Tricuspid atresia surgery: Difference between revisions

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{{CMG}}; '''Associate Editor-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] [[Priyamvada Singh|Priyamvada Singh, MBBS]] [[mailto:psingh@perfuse.org]]; '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [[mailto:kfeeney@perfuse.org]]
{{CMG}}; '''Associate Editor-In-Chief:''' {{Sara.Zand}} [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh13579@gmail.com]; '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]
 


==Overview==
==Overview==
[[Surgery]] is the mainstay of therapy for [[tricuspid atresia]]. In the first 8 weeks of life if there are severe [[ Cyanosis]] and [[pulmonary obstruction]] and normal positioning [[ aorta]] and [[ pulmonary artery]], making a [[shunt]] between systemic [[subclavian artery]] to the [[ pulmonary artery ]]is necessary which is called [[Blalock -Taussig]] [[(BT shunt)]]. If the [[pulmonary artery]] comes from the [[left ventricle]] and is overflowed, [[pulmonary artery]] banding is useful for lowering the [[pulmonary blood flow]]. In older children, [[bi-direction Glenn shunt]] which is the connection between [[superior vena cava]] to the [[ pulmonary artery]] is planned for transferring the blood to the pulmonary system. [[Fontan]] procedure is a conduit between the [[ inferior vena cava]] and the [[ pulmonary artery]] whether transfers the systemic venous blood to [[pulmonary circulation at the age of 2-3 years old.


==Surgery==
==Surgery==
===ACC/AHA 2008 Guidelines - Recommendation for Surgical Options for Patients With Single Ventricle - Tricuspid atresia (DO NOT EDIT)<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> ===
* [[Surgery]] is the mainstay of therapy for [[tricuspid atresia]].
** In the first 8 weeks of life if there are severe [[ Cyanosis]] and [[pulmonary obstruction]] and normal positioning [[ aorta]] and [[ pulmonary artery]], making a [[shunt]] between systemic [[subclavian artery]] to the [[ pulmonary artery ]]is necessary which is called [[Blalock -Taussig]] [[(BT shunt)]].<ref name="pmid26260095">{{cite journal |vauthors=Aykanat A, Yavuz T, Özalkaya E, Topçuoğlu S, Ovalı F, Karatekin G |title=Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease |journal=Pediatr Cardiol |volume=37 |issue=1 |pages=131–4 |date=January 2016 |pmid=26260095 |doi=10.1007/s00246-015-1251-0 |url=}}</ref>
** If the [[pulmonary artery]] comes from the [[left ventricle]] and is overflowed, [[pulmonary artery]] banding is useful for lowering the [[pulmonary blood flow]].<ref name="pmid30811802">{{cite journal |vauthors=Boucek DM, Qureshi AM, Goldstein BH, Petit CJ, Glatz AC |title=Blalock-Taussig shunt versus patent ductus arteriosus stent as first palliation for ductal-dependent pulmonary circulation lesions: A review of the literature |journal=Congenit Heart Dis |volume=14 |issue=1 |pages=105–109 |date=January 2019 |pmid=30811802 |doi=10.1111/chd.12707 |url=}}</ref>
** In older children, [[bi-direction Glenn shunt]] which is the connection between [[superior vena cava]] to the [[ pulmonary artery]] is planned for transferring the blood to the pulmonary system.
**[[Fontan]] procedure is a conduit between the [[ inferior vena cava]] and the [[ pulmonary artery]] whether transfers the systemic venous blood to [[pulmonary circulation at the age of 2-3 years old.<ref name="pmid8238751">{{cite journal |vauthors=Norwood WI, Jacobs ML |title=Fontan's procedure in two stages |journal=Am. J. Surg. |volume=166 |issue=5 |pages=548–51 |date=November 1993 |pmid=8238751 |doi=10.1016/s0002-9610(05)81151-1 |url=}}</ref>
**[[Endocarditis]] prophylaxis before every procedure is recommended.
 
 
 


{|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.'''Surgeons with training and expertise in congenital heart disease (CHD) should perform operations for single-ventricle anatomy or physiology.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}


===Surgical Interventions===
There are a number of interventional methods to address a tricuspid atresia. These include:
* [[PGE1]] to maintain [[patent ductus arteriosus]]
* Modified [[Blalock-Taussig]] shunt to maintain pulmonary blood flow by placing a Gortex conduit between the [[subclavian artery]] and the [[pulmonary artery]].
* Cavopulmonary anastomosis (hemi-Fontan or bidirectional Glenn) to provide stable pulmonary flow
* [[Fontan procedure]] to redirect [[inferior vena cava]] and [[hepatic vein]] flow into the pulmonary circulation


[[Image:TA_Fontan_procedure.jpg|left|450px]]
<br clear="left"/>


===ACC/AHA 2008 Guidelines - Recommendation for Evaluation and Follow-Up After Fontan Procedure - Tricuspid atresia (DO NOT EDIT)<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> ===


{|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.'''Lifelong follow-up is recommended for patients after a Fontan type of operation; this should include a yearly evaluation by a cardiologist with expertise in the care of adult congenital heart disease (ACHD) patients.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}


===ACC/AHA 2008 Guidelines - Recommendations for Surgery for Adults with Prior Fontan Repair - Tricuspid atresia (DO NOT EDIT)===


{|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.'''Surgeons with training and expertise in CHD should perform operations on patients with prior Fontan repair for single-ventricle physiology.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.'''Reoperation after Fontan is indicated for the following:''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''a.'''Unintended residual atrial septal defect (ASD) that results in right-to-left shunt with symptoms and/or cyanosis not amenable to transcatheter closure.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''b.'''Hemodynamically significant residual systemic artery-to-pulmonary artery shunt, residual surgical shunt, or residual ventricle-to-pulmonary artery connection not amenable to transcatheter closure.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''c.'''Moderate to severe systemic atrioventricular (AV) valve regurgitation.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''d.'''Significant (greater than 30-mm Hg peak-to-peak) subaortic obstruction.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''e.'''Fontan pathway obstruction.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''f.'''Development of venous collateral channels or pulmonary arteriovenous malformation not amenable to transcatheter management.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''g.'''Pulmonary venous obstruction.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''h.'''Rhythm abnormalities, such as complete AV block or sick sinus syndrome, that require epicardial pacemaker insertion.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''i.'''Creation or closure of a fenestration not amenable to transcatheter intervention.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}


{|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.'''Reoperation for Fontan conversion (i.e., revision of an atriopulmonary connection to an intracardiac lateral tunnel, intra-atrial conduit, or extracardiac conduit) can be useful for recurrent atrial fibrillation or flutter without hemodynamically significant anatomic abnormalities. A concomitant Maze procedure should also be performed.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}


{|class="wikitable"
[[Image:TA_Fontan_procedure.jpg|left|450px]]
|-
<br clear="left"/>
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
|-
| bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.'''Heart transplantation may be beneficial for severe SV dysfunction or protein-losing enteropathy (PLE).''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}


==References==
==References==
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[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Disease]]
[[Category:Needs overview]]
[[Category:Cardiovascular diseases]]
[[Category:Up-To-Date cardiology]]
[[Category:Up-To-Date]]
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{{WS}}
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Latest revision as of 19:42, 8 November 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Sara Zand, M.D.[2] Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]


Overview

Surgery is the mainstay of therapy for tricuspid atresia. In the first 8 weeks of life if there are severe Cyanosis and pulmonary obstruction and normal positioning aorta and pulmonary artery, making a shunt between systemic subclavian artery to the pulmonary artery is necessary which is called Blalock -Taussig (BT shunt). If the pulmonary artery comes from the left ventricle and is overflowed, pulmonary artery banding is useful for lowering the pulmonary blood flow. In older children, bi-direction Glenn shunt which is the connection between superior vena cava to the pulmonary artery is planned for transferring the blood to the pulmonary system. Fontan procedure is a conduit between the inferior vena cava and the pulmonary artery whether transfers the systemic venous blood to [[pulmonary circulation at the age of 2-3 years old.

Surgery








References

  1. ↑ Aykanat A, Yavuz T, Özalkaya E, Topçuoğlu S, Ovalı F, Karatekin G (January 2016). "Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease". Pediatr Cardiol. 37 (1): 131–4. doi:10.1007/s00246-015-1251-0. PMID 26260095.
  2. ↑ Boucek DM, Qureshi AM, Goldstein BH, Petit CJ, Glatz AC (January 2019). "Blalock-Taussig shunt versus patent ductus arteriosus stent as first palliation for ductal-dependent pulmonary circulation lesions: A review of the literature". Congenit Heart Dis. 14 (1): 105–109. doi:10.1111/chd.12707. PMID 30811802.
  3. ↑ Norwood WI, Jacobs ML (November 1993). "Fontan's procedure in two stages". Am. J. Surg. 166 (5): 548–51. doi:10.1016/s0002-9610(05)81151-1. PMID 8238751.

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