Quadrivalvular Involvement in Rheumatic Heart Disease: A Rare Case Report

CM Satvic; Arnab Ghosh Chaudhury; Lachikarathman Devegowda; Prabhavathi Bhat; CN Manjunath

doi:10.4103/jiae.jiae_56_20

INTERESTING CASE REPORT

Year : 2022 | Volume : 6 | Issue : 1 | Page : 56-58

Quadrivalvular Involvement in Rheumatic Heart Disease: A Rare Case Report

CM Satvic, Arnab Ghosh Chaudhury, Lachikarathman Devegowda, Prabhavathi Bhat, CN Manjunath
Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka, India

Date of Submission	01-Sep-2020
Date of Acceptance	27-Dec-2020
Date of Web Publication	29-Apr-2022

Correspondence Address:
Dr. Arnab Ghosh Chaudhury
Sambhram Apartment II, Gokulam Main Road, V V Mohalla, Mysore - 570 002, Karnataka
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_56_20

Abstract

Four-valve involvement (mitral, aortic, tricuspid, and pulmonary) in rheumatic heart disease (RHD) is an exceedingly rare entity. We report the case of a 24-year-old male, a known case of RHD, who presented with dyspnea and presyncope for 2 months. On examination, the patient had severe mitral stenosis, moderate mitral regurgitation, severe aortic stenosis, severe aortic regurgitation, and severe tricuspid regurgitation. Echocardiography revealed severe tricuspid stenosis with moderate pulmonary stenosis in addition to other clinically detectable valve lesions. The patient underwent aortic and mitral valve replacement, tricuspid valve repair with pulmonary valvotomy successfully.

Keywords: Echocardiography, regurgitation, rheumatic heart disease, stenosis

How to cite this article:
Satvic C M, Chaudhury AG, Devegowda L, Bhat P, Manjunath C N. Quadrivalvular Involvement in Rheumatic Heart Disease: A Rare Case Report. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:56-8

How to cite this URL:
Satvic C M, Chaudhury AG, Devegowda L, Bhat P, Manjunath C N. Quadrivalvular Involvement in Rheumatic Heart Disease: A Rare Case Report. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2023 Mar 20];6:56-8. Available from: https://jiaecho.org/text.asp?2022/6/1/56/344313

Introduction

All four-valve involvement in rheumatic heart disease (RHD) is an exceedingly rare entity. Four-valve involvement is also possible in patients with congenital pulmonary stenosis who develops rheumatic valvular heart disease later involving mitral, aortic, and tricuspid valves. Very rarely valve prolapses involving all four valves can present with regurgitant lesions in mitral, aortic, tricuspid, and pulmonary valves. However, stenotic lesions will not be associated in that case. We report the case of a 24-year-old man who presented with RHD with the involvement of mitral, aortic, tricuspid, and PVs.

Case Report

A 13-year-old male presented with New York Heart Association (NYHA) class III dyspnea in 2006. He was diagnosed to have RHD, severe mitral stenosis (MS), and mild aortic stenosis (AS). He underwent percutaneous transvenous mitral commissurotomy (PTMC) elsewhere. In 2009, the patient came to our hospital with similar symptoms. Review echo revealed severe MS with moderate AS. Repeat PTMC was done successfully. In June 2017, the patient presented again with NYHA class III dyspnea and multiple episodes of presyncope of 2-month duration. On clinical examination, the patient had a low volume regular pulse with carotid shudder suggestive of severe AS. Blood pressure was 110/70 mmHg. Jugular venous pulse was elevated with prominent C-V waves suggestive of severe tricuspid regurgitation (TR). The patient did not have any dysmorphic features including marfanoid habitus. Cardiovascular examination revealed left ventricular type of apex which was forceful and well sustained suggestive of predominant AS (pressure overload). On auscultation, S1 was loud, A2 and P2 were soft. The patient had mid-diastolic murmur at the mitral area, pansystolic murmur at the tricuspid area, ejection systolic murmur at the aortic area, early diastolic murmur at the neo aortic area suggestive of severe MS, severe AS, severe aortic regurgitation (AR), and severe TR.

Electrocardiogram revealed sinus rhythm, biatrial enlargement, left ventricular hypertrophy, and left axis deviation. Chest X-ray showed cardiomegaly and pulmonary venous hypertension. There was no evidence of active rheumatic activity as evidenced by normal antistreptolysin-O titer and c-reactive protein levels. Transthoracic echocardiography revealed severe MS with mitral valve orifice area of 0.48 cm² and gradient of 20/13 mmHg [Figure 1]a and [Figure 2]a, [Figure 2]b. There was moderate mitral regurgitation (MR) with eccentric jet [Figure 1]a, [Figure 1]b and [Video 1]. Severe AS was noted (aortic valve gradient was 154/91 mmHg) with severe AR [Figure 3]a, [Figure 3]b and [Video 2]. There was severe tricuspid stenosis (TS) with tricuspid valve gradient of 26/21 mmHg [Figure 4]a, [Figure 4]b and [Video 3] with severe TR [Figure 5]a, [Figure 5]b and [Video 4]. Pulmonary valve showed thickening with turbulence across the valve [Figure 6]a and [Video 5]. The pulmonary valve gradient was 40/18 mmHg suggestive of moderate pulmonary stenosis (PS) [Figure 6]b. Grade I pulmonary regurgitation (PR) was also noted. The final diagnosis was RHD, severe MS, moderate MR, severe AS, severe AR, severe TS, severe TR, moderate PS, and mild PR. The patient was treated with replacement of mitral and aortic valves with mechanical prosthetic valves, tricuspid valve repair, and surgical pulmonary valvotomy [Figure 7]. The patient is doing well after 6 months following surgery.

Figure 1: (a and b) Parasternal long-axis view, two-dimensional and color Doppler showing dilated left atrium, eccentric hypertrophy of left ventricle, mitral stenosis, mitral regurgitation (eccentric jet)

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Figure 2: (a) Parasternal short-axis view showing mitral valve orifice area of 0.48 cm² suggestive of severe mitral stenosis. (b) Continuous-wave Doppler showing mitral valve gradient of 20/13 indicating severe mitral stenosis

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Figure 3: (a) Apical five-chamber view with color Doppler showing severe aortic regurgitation. (b) Continuous-flow Doppler showed an aortic valve gradient of 154/91 suggestive of severe aortic stenosis

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Figure 4: (a) Apical four-chamber view, color flow Doppler showing tricuspid stenosis. (b) Continuous-wave Doppler measured the tricuspid valve gradient as 26/21 mmHg indicating severe tricuspid stenosis

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Figure 5: (a) Color flow Doppler showing severe tricuspid regurgitation. (b) Tricuspid regurgitation gradient measured by continuous-wave Doppler was 66 mmHg indicating severe tricuspid regurgitation

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Figure 6: (a) Parasternal short-axis view at the pulmonary valve level. Color flow Doppler showing significant gradient across the pulmonary valve with turbulence. (b) Continuous-wave Doppler measured the pulmonary gradient as 40/18 mmHg suggestive of moderate pulmonary stenosis

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Figure 7: (a) Transthoracic echocardiography apical five-chamber view showing prosthetic mitral and aortic valves in situ. (b) Continuous-wave Doppler showing 32/17 mmHg gradient across the prosthetic aortic valve. (c) Continuous-wave Doppler showing 11/5 mmHg gradient across the tricuspid valve (post repair)

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Video 1: Transthoracic echocardiography parasternal short axis view: colour flow Doppler showing mitral stenosis, moderate mitral regurgitation with eccentric jet.

[Additional file 1]

Video 2: Transthoracic echocardiography apical five chamber view: colour flow Doppler showing severe aortic stenosis with severe aortic regurgitation

[Additional file 2]

Video 3: Transthoracic echocardiography apical four chamber view: colour flow Doppler showing severe tricuspid stenosis.

[Additional file 3]

Video 4: Transthoracic echocardiography apical four chamber view: colour flow Doppler showing severe tricuspid regurgitation.

[Additional file 4]

Video 5: Transthoracic echocardiography parasternal short axis view: colour flow Doppler showing severe pulmonary stenosis with doming of the pulmonary valve.

[Additional file 5]

Discussion

Involvement of all the four cardiac valves due to a rheumatic process is rare.^[1] Rheumatic quadrivalvar damage has been found on necropsy (one in 586 patients with valve deformities) and by cardiac catheterization.^[2] There is a high incidence of multivalvular damage when Aschoff bodies are identified at necropsy. Earlier, pulmonary valve involvement was diagnosed only at the surgery. Organic tricuspid valve involvement is reported to occur in more than one-third of the patients with RHD studied at necropsy in the Indian subcontinent.^[3] Four-valve involvement is also possible in the case of a congenital pulmonary stenosis patient who develops rheumatic mitral, aortic, and tricuspid involvement later. In our case, old echocardiography findings of the same patient in 2006 and 2009 demonstrated normal pulmonary valve, ruling out the possibility of congenital pulmonary stenosis. The pulmonary valve was thickened suggestive of rheumatic pathology contrary to typical thin doming valves seen in congenital pulmonary stenosis. There are a few reports of echocardiographic diagnosis of rheumatic cardiopathy affecting all four cardiac valves.^[4] Preoperative echocardiographic diagnosis of rheumatic involvement of all four cardiac valves and successful surgical treatment has also been reported.^[5] A study done in our institute included 13,289 patients of valvular heart disease (2010–2012).^[6] Only one patient among the cases had combined mitral, tricuspid, and pulmonary valve involvement of rheumatic origin. Four-valve involvement was not reported in that study.^[6]

Conclusion

Quadrivalvular involvement in RHD can occur though it is extremely rare. Operator awareness regarding possible quadrivalvular damage is essential for appropriate diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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3.	Ewy GA. Tricuspid valve disease. In: Chatterjee K, Chetlin MD, Karliner J, et al., editors. Cardiology: An Illustrated Text Reference. Vol. 2. Philadelphia: JB Lippincott; 1991. p. 991.
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5.	Kumar N, Rasheed K, Gallo R, Al-Halees Z, Duran CM. Rheumatic involvement of all four heart valves – Preoperative echocardiographic diagnosis and successful surgical management. Eur J Cardiothorac Surg 1995;9:713-4.
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