Eisenmenger’s syndrome surgery: Difference between revisions

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==Overview==
==Overview==
[[Congenital heart defects]] should undergo surgical repair before progression to [[Eisenmenger's syndrome]]. Once the condition develops into [[Eisenmenger's syndrome]], no surgical cure is available except for heart and lung [[transplantation]]. However, [[Palliative care|palliative]] interventions may prolong the [[lifespan]] and improve the [[quality of life]].  
[[Congenital heart defects]] should undergo surgical repair before progression to [[Eisenmenger's syndrome]]. Once the condition develops into [[Eisenmenger's syndrome]], no surgical cure is available except for heart and lung [[transplantation]]. However, [[Palliative care|palliative]] interventions (as creation of an artificial [[Atrial septal defect|ASD]]) may prolong the [[lifespan]] and improve the [[quality of life]].  


==Surgery==
==Surgery==
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* [[Congenital heart defects]] should undergo surgical repair before progression to [[Eisenmenger's syndrome]].<ref name="pmid30586767">Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM et al. (2019) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=30586767 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.] ''Circulation'' 139 (14):e698-e800. [http://dx.doi.org/10.1161/CIR.0000000000000603 DOI:10.1161/CIR.0000000000000603] PMID: [https://pubmed.gov/30586767 30586767]</ref>
* [[Congenital heart defects]] should undergo surgical repair before progression to [[Eisenmenger's syndrome]].<ref name="pmid30586767">Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM et al. (2019) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=30586767 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.] ''Circulation'' 139 (14):e698-e800. [http://dx.doi.org/10.1161/CIR.0000000000000603 DOI:10.1161/CIR.0000000000000603] PMID: [https://pubmed.gov/30586767 30586767]</ref>
* Once the condition develops into [[Eisenmenger's syndrome]], no surgical cure is available except for heart and lung [[transplantation]].
* Once the condition develops into [[Eisenmenger's syndrome]], no surgical cure is available except for heart and lung [[transplantation]].
*This is because the defect itself may prevent worsening of the [[pulmonary vascular resistance]] in the face of increasing right ventricular pressure.<ref name="pmid30031003">{{cite journal| author=Fathallah M, Krasuski RA| title=A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease. | journal=Prog Cardiovasc Dis | year= 2018 | volume= 61 | issue= 3-4 | pages= 320-327 | pmid=30031003 | doi=10.1016/j.pcad.2018.07.017 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30031003  }}</ref>


* However, [[Palliative care|palliative]] interventions may prolong the [[lifespan]] and improve the [[quality of life]].  
* However, [[Palliative care|palliative]] interventions may prolong the [[lifespan]] and improve the [[quality of life]].  
* For example, creation of an artificial [[Atrial septal defect|ASD]] can decompress the high systolic right ventricular pressure in patients with suprasystemic [[pulmonary artery]] pressures and impending [[right ventricular failure]].
* For example, creation of an artificial [[Atrial septal defect|ASD]] can decompress the high systolic right ventricular pressure in patients with suprasystemic [[pulmonary artery]] pressures and impending [[right ventricular failure]].
*Surgical correction of the causative heart defect in adult patients is generally contraindicated. In patients who have developed PAH as a consequence of unrepaired CHD, the defect itself may be acting as a protective measure, preventing worsening of the pulmonary vascular resistance in the face of increasing right ventricular pressure. In children, development of pulmonary vascular disease (greater than 6 Woods units/m2) with poor vasodilator response are likely to face post-surgical complications, including right heart failure, increased pulmonary hypertension, and hypertensive crises.    Classically, it is thought to be of little benefit to undergo such risky procedures with a plethora of potential consequences. Additionally, even with clinical response to vasodilator therapies for the pulmonary arterial hypertension component, there are only a select few case reports that show benefit for the closure of the anatomical defect. However, there is promising evidence that cardiac surgery, along with close peri- and post-operational management of pulmonary vascular resistance with advanced PAH therapies, may improve safety and mitigate downstream problems
*In children, [[pulmonary vascular resistance]] > 6 Woods units/m2 and poor vasodilator response are predictors of post-surgical [[Complication (medicine)|complications]] as [[right heart failure]], [[pulmonary hypertension]], and [[Hypertensive crisis resident survival guide|hypertensive crises]].  
*However, [[surgery]] in general is though to lack benefit with a plethora of potential [[Complication (medicine)|complications]].<ref name="pmid22348268">{{cite journal| author=Huang JB, Liang J, Zhou LY| title=Eisenmenger syndrome: not always inoperable. | journal=Respir Care | year= 2012 | volume= 57 | issue= 9 | pages= 1488-95 | pmid=22348268 | doi=10.4187/respcare.01418 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22348268  }}</ref>


==References==
==References==

Latest revision as of 11:03, 20 January 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Abdelrahman Ibrahim Abushouk, MD[2], Cafer Zorkun, M.D., Ph.D. [3]

Overview

Congenital heart defects should undergo surgical repair before progression to Eisenmenger's syndrome. Once the condition develops into Eisenmenger's syndrome, no surgical cure is available except for heart and lung transplantation. However, palliative interventions (as creation of an artificial ASD) may prolong the lifespan and improve the quality of life.

Surgery

References

  1. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM et al. (2019) 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 139 (14):e698-e800. DOI:10.1161/CIR.0000000000000603 PMID: 30586767
  2. Fathallah M, Krasuski RA (2018). "A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease". Prog Cardiovasc Dis. 61 (3–4): 320–327. doi:10.1016/j.pcad.2018.07.017. PMID 30031003.
  3. Huang JB, Liang J, Zhou LY (2012). "Eisenmenger syndrome: not always inoperable". Respir Care. 57 (9): 1488–95. doi:10.4187/respcare.01418. PMID 22348268.

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