Tricuspid atresia medical therapy: Difference between revisions

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(/* ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT){{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...)
(/* Medical Therapy (DO NOT EDIT){{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=...)
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== Medical Therapy ==
== Medical Therapy ==
==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>==
==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>==
===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> ===


Treatment
Treatment
Medical Therapy
Medical Therapy
The mainstay of therapy for the cyanotic neonate with severe PS and small VSD is using prostaglandin E1 (PGE1) for keeping patency of ductus arteriosis.
*The mainstay of therapy for the cyanotic neonate with severe PS and small VSD is using prostaglandin E1 (PGE1) for keeping patency of ductus arteriosis.
The mainstay of therapy for heart failure symptoms is using diuretic for reloading and then starting ACEI.
*The mainstay of therapy for heart failure symptoms is using diuretic for reloading and then starting ACEI.
Surgery
Surgery
Surgery is the mainstay of therapy for tricuspid atresia.
Surgery is the mainstay of therapy for tricuspid atresia.

Revision as of 16:02, 24 August 2020

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

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Medical Therapy

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1][2]

Treatment Medical Therapy

  • The mainstay of therapy for the cyanotic neonate with severe PS and small VSD is using prostaglandin E1 (PGE1) for keeping patency of ductus arteriosis.
  • The mainstay of therapy for heart failure symptoms is using diuretic for reloading and then starting ACEI.

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 pulmonary artery is necessary which is called Blalock -Taussig(BT shunt). · If the pulmonary artery comes from the left ventricle and is overflowed, PA 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 will be done for transferring the blood to the pulmonary system. Fontan procedure is a conduit between the inferior vena cava and the pulmonary artery and transfers the systemic venous blood to pulmonary circulation in the age of 2-3 years old. Endocarditis prophylaxis before every procedure is recommended.



Class I
"1. Warfarin should be given for patients who have a documented atrial shunt, atrial thrombus, atrial arrhythmias, or a thromboembolic event. (Level of Evidence: C)"
Class IIa
"1. It is reasonable to treat systemic ventricular dysfunction with angiotensin-converting enzyme (ACE) inhibitors and diuretics. (Level of Evidence: C)"

Management Strategies for the Patient With Prior Fontan Repair (DO NOT EDIT)[1][2]

Class I
"1. Management of patients with prior Fontan repair should be coordinated with a regional ACHD center. Local cardiologists, internists, and family care physicians should develop ongoing relationships with such a center with continuous availability of specialists. (Level of Evidence: C)"
"2. At least yearly follow-up is recommended for patients after Fontan repair. (Level of Evidence: C)"
"3. Arrhythmia management is frequently an issue, and consultation with an electrophysiologist is recommended as a vital part of care. (Level of Evidence: C)"
"4. New-onset atrial tachyarrhythmia should prompt a comprehensive noninvasive imaging evaluation to identify associated atrial/baffle thrombus, anatomic abnormalities of the Fontan pathway, or ventricular dysfunction. (Level of Evidence: C)"

References

  1. 1.0 1.1 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.
  2. 2.0 2.1 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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