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Congenital heart Disease for the Adult CardiologistPatent Ductus Arteriosus
Douglas J. Schneider, and John W. Moore
Journal ReadingSeptember 2014
Bahagian KardiovaskularFakultas Kedokteran
Universitas Hasanuddin
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The patent ductus arteriosus (PDA) is avascular structure that connects the
proximal descending aorta to the roof ofthe main pulmonary artery near theorigin of the left branch pulmonaryartery.
Essential fetal structure normally closesspontaneously after birth.
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After the first few weeks of life, persistenceof ductal patency is abnormal.
The physiological impact and clinicalsignificance of the PDA depend largely onits size and the underlying cardiovascularstatus of the patient.
The PDA may be silent (not evidentclinically but diagnosed incidentally byechocardiography done for a different
reason), small, moderate, or large
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Normal and essential fetal structure
Abnormal if it remains patent after the
neonatal period. This transformation is complete by 8
weeks of fetal life. (Figure 1)
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Figure 1. Schematic of embryonicaortic arch system.
The 6 pairs of embryonic aorticarches are demonstrated (left-sided arches are numbered).
The portions that normallyinvolute are indicated by brokenlines.
The distal left sixth embryonicarch normally persists andbecomes the PDA, connecting
the left pulmonary artery to theproximal descending aorta.
The right distal sixth arch normallyinvolutes, as does the eighthsegment of the right dorsal aorta(*), which results in a leftward
aortic arch. RCA indicates right carotid artery;
LCA, left carotid artery; RSCA,right subclavian artery; and LSCA,left subclavian artery.
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Fetal Circulation
65% of the fetal CO is from RV
5% to 10% passes through the lungs
Ventricular output -> DA -> descending
aorta The fetal ductus arteriosus is thus an
important structure that is essential fornormal fetal development, permitting right
ventricular output to be diverted away fromthe high-resistance pulmonary circulation.
Premature constriction or closure -> rightheart failure -> fetal hydrops.
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Histology and Mechanisms of NormalClosure
Aorta & pulmonary artery : surroundedmediae are composed mainly ofcircumferentially arranged layers ofelastic fibers
Ductus arteriosus : the media iscomposed longitudinally and spirallyarranged layers of smooth muscle fiberswithin loose, concentric layers of elastic
tissue. The intima of the ductus arteriosusis thickened and irregular, with abundantmucoid material,sometimes referred toas intimal cushions.
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Before birth
Fetal patency of the ductus arteriosus is
controlled by many factors ; low fetal oxygen tension
cyclooxygenase-mediated products of
arachidonic acid metabolism (primarilyprostaglandin [PGE2] and prostacyclin [PGI2] -> vasodilator DA via ductal prostanoidreceptor)
Placenta -> PGE & PGI -> decreasedmetabolism in the fetal lungs.
ft bi th
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After birthIncrease oxygen tension
ductal constriction & PGE2 and PGI2 levels fall
lungs metabolism now functioning
ductus contract & wall thickening, lumen obliteration &shortening of the ductus arteriosus
Functional complete within 24 to 48 hours of birth in termneonates
next 2 to 3 weeks ->
endothelium infolding, subintimal disruption & proliferation->
fibrosis & a permanent seal ->
ligamentum arteriosum.
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The factors responsible for persistentpatency of the ductus arteriosus beyondthe first 24 to 48 hours of neonatal life arenot completely understood
Prematurity clearly increases theincidence of PDA
In term infants -> sporadically, but there
is increasing evidence with geneticfactors
Prenatal infection appear to play a role
in some cases.
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Incidence
PDA has been reported to be 1 in 2000
births This accounts for 5% to 10% of all
congenital heart disease
Children with silent patent ductus, theincidence has been estimated to be ashigh as 1 in 500.
Female : male ratio is 2:1 in most reports.
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Genetic Factors
Increased frequency in several genetic
syndromes(such as trisomy 21 and 4psyndrome), single-gene mutations (suchas Carpenters syndrome and Holt-Oramsyndrome), and X-linked mutations (suchas incontinentia pigmenti).
Genetic mechanism of patent ductus insome patients may be autosomal
recessive inheritance with incompletepenetrance
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In a family having 1 sibling with a PDA,
there is an 3% chance of a PDA in a
subsequent offspring. Genetic studies suggest that the
abnormalities in Char syndrome (aninherited disorder with PDA, facial
dimorphism, and hand anomalies) resultfrom derangement of neural crest cellderivatives.
There is also evidence of abnormalfibronectin-dependent smooth musclecell migration as a cause of PDA
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Infection and Environmental Factors
Rubella infection (first trimester of
pregnancy)
particularly in the first 4 weeks.
Environmental factors, such as in fetal
valproate syndrome
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The size, configuration,
and relationship toadjacent structuresare important withrespect to determiningresistance to bloodflow
Figure 2 demonstratesthe wide variability inpatent ductus size andconfiguration using theangiographicclassification originallydevised to help guidetranscatheter closureprocedures
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Figure 2. Variations in PDAconfiguration illustrated with theclassification of Krichenko et al.
A, Type A (conical) ductus, withwell-defined aortic ampulla andconstriction near the pulmonaryartery end.
B, Very large type B (window)
ductus, with very short length.
C, Type C (tubular) ductus,which is without constrictions.
D, Type D (complex) ductus,Which has multiple constrictions.
E, Type E (elongated) ductus,with the constriction remotefrom the anterior edge of the
trachea.
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Left-to-Right Shunting
The hemodynamic impact ofPDA,determined by the magnitude ofshunting(flow resistance of the ductusarteriosus, length, the narrowest diameter,
shape and configuration) Flow in the ductus is dynamic and pulsatile,
the elasticity of the ductus wall affect theblood flow
The magnitude of shunt flow depends notonly on the ductal resistance but also onthe pressure gradient between the aortaand the pulmonary artery
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Left-to-right shunting pulmonaryovercirculation and left heart volume
overload Increased pulmonary flow from the ductal
shunting increased pulmonaryfluid volume.
Shunts with moderate or largedecreased lung compliance
increased work of breathing
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Increased flow returning to the left heartincreased LA and LV end-diastolic
pressures.
LV compensates
Neuroendocrine adaptations
LVH
increased sympatheticactivity & catecholamines
(increased contractility & HR)
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EisenmengersSyndrome
long-standing left-to-right shunting
pulmonary artery system to high-pressure &increased flow
progressive morphological changes in thepulmonary vasculature (arteriolar medialhypertrophy, intimal proliferation & fibrosis)
progressive increase in pulmonary vascularresistance
ductal shunting reverses , right to left
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Medical History
Exercise intolerance
Reactive airways disease
Symptoms of congestive heart failure
Physical Examination
continuous murmur LUSB -> machinery
murmur
Murmur often radiates down the left side of
the sternum and into the back
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Thrill may be present
Diastolic rumble is audible at the cardiac
apex The peripheral pulses may be prominent
or bounding.
Auscultation may reveal a high-frequency diastolic decrescendomurmur of pulmonary regurgitationand/or a holosystolic murmur from
tricuspid valve regurgitation. Peripheral edema may be present late in
the course of disease when rightventricular dysfunction is present.
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Chest Radiograph
Cardiomegaly(specifically with signs of
left atrial and left ventricularenlargement).
Increased pulmonary vascular markings.
Main pulmonary artery is frequentlyenlarged, particularly in older adults withpulmonary hypertension, calcification ofthe ductus may be evident.
Electrocardiogram Sinus tachycardia or atrial fibrillation
LVH, LAE, RAE
Biventricular hypertrophy
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Echocardiogram
The echocardiogram is the procedure of
choiceto confirm the diagnosis and tocharacterize a PDA (Figure 3).
Echocardiogram is often useful inclassifying the PDA as silent, small,
moderate, or large. To identify and evaluate other
associated cardiac defects.
M-mode echocardiography is used tomeasure the cardiac chamber sizes andquantitate left ventricular systolicfunction
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Patient with a small ductus arteriosus,chamber sizes are usually normal,
although mild LA and/or LV enlargementmay be present.
Patient with a moderate or large patentductus, the LA and LV are enlarged.
Color Doppler is a very sensitive modalityin detecting the presence of a PDA andis frequently used to estimate the degreeof ductal shunting.
A complete pulmonary artery pressurecurve can be generated from thespectral Doppler signal of ductal flow.
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Magnetic Resonance Imaging andComputed Tomography
Computed tomography can assess thedegree of calcification, important ifsurgical therapy is considered.
To define the anatomy in patients withunusual PDA geometry, associatedabnormalities of the aortic arch (ductusarteriosus aneurysm), may present as a
chest mass.
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Cardiac Catheterization
Therapeutic catheterization is currently the
treatment of choice at most centers formost children and adults with patent ductus
Complete diagnostic assessment of
hemodynamics before transcatheter
closure.
Assessment of pulmonary vascular
resistance and its response to vasodilating
agents such as oxygen, nifedipine,prostacyclin, sildenafil, and nitric oxide may
be helpful in determining advisability of
ductus closure.
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Assessment of hemodynamics during
temporary test occlusion with a balloon
catheter may also provide important
information regarding advisability of
closure.
Detailed assessment of the ductal anatomy
is essential before transcatheter closure sothat the proper device and device size can
be chosen for the intervention (minimum
diameter, the largest diameter (usually at
the aortic ampulla), the length, and therelationship of the ductus to the anterior
border of the tracheal shadow, which helps
guide device positioning.)
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The natural history of PDA dependslargely on the size and magnitude of theshunt and the status of the pulmonaryvasculature.
patients with significant left heart volumeoverload, however, are at risk of
congestive heart failure or irreversiblepulmonary vascular disease,
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Congestive Heart Failure
Moderate to large patent ductus ->
congestive heart failure due topulmonary overcirculation and left heartvolume overload
Significant chronic volume overload ofthe left heart -> develop congestiveheart failure in adulthood, starting in the
third decade
In the adult, heart failure is frequentlyassociated with atrial flutter or fibrillation
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Hypertensive Pulmonary Vascular Disease
large nonrestrictive or minimally restrictivepatent ductus -> develop irreversible
pulmonary vascular disease. Secondary to long-standing pressure and
volume overload in the pulmonarycirculation -> Hypertensive PulmonaryVascular Disease
Endarteritis
Incidence of infective arteritis was reportedto
be 1% per year
In countries with limited health resourcesand access to health care, infectiveendarteritis associated with ductusarteriosus remains a significant health issue
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Aneurysm of Ductus Arteriosus
Reported incidence as high as 8%
Ductal aneurysm most commonlypresents in infancy
Reported in adults, and may develop
after infective endarteritis, surgicalclosure, or transcatheter coil occlusion
one fourth of patients, an underlyingdisorder such as trisomy 21, trisomy 13,Smith-Lemli- Opitz syndrome, type IVEhlers-Danlos syndrome, or Marfanssyndrome is present
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One potentially promising technique isplacement of a covered stent in theaorta to simultaneously exclude the
aneurysm and occlude the ductusarteriosus.
Other Complications Recurrent laryngeal nerve paralysis even
without aneurysm
Dissection and/or spontaneous rupture
of an aneurysmally dilated pulmonaryartery due to PDA with pulmonaryhypertension
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Symptomatic : diuretics and digoxin Afterload reduction : ACE-I
Antidysrhythmia (AF, flutter)
Cardiovertion, maintained in sinus rhythmafter closure
Anticoagulation (AF)
Infective endocarditis prophylaxis -> allpatients with PDA until 6 months afterclosure
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Patients with PDA and pulmonaryvascular disease who are considered
unacceptable candidates for definitiveclosure may be managed withpulmonary vasodilating agents such aschronic oxygen, PGI2, calcium channelblockers, endothelin antagonists, andphosphodiesterase type V inhibitors.
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Historical Perspective
Techniques for closure of PDA haveevolved since the first report of surgicalligation by Gross and Hubbard in 1939
Transcatheter methods, to avoidthoracotomy, were pioneered byPortsmann et al, who reported use of a
conical Ivalon plug in 1967, and byRashkind and Cuaso, who reported initialresults of an umbrella-type device in1979
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After Cambier et al67 in 1992 reportedthe use of Gianturco coils for
transcatheter closure of PDA,transcatheter coil occlusion quicklybecame a widely used technique forclosure of the small to moderate patent
ductus
Indications for Closure of PDA
indicated for any child or adult who issymptomaticfrom significant left-to-rightshunting through the PDA
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In asymptomatic patients with significantleft-to right shunting that results in left
heart enlargement,closure is indicatedto minimize the risk of complications inthe future
In patients with pulmonary vascular
resistance >8 U/m2, lung biopsy hasbeen recommended to determinecandidacy for closure.
The indications for closure of patent
ductus with small shunts, including thosethat are tiny and incidentally discovered(silent), are less certain, particularly inolder adults.
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Transcatheter Closure
Treatment of choice for most patent
ductus in children and adults. Cases with calcified ductus arteriosus
with increased pulmonary vascular
resistance, transcatheter closure offersconsiderable advantages over surgicalclosure
Frequently involves cardiopulmonarybypass with an anterior approachthrough a median sternotomy
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Figure 4 demonstrates an example ofductus occlusion with a standard
Gianturco coil Nit-Occlud coil occlusion designed for
occlusion of vascular structures otherthan the patent ductus
For closure of moderate and largepatent ductus, the Amplatzer ductoccluder is frequently used (Figure 6).
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Figure 4. Example ofGianturco coilocclusion of PDA.
A, Views of a
Gianturco coil in its stretched out
configuration (top)and in its naturalcoiled configuration
(bottom). Note theattached Dacronfibers, which promotethrombosis, along itslength.
B through D, Lateralangiogramsdemonstrating closureof a PDA with a single0.038-in diameterGianturco coil.
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Figure 5. Example ofPDA closure with a Nit-OccludPDA occlusiondevice.
A, Image of a Nit-Occlud coil with itsbiconical
configuration. Notethe reversed windingon the proximal end.
B through D, Lateral
angiogramsdemonstrating closureof a PDA with a singleNit-Occlud coil.
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Figure 6. Example ofPDA occlusion withan Amplatzer ductoccluder device.
A, Image of anAmplatzer ductoccluder device.
B through D, Lateralangiogramsdemonstrating
closure of a PDA withan Amplatzer ductoccluder device.
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The availability of a variety of devices andtechniques enhances the capability toclose the vast majority of patent ductus
with catheter-based techniques. Complete closure rates at follow-up
generally exceed 90% to 95% in moststudies
The most common complication is deviceembolization
Other potentially important complications :flow disturbance in the proximal leftpulmonary artery or descending aorta froma protruding device, hemolysis, femoralartery or vein thrombosis.
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Greater pain and morbidity thantranscatheter methods
Surgical ligation and surgical division aresafe and effective procedures
Surgical ligation or division of the PDA remains the treatment of choice -> very
large ductus.
Complete closure rates of surgicalligation (often accompanied by divisionof the ductus) in published reports rangefrom 94% to 100%, with 0% to 2% mortality
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Complications : bleeding,pneumothorax, infection.
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PDA is a cardiovascular disorder found in
patients of all ages and sizes, from tinypremature infants to older adults
The clinical implications vary depending onthe anatomy of the ductus arteriosus and
the underlying cardiovascular status of thepatient
Over the past 3 decades, transcathetertechniques have replaced surgical therapy
in most patients with PDA. Echocardiography have resulted in
improved detection and characterizationof PDA in patients of all ages.
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Thank you