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{{Pulmonary hypertension}}
{{Pulmonary hypertension}}
{{CMG}}, Richard Channick, M.D.; '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]], [[User:Lisa Prior|Lisa Prior]], [[Ann Slater|Ann Slater, R.N.]]
{{CMG}}, Richard Channick, M.D.; '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]]; [[User:Lisa Prior|Lisa Prior]]; [[Ann Slater|Ann Slater, R.N.]]; {{Rim}}; {{Jose}}
 
==Overview==
==Overview==
Patients with severe [[pulmonary hypertension classification#WHO Functional Classification|WHO functional class II or III]] pulmonary hypertension (PH) refractory to medical therapy are candidates for surgical intervention, such as [[atrial septostomy]] or [[lung transplantation]]. [[Pulmonary thromboendarterectomy]] (PTE) is a surgical procedure that is used for chronic [[thromboembolic]] pulmonary hypertension.
==Surgery==
==Surgery==
===Atrial Septostomy===
Surgical intervention such as [[atrial septostomy]] or [[lung transplantation]] should be considered among patients with pulmonary arterial hypertension (PAH) who fail to improve on optimal therapy or when medical therapy is unavailable.<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>
 
Failure of clinical improvement among PAH patients with [[pulmonary hypertension classification#WHO Functional Classification|WHO functional class II or III]] is defined as either:<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>
* A stable and unsatisfactory clinical status, or
* An unstable and deteriorating status


Atrial septostomy is a surgical procedure that creates a communication between the right and left [[atria]]. It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood (hypoxia). It is best performed in experienced centers[[Lung transplantation]] cures pulmonary arterial hypertension, but leaves the patient with the complications of transplantation, and a survival of about 5 years.
Failure of clinical improvement among PAH patients with [[pulmonary hypertension classification#WHO Functional Classification|WHO functional class IV]] is defined as either:<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>
* Absence of quick improvement to a WHO functional class III or less, or
* A stable and unsatisfactory clinical status


===Pulmonary thromboendarterectomy===
===Atrial Septostomy===
* [[Atrial septostomy]] is a surgical procedure that creates a shunt between the right and left [[atria]]. 
* It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood ([[hypoxia]]). It is best performed in experienced centers.
* Graded balloon dilatation of [[atrial septostomy]] is the recommended type of [[atrial septostomy]].<ref name="pmid9708453">{{cite journal| author=Sandoval J, Gaspar J, Pulido T, Bautista E, Martínez-Guerra ML, Zeballos M et al.| title=Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment. | journal=J Am Coll Cardiol | year= 1998 | volume= 32 | issue= 2 | pages= 297-304 | pmid=9708453 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9708453  }} </ref>
* [[Atrial septostomy]] might be considered among PH patients with [[pulmonary hypertension classification#WHO Functional Classification|WHO functional class IV]] and [[right heart failure]] refractory to therapy.<ref name="pmid17426198">{{cite journal| author=Kurzyna M, Dabrowski M, Bielecki D, Fijalkowska A, Pruszczyk P, Opolski G et al.| title=Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension. | journal=Chest | year= 2007 | volume= 131 | issue= 4 | pages= 977-83 | pmid=17426198 | doi=10.1378/chest.06-1227 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17426198  }} </ref> 
* [[Atrial septostomy]] should also be considered among PH patients who have [[Eisenmenger's syndrome]], idiopathic PAH, and those awaiting [[lung transplantation]].<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>


[[Pulmonary thromboendarterectomy]] (PTE) is a surgical procedure that is used for chronic thromboembolic pulmonary hypertension. It is the surgical removal of an organized [[thrombus]] (clot) along with the lining of the pulmonary artery; it is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
===Lung Transplantation===
* [[Lung transplantation]] is considered in the treatment of patients with idiopathic PH, PH associated with [[congenital heart disease]], or [[pulmonary veno-occlusive disease]] (PVOD) who fail to improve on optimal medical therapy. 
* Combined [[Lung transplantation|lung]] and [[heart transplantation]] might be considered in selected patients.<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>
* According to the Registry of the International Society for Heart and Lung Transplantation, the survival rates following [[lung transplantation]] are 61%, 49%, and 25 % at 3, 5, and 10 years respectively.<ref name="pmid16890108">{{cite journal| author=Trulock EP, Edwards LB, Taylor DO, Boucek MM, Keck BM, Hertz MI et al.| title=Registry of the International Society for Heart and Lung Transplantation: twenty-third official adult lung and heart-lung transplantation report--2006. | journal=J Heart Lung Transplant | year= 2006 | volume= 25 | issue= 8 | pages= 880-92 | pmid=16890108 | doi=10.1016/j.healun.2006.06.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16890108  }} </ref>
*If patients present with rapidly progressing disease, [[pulmonary transplantation]] should be considered earlier.<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>


Treatment for hypoxic and miscellaneous varieties of pulmonary hypertension have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.
===Pulmonary Thromboendarterectomy===
* [[Pulmonary thromboendarterectomy]] (PTE) is a surgical procedure that is used for the treatment of chronic [[thromboembolic]] pulmonary hypertension.
* It is the surgical removal of an organized [[thrombus]] along with the lining of the [[pulmonary artery]]. 
* [[PTE]] is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
* Treatment for [[hypoxic]] and miscellaneous varieties of PH have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.


==References==
==References==
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{{reflist|2}}
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[[Category:Medicine]]
[[Category:Cardiology]]
[[Category:Pulmonology]]
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Latest revision as of 17:37, 9 June 2021

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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.; Rim Halaby, M.D. [2]; José Eduardo Riceto Loyola Junior, M.D.[3]

Overview

Patients with severe WHO functional class II or III pulmonary hypertension (PH) refractory to medical therapy are candidates for surgical intervention, such as atrial septostomy or lung transplantation. Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is used for chronic thromboembolic pulmonary hypertension.

Surgery

Surgical intervention such as atrial septostomy or lung transplantation should be considered among patients with pulmonary arterial hypertension (PAH) who fail to improve on optimal therapy or when medical therapy is unavailable.[1]

Failure of clinical improvement among PAH patients with WHO functional class II or III is defined as either:[1]

  • A stable and unsatisfactory clinical status, or
  • An unstable and deteriorating status

Failure of clinical improvement among PAH patients with WHO functional class IV is defined as either:[1]

  • Absence of quick improvement to a WHO functional class III or less, or
  • A stable and unsatisfactory clinical status

Atrial Septostomy

Lung Transplantation

Pulmonary Thromboendarterectomy

  • Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is used for the treatment of chronic thromboembolic pulmonary hypertension.
  • It is the surgical removal of an organized thrombus along with the lining of the pulmonary artery.
  • PTE is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
  • Treatment for hypoxic and miscellaneous varieties of PH have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.

References

  1. 1.0 1.1 1.2 1.3 1.4 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.
  2. Sandoval J, Gaspar J, Pulido T, Bautista E, Martínez-Guerra ML, Zeballos M; et al. (1998). "Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment". J Am Coll Cardiol. 32 (2): 297–304. PMID 9708453.
  3. Kurzyna M, Dabrowski M, Bielecki D, Fijalkowska A, Pruszczyk P, Opolski G; et al. (2007). "Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension". Chest. 131 (4): 977–83. doi:10.1378/chest.06-1227. PMID 17426198.
  4. Trulock EP, Edwards LB, Taylor DO, Boucek MM, Keck BM, Hertz MI; et al. (2006). "Registry of the International Society for Heart and Lung Transplantation: twenty-third official adult lung and heart-lung transplantation report--2006". J Heart Lung Transplant. 25 (8): 880–92. doi:10.1016/j.healun.2006.06.001. PMID 16890108.
  5. Poch D, Mandel J (2021). "Pulmonary Hypertension". Ann Intern Med. 174 (4): ITC49–ITC64. doi:10.7326/AITC202104200. PMID 33844574 Check |pmid= value (help).

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