Case Presentation:

Congenital Heart Disease

Supervisors:
Dr. Raditya Prakoso, SpJP(K)
dr. Anna Ulfah Rahayoe, SpJP(K)

Lia Amanda
Raisha Basir
Karin Nadia Utami

April 9th, 2016

Cardiovascular Medicine
National Cardiovascular Health Center
CASE ILLUSTRATION
 Identity of the Patient
• Name : An. DNA
• Gender : Male
• DOB : February 13th, 2016
• Address : Lampung
• Religion : Islam
• Date of admission : March 24th, 2016
• Unit : Children Ward
 Chief Complaint
• Shortness of breath, with frequent choking
since birth
 History of Current Illness
• The patient was referred from a hospital in Lampung on March 24th 2016.
• Ever since his birth, the mother noticed that the patient frequently had
shortness of breath and choking especially after being given breastmilk.
• When the patient was choking, the mother pat his back to make the
choking stops.
• 25 days after the birth of the patient, the mother brought the patient to
the nearby hospital and was told that the patient has a leakage in his
heart.
• The patient often made grok-grok sounds, especially when crying.
• After crying, the mother noticed that the area around the patient’s eyes
and mouth becomes blue.
• The patient often sweats in the forehead area.
• The patient does not have significant weight gain since birth.
• The patient frequently coughs but stops since admitted to the hospital.
 History of Past Illness
• The patient had the same complaints since birth. No other
complaints were found before being admitted to the
hospital.

History of Pregnancy
• The mother of the patient has hypontension, she consumed
a medication that increases her blood pressure during
pregnancy, given by a midwife.
• No history of trauma, smoking, alcohol use, or infection
during pregnancy were found.
 History of Delivery
• The patient was born full term, assissted by a
midwife.
• The patient cried, but it took a few minutes
for him to cry after birth
• The patient had no yellowish or bluish color
on the skin.
• The patient was born with birth weight of 2.6
kg and birth length of 48 cm.
 History of Growth and Development
• The patient only had small increase in body weight ever since his
birth
– birth weight: 2.6 kg, body weight now: 3.1 kg
• The patient was breastfed since birth but stopped ever since he
was admitted to the hospital.
• The patient has been given immunization after he was born

History of Familial Disease

• No family members of the patient have ever experienced
the same sypmtoms as the patient when they were babies.
• No family members have heart disease.
• No family members has history of diabetes mellitus,
hypertension, or allergies.
 Physical Examination
• General condition : compos mentis
• Blood pressure : 125/80
• Heart rate : 150 x/minute
• Respiratory rate : 30 x/minute
• Oxygen saturation : 88%
• Eye : no icteric sclera, no anemic conjunctiva
• Heart : normal S1 and S2, no murmur, no gallop
• Lung : vesicular, no ronchi, no wheezing
• Abdomen : supple
• Extrimities : warm acral, no edema
• Weight : 3.1 kg
• Length : 52 cm
Electrocardiography
Electrocardiography
Electrocardiography
 Electrocardiography
• Sinus tachycardia
• Rate: 150 bpm
• Right axis deviation
• QRS duration 0.08 s
• P wave normal
• PR interval 0.10 s
• Right Ventricular Hyperthrophy
• Isoelectric
• Normal T-wave
• Conclusion: sinus tachycardia with right ventricular
hypertrophy
Chest X-ray
• CTR 55%
• Upwards apex
• Small pulmonary
segment
• Normal superior
mediastinum
• No aortal dilatation
• Boot-shaped
• No hillar dilatation
• Decrease in lung
vascularisation
• Normal sinus
costrophrenicus and
diaphraghm
• Normal bone and soft
tissue
Laboratory Examination
Laboratory Examination
 Echocardiography
• Situs solitus
• AV-VA concordance
• All pressure volume to left atrium;
pressure volume strong
• Good left ventricular function, good
right ventricular function Conclusion:
• Dilatation of left atrium and left • Tetralogy of fallot
ventricle
• Mild pulmonary stenosis
• Subaortic VSD with 50% overriding
aorta, bidirectional shunt • Patent ductus arteriosus
• Deviation of infundibular septum
• Twisted and narrowing of right
ventricular outflow tract
• Confluence of pulmonary artery (+)
• Patent ductus arteriosus (+)
• Left aortic arch, coarctatio (-)
 Diagnosis
• Tetralogy of fallot
• Patent ductus arteriosus
• Congestive heart failure
LITERATURE REVIEW
 Congenital Heart Disease
• Congenital heart disease: abnormality of the heart that is present
since birth
– Cyanotic
– Acyanotic
• Cyanotic heart disease:
– blue-purple discoloration on the skin
– caused by an increase concentration of deoxygenated hemoglobin
• Acyanotic heart disease:
– resulted from left-to-right shunt at the atrium, ventricle, or great
vessel level
– causes the volume of the pulmonary artery to increase
– in long period of time, the elevated pulmonary resistance forces the
direction of the shunt to reversecyanosis
 Patent Ductus Arteriosus

• Failure of the ductus arteriosus to close

• Ductus arteriosus: a vessel that connects the descending
aorta and the pulmonary artery
– Kept open due to the high level of circulating prostaglandin
– After a baby was born: the oxygen tension rises, the
prostaglandin level drops
• causing the constriction of the ductus arteriosus, making it closed
Patent Ductus Arteriosus
• PDA causes the blood to flow from the
aorta to pulmonary artery
• In longer period of time: the pulmonary
circulation, left atrium, and left ventricle
become volume-overload  left-sided
heart failure
• Right-sided heart failure might occurs
when there is presence of pulmonary
vascular disease
• The pulmonary vascular disease 
increased pressure of the pulmonary
artery, causing the direction of the shunt
to reversed from the pulmonary artery
to the aorta
 Management of PDA

• In most cases, A PDA will spontaneously close during the first

months after birth but this rarely occurs later
• A PDA should generally be therapeutically occluded in the
absence of other congenital cardiac abnormalities or severe
pulmonary vascular disease
• Constant risk of endarteritis and the minimal complications of
corrective procedures  a small asymptomatic PDA is
commonly referred for closure
 Management of PDA
• For neonates and premature infants
– Prostaglandin synthesis inhibitors  indomethacin or ibuprofen IV to
constrict the ductus.
– Ibuprofen is 10mg/kgBW bolus followed by 5 mg/kgBW for 2-3
additional days.
However this therapy is not responsive in mature baby

• If CHF develops
– Standard anticongestive measures with digoxin and diuretics
– Indication for definitive closure

• Prophylaxis for subacute bacterial endocarditis (SBE) is also indicated

when indication arise
 Definitive closure

• Trans-catheter  small ductus less than 4 mm closed

by coils and larger by an Amplatzer PDA device
• Surgical closure  done by ligation of the ductus.
• Contraindications of the surgical closure
– the presence of pulmonary vascular obstructive
disease
 Tetralogy of Fallot
• Started from the anterior
malalignment of the
infundibular part of
ventricular septum of the
heart
• Failure in the formation of
the membranous part of
interventricular septum
– Membranous part of
interventricular septum: the
fusion of endocardial
cushions and the right and
left bulbar ridges.
 Tetralogy of Fallot

• Ventricular septal
defect
• Pulmonary stenosis
• Overriding aorta
• Right ventricular
hyperthrophy
 Treatment of ToF
1. Recognize, treat hypoxic spells, give education to the parents
– The infant should be picked up and held in a knee-chest
position to increase blood flow to the lung
– Morphine sulfate (0.2 mg/kg subcutaneously or
intramuscularly)  suppresses the respiratory center and
abolishes hyperpnoea
– Oxygen
– Sodium bicarbonate (NaHCO3)  1 mEq/kg IV, it can be
repeated in 10-15 minutes  reduces the respiratory
center–stimulating effect of acidosis
 Hypoxic spells
If the hypoxic spells do not fully respond to the treatment

1. Vasoconstrictors, such as phenylephrine  raising systemic

arterial pressure (Neo-synephrine 0.02 mg/kg IV)
2. Ketamine (1-3 mg/kg average 2mg/kg over 60 seconds 
increase systemic vascular resistance and sedates infant
3. Propanolol (0.01-0.25 mg/kg average 0.05 mg/kg) by slow IV
push  reduce heart rate
 Treatment of TOF

2. Oral propranolol therapy (0.5-1.5 mg/kgBW every 6 hours)

 prevent hypoxic spells while waiting for corrective surgery
3. Balloon dilatation of the right ventricular outflow tract and
pulmonary valve to delay repair for several months
4. Maintenance of good dental hygiene and antibiotic
prophylaxis against infective endocarditis.
5. Treat the iron deficiency  more risk to have
cerebrovascular complications
 Surgical Treatment of ToF
Palliative shunt procedures
– Creating anatomic
communications between the
aorta (or one of its major
branches) to the pulmonary
artery
– Establishing left-to-right shunt
 increase pulmonary blood
flow
– Used in infants that definitive
repair is planned at an older
age
 Surgical Treatment of ToF
Complete repair surgery
Indications:
– Oxygen saturation less than 75% to 80% and the condition of hypoxic
spell
– Symptomatic infants who have favorable anatomy of the RVOT (right
ventricular outflow tract)
– Mildly cyanotic infants who have had previous shunt surgery may have
total repair 1-2 years after the shunt operation
– Asymptomatic children with coronary artery anomalies may have the
repair after 1 year of age
 Complete repair surgery

• Closure of the VSD and enlargement of the subpulmonary

infundibulum with the use of a pericardial patch
• Elective repair is usually recommended at 6 to 12 months of
age  decrease the likelihood of future complications
• Most patients who have undergone successful repair grow to
become asymptomatic adults
DISCUSSION
 Discussion - Treatment

• Treatment for the PDA

– Patient has CHF  digoxin and diuretic
– If CHF treated  surgical ligation can be postponed until 12-16 weeks
of age because there is a chance of spontaneous closure
– If not success  definitive closure by surgical ligation of the ductus
– Antibiotic prophylaxis is also usually given to prevent endocarditis

• Treatment for the TOF

– Oral propranolol therapy until 6 months
– Complete repair surgery or palliative shunt procedures
– If the patient has spell then give therapy for spell as indicated
 Prognosis
• Ad vitam: dubia ad bonam
• Ad sanationam: dubia ad bonam
• Ad fungsionam: dubia ad bonam