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Congenital tricuspid atresia and stenosis — Clinical Guidelines

Overview

Tricuspid atresia is a congenital heart defect characterized by the absence of the tricuspid valve, leading to reduced or absent blood flow from the right atrium to the right ventricle and inadequate pulmonary blood flow, necessitating surgical interventions like the Fontan procedure for survival. 1 2 19

Diagnosis

Clinical Presentation: Cyanosis, tachypnea, and signs of heart failure.

Echocardiography: Essential for confirming absence of tricuspid valve and assessing ventricular function and anatomy.

Cardiac Catheterization: Provides detailed hemodynamic assessment and guides surgical planning.

MRI: Useful for detailed anatomical evaluation, distinguishing between different subtypes of tricuspid atresia. 15 21

Management

Surgical Interventions:

- Fontan Procedure: Primary definitive repair for achieving systemic venous drainage to the pulmonary arteries. 4 14 24 - Fontan-Björk Modification: Enhances exercise performance with pulsatile systolic pulmonary flow. 2 - Total Cavopulmonary Connection (TCPC): Improves hemodynamic outcomes and ventricular function assessment via Doppler myocardial echocardiography. 3

Palliative Procedures:

- Glenn Shunt: Intermediate step before Fontan, improving oxygenation. - Blalock-Taussig Shunt: Provides additional pulmonary blood flow in early stages. 22 32

Device Therapy:

- Implantable Cardioverter Defibrillators (ICDs): Successful use in long-term management post-Fontan. 5

Special Populations

Pediatrics: Early surgical interventions like Glenn shunt and Fontan procedure are critical for survival and growth. 6 10 26

Comorbidities: Presence of pulmonary stenosis influences timing and approach of surgical interventions. 10

Key Recommendations

Perform Fontan procedure or its modifications for definitive repair in patients with tricuspid atresia to optimize long-term outcomes. (Evidence: Strong 4 24)

Consider preoperative left ventricular muscle mass as a predictor of Fontan procedure success, guiding patient selection and timing of surgery. (Evidence: Moderate 12)

Match surgical procedure to specific anatomical subtypes to improve surgical outcomes in neonates with tricuspid atresia. (Evidence: Moderate 6)

Monitor and manage wall motion abnormalities post-Fontan to address potential complications affecting ventricular function. (Evidence: Weak 9)

Evaluate and address atrial septal restrictions proactively in patients with right-sided atrioventricular valve atresia to prevent hemodynamic compromise. (Evidence: Moderate 4)

References

1 Cooke P, Goldrich D, Iloreta AM, Salama A, Shrivastava R. Intravascular Papillary Endothelial Hyperplasia of the Maxillary Sinus in Patient with Tricuspid Atresia. Head and neck pathology 2020. link 2 Ono M, Vogt M, Cleuziou J, Kasnar-Samprec J, Burri M, Strbad M et al.. Improved Exercise Performance in Patients With Tricuspid Atresia After the Fontan-Björk Modification With Pulsatile Systolic Pulmonary Flow. The Annals of thoracic surgery 2016. link 3 Eicken A, Petzuch K, Marek J, Vogel M, Hager A, Vogt M et al.. Characteristics of Doppler myocardial echocardiography in patients with tricuspid atresia after total cavopulmonary connection with preserved systolic ventricular function. International journal of cardiology 2007. link 4 Tzifa A, Gauvreau K, Geggel RL. Factors associated with development of atrial septal restriction in patients with tricuspid atresia involving the right-sided atrioventricular valve. American heart journal 2007. link 5 Lopez JA, Mihalick M. Successful use of a transvenous dual-chamber automatic implantable cardiac defibrillator after a classic Fontan operation for tricuspid atresia. Pacing and clinical electrophysiology : PACE 2006. link 6 Karamlou T, Ashburn DA, Caldarone CA, Blackstone EH, Jonas RA, Jacobs ML et al.. Matching procedure to morphology improves outcomes in neonates with tricuspid atresia. The Journal of thoracic and cardiovascular surgery 2005. link 7 Ikeda H, Aotsuka H, Nakajima H. Multiple fistulas from the coronary arteries to the left ventricle in tricuspid atresia. Cardiology in the young 2004. link 8 Rychik J, Jacobs ML, Norwood WI. Acute changes in left ventricular geometry after volume reduction operation. The Annals of thoracic surgery 1995. link00704-O) 9 Akagi T, Benson LN, Williams WG, Freedom RM. Regional ventricular wall motion abnormalities in tricuspid atresia after the Fontan procedure. Journal of the American College of Cardiology 1993. link90435-4) 10 Vogel M, Staller W, Bühlmeyer K, Sebening F. Influence of age at time of surgery on pre-operative left ventricular mass and postoperative outcome of Fontan operation in children with tricuspid atresia and native pulmonary stenosis. Herz 1992. link 11 Danilowicz D, Langsner A, Colvin SB. Successful modified Fontan procedure in an adolescent after left pneumonectomy. Pediatric cardiology 1991. link 12 Seliem M, Muster AJ, Paul MH, Benson DW. Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia. Journal of the American College of Cardiology 1989. link90121-6) 13 Grant GP, Mansell AL, Garofano RP, Hayes CJ, Bowman FO, Gersony WM. Cardiorespiratory response to exercise after the Fontan procedure for tricuspid atresia. Pediatric research 1988. link 14 Kreutzer GO, Allaria AE, Schlichter AJ, Roman MI, Capelli H, Berri GG et al.. A comparative long-term follow-up of the results of anterior and posterior approaches in bypassing the rudimentary right ventricle in patients with tricuspid atresia. International journal of cardiology 1988. link90077-0) 15 Fletcher BD, Jacobstein MD, Abramowsky CR, Anderson RH. Right atrioventricular valve atresia: anatomic evaluation with MR imaging. AJR. American journal of roentgenology 1987. link 16 Gussenhoven WJ, The HK, Schippers L, Bos E, Roelandt J, Ligtvoet C. Growth and function of the right ventricular outflow tract after Fontan's procedure for tricuspid atresia: a two-dimensional echocardiographic study. The Thoracic and cardiovascular surgeon 1986. link 17 Kurosawa H, Yagi Y, Imamura E, Koyanagi H, Satomi M, Nakazawa M et al.. A problem in Fontan's operation: sinus septal defect complicating tricuspid atresia. Heart and vessels 1985. link 18 Ottenkamp J, Wenink AC, Quaegebeur JM, Rohmer J, Gittenberger-de Groot AC, Brom AG et al.. Tricuspid atresia. Morphology of the outlet chamber with special emphasis on surgical implications. The Journal of thoracic and cardiovascular surgery 1985. link 19 Fontan F, Deville C, Quaegebeur J, Ottenkamp J, Sourdille N, Choussat A et al.. Repair of tricuspid atresia in 100 patients. The Journal of thoracic and cardiovascular surgery 1983. link 20 Rao PS. Further observations on the spontaneous closure of physiologically advantageous ventricular septal defects in tricuspid atresia: surgical implications. The Annals of thoracic surgery 1983. link61446-4) 21 Weinberg PM. Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy. The Annals of thoracic surgery 1980. link61476-2) 22 Annecchino FP, Fontan F, Chauve A, Quaegebeur J. Palliative reconstruction of the right ventricular outflow tract in tricuspid atresia: a report of 5 patients. The Annals of thoracic surgery 1980. link61478-6) 23 Ishizawa E, Horiuchi T, Sato S, Haneda K, Kahata O, Sato N et al.. Successful surgical correction in two patients with tricuspid atresia using autologous pericardial valve-bearing tube graft. The Journal of cardiovascular surgery 1980. link 24 Gale AW, Danielson GK, McGoon DC, Wallace RB, Mair DD. Fontan procedure for tricuspid atresia. Circulation 1980. link 25 Björk VO, Olin CL, Bjarke BB, Thorén CA. Right atrial-right ventricular anastomosis for correction of tricuspid atresia. The Journal of thoracic and cardiovascular surgery 1979. link 26 Sauer U, Mocellin R. Angiocardiographic left ventricular volume determination in tricuspid atresia. Comparison of patients with and without palliative surgery. Herz 1979. link 27 Sharratt GP, Johnson AM, Monro JL. Persistence and effects of sinus rhythm after Fontan procedure for tricuspid atresia. British heart journal 1979. link 28 Bowman FO, Malm JR, Hayes CJ, Gersony WM. Physiological approach to surgery for tricuspid atresia. Circulation 1978. link 29 Rao PS. Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications. British heart journal 1977. link 30 Gago O, Salles CA, Stern AM, Spooner E, Brandt RL, Morris JD. A different approach for the total correction of tricuspid atresia. The Journal of thoracic and cardiovascular surgery 1976. link 31 Kyger ER, Reul GJ, Sandiford FM, Wukasch DC, Hallman GL, Cooley DA. Surgical palliation of tricuspid atresia. Circulation 1975. link 32 Williams WG, Rubis L, Trulser GA, Mustard WT. Palliation of tricuspid atresia. Potts-Smith, Glenn, and Blalock-Taussig shunts. Archives of surgery (Chicago, Ill. : 1960) 1975. link 33 Walker DR, Sbokos CG, Lennox SC. Correction of tricuspid atresia. British heart journal 1975. link

Congenital tricuspid atresia and stenosis