Ebsteins anomaly of the tricuspid valve surgery: Difference between revisions

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__NOTOC__
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{{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]''' Associate Editor-In-Chief:''' {{CZ}}
{{CMG}} and Claudia P. Hochberg, M.D. [mailto:chochber@bidmc.harvard.edu]''' Associate Editor-In-Chief:''' {{CZ}},{{M.N}}


==Overview==
==Overview==
[[Tricuspid valve]] repair is indicated in [[patients]] in whom there are [[symptoms]] or deteriorating [[exercise capacity]], [[cyanosis]] ([[oxygen saturation]] less than 90%), [[paradoxical embolism]], progressive [[cardiomegaly]] on [[chest x-ray]] or progressive [[right ventricular dilation]] or reduction of  [[right ventricular]] [[systolic]] function.  When possible, repair is favored over replacement.


==Surgery==
==Indications==
*Decreasing [[exercise capacity]]<ref name="pmid16087085">{{cite journal |vauthors=Dearani JA, Danielson GK |title=Surgical management of Ebstein's anomaly in the adult |journal=Semin. Thorac. Cardiovasc. Surg. |volume=17 |issue=2 |pages=148–54 |date=2005 |pmid=16087085 |doi=10.1053/j.semtcvs.2005.02.008 |url=}}</ref><ref name="pmid431125">{{cite journal |vauthors=Danielson GK, Maloney JD, Devloo RA |title=Surgical repair of Ebstein's anomaly |journal=Mayo Clin. Proc. |volume=54 |issue=3 |pages=185–92 |date=March 1979 |pmid=431125 |doi= |url=}}</ref><ref name="pmid17198815">{{cite journal |vauthors=da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, Soares AM, Moreira LF, Barbero-Marcial M |title=The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results |journal=J. Thorac. Cardiovasc. Surg. |volume=133 |issue=1 |pages=215–23 |date=January 2007 |pmid=17198815 |doi=10.1016/j.jtcvs.2006.09.018 |url=}}</ref><ref name="pmid23743062">{{cite journal |vauthors=Dearani JA, Said SM, Burkhart HM, Pike RB, O'Leary PW, Cetta F |title=Strategies for tricuspid re-repair in Ebstein malformation using the cone technique |journal=Ann. Thorac. Surg. |volume=96 |issue=1 |pages=202–8: discussion 208–10 |date=July 2013 |pmid=23743062 |doi=10.1016/j.athoracsur.2013.02.067 |url=}}</ref>
*[[Cyanosis]]
*[[Paradoxical embolism]]
*[[Congestive cardiac failure]]
*Progressive RV( [[right ventricular dilation]])
*[[Right ventricle|RV]] [[systolic dysfunction]]


===ACC / AHA Guidelines- Recommendations for Surgical Interventions (DO NOT EDIT)===
Generally, surgical treatment improves the [[exercise intolerance]] from [[NYHA]]-FC III or IV to [[NYHA]]-FC I or II.


{|class="wikitable"
==Surgery==
The main purpose of the surgery is to improve tricuspid valve  function and there by increase the right heart output which can be accomplished by partial or complete closure of any interatrial communication, repair or replacement of the TV, and reduction of RA size.<ref name="pmid24294540">{{cite journal |vauthors=Romfh A, Pluchinotta FR, Porayette P, Valente AM, Sanders SP |title=Congenital Heart Defects in Adults : A Field Guide for Cardiologists |journal=J Clin Exp Cardiolog |volume= |issue=Suppl 8 |pages= |date=June 2012 |pmid=24294540 |pmc=3842121 |doi=10.4172/2155-9880.s8-007 |url=}}</ref>
<ref name="pmid23743062">{{cite journal |vauthors=Dearani JA, Said SM, Burkhart HM, Pike RB, O'Leary PW, Cetta F |title=Strategies for tricuspid re-repair in Ebstein malformation using the cone technique |journal=Ann. Thorac. Surg. |volume=96 |issue=1 |pages=202–8: discussion 208–10 |date=July 2013 |pmid=23743062 |doi=10.1016/j.athoracsur.2013.02.067 |url=}}</ref><ref name="pmid17198815">{{cite journal |vauthors=da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, Soares AM, Moreira LF, Barbero-Marcial M |title=The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results |journal=J. Thorac. Cardiovasc. Surg. |volume=133 |issue=1 |pages=215–23 |date=January 2007 |pmid=17198815 |doi=10.1016/j.jtcvs.2006.09.018 |url=}}</ref><ref name="pmid431125">{{cite journal |vauthors=Danielson GK, Maloney JD, Devloo RA |title=Surgical repair of Ebstein's anomaly |journal=Mayo Clin. Proc. |volume=54 |issue=3 |pages=185–92 |date=March 1979 |pmid=431125 |doi= |url=}}</ref>
{| class="wikitable"
|+Various surgical strategies employed in repair of Ebstein's anomaly
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Surgeon'''}}
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Effect on Tricuspid Valve'''}}
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Valve Coaptation'''}}
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Effect on Atrial Size'''}}
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Effect on Functional RV'''}}
|-
|Hunter and Lillehei
|Valve is brought to anatomic annulus by transverse plication of atrialized RV
|Anterior and posterior leaflets function as bicuspid valve
|Reduced
|No effect
|-
|Danielson
|Valve is repaired or replaced at level of functional annulus (high need for TVR
|Anterior leaflet functions as monocusp
|Reduced (transverse plication of atrialized RV)
|No effect
|-
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|Carpentier
|Valve is brought to the anatomic annulus (anterior/posterior leaflets detached, annular ring)
|Coaptation between leaflets and ventricular septum
|Reduced (longitudinal plication of atrialized RV)
|Increased
|-
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.'''  Surgeons with training and expertise in CHD should perform tricuspid valve repair or replacement with concomitant closure of an ASD, when present, for patients with Ebstein’s anomaly with the following indications:
|Da Silva
|Valve is brought to anatomic annulus
|Coaptation between leaflets
|Reduced
|Increased
|}


a. Symptoms or deteriorating exercise capacity. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])


b. Cyanosis (oxygen saturation less than 90%). ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])
{{#ev:youtube|QjfJK1zY7BM}}


c. Paradoxical embolism. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])
==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>==


d. Progressive cardiomegaly on chest x-ray. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])
===Surgical Interventions (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> ===


e. Progressive RV dilation or reduction of RV systolic function. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])<nowiki>"</nowiki>
{|class="wikitable"
|-
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.'''Surgeons with training and expertise in CHD should perform concomitant arrhythmia surgery in patients with Ebstein’s anomaly and the following indications:
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]


a. Appearance/progression of atrial and/or ventricular arrhythmias not amenable to percutaneous treatment. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Surgeons with training and expertise in CHD should perform tricuspid valve repair or replacement with concomitant closure of an ASD, when present, for patients with Ebstein’s anomaly with the following indications:
|-
| bgcolor="LightGreen"|'''a.''' Symptoms or deteriorating exercise capacity. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''b.''' Cyanosis (oxygen saturation less than 90%). ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''c.''' Paradoxical embolism. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''d.''' Progressive cardiomegaly on chest x-ray. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''e.''' Progressive RV dilation or reduction of RV systolic function. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>


b. Ventricular preexcitation not successfully treated in the electrophysiology laboratory. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.'''Surgeons with training and expertise in CHD should perform concomitant arrhythmia surgery in patients with Ebstein’s anomaly and the following indications:
|-
| bgcolor="LightGreen"|'''a.''' Appearance/progression of atrial and/or ventricular arrhythmias not amenable to percutaneous treatment. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''b.''' Ventricular preexcitation not successfully treated in the electrophysiology laboratory. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>


3. Surgical rerepair or replacement of the tricuspid valve is recommended in adults with Ebstein’s anomaly with the following indications:
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''3.''' Surgical rerepair or replacement of the tricuspid valve is recommended in adults with Ebstein’s anomaly with the following indications:
|-
| bgcolor="LightGreen"|'''a.''' Symptoms, deteriorating exercise capacity, or New York Heart Association functional class III or IV. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''b.''' Severe TR after repair with progressive RV dilation, reduction of RV systolic function, or appearance/ progression of atrial and/or ventricular arrhythmias. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''c.''' Bioprosthetic tricuspid valve dysfunction with significant mixed regurgitation and stenosis. (Level of Evidence: B)
|-
| bgcolor="LightGreen"|'''d.''' Predominant bioprosthetic valve stenosis (mean gradient greater than 12 to 15 mm Hg). ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''e.''' Operation can be considered earlier with lesser degrees of bioprosthetic stenosis with symptoms or decreased exercise tolerance. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|}


a. Symptoms, deteriorating exercise capacity, or New York Heart Association functional class III or IV. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])
==ACC/AHA 2018 Guidelines for the Management of Adults With Ebstein Anomaly(DO NOT EDIT)<ref name="pmid30121239">{{cite journal |vauthors=Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF |title=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 |journal=J. Am. Coll. Cardiol. |volume=73 |issue=12 |pages=e81–e192 |date=April 2019 |pmid=30121239 |doi=10.1016/j.jacc.2018.08.1029 |url=}}</ref><ref name="StoutDaniels2019">{{cite journal|last1=Stout|first1=Karen K.|last2=Daniels|first2=Curt J.|last3=Aboulhosn|first3=Jamil A.|last4=Bozkurt|first4=Biykem|last5=Broberg|first5=Craig S.|last6=Colman|first6=Jack M.|last7=Crumb|first7=Stephen R.|last8=Dearani|first8=Joseph A.|last9=Fuller|first9=Stephanie|last10=Gurvitz|first10=Michelle|last11=Khairy|first11=Paul|last12=Landzberg|first12=Michael J.|last13=Saidi|first13=Arwa|last14=Valente|first14=Anne Marie|last15=Van Hare|first15=George F.|title=2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease|journal=Journal of the American College of Cardiology|volume=73|issue=12|year=2019|pages=e81–e192|issn=07351097|doi=10.1016/j.jacc.2018.08.1029}}</ref>==


b. Severe TR after repair with progressive RV dilation, reduction of RV systolic function, or appearance/ progression of atrial and/or ventricular arrhythmias. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])c. Bioprosthetic tricuspid valve dysfunction with significant mixed regurgitation and stenosis. (Level of Evidence: B)
===Surgical Interventions (DO NOT EDIT)<ref name="pmid30121239">{{cite journal |vauthors=Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF |title=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 |journal=J. Am. Coll. Cardiol. |volume=73 |issue=12 |pages=e81–e192 |date=April 2019 |pmid=30121239 |doi=10.1016/j.jacc.2018.08.1029 |url=}}</ref><ref name="StoutDaniels2019">{{cite journal|last1=Stout|first1=Karen K.|last2=Daniels|first2=Curt J.|last3=Aboulhosn|first3=Jamil A.|last4=Bozkurt|first4=Biykem|last5=Broberg|first5=Craig S.|last6=Colman|first6=Jack M.|last7=Crumb|first7=Stephen R.|last8=Dearani|first8=Joseph A.|last9=Fuller|first9=Stephanie|last10=Gurvitz|first10=Michelle|last11=Khairy|first11=Paul|last12=Landzberg|first12=Michael J.|last13=Saidi|first13=Arwa|last14=Valente|first14=Anne Marie|last15=Van Hare|first15=George F.|title=2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease|journal=Journal of the American College of Cardiology|volume=73|issue=12|year=2019|pages=e81–e192|issn=07351097|doi=10.1016/j.jacc.2018.08.1029}} </ref>===


d. Predominant bioprosthetic valve stenosis (mean gradient greater than 12 to 15 mm Hg). ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])
{|class="wikitable"
 
|-
e. Operation can be considered earlier with lesser degrees of bioprosthetic stenosis with symptoms or decreased exercise tolerance. ([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]]) <nowiki>"</nowiki>
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]


|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Surgical repair or reoperation for adults with Ebstein anomaly and significant TR is recommended when one or more of the following are present :
|-
| bgcolor="LightGreen"|'''a.'''HF symptoms. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''b.'''Objective evidence of worsening exercise capacity . ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|'''c.'''Progressive RV systolic dysfunction by echocardiography or CMR . ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.'''Catheter ablation is recommended for adults with Ebstein anomaly and high-risk pathway conduction or multiple accessory pathways.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''
|}
|}


Indication for repair:
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
|bgcolor="LemonChiffon" |<nowiki></nowiki>'''1.'''Surgical repair or reoperation for adults with Ebstein anomaly and significant TR can be beneficial in the presence of:
|-
| bgcolor="LemonChiffon"|'''a.'''Progressive RV enlargement. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LemonChiffon"|'''b.'''Systemic desaturation from right-to-left atrial shunt. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LemonChiffon"|'''c.'''Paradoxical embolism. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
|-
| bgcolor="LemonChiffon"|'''c.'''Atrial tachyarrhythmias.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''


* Cyanosis (Risk of repair)
''<nowiki/>''
* Severe tricuspid regurgitaion
|}
* Bypass tract
* C:T ratio>60%
* Decrease exercise capacity


 
{|class="wikitable"
- Tricuspid valve repair or replacement (when possible repair is preferable to replacement as it is associated with a lower mortality rate and fewer complications).
|-
 
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
*[[Atrial septal defect]] repair.
|-
*Right atrial plication (unclear whther this improves right ventricular function).
| bgcolor="lemonchiffon" |<nowiki></nowiki>'''1.'''Bidirectional superior cavopulmonary (Glenn) anastomosis at time of Ebstein anomaly repair may be considered for adults when:
*Cardiac defibrillator implantation.
|-
*Accessory pathways may require surgical intervention or ablation.
| bgcolor="LemonChiffon"|'''a.'''Severe RV dilation or severe RV systolic dysfunction is present.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
 
|-
Hospital mortality at the Mayo for valve reconstruction is 6.7%. Only 1.6% in the Mayo series required reoperation. Long term follow up in the Mayo series showed 92% to have Class I or II symptoms and a 10 year mortality rate of 8%.
| bgcolor="LemonChiffon"|'''b.'''LV function is preserved. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''
 
|-
Generally, surgical treatment improves the exercise intolerance from NYHA-FC III or IV to NYHA-FC I or II.  
| bgcolor="LemonChiffon"|'''c.'''left atrial pressure and LV end diastolic pressure are not elevated. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
 
|}
Regardless of severity of the Ebstein’s Anomaly and type of treatment, risk of sudden death remains an important issue in patients with Ebstein's anomaly.
 
{{#ev:youtube|QjfJK1zY7BM}}


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


{{Congenital malformations and deformations of circulatory system}}
{{WH}}
{{WH}}
{{WS}}
{{WS}}


[[Category:Disease]]
[[Category:Cardiovascular system]]
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Surgery]]
[[Category:Medicine]]

Latest revision as of 22:33, 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

Tricuspid valve repair is indicated in patients in whom there are symptoms or deteriorating exercise capacity, cyanosis (oxygen saturation less than 90%), paradoxical embolism, progressive cardiomegaly on chest x-ray or progressive right ventricular dilation or reduction of right ventricular systolic function. When possible, repair is favored over replacement.

Indications

Generally, surgical treatment improves the exercise intolerance from NYHA-FC III or IV to NYHA-FC I or II.

Surgery

The main purpose of the surgery is to improve tricuspid valve function and there by increase the right heart output which can be accomplished by partial or complete closure of any interatrial communication, repair or replacement of the TV, and reduction of RA size.[5] [4][3][2]

Various surgical strategies employed in repair of Ebstein's anomaly
Surgeon Effect on Tricuspid Valve Valve Coaptation Effect on Atrial Size Effect on Functional RV
Hunter and Lillehei Valve is brought to anatomic annulus by transverse plication of atrialized RV Anterior and posterior leaflets function as bicuspid valve Reduced No effect
Danielson Valve is repaired or replaced at level of functional annulus (high need for TVR Anterior leaflet functions as monocusp Reduced (transverse plication of atrialized RV) No effect
Carpentier Valve is brought to the anatomic annulus (anterior/posterior leaflets detached, annular ring) Coaptation between leaflets and ventricular septum Reduced (longitudinal plication of atrialized RV) Increased
Da Silva Valve is brought to anatomic annulus Coaptation between leaflets Reduced Increased


{{#ev:youtube|QjfJK1zY7BM}}

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[6][7]

Surgical Interventions (DO NOT EDIT)[6][7]

Class I
"1. Surgeons with training and expertise in CHD should perform tricuspid valve repair or replacement with concomitant closure of an ASD, when present, for patients with Ebstein’s anomaly with the following indications:
a. Symptoms or deteriorating exercise capacity. (Level of Evidence: B)
b. Cyanosis (oxygen saturation less than 90%). (Level of Evidence: B)
c. Paradoxical embolism. (Level of Evidence: B)
d. Progressive cardiomegaly on chest x-ray. (Level of Evidence: B)
e. Progressive RV dilation or reduction of RV systolic function. (Level of Evidence: B)"
"2.Surgeons with training and expertise in CHD should perform concomitant arrhythmia surgery in patients with Ebstein’s anomaly and the following indications:
a. Appearance/progression of atrial and/or ventricular arrhythmias not amenable to percutaneous treatment. (Level of Evidence: B)
b. Ventricular preexcitation not successfully treated in the electrophysiology laboratory. (Level of Evidence: B)"
"3. Surgical rerepair or replacement of the tricuspid valve is recommended in adults with Ebstein’s anomaly with the following indications:
a. Symptoms, deteriorating exercise capacity, or New York Heart Association functional class III or IV. (Level of Evidence: B)
b. Severe TR after repair with progressive RV dilation, reduction of RV systolic function, or appearance/ progression of atrial and/or ventricular arrhythmias. (Level of Evidence: B)
c. Bioprosthetic tricuspid valve dysfunction with significant mixed regurgitation and stenosis. (Level of Evidence: B)
d. Predominant bioprosthetic valve stenosis (mean gradient greater than 12 to 15 mm Hg). (Level of Evidence: B)
e. Operation can be considered earlier with lesser degrees of bioprosthetic stenosis with symptoms or decreased exercise tolerance. (Level of Evidence: B)"

ACC/AHA 2018 Guidelines for the Management of Adults With Ebstein Anomaly(DO NOT EDIT)[8][9]

Surgical Interventions (DO NOT EDIT)[8][9]

Class I
"1. Surgical repair or reoperation for adults with Ebstein anomaly and significant TR is recommended when one or more of the following are present :
a.HF symptoms. (Level of Evidence: B)
b.Objective evidence of worsening exercise capacity . (Level of Evidence: B)
c.Progressive RV systolic dysfunction by echocardiography or CMR . (Level of Evidence: B)
"2.Catheter ablation is recommended for adults with Ebstein anomaly and high-risk pathway conduction or multiple accessory pathways.(Level of Evidence: C)
Class IIa
1.Surgical repair or reoperation for adults with Ebstein anomaly and significant TR can be beneficial in the presence of:
a.Progressive RV enlargement. (Level of Evidence: B)
b.Systemic desaturation from right-to-left atrial shunt. (Level of Evidence: B)
c.Paradoxical embolism. (Level of Evidence: B)
c.Atrial tachyarrhythmias.(Level of Evidence: B)

Class IIb
1.Bidirectional superior cavopulmonary (Glenn) anastomosis at time of Ebstein anomaly repair may be considered for adults when:
a.Severe RV dilation or severe RV systolic dysfunction is present.(Level of Evidence: B)
b.LV function is preserved. (Level of Evidence: B)
c.left atrial pressure and LV end diastolic pressure are not elevated. (Level of Evidence: B)"

References

  1. ↑ Dearani JA, Danielson GK (2005). "Surgical management of Ebstein's anomaly in the adult". Semin. Thorac. Cardiovasc. Surg. 17 (2): 148–54. doi:10.1053/j.semtcvs.2005.02.008. PMID 16087085.
  2. ↑ 2.0 2.1 Danielson GK, Maloney JD, Devloo RA (March 1979). "Surgical repair of Ebstein's anomaly". Mayo Clin. Proc. 54 (3): 185–92. PMID 431125.
  3. ↑ 3.0 3.1 da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, Soares AM, Moreira LF, Barbero-Marcial M (January 2007). "The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results". J. Thorac. Cardiovasc. Surg. 133 (1): 215–23. doi:10.1016/j.jtcvs.2006.09.018. PMID 17198815.
  4. ↑ 4.0 4.1 Dearani JA, Said SM, Burkhart HM, Pike RB, O'Leary PW, Cetta F (July 2013). "Strategies for tricuspid re-repair in Ebstein malformation using the cone technique". Ann. Thorac. Surg. 96 (1): 202–8: discussion 208–10. doi:10.1016/j.athoracsur.2013.02.067. PMID 23743062.
  5. ↑ Romfh A, Pluchinotta FR, Porayette P, Valente AM, Sanders SP (June 2012). "Congenital Heart Defects in Adults : A Field Guide for Cardiologists". J Clin Exp Cardiolog (Suppl 8). doi:10.4172/2155-9880.s8-007. PMC 3842121. PMID 24294540.
  6. ↑ 6.0 6.1 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.
  7. ↑ 7.0 7.1 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.
  8. ↑ 8.0 8.1 Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF (April 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". J. Am. Coll. Cardiol. 73 (12): e81–e192. doi:10.1016/j.jacc.2018.08.1029. PMID 30121239.
  9. ↑ 9.0 9.1 Stout, Karen K.; Daniels, Curt J.; Aboulhosn, Jamil A.; Bozkurt, Biykem; Broberg, Craig S.; Colman, Jack M.; Crumb, Stephen R.; Dearani, Joseph A.; Fuller, Stephanie; Gurvitz, Michelle; Khairy, Paul; Landzberg, Michael J.; Saidi, Arwa; Valente, Anne Marie; Van Hare, George F. (2019). "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease". Journal of the American College of Cardiology. 73 (12): e81–e192. doi:10.1016/j.jacc.2018.08.1029. ISSN 0735-1097.

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