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 Guidelines - Recommendation for Surgical Options for Patients With Single Ventricle (DO NOT EDIT)===
* [[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 Guidelines - Recommendation for Evaluation and Follow-Up After Fontan Procedure (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.'''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 Guidelines - Recommendations for Surgery for Adults with Prior Fontan Repair (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>
|}


===[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]===
* 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. (Level of Evidence: C)


===[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]===
[[Image:TA_Fontan_procedure.jpg|left|450px]]
* Heart transplantation may be beneficial for severe SV dysfunction or protein-losing enteropathy (PLE). (Level of Evidence: C)}}
<br clear="left"/>


==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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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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