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Congenital tricuspid regurgitation: review and a proposed new classification

  • Anubhav Gupta (a1), Vijay Grover (a1) and Vijay K. Gupta (a1)

Abstract

Background and aim

Congenital tricuspid regurgitation is an extremely rare condition. The morphologic heterogeneity makes it difficult to derive any conclusions regarding the best strategy for management of this condition.

Methods

We analysed the published literature on “congenital tricuspid regurgitation” using MEDLINE. In addition, the reference sections of all relevant articles were searched to identify additional cases. Studies published till June 2009 are included.

Results

As far as we could establish, there were 123 cases of congenital tricuspid regurgitation. Of these, 63 were documented during surgery, 38 during autopsy, and 22 were diagnosed by echocardiography or cardiac catheterisation. The mean age was 22.57 years, with a standard deviation of 23.42 years, and the age ranged from 1 day to 80 years, with a median of 16 years. There was a male predominance, with a male-to-female ratio of approximately 3:2.

Conclusion

Congenital tricuspid regurgitation is an uncommon clinical entity with wide anatomical variations. The severity of disease dictates the presentation in infancy, childhood, or adulthood. Tricuspid valve repair is the ideal treatment whenever feasible, especially in children. We propose a new classification for congenital tricuspid regurgitation, which not only includes the anatomical variations, but can also help the surgeon in deciding on the best strategy for management.

Copyright

Corresponding author

Correspondence to: Dr A. Gupta, Assistant Professor, Department of Cardiothoracic and Vascular Surgery, Dr RML Hospital, Postgraduate Institute of Medical Education and Research, Baba Kharak Singh Marg, Connaught Place, New Delhi – 110001, India. Tel: +91 11 23404480; Fax: +91 11 23747882; E-mail: dr.anubhav.gupta@gmail.com

References

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1. Aaron, BL, Mills, M, Lower, RR. Congenital tricuspid insufficiency: definition and review. Chest 1976; 69: 637641.
2. Becker, AE, Becker, MJ, Edwards, JE. Pathologic spectrum of dysplasia of the tricuspid valve. Arch Pathol 1971; 91: 167178.
3. Waller, BF, Bloch, T, Backer, BG, Roe, SJ, Brown, JW, Mahomed, Y. Evaluation of operated excised cardiac valves: etiologic determination of valvular heart disease. Cardiol Clin 1984; 2: 687716.
4. Hauck, AJ, Freeman, DP, Ackermann, DM, Danielson, GK, Edwards, WD. Surgical pathology of the tricuspid valve: a study of 363 cases spanning 25 years. Mayo Clin Proc 1988; 63: 851863.
5. Urban, CH. Congenital tricuspid insufficiency. J Forensic Sci 1904; 9: 396401.
6. Hotz, A. Uber angeborene Trikuspidalinsuffiziem. Jahrbuch fur Kinderheilkunde 1923; 102: 112.
7. Ariel, MB. Ein seltener Fall von angeborenem Herzfehler bei einem Neugeborenen. Virchow's Path Anat 1930; 277: 501506.
8. Abbott, ME. Atlas of Congenital Cardiac Disease. American Heart Association, New York, 1936, p 24.
9. Dubin, IN, Hollinshead, WH. Congenitally insufficient tricuspid valve accompanied by an anomalous septum in the right atrium. Arch Path 1944; 38: 225228.
10. Palladino, VS, Kinney, TD. Cardiac hypertrophy and congenital tricuspid insufficiency; report of two cases. Bull Internat AM Mus 1948; 28: 2333.
11. Barritt, DW, Urich, H. Congenital tricuspid incompetence. Br Heart J 1956; 18: 133138.
12. Kincaid, OW, Swan, HJC, Ongley, PA, et al. Congenital tricuspid insufficiency; report of two cases. Proc Staff Meeting Mayo Clinic 1962; 37: 840850.
13. Reisman, M, Hipona, FA, Bloor, CM, et al. Congenital tricuspid insufficiency. J Pediatr 1965; 66: 869876.
14. Jordan, JD, Taylor, WT. Case of combined congenital tricuspid and mitral insufEciency. Am J Dis Child 1966; 111: 105108.
15. Sanyal, SK, Bhargava, SK, Saxena, H, et al. Congenital insufficiency of the tricuspid valve. Indian Heart J 1968; 20: 214218.
16. Antia, AU, Osunkoya, BO. Congenital tricuspid incompetence. Br Heart J 1969; 31: 664666.
17. Barr, PA, Celermajer, MB, Bowdler, JD, et al. Severe congenital tricuspid incompetence in the neonate. Circulation 1974; 49: 962967.
18. Kanjuh, V, Stevenson, JE, Amplatz, K, Edwards, JE. Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia. Circulation 1964; 30: 911917.
19. Green, EW, Ziegler, RF, Mieher, WC. Physiologic and anatomic considerations in congenital tricuspid valvular defects. Circulation 1962; 26: 723.
20. Tutarel, O, Westhoff-Bleck, M. The double-orifice tricuspid valve: a review. J Heart Valve Dis 2007; 16: 508510.
21. Boon, R, Hazekamp, M, Hoohenkerk, G, et al. Artificial chordae for paediatric mitral and tricuspid valve repair. Eur J Cardiothorac Surg 2007; 32: 143148.
22. Ootaki, Y, Yamaguchi, M, Yoshimura, N, Oka, S, Yoshida, M, Hasegawa, T. Tricuspid valve repair with papillary muscle shortening for severe tricuspid regurgitation in children. Ann Thorac Surg 2004; 78: 14861488.
23. Motoyoshi, N, Tofukuji, M, Sakurai, M, Ohmi, M, Koichi, T. Cleft of tricuspid anterior leaflet. Ann Thorac Surg 2001; 71: 13501351.
24. Honjo, O, Ishino, K, Yoshizumi, K, et al. Repair of dysplastic tricuspid valve using artificial chordae: case report. J Heart Valve Dis 2006; 15: 392393.
25. Shibata, Y, Sato, M, Chanda, J, Sato, S, Fujiwara, R. Isolated tricuspid regurgitation due to atypical morphology of anterior-posterior leaflets in an adult: a case report and review of literature. J Cardiovasc Surg 1999; 40: 527530.
26. Okutan, H, Yavuz, T, Bigin, S, Duver, H, Kutsal, A. Congenital cleft of anterior tricuspid leaflet in adolescent. Asian Cardiovasc Thorac Ann 2002; 10: 262263.
27. Abrams, DJ, Kilner, P, Till, JA, et al. Prolapse of the antero-superior leaflet of the tricuspid valve secondary to congenital anomalies of the valvar and sub-valvar apparatus: a rare cause of severe tricuspid regurgitation. Cardiol Young 2005; 15: 417421.
28. Katogi, T, Aeba, R, Ito, T, et al. Surgical management of isolated congenital tricuspid regurgitation. Ann Thorac Surg 1998; 66: 15711574.
29. Eichhorn, P, Ritter, M, Suetsch, G, von Segesser, LK, Turina, M, Jenni, R. Congenital cleft of the anterior tricuspid leaflet with severe tricuspid regurgitation in adults. J Am Coll Cardiol 1992; 20: 11751179.
30. Mantovani, V, Grossi, C, Ferrarese, S, Sala, A. Edge-to-edge repair of congenital familiar tricuspid regurgitation: case report. J Heart Valve Dis 2000; 9: 641643.
31. McElhinney, DB, Silverman, NH, Brook, MM, Hanley, FL, Stanger, P. Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography. Cardiol Young 1999; 9: 300304.
32. Kobza, R, Kurz, DJ, Oechslin, EN, et al. Aberrant tendinous chordae with tethering of the tricuspid leaflets: a congenital anomaly causing severe tricuspid regurgitation. Heart 2004; 90: 319323.
33. Montero, CG, Tellez, G, Simarro, E, et al. A new technique for reconstruction of the tricuspid valve ring in congenital tricuspid insufficiency. Tex Heart Inst J 1983; 10: 209213.
34. Aoyagi, S, Maruyama, H, Akasu, K, Kawara, T. Isolated tricuspid valve regurgitation resulting from severe annular dilatation: case report. J Heart Valve Dis 1999; 8: 457459.
35. Sakai, K, Inoue, Y, Osawa, M. Congenital isolated tricuspid regurgitation in an adult. Am Heart J 1985; 110: 680681.
36. Hung, MJ, Kuo, LT, Wang, CH, Cherng, WJ. Reversible left ventricular function after tricuspid valve replacement for a patient with congenital isolated severe tricuspid regurgitation. Echocardiography 2002; 19: 517520.
37. Pernot, C, Hoeffel, JC, Henry, M, Piwnica, A. Congenital tricuspid insufficiency. Cardiovasc Radiol 1978; 1: 3744.
38. Yang, X, Wu, Q, Xu, J, Shen, X, Gao, S, Liu, F. Repair of flail leaflet of tricuspid valve by simple cusp remodelling technique. J Card Surg 2007; 22: 333335.
39. Reddy, VM, McElhinney, DB, Brook, MM, Silverman, NH, Stanger, P, Hanley, FL. Repair of congenital tricuspid valve abnormalities with artificial chordae tendinae. Ann Thorac Surg 1998; 66: 172176.
40. Girard, SE, Nishimura, RA, Warnes, CA, Dearani, JA, Puga, FJ. Idiopathic annular dilation: a rare cause of isolated severe tricuspid regurgitation. J Heart Valve Dis 2000; 9: 283287.
41. Yokoyama, S, Akimoto, T, Takemura, T, Harada, Y. A surgical case report of isolated congenital tricuspid valve insufficiency. J Jpn Assn Thorac Surg 1995; 43: 10631068.
42. Boucek, RJ Jr, Graham, TP Jr, Morgan, JP, Atwood, GF, Boerth, RC. Spontaneous resolution of massive congenital tricuspid insufficiency. Circulation 1976; 54: 795800.
43. Kilic, A, Ozkutlu, S, Gunal, N. Unguarded tricuspid orifice diagnosed by echocardiography: a clinical study. Turk J Pediatr 2000; 42: 227229.
44. Ozkutlu, S, Gunal, N, Caglar, M, Alehan, D, Gungor, C. Unguarded tricuspid orifice: a rare malformation of tricuspid valve diagnosed by echocardiography. Report of two cases and review of literature. Int J Cardiol 1996; 56: 125129.
45. Lang, D, Oberhoffer, R, Cook, A, et al. Pathologic spectrum of malformations of the tricuspid valve in prenatal and neonatal life. J Am Coll Cardiol 1991; 17: 11611167.
46. Lagarde, O, Garabedian, V, Coignard, A, Duret, JC, Piwnica, A, Droniou, J. Congenital tricuspid insufficiency due to valvular dysplasia. Review of the literature in light of a case in a 40-year-old adult. Arch Mal Coeur Vaiss 1980; 73: 387396.
47. McGrath, LB, Gonzales-Lavin, L, Bailey, BM, Grunkemeier, GL, Fernandez, J, Laub, GW. Tricuspid valve operations in 530 patients: twenty-five-year assessment of early and late phase events. J Thorac Cardiovasc Surg 1990; 99: 124133.
48. Van Nooten, GJ, Caes, F, Taeymans, Y, et al. Tricuspid valve replacement: postoperative and long-term results. J Thorac Cardiovasc Surg 1995; 110: 672679.
49. Pasque, M, Williams, WG, Coles, JG, Trusler, GA, Freedom, RM. Tricuspid valve replacement in children. Ann Thorac Surg 1987; 44: 164168.
50. Fundaro, P, Di Mattia, GG, Salati, M, Santoli, C. Free edge suture plication and remodelling: a technique for anterior leaflet prolapse repair. Ann Thorac Surg 1997; 63: 11861188.
51. Castedo, E, Cañas, A, Cabo, RA, Burgos, R, Ugarte, J. Edge-to-edge tricuspid repair for redeveloped valve incompetence after De Vega's annuloplasty. Ann Thorac Surg 2003; 75: 605606.
52. Klein, H. Uber einen seltenen Fall von herzmissbildung mit rudimentarer entwicklung des rechten ventrikels und defect der tricuspidal klappen. Virchows Arch 1938; 310: 136.
53. Anderson, RH, Silverman, NH, Zuberbuhler, JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein's malformation in association with pulmonary atresia and intact ventricular septum. Pediatr Cardiol 1990; 11: 8690.
54. Cascos, AS, Rabago, P, Sokolowski, M. Duplication of the tricuspid valve. Br Heart J 1976; 29: 943.
55. Pignoni, P, Carraro, R, Nicolosi, GL. Prolapse of atrioventricular valve leaflets in the setting of double orifice. Int J Cardiol 1988; 19: 115119.
56. Lynch, TG, Doty, DB. Duplication of the tricuspid valve. Cardiovasc Dis 1980; 7: 159164.
57. Goldberg, N, Schifter, D, Aron, M, Shapiro, RS, Krasnow, N, Stein, RA. Double orifice mitral and tricuspid valves. Echocardiography 1996; 13: 8590.
58. Badano, LP, Agricola, E, Perez de Isla, L, Gianfagna, P, Zamorano, JL. Evaluation of the tricuspid valve morphology and function by transthoracic real-time three-dimensional echocardiography. Eur J Echocardiogr 2009; 10: 477484.
59. Anwar, AM, Geleijnse, ML, Soliman, OI, et al. Assessment of normal tricuspid valve anatomy in adults by realtime three-dimensional echocardiography. Int J Cardiovasc Imaging 2007; 23: 717724.

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