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==Overview==
==Overview==
In 1891, a German physician Ernst von Romberg described pulmonary vascular sclerosis in an autopsy. In 1951 David Dresdale coined the term primary pulmonary hypertension for the first time. In 1951, David Dresdale coined the term primary pulmonary hypertension for the first time. In 1981, pulmonary hypertension registry landmark multi-center U.S. study characterizing natural history and clinical features of primary pulmonary hypertension (PPH).


==Historical Perspective==
==Historical Perspective==
The incidence of PAH associated with anorexigens is cyclical in nature and varies depending on the availability of specific appetite suppressants. The link was first identified in the 1960s when an epidemic of PAH occurred in Switzerland, Austria and Germany that was linked to the anorexigen aminorex fumarate. Use of the anorexigens fenfluramine and dexfenfluramine have also been linked with an increased risk for PAH.
* The historical perspective of the PH is as follows:<ref name="pmid2700428">{{cite journal |vauthors=Smith DB |title=Continent diversions: an overview |journal=Dimens Oncol Nurs |volume=3 |issue=4 |pages=18–23 |date=1989 |pmid=2700428 |doi= |url=}}</ref><ref name="pmid14033007">{{cite journal |vauthors=KIRKENDALL WM, GIFFORD RW, HORWITZ D, WILSON WR, GOLDBERG LI, GROLLMAN A |title=General aspects of hypertension; past, present and future |journal=Postgrad Med |volume=34 |issue= |pages=150–6 |date=August 1963 |pmid=14033007 |doi= |url=}}</ref>
 
** In 1891, a German physician Ernst von Romberg described pulmonary vascular sclerosis in an autopsy.
Prior to the development of disease-specific targeted PAH therapies, the median survival for subjects diagnosed with IPAH was approximately 2.8 years. However, 2.8 years likely underestimates current survival as the course of the disease has been favorably altered by therapeutic advances since that report from the 1980s. Prognosis is also dependent on the underlying etiology of the disease. The prognosis for patients with PAH associated with connective tissue disease appears to be worse than for those with IPAH. Estimates for 2-year survival in scleroderma patients with associated PAH are 40% compared with 48% for 3-year survival in patients with IPAH. Survival in patients with HIV-associated PAH is similar to patients with IPAH. With current HIV therapies, most of the deaths in patients with HIV and associated PAH are now attributed to PAH.
** In 1901, Abel Ayerza Key lecture integrating cyanosis and right heart failure, named the condition as '''''cardiac negro'' - black heart.'''
 
** In 1913, F. C. Arrillaga, assigned syphilitic arteriosclerosis as etiology of PH; changed the name of disease to '''''Ayerza’s Disease.'''''
 
** In 1929, Werner Forssman demonstrated that it was possible to perform [[Right heart catheterization|right sided catheterization]] in humans by performing [[catheterization]] on himself.
Although Ernst von Romberg, a German physician, described an autopsy in 1891 as ‘pulmonary vascular sclerosis,’ it is only since 1995 with the introduction of intravenous epoprostenol that disease-specific targeted medical therapies for PAH have become available. In addition, significant advances in the treatment of PAH have occurred during the past decade, with six medical therapies now having received regulatory approval worldwide targeting the prostacyclin pathway, the nitric oxide pathway and the endothelin pathway [Figure 1]. Furthermore, ongoing clinical trials are evaluating novel therapeutic approaches based on scientific insights gleaned over the past decade in the pathobiology of PAH
** In 1951, David Dresdale coined the term primary pulmonary hypertension for the first time.
 
** In 1956, Forssman, Cournand, and Richards were awarded the Nobel Prize for their contributions to the discovery of circulatory and cardiopulmonary systems.
From a therapeutic standpoint, why had it taken from 1891 until 1995 to develop a safe and efficacious therapeutic modality for the treatment of PAH? [Figure 3] Although several reports of young women dying of right heart failure without a diagnosis were published in 1940, it was not until pulmonary artery pressures could be recorded directly with the introduction of right heart catheterization that the physiology of the pulmonary circulation could be studied. In 1951, Dresdale tested the acute effects of tolzoline in a young woman with IPAH; the tolzoline caused a sudden decrease in pulmonary artery pressure and pulmonary vascular resistance without significant systemic effects. Unfortunately, no drugs were available at that time for chronic treatment. However, despite this, there remained little interest in PAH until the epidemic of the aminorex-induced PAH became apparent in the late 1960s. Prompted by the aminorex-induced PAH epidemic in 1973, the World Health Organization (WHO) held its first meeting in Geneva to assess what was known about IPAH and what remained unknown. In 1981, the National Heart, Lung and Blood Institute of the National Institutes of Health supported a national registry of patients with IPAH, which resulted in several reports over the next decade describing clinical features of IPAH and its natural history. Interestingly, despite the fact that IPAH was an orphan disease, significant interest from the scientific community rapidly ensued. Advances in the understanding of the mechanisms involved in the pathobiology of IPAH and PAH associated with other conditions have focused on molecular biology, developmental biology and genetics. Together with epidemiological and natural history studies, collaborative efforts between the scientific community and industry have led to a surge in clinical trials over the past decade: since the approval of intravenous epoprostenol for the treatment of IPAH in 1995, the prostacyclin analogue treprostinil has been approved for continuous subcutaneous infusion in 2002 and for continuous intravenous infusion in 2004. In addition, the prostacyclin analogue iloprost was approved in 2004 via inhalation. In 2001, bosentan, an endothelin ET A/ETB receptor antagonist, was the first oral therapy approved for the treatment of PAH; and sildenafil citrate, an oral phosphodiesterase type 5 inhibitor, was approved in 2005. In 2007, the oral ETA selective ERA ambrisentan was approved, and the oral ETA selective ERA sitaxsentan was approved in the EU.
** In 1958, Paul Wood published pulmonary hypertension with an association with a vasoconstrictive factor.
 
** During 1965–1970s, first pulmonary hypertension epidemic related to the approval of Aminorex.
Prompted by the scientific insights from the 1990s, in 1998 the second WHO meeting was held on the 25th anniversary of the original meeting; and with the dramatic advances over the next 5 years, the 3rd WHO Symposium on PAH was held in 2003 and the 4th World Symposium on PAH in 2008. Based on the clinical trials to date, current consensus evidence-based guidelines for the treatment of PAH are shown in [Figure 4]. What have we been able to achieve? The disease-specific PAH therapies, currently available in conjunction with anticoagulant, diuretic, digitalis and oxygen therapy, have improved exercise capacity, functional capacity, time to clinical worsening, hemodynamic parameters, overall quality of life and survival. However, PAH remains a devastating, life-threatening disorder. In more than 50% of patients, exercise capacity remains significantly limited, approximately 50% of patients remain WHO functional class III or IV, PAH patients continue to have frequent hospitalizations for PAH, right heart function remains significantly impaired in most patients, quality of life is suboptimal and despite an increase in survival for functional class III and IV patients with IPAH from a predicted survival of 33% (based on the NIH Registry) to 63% with our current therapeutic modalities, the outlook is far from ideal; we need to continue to aggressively pursue furthering our understanding of PAH if we ever hope to give these patients a near-normal life.
** In 1973, '''''1st WHO Meeting on pulmonary hypertension''''' the landmark meeting about pulmonary hypertension, provided recommendations for future directions.
** In 1981, pulmonary hypertension registry landmark multi-center U.S. study characterizing natural history and clinical features of primary pulmonary hypertension (PPH).
** In 1998, '''''2nd World Symposium on pulmonary hypertension,''''' formation of Evian Classification of pulmonary hypertension and the term pulmonary arterial hypertension (PAH) was coined.
** In 2003, '''''3rd World Symposium on pulmonary hypertension,''''' formation of Venice Classification; idiopathic PAH introduced to replace term PPH.
** In 2008, '''''4th World Symposium on pulmonary hypertension,''''' formation of Dana Point Classification; modification of genetic category, among others in Group 1.
** In 2013, '''''5th World Symposium on pulmonary hypertension,''''' formation of Nice Classification; further modifications of genetic category, among others in Group 1.
** In 2014, '''''International Right Heart Failure Foundation''''' - comprehensive nomenclature of RHF ([[right heart failure]]) defining distinction between [[right heart failure]] vs [[Right heart failure|right ventricular failure]] vs components of right heart system, and the definition of [[right heart failure]].


==References==
==References==
{{reflist|2}}
{{reflist|2}}


[[Category:Medicine]]
[[Category:Cardiology]]
[[Category:Pulmonology]]
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Latest revision as of 14:59, 27 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

In 1891, a German physician Ernst von Romberg described pulmonary vascular sclerosis in an autopsy. In 1951 David Dresdale coined the term primary pulmonary hypertension for the first time. In 1951, David Dresdale coined the term primary pulmonary hypertension for the first time. In 1981, pulmonary hypertension registry landmark multi-center U.S. study characterizing natural history and clinical features of primary pulmonary hypertension (PPH).

Historical Perspective

  • The historical perspective of the PH is as follows:[1][2]
    • In 1891, a German physician Ernst von Romberg described pulmonary vascular sclerosis in an autopsy.
    • In 1901, Abel Ayerza Key lecture integrating cyanosis and right heart failure, named the condition as cardiac negro - black heart.
    • In 1913, F. C. Arrillaga, assigned syphilitic arteriosclerosis as etiology of PH; changed the name of disease to Ayerza’s Disease.
    • In 1929, Werner Forssman demonstrated that it was possible to perform right sided catheterization in humans by performing catheterization on himself.
    • In 1951, David Dresdale coined the term primary pulmonary hypertension for the first time.
    • In 1956, Forssman, Cournand, and Richards were awarded the Nobel Prize for their contributions to the discovery of circulatory and cardiopulmonary systems.
    • In 1958, Paul Wood published pulmonary hypertension with an association with a vasoconstrictive factor.
    • During 1965–1970s, first pulmonary hypertension epidemic related to the approval of Aminorex.
    • In 1973, 1st WHO Meeting on pulmonary hypertension the landmark meeting about pulmonary hypertension, provided recommendations for future directions.
    • In 1981, pulmonary hypertension registry landmark multi-center U.S. study characterizing natural history and clinical features of primary pulmonary hypertension (PPH).
    • In 1998, 2nd World Symposium on pulmonary hypertension, formation of Evian Classification of pulmonary hypertension and the term pulmonary arterial hypertension (PAH) was coined.
    • In 2003, 3rd World Symposium on pulmonary hypertension, formation of Venice Classification; idiopathic PAH introduced to replace term PPH.
    • In 2008, 4th World Symposium on pulmonary hypertension, formation of Dana Point Classification; modification of genetic category, among others in Group 1.
    • In 2013, 5th World Symposium on pulmonary hypertension, formation of Nice Classification; further modifications of genetic category, among others in Group 1.
    • In 2014, International Right Heart Failure Foundation - comprehensive nomenclature of RHF (right heart failure) defining distinction between right heart failure vs right ventricular failure vs components of right heart system, and the definition of right heart failure.

References

  1. Smith DB (1989). "Continent diversions: an overview". Dimens Oncol Nurs. 3 (4): 18–23. PMID 2700428.
  2. KIRKENDALL WM, GIFFORD RW, HORWITZ D, WILSON WR, GOLDBERG LI, GROLLMAN A (August 1963). "General aspects of hypertension; past, present and future". Postgrad Med. 34: 150–6. PMID 14033007.


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