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    Patent Ductus Arteriousus

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    History

    In 1593 Giambattista Carcano described ductusarteriosus in book great cardiac vessels of the fetus

    Leo bottani falsely associated with ductus

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    Anatomy

    Normal fetal vascular channel between aorta andpulmonary artery.

    The pulmonary end is located to left of bifurcation of

    pulmonary trunk Aortic end is just beyond the origin of left subclavian

    artery

    With a right aortic arch, the ductus arteriosus may be

    on the right, joining the right pulmonary artery andthe right aortic arch just distal to the right subclavianartery

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    Introduction

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    microscopic structure of the ductus arteriosus differs

    the media of the ductus arteriosus consist largely oflayers of smooth muscle arranged spirally in both

    leftward and rightward directions The intimal layer of the ductus arteriosus is thicker

    than that of the adjoining arteries and contains anincreased amount of mucoid substance

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    Embryology

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    Physiology

    Role in the Fetus

    6 weeks of gestation, the ductus arteriosus isdeveloped sufficiently to carry most of the right

    ventricular output The right ventricle ejects about two thirds of

    combined ventricular output

    ductus arteriosus permits flow to be diverted away

    from the high-resistance pulmonary circulation

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    Normal Postnatal Closure

    closure of the ductus arteriosus is effected in twophases

    Immediately after birth, contraction and cellular

    migration of the medial smooth muscle in the wall ofthe ductus

    resulting in functional closure

    commonly occurs within 12 hours after birth

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    Normal Postnatal Closure

    The second stage usually is completed by 2 to 3weeks

    produced by infolding of the endothelium, disruption

    and fragmentation of the internal elastic lamina replacement of muscle fibers with fibrosis

    permanent sealing of the lumen to produce theligamentum arteriosum

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    mechanisms responsible for the initial postnatalclosure of the ductus arteriosus are not fullyunderstood

    increase in pO2, as occurs with ventilation after birth,constricts the ductus arteriosus

    prostaglandins play an active role in maintaining theductus arteriosus in a dilated state

    PGE1, PGE2, and PGI2 dilate isolated ductusarteriosus strips or rings from term fetal lambs

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    At birth, the placental source is removed, and themarked increase in pulmonary blood flow allowseffective removal of circulating PGE2

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    INCIDENCE OF PDA

    increased dramatically over the last two decades

    improved survival rate of premature infants

    incidence is approximately 0.02 to 0.04 percent

    among term infants born at sea level slight female predominance

    incidence is as high as 60 percent in infants bornbefore 28 weeks gestation

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    Clinical History

    The diagnosis of PDA canot be made at birth

    The murmur emerges after few days as thepulmonary vascular resistance falls

    History of prematurity is very important. Premature babies with respiratory distress tend to

    have large shunts

    PDA is moe common in females with a ratio of 2:1

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    Family history is important , as it tends to recur insiblings

    Rubella infection to mother during the first trimester

    is a common cause maternal coxsackie virus infection is another cause

    Low birth weight is common, even in small shunts

    More common in children born in October toJanuary

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    Another interesting point is the relation to thealtitude the patient was born

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    Small Ductus Arteriosus

    the resistance to flow across the ductus arteriosus ishigh

    only a small left-to-right shunt develops

    Pulmonary blood flow is increased only minimally left ventricular failure does not occur

    Most of the infants are asymptomatic

    Murmur is detected on routine physical examination.

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    Moderate Ductus Arteriosus

    In infants a moderate shunt produces symptoms ofheart failure

    Poor feeding, irritability, and tachypnea are present

    symptoms ordinarily increase until about the secondto third month

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    Large Ductus Arteriosus

    Infants with a large PDA are invariably symptomatic

    They are irritable, feed poorly, fail to gain weight andsweat excessively

    They have increased respiratory effort andrespiratory rates

    prone to develop recurrent upper respiratoryinfections and pneumonia

    symptoms indicative of severe left ventricular failurewith pulmonary edema may occur early in infancy

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    Patients with reversed shunt

    Small number of cases

    High pulmonary vascular resistance, from infancy

    Effort dyspnea is the most common symptom

    Symptoms of left ventricular failure are absent Hoaseness of voice may be present.

    Cyanosis may be overlooked.

    There is marked leg fatigue and absence of dyspnea Rarely patients may have swelling and pain in lower

    limbs.

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    Physical apperance

    Physical underdevelopment due to a large shunt

    Maternal rubella syndrome: cataract, deafness andmental retardation

    Rocker bottom feet and loose skin is present intrisomy 13

    In this syndrome assosiated VSD is usually present

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    Examination

    Differential cyanosis and clubbing is pesent in shuntreversal

    It can be brought out by exersise or a warm water

    bath Useful to have patient sit with hands and feet

    together.

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    Arterial pulse

    A wide pulse pressure is present

    Pulse has a brisk rise, single peak and rapid collapse

    Diastolic pressure is low, systolic is high

    The peripheral pulses are bounding If the shunt is small or if there is pulmonary

    hypertension the bounding pulse is absent

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    JVP

    JVP is normal in small shunts

    In patients of cardiac failure the jvp is elevated andprominent A and V waves are present

    Prominent A waves are present in high pulmonaryresistance

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    Auscultation

    The classic murmur of uncomplicated PDA rises topeak in latter systole

    Continues without interruption through the second

    heart sound Declines in intensity in diastole

    a silent interval may be present towards the end ofdiastole

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    Continious murmur

    A small duct results in a soft , high frequencycontinuous murmur

    A larger duct causes a loud noisy machinery murmur

    Loud murmur becomes soft if there is narrowing ofthe duct.

    The murmur is dependent on the pressure differencebetween aorta and pulmonary artery

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    Progression of murmur

    As the diastolic pressure of pulmonary arteryincreases the patient is left with a holosystolicmurmur

    As pulmonary hypertension progresses further thesystolic component also disappears

    Right to left flow across PDA does not have amurmur

    With increasing PH Gibson murmur is abolished Findings of pulmonary hypertension are present

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    Newborn

    In newborns the classic murmur is absent

    Only a soft systolic murmur may be present

    this is due to high pulmoary pressures

    However the signs of cardiac failure are present

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    Second heart sound

    Paradoxical spitting is present in patients of large leftto right shunts

    due to Prolonged LV ejection and short RV ejection

    Difficult to detect on auscultation With shunt reversal the second heart sound is closely

    split with loud pulmonary component

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    ECG

    Small ductus ECG may be normal

    Variation in ecg depend on the volume overload ofLV and pressure overload of RV

    Usually have sinus rhythm P waves are notched, bifid and prolonged

    s/o left atrial enlargement

    With development of pulmonary hypertension signsof biatrial enlargement are present

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    PR interval is prolonged in 10 to 20 percent ofpatients

    QRS axis is normal

    Volume overload of left ventricle results in deep swaves in v1, tall r waves I v5 to v6, deep q waves andtall t waves

    A large shunt with pulmonary hypertension results

    in features of biventricular hypertrophy Large equidiphasic complexes are present from v1 to

    v6

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    ECG in right to left shunt

    In PDA with right to left shunt peaked narrow rightatrial P waves appear in 2,3,and v1

    QRS axis shows right axis deviation

    Right ventricular hypertrophy R waves in v5, v6 remain tall bur the q waves and the

    tall t waves disappear

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    X ray chest

    Variation in XRAY depend on size, duration anddirection and pulmonary pressures

    The ductus may be seen as a convexity between aorta

    and pulmonary artery In older patients calcium may be depoisted

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    Left to right shunt causes increase in pulmoaryplethora

    Pulmonary trunk and main branches are dilated

    Ascending aorta is enlarged in adults LA and LV are enlarged

    RA and RV dilatation occurs when pulmonaryhypertension is present

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    Right to left shunt

    RV is hypertrophied

    Pulmonary trunk and main branches are dilated

    Peripheral vasculature is reduced

    Ascending aorta is normal sized Patients who have shunt reversal have larger hearts

    Both RV and LV enlargement is seen

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    Echocardiography

    A complete echocardiographic evaluation of the PDAincludes

    two-dimensional imaging of the ductus,

    evaluation of the degree of shunting at the ductus,and

    evaluation of pulmonary artery pressure usingDoppler echocardiography

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    Anatomic assessment

    Most commonly, the ductus is imaged in theparasternal and suprasternal notch views

    In the high parasternal short axis view, with the

    transducer oriented leftward toward the pulmonaryartery bifurcation, the ductus can be imagedcoursing between the pulmonary artery and thedescending aorta

    Absolute quantification of its diameter is the bestway to determine its presence or absence

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    In the suprasternal notch window, the ductus arisesfrom the descending aorta at the level of the leftsubclavian artery, and courses anteriorly to join thepulmonary artery

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    Color flow mapping is particularly helpful in thesetting of a small PDA,

    Determination of the origin of the retrograde flowinto the pulmonary artery using two-dimensionalimaging as well as color flow mapping is crucial toavoid confusion of the patent ductus with otheraortopulmonary shunts

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    The Size of the Left Atrium Left atrial (LA) enlargement signifies increased

    pulmonary venous return because of left-to-rightductal shunting

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    Left Ventricular Size

    This will enlarge as cardiac output increases withboth increased pulmonary venous return and with

    increased diastolic run-off Descending Aortic Flow in Diastole

    The presence of a significant ductal shunt resultsin diastolic run-off to the pulmonary circulation

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    Left Pulmonary Artery Diastolic FlowVelocity

    This is higher with large left-to-right

    shunts. Values less than 15cm/sec are seen whenthe duct is closed

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    Hemodynamic assessment

    The pulmonary to systemic flow ratio (Qp:Qs) can bedetermined echocardiographically

    When the pulmonary artery pressure is lower thansystemic arterial pressure, there is continuous left-to-right shunting demonstrated.

    The velocity of flow across the ductus, measured byeither pulsed or continuous wave Doppler,

    can be translated into the gradient between theaorta and the pulmonary artery

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    When the pulmonary artery pressure is equal tosystemic pressure, pulsed Doppler within the ductusdemonstrates systolic right-to-left shunting, withdiastolic left-to-right flow within the vessel

    Cardiac Catheterization

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    Cardiac Catheterization

    Color Doppler flow mapping is generally as sensitiveas cardiac catheterization for detecting even a smallPDA

    In children with pulmonary hypertension,determining the exact location of the shunt can bedifficult

    Right heart catheterization alone usually suffices to

    confirm the diagnosis an additional lesion such as ventricular septal defect

    is suspected

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    An increase of pulmonary arterial blood oxygencontent of >0.5 mL/dL or a saturation increase of>4% to 5% from that in right ventricular bloodindicates a significant left-to-right shunt at the

    pulmonary arterial level

    An increase in oxygen saturation in pulmonaryarterial blood is not diagnostic of a PDA, but may be

    present in lesions such as aortopulmonary windowor a high ventricular septal defect

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    a small communication, pulmonary arterial bloodpressures are normal, but systemic arterial pulsepressure may be slightly widened

    a moderate-sized defect,

    1. pulmonary arterial systolic, diastolic, and meanblood pressures may be slightly elevated.

    2. Systemic arterial diastolic blood pressure falls,

    3. whereas systemic arterial pulse pressure increases

    l h h d

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    large shunt hemodynamics

    pulmonary and systemic arterial pressures are equal, left atrial mean pressure may be increased substantially,

    and a prominent V wave is seen.

    Left ventricular end-diastolic pressure may be elevated,

    a diastolic pressure gradient between the left atrium andleft ventricle is demonstrated.

    A small systolic pressure difference between the left

    ventricle and aorta is also encountered

    Angiography

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    Angiography

    it is the most effective test for defining the anatomyof the PDA

    Contrast medium is injected into a catheter passedthrough the PDA into the aorta from the pulmonaryartery or into the aorta retrogradely from thefemoral artery

    PDA usually is widely dilated, and the ductus

    narrows down at the pulmonary arterial end

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    the lateral projection, or occasionally the left anterioroblique projection, demonstrates the anatomy mostclearly

    The AP camera can be positioned in the rightanterior oblique caudal position to demonstrate thePDA

    selective descending aortography is essential in

    patients of VSD or ASD to demonstrate PDA

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    M i R I i

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    Magnetic Resonance Imaging

    simpler techniques such as two-dimensionalechocardiography Doppler evaluation accuratelydefine the anatomy

    These studies can be of use in adolescents or adultswith poor echo windows

    Velocity-encoded cine MRI imaging for estimation ofleft-to-right shunting may have additional clinical

    utility

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    P t l

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    Percutaneous closure

    A variety of devices have been used for percutaneousclosure of a PDA

    It is the standard of care in most patients

    Exception in premature and small infants with largeshunts

    Have been available for last 20 years

    C il

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    Coils

    Stainless steel Gianturco coils Earlier used for AV malformations

    For duct closure the PDA should be less than 2 mm

    in diameter, long to accommodate loops and shouldhave sufficient aortic ductal diverticulum

    The coils are deployed in a retrograde fashion fromthe aorta

    Coil embolisation is a dangerous complication Modified coils with release mechanism are available

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    Multiple coils are used for large PDA PDA closure rate are around 95 to 100% at 2 years

    Residual shunt causes haemolysis

    Modifications available areGiantruco-grifika vascular occlusion device

    Nit occlud PDA occluder

    D t l d d i

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    Duct occluder device

    AMPLATZ duct occluder is only device which is FDAapproved

    Cone shaped device

    Antegrade venous approach Delivery cable- release notch

    98% closure at 6 months in large PDA

    Complications are left pulmonary artery stenosis,

    aortic coarctation

    Small ducts are avoided

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    Follow up

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    Follow up

    Anticoagulation for 6 months Endocarditis prophylaxsis

    Follow up 2d echo after 6 months

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    Thank you