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 Table of Contents  
ORIGINAL ARTICLE
Year : 2021  |  Volume : 10  |  Issue : 2  |  Page : 48-53

A study of spectrum of rheumatic heart disease in children at a tertiary care hospital in Western India


1 Department of Cardiology, U. N. Mehta Institute of Cardiology and Research Centre, Civil Hospital Campus, Ahmedabad, Gujarat, India
2 Department of Research, U. N. Mehta Institute of Cardiology and Research Centre, Civil Hospital Campus, Ahmedabad, Gujarat, India

Date of Submission29-Jul-2020
Date of Decision07-Dec-2020
Date of Acceptance17-Feb-2021
Date of Web Publication22-Jun-2021

Correspondence Address:
Dr. Pooja Vyas
Department of Cardiology, U. N. Mehta Institute of Cardiology and Research Centre, Civil Hospital Campus, Asarwa, Ahmedabad - 380 016, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcpc.jcpc_49_20

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  Abstract 


Context: Rheumatic heart disease (RHD) predominantly affects adults. However, in developing countries like India, it is the most common acquired heart disease in children. The prevalence of RHD also varies from the region to region in the same country due to the differences in educational, economic, and social status as well as health-care facilities of the region. Aim: The aim of the study is to establish and discuss the spectrum of RHD in children from Western India and to explore the relationship between severity of valvular lesion by the age and sex of the children with RHD. Methods: Echocardiographic findings of children (N = 820) ≤18 years with RHD seen at our institute from January 2018 to December 2018 were retrospectively analyzed. RHD was screened by echocardiography. All the echocardiogram were analyzed for the pattern and severity of various valve lesions and associated abnormalities. Results: Eight hundred and twenty children had a diagnosis of RHD. Maximum patients [476 (57.1%)] were from the age group of 13–18 years without any gender difference (418 male and 402 female). Mitral regurgitation (MR) was the most common lesion found in 81.46% children. Aortic regurgitation (AR) was seen in 29.76%, mitral stenosis (MS) was found in 20.48%, tricuspid regurgitation was seen in 66.83%, and aortic stenosis (AS) was found in 0.73% children. AR was found more common in males as compared to females (P = 0.0004). Majority of patients (69.5%) had single mitral valve involvement. Four hundred and eight patients (49.8%) had severe MR, 122 patients (14.9%) had severe MS, 62 patients (7.6%) had severe aortic regurgitation, and 2 (0.2%) patients had severe AS at the time of first presentation. Pulmonary hypertension was found in 69% of children. Children with MS and AS were older than those without MS and AS (P = 0.0001). Conclusion: Majority of children had severe valvular lesion and pulmonary hypertension at the time of first presentation. RHD is the leading cause of heart failure in children with requirement of surgical/catheter intervention and long-term medical management.

Keywords: Echocardiography, pediatric cardiology, rheumatic heart disease


How to cite this article:
Vyas P, Hasit J, Dake R, Patel I, Patel K. A study of spectrum of rheumatic heart disease in children at a tertiary care hospital in Western India. J Clin Prev Cardiol 2021;10:48-53

How to cite this URL:
Vyas P, Hasit J, Dake R, Patel I, Patel K. A study of spectrum of rheumatic heart disease in children at a tertiary care hospital in Western India. J Clin Prev Cardiol [serial online] 2021 [cited 2021 Jul 28];10:48-53. Available from: https://www.jcpconline.org/text.asp?2021/10/2/48/319043




  Introduction Top


Rheumatic heart disease (RHD) remains a major health complication in developing country like India. RHD contributed the most to the burden of valvular heart disease (64.3%). RHD continues to affect millions of children and young adults in the Indian subcontinent with the prevalence rates varying from 4.54 to 6 per 1000 with estimates as high as 51 per 1000, although some recent large series of school surveys has shown a decline in the prevalence of RHD (0.5–0.68 per 1000).[1]

Thirty million people are affected by RHD globally, and in 2015, RHD was estimated to have been responsible for 305,000 deaths and 11.5 million disability-adjusted life years lost. RHD persists in all WHO regions. The African, South-East Asia, and the Western Pacific regions are the worst affected, accounting for 84% of all prevalent cases and 80% of all estimated deaths due to RHD in 2015. India, in the South-East Asia Region, has the highest global prevalence, with about 27% of all cases globally.[2]

The major factors which contribute to of rheumatic fever and RHD are poverty, undernourishment, overcrowding, poor housing, improper sanitation, and a shortage of health-care resources. Although there are strategies and treatment for the prevention and control of rheumatic valve diseases available at effective cost, they remain underutilized due to the lack of awareness in most developing countries.[3],[4],[5]

Echocardiography has been proven to be more sensitive screening tool with detection rates of RHD considerably higher than those of its auscultation-based counterpart.[6] There are only few studies on the prevalence and spectrum of RHD in pediatric population as studied by echocardiography (Echo), although other studies have shown autopsy and surgical pathology series on the patterns of valvular heart disease.[7],[8],[9],[10],[11] Regional variation in the prevalence of RHD has also been reported in e-RHEUMATIC study in India.[6] There is no such study on spectrum of RHD in pediatric population from this part of India till date. The present study was aimed to establish and discuss the spectrum of RHD as studied by echocardiography in developing countries like India where it remains major cause of mortality and morbidity.


  Methods Top


Study population

The present retrospective study, carried out at tertiary cardiac care institute in Western India included all echocardiographically proven RHD patients with age up to 18 years. Data were collected from the medical data department from January to December 2018. The study was approved by the Institutional Ethics Committee.

Methods

Data were collected from the medical data department from January to December 2018. RHD was screened by echocardiography. All the echocardiogram were analyzed for type and severity of valvular lesions as well as morphological assessment of chambers and pulmonary arterial hypertension. Cardiac auscultation was done with the patient in the supine and left lateral decubitus positions on the same table before performing echocardiography for the accuracy of diagnosis. Electrocardiographic findings and diagnosis on clinical presentation were noted in all patients. Heart failure was diagnosed clinically by evaluating the symptoms, worsening in New York Heart Association class, physical examination and by evaluation of chest X-ray. Valvular cardiomyopathy was diagnosed if left ventricular ejection fraction (LVEF) was <60% in the absence of active carditis in cases of rheumatic aortic regurgitation (AR) or Mitral regurgitation (MR); or LVEF <50% in absence of active carditis in cases of rheumatic Mitral stenosis (MS) or Aortic stenosis (AS). Diagnosis of active carditis was made combining clinical evaluation (presence of fever, tachycardia, presence of new murmur, change in character of existing heart sounds or murmurs, and pericardial friction rub), laboratory investigations (raised crp, esr >30 mm/h), electrocardiogram findings, and echocardiographic evidence of pericardial effusion, left ventricular dysfunction or new regurgitant lesions. Definite infective endocarditis was diagnosed as per modified Duke criteria.

Echocardiography

Clinical echocardiographic examination was performed by trained cardiologist. The World Heart Federation criteria (2012) for age ≤20 years for the echocardiographic diagnosis of RHD were applied to reach the diagnosis.[12]

Definite rheumatic heart disease (either A, B, C, or D)

  1. Pathological MR and at least two morphological features of RHD of the MV
  2. MS mean gradient ≥4 mmHg
  3. Pathological AR and at least two morphological features of RHD of the AV
  4. Borderline disease of both the AV and MV.


Criteria for pathological regurgitation

Pathological MR (All four Doppler echocardiographic criteria must be met)

▄ Seen in two views ▄ In at least one view, jet length ≥2 cm* ▄ Velocity ≥3 m/s for one complete envelope ▄ Pan-systolic jet in at least one envelope.

Pathological AR (All four Doppler echocardiographic criteria must be met)

▄ Seen in two views ▄ In at least one view, jet length ≥1 cm* ▄ Velocity ≥3 m/s in early diastole ▄ Pan-diastolic jet in at least one envelope.

*A regurgitant jet length should be measured from the vena contracta to the last pixel of regurgitant color (blue or red).

Morphological features of rheumatic heart disease

Features in the MV ▄ AMVL thickening ≥3 mm ▄ Chordal thickening ▄ Restricted leaflet motion ▄ Excessive leaflet tip motion during systole.

Features in the AV ▄ Irregular or focal thickening ▄ Coaptation defect ▄ Restricted leaflet motion ▄ Prolapse.

MR, AS, AR, tricuspid stenosis and tricuspid regurgitation (TR) were classified into mild, moderate and severe lesions as per 2014 American Heart Association/American College of Cardiology (AHA/ACC) guidelines.[13] MS cases were classified into mild MS (mitral valve area 1.5–2 cm2, pressure half time <150 ms), moderate MS (mitral valve area 1–1.5 cm2, pressure half time between 150 and 220 ms), and severe MS (mitral valve area ≤1 cm2, pressure half time ≥220 ms).

Pulmonary arterial systolic pressure was measured on echocardiography by evaluation of TR jet Vmax as per modified Bernoulli equation. Pulmonary artery systolic pressure more than 40 mm of Hg was considered as pulmonary arterial hypertension. Left atrial size was calculated on PLAX view with the help of M-mode.

Statistical analysis

All statistical analysis was performed in IBM SPSS software version 20.0 (Chicago, IL, USA). Quantitative variables were expressed as the mean ± standard deviation and qualitative variables were expressed as percentage (%). A comparison of continuous variable between the two groups was performed using the independent sample t test. Categorical variables were compared using the Chi-square test. A nominal significance was taken as a two tailed P < 0.05.


  Results Top


Baseline characteristics of the population

A baseline characteristic of the population is shown in [Table 1]. Out of total 820 patients, 418 patients (51%) were male and 402 patients (49%) were female. There was no gender difference found in our study. We distributed total patients in three age groups and found that most of the study subjects belonged to 13–18 years age group (468 patients, 57.1%). While only 28 patients (3.4%) were in age group 0–6 years and 324 patients (39.4%) were found in the age group of 7–12 years. We found in our study that majority (85.85%) of patients were from the rural area.
Table 1: Baseline characteristic of population

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Echocardiographic parameters

Distribution of valve involvement in male and female gender is shown in [Table 2]. AR was found more common in males as compared to females (P = 0.0004). There was no significant relation between gender and other valvular lesions. MR was the most common echocardiographic diagnosis present in 668 (81.4%) patients, followed by TR in 548 (66.8%), AR in 29.76% and MS in 168 (20.4%) patients. Regurgitation lesions were found in higher number as compared to stenotic lesions in pediatric age group.
Table 2: Distribution of valve lesion according to gender

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Involvement of valve in different age groups is shown in [Table 3]. Numbers of MS cases were higher in the age group ≥13 years as compared to younger age groups. Youngest patient with severe MS and severe AS were of 7 years and 17 years, respectively, in our study. MR cases were distributed equally in all age group. We did not find any case of AS in the age group of 0–12 years. AR was found higher in patients with age ≥13 years.
Table 3: Valve involvement in different age group

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Distribution of valvular lesions according to severity is shown in [Table 4]. On assessing the severity of individual valve lesions, 122 (14.9%) patients had severe MS, 408 (49.8%) patients had severe MR, 12 (1.4%) patients had hemodynamically significant TS, and 126 (15.4%) patients had severe TR.
Table 4: Distribution of valvular lesions according to severity (n=820)

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The echocardiographic parameters such as number of valve involvement, pulmonary artery pressure, and size of left atrium are given in [Table 5]. Majority of patients had single valve lesions with mitral valve involvement. 32.68% patients in the study had severe PAH with PA systolic pressures >60 mmHg. Average LA size was found significantly higher in patients with MS and MR.
Table 5: Echocardiographic parameters like number of valve involvement, pulmonary artery systolic pressure and size of left atrium

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Complications of rheumatic heart disease and treatment strategy offered

The complication of RHD found in our study is shown in [Table 6]. We studied the complications of RHD in this population and found that 566 (69.03%) patients had pulmonary arterial hypertension; 220 patients (27%) presented with heart failure; 36 (4.3%) patients had atrial fibrillation; 106 patients (12.9%) had arrhythmias other than atrial fibrillation like atrial premature contractions, ventricular premature contractions and atrial flutter; 6% patients had active carditis; 3.6% patients had valvular cardiomyopathies; 2.4% patients had infective endocarditis and 6 patients (0.73%) had left atrial appendage clot.
Table 6: Complications found in the study

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[Table 7] shows treatment strategy offered to these patients with RHD. These RHD patients were given either of three treatment options: Surgery, balloon mitral valvotomy, or medical management. Majority (47%) of patients had been advised for surgery, 10% of patients were given BMV as treatment option, and 42.9% patients were advised for the medical management.
Table 7: Treatment groups

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  Discussion Top


RHD is a major preventable cause for death and disability in children with age less than or equal to 18 years. Moreover, that can be done by early diagnosis and complete treatment of rheumatic fever. 15.6–19.6 million patients are suffering from RHD globally. Majority of these patients lie within Asian countries with approximately 10.8–15.9 million patients.[14]

Global burden of RHD is significantly higher for Asian children.

In our study, we did not find any gender difference for having RHD. This may probably be due to positive change in the mindset of society who is seeking and providing equal healthcare and treatment for female children in childhood. Previous studies showed that females were predominantly involved due to RHD in rural communities.[15],[16] In our study, we found the maximum numbers (54.63%) of RHD patients in age group (13–18) years. This can be explained by the natural history for the development of RHD with increasing age in the pediatric population.

Our study showed that single valve involvement is more common in RHD with frequent mitral valve involvement (69.5%) as compared to aortic valve involvement (10.73%). Combined mitral and aortic valvular lesions were found in 20.97% of the patients. Recent Asian study on RHD by Laudari S et al. showed that multivalve involvement was found in 38% of MS patients with involvement of aortic valve in 35% of patients approximately.[14]

Our study highlighted the fact that MR isolated or along with combination was the most common RHD follow by AR (29.7%). Sanyal et al.[16] also showed that the chronic MR, as the most common form of RHD in children and adults, whereas MS is more common in 4th–6th decades of life.

In our study, MS was found in 20.48% of patients. Females were more likely to develop rheumatic MS than men but association was not found to be statistically significant. Youngest patient with severe MS in our study was of 7 years. In MS, LV inflow obstruction progresses gradually due to fibrosis and partial fusion of mitral valve leaflets. The mean age of RHD patients with MS is 33 years.[15] More rapid progression here may be due to recurrent rheumatic fever episodes.

In our study, 29.7% patients had rheumatic AR. Leaflet thickening, fibrosis, and leaflet contracture result in abnormal leaflet coaptation and regurgitant orifice leading to development of AR. AR was found less common in females with P value of 0.0004. A study by Lubega et al. also showed male dominance in pediatric rheumatic AR.[14] We found only 6 cases (0.7%) of AS in study. In our study, youngest patient with severe AS was of 17 years. This can be explained by the natural history of AS.

Rheumatic AS occurs 20–40 years after acute illness as adhesions, leaflet thickening, fibrosis, commissural fusion, and calcific nodules may develop over period of time.[14] TR was found in 66.8% of cases. Organic tricuspid stenosis with TR was found in 12 patients (1.4%). A study by Chockalingam et al.[17] showed that 0.45% patients had organic tricuspid valve stenosis and 38.9% patients had functional TR. Study by Sulaiman Lubega et al. done at Uganda Heart Institute, Mulago showed that 86.7% of children had TR.[16]

Pulmonary hypertension was present in 69% patients. 32.6% patients had severe pulmonary hypertension. Many patients in our study had pulmonary hypertension. This could be due to delay in seeking treatment by parents in advanced stage of RHD with severe valvular lesion. In our study, 36 (4.39%) patients had atrial fibrillation, and 6 (0.73%) patients had LA thrombus. Twenty-seven percent of patients presented to the hospital with heart failure. Twenty (2.4%) patients had infective endocarditis, 3.65% patients had developed cardiomyopathies secondary to severe valvular lesions.

In our study, 386 (47%) patients were given surgery as the option of treatment, 253 (42.9%) patients were put on medical management, and 82 (10%) patients were offered BMV as treatment option. Asymptomatic patients with mild-to-moderate valve lesions and some cases of severe valve involvement can be followed conservatively as most of them remain stable for years. Medical management will include serial echocardiographic and clinical evaluation for symptoms, change in valve function, severity of valvular lesion, change in chamber size, and ventricular function. Dominant valvular lesion usually needs the management in multivalvular disease. Percutaneous balloon mitral valvotomy is preferred treatment option in isolated or dominant MS with suitable mitral valve morphology in selected cases. Mitral valve and aortic valve repair/replacement can done for patients with severe MR/AR with symptoms and or evidence of marked LV enlargement and or LV dysfunction. A tricuspid annuloplasty may be done for TR with annular dilation. Tricuspid commissurotomy is preferred in organic tricuspid stenosis. Tricuspid valve replacement in kept reserved for the selected cases of severe tricuspid stenosis with significant regurgitation.

Limitation of study

Our study is a hospital-based study. Moreover, the patients who presented to tertiary cardiac care institute are included in the study. These are all the clinical cases of RHD presented with symptoms. Thus, we missed those who had subclinical RHD in the community. Hence, the results may not replicate the true epidemiology and prevalence of RHD in the community; and the fact that these subclinical cases of RHD are the ones which would benefit the most from early institution of secondary prophylaxis regimen to avert progression to symptomatic disease.


  Conclusion Top


Mitral valve involvement with MR was the most common form of RHD in children. Majority of children had severe valve lesion and pulmonary hypertension at the time of presentation. Children with MS and AS were predominately above 12 years. Severe AR was found more common in males. RHD is the leading cause of morbidity and mortality among young population which requires prolonged duration of medical management, catheter, or surgical intervention. There is a huge burden of RHD cases in our country. Although many studies have shown declining the prevalence of RHD, systematic, large scale, echocardiographic screening program for RHD should be structured to evaluate possible economic and health outcomes in countries like India.

Financial support and sponsorship

This work was financially supported by U. N. Mehta Institute of Cardiology and Research Centre itself and received no specific grant from any funding agency, commercial or not for profit sectors.

Conflicts of interest

There are no conflicts of interest.



 
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Manjunath CN, Srinivas P, Ravindranath KS, Dhanalakshmi C. Incidence and patterns of valvular heart disease in a tertiary care high-volume cardiac center: a single center experience. Indian Heart J. 2014 May 1;66(3):320-6.  Back to cited text no. 1
    
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Dare AJ, Harrity PJ, Tazelaar HD, et al. Evaluation of surgically excised mitral valves: revised recommendations based on changing operative procedures in the 1990s. Hum Pathol. 1993;24:1286-93.  Back to cited text no. 10
    
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Duren DR, Becker AE, Dunning AJ. Long-term follow-up of idiopathic mitral valve prolapse in 300 patients: a prospective study. J Am Coll Cardiol. 1988;11:42-47.  Back to cited text no. 11
    
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Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014;63:2438-88.  Back to cited text no. 13
    
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Laudari S, Subramanyam G. A study of spectrum of rheumatic heart disease in a tertiary care hospital in Central Nepal. Int J Cardiol Heart Vasc. 2017 Jun 1;15:26-30.  Back to cited text no. 14
    
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    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7]



 

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