Coarctation of Aorta (CoA) in 1-year 4-month Old Girl

Coarctation of aorta (CoA) can be simply defined as narrowing in aorta causing

obstruction to the flow of blood in the aorta. CoA can occur at any region in the thoracic and

abdominal aorta. Most common location for CoA is just distal to the left subclavian artery at

the point where ductus arteriosus connects to the aorta. CoA accounts for 6–8% of all CHD

with an approximate incidence of four per 10,000 live births. 1,2

In babies with coarctation, the aortic arch may also be small (hypoplastic).

Coarctation may also occur with other cardiac defects, typically involving the left side of the

heart. Coarctation of the aorta is common in some patients with chromosomal abnormalities,

such as Turner's syndrome. In the presence of a coarctation, the left ventricle has to work

harder, since it must generate a higher pressure than normal to force blood through the

narrow segment of aorta to the lower part of the body. If the narrowing is very severe, the

ventricle may not be strong enough to perform this extra work, resulting in congestive heart

failure or inadequate blood flow to the organs of the body. 1,2

Pediatric patients are usually diagnosed due to weak femoral pulse, upper extremity

hypertension, a systolic murmur over upper sternal border with radiation to the back, and

upper-lower extremity systolic blood pressure gradient. Based on type of coarctation, size of

patient, severity of lesion, and associated abnormalities various management options like

surgical treatment, transcatheter balloon angioplasty and transcatheter stent implantation are

available. 3

We report the case of a 1-year and 4 months-old girl with Coarctation of aorta (CoA)

and acute respiratory tract infection.

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CASE REPORT

I. PATIENT’S IDENTITY
Name : ES
Medical Record : 90-95-31
Date of birth : October 23rd 2018
Age : 1 years 4 month
Gender : Female
Actual Body Weight : 8 kg
Ideal Body Weight : 9,5 kg
Body Height : 76 cm
Admission date : January 31st2020
Address : Fak-fak
Phone Number : 081322154811

PARENT’S INDENTITIES
Father Mother
Name Charles Marlinda

Date of Birth 31/12/1983 01/02/1991

Age 35 years old 28 years old

Last Education Elementary School Junior High School

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 Occupation Farmer Housewife

II. PATIENT’S HISTORY

Based on alloanamnesis from the mother.
Chief complaint : Oedema on periorbital
1. History of present illness
- Recurrent upper respiratory tract infection was noticed since a year ago, once
until twice in a month. There is cough and rhinore since a week before admitted
to hospital. Currently no dispnoe and cyanosis. There is no fever, seizure,
vomiting. The appetite is normal. Defecation is ordinary yellow, urinate is
sufficient. Patient referred to Wahidin Sudirohusodo pediatric cardiology
outpatient department for echocardiography screening because her twin being
suspected of acyanotic congenital heart disease.
2. History of previous illness
- History of recurrent respiratory tract infection almost every month since six
month of age.
- There was no history of blue and dispone before.
3. History of illness in the family
The patients’s mother said that her twin had the same complaints as the patients.
4. Patient’s personal and social history
a. Prenatal history
During pregnancy, the mother regularly controls to obstetrician, consume
blood and vitamin supplements tablets, never consumes herbs and other medicine
beside doctor’s prescription. Mother’s pregnancy was aterm and never felt sick.
b. History of delivery

Patient born spontaneously at primary health care by midwife, immediately

crying, with body weight 2300 gram, body height 46 cm and head circumference
33 cm.

c. Post-natal history

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 There were no pale, jaundice, seizures, cyanosis and bleeding in the post-
natal history. She got vitamin K injection and hepatitits B immunization.
d. Feeding History
The patient got breastfeeding for 1 month, then her mother gave formula
milk, banana weaning food in the age of 6 months, got milk porridge and then got
team rice in the age of 9 months. Finally, he got family food when the baby was 1
year old. The composition of family food were rice, fish, eggs, tofu, tempe,
vegetables and fruits. The family food given 3 times a day.
e. Growth and developmental history
Growth
The mother regularly took him to posyandu to get growth monitoring until
now. Based on Kartu menuju Sehat (KMS), growth curve was always above the
green line, hence normal growth.
Developmental
Start learning how to social smile in the age of 3 months, prone at 4 months
old, sat at 8 months old, start standing at 11 months and was able to walk 13
months old. She can speaks 2-3 words isentence.
f. History of immunization
Basic vaccination were complete; hepatitis B 4 times (age 0,2,3,4 months),
orally polio 4 times (age 0,2,3,4 months), HiB 4 times (age 0,2,3,4 months), BCG
at 1 month, DPT 3 times (age 2,3,4 months), measles once (age 9 months).
g. Basic needs
Physical-biomedic needs
Since she was born,her parents fully to care of her. Patient has got formula
milk beside complementary food and family meal. Clothing needs was also
fulfilled. She has received basic vaccination.her parents have a good awareness of
patient condition, therefore they made an effort for best medication with referred
this patient and her twin to hospital.

Emotional needs

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 There was a close and loving relationship between her family and her.
Father and mother were affectionate her. The parents were patient to pay attention
to her illness.
Mental stimulation needs
Early stimulation were given by both parents since early age that includes
touch and hug, playing together, and talking. The mother was dominant because
she had more time at home.
h. Family socio-economy history/environment/housing
Her father is 36 years old, a christian-papuaness, Elementary school
education, working as a farmer, her mother s 28 years old, a christian-papuaness,
Junior high school education, as a housewive. The patient had a sibling.her age is
6 years old.
She lives together with her parents and grandparents in permanent house
size ± 7x10m2, consisted of a living room, a family room, 2 bedrooms, a
bathroom and a kitchen. Ventilation and lighting were sufficient. Water source of
daily need came from PDAM, but for drinking came from refill gallon water. The
nearest distance from Community Health Center (Puskesmas) 1000 meters.
Patient’s health services are covered by BPJS.

III. PATIET’S DATA IN THE BEGINNING CASE

1. Physical Examination (Pediatric Ward, January 31st, 2020)
a. Present status
General condition : Moderate illness
Consciousness : GCS 15 (E4M5V6)
Vital sign
Blood pressure : 90/60 mmHg (upper arm)
80/50 mmHg (lower leg)
P50 85/40 mmHg;
P90 99/52 mmHg;
Heart rate : 118 x/minute
Respiratory rate : 28x/minute
Temperature : 36,8oC
Pain scale : 0 FLACC
General examination
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 Head : Mesocephal, normocephal, open fontanelle, black hair, difficult to
remove.
Face : no dismorfic, no edema
Eyes : no anemic conjunctiva, no icteric sclera. Eye movement was within
normal, no strabismus, round and isocor pupil, diameter
2,5mm/2,5mm, light relex normal.
Ear : No secret, intact tympanic membrane
Nose : Septum nasal was in the middle, no secret, mucosa was not
hyperemic
Mouth : No orally ulcer, no stomatitis
Teeth : No caries dentis
Throat : Pharyngs not hyperemic, no tonsilar enlargement
Neck : No thyroid glands enlargement. Normal jugular vein pressure, no
nuchal rigidity.
Chest : Shape and movement were symmetric, no deformities, no chest
indrawing.
Lung : Vocal fremitus symmetrical, percussion, sonor, vesicular breath
sound, no additional breath sound (wheezing and ronchi)
Heart : Ictus cordis was not visible, heart sound I-II normal, no murmur or
gallop.
Abdominal: No distension, supple, normal bowel sound, liver and spleen not
palpable, no ascites.
Genitalia : Girl, pubertal status A1M1P1
Lymph nodes : No enlargement
Extremities : Warm extremities, capillary refill time less the 2 seconds, no
oedema at pretibial and dorsum pedis. There is BCG scar on
deltoid region right upper arm. No oedema/pain on the joints.
Motoric: power and tonus are within normal limit, normal
physiological reflexes, no pathologic reflexes. No erythema.
b. Anthropometric status
Actual Body Weight : 8 kg
Ideal Body Weight : 9,5 kg
Body Height : 76 cm
Head circumference : 46 cm (Normal: 44 – 49 cm)
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 Weight-for-height : between -2SD and -1SD
Weight-for-age : between -2SD and Median
Height-for-age : between -2SD and Median

2. Laboratory Examination
Date of Examination Examination Result Normal Range
Januari 31st, 2020 Hb 11 11-13,5 g/dL
Ht 33,3 37-48 %
Leucocyte 16.650 4.000-10.000 /µL
Platelet 434.000 150.000-400.000/µL
MCV 70 80,0-97,0 fL
MCH 23 26,5-33,5 pg
MCHC 32 31,5-35,0 g/dL
Glucose 128 140 mg/dL
Sodium 137 136 – 145 mmol/l
Potassium 4,2 3,5 – 5,1 mmol/l
Chloride 109 97 – 111 mmol/l

Radiology Finding (Thorax imaging) (31/1/2010)

Expertise: Left to Right shunt suspect ASD

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IV. WORKING DIAGNOSIS
- Suspect Congenital Acyanotic Heart Disease
- Acute Respiratory Tract Infection

V. TREATMENT PLANNING
Therapy Echocardiography Examination
Ambroxol 4mg/8jam/orally
Pediatric  Diet according to Recommended Daily Allowance (RDA),
Nutrition
solid food 3x/day and snack 2x/day/orally, consist of:
Care
- energy 800 kcal/day
- protein 1-6 g/kgBW/day ≈ 8-48 gram/day
- fat 30% of total calory = 270 kcal = 30 gram/day
Monitoring - Vital sign especially Hemodinamic
- Nutritional state
Education  Parents were informed about patient’s current condition,
possible treatment that will be given
 Adherence and routine check up

VI. OBSERVATION IN PEDIATRIC CARDIOLOGY OUTPATIENT DEPT.

February 5th 2020

S There is cough, no dispone and cyanosis

The appetite is normal
No other symptomps
O Compos mentis
Blood pressure :
90/60 mmHg (upper arm)
80/50 mmHg (lower leg)
P50 85/40 mmHg;
P90 99/52 mmHg;
Heart rate : 120 beats perminute, regular, adequate volume
Respiratory rate : 28 breaths perminute, regular, adequate depth
Temperature : 36,8 OC
Echocardiography (5/2/2020)

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 Expertise:
- Situs solitus

- AV-VA Concordance

- RV and LV systolic function within normal

- Coarcatio Aorta (MaxPG 40 mmHg, Vmax 3,1 m/s)

- ASD (-) VSD (-) PDA (-)

A 1. Coarcatio Aorta
2. Upper Respiratory Tract Infection
P 1. Thorax MRI
2. Monitoring Hemodinamic signs
3. Diet according to Recommended Daily Allowance (RDA), solid food 3x/day
and snack 2x/day/orally, consist of:
- energy 800 kcal/day
- protein 1-6 g/kgBW/day ≈ 8-48 gram/day
fat 30% of total calory = 270 kcal = 30 gram/day

VII.OBSERVATION IN PEDIATRIC CARDIOLOGY OUTPATIENT DEPT.

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February 10th 2020

S There is cough (decrease), no dispone and cyanosis

The appetite is normal
No other symptomps
O Compos mentis
Blood pressure :
90/60 mmHg (upper arm). P50 85/40 mmHg;
80/50 mmHg (lower leg). P90 99/52 mmHg;
Heart rate : 122 beats perminute, regular, adequate volume
Respiratory rate : 28 breaths perminute, regular, adequate depth
Temperature : 36,8 OC

THORAX MRI (7/2/2020)

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 Expertise:
- Coarctatio aortae at decending aortae after sinistra subclavian artery arch
(narrowing of lumen with smallest diameter was 5 mm, long was 9mm )

- No collateral vascular and vascular ring at aortae thoracic level

- No patent ductus arteriosus appear

A 1. Coarcatio Aorta

2. Upper Respiratory Tract Infection

P 1. Referred to Harapan Kita Hospital

2. Monitoring Hemodinamic signs
3. Diet according to Recommended Daily Allowance (RDA), solid food 3x/day
and snack 2x/day/orally, consist of:
- energy 800 kcal/day
- protein 1-6 g/kgBW/day ≈ 8-48 gram/day
- fat 30% of total calory = 270 kcal = 30 gram/day

VIII. DEFINITIVE DIAGNOSIS

1. Coarctatio Aorta

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2. Upper Respiratory Tract Infection

IX. PROGNOSIS
Qua ad vitam : Dubia ad bonam
Qua ad fungtionam : Dubia ad bonam
Qua ad sanationem : Dubia ad bonam

X. DISCUSSION

Sketch showing heart with coarctation of the aorta. A: Coarctation (narrowing) of the aorta. 1:Inferior vena cava,
2:Right pulmonary veins, 3: Right pulmonary artery, 4:Superior vena cava, 5:Left pulmonary artery, 6:Left
pulmonary veins, 7:Right ventricle, 8:Left ventricle, 9:Pulmonary artery, 10:Aorta

About 80% of cardiac disease in children are congenital. The 9 most congenital heart

diseases that always found are Ventricular Septal Defect/VSD 30%, Patent Ductus

Arteriosus/PDA 12%, Atrial Septal Defect/ASD 7%, Tetralogy of Fallot 5%, Transposition

Great Artery/TGA 5%, Complete Atrioventricular Septal Defect/AVSD 2%, Pulmonary

Stenosis/PS 7%, Aortic Stenosis/AS 5% dan Coarctatio Aorta 6-8%. 1,2

This patient was diagnosed with coarctation of aorta (CoA) based on history taking,

physical examination, echocardiography and thorax MRI. From history taking there is

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recurrent upper respiratory tract infection noticed since a year ago, the frecuency is once until

twice in a month. No history of dispnoe and cyanosis. Appetite was normal. Patient referred

to Wahidin Sudirohusodo pediatric cardiology outpatient department for echocardiography

screening because her twin being suspected of acyanotic congenital heart disease. From

physical examination, the cardiac revealed no murmur, there is difference between upper and

lower extremity blood pressure, Upper was 90/60 mmHg and lower was 80/50 mmHg. Then

the diagnosed of CoA Established by echocardiography and MRI (there found narrowing of

aorta, narrowing of lumen with smallest diameter was 5 mm, long was 9 mm).

The clinical presentation and examination findings are variable based on patient’s age

and aortic narrowing. The condition can range from mild to severe, and might not be detected

until adulthood, depending on how much the aorta is narrowed. Children with serious aortic

narrowing may show signs and symptoms earlier in life, but mild cases with no symptoms

might not be diagnosed until adulthood. 1,3

Newborns and neonates are usually asymptomatic right after birth as patent ductus

arteriosus (PDA) helps perfuse lower body irrespective of severity of CoA. Neonates with

severe / critical CoA develop signs and symptoms of: 1,3

 Cardiogenic shock as the ductus arteriosus closes after birth.

 Absent femoral pulse

 Delayed capillary refill

 Feeding problems

 Decreased responsiveness

 Metabolic acidosis

 Myocardial depression.

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 Older pediatric patients are usually diagnosed due to

 Weak femoral pulse

 Upper extremity hypertension

 A systolic murmur over upper sternal border with radiation to the back

 Upper-lower extremity systolic blood pressure gradient. 1,3

Because of the CoA occurs beyond the blood vessels that branch off to upper body

and before the blood vessels that lead to lower body, this can lead to high blood pressure

in arms but low blood pressure in your legs and ankles. This is found clearly remarkable

in our patient. With coarctation of the aorta, the lower left heart chamber (left ventricle)

works harder to pump blood through the narrowed aorta, and blood pressure increases in the

left ventricle. This may cause the wall of the left ventricle to thicken (hypertrophy). 1,3

Children with congenital heart disease often experience abnormalities in several organ

systems and the most frequent complications is in the respiratory system. Respiratory tract

infections are the main reasons for mortality and morbidity, prolonged hospitalization,

respiratory failure, prolonged use of mechanical ventilators and delayed definitive surgery.

So that, early detection and prompt treatment of congenital heart disease in recurrent

respiratory tract infections can give the child sufficient time for growth and development,

decrease risk of morbidity and mortality, and reduce risk of long-term morbidity. 10,11

The mechanism that responsible for increasing breathing effort varies depending on

the mechanical changes that are caused by each cardiovascular anomaly. Children with

congenital heart disease who experience left to right shunt have increased pulmonary blood

flow resulting in pulmonary edema and decreased functional residual capacity. These changes

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then lead to atelectasis and a mismatch of ventilation and perfusion which will lead to

hypoxia. In this case, there found recurrent upper respiratory tract infection. 10,11

Diagnostic imaging

Transthoracic echocardiography (TTE) is the preferred diagnostic modality for

diagnosis and follow-up of CoA. TTE remains the mainstay of postnatal diagnosis and

follow-up for aortic arch anomalies. Goal of TTE is to identify arch anatomy, site of

coarctation, determine severity, and assess for associated intracardiac abnormalities. Cardiac

MRI (cMRI) have emerged as a sophisticated second line of advanced imaging that provides

excellent image resolution and anatomical details.. cMRI is a preferred non-invasive

advanced imaging for patients with CoA. cMRI does not include any exposure to ionizing

radiation but provides excellent image resolution which makes it ideal for initial imaging and

serial follow-ups. cMRI also provides valuable functional and anatomical data regarding

other intracardiac structures like aortic valve anatomy, myocardial mass, ventricular function,

valve function, etc. So, in this patient we use echocardiography and cMRI to establised the

CoA diagnosed. 4,7,8

Figure 1.Transthoracic echocardiographic views of coarctation of aorta. (A) Suprasternal sagittal two-dimensional
view showing narrowing in the aortic lumen at the isthmus (arrow). (B) Suprasternal sagittal color Doppler imaging
showing turbulent flow across the coarctation site (arrow). (C) Continuous wave spectral Doppler imaging across the

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coarctation segment in suprasternal view. Doppler shows increased flow velocity in systole with continuation of flow
in diastole (diastolic run-off). (D) Abnormal Doppler pattern in abdominal aorta in coarctation of aorta. Spectral
Doppler shows blunted velocity and systolic upstroke with continuous forward flow.

MRI from our patient show that the Coarcatio aortae located at decending aortae after

sinistra subclavian artery arch. It showed narrowing of lumen with smallest diameter was 5

mm and long was 9 mm.

Figure 2. Location of CoA

Treatment

This patient referred to Harapan Kita Cardiac centre hospital Jakarta for further

treatment. In adults and children found to have coarctation, treatment is conservative if

asymptomatic, but may require surgical resection of the narrow segment if there is arterial

hypertension. In some cases angioplasty can be performed to dilate the narrowed artery, with

or without the placement of a stent graft. Some of the widely accepted indications for

treatment of native coarctation are as below: 1,5,6,9,12

 Non-invasive systolic blood pressure gradient of >20 mmHg between upper and lower

limbs - Peak-to-peak transcatheter gradient of >/= 20 mmHg across the coarctation

site

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  Peak-to-peak transcatheter gradient of <20 mmHg in the setting of extensive collateral

circulation around the coarctation site

 Significant left ventricular hypertrophy

 Left ventricular systolic dysfunction

 Uncontrolled systemic hypertension in the setting of coarctation of aorta

 Abnormal blood pressure response during exercise stress test

Surgical Repair

Since the first surgical repair of CoA in early 1940s by Dr. Crafoord, surgery remains

a major treatment option for patients of all age group with CoA. In neonates and young

infants surgery is widely accepted as initial intervention of choice for significant CoA. Based

on the arch anatomy, site of coarctation and age of the patient, various surgical options are

available. Vast majority of surgeries for CoA in current era include end-to-end and extended

end-to-end anastomosis. Overall, surgical mortality after CoA repair is fairly low. Immediate

postoperative course may be complicated by paradoxical hypertension, recurrent laryngeal

nerve injury, bleeding, subclavian steal, residual coarctation, etc. Depending on the surgical

technique employed and complexity of the lesion some of the long-term complications

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include recurrent CoA, aneurysm formation, persistent hypertension, and very rarely spinal

cord ischemia. 6,9,12

Transcatheter Stent Implantation

This is a preferred treatment method for native and recurrent CoA in older children,

adolescents and adults. Results from the Congenital Cardiovascular Interventional Study

Consortium (CCISC) and the Coarctation of Aorta Stent Trial (COAST) trials show that stent

patients have lower rate of acute complications compared to surgery and balloon angioplasty

cohort. But they are more likely to require a planned reintervention for stent dilatation,

especially when implanted in younger patient. Acute complications after stent implantation

include stent migration, stent embolization, “jailing” of blood vessel, and aortic dissection.

Similar survival rate of about 93% at 10 years, 86% at 20 years, and 74% at 30 years

postoperatively is reported in older and contemporary postsurgical cohort. 6,9,12

Transcatheter Balloon Angioplasty

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 Appropriate sized balloon is chosen based on the measurements to dilate the

coarctation region. Goal of the procedure is to cause tear in the intima media by

overstretching of the narrow vessel site. After this dilatation and creation of tear, aortic wall

remodeling is expected to result in long-term resolution of CoA and prevent recoil. Balloon

angioplasty is preferred option in older children. It is also preferred choice in younger

patients with recoarctation. At present time use of balloon angioplasty in neonates and young

infant is mainly reserved in patients with associated ventricular dysfunction to get them

stabilized for definitive surgical repair. Its utility as an initial intervention in this very young

age group has fallen out of favor due to high recurrence rate and risk of vascular

complications. 6,9,12

The family education about the symptoms, the course of the disease, the posibility

treatment, the prognosis were most important. Families need to understand that the illness

requires a lifelong follow-up to monitor growth, developmental and any late complications. 6

XI. SUMMARY
A case of Coarcatio of Aorta in a 1-year 4-month-old boy with recurrent respiratory tract

infection. Diagnosis is based on history taking, physical examinations, echocardiography and

MRI. The patient was planned to referred to Harapan Kita Cardiac centre hospital Jakarta for

further treatment. The prognosis of this patient is dubia.

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