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| __NOTOC__ | | __NOTOC__ |
| {{Template:Tricuspid atresia}} | | {{Template:Tricuspid atresia}} |
| {{CMG}}; '''Associate Editor-In-Chief:''' [[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] | | {{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] |
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| | ==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. |
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| ==Surgery== | | ==Surgery== |
| | * [[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. |
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| * [[Surgery]] is the mainstay of therapy for [[tricuspid atresia]].
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| *** 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)]].
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| *** If the [[pulmonary artery]] comes from the [[left ventricle]] and is overflowed, [[pulmonary artery]] banding is useful for lowering the [[pulmonary blood flow]].
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| *** In older children, [[bi-direction Glenn shunt]] which is the connection between [[superior vena cava]] to the[[ pulmonary artery]] will be done for transferring the blood to the pulmonary system.
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| ***[[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.
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| ***[[Endocarditis]] prophylaxis before every procedure is recommended.
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| ===Surgical Interventions===
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| There are a number of interventional methods to address a tricuspid atresia. These include:
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| * [[PGE1]] to maintain [[patent ductus arteriosus]]
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| * Modified [[Blalock-Taussig]] shunt to maintain pulmonary blood flow by placing a Gortex conduit between the [[subclavian artery]] and the [[pulmonary artery]].
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| * Cavopulmonary anastomosis (hemi-Fontan or bidirectional Glenn) to provide stable pulmonary flow
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| * [[Fontan procedure]] to redirect [[inferior vena cava]] and [[hepatic vein]] flow into the pulmonary circulation
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| [[Image:TA_Fontan_procedure.jpg|left|450px]] | | [[Image:TA_Fontan_procedure.jpg|left|450px]] |
| <br clear="left"/> | | <br clear="left"/> |
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| ==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>==
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| ===Surgical Options for Patients With Single Ventricle (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> ===
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| {|class="wikitable"
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| | colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
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| | 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>
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| |}
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| ===Evaluation and Follow-Up After Fontan Procedure (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> ===
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| {|class="wikitable"
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| | colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
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| | 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>
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| |}
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| ===Surgery for Adults With Prior Fontan Repair (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> ===
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| {|class="wikitable"
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| | colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
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| | 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>
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| | 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>
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| | 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>
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| | 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>
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| | 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>
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| | 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>
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| | bgcolor="LightGreen"|<nowiki>"</nowiki>'''e.''' Fontan pathway obstruction. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
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| | 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>
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| | bgcolor="LightGreen"|<nowiki>"</nowiki>'''g.''' Pulmonary venous obstruction. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
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| | 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>
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| | 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>
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| {|class="wikitable"
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| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
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| | 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>
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| {|class="wikitable"
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| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
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| | 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>
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| ==References== | | ==References== |
