SCENARIO

A 4-year-old boy comes with a complaint often too tight and easily tired when playing. This has been
experienced since a baby. Children never look blue. Children often suffer from repeated colds and
sweating a lot. Physical analysis was found: small and thin stature. Cyanosis (-). Normal pulse and blood
pressure. Thoracic examination: visible voussure cardiac (+). Left and right ventricular activity increases.
Thrill is limited to LSB 4. BJ: 1 and 2 sound hard. A 4/6 p.m degree pansystolic noise at LSB 4 was spread
to RSB, axillary and suprasternal. The femoral artery is normally palpable. There is no clubbing.

B. DIFFICULT WORDS
1. Cyanosis
Skin color and mucous membrane are bluish and pale because of the low O2 content in the blood
2. Voussure cardiac
Wide local protrusion in the precordium area, between the sternum and cordis apex. Sometimes it
shows heart pulses.
3. Thrill
Vibration sensation felt by the examination of body palpation. As found in hard and rough heart
murmurs.
4. Pansystolic
5. clubbing
Finger deformity due to proliferation of soft tissue around the terminal phalanx of the fingers or feet
Without persistent osseous changes.

Voussure Cardiac
- Wide protrusion in the precordium area, between the sternum and apex cordis.
• Thrill is vibration
• LSB: Left Sternal Border
• 4/6 degree Pansystolic Noise
- Noisy caused by an abnormal flow of blood from the higher pressure left ventricle to the lower
chamber of the heart.

• RSB: Sternal border Right

• Taboo finger
- Karen-induced deformity of the proliferation of fat tissue around the phalanx of the terminal
fingers and toes.

C. KEY SENTENCE
1. A 4-year-old boy.
2. Complaints are often too tight and easily tired when playing.
3. Children often suffer from repeated colds and sweating a lot.
4. Small and thin stature. Cyanosis (-).
5. Normal pulse and blood pressure.
6. Voussure cardiac (+).
7. Left and right ventricular activity increases.
8. Thrill unfolds on LSB 4. BJ: 1 and 2 sound hard.
9. A 4/6 p.m degree pansystolic noise at LSB 4 spreads to RSB, axillary and suprasternal.
10. The femoral artery is normally palpable.
11. There is no clubbing.

D. QUESTIONS
1. What is the fetal circulation?
2. Explain why:
a. Coughs and colds occur repeatedly, sweating a lot, tightness and fatigue easily since a baby
b. Palpable thrill, 4/6 p.m of pansystolic noise from LSB 4 spreads to RSB, axillary and suprasternal and
positive cardiac voussure
c. S1 and S2 harden at LSB 4
3. Does the illness affect the child's growth?
4. Explain the steps of the diagnosis?
5. Differential diagnosis?
6. Classification of heart abnormalities in children!
7. Explain the pathomechanism of symptoms that arise in the scenario!
8. What is the investigation for the case?
9. How is the treatment and management?
10. What are the prognoses and complications?
11. Explain the mechanism of breathlessness in the scenario!
12. What is the relationship between symptoms in the scenario and how is the interpretation of physical
and thoracic examinations?
13. What causes children to repeat coughing and sweating a lot?
14. Why do children complain of being short and easily tired when playing?
15. Mention cyanotic and asianotik congenital heart disease!
16. What is the pathophysiology of cyanosis?
17. Explain the difference between central and peripheral cyanosis!
18. How is emergency management for patients in this scenario?

ANSWER :

ANSWER
1. Fetal circulation
The blood needed by the fetus comes from the maternal blood circulation which is connected by
the placenta. Which in the initial circulation through the umbilical vein will then enter the liver.
In the liver the blood flow will be divided into two, which is flowing to the liver sinusoids and
flowing into the ductus venosus. Then it flows to the vena cava caudalis and then empties into
the right atrium. The blood stored in the right atrium is the result of the estuary of the vena cava
caudalis and vena cava cranialis and the flow will be divided into two, namely directly into the
left atrium and some into the left ventricle. Blood that directly flows into the left atrium will
pump blood to the left ventricle. Then the collected blood will be pumped by the left ventricle
into the aorta, the blood from the aorta will flow throughout the body from the fetus which flows
to the upper limb and then the head to the lower extremities and lower visceral organs. Blood
flowing to the lower extremities and lower visceral organs will flow out of the fetal body through
the umbilical arteries and some will return to the vena cava caudalis.
As explained earlier that, in the right atrium the blood flowing is divided into two, some to the
left atrium and one to the right ventricle. Blood to the right ventricle will pump blood to the
pulmunol trunk, which in adults will enter the lungs but the fetus has not yet developed and is
filled with fluid so that the pressure on the lungs is higher than the truncal pressure of the
polmon, so that the blood flowing to the lungs is only minimal, and due to high pressure in the
lungs causing blood to flow more away from the lungs that will enter the aorta through the
ductus arteriosum. Then the blood will return to flow throughout the fetal body.

2. a. Coughs and colds occur repeatedly, sweating a lot, tightness and fatigue easily since a
baby.
Children experience tightness, coughing, runny nose, and sweating a lot due to the presence of
dams in the lungs and increased heart activity. In the event of a pathological condition in the
form of atrial bulkhead defect or ventricular bulkhead defect, a hemodynamic disorder occurs in
the circulatory system. For example, the ventricular bulkhead defect in the left ventricle which
must pump blood throughout the body also pumps blood to the right ventricle due to holes in the
interventricular septum (holes in the septum have various sizes, shapes, and numbers) so that the
right ventricle is prealoaded . According to Frank-Starling's law, on increasing preload
compensation of intrinsic factors increases the length of muscle fibers, the heart stretches, and
the strength of contraction and the contents of the stroke increase. In addition, extrinsic factors
also compensate for the sympathetic nerve and epinephrine stimulation which increases cardiac
contractility so the heart pumps more strongly to squeeze out more blood it contains. A lot of
blood volume in the right ventricle will enter the lungs, causing pulmonary hypertension and
excess blood volume in the lungs, a lot of blood in the lungs causing the pulmonary blood
vessels to dilate and break, the blood will enter the intestine, causing a dam in the lungs that
cause children to experience tightness, coughs, colds, and recurrent respiratory infections. In
addition, the blood leading to the systemic is less, there is stimulation of the sympathetic nerves
in the heart which speeds up the work of the heart pump, the metabolism gets faster so that the
child is easy to experience fatigue and sweating a lot. (6)

3. Nutrition intake (8)

Nutrition is a basic need to guarantee energy needs, metabolism, body growth and physical activity.
Infants and children who have high metabolic rates but little energy reserves so that there is an easy
lack of energy when sick, especially if sick for a long time.
Infants and children with congenital heart disease (CHD) have a significant risk of experiencing energy
imbalances because in children with CHD there is an increase in energy use and enter unbalanced
nutrition.
This energy imbalance results in malnutrition, which in turn will result in growth disorders, impaired
cognitive abilities and their motor development. Growth failure is also known to have a very important
role in the length of time after postoperative healing.
Malnutrition
According to Dr. Rubiana Sukardi SpA (K) malnutrition is a condition of babies or children with poor
nutrition so that they experience growth disorders. While the definition of a growth disorder is if the
child's weight, or the ratio of the child's weight and height, is less than 2 standard deviations below his
age.
It is estimated that four out of five children with CHD experience acute or chronic malnutrition. The
severity of malnutrition is influenced by the type and complexity of heart abnormalities, "said the RSCM
pediatric cardiology consultant. He further emphasized
the chances of blue type CHD are more often malnourished than PJB that is not blue. Boys are more
likely to experience impaired weight gain than their peers. While girls more often experience a delay in
increasing height compared to their age. Before 1980 when children with blue PJB and those who did
not show blue who had failed to grow when they were babies, they will continue until the children.
Nowadays with the advancement of technology, where heart surgery can be done at the age of a few
days after birth, there are fewer and fewer children who still fail to grow after heart surgery. "But one of
the studies found that catching height was very closely related to the severity of failure at the time of
surgery but did not depend on the age at surgery" continued the doctor who was taking a PhD program
at the University of Indonesia.

The mechanism of failure to thrive

If seen from the growth curve in children with small or light heart abnormalities it will not affect
their growth and development. But in the blue type of CHD, the risk of failure to thrive is much
higher. Why did this happen? There are three reasons according to Dr. Rubiana Sukardi SpA (K),
namely:
1. INCREDIBLE CALORIAL ORIGIN
2. FOOD DIGITAL DISORDERS (MALABSORPSI)
3. INFLUENCE OF GROWTH HORMONES
Dr. Rubiana Sukardi SpA, a child cardiology consultant, explained that there are six nutrients
that play a role in the growth of infants and children, namely carbohydrates, fats, proteins,
vitamins, minerals and water, which when combined well will provide optimal nutrition and
good growth of children. "The choice of nutrition for babies is breast milk (ASI) or if there is no
or insufficient breast milk can be given formula milk specifically for premature babies and term
infants" he said. He further explained that for calorie needs, healthy infants aged over 6 months
are recommended to give calories between 108 to 117 kilocalories per kilogram of body weight
per day. Because the protein needs at the beginning of the baby's age is high (due to the rapid
growth of muscles), it is recommended that the administration of nutrients approximately 2.2
grams per kilogram of body weight.
In infants with CHD, more nutritional support is needed to ensure a caloric intake for higher
metabolism than babies of the same age. In infants with a moderate degree of recommended
caloric intake between 130 to 150 kilocalories / kilogram of body weight / day, while in sick
infants it weighs between 170 to 180 kilocalories / kilogram of body weight / day. For micro
nutrients needed are nutrients that contain iron, magnesium, phosphorus, manganese, selenium
and zinc. This micro nutrient is usually also present in breast milk or formula or vitamins that are
sold in pharmacies.
To maximize calorie intake there are several ways that can be taken such as by increasing the
volume or can also be by increasing the density (density) of calories, giving medicines for heart
failure in the event of heart failure and the most important is to correct heart defects /
abnormalities by
surgery or transcatheter. Given the baby or child with PJB easily tired when drinking, an
alternative that can be done to include these caloric needs is to install a hose that connects the
mouth to the stomach (installation of naso-gastric tube, NGT).
At present according to Dr. Rubiana Sukardi SpA (K) more and more nutrition and child
nutrition experts can be found in hospitals. Parents can consult on choosing the type of milk or
type of food (if the child has started eating), the time of administration, counting calories, and
helping to monitor the intake and increase in weight and height.
With good nutrition, it is expected that good infants or children with PJB do not experience
failure to grow so that they have a strong immune system and if it is time to undergo surgery the
condition of the baby or child is better prepared and can pass the recovery period in the ICU and
the treatment room is faster.