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Year : 2020  |  Volume : 8  |  Issue : 2  |  Page : 116-118

Rheumatic valve disease in a 2-year-old child: A rare case report

Department of Cardiology, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi, India

Date of Submission19-Feb-2020
Date of Decision05-Mar-2020
Date of Acceptance27-Apr-2020
Date of Web Publication4-Aug-2020

Correspondence Address:
Dr. Rajeev Bharadwaj
Department of Cardiology, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi - 110 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/heartindia.heartindia_7_20

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Rheumatic fever (RF) and rheumatic heart disease (RHD) are major health hazards in developing countries. Affection with RF-RHD in preschool years is uncommon, but prognostically worse as management is technically difficult. We report a rare early presentation of rheumatic valve disease in a 2-year-old child. This is probably the youngest case of rheumatic mitral obstruction documented in literature till date. It highlights that RF-RHD is still a very prevalent disease in India yet is underdiagnosed and underreported.

Keywords: Juvenile, mitral stenosis, pediatric, rheumatic

How to cite this article:
Nath RK, Bharadwaj R. Rheumatic valve disease in a 2-year-old child: A rare case report. Heart India 2020;8:116-8

How to cite this URL:
Nath RK, Bharadwaj R. Rheumatic valve disease in a 2-year-old child: A rare case report. Heart India [serial online] 2020 [cited 2020 Oct 31];8:116-8. Available from: https://www.heartindia.net/text.asp?2020/8/2/116/291363

  Introduction Top

Acquired mitral stenosis (MS) is mostly considered a “rheumatic fever (RF)-rheumatic heart disease (RHD)” sequelae and juvenile MS term was coined by Dr. Roy Sujoy,[1] which indicates early involvement of valve tissues by this disease. Pediatric and juvenile MS are defined as significant MS up to the age of 12 and 20 years, respectively,[2] RF is still endemic in Southeast Asian, Sub-saharan, and North Australian subcontinents. Critical MS may present in juvenile age in these areas, while the latency period after RF episode extends from 20 to 50 years in developed countries. Up to 50% of RHD patients present without any past history suggestive of RF.[2]

There are only scanty case reports of RHD cases in preschool (<5 year) age group and the youngest such case report was of a 2.5-year-old child.[3],[4],[5],[6],[7],[8] Here, we report an unusual case of rheumatic MS in a 1 year and 11-month-old child which is the earliest presentation of RHD-MS ever documented to the best of our knowledge.

  Case Report Top

A 1-year and 11-month-old female child presented to our hospital with a history of fever, bilateral joint pain, cough, and restlessness for the past 3 weeks after not responding to treatment given by a local pediatrician. The mother also gave a history of respiratory distress and tachypnea. She had a past history of similar fever which lasted for 4–5 weeks at the age of 1.5 years, for which she was treated by the local pediatrician but was not evaluated for possible RF. She was born out of a full-term normal vaginal delivery, had no siblings, and was adequately immunized till date. The family belonged to lower socioeconomic class.

On examination, the child was thin built, had mild pallor, pulse rate was 130/min/regular, and no radiofemoral delay; respiratory rate was 28/min; blood pressure was 90/54 mmHg in RUL; and cardiovascular system examination revealed a tapping apex, loud P2, an apical soft pansystolic murmur, apical mid-diastolic murmur, and a soft pansystolic murmur at the right lower sternal border. Peripheral large joints were tender but showed no other signs of inflammation. The examination of other systems revealed no significant signs and there were no cutaneous manifestations of RF.

Blood investigation revealed hemoglobin of 8.6 g%, very high erythrocyte sedimentation rate (80 mm/h), high C-reactive protein (30), and a very high antistreptolysin O (ASO) titer of 1200 IU/ml, with normal total and differential counts. The patient was admitted under pediatrician's care and was initially treated for suspected connective tissue disorder.

Electrocardiogram revealed sinus tachycardia, right atrial (RA) enlargement, and QRS axis +120°, with normal PR, QRS, and QT interval [Figure 1]. Chest radiograph showed cardiomegaly with right ventricular (RV) type of apex, RA enlargement, splaying of carinal angle indicating left atrial enlargement, characteristic straightening of left heart border, and prominent upper lobe vascular markings indicating pulmonary venous hypertension [Figure 2].
Figure 1: 12-lead electrocardiogram showing sinus rhythm with right atrial enlargement

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Figure 2: Posteroanterior view of chest X-ray showing cardiomegaly, bi-atrial enlargement, and pulmonary venous hypertension

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In view of heart murmurs and radiographic findings, transthoracic two-dimensional echocardiography was done, which revealed an enlarged left atrium, thickened anterior and posterior mitral leaflets with restricted mobility with no calcification, and severely thickened subvalvular apparatus up to the papillary muscles. Moderate mitral regurgitation was present [Figure 3]a. The mean gradient across mitral valve was 20 mmHg [Figure 3]d. Maximal leaflet separation was 4 mm [Figure 3]e. There was moderate eccentric tricuspid regurgitation with RV systolic pressure of 55 mmHg [Figure 3]c. Mild regurgitation across the aortic valve was also noted [Figure 3]b. Pulmonary valve and both ventricular functions were normal.
Figure 3: Transthoracic echocardiographic and color Doppler showing (a) thickened mitral leaflets and mitral regurgitation, (b) enlarged left atrium and mild aortic regurgitation, (c) moderate tricuspid regurgitation and moderate pulmonary artery hypertension, (d) transmitral diastolic gradient, and (e) restricted opening of mitral leaflets

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A diagnosis of RHD with probable RF recurrence was made due to characteristic multivalvular involvement and rise of ASO titer along with a history of fever and polyarthralgia. The child was initiated on oral suspensions with weight-based dose of frusemide, spironolactone, digoxin, penicillin, and hematinics. She responded well to treatment and showed symptomatic improvement and weight gain over 6-month follow-up. The parents were counseled regarding the importance of continued RF prophylaxis, close follow-up, and need of surgical intervention in the near future, as surgeons refused to operate in such an early age and as she was responding to the therapy.

  Discussion Top

RHD is most significant sequelae of RF. It is well known that RHD patients in developing countries have a much shorter latency period after RF episode. The diagnosis of RF in young age is often missed, missing the only opportunity for “primary” prophylaxis. Many patients do not have classical symptoms and suffer from subclinical carditis, indolently damaging the valves.[2]

RHD prevalence in school-aged children varies from 0.6 to 4.5/1000 in India.[2] However, the proportion of RHD cases in <5 year age (pre-school) group is much lower. Our case is probably the youngest documented case of rheumatic valve disease till date. At a time when the occurrence of RF is considered uncommon under 2 years of age, this early presentation of RF and RHD is not only surprising but also a revelation that there is more to learn about RHD pathogenesis and RF-RHD is still a rampant disease in India.

The management of pediatric RHD raises relevant concerns that includes prophylaxis, medication, intervention (timing, mode, and technical difficulties), and probable need of re-intervention (recurrence and increased physiological demand with age).

In conclusion, RF-RHD largely being a disease of the poor and unprivileged, is grossly underdiagnosed, underreported, and undertreated. It is to note that failure to detect and treat RF at young age leads to a huge burden of RHD subsequently, leading to increased morbidity, mortality, and loss of productive years.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

Ethical Approval

Ethical clearance is not required as proper consent was taken from the patient and no personal details of the patient were revealed.

  References Top

Roy Sujoy B, Lazaro EJ, Ramalingaswami V. Juvenile MS in India. Lancet 1963;2:1193-6.  Back to cited text no. 1
Kumar RK, Tandon R. Rheumatic fever and rheumatic heart disease: The last 50 years. Indian J Med Res 2013;137:643-58.  Back to cited text no. 2
[PUBMED]  [Full text]  
Tandon R, Potti S, Mathur VS, Roy SB. Critical rheumatic mitral stenosis in children. Indian Pediatr 1972;9:171-3.  Back to cited text no. 3
Chadha SK, Thareja RN, Durairaj M, Alurkar VM, Palnitkar SD, Rajan RS, et al. Mitral valve disease in young; review of surgical treatment. Indian J Thorac Cardiovasc Surg 1983;2:29-35.  Back to cited text no. 4
Kapoor A, Moorthy N, Kumar S. Inoue balloon mitral valvotomy in a 4-year-old boy: To treat fulminant rheumatic mitral stenosis. Tex Heart Inst J 2012;39:108-11.  Back to cited text no. 5
Vijaylaksmi IB. Acute rheumatic fever: Current scenario in India. Med Update 2012;22:199-212.  Back to cited text no. 6
Sarkar A, Patil S, Ahmed I. Balloon mitral valvotomy in youngest documented rheumatic mitral stenosis patient. Catheter Cardiovasc Interv 2015;86:E213-6.  Back to cited text no. 7
Modi R, Patted SV, Halkati PC, Porwal S. Youngest reported case of juvenile mitral stenosis. Indian Heart J 2015;67:S1-2.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]


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