Ebsteins anomaly of the tricuspid valve medical therapy: Difference between revisions

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__NOTOC__
{{Template:ebstein's anomaly of the tricuspid valve}}
{{Template:ebstein's anomaly of the tricuspid valve}}
{{CMG}} and Claudia P. Hochberg, M.D. [mailto:chochber@bidmc.harvard.edu]
{{CMG}} and Claudia P. Hochberg, M.D. [mailto:chochber@bidmc.harvard.edu]'''Associate Editor-In-Chief:''' {{CZ}},{{M.N}}
__NOTOC__
 
'''Associate Editor-In-Chief:''' {{CZ}}
==Overview==
[[Medical]] management of [[patients]] with Ebstein’s anomaly consists of supportive care such as control of the [[heart rhythm]] with [[antiarrhythmic drugs]], [[Inotropic agent|inotropic agents]] and [[diuretics]] for [[heart failure]], [[anticoagulation]] in [[patients]] with [[atrial fibrillation]] and [[paradoxical embolization]].


==Medical Therapy==
==Medical therapy==
===Antiarrhythmics===
*Some Ebstein's anomaly [[patients]] present with an ([[antidromic]]) [[AV nodal reentrant tachycardia]] with associated [[pre-excitation]]. Among these [[patients]], the preferred [[pharmacological]] treatment agent is [[procainamide]]. Since AV-blockade may promote [[Conduction System|conduction]] over the [[accessory pathway]], [[drugs]] such as [[beta blockers]], [[calcium channel blocker]]s and [[digoxin]] are contraindicated. If there is [[atrial fibrillation]] with [[pre-excitation]], treatment options include [[procainamide]], [[flecainide]], [[propafenone]], [[dofetilide]] and [[ibutilide]] since these [[medications]] slow [[Conduction System|conduction]] in the [[accessory pathway]] causing the [[tachycardia]] and should be administered before considering electrical [[cardioversion]].  Intravenous [[amiodarone]] may also convert [[atrial fibrillation]] and/or slow the [[ventricular]] response<ref name="pmid28072699">{{cite journal |vauthors=Luo M, Lin J, Qin Z, Du L |title=Prevention preferable to treatment: 3 case reports of patients experiencing right-sided heart failure after Ebstein anomaly correction |journal=Medicine (Baltimore) |volume=96 |issue=1 |pages=e5627 |date=January 2017 |pmid=28072699 |pmc=5228659 |doi=10.1097/MD.0000000000005627 |url=}}</ref><ref name="pmid27709852">{{cite journal |vauthors=Kim HY, Jang SY, Moon JR, Kim EK, Chang SA, Song J, Huh J, Kang IS, Yang JH, Jun TG, Park SW |title=Natural Course of Adult Ebstein Anomaly When Treated according to Current Recommendation |journal=J. Korean Med. Sci. |volume=31 |issue=11 |pages=1749–1754 |date=November 2016 |pmid=27709852 |pmc=5056206 |doi=10.3346/jkms.2016.31.11.1749 |url=}}</ref>


=== Mechanical and Device Based Therapy ===
===Anticoagulation===
*[[Warfarin]] is recommended for [[patients]] with Ebstein’s anomaly with a history of [[Paradoxical embolization|paradoxical embolus]] or [[atrial fibrillation]].


The protracted natural history of this disease in contrast to the uncertainty of long-term results of surgical intervention supports the policy of postponing surgical intervention until the onset of disability.
===Heart Failure Management===
*[[Diuretics]] may aid in reducing [[volume]] overload.  [[Cardiac glycosides]] like [[digoxin]] can be used to maintain proper [[heart rhythm]] and increase [[contractility]].


=== Pharmacological Management ===
==ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)<ref name="pmid18997168">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). | journal=Circulation | year= 2008 | volume= 118 | issue= 23 | pages= 2395-451 | pmid=18997168 | doi=10.1161/CIRCULATIONAHA.108.190811 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18997168  }} </ref><ref name="pmid19038677">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 23 | pages= e1-121 | pmid=19038677 | doi=10.1016/j.jacc.2008.10.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19038677  }} </ref>==


Ebstein's cardiophysiology typically presents as an (antidromic) [[AV nodal reentrant tachycardia]] with associated [[pre-excitation]]. In this setting, the preferred pharmacological treatment agent is [[procainamide]]. Since AV-blockade may promote conduction over the accessory pathway, drugs like [[beta blockers]], [[calcium channel blocker]]s and [[digoxin]] are contraindicated.
===Medical Therapy (DO NOT EDIT)<ref name="pmid18997168">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). | journal=Circulation | year= 2008 | volume= 118 | issue= 23 | pages= 2395-451 | pmid=18997168 | doi=10.1161/CIRCULATIONAHA.108.190811 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18997168  }} </ref><ref name="pmid19038677">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 23 | pages= e1-121 | pmid=19038677 | doi=10.1016/j.jacc.2008.10.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19038677  }} </ref> ===


If there is [[atrial fibrillation]] with pre-excitation, treatment options include [[procainamide]], [[flecainide]], [[propafenone]], [[dofetilide]] and [[ibutilide]] since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical [[cardioversion]]. Intravenous [[amiodarone]] may also convert atrial fibrillation and/or slow the ventricular response.
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]


|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Anticoagulation with [[warfarin]] is recommended for patients with Ebstein’s anomaly with a history of paradoxical embolus or [[atrial fibrillation]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|}


==References==
==References==
{{Reflist}}
{{Reflist|2}}


{{WH}}
{{WS}}


{{Congenital malformations and deformations of circulatory system}}
[[Category:Cardiovascular system]]
[[Category:DiseaseState]]
[[Category:Cardiology]]
[[Category: Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
 
[[Category:Medicine]]
{{WH}}
[[Category:Surgery]]
 
{{WS}}

Latest revision as of 22:32, 7 July 2020

Ebsteins anomaly of the tricuspid valve Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] and Claudia P. Hochberg, M.D. [2]Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [3], Maneesha Nandimandalam, M.B.B.S.[4]

Overview

Medical management of patients with Ebstein’s anomaly consists of supportive care such as control of the heart rhythm with antiarrhythmic drugs, inotropic agents and diuretics for heart failure, anticoagulation in patients with atrial fibrillation and paradoxical embolization.

Medical therapy

Antiarrhythmics

Anticoagulation

Heart Failure Management

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[3][4]

Medical Therapy (DO NOT EDIT)[3][4]

Class I
"1. Anticoagulation with warfarin is recommended for patients with Ebstein’s anomaly with a history of paradoxical embolus or atrial fibrillation. (Level of Evidence: B)"

References

  1. ↑ Luo M, Lin J, Qin Z, Du L (January 2017). "Prevention preferable to treatment: 3 case reports of patients experiencing right-sided heart failure after Ebstein anomaly correction". Medicine (Baltimore). 96 (1): e5627. doi:10.1097/MD.0000000000005627. PMC 5228659. PMID 28072699.
  2. ↑ Kim HY, Jang SY, Moon JR, Kim EK, Chang SA, Song J, Huh J, Kang IS, Yang JH, Jun TG, Park SW (November 2016). "Natural Course of Adult Ebstein Anomaly When Treated according to Current Recommendation". J. Korean Med. Sci. 31 (11): 1749–1754. doi:10.3346/jkms.2016.31.11.1749. PMC 5056206. PMID 27709852.
  3. ↑ 3.0 3.1 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease)". Circulation. 118 (23): 2395–451. doi:10.1161/CIRCULATIONAHA.108.190811. PMID 18997168.
  4. ↑ 4.0 4.1 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.

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