Congenital Heart Disease :

The challenge of optimal

surgical treatment in NTB

Maz Isa Ansyori

Sejarah Bedah Jantung
• Leoanardo da vinci (abad 15 ) : berhasil
menggambr anatomi jantung secara terperinci
• Ludwig rehn (1896) : berhasil menjahit ventrikel
kanan pada luka tusuk jantung
• Frey dan Grubner (1885) : menciptakan konsep
mesin jantung Paru pertama dan disempurnakan
dan digunakan secara klinis oleh John Gibbon
(1953)
• Christian Barnard (1969) : Transplantasi Jantung
pertama
Penduduk Provinsi NTB menurut Jenis
Kelamin dan Kepadatan Penduduk
PersentasePenduduk Miskin di
Provinsi NTB Tahun 2013-2017
Kasus Kematian Bayi di Provinsi
NTB Tahun 2011-2017
Peta Persebaran Anggota HBTKVI
Aceh 2

Medan 7

Samarinda2 Manado1
Pekanbaru3
Riau 2

Padang 2
Palembang 3
Banjarmasin1

Jkt 53
Sby 15
Bdg 6
Makasar 2
Banten 10
Jogja 3
Solo 2
Malang2 Bali 4
Smrng 4
Lombok 1

10
Congenital heart disease (CHD)
Congenital heart disease (CHD) refers to the
presence of a structural abnormality of the heart and
/ or great vessels that is present at birth and is of
actual or potential functional significance.
(Hoffman & Kaplan)
Congenital heart disease (CHD)
• Because of the paucity of treatment facilities in
developing regions and the known fact that
without treatment 60% of congenital heart
diseases are lethal within the first two years of life
• In addition to the deaths that may be directly
attributable to heart disease, many children with
congenital heart disease also die from some of the
infectious diseases
Burden of congenital heart disease
incidence steadily
increased from about 4
to 5 per 1000 live births
in the 1950’s to as
much as 50 to 75 per
1000 live births more
recently
(Hoffman & Kaplan).
Estimated number of congenital
heart disease live births in selected
developing
countries
Burden of congenital heart disease
• The number of children born with congenital heart
disease in developing region have generally been
extrapolated (based on the assumption of similar
worldwide incidence)
• Indeed, there generally is a paucity of data on the
birth prevalence of all congenital malformations
from developing countries, so that the impacts of
these defects have been systematically
underestimated in the past (Howson et al., 2004).
strategies
In recent decades, the management of congenital
malformations of the heart has improved remarkably
in the developed world such that even very
complicated lesions are now amenable to treatment.
strategies
These strategies
few lucky children who benefit from the timely
detection of CHD, but there may still be many more
who die due to undetected CHD or inappropriate
treatment or care.
strategies
• Transporting a few affected children center for
treatment.
• to short-term visits experts from advanced center
to perform surgical procedures on a few children.
• establishing regional centres of excellence
treatment.
(Stolf , Pezzella 2010).
factors that have contributed
both to the higher proportion of
birth defects
• Higher proportion of young / older mothers,
• exposure to environmental teratogens
• poverty, which is very often associated with
maternal malnutrition and more frequent
infections.
Typically Congenital heart disease
patients in developing regions

• Late diagnosis
• Late presentation
• First presentation to hospital may be because of
complications
• late treatment
• loss follow-up
Typically Congenital heart disease
patients in developing regions

• Due to late presentation + lack of treatment +

infections and nutritional deficiencies →patients
with congenital heart disease with complications
Complication
• Failure to thrive and Respiratory tract infection
• severe hypoxia, frequent and severe hypercyanotic
attacks, cerebrovascular accidents, and infective
endocarditis
• heart failure and Eisenmenger syndrome
Challenges of congenital heart
disease in developing region
• The prevention of congenital heart disease, like
that of any other disease condition hinges on a
basic understanding of its causes
• There is widespread ignorance and misconception
about the aetiology of congenital heart disease and
other birth defects.
Challenges of congenital heart disease
prevention in developing region
three broad groups now widely recognized:
• pre-conception period and due primarily to genetic
(chromosomal and single gene defects) and partly
genetic causes (multifactorial inheritance involving
interaction of genes and the environment);
• after conception before birth (these are usually
due to teratogens)
pre-conception period
• Down syndrome or trisomy 21, the most common
chromosomal disorder is associated with congenital
heart disease in about 50% of cases.
• Trisomies 18 and 13 are much less common and are
each associated with congenital heart disease in
over 90% of cases
after conception period
• Teratogen-induced heart defects, though more
readily preventable →higher frequency of
intrauterine infection (rubella and CMV)
• 10- 15 % of birth defects in develop countries are of
post-conception.
Challenges of congenital heart disease
prevention in developing countries
Other factors that contribute to the higher burden of
congenital heart disease in developing countries
include:
• poverty, which predisposes women to malnutrition
before and during pregnancy, and to a greater risk
of exposure to environmental teratogens,
• in some developing countries and inadequate
access to health care which hinders the control of
some of the risk factors for congenital heart disease
Reducing congenital heart disease
in developing Region
• The need for community based studies to generate
accurate statistics on incidence, mortality and
survival data → for planning intervention
programmes and to justify more resource
allocation.
Reducing the toll of congenital heart
disease in developing countries
• Improved prenatal and peri-natal services including
promotion of family planning, rubella immunization
before pregnancy and folic acid fortification of
commonly consumed foods.
• Educating the public on steps to take to promote a
healthy pregnancy outcome including avoidance of
teratogens during gestation.
optimal surgical treatment
let’s focus on sensitive life-saving treatments and
practical management of the child with congenital
heart lesion

Any infant < 1 month of age with cyanosis or shock

should be considered to have duct-dependent critical
congenital cardiac disease until proven otherwise.
• left heart lesion/ductal dependent lesion
• Shunting or mixing lesions
optimal surgical treatment
The big 4 Causes of Neonatal Cyanosis
• Congenital Heart Disease
• Sepsis
• Respiratory disorders (i.e., pneumonia, ARDS)
• Hemaglobinopathy (i.e., polycythemia,
methemoglobinemia)
optimal surgical treatment
• To distinguish central cyanosis
from peripheral cyanosis, look for bluish
discoloration inside the mouth – tongue, mucous
membranes and lips.
optimal surgical treatment
Blue: think right obstructive duct-dependent in the
first month of life or mixing lesion (inadequate
pulmonary blood flow: usually right-sided
obstructive ductal-dependent lesion or a mixing
lesion) after one month of life
optimal surgical treatment
Physical Examination Tests in Congenital Heart
Disease → Hyperoxia Test

• patient receives 100% oxygen (or as close to a

100% FIO2 as possible) for 5-10 minutes
• If the oxygen saturation improves the underlying
cause favours a respiratory etiology, whereas if the
oxgygen saturation does not improve, a cardiac
cause is favoured.
optimal surgical treatment
CXR Clues for Congenital Heart Disease
optimal surgical treatment
Early empiric antibiotics should be started as soon as
possible. Prostaglandin therapy should be
implemented for blue infant less than a month of
age to keep the PDA open.

Ventilation Considerations →Positive pressure

ventilation can increase PVR and decrease SVR and
preload → affect shunting flow
optimal surgical treatment
Tetralogy of Fallot is a
rare condition caused
by a combination of
four heart defects that
are present at birth
optimal surgical treatment
In transposition of the
great arteries, the aorta
is connected to the
right ventricle, and the
pulmonary artery is
connected to the left
ventricle — the
opposite of a normal
heart's anatomy.
optimal surgical treatment
abnormal pulmonary
venous return (TAPVR),
the pulmonary veins
connect to other veins
and ultimately drain
their
Conclusion
• There is a need to increase public awareness about
congenital heart disease so as to encourage early
presentation and enhance early diagnosis.
Terima Kasih