeper MM et al (2013) Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol 168(2):871–880CrossRefPubMed
26.
Zurück zum Zitat Campbell EG et al (2010) Physician professionalism and changes in physician-industry relationships from 2004 to 2009. Arch Intern Med 170(20):1820–1826CrossRefPubMed Campbell EG et al (2010) Physician professionalism and changes in physician-industry relationships from 2004 to 2009. Arch Intern Med 170(20):1820–1826CrossRefPubMed
Metadaten
Titel
Definition, epidemiology and registries of pulmonary hypertension
verfasst von
R. Awdish
H. Cajigas
Publikationsdatum
05.10.2015
Verlag
Springer US
Erschienen in
Heart Failure Reviews / Ausgabe 3/2016
Print ISSN: 1382-4147
Elektronische ISSN: 1573-7322
DOI
https://doi.org/10.1007/s10741-015-9510-y

Weitere Artikel der Ausgabe 3/2016

Heart Failure Reviews 3/2016 Zur Ausgabe

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.