DIAGNOSTIC CASE REPORT

20 YEARS OLD WOMAN WITH PATENT

DUCTUS ARTERIOSUS

By :
dr. Daniel Nugraha

Supervisor:
dr. Yan Herry Sp.JP(K), FIHA, FSCAI, FAsCC
dr. Sefri Noventi Sofia, MSi.Med, Sp.JP, FIHA, FAsCC

Department of Cardiology and Vascular Medicine

Faculty of Medicine Diponegoro University
Dr. Kariadi Central General Hospital
Semarang
2021
 ABSTRACT

This case reported a 20 years-old woman that came to Kariadi Hospital with chief
complaint shortness of breath accompanied by fatigue and tiredness since 3 months before
admission, shortness of breath exaggerated during strenuous activities such as running or
climbing up to stairs and relieved with rest. She was never awakened at night due to shortness
of breath and also never experienced shortness of breath when lying down. She never had feet
or ankle swelling. She also had hoarseness that started 2 months before admission. She was
diagnosed with congenital heart disease since 2.5 years old.
On physical examination the patient was classified as underweight due to Body Mass
Index (WHO criteria) blood pressure 110/60 mmHg, pulse 90 times/minute, respiration 18
times/minute with 97% oxygen saturation on all four extremities. JVP was not increase.
Cardiac examination showed a loud S2 (P2), parasternal heave, continous murmur, grade
IV/VI, punctum maximum at 2nd spasium intercostal linea parasternalis sinistra, and
pansistolic murmur, grade III/VI, punctum maximum at LLSB. Her electrocardiogram
examination showed sinus ryhtm with heart rate 94 bpm, normoaxis, biventricular
hypertrophy. Chest X-ray showed cardiomegaly LA and RV. In laboratory test we found a
normal blood test value.
Based on five fingers diagnostic, the patient has chronic heart failure functional class
nyha II, suspect patent ductus arteriosus and tricuspid regurgitation with possibility of
pulmonary hypertension. Further diagnostic tests must be done to identify the etiology and to
determine the most appropriate treatment.

Keyword: Congenital Heart Disease, Patent Ductus Arteriosus, Chronic Heart Failure

i
 CONTENTS

Abstract ............................................................................................................................... i
Contents .............................................................................................................................. ii
Figures................................................................................................................................. iii
Tables .................................................................................................................................. iv
Case Illustration .................................................................................................................. 1
Literature Review................................................................................................................ 9
Discussion .......................................................................................................................... 30
References .......................................................................................................................... 31

ii
 FIGURES

Figure 1. Comparison of fetal and neonatal circulation after ductus arteriosus closure. .... 11
Figure 2. Patent ductus arteriosus ....................................................................................... 12
Figure 3. Auscultation of the patent ductus arteriosus ........................................................ 15
Figure 4. Preload curve of the relationship between LV end diastolic volume or LV end
diastolic pressure and cardiac work, which is measured as cardiac output ........................ 23
Figure 5. Normal curve of the left ventricular pressure-volume relationship..................... 24
Figure 6. Conditions that cause impaired left ventricular systolic and diastolic function
leading to left heart failure .................................................................................................. 25
Figure 7. Diagnostic algorithm for heart failure ................................................................. 28

iii
 TABLES

Table 1. PDA classification based on clinical sign ............................................................. 16

Table 2. ECG Findings in PDA .......................................................................................... 17
Table 3. Classification of heart failure based on ACCF/AHA &NYHA functional class .. 21
Table 4. Sign and Symptoms of Heart Failure .................................................................... 26
Table 5. Framingham Criteria ............................................................................................. 28
Table 6. Definition of Pulmonal Hypertension ................................................................... 29

iv
 CASE ILLUSTRATION

A. Patient Identity

Name : Ms. PDP

Age : 20 th
Gender : Female
Address : Semarang
Occupation : Student
Status : Not Married
Admission : 20 January 2022
Insurance : BPJS Non PBI

B. Anamnesis
Auto anamnesis in Elang I RPO Ward, at 21 January 2022.

Chief Complaint: Shortness of breath

History of Current Illness:

Patient came with a complaint of shortness of breath accompanied by fatigue and
tiredness since 3 months before admission. She feels tired easily and sometimes
breathless when doing strenuous activities such as running or climbing up to stairs when
she was at school, the symptomps was relieved with rest. She was never awakened at
night due to shortness of breath and able to sleep comfortably with 1 pillow. She also
never experienced shortness of breath when lying down and slept well in flat position.
She never had feet or ankle swelling nor she had enlargement of waist circumference.
There was no episode of chronic cough, fever, chest pain, nausea vomiting, cold sweat,
palpitations or syncope.
She also had mild hoarseness that started 2 months before admission.
Hoarseness did not accompany with sore throat, dyspneu, fever, and eating or
swallowing difficulty.
She was diagnosed with congenital heart disease since 2.5 years old. She never
had bluish discoloration of her lips or fingertips. Her parents already consult with
several pediatrician and cardiologist, and was offered operation, but did not agree.
1
 She was denied covid-19 vaccination because of her cardiac history 3 months
ago and felt the need to know her current cardiac conditions. Thus, she came to
cardiologist at Kariadi Hospital and agreed to do an elective diagnostic program.

History of perinatal
o History of mother consume drugs use (amphetamines, anticonvulsants, ACE
inhibitors, valproic acids, etc) outside health care provider evaluation during
pregnancy period was denied
o History of fever or other infection during mother’s pregnancy was denied
o History of mother consume alcohol was denied
o Antenatal care routinely with gynecologist during pregnancy

Growth History
o Patient was born spontaneously, aterm, birth weight 2500 grams, with the
help of gynecologist and no complication happens in labour process
o History of cyanosis on lips or fingertips when born until childhood was
denied
o History of breastfeeding interruption was denied
o History of immunization was completed
o Patient body always small (underweight) compare too her peer group and
growth chart
o History of hearing or vision impairment was denied
o History of asthma or allergy was denied
o History of reccurent respiratory tract infection was denied
o Patient actively interacted with surrounding environment and her peer
group.
o Patient did not have difficulty participating learning activities in her school
o History of drug abuse and alcohol consumption was denied

History of Family Illness

• No family member has the same illness
• No family member has congenital heart disease

2
 Socio-Economic
The patient is a student and live with her parents. Her parents work as a civil servant.
Medical fee was paid by BPJS insurance.

C. Physical Examination
Physical examination in Elang I RPO, at 21 January 2022.
General Appereance : Mild Illness
Awareness : GCS E4M6V5 = 15, compos mentis
General Status : Weight : 40 kg
Height : 160 cm
Body Mass Index : 15.6 kg/m2 (underweight)
WHO Criteria -Asian IMT
Vital Sign
o Blood Pressure: 110/60 mmHg
o Heart Rate: 90 bpm reguler
o Respiratory rate: 18 time per minute
o Temperature: 370C
o Oxygen saturation: 97% room air, all extremities

Head : Eyes : Pale conjungtiva (-/-), Scleral icterus (-/-)

Lips and mouth : Cyanosis (-)
Neck : JVP R+2 cm H20, Hepatojuguler reflux (-)
Chest : Normal appearance
Heart :
Inspection : Ictus cordis seen at 5th ICS, LMCS
Palpation : Ictus cordis palpate at 5th ICS, LMCS, thrill (+), parasternal heave (+),
epigastrial pulsation (-)
Auscultation : S1 normal
S2 (P2) loud
Continous murmur, grade IV/VI, punctum maximum at 2nd spasium
intercostal linea parasternalis sinistra, rough quality
Pansistolic murmur, grade III/VI, punctum maximum at Left Lower
Sternal Border, blowing, increased in inspiration
3
 Gallop (-)
Pulmo : Inspection : Symetric in both left and right chest in static
and dynamic state
Palpation : Stem fremitus symetric in both chest
Percussion : Sonor in both left and right chest
Auscultation : Vesicular (+/+) symetric in both chest
Rhonki (-/-)
Wheezing (-/-)
Abdomen : Inspection : Flat
Palpation : Suple, pain (-), liver and lien not palpable
Percussion : Tympani, shiffting dullness (-)
Auscultation : bowel movement sound (+) normal

Extremities : Cyanosis (-/-), Clubbing fingers (-/-), oedema (-/-), warm (+/+)

4
D. Supporting Examination
1. Electrocardiogram (20 January 2022)

ECG Description :
-Rythme : Sinus
-Rate : 94 beats over minute
-Axis : Normal (axis 64o)
-P wave : lead II: duration 0.08 s, amplitudo 0.15 mV, notch (-), P pulmonal/P
mitral (-)
-PR interval : 0,08 second
-QRS complex : Duration 0.10 second, no pathologic Q,
S in V1 = 1,4 mV ; R in V6 = 2,6 mV ; R in V6 + S in V1 = 4 mV (>3.5 mV – Sokolow
Lyon criteria for LVH)
R in V1 = 1,2 mV ; S in V5 / V6 = 0 mV ; R in V1 +S in V5 =1,2 (>1.05 mV – Sokolow
Lyon criteria for RVH) and R in V1 =1,2 mV (>0,6 mV – Myers criteria for RVH)
- ST segment : no ST-T changes
-T wave : Tall T (-), inverted T (-)
Interpretation : sinus rythme, rate 94 x/m, normoaxis, biventricular hypertrophy

5
2. X-ray Thorax (21 January 2022)

Description:
o Normal soft tissue, costaes and clavicula compact, vertebrae compact at midline,
enough inspiration
o Trachea at midline
o Cardio Torachic Ratio 54 %
o Elongatio aorta (-)
o Waist flattening(+)
o Prominent main pulmonary artery/conus pulmonalis
o Apex cranial
o Bronkhovaskular patern increase
o Right Heart limit < 1/3 right hemithorax
o Double contour (-)
o Sharp costophrenicus angle both lung
o Retrosternal space : Narrowed
o Retrocardiac space: Not Narrowed

X-ray interpretation :
o Cardiomegaly LA, RV
o Increase bronchovascular pattern

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3. Laboratorium Result (20 January 2022)
EXAMINATION RESULT UNIT NORMAL
RANGE

Hemoglobin 13.5 g/dL 13.2-17.3

Hematokrit 41.2 % 32- 62

Eritrosit 4.70 106/uL 4.4 - 5.9

MCH 28.7 pg 24 - 36

MCV 87.7 fL 76 - 96

MCHC 32.8 g/dL 29 - 36

Leukosit 8.9 103/uL 3.8 – 10.6

Trombosit 178 103/uL 150-400

Glukosa Sewaktu 80 mg/dL 80-160

Ureum 21 mg/dL 15-39

Creatinin 0.8 mg/dL 0,6-1.3

Natrium 139 Mmol/L 136-145

Kalium 3.8 Mmol/L 3.5-5.0

Chlorida 107 Mmol/L 95-105

HBsAg 0.10 - Negative <

1.00

Waktu Prothombin (PPT) 13.8 detik 9.4-11.3

PPT control 15.6 Detik

Waktu Thromboplastin 33.5 detik 23.4-31.5

(PTTK)

APPT Kontrol 31.2 Detik

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E. Working Diagnosis
1. Functional Diagnosis: Chronic Heart Failure Functional Class NYHA II
2. Anatomical Diagnosis:
o Suspect Patent Ductus Arteriosus dd/ VSD with Aorta Regurgitation, AV Fistula
o Suspect Tricuspid Regurgitation
3. Etiology Diagnosis: Acyanotic Congenital Heart Disease
4. Additional Diagnosis: Suspect Pulmonary Hypertension

8
 LITERATURE REVIEW

2.1 Patent Ductus Arteriosus

2.1.1 Definition
Patent ductus arteriosus (PDA) is a congenital heart disease in which the ductus
arteriosus does not close so that there is a connection between the aorta and the
pulmonary artery. The ductus arteriosus originates from the sixth aortic arch in the
fetus, which connects the pulmonary artery with the descending aorta. In normal infants,
this duct will close functionally 24-48 hours after birth and anatomically become the
ligamentum arteriosum at 2-3 weeks of age.1

2.1.2 Epidemiology
PDA is one of the most common congenital heart defects found in infants and
children and is the most common extra-cardiac shunt abnormality. The incidence of
PDA is about 5-10% of all CHD disorders. The constrictive response of the ductus
arteriosus to oxygen and the dilating response to prostaglandins are closely related to
gestational age. This causes a high incidence of PDA in preterm infants, especially
those with low birth weight or with pulmonary abnormalities. Approximately 45% of
infants with birth weight below 1750 grams have a patent ductus arteriosus. The
incidence of PDA in preterm infants is about 8 per 1000 live births. PDA is relatively
rare in the adult population.1,2
In contrast to the ductus arteriosus in preterm infants, in which failure to close
the ductus arteriosus results from a physiological developmental delay, failure to close
the ductus arteriosus in term infants is often associated with significant structural
abnormalities. Usualy persistent patency occurs because of hypoxaemic conditions that
cause ductal vasodilation. The incidence of PDA in term infants is approximately 1 in
2000 live births.
Mothers who are infected with rubella in the first trimester of pregnancy are
associated with an increased incidence of multiple congenital abnormalities which is
60% accompanied by involvement of cardiovascular system disorders such as PDA.

9
2.1.3 Etiology

The cause of congenital heart disease is not known for certain, but there are
several factors that are thought to have an influence on increasing the incidence of
congenital heart disease: 2,3
1. Prenatal Factor
a. Infection of Rubella at pregnancy period
b. Alcoholic mother
c. Mother age > 40 years old when pregnant
d. Insulin dependent diabetes mellitus of mother

2. Genetic Factor
a. History parents suffered congenital heart disease
b. History siblings suffered congenital heart disease
c. Chromosomal abnormalities like Sindrom Down.
d. Born with other congenital disorder

2.1.4 Anatomy and Physiology

The ductus arteriosus is an important component in the development of the
cardiovascular system, originating from the distal arch of the aorta. Ductus arteriosus
generally funnel-shaped with the tip located at the aortic arch, distal to the origin of the
subclavian artery, the left is larger (ampulla) and the lumen size narrows as it
approaches the insertion (where it connects) with the main pulmonary artery or left
pulmonary artery.4
In the case of a right-sided aortic arch, the anatomy of the ductus arteriosus can
vary significantly. The ductus arteriosus can arise from the left brachiocephalic trunk
and connect proximal to the left pulmonary artery or it can arise distal to the origin of
the right subclavian artery and connect proximal to the right pulmonary artery. Bilateral
ductus arteriosus may also occur
The presence of the ductus arteriosus in the fetal circulation is very important as
a bypass the flow of oxygen-rich blood and nutrients from the maternal placenta to the
systemic circulation without having to pass through the fetal pulmonary circulation. In
normal fetal circulation, oxygen-rich blood flows from the maternal circulation to the
placenta, continuing through the fetal inferior vena cava to the right atrium, right
10
 ventricle and main pulmonary artery. The fetal pulmonary arteries are constricted and
have high vascular resistance. This causes the oxygen-rich blood to enter the ductus
arteriosus and flow into the systemic circulation, which has a lower vascular resistance.
In the fetus, the ductus arteriosus remains open due to low arterial oxygen levels and
prostaglandin E2 (PGE2) derived from the maternal placenta.
At birth, several changes occur that initiate functional closure of the ductus
arteriosus in the first 15-18 hours of life. Respiration spontaneously occurring in the
newborn causes an increase in arterial oxygen levels. Prostaglandin levels also decrease
with the separation of the placenta and increased prostaglandin metabolism in the
infant's pulmonary circulation. The combination of increased blood oxygen levels and
decreased circulating levels of prostaglandins usually results in closure of the ductus
arteriosus.
Generally, the ductus arteriosus has no hemodynamic impact in the first 15 hours
from birth and closes completely by 2 to 3 weeks of life. The obliterated remnant of this
structure persists into adulthood and is called the ligamentum arteriosum. Spontaneous
closure of the ductus arteriosus is very rare in term infants older than 3 months and very
rarely occurs in preterm infants older than 12 months.

Figure 1. Comparison of fetal and neonatal circulation after ductus arteriosus closure.5

2.1.5 Pathophsyiology
The ductus arteriosus in the fetus opens wide and carries deoxygenated blood
from the right ventricle to the descending aorta and the placenta where oxygen-rich
blood is found. The mechanism of postnatal ductus arteriosus closure occurs in two
phases. The first phase occurs within 12 hours of birth (in term infants) in which cellular
contraction and migration of smooth muscle cells in the tunica media of the ductus

11
arteriosus results in shortening, thickening of the walls leading to closure of the ductus.
The second phase is usually completed when the baby is 2-3 weeks old. In this phase,
there is endothelium indentation, disruption and fragmentation of the elastic internal
lamina, proliferation of the sub-intima layer, and bleeding and necrosis in the tunica
intima. Finally, connective tissue and fibrosis are formed in the lumen of the duct so that
the ligamentum arteriosum eventually forms. 2,3
The exact cause that initiates the closure of the ductus arteriosus is still not
known with certainty. From the results of research on animal fetuses, it shows an
increase in PO2 that occurs due to the ventilation process after birth which then causes
constriction of the ductus arteriosus. With increasing gestational age, the response of
ductal constriction to an increase in PO2 increases, and the level of PO2 required to
initiate ductal closure decreases.
Prostaglandins (PGE1, PGE2 and PGI2) cause dilation of the lumen of the
ductus arteriosus, on the other hand, a prostaglandin synthesis inhibitor such as
Indomethacin can cause constriction of the ductus arteriosus. The patency or closure of
the ductus arteriosus represents a balance between the constrictive effects of oxygen,
and possibly several other constrictors, and the relaxing effects of prostaglandins.

Figure 2. Patent ductus arteriosus. A. The direction of the arrow indicates the shunt
flow from the descending aorta (Ao) to the pulmonary artery (PA). B. Schematic of
blood flow in the PDA. The direction of the arrow shows increased back blood flow to
the left heart as a result of the shunt, which causes enlargement of the left atrium (LA)
and left ventricle (LV) and Ao. IVC: inferior vena cava; RV: right ventricle; SVC,
superior vena cava2

12
 The hemodynamic impact of PDA on the cardiovascular system is determined by
the extent of the shunt flow, which is largely dependent on the resistance to flow within
the ductus arteriosus. The length, smallest diameter and shape and configuration of the
ductus arteriosus determine the resistance to this flow. In addition, because the flow
within the ductus arteriosus is dynamic and pulsatile, the elasticity of the ductus walls
can also affect the resistance to blood flow.6
The magnitude of the shunt flow depends not only on the ductal resistance but
also on the pressure gradient between the aorta and the pulmonary artery. This pressure
gradient is dynamic, with systolic and diastolic components, and is largely dependent on
systemic and pulmonary vascular resistance, and cardiac output. The impact of changes
in systemic and pulmonary vascular resistance is more pronounced in large ducts with
low resistance.
A left-to-right shunt through the ductus arteriosus creates excess blood flow into
the pulmonary circulation and causes volume overload in the left heart. An increase in
pulmonary blood flow results in an increase in pulmonary fluid volume, and in patients
with moderate or large shunts, this can result in decreased lung compliance, which can
lead to an increased effort to breathe. Pulmonary edema is rare but may occur in elderly
patients with severe congestive heart failure. Increased blood flow returning to the left
heart causes an increase in left atrial pressure and an increase in left ventricular end-
diastolic pressure. The left ventricle will then compensate by increasing stroke volume
and ultimately through hypertrophy to normalize wall stress.
Neuroendocrine adaptation also occurs, with increased sympathetic activity and
circulating catecholamines resulting in increased contractility and heart rate. Diastolic
blood pressure in the aorta is decreased because there is blood flowing through the
ductus arteriosus during diastole, and when combined with the shortened diastolic effect
due to tachycardia, increased intramyocardial stretch due to left ventricular dilatation,
and increased myocardial oxygen demand, it can result in subendocardial ischemia.
With continued left-to-right shunts, exposure to increased flow and high pressure
in the pulmonary arterial system leads to progressive morphological changes in the
pulmonary vasculature. These changes include hypertrophy of the tunica media arteries,
intima proliferation and fibrosis and ultimately obliteration of the pulmonary arterioles
and capillaries leading to a progressive increase in pulmonary vascular resistance. When
pulmonary vascular resistance approaches and exceeds systemic vascular resistance, the
shunt flow changes direction from right to left.
13
2.1.6 Natural History of PDA
The course of disease in patients with patent ductus arteriosus is highly
dependent on the size of the PDA, the direction of the shunt flow, and the development of
associated complications. At birth, 95% of patients with isolated PDA have a left-to-right
shunt and a normal or near-normal pulmonary pressure. Patients with normal pulmonary
artery pressures and no evidence of chronic left ventricular volume overload have a better
prognosis. If left untreated, the life expectancy of patients with PDA is shortened. 1/3 of
patients with PDA are expected to die by the age of 40 years and almost 2/3 of them will
die by the age of 60 years.4
Congestive heart failure can occur in PDA due to a chronic increase in left heart
volume. In patients with PDA-related deaths, congestive heart failure was the leading
cause of death. The occurrence of a right-to-left shunt is also a bad sign because this
condition indicates the development of severe pulmonary vascular disease and causes an
increase in right heart pressure.

2.1.7 Clinical Manifestation

Initial clinical assessment of patients with suspected cardiac abnormalities
consists of five modalities of cardiology examination, namely history taking,
physical examination including inspection, palpation and auscultation, ECG, and
chest X-ray. The weight of information obtained from the five modalities varies,
depending on the type and severity of heart disease.1
The symptoms experienced by patients are highly dependent on the degree
of left to right shunt which is influenced by the size of the PDA, ductal resistance,
cardiac output, and systemic and pulmonary vascular resistance.3
Between 25-40% of patients with PDA are asymptomatic, especially
patients with small PDA. These patients are often found incidentally on diagnostic
examination, which is auscultated with a continuous murmur. On a PDA with a
larger size symptoms may be found. Symptoms that patients often complain about
are shortness of breath, peripheral edema, and palpitations.

2.1.8 Physical Examination

Patients with PDA may exhibit a wide range of physical examination findings. A
wide pulse pressure can be found and a bounding pulse is found at the periphery.
14
Jugular venous pressure may be normal in small PDAs, and in larger PDAs there may
be prominent a and v waves. On precordial palpation a small PDA is usually within
normal limits, while a large PDA shows left ventricular enlargement.2,4
On auscultation, a harsh continuous murmur was found in the ICS I-II left
sternal border. The murmur passes through S2 and decreases in intensity during
diastole. A small PDA may produce a soft, high-frequency continuous murmur. While
the larger PDA delivers a distinctive machinery-like-loud murmur.3

Figure 3. Auscultation of the patent ductus arteriosus. Systolic thrill can be found in
the dotted area.2

If pulmonary hypertension is present, right ventricular lift can be obtained

with an increase in the intensity of the pulmonary component. The duration of the
diastolic murmur reflects the pulmonary artery pressure. An increase in pulmonary
artery pressure causes a decrease in the left-to-right flow gradient in the PDA
during diastole. This causes a shorter diastolic murmur. Likewise, the systolic
component of the murmur will also decrease. If there is a right-to-left flow, the
systolic murmur will disappear. In this right-to-left flow, the pathognomonic
physical finding is distinct cyanosis in the lower extremities and the left hand.2,3
The clinical manifestations of PDA in adults are often asymptomatic for
many years. PDA can manifest as a result of conditions such as recurrent
pneumonia, COPD, due to valvular manifestations or ischemic heart disease.
From a clinical perspective, PDA in adults can be divided according to the
following table:

15
 Table 1. PDA classification based on clinical sign3

PDA Type Murmur Wide pulse Left Ventricle Pulmonal

Pressure Dilatation Hypertension
Silent - - - -
Small continous - - -
Moderate continous + + +
Large Systolic + + ++ ++

Diastolic +
Eisenmenger Ejection murmur - ++ +++

In simple terms, PDAs can also be classified as follows:2,3

- Small: a long-ejection or continuous murmur is heard, radiating to the back.
Normal peripheral pulse, normal left atrial and left ventricular size without
pulmonary hypertension.
- Moderate: generally there is a wide peripheral pulse and a continuous
murmur is heard. There is enlargement of LA and LV and PH begins to
occur (usually still reversible)
- Large: generally in adult patients with physiological Eisenmenger. There
are signs of pulmonary hypertension. No more continuous murmurs heard.
Causes differential cyanosis and clubbing.
The other classification to determine the degree of PDA based on noise and
pulmonary-systemic blood flow ratio is: 3,7
- Silent: PDA is so small that only detectable by investigation (usually
echocardiography)
- Small: continuous murmur is common; Qp/Qs < 1.5
- Moderate: continuous murmur is common; Qp/Qs of 1.5 to 2.2
- Large: continuous murmur ; Qp/Qs > 2.2
- Eisenmenger: no continuous murmur, marked pulmonary hypertension,
differential hypoxemia, and differential cyanosis (pink fingers, blue toes)

16
2.1.9 ECG, X-ray, and Laboratorium Examination in PDA
ECG and chest X-ray have their respective advantages and disadvantages to
assess the severity of heart disease. ECG detects hypertrophy well, so it can detect the
presence of pressure overload, but is less accurate in detecting dilatation due to
volume overload. While the chest X-ray is most suitable for detecting volume
overload, it is not good at diagnosing hypertrophy without dilatation.4

Table 2. ECG Findings in PDA

ECG PDA
Normal Small
LVH, LAE, Tachycardia, AF Moderate- Large
BVH, RVH, RAE Eissenmenger’s

ECG examination is also not sensitive or specific for PDA, sinus tachycardia
or atrial fibrillation can be found, left ventricular hypertrophy, and left atrial dilatation
in cases of PDA with moderate to wide shunt. In increased pulmonary artery pressure,
marked appearance of biventricular hypertrophy and enlargement of the right atrium.
For cases with a small PDA size, a normal ECG is found.3,4
X-ray examination is non-specific and insensitive for PDA cases. Small PDA
will not be found abnormalities on chest X-ray. Whereas in a wide PDA will be found
dilatation of the left atrium, left ventricle and increased vascular markings in the
lungs. If pulmonary hypertension is found, there will be enlargement of the pulmonary
arteries, calcification of the PDA can be found in elderly patients. 4,5
Blood laboratory examination did not find a typical PDA picture,
erythrocytosis may be found as compensation if cyanosis occurs in a long period.

2.1.10 Different Diagnosis

The hallmark of PDA is the presence of a continuous murmur on auscultation of
the heart. The differential diagnosis for a continuous murmur is as follows: 4.5
1. Coronary arteriovenous fistula: continuous murmur is heard maximally at the
right sternal border.
2. Systemic arteriovenous fistula: found a bounding pulse, wide pulse pressure,
found signs of chronic heart failure. Continuous murmurs are not found in the
17
 precordial area but in fistula areas such as the head or liver.
3. Pulmonary arterioveous fistula: a continuous murmur can be heard in the back.
There are signs of cyanosis and clubbing without signs of cardiomegaly.
4. Venous Hum: maximal audible in the right and or left area around the clavicle
when the patient is examined in a sitting position. Usualy this sound will
disappear when the patient is examined in the supine position.
5. Collateral in CoA: continuous murmur heard over the intercostal spaces, and
bilateral.
6. VSD with AR: to and fro murmur, tends to be similar to continuous murmur,
usually heard over the mid-left sternal border or lower left sternal border.
7. Pulmonary valve loss: to and from a murmur (sawing wood) heard at the upper
left sternal border. Very enlarged pulmonary artery hilar, presence of RVH on
ECG. The patient is usually cyanotic, as this disorder usually accompanies
Tetralogy of Fallot.
8. Persistent Truncus Arteriosus: a continuous murmur can be heard in the right
second intercostal space or on the back of a cyanotic infant. The EKG shows
biventricular hypertrophy, the chest radiograph shows cardiomegaly.
Sometimes found a picture of the right aortic arch.
9. Aortopulmonary septal defect (aortopulmonary window) is a very rare
condition, with a bounding pulse, but a murmur that reflects a VSD. Chronic
heart failure develops early in infant development.
10. Peripheral PA stenosis: a continuous murmur is heard over the entire thoracic
field. The EKG may depict an RV if the stenosis is severe. It occurs in
association with Williams syndrome or rubella syndrome.
11. Ruptured sinus valsalva aneurysm: sudden onset of chest pain and signs of
heart failure with rapidly developing shortness of breath. To and from
murmurs can be heard at the base of the heart. This condition is often found in
Marfan's Syndrome.
12. TAPVR (total anomalous pulmonary venous return) flowing to RA: similar to
venous hum, heard at the right sternal border. The ECG showed RVH, the
chest X-ray showed cardiomegaly and increased vascular markings in the
lungs.

18
 2.1.11 Complication

The most common complications of PDA are chronic heart failure, infective
endocarditis and pulmonary hypertension. Chronic heart failure occurs due to
volume overload on the left side of the heart. As a result of this often also occurs
atrial fibrillation. Vegetation is often found on the pulmonary side, as a result of
which septic pulmonary embolism may occur. Untreated PDA has a 0.45% risk of
infective endocarditis per year after the second decade of life. 3,5
In general, complications that can occur in PDA patients are:4
o Endarteritis: quite rare
o PDA aneurysm: found in young adults or occurs after endarteritis
o PDA calcification: common in elderly patients
o Atrial arrhythmias: often found in moderate PDA
o Progressive Pulmonary Hypertension: depending on the size of the PDA and the
degree of shunt. Over time, Eisenmenger syndrome may develop, with clinically
differential cyanosis.

2.2 Heart Failure

2.2.1. Definition
Heart failure (HF) is a clinical syndrome characterized by structural and/or functional
disturbances in filling or pumping blood by the ventricles.9
The European Society of Cardiology (ESC) guidelines for acute & chronic heart failure
stated that heart failure is a set of clinical symptoms including shortness of breath and fatigue
which accompanied by signs of increased jugular venous pressure, rales, and peripheral
edema caused by structural and/or cardiac function abnormalities resulting in heart failure to
deliver normal oxygen required for tissue metabolism both at rest and during activity.
Therefore, heart failure is a clinical syndrome consisting of variable symptoms & signs
such as shortness of breath, fatigue, tachycardia, tachypnea, increased jugular venous
pressure, rhonchi in both lung fields, hepatomegaly, and peripheral edema.10

2.2.2 Epidemiology
Approximately 1-2% of adult population in developed countries suffer from heart
failure, which more than 10 percent of population are in the age group 70 years or older. In
general, men over the age of 55 have a 33% risk of developing heart failure, while women

19
 have a slightly lower risk of 28%. Heart Failure with Preserved Ejection Fraction (HFpEF)
affects 22 to 73% of the population, depending on the definition, location of medical service
facilities (primary care, clinics, hospitals), age, gender, history of myocardial infarction, and
year of publication of the research.2 An epidemiological study in the United States stated that
5.7 million people have heart failure, and it is estimated that by 2030 the number will increase
to 25% from recent study

2.2.3 Classification
Based on left ventricular ejection fraction (LVEF)
Terminology used to classify heart failure based on LVEF. EF is the ratio of stroke
volume (end-diastolic volume – end-systolic volume) to end-diastolic volume. Based on the EF,
heart failure is divided into three types:10
- Heart failure with reduced EF (HF-Reduced EF or systolic heart failure). Heart failure with
EF <40%
- Heart failure with minimal EF reduction (HF-mildly impaired EF). Heart failure with EF >
40-49%
- Heart failure without EF reduction (HF-preserved EF or diastolic heart failure).
- Heart failure with EF ≥50%

Based on Onset
Heart failure can be classified based on the onset or timeline of the patient clinical
symptoms, such as:
- Chronic HF; patients with chronic heart failure
- Chronic stable HF; Patients receiving treatment whose condition has persisted for at least 1
month
- Acute decompensated HF; patients with chronic HF experience worsening (decompensated),
often bringing the patient to the hospital.
- New (acute de novo) HF; occurs acutely, usually as a result of acute myocardial infarction
- Congestive HF; a term still sometimes used, especially in America, to describe both acute and
chronic heart failure with conditions of congestion (water and sodium retention).

Based on the severity of heart failure

American College of Cardiology Foundation/American Heart Association
(ACCF/AHA) and New York Heart Association (NYHA) classified heart failure based on the
20
stage of the disease or by its functional class, which can provide useful information about the
severity of heart failure. The ACCF/AHA classification of heart failure emphasizes the
development and progression of the disease, while the NYHA functional class focuses on the
patient's physical activity capacity and symptoms. Classification of heart failure based on
ACCF/AHA and NYHA functional class is shown in Table 1.11,12

Table 3. Classification of heart failure based on ACCF/AHA and NYHA functional class
ACCF/AHA Stages of HF NYHA Functional Classification
A At high risk for HF but without I No limitation of physical activity. Ordinary
structural heart disease or physical activity does not cause symptoms
symptoms of HF of HF.
B Structural heart disease but II Slight limitation of physical activity.
without signs or symptoms of HF Comfortable at rest, but ordinary physical
activity results in symptoms of HF.
C Structural heart disease with prior III Marked limitation of physical activity.
or current symptoms of HF Comfortable at rest, but less than ordinary
physical activity causes symptoms of HF.
D Refractory HF are requiring IV Unable to carry on any physical activity
specialized interventions without symptoms of HF, or symptoms of
HF at rest.

2.1.4 Etiology
Etiology of heart failure can be classified into various diseases or conditions such as:13,14
• Myocardial disease
• Coronary artery disease
• Hypertension
• Immunity /inflammation: viral myocarditis, Chagas’ disease
• Metabolic /infiltratif: thiamine deficiency, hemochromatosis, amyloidosis,
sarcoidosis
• Endocrin: thyrotoxicosis
• Toxic: alcohol, cytotoxics, negative inotropic drugs (eg CCBs)
• Idiopathic: cardiomyopathy (dilated, hypertrophic, restrictive, peri partum)
• Valve disease
• Stenosis/ mitral regurgitation
• Stenosis/ aorta regurgitation
• Stenosis / pulmonal regurgitation
• Stenosis/ tricuspid regurgitation
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 • Pericardial disease
• Effusion
• Constriction
• Endomyocardial disease
• Endokarditis Loeffler
• Endomyocardial Fibrosis
• Congenital heart disease
• Such as: atrial/ventricular septal defect
• Genetic
• Such as: familial dilated cardiomyopathy
• Arrhythmia (bradycardia and tachycardia)
• Atrial
• Ventricle
• Conduction disorders
• Sinus node dysfunction
• 2nd degree atrioventricular block
• 3rd degree atrioventricular block
• High output states
• Anemia
• Sepsis
• Thyrotoxicosis
• Paget’s disease
• Arteriovenous fistula
• Volume overload
• Renal failure
• Iatrogenic

2.1.5. Pathophysiology
Heart failure occurs due to failure of right ventricular and left ventricular function.
However, left heart failure is the most common cause of heart failure. The function of the heart

22
that we can see from cardiac output is influenced by two main components, namely heart rate
and stroke volume. 9,11
Stroke volume is affected by preload, cardiac muscle contractility, afterload. Preload is
defined as the tension of the ventricular wall in end-diastole, which is the stretch of the
ventricular muscle fibers just before contraction, often equated with end-diastolic volume or
end-diastolic pressure.

Figure 4. Preload curve of the relationship between LV end diastolic volume or LV end
diastolic pressure and cardiac work, which is measured as cardiac output.

Afterload is defined as the pressure of the ventricular wall during contraction to expel
the contents inside. Afterload is often equated with ventricular systolic pressure.
Cardiac muscle contractility is the ability of the heart muscle to adapt to different
preload and afterload that occurs due to the influence of hormones and chemical reactions.

Pressure – Volume Loops Curve

The curve below depicts the relationship between changes in ventricular volume in
response to pressure changes that occur during the cardiac cycle. In the left ventricle, the
process of filling in the ventricular chambers begins after the opening of the mitral valve at the
beginning of diastole.
As volume increases during diastole, there is an increase in internal pressure,
accompanied by passive elongation of the myocardium. Furthermore, the onset of left
ventricular systolic contraction causes an increase in pressure in the ventricles.
When the pressure in the left ventricle exceeds the pressure in the atria, the mitral valve
closes. Then there is an increase in pressure, which is not accompanied by an increase in
23
ventricular volume. This pressure is then increased through the process of isovolumetric
contraction. When this pressure exceeds the aortic diastolic pressure, the aortic valve opens and
ejection of blood from the left ventricle into the aorta occurs.
During the ejection process, the volume in the ventricle decreases, but the pressure
continues to increase until the ventricular relaxation process occurs. After this process, the
ventricle then relaxes, and there is a decrease in pressure in the ventricle with a volume that is
still constant because the mitral valve has not opened. This process is known as isovolumetric
relaxation. The process is restarted when the pressure in the ventricles drops below the atria,
and the mitral valve opens again.11

Figure 5. Normal curve of the left ventricular pressure-volume relationship.11

Heart failure occurs due to disruption of the three processes that have been mentioned,
namely disturbances in preload, afterload, and contractility of the heart muscle.

24
Figure 6. Conditions that cause impaired left ventricular systolic and diastolic function leading
to left heart failure.10

In patients with LV systolic dysfunction, myocytes alteration and extracellular matrix

formation after myocardial injury (eg, myocardial infarction) leading to ventricular remodeling,
with dilatation and impaired contractility, as seen with decreased EF. If this systolic
dysfunction is not treated, it will progressively worsen over time, with enlargement of the LV
and EF reduction, even though the patient is asymptomatic.
There are two mechanisms that can lead to worsening of the disease. First, events that
can increase myocyte death (eg recurrent myocardial infarction). Second, the emergence of a
systemic response due to a decrease in systolic function, especially neurohormonal activation.
Two important neurohormonal systems, the renin-angiotensin-aldosterone system and the
sympathetic nervous system. In addition, the responses of these two systems exert effects on the
blood vessels, kidneys, muscles, bone marrow, lungs, and liver. Termination of these two
processes is the basis of effective heart failure therapy.10,14

25
2.1.6. Sign and Symptoms of Heart Failure

The main clinical manifestations of patients with heart failure are shortness of breath,
fatigue, and decreased tolerance for physical activity, accompanied by fluid retention in the
form of pulmonary and peripheral edema. These various symptoms and signs result in a
decrease of the heart function and patient quality of life.
However, not all of these symptoms and signs appear at the same time, some patients
may experience a decrease in functional capacity without significant fluid retention, on the
other hand, some patients experience significant signs of fluid retention without significant
shortness of breath or fatigue. This happens because not all patients with heart failure
experience fluid congestion in the early stages of heart failure, so the term congestive heart
failure which is often used in the past is not appropriate anymore.
The diagnosis of heart failure is quite difficult to establish in the early stages of the
disease. Some of the symptoms that patients complain during treatment are not specific to heart
failure. Symptoms specific to heart failure, such as orthopnea and paroxysmal nocturnal
dyspnea, are less common in patients, especially those with mild symptoms. Most of the signs
of heart failure are caused by water and sodium retention, so these signs can be reduced quickly
with diuretic therapy

26
 Table 4. Sign and Symptoms of Heart Failure14

The Framingham criteria are epidemiological criteria that have been widely used. A
minimum of two major criteria or one major and two minor criteria are required to make a
diagnosis of congestive heart failure. These minor criteria are also considered valid if they are
not associated with other related diseases such as pulmonary hypertension, chronic obstructive
pulmonary disease, liver cirrhosis, or nephrotic syndrome. The Framingham criteria that can be
used to establish a diagnosis of congestive heart failure are listed in Table 5.15

27
 Table 5. Framingham Criteria15
Major criteria Minor criteria
Paroxysmal nocturnal dyspnea Cough at night
Neck vein distention Shortness of breath on normal activities
Ronchi Hepatomegaly
Gallop S3 Pleural effusion
Cardiomegaly (cardiothoracic ratio Decreased vital capacity to 1/3 of the
>50% on a chest x-ray) maximum recorded value
Acute pulmonal edema Tachycardia (heart rate > 120x per
minute)
Hepatojugular reflux Bilateral ankle edema
Elevated central venous pressure
>16cm H2O in right atrium
Weight loss >4.5kg in 5
days in response to treatment
Circulation time is more than 25
seconds

2.1.7. Diagnostic Algorithm for Heart Failure

Figure 7. Diagnostic Algorithm for Heart Failure

28
2.3 Pulmonal Hypertension
2.2.1. Definition
Pulmonary hypertension is a condition that requires special attention. Until
recently, pulmonary hypertension was defined by an increase in PAP (pulmonary arterial
pressure) as measured when right heart catheterization was > 20 mmHg. 16

Table 6. Definition of Pulmonal Hypertension

2.2.2. Diagnosis
To diagnose pulmonary hypertension, including by looking at past medical
history, physical examination, lung function, blood gas analysis, imaging (especially with
echocardiography) and laboratories. In general, right heart catheterization with
compartment oximetry is required for decisions such as initiating and following up
vasodilator therapy, pregnancy, or surgery. Invasive hemodynamic assessment is not
usually necessary to guide therapeutic intervention over time. Since a higher hematocrit
level leads to a higher PVR, it may therefore have to be taken into account in the
evaluation of therapy.16

29
 DISCUSSION

The diagnosis in this patient was based on anamnesis, physical examination, which
was supported by additional examinations, namely electrocardiogram, chest x-ray, and
laboratory test to confirm the diagnosis.
Anamnesis showed a 20 years-old woman that came to Kariadi Hospital with chief
complaint fatigue and tiredness since 3 months before admission, during strenuous activities
such as running or climbing up to stairs. She was never awakened at night due to shortness of
breath and also never experienced shortness of breath when lying down. She never had feet or
ankle swelling. She had hoarseness that started 2 months before admission. She was diagnosed
with congenital heart disease since 2.5 years old.
On physical examination, the patient was classified as underweight due to Body
Mass Index (WHO criteria) blood pressure 110/60 mmHg, pulse 90 times/minute, respiration
18 times/minute with 98% oxygen saturation on all four extremities. JVP was not increase. On
cardiac examination we found a loud S2 (P2), parasternal heave, continous murmur, grade
IV/VI, punctum maximum at 2nd spasium intercostal linea parasternalis sinistra, and pansistolic
murmur, grade III/VI, punctum maximum at LLSB. Based on the history and physical
examination, according to the Framingham criteria, the diagnosis of heart failure has been
fulfilled.
Her electrocardiogram examination showed sinus ryhtm with heart rate 94 bpm,
normoaxis, biventricular hypertrophy. Chest X-ray showed cardiomegaly LA and RV. In
laboratory test we found a normal blood test value.
Based on five fingers diagnostic, the patient has chronic heart failure functional class
nyha II, patent ductus arteriosus, and tricuspid regurgitation with possibility of pulmonary
hypertention. Further diagnostic tests must be done to identify the etiology and to determine the
most appropriate treatment.

30
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