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Year : 2014  |  Volume : 2  |  Issue : 3  |  Page : 83-85

Unusual presentation of isolated tricuspid valve disease

Department of Cardio-Vascular and Thoracic Surgery, S.M.S. Medical College, Jaipur, Rajasthan, India

Date of Web Publication5-Sep-2014

Correspondence Address:
Mohit Sharma
Department of Cardio-Vascular and Thoracic Surgery, S.M.S. Medical College, Jaipur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2321-449x.140232

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Here we present a case 20 year old boy with isolated calcific tricuspid stenosis (TS) and tricuspid regurgitation (TR) underwent TV replacement with bioprosthetic valve. After 3 months patient was presented in our emergency with sudden cyanosis, dyspnea at rest, TS and the large patent foramen ovale with large right to left shunt. Patient was re-operated, bioprothetic valve was explanted and mechanical valve was implanted. Patient developed acquired dysfibrogenemia in early post-op period with valvular dysfunction.

Keywords: Acquired dysfibrogenemia, bioprosthetic valve, tricuspid disease, tricuspid valve replacement

How to cite this article:
Sharma A, Dixit S, Sharma M, Sharma N. Unusual presentation of isolated tricuspid valve disease. Heart India 2014;2:83-5

How to cite this URL:
Sharma A, Dixit S, Sharma M, Sharma N. Unusual presentation of isolated tricuspid valve disease. Heart India [serial online] 2014 [cited 2022 Jan 21];2:83-5. Available from: https://www.heartindia.net/text.asp?2014/2/3/83/140232

  Introduction Top

Gross calcific change of the tricuspid valve (TV) is usually rheumatic and usually associated with the changes of aortic and mitral valve. Isolated rheumatic tricuspid valvular disease is a recognized but extremely rare entity. Calcification in dysplastic tricuspid valve is common but gross calcification is usually not present. [1] Though dysfunction of bioprosthetic valve after implantation at tricuspid position is known but is quite uncommon. [2] Rheumatic pathology of tricuspid valve and development of acquired dysfibrogenemia in the post operative period is unusual. Development of acquired dysfibrogenemia and the formation of pannus as early as 3 months suggest that his body has reacted strongly to valvular tissue and warrants documentation. Most noticeable part in this case was postoperative development of acquired dysfibrogenemia with valvular dysfunction just 3 months later.

  Case report Top

The case we present here is about a 20 year old boy presented in our institute with the complaint of vague chest pain in left side unrelated to physical exercise. There was no history of acute rheumatic fever in the past. He was known case of structural heart disease since 8 years of age. General physical examination revealed distended jugular veins with raised jugular venous pressure. Auscultation of the lungs was normal. The apical impulse was at the fifth intercostal space in the posterior axillary line. Heart sounds were regular; the second heart sound was split in inspiration: A grade 4/6 systolic ejection murmur and a grade 4/6 high frequency decrescendo diastolic murmur were heard at the fourth intercostal space along the left sternal border. Laboratory data and coagulation profile were normal. The electrocardiogram showed tall P waves. Chest x-ray was essentially normal. On M-mode echocardiogram, tricuspid valve (TV) was thickened and the slope of the diastolic closure was diminished. Two- dimensional echocardiography revealed the TV thick, resulting in reduced pliability and restricted opening. Mean diastolic gradient was 7 mmHg. Right ventricular systolic pressure was right atrial pressure + 20 and color flow doppler echocardiography showed moderate tricuspid regurgitation (TR). Origin of the TV change was considered dysplastic. Mitral and aortic valves were normal. Interatrial septum was intact. Left ventricular ejection fraction was 60%.

At surgery, on inspection TV was pale white, firm and calcified. TV orifice was <1 cm 2 . There were fused commissures and chordae tendinae. TV replacement (TVR) was performed with 27 mm Perimount Bioprosthetic valve. Microscopy of the TV specimen submitted through surgical pathology showed thick bands of hyalinized collagenous tissue with large foci of calcification favoring rheumatic pathology. Patient recovered well clinically but developed coagulation abnormality as early as post operative day 2. Investigations revealed high prothrombin, thrombin and reptilase time, thus diagnosis of dysfibrogenemia was made by our hematologists. He was discharged on only asprin 75 mg/day with advice for close follow-up. At 3 months following surgery, patient was readmitted in emergency with sudden development of cyanosis and dyspnea at rest. Trans-esophageal echocardiography revealed large patent foramen ovale (PFO) with significant tricuspid stenosis (TS) with large right to left shunt. He refused for immediate surgery and was managed medically and symptoms improved. He came up for surgery after 6 months and was re-operated after giving cryoprecipitate infusions 2 days before, during and 1 day after surgery. Pannus was found covering the rim of valve from right atrial side and ventricular side with some of it obstructing the valve orifice [Figure 1] and [Figure 2]. Pannus was removed and bioprosthetic valve explanted. The 29 mm St. Jude medical mechanical valve was implanted. PFO was closed directly. Biopsy of pannus revealed only hyalinized collagen tissue with some nonspecific cellular infiltrate. The patient did well after surgery and was discharged on only asprin 75 mg/day. No acitrom (acecumanolone) was given as he still continued to have high international normalized ratio.
Figure 1: Explanted valve showing overgrowth of tissue leading to obstructed valve orifi ce from ventricular side

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Figure 2: Explanted valve showing overgrowth of tissue leading to obstructed valve orifi ce from atrial side

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  Discussion Top

Organic TR is caused by myocardial infarction, [3] infective endocarditis, [4] trauma, [5] and so on. Rheumatic fever may attack TV directly and when it does, it usually involves mitral and/or aortic valves. In the present case, gross and microscopic features were suggestive of rheumatic pathology but the absence of history of acute rheumatic fever and diagnosis of valvular lesion as early as 8 years favored congenital lesion. Keefe et al. thought that all these cases of isolated TS were congenital in origin.

Other intriguing issue was the development of acquired dysfibrogenemia in immediate postoperative period, which was not present preoperatively. Much of investigations and review of literature were not helpful. One hypothesis was a reaction of his body to bioprosthetic valve leading pannus and reactionary dysfibrogenemia.

Another issue was a development of large PFO. Missing such PFO both in preoperative echo and at the time of surgery made little sense. Here also review of literature did not prove much useful. Valvular dysfunction of bioprosthetic valve at tricuspid position is a known entity with as many as 12% patients requiring redo TVR. [2] However, obstruction of isolated bioprosthetic valve and its redo surgery has not been reported yet. This case is easily first of its kind. Pannus formation is the most common cause of nonstructural valvular dysfunction. [2]

Patient is under follow-up with good recovery, but still with high prothrombin time without any anticoagulation.

  Comments Top

This case has given us insights into some of unusual presentations of isolated tricuspid valve disease and popped up some controversies. As our database about this disease grows with reporting of more such cases, we hope to resolve these issues in the near future.

  References Top

1.Fujii S, Funaki K, Denzumi N. Isolated rheumatic tricuspid regurgitation and stenosis. Clin Cardiol 1986;9:353-5.  Back to cited text no. 1
2.Nakano K, Ishibashi-Ueda H, Kobayashi J, Sasako Y, Yagihara T. Tricuspid valve replacement with bioprostheses: Long-term results and causes of valve dysfunction. Ann Thorac Surg 2001;71:105-9.  Back to cited text no. 2
3.Zone DD, Botti RE. Right ventricular infarction with tricuspid insufficiency and chronic right heart failure. Am J Cardiol 1976;37:445-8.  Back to cited text no. 3
4.Ginzton LE, Siegel RJ, Criley JM. Natural history of tricuspid valve endocarditis: A two dimensional echocardiographic study. Am J Cardiol 1982;49:1853-9.  Back to cited text no. 4
5.Bardy GH, Talano JV, Meyers S, Lesch M. Acquired cyanotic heart disease secondary to traumatic tricuspid regurgitation. Case report with a review of the literature. Am J Cardiol 1979;44:1401-6.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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