ABSTRACT: Pulmonary arterial hypertension (PAH) is an increasingly recognized cause of dyspnea in elderly patients. The initial workup typically includes electrocardiography, chest adiography, echocardiography, and pulmonary function tests. If echocardiography shows signs of PAH, the diagnosis should be confirmed by right heart catheterization. Radiographic evidence of long-standing PAH includes enlargement of the central pulmonary arteries with abrupt narrowing of the more distal branches, giving a "pruned-tree" appearance, and right ventricular (RV) enlargement. The classic radiographic signs of RV enlargement include increased transverse diameter of the heart, elevation of the cardiac border on the posteroanterior view, and narrowing or loss of the retrosternal airspace on the lateral projection. (J Respir Dis. 2008;29(11):443-450)
Dyspnea has been defined as a "subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity."1 It is a common complaint that becomes more frequent with age, with an incidence ranging from 17.4% in patients younger than 45 years to 38% in those older than 65 years.1 Dyspnea has been associated with poor perceived health, increased anxiety and depressive symptoms, impaired daily functioning, and reduced happiness. 2 Furthermore, moderate to severe dyspnea was associated with an increased risk of death in a cohort of 124 patients who were older than 70 years.2
Dyspnea is associated with a variety of diagnoses in the elderly, most commonly obstructive lung disease (emphysema, asthma), restrictive lung disease (pulmonary fibrosis), and cardiac disease (congestive heart failure). An aggressive evaluation to determine the cause of dyspnea is necessary. A simple history and physical examination with screening tests can often determine the cause. However, persistent dyspnea despite an extensive evaluation can be puzzling for both the clinician and the patient.
Pulmonary hypertension (PH) has been increasingly recognized as a cause of insidious dyspnea in the elderly and should be added to the differential diagnosis when common causes of this symptom are absent. Advances in recognition, diagnosis, and management of PH have led to earlier intervention and improved prognosis over the past decade. Of course, successful therapy first requires a suspicion for the disease followed by an appropriate evaluation.
In part 1 of this article, we will review the diagnostic evaluation of PH in elderly patients. In part 2, we will focus on management.
PH is defined as a mean pulmonary artery pressure (PAP) of greater than 25 mm Hg.3 It can be an insidious cause of dyspnea and is increasingly discovered as a result of the ease of obtaining a screening echocardiogram.
1. Mahler DA, Fierro-Carrion G, Baird JC. Evaluation
of dyspnea in the elderly. Clin Geriatr Med.
2. Huijnen B, van der Horst F, van Amelsvoort L, et
al. Dyspnea in elderly family practice patients. Occurrence,
severity, quality of life and mortality over
an 8-year period. Fam Pract. 2006;23:34-39.
3. Rubin LJ; American College of Chest Physicians.
Introduction: diagnosis and management of pulmonary
arterial hypertension: ACCP evidencebased
clinical practice guidelines. Chest. 2004;126(1 suppl):7S-10S.
4. Simonneau G, Galiè N, Rubin LJ, et al. Clinical
classification of pulmonary hypertension. J Am Coll
Cardiol. 2004;43(12 suppl S):5S-12S.
5. D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival
in patients with primary pulmonary hypertension.
Results from a national prospective registry. Ann Intern
Nataro S, Strasser T, eds. Primary Pulmonary
Hypertension: Report of WHO Meeting. Geneva:
World Health Organization; 1975:1-45.
Carrillo M, Cajigas H, Greenbaum A, Chan KM.
Pulmonary hypertension in the elderly: demo-graphics and outcomes following therapy with
bosentan [abstract]. Chest. 2005;128:175S-176S.
8. Phipps B, Wong G, Chang CH, Dunn M. Unexplained
severe pulmonary hypertension in the old
age group. Chest. 1983;84:399-402.
9. Braman SS, Eby E, Kuhn C, Rounds S. Primary
pulmonary hypertension in the elderly. Arch Intern
10. Yigla M, Kramer MR, Bendayan D, et al. Unexplained
severe pulmonary hypertension in the elderly:
report on 14 patients. Isr Med Assoc J. 2004;6:78-81.
11. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary
arterial hypertension in France: results from
a national registry. Am J Respir Crit Care Med.
12. Shapiro BP, McGoon MD, Redfield MM. Unexplained
pulmonary hypertension in elderly patients.
13. Martin-Duran R, Larman M, Trugeda A, et al.
Comparison of Doppler-determined elevated pulmonary
arterial pressure with pressure measured
at cardiac catherization. Am J Cardiol. 1986;57:859-863.
14. Finkelhor RS, Yang SX, Bosich RM, Bahler RC.
Unexplained pulmonary hypertension is associated
with systolic arterial hypertension in patients undergoing
routine Doppler echocardiography.
Dow L, Coggon D, Osmond C, Holgate ST. A
population survey of respiratory symptoms in the
elderly. Eur Respir J. 1991;4:267-272.
16. Horsley JR, Sterling IJ, Waters WE, Howell JB.
Respiratory symptoms among elderly people in the
New Forest area as assessed by postal questionnaire.
Age Ageing. 1991;20:325-331.
17. Pedersen F, Raymond I, Mehlsen J, et al.
Prevalence of diastolic dysfunction as a possible
cause of dyspnea in the elderly. Am J Med. 2005;118:25-31.
18. Ahearn GS, Tapson VF, Rebeiz A, Greenfield JC
Jr. Electrocardiography to define clinical status in
primary pulmonary hypertension and pulmonary
arterial hypertension secondary to collagen vascular
disease. Chest. 2002;122:524-527.
19. Ravin CE, Greenspan RH, McLoud TC, et al.
Redistribution of pulmonary blood flow secondary
to pulmonary arterial hypertension. Invest Radiol.
20. McGoon M, Gutterman D, Steen V, et al; American
College of Chest Physicians. Screening, early
detection, and diagnosis of pulmonary arterial hypertension.
ACCP evidence-based clinical practice
guidelines. Chest. 2004;126(1 suppl):14S-34S.
21. Torregrosa M, Genesca J, Gonzalez A, et al.
Role of Doppler echocardiography in the assessment
of portopulmonary hypertension in liver transplantation
candidates. Transplantation. 2001;71:572-574.
22. Arcasoy SM, Christie JD, Ferrari VA, et al. Echocardiographic
assessment of pulmonary hypertension
in patients with advanced lung disease. Am J
Respir Crit Care Med. 2003;167:735-740.
23. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis
and differential assessment of pulmonary arterial
hypertension. J Am Coll Cardiol. 2004;43(12
24. Badesch DB, Abman SH, Simonneau G, et al.
Medical therapy for pulmonary arterial hypertension:
updated ACCP evidence-based clinical practice
guidelines. Chest. 2007;131:1917-1928.
25. Sitbon O, Humbert M, Jaïs X, et al. Long-term
response to calcium channel blockers in idiopathic
pulmonary arterial hypertension. Circulation. 2005;111:3105-3111.