Patent Ductus Arteriousus

History
 In 1593 Giambattista Carcano described ductus

arteriosus in book “great cardiac vessels of the fetus”  Leo bottani falsely associated with “ ductus”

Anatomy
 Normal fetal vascular channel between aorta and

pulmonary 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 and the right aortic arch just distal to the right subclavian artery

Introduction

 microscopic structure of the ductus arteriosus differs

 the media of the ductus arteriosus consist largely of

layers 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 an increased amount of mucoid substance

Embryology

Physiology
Role in the Fetus  6 weeks of gestation, the ductus arteriosus is developed 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

Normal Postnatal Closure
 closure of the ductus arteriosus is effected in two

phases  Immediately after birth, contraction and cellular migration of the medial smooth muscle in the wall of the ductus  resulting in functional closure  commonly occurs within 12 hours after birth

Normal Postnatal Closure
 The second stage usually is completed by 2 to 3

weeks  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 the ligamentum arteriosum

 mechanisms responsible for the initial postnatal

closure of the ductus arteriosus are not fully understood  increase in pO2, as occurs with ventilation after birth, constricts the ductus arteriosus  prostaglandins play an active role in maintaining the ductus arteriosus in a dilated state  PGE1, PGE2, and PGI2 dilate isolated ductus arteriosus strips or rings from term fetal lambs

 At birth, the placental source is removed, and the

marked increase in pulmonary blood flow allows effective removal of circulating PGE2

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 born before 28 weeks gestation

Clinical History
 The diagnosis of PDA canot be made at birth

 The murmur emerges after few days as the

pulmonary 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

 Family history is important , as it tends to recur in

siblings  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 to

January

 Another interesting point is the relation to the

altitude the patient was born

Small Ductus Arteriosus
 the resistance to flow across the ductus arteriosus is  


 

high 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.

Moderate Ductus Arteriosus
 In infants a moderate shunt produces symptoms of

heart failure  Poor feeding, irritability, and tachypnea are present  symptoms ordinarily increase until about the second to third month

Large Ductus Arteriosus
 Infants with a large PDA are invariably symptomatic

 They are irritable, feed poorly, fail to gain weight and

sweat excessively  They have increased respiratory effort and respiratory rates  prone to develop recurrent upper respiratory infections and pneumonia  symptoms indicative of severe left ventricular failure with pulmonary edema may occur early in infancy

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.

Physical apperance
 Physical underdevelopment due to a large shunt

 Maternal rubella syndrome: cataract, deafness and

mental retardation  Rocker bottom feet and loose skin is present in trisomy 13  In this syndrome assosiated VSD is usually present

Examination
 Differential cyanosis and clubbing is pesent in shunt

reversal  It can be brought out by exersise or a warm water bath  Useful to have patient sit with hands and feet together.

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

JVP
 JVP is normal in small shunts

 In patients of cardiac failure the jvp is elevated and

prominent A and V waves are present  Prominent A waves are present in high pulmonary resistance

Auscultation
 The classic murmur of uncomplicated PDA rises to

peak 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 of diastole

Continious murmur
 A small duct results in a soft , high frequency

continuous murmur  A larger duct causes a loud noisy machinery murmur  Loud murmur becomes soft if there is narrowing of the duct.  The murmur is dependent on the pressure difference between aorta and pulmonary artery

Progression of murmur
 As the diastolic pressure of pulmonary artery

   

increases the patient is left with a holosystolic murmur As pulmonary hypertension progresses further the systolic component also disappears Right to left flow across PDA does not have a murmur With increasing PH Gibson murmur is abolished Findings of pulmonary hypertension are present

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

Second heart sound
 Paradoxical spitting is present in patients of large left

to 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

ECG
 Small ductus ECG may be normal

 Variation in ecg depend on the volume overload of


 

LV 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 signs of biatrial enlargement are present

 PR interval is prolonged in 10 to 20 percent of  

 

patients QRS axis is normal Volume overload of left ventricle results in deep s waves in v1, tall r waves I v5 to v6, deep q waves and tall t waves A large shunt with pulmonary hypertension results in features of biventricular hypertrophy Large equidiphasic complexes are present from v1 to v6

ECG in right to left shunt
 In PDA with right to left shunt peaked narrow right

atrial 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

X ray chest
 Variation in XRAY depend on size, duration and

direction and pulmonary pressures  The ductus may be seen as a convexity between aorta and pulmonary artery  In older patients calcium may be depoisted

 Left to right shunt causes increase in pulmoary  


plethora Pulmonary trunk and main branches are dilated Ascending aorta is enlarged in adults LA and LV are enlarged RA and RV dilatation occurs when pulmonary hypertension is present

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

Echocardiography
 A complete echocardiographic evaluation of the PDA

includes  two-dimensional imaging of the ductus,  evaluation of the degree of shunting at the ductus, and  evaluation of pulmonary artery pressure using Doppler echocardiography

Anatomic assessment
 Most commonly, the ductus is imaged in the

parasternal and suprasternal notch views  In the high parasternal short axis view, with the transducer oriented leftward toward the pulmonary artery bifurcation, the ductus can be imaged coursing between the pulmonary artery and the descending aorta  Absolute quantification of its diameter is the best way to determine its presence or absence

 In the suprasternal notch window, the ductus arises

from the descending aorta at the level of the left subclavian artery, and courses anteriorly to join the pulmonary artery

 Color flow mapping is particularly helpful in the

setting of a small PDA,  Determination of the origin of the retrograde flow into the pulmonary artery using two-dimensional imaging as well as color flow mapping is crucial to avoid confusion of the patent ductus with other aortopulmonary shunts

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

pulmonary venous return because of left-to-right ductal shunting

 Left Ventricular Size

 This will enlarge as cardiac output increases with

both increased pulmonary venous return and with increased diastolic run-off  Descending Aortic Flow in Diastole  The presence of a significant ductal shunt results in diastolic run-off to the pulmonary circulation

 Left Pulmonary Artery Diastolic Flow

Velocity  This is higher with large left-to-right shunts. Values less than 15cm/sec are seen when the duct is closed

Hemodynamic assessment
 The pulmonary to systemic flow ratio (Qp:Qs) can be

determined echocardiographically  When the pulmonary artery pressure is lower than systemic arterial pressure, there is continuous leftto-right shunting demonstrated.  The velocity of flow across the ductus, measured by either pulsed or continuous wave Doppler,  can be translated into the gradient between the aorta and the pulmonary artery

 When the pulmonary artery pressure is equal to

systemic pressure, pulsed Doppler within the ductus demonstrates systolic right-to-left shunting, with diastolic left-to-right flow within the vessel

Cardiac Catheterization
 Color Doppler flow mapping is generally as sensitive

as cardiac catheterization for detecting even a small PDA  In children with pulmonary hypertension, determining the exact location of the shunt can be difficult  Right heart catheterization alone usually suffices to confirm the diagnosis  an additional lesion such as ventricular septal defect is suspected

 An increase of pulmonary arterial blood oxygen

content of >0.5 mL/dL or a saturation increase of >4% to 5% from that in right ventricular blood indicates a significant left-to-right shunt at the pulmonary arterial level  An increase in oxygen saturation in pulmonary arterial blood is not diagnostic of a PDA, but may be present in lesions such as aortopulmonary window or a high ventricular septal defect

 a small communication, pulmonary arterial blood

pressures are normal, but systemic arterial pulse pressure may be slightly widened  a moderate-sized defect, 1. pulmonary arterial systolic, diastolic, and mean blood pressures may be slightly elevated. 2. Systemic arterial diastolic blood pressure falls, 3. whereas systemic arterial pulse pressure increases

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 and

left ventricle is demonstrated.  A small systolic pressure difference between the left ventricle and aorta is also encountered

Angiography
 it is the most effective test for defining the anatomy

of the PDA  Contrast medium is injected into a catheter passed through the PDA into the aorta from the pulmonary artery or into the aorta retrogradely from the femoral artery  PDA usually is widely dilated, and the ductus narrows down at the pulmonary arterial end

 the lateral projection, or occasionally the left anterior

oblique projection, demonstrates the anatomy most clearly  The AP camera can be positioned in the right anterior oblique caudal position to demonstrate the PDA  selective descending aortography is essential in patients of VSD or ASD to demonstrate PDA

Magnetic Resonance Imaging
 simpler techniques such as two-dimensional

echocardiography Doppler evaluation accurately define the anatomy  These studies can be of use in adolescents or adults with poor echo windows  Velocity-encoded cine MRI imaging for estimation of left-to-right shunting may have additional clinical utility

Percutaneous closure
 A variety of devices have been used for percutaneous

closure of a PDA  It is the standard of care in most patients  Exception in premature and small infants with large shunts  Have been available for last 20 years

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 should have sufficient aortic ductal diverticulum  The coils are deployed in a retrograde fashion from the aorta  Coil embolisation is a dangerous complication  Modified coils with release mechanism are available

 Multiple coils are used for large PDA

 PDA closure rate are around 95 to 100% at 2 years
 Residual shunt causes haemolysis  Modifications available are

Giantruco-grifika vascular occlusion device Nit occlud PDA occluder

Duct occluder device
 AMPLATZ duct occluder is only device which is FDA  


  

approved 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

Follow up
 Anticoagulation for 6 months

 Endocarditis prophylaxsis
 Follow up 2d echo after 6 months

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