Pulmonary hypertension diagnostic study of choice

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Diagnostic Study of Choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Richard Channick, M.D.; Assistant Editor(s)-in-Chief: Ralph Matar, Lisa Prior, Ann Slater, R.N.; José Eduardo Riceto Loyola Junior, M.D.[2]

Overview

Pulmonary hypertension is defined by a mean pulmonary arterial pressure higher than 25mmHg. It can be assessed by echocardiography or right heart cardiac catheterization.

Diagnosis

Diagnostic Study of Choice

  • Echocardiography is the diagnostic study of choice, as it can:
    • Estimate the pulmonary artery systolic pressure (PSAP);
    • Evaluate for possible causes of pulmonary hypertension;
    • Asses the presence of right atrial or ventricular enlargement, hypertrophy, or decreased right ventricular function;
    • May also evaluate left heart chambers, especially if they are causing pulmonary hypertension;


  • A physical examination is performed to look for typical signs of pulmonary hypertension. These include extra sounds, murmurs and signs of RV failure.
  • Further procedures are required to confirm the presence of pulmonary hypertension and exclude other possible diagnoses. These generally include:
  1. Pulmonary function tests
  2. Blood tests
  3. Electrocardiography (ECG)
  4. Arterial blood gas measurements
  5. X-rays of the chest (followed by high-resolution CT scanning if interstitial lung disease is suspected), and ventilation-perfusion or V/Q scanning to exclude chronic thromboembolic pulmonary hypertension.
  6. Biopsy of the lung is usually not indicated unless the pulmonary hypertension is thought to be due to an underlying interstitial lung disease. But lung biopsies are fraught with risks of bleeding due to the high intrapulmonary blood pressure.
  7. Clinical improvement is often measured by a "six-minute walk test", i.e. the distance a patient can walk in six minutes. Stability and improvement in this measurement correlate with better survival.
  8. Although pulmonary arterial pressure can be estimated on the basis of echocardiography, pressure sampling with a Swan-Ganz catheter provides the most definite measurement. PAOP and PVR can not be measured directly with echocardiography. Therefore diagnosis of PAH requires a cardiac catheterization. A Swan-Ganz catheter can also measure the cardiac output, which is far more important in measuring disease severity than the pulmonary arterial pressure.
  • Normal pulmonary arterial pressure in a person living at sea level has a mean value of 12–16 mm Hg (1600–2100 Pa). Definite pulmonary hypertension is present when mean pressures at rest exceed 25 mm Hg (3300 Pa). If mean pulmonary artery pressure rises above 30 mm Hg (4000 Pa) with exercise, that is also considered pulmonary hypertension.

Diagnostic criteria

Diagnosis of PAH requires the presence of pulmonary hypertension with two other conditions.

  • Pulmonary artery occlusion pressure (PAOP or PCWP) must be less than 15 mm Hg (2000 Pa)
  • Pulmonary vascular resistance (PVR) must be greater than 3 Wood units (240 dyn•s•cm-5 or 2.4 mN•s•cm-5).

References

  1. Shitrit, D.; Bendayan, D.; Rudensky, B.; Izbicki, G.; Huerta, M.; Fink, G.; Kramer, MR. (2002). "Elevation of ELISA d-dimer levels in patients with primary pulmonary hypertension". Respiration. 69 (4): 327–9. doi:63270 Check |doi= value (help). PMID 12169745.
  2. Shitrit, D.; Bendayan, D.; Bar-Gil-Shitrit, A.; Huerta, M.; Rudensky, B.; Fink, G.; Kramer, MR. (2002). "Significance of a plasma D-dimer test in patients with primary pulmonary hypertension". Chest. 122 (5): 1674–8. PMID 12426270. Unknown parameter |month= ignored (help)
  3. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM; et al. (1987). "Primary pulmonary hypertension. A national prospective study". Ann Intern Med. 107 (2): 216–23. PMID 3605900.
  4. Budev MM, Arroliga AC, Jennings CA (2003). "Diagnosis and evaluation of pulmonary hypertension". Cleve Clin J Med. 70 Suppl 1: S9–17. PMID 12716138. Unknown parameter |month= ignored (help)
  5. Carolyn H. Welsh; Michael E. Hanley (2003). Current diagnosis & treatment in pulmonary medicine. New York: Lange Medical Books / McGraw-Hill. ISBN 0-07-121971-4.
  6. Wiedemann, R.; Ghofrani, HA.; Weissmann, N.; Schermuly, R.; Quanz, K.; Grimminger, F.; Seeger, W.; Olschewski, H. (2001). "Atrial natriuretic peptide in severe primary and nonprimary pulmonary hypertension: response to iloprost inhalation". J Am Coll Cardiol. 38 (4): 1130–6. PMID 11583893. Unknown parameter |month= ignored (help)
  7. Bossone E, Paciocco G, Iarussi D; et al. (2002). "The prognostic role of the ECG in primary pulmonary hypertension". Chest. 121 (2): 513–8. PMID 11834666. Unknown parameter |month= ignored (help)
  8. Kawut SM, Silvestry FE, Ferrari VA; et al. (1999). "Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension". Am. J. Cardiol. 83 (6): 984–6, A10. PMID 10190427. Unknown parameter |month= ignored (help)
  9. Simon O'Connor MBBS FRACP DDU; Nicholas P. Hirsch MBBS FRCA FRCP (2009). Clinical Examination: A Systematic Guide to Physical Diagnosis. Edinburgh: Churchill Livingstone. ISBN 0-7295-3905-9.
  10. Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA; et al. (2009). "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. 30 (20): 2493–537. doi:10.1093/eurheartj/ehp297. PMID 19713419.
  11. Thompson, Paul Richard; Topol, Eric J.; Califf, Robert M.; Prystowsky, Eric N.; Thomas, James Alan (2007). Textbook of cardiovascular medicine. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7012-2.
  12. Nagaya, N.; Nishikimi, T.; Uematsu, M.; Satoh, T.; Kyotani, S.; Sakamaki, F.; Kakishita, M.; Fukushima, K.; Okano, Y. (2000). "Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension". Circulation. 102 (8): 865–70. PMID 10952954. Unknown parameter |month= ignored (help)
  13. Kucher, N.; Printzen, G.; Goldhaber, SZ. (2003). "Prognostic role of brain natriuretic peptide in acute pulmonary embolism". Circulation. 107 (20): 2545–7. doi:10.1161/01.CIR.0000074039.45523.BE. PMID 12742987. Unknown parameter |month= ignored (help)
  14. ten Wolde, M.; Tulevski, II.; Mulder, JW.; Söhne, M.; Boomsma, F.; Mulder, BJ.; Büller, HR. (2003). "Brain natriuretic peptide as a predictor of adverse outcome in patients with pulmonary embolism". Circulation. 107 (16): 2082–4. doi:10.1161/01.CIR.0000070020.79932.DB. PMID 12707233. Unknown parameter |month= ignored (help)
  15. Leuchte, HH.; Baumgartner, RA.; Nounou, ME.; Vogeser, M.; Neurohr, C.; Trautnitz, M.; Behr, J. (2006). "Brain natriuretic peptide is a prognostic parameter in chronic lung disease". Am J Respir Crit Care Med. 173 (7): 744–50. doi:10.1164/rccm.200510-1545OC. PMID 16415273. Unknown parameter |month= ignored (help)

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