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Global burden of cardiovascular disease

Paediatric heart care in India


R K Kumar,1 S Shrivastava2
1
Department of Pediatric ABSTRACT These regional differences are likely to reflect on
Cardiology, Amrita Institute of There have been few systematic efforts to define the cardiovascular disease profiles in various parts of
Medical Sciences, Kochi, Kerala,
burden of paediatric heart disease in India. Estimates India. While most of India has witnessed an
India; 2 Department of Pediatric
Cardiology and Congenital Heart based on published studies on congenital heart disease increase in lifestyle-related cardiovascular disease
Diseases, Escorts Heart Institute (CHD) at birth suggest a massive CHD burden. Absolute and diabetes, the problem appears particularly
and Research Center, Okhla numbers of children with other heart diseases are also worrying in some areas.6 Rheumatic heart disease
Road, New Delhi, India often mirrors human development and striking
likely to be substantial. Given the enormity of the problem
Correspondence to: the number of paediatric heart programmes and specially regional contrasts in disease trends can therefore be
Dr Raman Krishna Kumar, trained paediatric cardiologists and paediatric cardiac expected.7
Amrita Institute of Medical surgeons are woefully inadequate. They are largely Unlike acquired conditions, the prevalence and
Sciences, Elamakkara PO, Kochi, profile of congenital heart disease (CHD) at birth is
Kerala, India; rkrishnakumar@ clustered in those parts of India that are experiencing
aims.amrita.edu improving economy and human development. For the unlikely to be significantly influenced by the above
average family the cost of care of a child with heart mentioned factors. However, the condition of
Accepted 1 April 2008 disease is prohibitive because care of children with heart healthcare delivery systems can significantly influ-
disease often requires considerable human and material ence the early survival of newborns and infants
resources, together with sophisticated technology. While with serious forms of CHD.8 This is turn can have
paediatric cardiology is only now being recognised in India an important effect on the profile of CHD
as a major specialty, there is a need to intensify efforts to encountered beyond infancy.
develop the specialty especially in those parts of India The rapid economic growth in the recent years has
where there are very few centres. The present challenges had a powerful impact on healthcare delivery in
are many and they include obtaining representative data India. Tertiary health care has largely been taken
on disease burden, establishing quality institutions with over by the private sector. This sector has grown
comprehensive paediatric cardiac programme training a impressively in terms of revenue generated and
cadre of professionals for paediatric heart care, develop- number of hospitals and healthcare facilities.9
ing cost-effective management strategies and improving Comprehensive paediatric heart care that includes
awareness on diagnosis and management of paediatric prenatal diagnosis, newborn and infant heart
heart diseases. surgery and catheter interventions can only be
provided in sophisticated tertiary hospitals. In the
absence of health insurance the costs of paediatric
The extraordinary geographical and cultural diver- heart operations and catheter interventions are
sity of India was recognised as independent India prohibitive for most Indian families and only a small
was being established.1 One of the early challenges proportion of India’s population (mostly urban) can
faced by the nation at the time of independence afford it. In this review we will focus on the current
related to human development. The infant and situation of paediatric cardiac diseases in India. The
maternal mortality rates were extremely high in growth and development of services relating to
the early 1950s for most of India.2 Since indepen- paediatric heart care in India will also be discussed.
dence, selected states in India showed impressive
improvement in various health indices, largely as a DEVELOPMENT OF PAEDIATRIC CARDIOLOGY AND
result of improving literacy, specifically female PAEDIATRIC CARDIAC SURGERY
literacy.2 3 However, the track record for most of The first cardiac centres in India were established
the Indian states in improving human develop- in the early 1960s. For the next 30 years, facilities
ment and health indices has been abysmal.4 for advanced heart care including cardiac surgery
In recent years selected areas of India are for both adults and children were essentially
witnessing unprecedented economic growth, lar- limited to very few large teaching institutions.
gely ushered in by the forces of globalisation. This These programmes trained a number of cardiolo-
has rapidly changed lifestyles, particularly in urban gists and cardiac surgeons over a 30 year period.
India.5 Globalisation has also had a ‘‘flattening Training was simultaneously for both adult and
effect’’ on India’s cultural diversity. India’s diver- paediatric heart care. All trainees, with very few
sity today stems from sharp regional differences in exceptions, had their basic background in adult
economic growth and human development, in medicine. Paediatric cardiology therefore did not
addition to previously existing differences in evolve as a subspecialty of paediatrics. Only a small
geography, language and religion. Large parts of handful of individuals seriously pursued paediatric
rural India still have among the worst human cardiology and the number of dedicated paediatric
development in the world, with extremely high cardiac surgeons in the country was limited to less
infant and maternal mortality and alarming levels than half a dozen.10 Pioneering and dedicated
of malnutrition.4 As a result, it is perhaps efforts from these individuals was responsible for
impossible to make generalisations on health the introduction of echocardiography, catheter
indices and disease prevalence in India. interventions11 and infant heart surgery in India.

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Global burden of cardiovascular disease

However, because of the paucity of institutions willing to take c Lifestyle-related conditions—childhood obesity and hyper-
care of children with heart disease, very little could be offered to tension
Indian children with heart disease until recently. c Other conditions—Kawasaki disease, cardiomyopathy and
In the 1990s a few dedicated paediatric heart programmes myocarditis.
were established in the private sector in spite of serious No population-based systematic surveys on disease preva-
questions about their economic viability. With the establish- lence have been performed for CHD at birth. Although school
ment these programmes and the formation of the Paediatric surveys are available for CHD,14–16 they cannot be used to
Cardiac Society of India in the late 1990s, paediatric cardiology generate population-based prevalence data because of attrition
and cardiac surgery are being recognised as separate specialties. in early years, low school enrolment rates in many parts of India
Dedicated training programmes in paediatric cardiology have and the possibility of children with CHD being absent from
been created in these new programmes. Trainees from these school because of illness. Similarly, published studies based on
programmes are in turn seeking to establish newer centres. The hospital statistics17 are also unlikely to be representative of the
total number of infant and newborn congenital heart operations population. An ideal study that can generate reliable data for
performed in all the existing Indian centres put together was the population requires an organised infrastructure necessary
less than 2500 in the year 2004. For a population of one billion, for optimal care of newborns and infants with CHD
it can be expected that there would perhaps be approximately (table 1).18 19 Such a situation does not exist even in the large
100 000 newborns with congenital heart disease requiring some metropolitan areas of India. Given the reasonable uniformity in
form of intervention during infancy. Thus, heart surgery CHD prevalence among various racial and ethnic groups in
appears realistic today for only 2–3% of infants requiring previous large population-based studies from various parts of
congenital heart surgery in India.12 13 This situation is likely to the world,8 it may be fair to assume that the overall prevalence
change with rapid changes in the economy and human of CHD at birth may not be substantially different in India.
development that we are now witnessing in many parts of There may, however, be differences in the relative prevalence of
India. It may be useful, therefore, to chart a roadmap for certain specific conditions and this may be worth investigating.
development of paediatric heart care for the country. It may, therefore, be appropriate to screen representative
Figure 1 shows the various components of comprehensive cohorts of live births in selected parts of India to obtain an
paediatric heart care from a national perspective. This model idea of the typical profile of CHD in various parts of the
can perhaps be applied to any specialty. This figure will form country.
the basis of this section of the review and each of the headings The epidemiology of rheumatic heart disease in India will be
will be discussed individually. discussed separately. The information on other common
acquired heart diseases is limited to small case reports and
descriptive studies. Kawasaki disease (KD) is increasingly being
Defining disease burden recognised and a number of reports have appeared in recent
The major categories of paediatric heart disease from the years after initial descriptions in 1970s.20 21 Most reports are
standpoint of relative numbers can be classified under the from urban populations and there is a strong possibility that a
following major categories: number of cases are missed.22 Awareness of manifestations of
c Rheumatic heart disease KD has not been formally evaluated and may be quite limited in
c Congenital heart disease (CHD) many parts of India. There is a need to develop a comprehensive

Figure 1 Chart showing a blueprint for


the development of paediatric heart care
from a national perspective. OB Gyn,
obstetrics and gynaecology.

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Global burden of cardiovascular disease

Table 1 Estimation of prevalence of congenital heart populations with similar body mass index (BMI) levels. Small
disease (CHD) among liveborn infants: requirements of an increases in BMI among them may translate into a substantial
ideal study increase in the CVD burden.32 For this reason the trends
reported in India are alarming even though the overall
1 An area with a clearly identified resident population
2 All deliveries from this area should be medically supervised,
proportion of obesity and overweight children is lower than
ideally in a hospital reported in other countries. The magnitude of the problem is
3 All the hospitals in the area should be included already substantial and this is likely to rapidly increase in the
5 A well defined clinical protocol for screening CHD among near future. More studies are needed with representation from
newborns various regions. There is also an urgent need to develop
6 Complete referral of all suspected newborns with CHD to a normative data for BMI and other anthropometric indices,
paediatric cardiology facility
blood pressures and lipid profiles in Indian children.
7 Remaining newborns of the birth cohort should be followed up
at specified intervals and referred whenever CHD is suspected
8 A reliable echocardiogram for confirmation of CHD Delivering paediatric heart care; developing quality institutions
9 A postmortem facility for all infants or newborns dying Perhaps the most critical component of delivering paediatric
without a clearly specified cause heart care relates to development of quality institutions that
should ideally become centres of excellence. Like many other
technology and resource intensive subspecialties, the success of
national disease registry for KD.23 Other forms of inflammatory
a paediatric cardiac programme is largely dependent on the
vasculitis, such as aortoarteritis, are well recognised in Indian
creation of a cohesive team of individuals. This team is
children. Several unique features characterise the disease in
constituted by paediatric cardiologists, paediatric cardiac sur-
Indian children.24 25 They include a strong association with
geons, anaesthesiologists, intensive care experts, nurses, tech-
tuberculosis, occurrence of heart failure and myocardial nologists (catheterisation, perfusion, ultrasound) and other
dysfunction and involvement of the thoracic and abdominal support personnel (fig 2). Additional closely related specialties
aorta. include obstetrics and neonatology. A hugely challenging facet
Few epidemiological data are currently available about of delivering paediatric cardiac care in the developing world
myocarditis and cardiomyopathy in Indian children. Clinical relates to establishment of new paediatric heart programmes.
features and diagnostic approaches for a child with suspected Because of the paucity of pre-existing training programmes,
myocarditis are largely based on descriptions from studies there is a tremendous shortage of trained staff in India.
published in Western literature and the disease has not been Additional challenges include costs of infrastructure and
well characterised in Indian children.26 27 equipment and development of support services. Perhaps
Changing lifestyles have started to have an impact in Indian because of these reasons all existing paediatric heart pro-
children. This is particularly true for urban children. Surveys grammes in India are in hospitals with busy adult cardiology
from selected cities have started to highlight the growing and cardiac surgery programmes. The infrastructure, equipment
prevalence of obesity in Indian children.28–31 Evidence from and many of the personnel are completely shared with adult
prospective studies suggests that childhood obesity is directly services. Paediatric heart programmes in India have not evolved
related to the incidence of hypertension, type 2 diabetes and in children’s hospitals, unlike many programmes in the West.
hypercholesterolaemia. According to the World Health Paediatric heart care in India is currently being delivered in
Organization, chronic diseases are the major cause of death in two settings. The first is a handful of large programmes with
almost all countries, including those in Asia. It is estimated that impressive case volumes. As of 2007 there are only half a dozen
70% of these deaths will happen in developing Asian nations programmes in India with annual case volumes of more than
like China, India, Pakistan, Cambodia and Vietnam.32 500 for paediatric heart surgery. The second setting is small
Cardiovascular diseases (CVD) are responsible for the major centres, mostly private establishments, with small case volumes
share of these chronic disease deaths. South Asians appear to and limited personnel. The scope of comprehensive paediatric
have worse CVD risk profiles when compared to white cardiac care, particularly newborn and infant heart surgery, is

Figure 2 Chart showing the human resources associated with comprehensive paediatric heart care.

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Global burden of cardiovascular disease

very limited in smaller centres. Most centres are clustered in from government funds are limited to selected patients in a few
large cities in selected regions in India. For large parts of the Indian states (such as Gujarat and Andhra Pradesh). This is not
country there are no paediatric cardiac centres. The most poorly feasible on a larger scale. From a public health perspective,
served regions include most eastern and all north-eastern states, subsidies for expensive heart surgery or interventions would
vast regions in western and central India.10 12 13 The majority of perhaps amount to significantly misplaced priorities and are not
children with CHD in these regions escape detection. For the likely to be sustainable. Overseas agencies such as the Gift of Life
small percentage of cases where the condition is diagnosed early programme of Rotary International (www.giftoflifeinternational.
enough for treatment, families are often intimidated by org) also fund paediatric heart operations in selected centres in a
financial implications and the prospect of seeking care at a limited way. However, donations and logistic support for capacity
far-off centre. However, a small but growing number of families building of selected centres in India may have a more lasting effect
do travel large distances to obtain treatment in selected Indian when compared to funding assistance for selected heart opera-
centres that deliver comprehensive paediatric heart care. The tions. The Children’s Heart Link (www.childrensheartlink.org) is
larger and relatively less expensive programmes in India are an example of an organisation that is seeking to improve quality
often overwhelmed with numbers and have significant waiting of paediatric heart care by facilitating collaborations between
lists. The low rate of detection of CHD, and economic and established centres in North America and new programmes in the
logistic considerations have perhaps ensured that only a tiny developing world.
proportion of cases from the most poorly served regions reach Another challenge relates to quality of care. The outcomes
these centres and keep the numbers to ‘‘manageable’’ levels. after paediatric cardiac surgery in infants and newborns are
A significant challenge relates to the prevailing mindset critically dependent on the skills and cohesiveness of the
regarding the financial viability of paediatric heart programmes. individual team members and there is a fairly steep learning
Most private hospitals have been reluctant to establish these curve for the institution.33 Newly established programmes
programmes because the costs of care are substantial and often therefore often need to have careful case selection policies in
cannot be recovered from patients. Open-heart operations for the beginning. Careful prioritisation of care is often necessary in
CHD typically cost $4000–$6000 (£2500–£3000) in most private the face of large numbers and limited resources. Most Indian
Indian hospitals. Third-party payments are limited to situations centres do not therefore routinely perform the Norwood
where either parent is employed by selected organisations operation for hypoplastic left heart syndrome. Lesions that
(mostly government) that provide comprehensive family health can be corrected through a single operation often receive greater
coverage. Most insurance companies exclude congenital heart priority over multistaged palliative procedures. Nosocomial
defects from the list of conditions covered. Efforts to provide infections are a significant cause of mortality in this particularly
financial support for individual patients through donations vulnerable group of patients and it is essential to establish a
robust infection control programme as new programmes are
being established.32
Table 2 Differences encountered between patient populations with When compared to established programmes in developed
congenital heart disease (CHD) from India and those from developed nations, there are noticeable differences in how paediatric heart
nations
programmes are structured in India. Most paediatric cardiolo-
Specific differences Implications gists are forced to multitask and do not focus on specific areas
Pulmonary hypertension from Need to evaluate carefully for development of with in the specialty. For example, a paediatric cardiologist
late presentation pulmonary vascular obstructive disease (PVOD) could perform echocardiography, catheter interventions proce-
before management of congenital heart defects dures and look after intensive care. Dedicated services for
associated with increased pulmonary blood flow
specific areas such as adult congenital heart disease, fetal
Neurological insults: hypoxic Routine iron supplementation for infants with
cardiology and paediatric electrophysiology have not been
insults to the developing brain, cyanotic CHD, careful preoperative neurological developed in Indian centres.
brain abscess, stroke evaluation, parental counselling on late neuro-
developmental consequences
Table 3 Cost-effective strategies for paediatric catheter-based
Malnutrition and anaemia Early correction whenever feasible, aggressive interventions
because of both delayed dietary counselling of children with CHD Problem ‘‘Solution’’
correction and high prevalence in
population Cost of dedicated Sharing of catheterisation laboratory and personnel with
catheterisation adult cardiology programmes. Performing paediatric
laboratories for paediatric catheterisation procedures using single plane equipment;
Lung infections are associated Early correction after initial control of lung infection
catheterisation avoiding diagnostic procedures if alternative methods
with shunt lesions
provide the information

Frequent occurrence of infective Need for greater emphasis on dental hygiene for
Cost of disposable Reuse of catheter disposables after ethylene oxide
endocarditis children with structural heart lesions
hardware sterilisation; using ‘‘adult’’ catheter hardware as alternatives
to selected paediatric catheter items
High prevalence of neonatal Careful septic screen is warranted before neonatal
sepsis heart surgery, judicious antibiotic use to prevent
Cost of dedicated Performing a majority of procedures under conscious
emergence of resistant strains of bacteria
anaesthesiology support sedation or ketamine anaesthesia with careful monitoring
for paediatric procedures
High prevalence of low birth Need to recognise that limited reserves in low
weight birthweight newborns with heart defects are further
Cost of imported Offering less expensive surgical alternatives to those who
limited.
occlusive devices cannot afford occlusive devices; using alternatives to
expensive occlusive devices such as multiple coils to
Pregnancy in the presence of a Need to educate and counsel women in occlude large patent arterial ducts and other arterio-venous
significant heart lesion reproductive age group; discussion of the situation communications; the less expensive duct occlude in place
with spouse of the ventricular septal occluder

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Global burden of cardiovascular disease

Role of institutions institutions with paediatric heart programmes, highlight the


Training unique problems encountered in India and suggest solutions to
Apart from delivering care, institutions serve a number of other deal with them.33–45
vital purposes. Training of staff in all the facets of paediatric
heart care is largely possible only in busy institutions. Small Cost-effective practices
programmes with low case volumes cannot develop robust Because of severe cost constraints typically encountered in most
training programmes. Until recently, training was largely Indian institutions, a number of cost-effective practices have
limited to a small number of institutions in India. There were evolved in India.46–53 These relate to management strategies for
no formal structured programmes exclusively for paediatric individual conditions, institutional policies for catheter inter-
cardiology. Paediatric cardiology had to be learned as a part of ventions and for cardiac surgery, as well as development of
training in adult cardiology. After completion of training very specific operations and catheter-based procedures46 (tables 3 and
few saw opportunities to practise paediatric cardiology; besides, 4). Some of the less expensive management strategies practised
most trainees did not have a basic background in general for patients with CHD may not be perceived as ideal and their
paediatrics. As a result in the mid-1990s the total number of long-term outcomes are likely to be less acceptable than those
dedicated paediatric cardiologists in all of India was less than a that conform to the standards of care in developed nations. A
dozen. A beginning was made recently with the establishment good example relates to patients requiring the use of conduits
of a structured training programme in paediatric cardiology by for congenital heart surgery. Conduits are very expensive in all
the national board in four of the existing large paediatric heart but one or two programmes in India that have a homograft
programmes in the country. The numbers of trainees emerging bank. More importantly, conduits need regular replacement and
from these programmes are grossly inadequate for the country’s the prospect of repeated open-heart operations is completely
needs. The situation is far worse for paediatric cardiac surgery; unrealistic for a number of patients and their families. As a
there are no dedicated training programmes yet. As a result result, many Indian programmes attempt to postpone the
there is a great shortage of paediatric cardiac surgeons. The implantation of conduits through palliative procedures such as
shortfall is likely to continue for many years and existing aorto-pulmonary shunt and the Glen shunt.
centres may find it particularly difficult to deal with growing
case numbers.
Indigenous technology
Cardiology and cardiac surgery have been largely dependent on
Developing specific strategies for Indian patients and driven by imported technology. Because of the paucity of
Because of great differences in the CHD patient populations institutions that focus on medical technology development,
that are typically encountered in India, specific management there have been very few Indian innovations in cardiology and
approaches often need to be developed for Indian patients cardiac surgery. The success of the TTK Chitra heart valve
(table 2). The differences relate to older age at presentation for underscores the feasibility and scope of innovative technological
many of the congenital heart defects, high prevalence of solutions developed in India.54 55 This valve is a tilting disc
malnutrition and associated infections. Economic considera- artificial heart valve prosthesis designed and developed by Sree
tions (below) also impact treatment practices significantly. A Chitra Tirunal Institute for Medical Sciences and Technology,
number of important publications, mostly from selected Indian (SCTIMST), an autonomous institute under the Department of
Science and Technology, Government of India. TTK Chitra
Table 4 Cost-effective strategies for paediatric cardiac surgery heart valves are being used in over 175 major cardiac
programmes in the country. More than 15 000 valves have
Problem ‘‘Solution’’
been implanted since its first implant on 6 December 1990
Limited availability of Delaying initial corrective operations for conditions requiring (www.sctimst.ac.in/bmt/technologies3). Additional innova-
conduits conduits, using the bidirectional Glen shunt—as a venous
shunt—as interim palliation for longer durations
tions in cardiovascular technology that have found applications
Greater emphasis on alternatives for corrective operations
include the bubble oxygenator and cardiotomy reservoir
that do not involve the use of conduits—for example, the (SCTIMST) and the permanent pacemaker (Shree Pacetronics;
double barrel repair for tetralogy of Fallot with coronary www.pacetronix.com/clinic) and indigenously processed por-
crossing right ventricular outflow tract-conduit, Barbero- cine xenograft conduits (www.frontierlifeline.com, www.
Marcials repair for persistent truncus arteriosus
biotech.iitm.ac.in).
Cost of imported bypass Re-sterilisation and reuse of tubings and cannulas for 3–4
disposables uses; performing the bidirectional Glen operation under right
heart bypass without the use of a oxygenator
IMPROVING AWARENESS REGARDING PAEDIATRIC HEART
DISEASES
Cost of suture materials Indigenous suture materials A number of problems relating to inadequate healthcare
delivery for children with congenital heart disease are the
Human resources for Multitasking—for example, in absence of dedicated result of poor awareness among care givers (table 5). This is
intensive care unit care paediatric cardiac intensivists, postoperative care is often particularly true for many paediatricians in India. Most
delivered by cardiac surgeons, cardiologists and postgraduate training programmes in India are in regions
anaesthesiologists
with no paediatric heart programmes. As a result the average
Complex heart defects Attempts to perform single-stage corrections albeit at
postgraduate trainee in general paediatrics receives little
requiring single/ marginally higher risk—for example, performing aortic arch exposure in paediatric cardiology.10 This translates into a
multistage corrections repair with correction of intracardiac defects such as situation where most children with heart disease escape early
ventricular septal defects closure or arterial switch options detection. There is, therefore, an urgent need to educate
practising paediatricians through current medical education
Prosthetic materials Pericardial autografts as alternatives to expensive synthetic
materials such as polytetrafluoroethylene
programmes. Professional bodies such as the Indian Academy
of Paediatrics can help facilitate this process. The newly

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Global burden of cardiovascular disease

Table 5 Shortcomings in paediatric heart care in India


Broad category Shortcomings in India Reasons

Detection of CHD Only a tiny fraction of CHD detected Limited access to health care. Small proportion of supervised live
at birth and during infancy births
Limited ability of many paediatricians to detect heart disease
because of limited exposure of paediatricians to paediatric
cardiology during their postgraduate training
Limited use of pulse oximeter for routine screening of newborns

Diagnosis Inaccurate diagnosis in a sizeable Very limited specialised paediatric echocardiography expertise
proportion

Referral Delayed referral of many infants Limited knowledge of natural history of many forms of CHD; lack of
and children awareness about newer developments in the specialty; lack of
awareness about what is available within the country

Treatment Small proportion of referred infants Few institutions with good standards of care, care too expensive for
and newborns actually receive most people
definitive treatment for CHD

Prevention Very few termination of Very limited fetal echocardiography expertise


pregnancies because of diagnosis
of CHD
Very few families adequately Very few centres with infrastructure for advanced chromosomal
counselled analysis and genetic studies

Healthcare planning No national policy for CHD Very little data on CHD epidemiology, CHD not perceived as an
treatment important paediatric health problem

formulated Paediatric Cardiac Society of India (PCSI) has exponentially in India. However, the challenge of delivering
defined education of paediatricians as one of its most quality care at a cost that is affordable to the majority of
important goals. The PCSI is working earnestly in educating affected families is likely to remain for a very long time.
paediatricians, postgraduate students in paediatrics through
Competing interests: None.
regular continuing medical education programmes, news-
letters and annual conferences. There is also a need to include
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990 Heart 2008;94:984–990. doi:10.1136/hrt.2007.139360


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Paediatric heart care in India

R K Kumar and S Shrivastava

Heart 2008 94: 984-990


doi: 10.1136/hrt.2007.139360

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