CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 4, May 2013
AFRICA
141
Review Article
The global burden of congenital heart disease
JULIEN IE HOFFMAN
Abstract
Although the incidence of congenital heart disease (CHD) is
similar worldwide, the burden of supporting these patients
falls more heavily on countries with high fertility rates. In a
country with a fertility rate of about eight per woman, the
population has to support four times as many children with
CHD as in a country with a fertility rate of two. Countries
with the highest fertility rates tend to have the lowest incomes
per capita, thus accentuating the disparity. Countries with
high fertility rates have more children with congenital heart
disease per wage earner. Improving local health services and
controlling infectious diseases (diarrhoeal illness, rheumatic
fever, measles, rotoviral infection) are important but are
mere ‘band-aids’ compared to improving education, empow-
ering women and reducing birth rates.
Keywords:
fertility rate, per capita income, population age
structure, cost disparities
Submitted 11/3/13, accepted 17/4/13
Cardiovasc
J Afr
2013;
24
: 141–145
DOI: 10.5830/CVJA-2013-028
Patients
Congenital heart disease, a major cause of serious morbidity
and mortality, is common. It is usually defined as clinically
significant structural heart disease present at birth;
1
anomalies
such as minor changes in the vena caval drainage that have
no clinical importance are not included. Some caveats apply.
For example, subaortic stenosis almost always develops well
after birth but is included because it resembles other congenital
forms of left ventricular outflow tract obstruction. Furthermore,
some structural abnormalities that are genetically determined
but usually present after infancy are not usually included in the
totals; examples include the valve lesions of Marfan’s syndrome
or obstruction due to hypertrophic cardiomyopathy. These genetic
forms of heart disease are uncommon (Table 1) and including or
excluding them from the totals makes little difference to the total
incidence of congenital heart disease.
The incidence of congenital heart disease at birth (sometimes
referred to as birth prevalence) depends on how a population is
studied.
2,3
Before the introduction of echocardiography, incidence
figures ranged from five to eight per 1 000 live births but better
diagnosis has detected many more with milder forms, so that
current estimates range from eight to 12 per 1 000 live births.
Much depends on how early and how intensively the diagnosis
is made.
Performing echocardiograms on all neonates has shown that
about 5% have small muscular ventricular septal defects, most of
which close spontaneously before a year of age. Even more have
small atrial septal defects that also close spontaneously. Many
neonates have delayed closure of a patent ductus arteriosus. It
therefore makes more sense to consider only lesions that need
treatment in infancy or are present at a year of age. I think that
the best current figure to use is 10–12 per 1 000 live births. Even
though this includes some with small and mild defects, these
patients still have significant cardiac murmurs, often come to
medical attention, and cause much distress to families.
In addition to these, another 10–12 children per 1 000 live
births have bicuspid aortic valves that are not stenotic. These
seldom cause problems in childhood but account for a large
Department of Paediatrics, Cardiovascular Research Institute,
University of California, San Francisco, California, USA
JULIEN IE HOFFMAN, BSc Hons (Wits), MD (Wits), FRCP
(London),
Fig. 1. Annual births in selected countries. Data from
United Nations Children’s Fund accessed at
.
un.org/Data.aspx?d
=
SOWC&f
=
inID%3A75. Map from
Wikipedia accessed at ttp://en.wikipedia.org/wiki/World_
population. The panel at the side indicates the population
ranges of the different countries, with China and India
each having over one billion inhabitants.
TABLE 1. BIRTH INCIDENCE OF DIFFERENT FORMS OF
CONGENITAL HEART DISEASE
Disease
Incidence/million
live births
Structural CHD
10 000–12 000
Bicuspid aortic valve
10 000–12 000
Genetic disorders
776
Dilated cardiomyopathy
6
Hypertrophic cardiomyopathy
50
Neuromuscular dystrophies
300
Connective tissue disorders: large countries, Marfan,
mucopolysaccharidoses, etc
420
