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==Diagnosis==
==Diagnosis==
The diagnostic approach to PH aims to:
The diagnostic approach to PH aims to:<ref name="pmid3605900">{{cite journal| author=Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM et al.| title=Primary pulmonary hypertension. A national prospective study. | journal=Ann Intern Med | year= 1987 | volume= 107 | issue= 2 | pages= 216-23 | pmid=3605900 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3605900  }} </ref><ref name="pmid3605900">{{cite journal |author=Rich S, Dantzker DR, Ayres SM, ''et al.'' |title=Primary pulmonary hypertension. A national prospective study |journal=Ann. Intern. Med. |volume=107 |issue=2 |pages=216–23 |year=1987 |month=August |pmid=3605900 |doi= |url=}}</ref><ref name="pmid3605900">{{cite journal |author=Rich S, Dantzker DR, Ayres SM, ''et al.'' |title=Primary pulmonary hypertension. A national prospective study |journal=Ann. Intern. Med. |volume=107 |issue=2 |pages=216–23 |year=1987 |month=August |pmid=3605900 |doi= |url=}}</ref><ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref><ref name="isbn0-07-121971-4">{{cite book |author=Carolyn H. Welsh; Michael E. Hanley |title=Current diagnosis & treatment in pulmonary medicine |publisher=Lange Medical Books / McGraw-Hill |location=New York |year=2003 |pages= |isbn=0-07-121971-4 |oclc= |doi= |accessdate=}}</ref><ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref>
**However, there have also been reports of angina due to decreased [[Myocardium|myocardial]] oxygen supply from compression of the left main [[Coronary circulation|coronary artery]] by a dilated pulmonary artery.<ref name="pmid10190427">{{cite journal |author=Kawut SM, Silvestry FE, Ferrari VA, ''et al.'' |title=Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension |journal=Am. J. Cardiol. |volume=83 |issue=6 |pages=984–6, A10 |year=1999 |month=March |pmid=10190427 |doi= |url=}}</ref><ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref><ref name="isbn0-07-121971-4">{{cite book |author=Carolyn H. Welsh; Michael E. Hanley |title=Current diagnosis & treatment in pulmonary medicine |publisher=Lange Medical Books / McGraw-Hill |location=New York |year=2003 |pages= |isbn=0-07-121971-4 |oclc= |doi= |accessdate=}}</ref<ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref><ref name="isbn0-7295-3905-9">{{cite book |author=Simon O'Connor MBBS FRACP DDU; Nicholas P. Hirsch MBBS FRCA FRCP |title=Clinical Examination: A Systematic Guide to Physical Diagnosis |publisher=Churchill Livingstone |location=Edinburgh |year=2009 |pages= |isbn=0-7295-3905-9 |oclc= |doi= |accessdate=}}</ref><ref name="isbn0-7295-3905-9">{{cite book |author=Simon O'Connor MBBS FRACP DDU; Nicholas P. Hirsch MBBS FRCA FRCP |title=Clinical Examination: A Systematic Guide to Physical Diagnosis |publisher=Churchill Livingstone |location=Edinburgh |year=2009 |pages= |isbn=0-7295-3905-9 |oclc= |doi= |accessdate=}}</ref><ref name="pmid19713419">{{cite journal| author=Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA et al.| title=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). | journal=Eur Heart J | year= 2009 | volume= 30 | issue= 20 | pages= 2493-537 | pmid=19713419 | doi=10.1093/eurheartj/ehp297 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19713419  }} </ref><ref name="isbn0-7295-3905-9">{{cite book |author=Simon O'Connor MBBS FRACP DDU; Nicholas P. Hirsch MBBS FRCA FRCP |title=Clinical Examination: A Systematic Guide to Physical Diagnosis |publisher=Churchill Livingstone |location=Edinburgh |year=2009 |pages= |isbn=0-7295-3905-9 |oclc= |doi= |accessdate=}}</ref><ref name="isbn0-7817-7012-2">{{cite book |author=Thompson, Paul Richard; Topol, Eric J.; Califf, Robert M.; Prystowsky, Eric N.; Thomas, James Alan |title=Textbook of cardiovascular medicine |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2007 |pages= |isbn=0-7817-7012-2 |oclc= |doi= |accessdate=}}</ref><ref name="Nagaya-2000">{{Cite journal  | last1 = Nagaya | first1 = N. | last2 = Nishikimi | first2 = T. | last3 = Uematsu | first3 = M. | last4 = Satoh | first4 = T. | last5 = Kyotani | first5 = S. | last6 = Sakamaki | first6 = F. | last7 = Kakishita | first7 = M. | last8 = Fukushima | first8 = K. | last9 = Okano | first9 = Y. | title = Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. | journal = Circulation | volume = 102 | issue = 8 | pages = 865-70 | month = Aug | year = 2000 | doi =  | PMID = 10952954 }}</ref><ref name="Kucher-2003">{{Cite journal  | last1 = Kucher | first1 = N. | last2 = Printzen | first2 = G. | last3 = Goldhaber | first3 = SZ. | title = Prognostic role of brain natriuretic peptide in acute pulmonary embolism. | journal = Circulation | volume = 107 | issue = 20 | pages = 2545-7 | month = May | year = 2003 | doi = 10.1161/01.CIR.0000074039.45523.BE | PMID = 12742987 }}</ref><ref name="ten Wolde-2003">{{Cite journal  | last1 = ten Wolde | first1 = M. | last2 = Tulevski | first2 = II. | last3 = Mulder | first3 = JW. | last4 = Söhne | first4 = M. | last5 = Boomsma | first5 = F. | last6 = Mulder | first6 = BJ. | last7 = Büller | first7 = HR. | title = Brain natriuretic peptide as a predictor of adverse outcome in patients with pulmonary embolism. | journal = Circulation | volume = 107 | issue = 16 | pages = 2082-4 | month = Apr | year = 2003 | doi = 10.1161/01.CIR.0000070020.79932.DB | PMID = 12707233 }}</ref><ref name="Leuchte-2006">{{Cite journal  | last1 = Leuchte | first1 = HH. | last2 = Baumgartner | first2 = RA. | last3 = Nounou | first3 = ME. | last4 = Vogeser | first4 = M. | last5 = Neurohr | first5 = C. | last6 = Trautnitz | first6 = M. | last7 = Behr | first7 = J. | title = Brain natriuretic peptide is a prognostic parameter in chronic lung disease. | journal = Am J Respir Crit Care Med | volume = 173 | issue = 7 | pages = 744-50 | month = Apr | year = 2006 | doi = 10.1164/rccm.200510-1545OC | PMID = 16415273 }}</ref> N-terminal fragment of BNP (NT-proBNP) has been studied as an alternative biomarker to BNP in various classes of pulmonary hypertension. Higher NT-proBNP and peak oxygen uptake were shown to be independent predictors of mortality and a supramedian NT-proBNP level indicated a significantly lower survival.<ref name="Andreassen-2006">{{Cite journal  | last1 = Andreassen | first1 = AK. | last2 = Wergeland | first2 = R. | last3 = Simonsen | first3 = S. | last4 = Geiran | first4 = O. | last5 = Guevara | first5 = C. | last6 = Ueland | first6 = T. | title = N-terminal pro-B-type natriuretic peptide as an indicator of disease severity in a heterogeneous group of patients with chronic precapillary pulmonary hypertension. | journal = Am J Cardiol | volume = 98 | issue = 4 | pages = 525-9 | month = Aug | year = 2006 | doi = 10.1016/j.amjcard.2006.02.061 | PMID = 16893710 }}</ref><ref name="Fijalkowska-2006">{{Cite journal  | last1 = Fijalkowska | first1 = A. | last2 = Kurzyna | first2 = M. | last3 = Torbicki | first3 = A. | last4 = Szewczyk | first4 = G. | last5 = Florczyk | first5 = M. | last6 = Pruszczyk | first6 = P. | last7 = Szturmowicz | first7 = M. | title = Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. | journal = Chest | volume = 129 | issue = 5 | pages = 1313-21 | month = May | year = 2006 | doi = 10.1378/chest.129.5.1313 | PMID = 16685024 }}</ref> 
* Confirm the diagnosis of PH
* Confirm the diagnosis of PH
* Determine the classification group of PH
* Determine the classification group of PH

Revision as of 20:40, 1 March 2018

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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.

Overview

Pulmonary hypertension (PH) is a pathological condition of the pulmonary vasculature present in several disease states and leading to hemodymical derangement. PH is defined as an elevated mean pulmonary artery pressure (PAP) as measured by right heart catheterization at rest.

Diagnosis

The diagnostic approach to PH aims to:[1][1][1][2][3][2]

    • However, there have also been reports of angina due to decreased myocardial oxygen supply from compression of the left main coronary artery by a dilated pulmonary artery.[4][2][5][5][6][5][7][8][9][10][11] N-terminal fragment of BNP (NT-proBNP) has been studied as an alternative biomarker to BNP in various classes of pulmonary hypertension. Higher NT-proBNP and peak oxygen uptake were shown to be independent predictors of mortality and a supramedian NT-proBNP level indicated a significantly lower survival.[12][13]
  • Confirm the diagnosis of PH
  • Determine the classification group of PH
  • Specify the possible etiology for PH, if present
  • Evaluate the hemodynamic status of the patient

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.

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) and 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. ↑ 1.0 1.1 1.2 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.
  2. ↑ 2.0 2.1 2.2 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)
  3. ↑ 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.
  4. ↑ 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)
  5. ↑ 5.0 5.1 5.2 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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)
  9. ↑ 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)
  10. ↑ 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)
  11. ↑ 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)
  12. ↑ Andreassen, AK.; Wergeland, R.; Simonsen, S.; Geiran, O.; Guevara, C.; Ueland, T. (2006). "N-terminal pro-B-type natriuretic peptide as an indicator of disease severity in a heterogeneous group of patients with chronic precapillary pulmonary hypertension". Am J Cardiol. 98 (4): 525–9. doi:10.1016/j.amjcard.2006.02.061. PMID 16893710. Unknown parameter |month= ignored (help)
  13. ↑ Fijalkowska, A.; Kurzyna, M.; Torbicki, A.; Szewczyk, G.; Florczyk, M.; Pruszczyk, P.; Szturmowicz, M. (2006). "Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension". Chest. 129 (5): 1313–21. doi:10.1378/chest.129.5.1313. PMID 16685024. Unknown parameter |month= ignored (help)

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