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 Table of Contents  
Year : 2019  |  Volume : 3  |  Issue : 2  |  Page : 97-98

Quadrivalvular rheumatic heart disease

Department of Cardiology, MMC, Chennai, Tamil Nadu, India

Date of Web Publication29-Aug-2019

Correspondence Address:
Deepak Ramchand Nenwani
Room No. 115, MMC Men's Hostel, Near Broadway Bus Stand, Opposite to Tamil Nadu Public Service Commission Office, Chennai - 600 003, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_45_18

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Rarely, rheumatic heart disease (RHD) involves all the four valves and causes could be rheumatic, congenital, infective, or degenerative. Here, we report a case of a 42-year-old female known case RHD for 30 years and underwent closed mitral commissurotomy 25 years back presented with progressive exertional dyspnea for the past 1 year. On clinical examination, she was in atrial fibrillation with evidence of the involvement of the mitral, aortic, and tricuspid valves. Transthoracic echocardiography showed the quadrivalvular involvement including pulmonary valves. Only few case reports are available describing rheumatic quadrivalvular damage.

Keywords: Quadrivalvular heart disease, rare case, rheumatic heart disease

How to cite this article:
Nenwani DR, Ganesan G, Venkatesan S, Babu S S. Quadrivalvular rheumatic heart disease. J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:97-8

How to cite this URL:
Nenwani DR, Ganesan G, Venkatesan S, Babu S S. Quadrivalvular rheumatic heart disease. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2021 Dec 5];3:97-8. Available from: https://www.jiaecho.org/text.asp?2019/3/2/97/265758

  Introduction Top

In developing countries, rheumatic heart disease (RHD) is the most common cause of multi-valvular involvement. Acquired pulmonary valve disease is uncommon, and it usually occurs in combination with all other valves. Rheumatic tricuspid valve disease is reported to occur in more than one-third of RHD patients studied at autopsy in the Indian subcontinent. Rheumatic involvement of all four cardiac valves is rare.[1]

  Case Report Top

A 42-year-old female presented with gradually progressive exertional dyspnea of 1-year duration. She had a history of breathlessness 30 years back and diagnosed with rheumatic mitral stenosis (MS) for which she underwent closed mitral commissurotomy. She also has a history of palpitation and abdominal distension. General examination revealed bilateral pedal edema. Her blood pressure in the right upper limb in supine posture was 100/60 mm Hg. Pulse rate was 82–100/min, irregularly irregular, variable in volume. Mean jugular venous pressure was raised with absent A wave, a prominent V wave, and Y descent. Apex beat was situated in the sixth intercostal space, 2-cm lateral to the midclavicular line, and tapping in character. A parasternal heave and palpable P2 were present. The S1 was variable and loud P2 was present. Mid-diastolic murmurs at the apex and the lower left sternal border of variable length, high-pitched blowing early diastolic murmur in the left second intercostal space, a grade 2 ejection systolic murmur in the aortic area conducted to the carotids, and a grade 3 pansystolic murmur at the lower left sternal border increasing on inspiration were present. Hepatomegaly present 3 cm below the right costal margin. Electrocardiography (ECG) showed QRS axis of 90°, atrial fibrillation with controlled ventricular rate, ventricular ectopics, and right ventricular hypertrophy. A chest radiograph in the posterior-anterior view showed cardiomegaly with straightening of the left border of the heart, double-atrial shadow with right atrial enlargement as described in [Figure 1]. Transthoracic echo evaluation revealed severe MS with mitral valve area of 0.7 cm 2 and mild mitral regurgitation as shown in [Figure 2]. The mean gradient across the mitral valves was 18 mm Hg. There were mild aortic stenosis and moderate aortic regurgitation. There were thickened and doming pulmonary valves with mild pulmonary valve stenosis and regurgitation as shown in [Figure 3]. There was thickened and doming tricuspid valve with organic tricuspid stenosis and low-pressure tricuspid regurgitation as shown in [Figure 2]. Normal biventricular function was present. Right atrial and right ventricular enlargement was severe and was referred to cardio-thoracic surgery for further management.
Figure 1: (a) Posteroanterior view of the chest X-ray showing cardiomegaly. (b) Echocardiography showing atrial fibrillation with ventricular ectopics and controlled ventricular rate

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Figure 2: (a) Mitral valve by planimetry. (b, d and e) Transthoracic echocardiogram showing the involvement of mitral and tricuspid valves. (c) Continuous-wave Doppler across the mitral valve. (f) Transthoracic echocardiogram showing involvement of mitral valve

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Figure 3: (a) Continuous-wave Doppler across the aortic valve. (b) Modified short-axis view showing the pulmonic valve. (c) Continuous-wave Doppler across pulmonary valve. (d) Modified short-axis view showing the pulmonary valve

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  Discussion Top

Acquired disease of the pulmonary valve is distinctly unusual. Any of the disease that may cause lesions of other valves rarely attacks the pulmonary valve. Significant involvement of the pulmonary valve is distinctly unusual; when it occurs, it is usually in patients with quadrivalvular involvement.[2],[3],[4],[5] Involvement of all the four cardiac valves due to a rheumatic process is rare with stenosis in all valves being still rarer. Only a few cases of RHD with stenosis of all four valves have been reported.[1] Only one case of the quadrivalvular disease occurred in 585 patients with RHD and only 1 in 400 patients undergoing cardiac catheterization at the New York Hospital.[4] Earlier, pulmonary valve involvement was diagnosed only at surgery.[3] With the advent of echo, it has become much easier. Organic tricuspid valve involvement is reported to occur in more than one-third of patients with RHD studied at necropsy in patients of the Indian subcontinent. There are a few reports of echocardiographic diagnosis of the RHD affecting all four cardiac valves.[5] Preoperative echocardiographic diagnosis of the rheumatic involvement of all four cardiac valves and successful surgical treatment has also been reported.[3] Rheumatic manifestation in the Indian subcontinent is different from Western population - being younger age of involvement, multivalvular affliction, and fulminant course.[6] This case highlights the importance of echo to diagnose all four valve involvement.

Declaration of patient consent

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


The authors would like to thank technical help for echocardiography machine from the Department of Cardiology, MMC, Chennai.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Paraskos JA. Combined valvular disease. In: Dalen JE, Alpert JS, editors. Valvular Heart Disease. 2nd ed. Boston: Little, Brown Combined Valvular Disease; 1987. p. 439-508.  Back to cited text no. 1
Gialloreto O, Aerichide N, Allard PP. Stenotic involvement of all four heart valves. Report of three cases. Am J Cardiol 1961;7:865-73.  Back to cited text no. 2
Kumar AS, Iyer KS, Chopra P. Quadrivalvular heart disease. Int J Cardiol 1985;7:66-9.  Back to cited text no. 3
Ayres SM, Arditi LI, Lambrew CT, Lukas DS. Quadrivalvular rheumatic heart disease; report of a case, with marked stenosis of all valves. Am J Med 1962;32:467-70.  Back to cited text no. 4
Krishnamoorthy KM. Images in cardiovascular medicine. Rheumatic stenosis of all four valves. Tex Heart Inst J 2002;29:224-5.  Back to cited text no. 5
Padmavati S. Rheumatic heart disease: Prevalence and preventive measures in the Indian subcontinent. Keywords: Rheumatic heart disease; rheumatic fever. Heart 2001;86:127.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3]


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