CASE PRESENTATION

CONGENITAL HEART DISEASE

Mentor :
dr. Ulynar Marpaung, Sp. A
Created by: Charifa Sama

Department of Pediatric
Raden Said Sukanto Police Center Hospital
Faculty of Medicine UIN Syarif Hidayatuallah Jakarta
Rotation Period 19th March – 25th May 2017
 Patient identity
Name : Child K
Birth Date : June 7th 2017
Age : 10 months
Gender : Female
Address : Walang, North Jakarta
Nationality : Indonesia
Religion : Islam
Date of admission : April 6th 2018
Date of examination : April 6th 2018
 Parent identity

Father Mother

Name Mr. B Mrs. N

Age 40 years old 38 years old

Job Entrepreneur Housewife

Nationality Javanese Javanese

Religion Islam Islam

Education Bachelor Bachelor

Earning/month Approximately Rp 3.500.000,- -

Address Walang, North Jakarta

 History
Chief complain :
Breath shortness since 3 day before admission to the hospital

Additional complains:
fever, cough with phlegm, runny nose.
 Present history
shortness of breath since 3 day
before admission the hospital After hospital care,
Breath shortness is
1week ago she was admission in recurrent
another hospital with the same
complain
She was Patient
undergoing 2D admission at
She was diagnosed as
echocardiogram Raden Said
broncopneumonia and
Sukanto Police
Down Syndrome
Center Hospital
 Present history
Cough Fever

Since 4 days before admission, Since 4 days before

Cough with white phlegm, no admission.
blood. Goes up slowly. Occur up and
Following with breath down, increase at night to
shorteness morning.
Goes down with Paracetamol
History of past illness
Pharyngitis/Tonsilitis -
Bronchitis -
Pneumonia +
Morbilli -
Pertussis -
Varicella -
Diphteria -
Malaria -
Polio -
Enteritis -
Bacillary Dysentry -
Amoeba Dysentry -
Diarrhea -
Thypoid -
Worms -
Surgery -
Brain Concussion -
Fracture -
Drug Reaction -
• The mother routinely checked • Labor : Hospital
her pregnancy to the doctor in • Birth attendants : Doctor
the hospital. She denied any • Mode of delivery : Pervaginam
problem noted during her • Gestation : 38 weeks
pregnancy. She took vitamins • Infant state : Healthy
routinely given. No herbal or • Birth weight : 3000 grams
other consume • Body length : 48 cm
• According to the mother, the baby
started to cry and the baby's skin is red.

Mother’s
Pregnancy Child’s Birth History
History
Growth and Development History

Slant : 5 months
Prone : 7 years
Sitting : not yet
Crawling :-
Standing :-
Walking :-

Conclusion, poor status of growth and development

 Immunisation History

Immunization Frequency Time

BCG 1 time 1 month old

Hepatitis B 3 times 0, 1, 6 months old

DPT 3 times 2, 4, 6 months old

Polio 4 times 0, 2, 4, 6 months old

Hib 4 times 2, 4, 6, months old

Family History
• Patient’s both parents were married when they
were 28 years old and 25 years old, and this is
their first marriage

• Patient is the 3rd child, her mother pregnant

when 38 yo.

• There are not any significant illnesses or chronic

illnesses in the family declared
Physical Examination
General Status
General condition : Mild ill
Awareness : Apatis
Pulse : 121x/min
Breathing rate : 37x/min
Temperature : 37.3oC (per axilla)
Saturation O2 : 98% (with Oxygen 3L/m)
Physical Examination
Antropometry Status
Weight : 5,0 kilograms
Length : 65 cm
Head circumference : 36,5 cm
Physical Examination
Physical Examination
Physical Examination
Head to Toe Examination
Head
microcephaly, hair (black, rare distribution or almost bald, not easily removed) sign of trauma (-)
Eyes
Icteric sclera -/-, pale conjunctiva -/-, hyperaemia conjunctiva -/- , lacrimation -/-, sunken eyes +/+,
pupils cant be examined/3mm isokor, direct and indirect light response +/+ and +/+
Ears
AD: Normal shape, no wound, no bleeding, secretion or serumen
AS: Normal shape, no wound, no bleeding, secretion or serumen
Nose
Normal shape, midline septum, secretion -/-
Mouth
Lips: moist
Teeth: no caries
Mucous: moist
Tongue: no dirty, normal
Tonsils: T1/T1, no hyperemia
Pharynx: hyperemia (-)
Neck
Lymph node enlargement (-), scrofuloderma (-)
Head to Toe Examination

Thorax :
• Inspection : symmetric when breathing , retraction
(+), ictus cordis is not visible
• Palpation : mass (-), tactile fremitus -/-
• Percussion : sonor on both of lungs
• Auscultation:
Cor : regular S1-S2, murmur (+), gallop (+)
Pulmo : vesicular +/+, Wheezing -/- ,
Rhonchi +/+
Head to Toe Examination
Abdomen :
• Inspection : Convex, epigastric retraction (-), there is no a
widening of the veins, no spider nevi
• Palpation : supple, liver and spleen not palpable, fluid wave
(-), abdominal mass (-)
• Percussion : The entire field of tympanic abdomen, shifting
dullness (-)
• Auscultation: normal bowel sound, bruit (-)

Vertebra : There are no scoliosis, kyphosis, and lordosis, no mass

along the vertebral line
Extrimities : warm, capillary refill time < 2 seconds, edema(-)
Skin : Good turgor.
• Nuchal rigidity (-) • Defecation: Normal
• Kernig sign(-)
• Urination: Normal ( 3-4 times
• Lasegue sign(-)
• Brudzinski I(-)
daily )
• Brudzinski II(-) • Sweating: Normal

Neurological Autonom
Examination Examination
 Laboratorium finding
Hematology Results Normal Value

Haemoglobin 10,9 g/dL 13-16 g/dL

Leukocytes 14,000 /µL 5,000 – 10,000/µL

Hematocrits 36,7 % 40 – 48 %

Trombocytes 217,000/ µL 150,000 – 400,000/µL

Erythrocytes 4,03 million/µL 4 – 5 million/µL

MCV 91,1 fL 82-92 fL

MCH 27,00 pg 27-31 pg

MCHC 29,70 g/dL 32-36 g/dL

LED 15 mm/jam <= 15mm/jam

CRP kuantitatif >120 < 6,0 mg/L

 2D Echocardiogram
2D Echo
- Situs solitus
- AV-VA konkordans
- Dilatasi RA-RV
- Muara vena sistemik dan vena pulmonal normal
- Moderate TR PG 48 mmHg
- Moderate PR
- Moderate ASD secundum 7 mm bidirectional shunt
- Moderate PDA 3-4 mm L to R
- Arcus aorta on left side, no coartasio aorta
- No effusion pericardial
M-Mode : good contraction of heart

Conclusion : Moderate ASD secundum, small VSD, Moderate PDA, and

Moderate pulmonary hypertenssion
 Working diagnosis

• CHF ec CHDs (ASD, VSD, PDA)

• Pulmonary hypertension
• Broncopneumonia
• Down Syndrome
 Management
O2 3 l/m
IVFD KaEn 3B 125 cc / day
Meropenem 3 x 100 mg
Furosemid 3x 5 mg
Captopril 3x 3,125 mg
Spironolacton 1x 6,25 mg
Lasix drip 2mg/hour
Inh. Ventolin ½ amp + NaCl 2 cc
 Prognosis

Quo ad vitam : dubia ad bonam

Quo ad functionam : dubia ad malam
Quo ad sanationam : dubia ad bonam
Documentation
 Literature review
Congenital Heart disease
(ASD , VSD , PDA)
 • ASD is persistent opening in

1 the interatrial septum after

birth that allows direct
communication between the
ACYANOTIC left and right atria

Atrial  occurring with an incidence

of 1 in 1,500 live births
Septal
Defect  (5-10% CHDs)
 Male : female
(ASD) 1:2
 PATHOPHYSIOLOGY
Oxygenated blood from LA is shunted
into the RA

Volume overload & Right ventricular compliance will

Enlargement in RA be decreases over time
& RV

the left to-right shunt

shunt reverse : right-to- may lessen
left flow

If severe pulmonary
vascular disease develops
hypoxemia and cyanosis (e.g., Eisenmenger
syndrome)
Source : Lilly, Leonard S. Pathophysiology of Heart Disease. Fifth Edition. Lippincott Williams & Wilkins : Harvard Medical School. 2011. Page 361—385.
 Clinical findings
Symtoms
 Most infants with ASD →ASYMPTOMATIC !

 If symptoms do occur :
- Dyspnea on exertion
- Fatigue
- Recurrent lower respiratory infection
- The most common symptoms in adults are decreased stamina and
palpitations due to atrial tachyarrhythmias resulting from RIGHT
ATRIAL ENLARGEMENT
PHYSICAL EXAMINATION

• A prominent systolic impulse may be palpated along the lower-

left sternal border → contraction of the dilated RV (termed an
RV heave)
• S2 demonstrates a widened, fixed splitting pattern

• ↑ volume of blood flowing across the pulmonary valve 

Systolic murmur at the upper-left sternal border

• ↑ flow across the tricuspid valve Mid-diastolic murmur at the

lower-left sternal border
 Diagnostic studies
• Heart is usually • RVH
enlarged because of • RAE • Right atrial and
right atrial and right • Incomplete or complete right ventricular
ventricular dilatation right bundle branch block enlargement
• pulmonary artery is • Ostium primum ASD  • Visualizes ASD
prominent with Shunt direction
increased pulmonary displacement dan
vascular markings hypoplasia fesikulus (doppler)
anterior Left boundle • Right ventricular
branch  LAD systolic pressure
Chest can be determined
radiographs Electro
Cardiography Echo
cardiography
 Treatment
 Elective surgical repair → to prevent the development of
Heart Failure or Pulmonary Vasculary Disease

 Direct suture closure

 Amplatzer Septal Occluder
 2 • VSD is an abnormal
opening in the
interventricular
ACYANOTIC
septum

Ventricul  Incidence of 1.5 to 3.5

ar Septal per 1,000 live births
Defect
(VSD) • 15% - 20% of all
CHDs
Ventricular septal defect The hemodynamic changes that
accompany VSDs depend on the
size of the defect

• offer more resistance to flow than the pulmonary or systemic

small vasculature
VSDs

• RV, pulmonary volume stroke volume ↗ &

chamber dilatation
circulation, LA, and LV
larger
VSDs
overload (overtime)

systolic
• The augmented circulation dysfunction
HF through the pulmonary
symptoms vasculature can cause
pulmonary vascular disease as
early as 2 years of age

Source : Lilly, Leonard S. Pathophysiology of Heart Disease. Fifth Edition. Lippincott Williams & Wilkins : Harvard Medical School. 2011. Page 361—385.
 Pathophysiology

Source : Lilly, Leonard S. Pathophysiology of Heart Disease. Fifth Edition. Lippincott Williams & Wilkins : Harvard Medical School. 2011. Page 361—385.
 Clinical findings

Symptoms

 Asymptomatic
 CHF symptoms
 Tachypnea
 Poor feeding
 Failure to thrive
 Frequent lower respiratory
tract infection
 VSD complicated by
pulmonary vascular
disease and reverse shunt
→ dyspneu and cyanosis
 Pysical examination

 Harsh holosystolic
murmur at left sternal
border
 Smaller defect →loudest
Systolic murmur
 Systolic thrill (commonly
be palpated over the
region of the murmur)
 Diagnostic studies
• Cardiomegaly • LAE • Determine the
• prominent
pulmonary • LVH location of VSD
vascular markings • Identify direction
are present • RVH in of the shunt
• Enlargement of pulmonary • Provide an
pulmonary arteries
vascular disease estimate of right
ventricular
Chest systolic pressure
radiographs Electro
Cardiography Echo
cardiography
 Treatment
 By age 2  50% small-
moderate VSDs  complete
spontaneous closure

 Surgical correction
 First few months of life in
children w/ CHF or pulmonary
vascular disease
 Moderate sized defect without
pulmonary vascular disease 
later childhood

Source : Lilly, Leonard S. Pathophysiology of Heart Disease. Fifth Edition. Lippincott Williams & Wilkins : Harvard Medical School. 2011. Page 361—385.
 • Patent ductus arteriosus

3 (PDA) results when the

ductus
fails to close after birth,
ACYANOTIC
resulting in a persistent
connection between the
Patent great vessels
Ductus
Arterios • Incidence of about 1 in
us (PDA) 2,500 to 5,000 live term
births

• 5%-10% of all CHDs

 • first trimester maternal
Pathophysiology rubella infection,
• prematurity,
AFTER • and birth at a high
BIRTH altitude
↑ O2 ductus arteriosus
↓ PG usually constricts Risk Factor
after birth

Failure of the cyanosis of the

intimal ductus to
proliferation and close feet
fibrosis
unless
left-to-right pulmonary
Permane shunt vascular disease
ensues
nt closure
Volume left ventricular
Lig. overload in
arteriosum dilatation and
LA, LV → PA
left-sided
NORMALLY heart failure
 Clinical Findings
 Asymptomatic
 CHF symptoms (large shunt)
 Tachypnea

 Poor feeding

 Failure to thrive

 Frequent lower respiratory tract infection

 Fatigue, Dyspnea (moderated-size),

palpitation in adolescence and
adult life
 AF
 Endovascular infection
 Pysical examination

 Continuous machine-
like murmur at Left
subclavicular region
 Eisenmenger
syndrome present →
lower extremity
cyanosis & clubbing
 Diagnostic studies
• Visualize the defect -
enlarged cardiac • LAE, LVH Demonstrate flow
silhouette (left atrial through it
and left ventricular • Left atrial estimate right-sided
enlargement)
• Calcification of
enlargment systolic pressures
ductus may be
visualized in adult Electro Echo
Cardiography cardiography
Chest
radiographs

cath
Angiography /
• Look O2 saturation in
the pulmonary artery
compared with the RV
• Abnormal flow of blood
through PDA
 Treatment

 Neonatus and premature infants

 prostaglandin synthetic
inhibitors (indomethacin)

 Devinitive Closure : Surgical

division / ligation of the ductus /
transcatheter techniques
THANK
YOU