paper / makalah cardio Trancatheter PDA Closure (Autosaved)

CASE REPORT

TRANSCATHETER PATENT DUCTUS ARTERIOSUS CLOSURE

Compiled by :

Rhea Tiara Dinata (110100029)

Supervisor:

dr. Muhammad Ali, Sp. A(K)

PEDIATRIC DEPARTMENT

HAJI ADAM MALIK GENERAL HOSPITAL

FACULTY OF MEDICINE

UNIVERSITY OF NORTH SUMATERA

MEDAN

2016

i

ACKNOWLEDGEMENTS

We are greatly indebted to the Almighty One for giving us blessing to

finish this case report, “Transcatheter PDA Closure”. This case report is a

requirement to complete the clinical assistance program in Pediatric Department

in Haji Adam Malik General Hospital, Faculty of Medicine, University of North

Sumatera.

We are also indebted to our supervisor and adviser, dr. Muhammad Ali,

Sp.A (K) for much spent time to give us guidances, comments, and suggestions.

We are grateful because without him, this case report wouldn’t have taken its

present shape.

This case report has gone through series of developments and corrections.

There were critical but constructive comments and relevants suggestions from the

reviewers. Hopefully the content will be useful for everyone in the future. Finally,

we thank you.

Medan, January 16th 2016

Presentator

CONTENTS

ACKNOWLEDGEMENTS........................................................................ i

CONTENTS.................................................................................................. ii

CHAPTER 1 INTRODUCTION................................................................ 1

1.1. Background................................................................................. 1

1.2. Objective..................................................................................... 2

CHAPTER 2 LITERATURE REVIEW.................................................... 2

2.1. Definition and Classification...................................................... 2

2.2. Epidemiology.............................................................................. 2

2.3. Etiologi........................................................................................ 3

ii

2.4. Pathophysiology.......................................................................... 4

2.5. Clinical Manifestasion................................................................ 7

2.6. Diagnosis..................................................................................... 11

2.7. Management................................................................................ 17

2.8. Complication............................................................................... 19

CHAPTER 3 CASE REPORT.................................................................... 21

CHAPTER 4 DISCUSSION....................................................................... 30

CHAPTER 5 SUMMARY........................................................................... 31

REFERENCES............................................................................................. 32

1

CHAPTER I

INTRODUCTION

1.1. BackgroundPatent Ductus Arteriosus is patent in all newborns at the time of delivery. It is

closed by 48 hours after birth in 100% of infants delivered at ≥40 weeks gestation

and by 72 hours after birth in 90% of infants delivered at ≥30 weeks gestation. A

persistently patent ductus arteriosus (PDA) has significant clinical consequences

in preterm neonates during the recovery period from respiratory distress

syndrome.1

The ductus arteriosus is an essential component of fetal circulation allowing

for communication between the pulmonary artery and the aorta. After birth, it

usually closes within 48 hours. A persistently patent ductus arteriosus (PDA) is

diagnosed when the ductus arteriosus fails to close after 72 hours. Patent ductus

arteriosus increases pulmonary blood flow and left atrial and ventricular

volumes, and produces a redistribution of systemic blood flow. Clinical

complications are dependent on the degree of left to right shunting through the

ductus. Hemodynamic symptoms from a PDA are present in 55–70% of infants

delivered below 1000g or prior to 28 weeks of gestation and may require either

medical or surgical intervention.2

Patent Ductus Arteriosus (PDA) is the most common cardiovascular

abnormality in preterm neonates with a reported incidence as high as 60% in

Extremely-Low-Birth-Weight (ELBW) newborns less than 28 weeks gestation.

While the Ductus Arteriosus (DA) is important for prenatal and immediate

postnatal circulation, its persistence beyond the transitional period is associated

with neonatal morbidity and mortality.3

1.2 Objective

The aim of this study is to explore more about the theoretical on Patent

Ductus Arteriosus (PDA). It is also to integrate the theory and application of

pediatric with PDA in children.

2

CHAPTER II

LITERATURE REVIEW

2.1. Definition

The arterial duct (ductus arteriosus) is a short blood vessel connecting the

two main arteries of the heart - the aorta and the pulmonary artery. Before a baby

is born the arterial duct allows blood to go around their lungs. After the baby is

born and the lungs fill with air, the arterial duct is no longer needed - it usually

closes by itself within the first week after birth. Sometimes the duct fails to close

by itself and remains open (patent). This is called patent ductus arteriosus or PDA

for short. It is sometimes called persistent arterial duct.4

Figure 1. Difference between normal heart and heart with PDA [14]

2.2. Incidence of PDA

According to the applicant, the prevalence of congenital heart disease in

Australia is about 1 in 100 and PDAs would represent 10% of the disease burden

(incidence 1 in 1000). On average in the United States, PDA occurs in about 8

out of every 1,000 premature babies, compared with 2 out of every 1,000 full-term

3

babies. Premature babies also are more vulnerable to the effects of PDA (National

Heart Lung and Blood Institute 2011). European statistics indicate an incidence of

1 in 2000 full term infants. A higher prevalence is found in low birth weight

premature babies15.

The incidence of PDA is inversely related to gestational age and birth

weight. A hemodynamically significant shunt due to PDA has been reported in

40% of infants less than 1000 grams and 20% of infants between 1000-1500

grams. Respiratory distress syndrome (RDS) in preterm neonates needing

ventilation and surfactant is an additional risk factor for PDA. Prophylactic use of

synthetic surfactant has been associated with an increased risk of PDA. Lack of

antenatal steroids, presence of sepsis, and liberal fluid therapy are other risk

factors for developing PDA.5

PDA is closed by 48 hours after birth in 100% of infants delivered at ≥40

weeks gestation and by 72 hours after birth in 90% of infants delivered at ≥30

weeks gestation.1

2.3. Etiology

The ductus arteriosus is a normal fetal artery connecting the main body

artery (aorta) and the main lung artery (pulmonary artery). The ductus allows

blood to detour away from the lungs before birth. Every baby is born with a

ductus arteriosus. After birth, the opening is no longer needed and it usually

narrows and closes within the first few days.

Sometimes, the ductus doesn’t close after birth. Failure of the ductus to

close is common in premature infants but rare in full-term babies. In most

children, the cause of PDA isn’t known. Some children can have other heart

defects along with the PDA.5

4

2.4. Pathophysiology

Initially, oxygen and endothelin are very strong vasoconstrictors and

prostaglandins E2 and I2 are strong vasodilators of the DA. Lower oxygen

concentrations in utero and high circulating PgE2 and PgI2 levels help in keeping

the ductus patent. Sudden elevation in circulating oxygen tension and fall in

prostaglandin levels soon after delivery results in strong vasoconstriction and

functional closure of the DA soon after delivery. It is believed that this action of

oxygen is mediated via the formation of the endothelin molecule. This functional

closure is followed by anatomical closure in the next 1-3 months.6

In utero, low fetal systemic arterial oxygen tension (PaO2) and elevated

circulating PGs (in addition to PGs made within the ductus wall itself) play a

significant role in keeping the lumen of the ductus arteriosus patent. This is

necessary for fetal circulation and survival. After a full-term birth, the ductus

closes within 24– 48 hours of delivery6.

The closure of the ductus arteriosus in full-term infants occurs in two steps.

Initially, within the first few hours after birth, increased arterial PaO2 and

decreased circulating PGs allow the smooth muscle media of the ductus to

constrict. As a result of the constriction, the inner muscle wall of the ductus

arteriosus develops profound ischemic hypoxia which leads to the formation of

vascular endothelial growth factor, transforming growth factor- beta, and other

inflammatory mediators and growth factors that transform the ductus into a non-

contractile ligament6.

Conversely, in preterm infants, the ductus often fails to constrict in the days

following birth. Even in those preterm infants who achieve ductus constriction,

the ductus frequently fails to develop the level of profound hypoxic ischemia

needed to cause remodeling of the artery. As a result, many premature infants with

a closed ductus can reopen their ductus and develop clinical symptoms related to

the PDA.2

5

2.5. Clinical Manifestation

Despite the increased left ventricular output, there is significant

redistribution of blood flow to major organ systems, with the presence of ductal

steal seen in PDA due to left to right shunt. There is flow across the ductus all

throughout the cardiac cycle, the direction of which depends on the difference

between systemic and pulmonary pressures. Usually there is shunting from

systemic to pulmonary circulation called ductal steal, the maximum of which

occurs at the beginning of the cardiac systole when the pressure gradient is

maximum. Contrary to the belief that ductal run off occurs only in diastole, it is

present all throughout the cardiac cycle. However, its effect on systemic

circulation is best demonstrated on echocardiogram during diastole, as a

retrograde flow in the descending aorta, or other systemic vessels on Doppler,

instead of the normal low velocity forward flow. This steal phenomenon may

lead to systemic hypoperfusion, despite increased cardiac output. Hence

hemodynamically significant PDA has negative effect on cerebral circulation and

oxygenation, which may lead to injury to the immature brain.7

Patent ductus arteriosus is associated with several neonatal morbidities.

Both animal and human studies have demonstrated alterations in blood pressure

with a drop in the mean, as well as, systolic and diastolic pressures. Left

ventricular output can increase by as much as 100%. In spite of this increased

cardiac output, redistribution in blood flow results in increased flow to the lungs

and decreased flow to the pressure passive organs like the intestines, skin, muscle,

and kidneys. This redistribution may result in metabolic acidosis, necrotizing

enterocolitis (NEC), and pulmonary edema/hemorrhage.

Timing is important in evaluating when different morbidities will present.

Intracranial hemorrhage usually occurs within the first 3 days after birth.

Similarly, marked pulmonary edema and pulmonary hemorrhage secondary to

fluid shifts and ductus patency occurs within 2–3 days after birth. Necrotizing

enterocolitis usually presents 5 days after birth.2

6

Patients can present at any age. Three-week to 6-week-old infants can

present with tachypnoea, diaphoresis, inability or difficulty with feeding, and

weight loss or no weight gain. A PDA with a moderate-to-large left-to-right shunt

may be associated with a hoarse cry, cough, lower respiratory tract infections,

atelectasis (collapse of the lung tissue), or pneumonia. With large defects, the

patient may have a history of feeding difficulties and poor growth during infancy,

described as failure to thrive. However, frank symptoms of congestive heart

failure are rare.

The typical child with a PDA is asymptomatic. At times, the patient may

report decreased exercise tolerance or pulmonary congestion in conjunction with a

murmur. Adults with a PDA that has gone undiagnosed may present with signs

and symptoms of heart failure, atrial arrhythmia, or even differential cyanosis

limited to the lower extremities

Left untreated, patients with a large PDA are at risk to develop Eisenmenger

Syndrome, in which the pulmonary vascular resistance (PVR) can exceed

systemic vascular resistance, and the usual left-to-right shunting reverses to a

right-to-left direction. At this stage, the PVR is irreversible, closure of the PDA is

contraindicated, and lung transplantation may be the only hope for long-term

survival16.

7

Table 1. Classification of PDAs based on size

2.6. Diagnosis

The literature on diagnosis of PDA is confused by poorly defined

terminology with terms such as ‘symptomatic’, ‘clinically apparent’ and

‘haemodynamically significant’ often used interchangeably. There are no standard

definitions but below would be our interpretation of the meaning of these terms:

Clinically apparent PDA refers to presence of physical signs consistent

with a PDA such as murmur, active praecordium or full pulses, preferably

confirmed by cardiac ultrasound. Symptomatic PDA implies that the PDA is

having a clinical impact on the baby’s condition. This is really hard to define as

most ‘clinical symptoms’ of a PDA are non-specific and may or may not relate to

a concurrent PDA. Probably the respiratory and circulatory effects of a PDA are

easiest to define and may include persistent ventilator dependence, deteriorating

respiratory status, increasing recurrent apnoea, pulmonary haemorrhage and

hypotension. Probably the most specific of these would be pulmonary

haemorrhage8 particularly if it occurs within the first week of life and is associated

with a respiratory deterioration.

8

Haemodynamically significant PD’ refers to ultrasound findings that are

consistent with a large shunt volume. Again there are no standard definitions but

this will often be based on ductal diameter together with a range of indirect

markers of shunt size as discussed below.

2.6.1. Clinical diagnosis.

Reliance on clinical signs, such as an active praecordium, full pulses or a

systolic murmur, will eventually make the diagnosis of a PDA but only after the

left to right shunt through the duct has been significant for some days. Blinded

comparison of these clinical signs to cardiac ultrasound criteria of ductal

haemodynamic significance (discussed below)have shown it is normal for

haemodynamically significant ducts to be clinically silent for the first 2 to 3 days

of life. From day 4 onwards, physical signs, particularly the murmur, become

more accurate but some inaccuracy persists up to day 7 of life. Wide pulse

pressure also does not accurately diagnose patent ducts in the first week of life.

Thus a ‘haemodynamically significant” PDA and/or ‘symptomatic’ PDA

may not be ‘clinically apparent’ particularly in the first few postnatal days.

Accurate and early diagnosis of significant ductal shunting depends on cardiac

ultrasound.

Figure 2. Abnormal Heart Sounds

9

Continuous precordial murmur is a typical continuous murmur that can be

heard at the patients with patent ductus arteriosus, which is the most frequently

described in literature. It was first described in 1847 in "London Medical Gazette"

as "murmur that accompanying first sound.. extended to the second sound, so

there is no interruption of the murmur before the second sound had already

started". Later, in 1900, George Gibson presented a more precise description. "It

persists trough the second sound and dies away gradually during the long pause.

The murmur is rough and trembling. It begins softly and increases in intensity so

as to reach its acme just about, or immediately after, the incidence of the second

sound, and from that point gradually wanes until its termination17.

Continuous murmurs of patent ductus arteriosus consists of two

components: a crescendo systolic one and a decrescendo diastolic one, with a peak

of intensity around second sound. It is best heard at second left intercostals space

or immediately left infraclavicular. It is continuous because the ductus arteriosus

provides a permanent communication between the systemic circulation, with high

pressure, and the pulmonary circulation, where pressure level is much lower.

About half of patent ductus arteriosus murmurs in children are not truly

continuous and many are only systolic. This is because with the pulmonary

vasoconstriction secondary to a large shunt, there is often a moderate degree of

pulmonary hypertension, which decreases the aortic–pulmonary artery gradient

more in diastole than in systole. Those with large ductus have been described by

Eddy sounds, clicks or crackles at the end of systole and at the beginning of the

diastole. With the equilibration of aortic and pulmonary artery pressure, systolic

flow across the shunt diminishes and finally disappears, leaving the ductus silent

(Eisenmenger patent ductus arteriosus). Sometimes, these patients can be

recognized by the existence of cyanosis of the lower limbs and left upper limb

instead the face, lips and upper right limb are colored as normal (with pulmonary

hypertension, unsaturated blood flows through a ductus from the pulmonary artery

to the aorta; the ductus often joins the pulmonary artery to the aorta just beyond

the left subclavian artery. Unsaturated pulmonary artery blood will then pass

beyond the left subclavian artery, and both hands will be less clubbed and

10

cyanotic than the feet. If, however the ductus is at the junction of the aorta and the

left subclavian artery, the left hand will be as cyanotic and clubbed as the feet)17.

2.6.2. Ultrasound diagnosis

Accurate diagnosis relies on cardiac ultrasound.

Patency can be confirmed by diastolic turbulence on Doppler in the pulmonary

artery. The picture below shows the diastolic turbulence seen with a patent

duct. This is a reasonably accurate method for diagnosing ductal patency with

left to right shunting but tells you little about the haemodynamic significance.

Shunt direction is demonstrated with pulsed wave and colour Doppler. There

are broadly three direction patterns which are shown below. Pure left to right

(A), bidirectional (B) and right to left (C). Most babies even in the early hours

after birth have left to right or bidirectional shunt with a dominant left to right

component. Predominantly right to left shunting is unusual.

2.6.3 Haemodynamic significance

In studies done within this group, the best markers of haemodynamic

significance were colour Doppler diameter of the duct and absent or retrograde

diastolic flow in the post-ductal aorta. The pictures below contrast three preterm

ductal ultrasound assessments. (A) is closed with no ductal shunt apparent on

colour Doppler. (B) is well constricted at less than 1.0 mm diameter. Constriction

has failed in (C) which is over 2.0 mm in diameter and has a large left to right

shunt draining blood from the systemic circulation. These studies were done

mainly within the first week of life and shunt velocities will often rise with age, so

after 7 days it will be important to take into account the velocity of the left to right

shunt.

Another useful marker is the velocity of flow in the left pulmonary artery

(LPA).11 This reflects the increased flow volumes into the pulmonary circulation.

A mean LPA velocity of more than 0.42 m/sec and/or an end-diastolic LPA

velocity of more than 0.2 m/sec predicts haemodynamic significance

11

(approximately a Qp:Qs> 2:1) with greater than 90% specificity and sensitivity.

We use the colour Doppler diameter of the ductus at its narrowest point (usually

the pulmonary end) as the primary determinant of haemodynamic significance but

assessment should include other secondary determinants. This haemodynamic

impact can be broadly categorised based primarily on ductal diameter:

• Large PDA: Minimum Ductal colour Doppler diameter greater that 2.0

mm with a predominantly left to right shunt. With a large PDA there will

usually be retrograde diastolic flow in the post-ductal descending aorta and

mean diastolic velocity in the left pulmonary artery of more than 0.43

m/sec or end-diastolic velocity more than 0.2 m/sec but they should be

checked as back up measures to confirm the accuracy of the diameter

measurement.

• Moderate PDA: Minimum Ductal colour Doppler diameter between 1.5

and 2.0 mm with a predominantly left to right shunt. With a moderate

PDA, the measures in the descending aorta and left pulmonary artery will

be more variable but should be assessed.

• Small PDA: Minimum Ductal colour Doppler diameter less than 1.5 mm

with a predominantly left to right shunt. With a small PDA, the diastolic

flow in the descending aorta will usually be antegrade and left pulmonary

artery velocities will be below the thresholds defined above.

• Closed PDA: There is no shunt within the ductus that is apparent on

colour Doppler.

2.7. Treatment

Spontaneous closure of patent ducutus arteriosus (PDA) is common. If

significant respiratory distress or impaired systemic oxygen delivery is present,

therapy usually prudent11. Treatment options include conservative medical

management, pharmacological therapy or surgical ligation. Conservative medical

management involves fluid restriction, watchful waiting and ventilator

support.This approach is associated with a high failure rate, especially in low-

birth-weight infants. Pharmacological treatment with one of the two available

12

cyclo-oxygenase (COX) inhibitors is effective in 70 to 80% of ELBW infants.

Surgical ligation involves thoracotomy and is associated with such significant

morbidities as pneumothorax, chylothorax, infection, laryngeal nerve paralysis,

respiratory compromise, blood pressure fluctuations, BPD, retinopathy of

prematurity andinjury have been reported with ductal ligation.

2.7.1 Pharmacological therapy

Inhibitors of the cyclo-oxygenase component of prostaglandin-H2

synthetase, namely indomethacin and ibuprofen, are commonly used in the

treatment of hemodynamically significant patent ductus arteriosus. These drugs

are associated with serious adverse events, including gastrointestinal perforation,

renal failure and bleeding. The role of paracetamol, an inhibitor of the peroxidase

component of prostaglandin-H2 synthetase, has been proposed for the treatment

of

patent ductus arteriosus18.

There is still ongoing debate regarding when to treat a PDA in premature

infants who are born before 28 weeks of gestation. Physicians for decades have

weighed the pros and cons of early prophylactic therapy versus late treatment for

infants who are most at risk for developing complications from a PDA. Clinical

experience reveals that if a delayed treatment approach is used (waiting until

hemodynamic symptoms of a PDA appear), 55–70% of infants born before 28

weeks of gestation and weighing less than 1000 g at birth ultimately will be

treated for a PDA during their hospitalization. The evidence suggests that preterm

infants require a tighter degree of constriction than full-term infants to develop the

anatomic changes that lead to permanent ductus closure. Even minimal degrees of

ductus patency after indomethacin treatment will prevent remodeling and lead to

subsequent clinical reopening. Clinical and laboratory experience also indicates

that PG inhibitors given as prophylaxis, early after birth, are more effective in

producing tight ductus constriction than waiting several days for symptoms to

develop. The delay of several days may necessitate the use of other

13

vasoconstrictive agents, in addition to indomethacin, to produce the same degree

of ductus closure. Preterm infants who are born before 28 weeks of gestation and

who receive prophylaxis with indomethacin by 6–15h after birth consistently have

a lower incidence of serious pulmonary hemorrhage, intracranial hemorrhage

(grade III/IV), and need for ductus ligation.2

A platelet count of at least 50 000 is necessary prior to the first dose of

indomethacin. PT and APTT should be obtained if there is any concern about

bleeding. Serum creatinine and platelet count should be checked prior to second

and third doses. Because of decreased gastrointestinal blood flow secondary to

indomethacin, the infant is restricted to no oral intake during treatment and for 48

hours after treatment is completed2. Urine output should be monitored since the

infant may experience a transient decrease in output requiring alterations in fluid

administration. Indomethacin may be restarted when urine output is greater than 1

ml/kg/h.2

Treatment Indomethacin based on age:

1) Gestation ≥28 weeks at birth: These infants are usually treated only when

a hemodynamically significant PDA is present. This practice is aimed to

avoid unnecessary treatment. A PDA is considered hemodynamically

significant if, in addition to a murmur, two or more of the following signs

are present: increased pulse volume or widened pulse pressure

hyperactive precordium, or increased pulmonary vascular markings on

chest radiograph

An untreated hemodynamically significant PDA will prolong the need for

oxygen therapy and delay the establishment of feedings. An echocardiogram

should be obtained to rule out structural congenital heart disease. Indomethacin

Dosage (IV):

• Birthweight >1,250 g: Give three doses of 0.2 mg/kg; give the 2nd dose

12 h after the first, and the 3rd dose 24 h after the 2nd.

• Birth weight 1,000-1,250 g: 1st dose is 0.2 mg/kg; 12 h later, give 0.1

mg/kg for 2nd dose; 24 h after the 2nd dose, give 0.1 mg/kg.

14

2) Gestation <28 weeks at birth – (Prophylactic Indomethacin, UCSF

approach): Seventy percent of infants <28 weeks gestation will require

therapy for a PDA at some point during their hospitalization. Indomethacin

is not as effective in this group and surgery is frequently necessary. The

earlier infants are treated with indomethacin, the more effective it is in

producing permanent closure. At UCSF, all infants <28 weeks gestation are

treated prophylactically with indomethacin by 12-15 h after birth. An

echocardiogram and serum creatinine are not usually obtained prior to

giving the 1st dose. Platelet count >100,000 should be demonstrated prior to

the first dose. The 1st dose should be delayed if there is any concern about a

bleeding diathesis or coagulopathy. PT and PTT should be measured if there

is concern about a coagulopathy. Prophylactic Indomethacin Dosage (IV) :

Three initial doses:

• 1st = 0.2 mg/kg,

• 2nd = 0.1 mg/kg (24 h after the first dose),

• 3rd = 0.1 mg/kg (24 h after the 2nd dose). Serum creatinine and platelet

count should be checked before 2nd and 3rd doses.

Just prior to the 3rd indomethacin dose, obtain an echocardiogram. If there

is echocardiographic evidence of patency of the ductus (even if there are no

clinical signs), give 4th, 5th, and 6th doses of indomethacin (0.1 mg/kg at 24 h

intervals). Repeat the echocardiogram after the 6th dose.

Contraindications to Indomethacin:

- Active bleeding: GI and other (Note that presence of an ICH is not a

contraindication)

- Active or suspected Necrotizing Enterocolitis (NEC)

- Creatinine ≥2.0 mg/dL

- Urine output <1 mL/kg/h (indomethacin may be restarted when urine

output>1mL/kg/h)

15

- Platelet count <50,000 (consider platelet transfusion prior to indomethacin)

- Active (and untreated) infection

- Suspected Congenital Heart Disease

- Known gastrointestinal or renal anomaly

Neonates with hs-PDA were treated with paracetamol in the presence of

contraindications to ibuprofen or indomethacin (i.e. urine output < 1 ml/kg/h,

intraventricular hemorrhage, platelet count < 60,000/mm3, hyperbilirubinemia

requiring exchange transfusion, signs of feeding intolerance or gastrointestinal

bleeding). Paracetamol was given at doses ranging from 7.5 to 15 mg/kg every 4–

6 hours, with a maximum daily dose of 60 mg/Kg.

2.7.2 Catheterization

The use of the percutaneous route to close the PDA is becoming more

common. Transcatheter occlusion is an effective alternative to surgical

intervention and is becoming the treatment of choice for more cases of PDA in

children and adults12,13.

Most patient with an isolated PDA can have successful treatment by

catheterization after the first few month of life. After the first birthday, the most

common treatment for a PDA is occlucion at cardiac catheterization. In fact, as

catheterization techniques advance, the ability to close defects in smaller infants

has also been reported with high level success. Over the last decades, many

techniques and devices have been used for patent ductus arteriosus (PDA)

occlusion, although definitive closure rates do nor approach those of surgery.

Contraindications to catheter-based closure involve the sixe of patient12,13.

a. Gianturco spring occluding coils

Introduce in 1992, Gianturico spring occluding coils have been the most

common device used in PDA occlusion for many years. The coils are delivered

to the PDA via venous or arterial systems : 1-5 coils are placed in the ductus. In

experience hands with proper patient selection, this has become a procedure

associated with high success and low morbidity. This method has been reported

16

to be 75-100% effective but is limited to ductus that are only 4-5 mm in

diameter. Coil occlusion is best suited to close a patet ductus arteriosus (PDA)

with a minimal internal diameter of less than 2,5 mm. Fue et al showed that

very high closure rates could be obtained in ducts less than 3 mm using coils,

but that success significantly dropped when the ducts exceeded 3 mm12,13.

b. Amplatzer ducto occlude (ADO)

More recently, the amplazer device has expanded the ability to close patet

ductus arteriosus (PDA) than spring oclluding coils. The major disanvantages

of the desgn is that the aortic part of the device can protrude into the decending

aorta and partly obstruct the lumen, especially in infants. Howefer, the

amplatzer duct occlude II (ADO), a nitinol flexible mesh with a symmetrical

design to provide high conformability, has been approved in Europe for

treatment of all types of patet ductus arteriosus (PDA) 12,13.

Figure 2. Occluder for ASO, ADO, AmVSDO, AVPII, AVPIII.

17

Figure 3. The Amplatzer Duct Occluder II

Table 3. Amplatzer Duct Occluder II and delivery system sizes

Tabel 4. Amplatzer Duct Occluder II sizing chart

18

Figure 4. Contraindications to participate Amplatzer Duct Occluder II in

clinical study

c. Rashkind ductus occlusion device

This kind of device consists of 2-umbrela system delivered to the ductus in

either the transvenous pathway or transarterial pathway. This therapy has a

reported occlusion rate of 83%. Although used internationally, it is not

approved to use in the United States12,13.

3.7.3 Surgical Closure

Closure of a PDA in adults or older children is often a surgical challenge.

Its fragile wall, as the result of calcification and atherosclerotic lesions, together

with the frequent coexistence of pulmonary hyperten- sion, make conventional

ligation and division of the PDA an extremely hazardous procedure, sometimes

impossible.6,9 In these cases, the use of cardiopulmo- nary bypass and ECC (or a

bypass shunt) is essential. Of course, apart from the known consequences of such

support (systemic inflammatory reaction, etc.) and its financial cost, it entails the

full heparinisation of the patient with an unstable surgical field (PDA wall). We

consider, however, that its use in specific cases of PDA is essential in order to

provide the pa- tient with the greatest possible degree of safety.

Various techniques have been invented for clos- ing the “difficult” PDA:

approach via pulmonary arteriotomy or aortotomy, followed by closure of the

pulmonary or aortic end of the ductus, respec- tively. Closure may be achieved

simply, with sutures reinforced by Teflon felts.10 However, closure with a

synthetic patch is safer to prevent a possible recanali- sation of a closed PDA.

19

Surgical should be considered in the following situations:

- Failure of indomethacin therapy

-Hemodynamically significant PDA and presence of contraindication(s)

toindomethacin

- Presence of PDA and NEC. In this circumstance, operative closure of the PDA is

almost always necessary before the NEC will resolve and may be required as an

emergency procedure.1

Figure 5.Closure of a patent ductus arteriosus (PDA) using extracorporeal

circulation (ECC). A. Under ECC with reduced flow, arteri- otomy is

performed at the pulmonary bifurcation. B. The PDA is pressed with a

finger from outside, reducing flow. C. Single sutures, rein- forced with

pledgets, are placed at the pulmonary end of the PDA. D. Closure of the

PDA is completed with a Dacron patch16.

2.8. Complication

• Patent ductus arteriosus, is a major morbidity in preterm infants, with

incidence inversely related to gestation and birth weight. Antenatal

steroids and judicious fluid therapy seems to be protective against PDA,

whereas RDS requiring mechanical ventilation, delay spontaneous closure

of the ductus arteriosus.

20

• Bedside functional echocardiography is a valuable tool for early diagnosis,

assessment of hemodynamic effects and response to therapy. It may

emerge as a valuable tool in all tertiary level NICUs.

• Ibuprofen with its superior safety profile, especially reduced risk of NEC

and comparable efficacy to indomethacin, is currently the drug of choice.

Oral ibuprofen is emerging as safe and effective alternative to intravenous

indomethacin in treatment of PDA.

• There is increasing advocacy for reserving pharmacological treatment of

PDA for compelling indications like refractory hypotension or congestive

heart failure as early medical treatment is not associated with proven long

term benefits and in nearly one half of the infants, PDA spontaneously

closes.9

The variation in management of PDA due to conflicting evidence that it truly

impacts the long-term outcome brings a sense of urgency to conduct robust trials

that could identify which group of preterm infants benefit most from early

treatment of PDA and which group can be managed expectantly with watchful

waiting or delayed treatment. Lack of benefit of PDA treatment on major neonatal

outcomes may relate to the lack of standardization of diagnosis of hemodynamic

significance, variability in timing of intervention and diagnosis, difference in

therapeutic regime, and failure of consideration of co-morbidities. A scoring

system that combines echocardiographic, clinical and lab criteria of PDA that

correlate with adverse outcome and is subsequently validated in a well designed

trial will go a long way in standardizing care and improving outcomes in this

vulnerable population. Until more evidence comes up, the risk of treatment has to

be carefully weighed against benefit offered.10

21

CHAPTER 3

CASE REPORT

Name : RYY

Age : 4 years 7 month 23 day

Sex : Male

Date of Admission : 15th December 2015

Chief Complaint : history of shortness of breath

History of disease :

It is experienced by the patient since 3 weeks ago and has been worsening

since the 1 week before hospital admission.

The patient has been tire easily, especially during exercise

(running,jumping), since 2 weeks ago. The shortness of breath is not

associated with position, weather and temperature.

Experience of fever, cough, flu, nausea and vomitting were denied.

Defecation and urination were normal, no pain, difficulty, or bleeding

About 3 weeks ago, the child was admitted to the cardiology department

of Bunda Thamrin Hospital and was examined with echocardiography.

The child was diagnosed with PDA.

Patient was referred to H. Adam Malik General Hospital to do trancatheter

PDA closure.

History of medication : unclear

History of family : none

History of parent’s medication : none

History of pregnancy : The mother was 25 years old when

pregnant. During her pregnancy, she

frequently visited the doctor. RYY was

born with 37 weeks of pregnancy. History

22

of hypertension in pregnancy and diabetes

in pregnancy was denied.

History of birth : RYY was born with 37 weeks of

pregnancy and was helped by doctor by

normal method. BW when born is

3400g. BL is unclear. Crying

immediately.

History of feeding : The patient got exclusive breast feeding

for 6 month 10-12 times daily. Patient

get formula milk when he was 6 month

4-5 times daily.

History of immunization : BCG : soon after birth

Hepatitis : 0 – 1 - 6 mo

Polio : 0 – 2 – 4 – 6 mo

DPT : 2 – 4 – 6 mo

Campak : 9 mo

History of growth and development : The patient can hold his head when he

was 3 months, face down when he was

5 months, sit when he was 8 months,

crawling when he was 10 months, stood

when he was 12 months, walk when he

was 18 months, and talking when he

was 15 months.

Physical Examination :

Present status :

Level of consciousness: Alert, Compos mentis. Blood pressure : 90/50 mmHg,

Respiratory rate: 24 x/i, regular. Pulse : 108 x/i reguler, Body temperature:

23

36,8°C, Body weight : 15 kg, Body height : 105 cm. BW/BH : -2 < z-score < +2,

anemic (-), icteric (-), dyspnea (-), cyanotic (-), edema (-).

Localized status :

Head : Eyes: Light reflex: (+/+), isochoric pupil, anemic inferior

palpebral conjunctiva (-/-). Ear, nose and mouth are normal.

Neck : Lymph node enlargement (-), TVJ : R+2 cm H2O.

Thorax : Symmetrical fusiform, chest retraction (-).

HR: 108 x/i, regular, Continuous murmur (+) grade III/ ICS II-

III LMCS

RR: 24 x/i regular, rales (-/-), breath sound : vesicular,

additional sound (-).

Abdomen : Soepel, normal peristaltic, liver and spleen was not palpable.

Ascites (-). Tumor (-).

Extremities :Pulse 108 x/i, regular, p/v adequate, warm acral, CRT < 3”.

Blood Pressure : 90/50 mmHg.

Anogenitalia : Male, normal.

Laboratory finding:

Complete blood analysis (December 17th 2015)

Test Result Unit References

Hemoglobin 11,40 g% 11,3-14,1

Erythrocyte 4,04 106/mm3 4,40-4,48

Leucocyte 11,85 103/mm3 4,5-13,5

Thrombocyte 315 103/mm3 150-450

Hematocrite 33,30 % 37-41

Eosinophil 3,80 % 1-6

Basophil 0,700 % 0-1

Neuthrophil 46,10 % 37-80

Lymphocyte 37,20 % 1,7-5,1

Monocyte 12,20 % 2-8

24

MCV 82,40 Fl 81-95

MCH 28,20 Pg 25-29

MCHC 13,00 g% 29-31

Haemostatic Factor

Bleeding time 3’ Min <5

PT 13,7 Sec 13,7

APTT 30,3 Sec 35,0

TT 15,7 sec 17,6

LFT

Total billirubin 0,22 mg/dl <1

Direct billirubin 0,09 mg/dl 0-0,2

ALP 128 U/L <269

AST/SGOT 34 U/L <38

ALT/SGPT 15 U/L <41

RFT

Ureum 10,40 mg/dl <50

Creatinine 0,31 mg/dl 0,31-0,47

Uric acid 2,6 mg/dl <7,0

Electrolite

Ca 9,0 mg/dl 8,4-10,4

Na 137 mEq/L 135-155

K 5,0 mEq/L 3,6-5,5

Cl 105 V 96-106

KGDar 81,90 mg/dl <200

Immunoserology

HBsAg Negative negative

25

Anti HBs Negative IU/L negative

Anti HAV IgM Negative negative

Anti HAV total 14,97 negative

Anti HCV Negative negative

Anti HIV (RapidI) non reactive non reactive

Thorax Imaging

Interpretation : CTR 61%, apex upward, Aorta is within normal range

Superior mediastinus is not wide, Trachea is in middle.

Both of hillus are not hiperdense,

Bone and soft tissue are good, Nodule (-) infiltrate (-)

Bronkovascular pattern is within normal range

Costofrenicussinus and diafragma are within normal range

Conclusion : Cardiomegaly, suspect CHD

Electrocardiography

26

Interpretation : Sinus tachycardia, LVH

Echocardiography (Dec 15th 2015)

Result : Situs solitus, AV-VA concordance

Normal pulmonary venous drainage

Dilated LA, LV, MPA, Mild AR, Mild TR

Intact IAS and IVS

Large PDA ( 7,7-8,6 mm, L-R shunt)

Left aortic arch, no coarctation

Good ventricular fungtion (EF 68%, FS 34%)

Conclusion : Large PDA/ Left Enlargement/Mild AR/Mild TR

Advice : Trancatheter PDA closure

Working diagnosis : Large PDA + LVH

Therapy : - IVFD Ringer Lactat 10gtt/i

27

- Inj. Ceftriaxone 50 mg/kgBB/iv

- Fasting 6-8 hours before transchateter operation.

Further Plan : - Transcatheter PDA Closure

- Consult to POSYANSUS

- Consut to ENT

- Consult to Dentist

- Consult to Anesthesiology

FOLLOW UP

December, 22nd 2015 10.00 WIB

Consult Answer : (ANASTHESIA DEPARTEMENT) 21/12/15

S: Compos Mentis

O : RR : 24 x/m, vesikuler

HR : 100 x/m , desah (+)

A : Large PDA

O : ACC anasthesia procedure

1. Anasthesia letter of permission2. Fasting 6 – 8 hours berfore procedure3. Put IVFD on , make sure the drip is clear4. Personal hygine5. Praying

Consult Answer : ( POSYANSUS ) 21/12/2015

Pre test counseling + HIV test

Consult Answer : ( ENT DEPARTMENT ) 21/12/15

S : Earache ( - ) earorhea ( - ) hearing and balance disturbance ( - )

Clogged nose ( - ) Smelling problem ( - ) Odynophagia ( - )

Shortness of breath ( - )

O :Within normal range

A :Withinnormal range

P :ENT division found no problem

28

S ADO preparation

O Sensorium : CM, Temp: 36,6o C

Head : Eyes : Light reflexes (+/+), pupile were isochoric, anemic

inferior palpebra conjunctiva (-/-). Ear, nose and mouth are

normal.

Thorax : Symmetrical fusiform. Chest retraction (-). Heart rate 100

x/i, regular, Continuous Murmur (+) ICS II-III LMCS.

Respiratory Rate 24x/i, regular, ronchi (-/-).

Abdomen : Soepel. Peristaltic (+) normal. Liver and spleen unpalpable.

Extremities : Pulse 100 x/i, regular, adequate volume and pressure, warm

CRT < 3”.

A Large Patent ductus arteriosus

P - IVFD Ringer Lactat 50 mg/kgBB 10gtt/i (micro)

- Inj. Ceftriaxone 50 mg/kgBB/iv 1 hour befor procedure

- Fasting 6-8 hours before transchateter procedure

- Avoid substacting blood from the inguinal area

- Pray

December, 22nd 2015 14.00 WIB

S Post ADO

O Conciousness : CM, Temp: 36,8o C

Head : Eyes : Light reflexes (+/+), pupile were isochoric, anemic

inferior palpebra conjunctiva (-/-). Ear, nose and mouth are

normal.

Thorax : Symmetrical fusiform. Chest retraction (-). Heart rate 102

x/i, regular, Continuous Murmur (-).

Respiratory Rate 20x/i, regular, ronchi (-/-).

Abdomen : Soepel. Peristaltic (+) normal. Liver and spleen unpalpable.

Extremities : Pulse 102 x/i, regular, adequate volume and pressure, warm

CRT < 3”.

A Post Transcatheter PDA Closure

P - IVFD Ringer Lactat 10gtt/i

29

- Diet : regular meals

- Patient is planned to be discharged after stabilisation

- Patient is planned to re-echocardiography

CHAPTER IV

DISCUSSION

30

THEORY CASE

Epidemiology

- PDA is closed by 48 hours after birth

in 100% of infants delivered at ≥40

weeks gestation and by 72 hours after

birth in 90% of infants delivered at

≥30 weeks gestation.

Epidemiology

- The pregnancy was 37 weeks long.

Clinical Manifestations

- Reliance on clinical signs, such as an

active praecordium, full pulses or a

systolic murmur, will eventually make

the diagnosis of a PDA

Clinical Manifestation

-Continous Murmur was found in this

patient

Diagnosis

- Accurate diagnosis of PDA relies on

cardiac ultrasound.

Diagnosis

- The patient was diagnosed with PDA

through Echocardiography

Treatment

- Pharmacotherapy

- Catheterization

- Surgical

Treatment

- This patient was refer to Adam Malik

Hospital to do trancatheter PDA

closure

CHAPTER V

SUMMARY

31

RYY, male, 4 years 7 month 23 day old, came to Haji Adam Malik

General Hospital on December 15th2015 with chief complaint of history of

Dyspnoe. Based on anamnesis, physical examination, and laboratory assessment

dan echocardiography, he was diagnosed with PDA. In physical examination

heard continuous murmur in LMCS ICS II-III and in echocardiography found a

large PDA. He was treated with transcatheter closure of PDA. After the ADO,

there is no simptomp and no murmur anymore. The patient was discharged from

hospital after 6 hours of maintenance in hospital and planned to do

echocardiography post ADO in Bunda Thamrin hospital the day after.

REFERENCES

1. Tooley, W.H, et al. Intensive Care Nursery House Staff Manual. University

32

of California. 2004.

2. Clyman,RI, De-Santis, ER.H. Patent Ductus Arteriosus: Pathophysiology and Management. 2006. www.nature.com/jp

3. Sinha, B. Controversies in Management of Patent Ductus Arterious in the Preterm Infant. 2013. JPRM.

4. Understanding Your Child’s Heart: Patent Ductus Arteriosus. British Heart Foundation. 2012.

5. Schneider, DJ. Patent Ductus Arteriosus (PDA).American Heart Association. 2009.

6. Patent Ductus Arteriosus in Preterm Neonates. AIIMS- NICU protocols 2007.

7. Deorari,A, Sasi. Patent Ductus Arteriosus in Preterm Infants.Arun 2011. Indian Pediatrics.

8. Evans,N. Patent Ductus Arteriosus.. 2013

9. Sekar.KC, Corff, KE. Treatment of patent ductus arteriosus: indomethacin or ibuprofen?. 2008.

10. Noori,S. Patent Ductus Arteriosus in The Preterm Infant: to treat or not to treat?. 2010.

11. Aranda JV,Clyman R. Cox B. Randomized, double blind, placebo-cotrolled trial on intravenous ibuprofen L lysine for the early closure of nonsymptomatic patent ductus arteriosus within 72hours of birth in extremely low birth weight infants.Americal Journal of Perinatology. 2009 ; 26 (3) : 235-245

12. Rao PS,Sideris, et al. Transcatheter occlusion of patent ductus arteriousus with adjustable buttoned device : initial clinical experience. Circulation. 2012:1119-26

13. Rashkind WJ, Mullins, et al. Non-Surgical closure of PDA. Clinical application of Rahkind PDA occlude system. Circulation. 2007;75:583-92

14. Heart Disease An introduction to problems that can occur in the heart Partnership for Environmental Education and Rural Health Texas A&M University. http://peer.tamu.edu DVM (accessed dec 23th 2015)

16. Kalavrouziotis,G, et al. Closure of a Large Patent Ductus Arteriosus in Children and Adults with Pulmonary Hypertension. Hellenic J Cardiol 2010; 51: 15-18

33

17. Ginghină C, et al. Continuous murmur-the auscultatory expression of a variety

of pathological conditions. Bucharest. Journal of Medicine and Life Vol. 5,

Issue 1, January‐March 2012, pp.39‐46

18. Terrin,G, Conte,F, et al. 2014. Efficacy of paracetamol for the treatment of

patent ductus arteriosus in preterm neonates. Biomed Central. pp.1