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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
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.
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
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
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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Notes