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__NOTOC__
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{{Pulmonary hypertension}}
{{Pulmonary hypertension}}
{{CMG}}; '''Assistant Editor(s)-in-Chief: '''[[User:Ralph Matar|Ralph Matar]]
{{CMG}}; '''Assistant Editor(s)-in-Chief: '''[[User:Ralph Matar|Ralph Matar]]; {{Jose}}


==Overview==
==Overview==


In the NHLBI registry for primary pulmonary hypertension, the mean interval from the onset of symptoms to diagnosis was 2 years, and the most common initial symptoms were [[dyspnea]], [[fatigue]], and [[syncope]]. There was an estimated median survival of 2.8 years for symptomatic patients who do not recieve any treatment, with the most common cause of death as [[cor pulmonale]].
[[Pulmonary hypertension]] most common initial symptoms are [[dyspnea]], [[fatigue]], and [[syncope]]. There was an estimated median survival of 2.8 years for symptomatic patients who do not receive any treatment, with the most common cause of death as [[cor pulmonale]], but survival rates have been increasing as new forms of treatment become available. Despite such advances, [[prognosis]] is still poor.


==Natural History==
==Natural History==


*The National Institutes of Health (NIH) Registry estimated a median survival of 2.8 years for symptomatic patients with idiopathic pulmonary hypertension who do not recieve any treatment, with the cause of death usually being right ventricular failure ([[cor pulmonale]]).<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>  The 1, 3,and 5-year survival rates for untreated patients with idiopathic pulmonary hypertension were 68%, 48%, and 34%, respectively.<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>
*The National Institutes of Health (NIH) Registry estimated a median survival of 2.8 years for symptomatic patients with idiopathic pulmonary hypertension who do not receive any treatment, with the cause of death usually being right ventricular failure ([[cor pulmonale]]).<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>   
*The 1, 3,and 5-year survival rates for untreated patients with idiopathic pulmonary hypertension were 68%, 48%, and 34%, respectively.<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>


*The median survival duration was even lower for patients with pulmonary hypertension that was associated with other diseases like [[portal hypertension]], and [[scleroderma]] (2-year survival of 40% if untreated).
*The median survival duration was even lower for patients with pulmonary hypertension that was associated with other diseases like [[portal hypertension]], and [[scleroderma]] (2-year survival of 40% if untreated).
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*A recent outcome study of those patients who had started treatment with [[bosentan]] demonstrated that 86% patients were alive at 3 years.   
*A recent outcome study of those patients who had started treatment with [[bosentan]] demonstrated that 86% patients were alive at 3 years.   


*With multiple agents now available, combination therapy is increasingly used. Impact of these agents on survival is not known, since many of them have been developed only recently.  It would not be unreasonable to expect median survival to extend past 10 years in the near future.
*With multiple agents now available, combination therapy is increasingly used. Impact of these agents on survival is not known, since many of them have been developed only recently.  It would not be unreasonable to expect median survival to extend past 10 years in the near future.


==Complications==
==Complications==
* [[Right-sided heart failure]] ([[cor pulmonale]]).
Complications that can develop as a result of [[pulmonary hypertension]] are:<ref name="pmid15249497" />
* Blood clots.
* [[Right-sided heart failure]] ([[cor pulmonale]])
* [[Arrhythmia]] (irregular heart beats).
* Blood clots
* Bleeding into the lungs and [[coughing up blood]].
* [[Arrhythmia]]
* [[Hemoptysis]]


==Prognosis==
==Prognosis==
*The long-term prognosis has been known to be poor, however outcomes have changed dramatically over the last two decades. This may be attributed to the use of newer drug therapy, better overall care, and earlier diagnosis (lead time bias).
The prognosis of [[pulmonary hypertension]] is poor, but good with treatment. Without treatment, [[pulmonary hypertension]] will result in [[cor pulmonale]]. [[Pulmonary hypertension]] is associated with a 7 year mortality of 49%.
*The long-term [[prognosis]] has been known to be poor, however outcomes have changed dramatically over the last two decades. This may be attributed to the use of newer [[drug therapy]], better overall care, and earlier diagnosis (lead time bias).
 
*[[Elevated pulmonary arterial pressure]] on heart catheterization is a predictor of [[mortality]], especially if it is happening not only in the setting of [[myocarditis]] or [[decreased right ventricular ejection fraction]], but also [[COPD]]. Treatment of [[pulmonary hypertension]] in these morbidities does not affect outcomes.<ref name="pmid33844574">{{cite journal| author=Poch D, Mandel J| title=Pulmonary Hypertension. | journal=Ann Intern Med | year= 2021 | volume= 174 | issue= 4 | pages= ITC49-ITC64 | pmid=33844574 | doi=10.7326/AITC202104200 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33844574  }} </ref>
 
*Survival rate at 5 years is 57%.<ref name="pmid33844574">{{cite journal| author=Poch D, Mandel J| title=Pulmonary Hypertension. | journal=Ann Intern Med | year= 2021 | volume= 174 | issue= 4 | pages= ITC49-ITC64 | pmid=33844574 | doi=10.7326/AITC202104200 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33844574  }} </ref>
 
*Persistently elevated [[BNP]] or [[NT-proBNP]] levels, [[PAH]] associated with [[connective tissue disease]] or [[portal hypertension]] indicate poorer prognosis.<ref name="pmid33844574">{{cite journal| author=Poch D, Mandel J| title=Pulmonary Hypertension. | journal=Ann Intern Med | year= 2021 | volume= 174 | issue= 4 | pages= ITC49-ITC64 | pmid=33844574 | doi=10.7326/AITC202104200 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33844574  }} </ref>


*Some people with this condition may have [[heart failure]] that could lead to death. Assessment of prognosis in patients with pulmonary arterial hypertension (PAH) is important since it influences both medical therapy and referral for [[transplantation]].
*Some people with this condition may have [[heart failure]] that could lead to death. Assessment of prognosis in patients with pulmonary arterial hypertension (PAH) is important since it influences both medical therapy and referral for [[transplantation]].


*Surprisingly, patients with [[Eisenmenger syndrome]] have a more favorable hemodynamic profile and prognosis than adults with idiopathic or primary pulmonary hypertension.<ref> Hopkins WE,Ochoa LL, Richardson GW, Trulock EP(1996) Comparison of the hemodynamics and survival or patients with severe pulmonary hypertension or Eisenmenger Syndrome.</ref>
*Mortality rate is 5.2-5.4 per 100,000 and is more common in African-Americans and women.


===Indicators of Poor Prognosis===
*The most common cause of hospitalization is [[heart failure]].
* Age>45 at presentation
* Poor functional capacity
* Poor exercise tolerance as assessed by the 6-minute-walk distance (6MWD)
* Elevated [[Brain Natriuretic Peptide]]
* [[Pericardial effusion]]
* Elevated right atrial size and pressure
* Significant right ventricular dysfunction or failure
* Low [[cardiac index]]
* Decreased pulmonary arterial [[capacitance]]
* Underlying [[Scleroderma]] or [[liver disease]]


== ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) <ref name="pmid16935995">{{cite journal| author=Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M et al.| title=ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. | journal=Circulation | year= 2006 | volume= 114 | issue= 10 | pages= e385-484 | pmid=16935995 | doi=10.1161/CIRCULATIONAHA.106.178233 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16935995}}</ref> ==
*The most common cause of death is [[right ventricular failure]] and not chronic lower [[respiratory disease]] as was once thought.
=== Recommendations for Pulmonary Arterial Hypertension===


{|class="wikitable"
*Surprisingly, patients with [[Eisenmenger syndrome]] have a more favorable hemodynamic profile and prognosis than adults with idiopathic or primary pulmonary hypertension.<ref>Hopkins WE,Ochoa LL, Richardson GW, Trulock EP(1996) Comparison of the hemodynamics and survival or patients with severe pulmonary hypertension or Eisenmenger Syndrome.</ref>
|-
|colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class III]]


|-
===Indicators of Poor Prognosis===
|bgcolor="LightCoral"|<nowiki>"</nowiki>'''1.''' Prophylactic antiarrhythmic therapy generally is not indicated for primary prevention of SCD in patients with pulmonary arterial hypertension (PAH) or other pulmonary conditions.''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
Indicators of poor prognosis include:
|}
* Poor functional class<ref name="pmid15249497">{{cite journal| author=McLaughlin VV, Presberg KW, Doyle RL, Abman SH, McCrory DC, Fortin T et al.| title=Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. | journal=Chest | year= 2004 | volume= 126 | issue= 1 Suppl | pages= 78S-92S | pmid=15249497 | doi=10.1378/chest.126.1_suppl.78S | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15249497  }} </ref>
* Poor exercise tolerance as assessed by the 6-minute-walk distance (6MWD)<ref name="pmid8532025">{{cite journal| author=Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB et al.| title=A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. | journal=N Engl J Med | year= 1996 | volume= 334 | issue= 5 | pages= 296-301 | pmid=8532025 | doi=10.1056/NEJM199602013340504 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8532025  }} </ref>
* Elevated [[brain natriuretic peptide]] ([[BNP]])<ref name="pmid20585012">{{cite journal| author=Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS et al.| title=Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 164-72 | pmid=20585012 | doi=10.1161/CIRCULATIONAHA.109.898122 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20585012  }} </ref> or [[NT-proBNP]] levels.<ref name="pmid33844574">{{cite journal| author=Poch D, Mandel J| title=Pulmonary Hypertension. | journal=Ann Intern Med | year= 2021 | volume= 174 | issue= 4 | pages= ITC49-ITC64 | pmid=33844574 | doi=10.7326/AITC202104200 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33844574  }} </ref>
* [[Pericardial effusion]]<ref name="pmid20585012">{{cite journal| author=Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS et al.| title=Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 164-72 | pmid=20585012 | doi=10.1161/CIRCULATIONAHA.109.898122 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20585012  }} </ref>
* Persistently elevated right atrial size and pressure<ref name="pmid1863023">{{cite journal| author=D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM et al.| title=Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. | journal=Ann Intern Med | year= 1991 | volume= 115 | issue= 5 | pages= 343-9 | pmid=1863023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1863023  }} </ref>
* Significant right ventricular dysfunction or failure<ref name="pmid20585012">{{cite journal| author=Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS et al.| title=Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 164-72 | pmid=20585012 | doi=10.1161/CIRCULATIONAHA.109.898122 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20585012  }} </ref>
* Low [[cardiac index]]<ref name="pmid1863023">{{cite journal| author=D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM et al.| title=Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. | journal=Ann Intern Med | year= 1991 | volume= 115 | issue= 5 | pages= 343-9 | pmid=1863023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1863023  }} </ref>
* Underlying connective tissue disease<ref name="pmid20585012">{{cite journal| author=Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS et al.| title=Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 164-72 | pmid=20585012 | doi=10.1161/CIRCULATIONAHA.109.898122 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20585012  }} </ref>
* Decreased predicted carbon monoxide diffusing capacity<ref name="pmid20585012">{{cite journal| author=Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS et al.| title=Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 164-72 | pmid=20585012 | doi=10.1161/CIRCULATIONAHA.109.898122 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20585012  }} </ref>
*[[PAH]] associated with [[connective tissue disease]] or [[portal hypertension]].


==References==
==References==
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor(s)-in-Chief: Ralph Matar; José Eduardo Riceto Loyola Junior, M.D.[2]

Overview

Pulmonary hypertension most common initial symptoms are dyspnea, fatigue, and syncope. There was an estimated median survival of 2.8 years for symptomatic patients who do not receive any treatment, with the most common cause of death as cor pulmonale, but survival rates have been increasing as new forms of treatment become available. Despite such advances, prognosis is still poor.

Natural History

  • The National Institutes of Health (NIH) Registry estimated a median survival of 2.8 years for symptomatic patients with idiopathic pulmonary hypertension who do not receive any treatment, with the cause of death usually being right ventricular failure (cor pulmonale).[1]
  • The 1, 3,and 5-year survival rates for untreated patients with idiopathic pulmonary hypertension were 68%, 48%, and 34%, respectively.[1]
  • The median survival duration was even lower for patients with pulmonary hypertension that was associated with other diseases like portal hypertension, and scleroderma (2-year survival of 40% if untreated).
  • A recent outcome study of those patients who had started treatment with bosentan demonstrated that 86% patients were alive at 3 years.
  • With multiple agents now available, combination therapy is increasingly used. Impact of these agents on survival is not known, since many of them have been developed only recently. It would not be unreasonable to expect median survival to extend past 10 years in the near future.

Complications

Complications that can develop as a result of pulmonary hypertension are:[2]

Prognosis

The prognosis of pulmonary hypertension is poor, but good with treatment. Without treatment, pulmonary hypertension will result in cor pulmonale. Pulmonary hypertension is associated with a 7 year mortality of 49%.

  • The long-term prognosis has been known to be poor, however outcomes have changed dramatically over the last two decades. This may be attributed to the use of newer drug therapy, better overall care, and earlier diagnosis (lead time bias).
  • Survival rate at 5 years is 57%.[3]
  • Some people with this condition may have heart failure that could lead to death. Assessment of prognosis in patients with pulmonary arterial hypertension (PAH) is important since it influences both medical therapy and referral for transplantation.
  • Mortality rate is 5.2-5.4 per 100,000 and is more common in African-Americans and women.
  • Surprisingly, patients with Eisenmenger syndrome have a more favorable hemodynamic profile and prognosis than adults with idiopathic or primary pulmonary hypertension.[4]

Indicators of Poor Prognosis

Indicators of poor prognosis include:

References

  1. ↑ 1.0 1.1 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 McLaughlin VV, Presberg KW, Doyle RL, Abman SH, McCrory DC, Fortin T; et al. (2004). "Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines". Chest. 126 (1 Suppl): 78S–92S. doi:10.1378/chest.126.1_suppl.78S. PMID 15249497.
  3. ↑ 3.0 3.1 3.2 3.3 Poch D, Mandel J (2021). "Pulmonary Hypertension". Ann Intern Med. 174 (4): ITC49–ITC64. doi:10.7326/AITC202104200. PMID 33844574 Check |pmid= value (help).
  4. ↑ Hopkins WE,Ochoa LL, Richardson GW, Trulock EP(1996) Comparison of the hemodynamics and survival or patients with severe pulmonary hypertension or Eisenmenger Syndrome.
  5. ↑ Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB; et al. (1996). "A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension". N Engl J Med. 334 (5): 296–301. doi:10.1056/NEJM199602013340504. PMID 8532025.
  6. ↑ 6.0 6.1 6.2 6.3 6.4 Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS; et al. (2010). "Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)". Circulation. 122 (2): 164–72. doi:10.1161/CIRCULATIONAHA.109.898122. PMID 20585012.
  7. ↑ 7.0 7.1 D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM; et al. (1991). "Survival in patients with primary pulmonary hypertension. Results from a national prospective registry". Ann Intern Med. 115 (5): 343–9. PMID 1863023.

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