Pulmonary hypertension: prevalence and mortality in the Armadale echocardiography cohort

Geoff Strange, David Playford, Simon Stewart, Jenny A Deague, Helen Nelson, Aaron Kent, Eli Gabbay, Geoff Strange, David Playford, Simon Stewart, Jenny A Deague, Helen Nelson, Aaron Kent, Eli Gabbay

Abstract

Background: Pulmonary hypertension (PHT) lacks community prevalence and outcome data.

Objective: To characterise minimum 'indicative' prevalences and mortality data for all forms of PHT in a selected population with an elevated estimated pulmonary artery systolic pressure (ePASP) on echocardiography.

Design: Observational cohort study.

Setting: Residents of Armadale and the surrounding region in Western Australia (population 165 450) referred to our unit for transthoracic echocardiography between January 2003 and December 2009.

Results: Overall, 10 314 individuals (6.2% of the surrounding population) had 15 633 echo studies performed. Of these, 3320 patients (32%) had insufficient TR to ePASP and 936 individuals (9.1%, 95% CI 8.6% to 9.7%) had PHT, defined as, ePASP>40 mm Hg. The minimum 'indicative' prevalence for all forms of PHT is 326 cases/100 000 inhabitants of the local population, with left heart disease-associated PHT being the commonest cause (250 cases/100 000). 15 cases of pulmonary arterial hypertension/100 000 inhabitants were identified and an additional 144 individuals (15%) with no identified cause for their PHT. The mean time to death for those with ePASP >40 mm Hg, calculated from the first recorded ePASP, was 4.1 years (95% CI 3.9 to 4.3). PHT increased mortality whatever the underlying cause, but patients with PHT from left heart disease had the worst prognosis and those with idiopathic pulmonary arterial hypertension receiving disease-specific treatment the best prognosis. Risk of death increased with PHT severity: severe pulmonary hypertension shortened the lifespan by an average of 1.1 years compared with mild pulmonary hypertension.

Conclusions: In this cohort, PHT was common and deadly. Left heart disease was the most common cause and had the worst prognosis and treated pulmonary arterial hypertension had the best prognosis.

Conflict of interest statement

Competing interests: GS receives honoraria from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ). The PHSANZ receives grant funding from Actelion, Bayer, GSK, Lilly, Novartis and Pfizer Australia. DP has received honoraria for speaking from Actelion and Pfizer Australia. SS, JD, HN, AK have no conflict of interest to declare. EG has received research funds from Actelion, Bayer, GSK and Pfizer Australia. EG has received honoraria for speaking from Actelion, Bayer, GSK and Pfizer. EG in currently a member of the Advisory Boards for Actelion, Eli Lilly, GSK and Pfizer Australia.

Figures

Figure 1
Figure 1
Cases detected: the ‘indicative’ prevalence of pulmonary hypertension in Armadale and surrounding regions, December 2009. CHD, congenital heart disease; CTEPH, chronic thromboembolic pulmonary hypertension; iPAH, idiopathic PAHs; PAH, pulmonary arterial hypertension; Misc PHT, pulmonary hypertension due to other causes; PLHD, pulmonary hypertension secondary to left heart disease; RPHT, respiratory-associated pulmonary hypertension; SSc, systemic sclerosis.
Figure 2
Figure 2
Kaplan–Meier survival and proportional OR for all causes of pulmonary hypertension. (A) Kaplan–Meier survival for all causes of pulmonary hypertension. (B) Independent variables associated with mortality. PAP, pulmonary artery pressure on echocardiogram in mm Hg.
Figure 3
Figure 3
Kaplan–Meier survival estimates. (A) Each subtype of PHT. (B) Clinical severity of PASP. PAH, pulmonary arterial hypertension; Left heart disease, pulmonary hypertension secondary to left heart disease; Respiratory disease, respiratory-associated pulmonary hypertension; Chronic thromboembolic, chronic thromboembolic pulmonary hypertension; Miscellaneous, miscellaneous pulmonary hypertension; Unknown cause, pulmonary hypertension of unknown cause. Mortality for mild, moderate and severe pulmonary hypertension for all causes of pulmonary hypertension during the period of follow-up. p

Figure 4

Survival for all forms of…

Figure 4

Survival for all forms of pulmonary hypertension. (A) Gender; (B) quartiles of age.…

Figure 4
Survival for all forms of pulmonary hypertension. (A) Gender; (B) quartiles of age. p80 years; 1325±66 days. *p
Similar articles
Cited by
References
    1. Rubin LJ; American College of Chest Physicians Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126(Suppl 1):7S–10 - PubMed
    1. Galiè N, Hoeper MM, Humbert M, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Eur Heart J 2004;25:2243–78 - PubMed
    1. Barst RJ, Gibbs JS, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2009;54:S78–84 - PMC - PubMed
    1. Nef HM, Möllmann H, Hamm C, et al. Pulmonary hypertension: updated classification and management of pulmonary hypertension. Heart 2010;96:552–9 - PubMed
    1. Swiston JR, Johnson SR, Granton JT. Factors that prognosticate mortality in idiopathic pulmonary arterial hypertension: a systemic review of the literature. Respir Med 2010;104:1588–607 - PubMed
Show all 37 references
Publication types
MeSH terms
Related information
Full text links [x]
[x]
Cite
Copy Download .nbib
Format: AMA APA MLA NLM
Figure 4
Figure 4
Survival for all forms of pulmonary hypertension. (A) Gender; (B) quartiles of age. p80 years; 1325±66 days. *p

References

    1. Rubin LJ; American College of Chest Physicians Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126(Suppl 1):7S–10
    1. Galiè N, Hoeper MM, Humbert M, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Eur Heart J 2004;25:2243–78
    1. Barst RJ, Gibbs JS, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2009;54:S78–84
    1. Nef HM, Möllmann H, Hamm C, et al. Pulmonary hypertension: updated classification and management of pulmonary hypertension. Heart 2010;96:552–9
    1. Swiston JR, Johnson SR, Granton JT. Factors that prognosticate mortality in idiopathic pulmonary arterial hypertension: a systemic review of the literature. Respir Med 2010;104:1588–607
    1. Shafazand S, Goldstein MK, Doyle RL, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Chest 2004;126:1452–9
    1. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010;137:376–87
    1. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006;173:1023–30
    1. Peacock AJ, Murphy NF, McMurray JJ, et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007;30:104–9
    1. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987;107:216–23
    1. D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991;115:343–9
    1. von Elm E, Altman DG, Egger M, et al. The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement: guidelines for reporting observational studies. Lancet 2007;370:1453–7
    1. Thomas JD, Adams DB, Devries S, et al. Guidelines and recommendations for digital echocardiography. J Am Soc Echocardiogr 2005;18:287–97
    1. Lang RM, Bierig M, Devereux RB, et al. Recommendations for chamber quantification: a report from the American Society of Echocardiography's guidelines and standards Committee and the chamber quantification Writing group, developed in conjunction with the European association of echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 2005;18:1440–63
    1. Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009;179:151–7
    1. Hatle L, Angelsen BA, Tromsdal A. Non-invasive estimation of pulmonary artery systolic pressure with Doppler ultrasound. Br Heart J 1981;45:157–65
    1. Galiè N, Hoeper MM, Humbert M, et al. ; ESC Committee for Practice Guidelines (CPG) 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
    1. Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43(Suppl 12):5S–12
    1. Australian Government. 2006 Census.
    1. Enea I, Ghio S, Bongarzoni A, et al. [Echocardiographic alterations suggestive of pulmonary hypertension in the Italian ultrasonography laboratories. Epidemiological data from the INCIPIT study (INCidence of Pulmonary Hypertension in Italian ulTrasonography laboratories)]. G Ital Cardiol (Rome) 2010;11:402–7

    2. Alterazioni ecocardiografiche suggestive di ipertensione polmonare nei laboratori italiani di ultrasonografia. Dati epidemiologici dallo studio INCIPIT (INCidenza di Ipertensione Polmonare nei laboratori Italiani di ulTrasonografia).

    1. Strange G, Keogh A, Stewart S, et al. Symptoms to definitive diagnosis of pulmonary arterial hypertension (PAH). Am J Resp Crit Care 2011;183:A1923
    1. Bursi F, McNallan SM, Redfield MM, et al. Pulmonary pressures and death in heart failure: a community study. J Am Coll Cardiol 2012;59:222–31
    1. Lam CS, Borlaug BA, Kane GC, et al. Age-associated increases in pulmonary artery systolic pressure in the general population. Circulation 2009;119:2663–78
    1. Denton CP, Cailes JB, Phillips GD, et al. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol 1997;36:239–43
    1. Gabbay E, Reed A, Williams TJ. Assessment and treatment of pulmonary arterial hypertension: an Australian perspective in 2006. Intern Med J 2007;37:38–48
    1. Proudman SM, Stevens WM, Sahhar J, et al. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Intern Med J 2007;37:485–94
    1. Phung S, Strange G, Chung LP, et al. Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis. Intern Med J 2009;39:682–91
    1. Mukerjee D, St George D, Knight C, et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford) 2004;43:461–6
    1. Kilner PJ. Non invasive assessment of pulmonary hypertension severity and disease progression. Heart 2011;97:1196–7
    1. Rubin LJ, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 1990;112:485–91
    1. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346:896–903
    1. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;334:296–302
    1. Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002;347:322–9
    1. Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001;358:1119–23
    1. Keogh A, Strange G, Kotlyar E, et al. Survival after the initiation of combination therapy in patients with pulmonary arterial hypertension: an Australian collaborative report. Intern Med J 2011;41:235–44
    1. Keogh A, Strange G, McNeil K, et al. The bosentan patient registry: long-term survival in pulmonary arterial hypertension. Intern Med J 2011;41:227–34
    1. Janda S, Shahidi N, Gin K, et al. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis. Heart 2011;97:612–22

Source: PubMed

Sponsoren und Mitarbeiter

Krankheiten

Drogeninterventionen

3
Abonnieren