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PRESENTED BY : DR.PRESENTED BY : DR. ADILADILLANKERLANKER

MODERATED BY :MODERATED BY : DR.GULAMDR.GULAM

RASOOLRASOOL

APPROACH TO A CHILD WITH CYANOTIC

CONGENITAL HEART DISEASE

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INTRODUCTIONINTRODUCTIONy The initial evaluation for suspected congenital heart disease involves a

systematic approach with three major components.

1. Congenital cardiac defects can be divided into two major groupsbased on the presence or absence of cyanosis, which can bedetermined by physical examination aided by pulse oximetry .

2. These two groups can be further subdivided according to whether thechest radiograph shows evidence of increased, normal, or decreasedpulmonary vascular markings.

3. The electrocardiogram can be used to determine whether right, left,or biventricular hypertrophy exists.

y The character of the heart sounds and the presence and character of anymurmurs further narrow the differential diagnosis.

y The final diagnosis is then confirmed by echocardiography or cardiaccatheterization, or by both.

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Cyanotic Congenital Heart Lesions

y

This group of congenital heart lesions can also be further divided according topathophysiology: whether pulmonary blood flow is decreased or increased.

The CXR is a valuable tool for initial differentiation between these two categories.

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Cyanosis

y Cyanosis in children happens when there is clinically apparent amount of desaturated

hemoglobiny Usually requires 3-4 grams/ dL of reduced hemoglobin

y This usually correspond to oxygen saturation of 70-80%

y Therefore mild desaturation may clinically be missed

Mechanisms of Cardiac Cyanosis

Right to left

shuntMixing Recirculation

TOF Single ventricle TGA

Chest radiography

Electrocardiogram

Hyperoxia test

Echocardiogram

Testing

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TETRALOGY OF FALLOT

y Commonest cause of cyanotic heart disease after one yr of age.

y Cl presentation depends on severity of RV outflow obstruction.

y Most are cyanotic since birth.

y May presents with exertional dyspnoea.y Hypoxic spell

y squatting

SIGNS

a) RV type impulse

b) Systolic thrill at left 3rd ics

c) S2single(A2)

d) Ejection systolic murmur at left 3rd ics

e) Continuous murmur faintly audible over the ant & post chest

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CHEST X RAYy normal-sized, boot-shaped heart (coeur

en sabot)

y prominence of the right ventricle and a

concavity in the region of theunderdeveloped right ventricular outflow

tract and main pulmonary artery.

y The pulmonary vascular markings are

typically diminished,y the aortic arch may be on the right side

(25 percent).

y Dilatation of the ascending aorta

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y Electrocardiography

1. Right axis deviation

2. Right ventricular and right atrialhypertrophy

3. Adults with repaired tetralogy ofFallot, a complete right bundle

branch block following repair hasbeen the rule.

y Cardiaccatheterizationdemonstrates a systolic pressure inthe right ventricle equal to systemicpressure.

y Selectiverightventriculographybest demonstrates the anatomy of thetetralogy of Fallot. Contrast mediumoutlines the heavily trabeculated rightventricle.

y ECHOCARDIOGRAPHY

1. malaligned and nonrestrictiveVSD

2. overriding aorta (

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MedicalManagementy

Polycythemiay HCT should be 45-50%y HCT > 60% dramatically increases risky > 65% serious hyperviscosity risk

y Neurologic sequelae

y Clotting abnormalities

y

Consider phlebotomy pre-operativelyy Infection

y R->L shunt, direct route to bodyy Bacterial endocarditisy Brain abscess

y TET spellsy K

nee-chesty O2, volume, MSO4, vasoconstrictorsy surgery

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SurgicalM

anagementy VSD closure

y transatrial access if possibley Infundibular resection for visualizationy

Patch closurey ReliefofRVOTobstruction

y Infundibular resection vs.transannular patch

y Resultsy

Surgical Mortality is quite low, 1.6%y Residual obstruction is commony Residual VSDs may occury PI - initially well tolerated, but

significant late consequences

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1.Primaryrepairduring infancy has become the treatment of choice of

many centers with surgery scheduled at 2-4 months of age for

asymptomatic infants. This is to normalize the physiology sooner and

promote normal growth of the pulmonary arteries. It requires CP

bypass.

2. ModifiedBlalock Taussig Procedure the most commonly

performed in some symptomatic NBs that are poor candidates for

primary repair. This is a lower risk surgical procedure by creating a

systemic pulmonary artery shunt to increase the pulmonary bloodflow. This is usually not done with the child in CP bypass.

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Pulmonary Atresia with Ventricular Septal

Defect

y Pulmonary atresia with VSD is an extreme form of the tetralogy ofFallot.

y The pulmonary valve is atretic, rudimentary, or absent, and the

pulmonary trunk is atretic or hypoplastic. The entire right ventricularoutput is ejected into the aorta. Pulmonary blood flow is thendependent on a PDA or on bronchial collateral vessels.

y Most patients are severely cyanotic and require urgent prostaglandin E1infusion and palliative surgical intervention

y Some patients have heart failure caused by increased pulmonary bloodflow via bronchial collateral vessels (MAPCAs),and

y Some infants have adequate pulmonary blood flow and can be managedlike patients with uncomplicated tetralogy of Fallot.

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y The surgical procedure of choice depends on whether the mainpulmonary artery segment is adequate and on the size of the branch

pulmonary arteries.y In patients with small branch pulmonary arteries, surgical intervention is

directed toward increasing pulmonary blood flow in the hope thatpulmonary artery growth will be stimulated.

y Two options are currently considered:

1. an aortopulmonary (Blalock-Taussig or central) shunt or2. the establishment of a connection from the right ventricle directly to the

pulmonary artery,

a. either bypatch unroofingof the outflow tract or

b. by implanting a homograft conduit.

y To be a candidate for full repair, the pulmonary arteries must be ofadequate size to accept the full volume of right ventricular output.

y Complete repair includes closure of the VSD and placement of ahomograft conduit from the right ventricle to the pulmonary artery

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Pulmonary Atresia with Intact Ventricular

Septum

y In pulmonary atresia with an intact ventricular septum, thepulmonary valve leaflets are completely fused to form amembrane and the right ventricular outflow tract is atretic.

y Because no VSD is present, no egress of blood from the rightventricle occurs. Right atrial pressure increases, and blood shunts

via the foramen ovale into the left atrium, where it mixes withpulmonary venous blood and enters the left ventricle. Thecombined left and right ventricular output is pumped solely by theleft ventricle into the aorta.

y In a newborn with pulmonary atresia, the only source of

pulmonary blood flow occurs via a PDA.y The right ventricle is usually hypoplastic, although the degree of

hypoplasia varies considerably.

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ClinicalManifestations

y As the ductus arteriosus closes in the 1st hr or days of life, infants with pulmonary atresia

and an intact ventricular septum become markedly cyanotic.

y Untreated, most patients die within the 1st wk of life.

y Physical examination reveals severe cyanosis and respiratory distress.

y The 2nd heart sound is single and loud.

y Often, no murmurs are audible.

Treatmenty Infusion of prostaglandin E1 is usually effective before surgery.

y A surgicalpulmonaryvalvotomyis carried out to relieve outflow obstruction.

y To preserve adequate pulmonary blood flow, an aortopulmonaryshuntis often

performed during the same procedure.

y Surgicalunroofing of the right ventricular outflow tract and patch grafting.

y Interventionalcatheterization, in which the imperforate pulmonary valve is first

punctured with a wire or radiofrequency ablation catheter, followed by balloon

valvuloplasty.

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Tricuspid Atresiay Complete absence of communication

between the right atrium and right ventricle

y About 3 % of congenital heart disease

y There is an obligate interatrial

communicationy Usually associated with VSD

y The pulmonary blood flow is dependent onthe size of the VSD

y Pulmonary blood flow can be increased or

decreased causing variable presentingsymptoms

y If there is no VSD ( also called Hypoplasticright ventricle) the pulmonary blood flow isdependent on the PDA

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Clinical presentationy The presentation will depend on the amount of pulmonary blood flow

y If the PBF is decreased, the main presenting symptom is cyanosis

y If the PBF is increased the presentation is that ofcongestive heart failure

y Severe cyanosis since birth

y Hypoxic spell

y Prominent a wave in jvpy LV type impulse

y Tender hepatomegaly with presystolic pulsation

y S2 ---single loud( A2)

y A holosystolic murmur at left mid & lower sternal border

y CXR will also reflect the amount of pulmonary blood flow

y Two types----1.TA with normal related great art(70%)

2.TA with TGA ( 30%)

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ECG

1. Left axis deviation,

2. Tall peaked rt atrial P wave

3. Left ventricular hypertrophy.

4. Left atrial enlargement may be present

if pulmonary flow is high.

CHEST RADIOGRAPHY

1. Left ventricular configuration

2. Oligaemic lung field

3. The main pulmonary trunk is inapparent.

4. A right aortic arch exists in 25 percent of

patients

ECHO

1. Atretic tricuspid valve in Apical subxiphoid view

2. Hypoplastic RV

3. Large LV

4. ASD ,VSD,Aortic arch anomaly

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TherapeuticManagement:

a.MedicalManagement

PGE1 infusion is initiated for infants who depend on the PDA for pulmonaryblood flow; and the infant is stabilized and readied for surgery.

b. Interventional Cardiac catheterization

Balloon atrial septostomy during cardiac catheterization to allow blood to flowfrom the RA to the LA.

Single ventricle paliation

y First stage : to establish a reliable source of PBFy Aorta to pulmonary artery shunt ( BT shunt)

y Pulmonary arterial banding in cases of increased PBFy Second stage: GlennAnastomosis ( superior vena cava to pulmonary

artery

y Third stage : Fontan anastomosis ( Inferior vena cava to pulmonary artery

c.SurgicalManagement

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DoubleDouble--Outlet Right Ventricle with PulmonaryOutlet Right Ventricle with Pulmonary

StenosisStenosis

y Both the aorta and pulmonary artery arising from the right ventricle characterizedouble-outlet right ventricle with pulmonary stenosis; the outlet from the leftventricle is a VSD into the right ventricle.

y The aortic and mitral valves are separated by a smooth muscular conus, similarto that seen under the normal pulmonary valve.

y The aorta may override the VSD by a variable amount but is at least 50%committed to the right ventricle.

y This defect may be viewed as part of a continuum with the tetralogy ofFallot,depending on the degree of aortic override.

y

The physiology as well as the history, physical examination, electrocardiogram,and roentgenograms are similar to that in the tetralogy ofFallot,

y Surgical correction consists of creating an intraventricular tunnel so that the leftventricle ejects blood through the VSD, through the tunnel, and into the aorta.

y The pulmonary obstruction is relieved either with an outflow patch or with apulmonary or aortic homograft conduit (Rastelli operation).

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EBSTEIN ANOMALY

y Septal & post leaflet of tricuspid valve displaced downward ----atrialisation of RV

y h/o maternal use of lithium

y h/o cyanosis,effort intolerance &

fatigue,arrythmia

y LV impulse

y S1 widely split loud T1(the sail sound)

y S2 widely split

y S3,S4---triple & quardruple rhythm

ySystolic TR murmur not with resp

y Cyanosis with normal or reduced pulmonary blood flow and a dominant leftventricle with type B WPW is diagnostic

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Electrocardiography1. Himalayan p wave

2. Prolonged PR

3. RBBB

4. WPW

5. AF

6. Deep q wave in inf & V1-V4

CXR

1. RT BORER OF HEART PROMINENT WITH

LEFTWARD CONVEXITY

2. WATER BOTTLEAPPEARANCE

3. OLIGEMIC LUNG FIELD

4. AORTA & PUL ART INCONSPICOUS

ECHOCARDIOGRAPHY

1. Apical displacement of the septal leaflet of the tricuspid valve by 8

mm/m2 or more &

2. An elongated sail-like appearance of the anterior leaflet, confirms the

diagnosis

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Treatment

Inneonates:an aortopulmonary shunt alone or

by surgical patch closure of the tricuspid valve, atrial septectomy, and placementof an aortopulmonary shunt (Starnes procedure).

y This operation creates a functional tricuspid atresia, which can then be furtherrepaired with first a Glenn and then a Fontan.

Inolderchildren:y with mild or moderate disease,

control of supraventricular dysrhythmias is of primary importance;

surgical treatment may not be necessary until adolescence or young adulthood.

y with severe tricuspid regurgitation,

repair or replacement of the abnormal tricuspid valve along with closure of theatrial septal defect is then carried out.

In some patients, a bidirectional Glenn shunt is performed, with the superiorvena cava anastomosed to the pulmonary arteries. This procedure reduces thevolume of blood that the dysfunctional right side of the heart has to pump, thuscreating a one-and-one-half ventricle repair.

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Transposition of Great Areries (TGA)

y Aorta originating from the rightventricle, and pulmonary arteryoriginating from the left ventricle

y Accounts for 5-7% of all congenitalheart disease

y Survival is dependent on thepresence of mixing between thepulmonary and systemic circulation

y Atrial septal defect is essential forsurvival

y50% of patients have a VSD

y Usually presents in the first day oflife with profound cyanosis

y More common in boys

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TRANSPOSITION OF GREAT ARTERIES

(TGA )

COMPLETE TGA --------------- CONGENITALY

CORRECTED TGA

WITHOUT VSD ------------------- WITH VSD

(ASD,PDA)

WITH PS---------------------------WITHOUT PS

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COMPLETETRANSPOSITION OF THE GREAT ARTERIES

y d-TGA with an intact ventricular septum is

also referred to as simple TGAor isolated TGAy Male : female 4:1

y Average birth weight and size are greater than

normal.

y Dyspnea and cyanosis from birth, progressive

hypoxemia, and congestive heart failure usualpresentation

y Sever cyanosis & hypoxemia in infants who

have only a small patent foramen ovale or

ductus arteriosus and an intact ventricular

septum; or left ventricular outflow tractobstruction.

y cyanosis can be minimal, and heart failure is

the dominant after the first few weeks of life

if large VSD or PDA present

y Signs :

1. Pulsefull volume

2. JVP---N or increased in CHF

3. RV type impulse

4. Palpable S2 at left base----originate in

aortic valve5. early or holosystolic murmur of VSD may

be audible in less cyanotic infants with

associated VSD.

6. A soft midsystolic murmur of pulmonary

stenosis (PS or LVOT obstruction) may beaudible.

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CXR

1. Cardiomegaly with increased

pulmonary vascularity is typically

present.

2. An egg-shaped cardiac silhouette

with a narrow, superior

mediastinum is characteristic

Electrocardiography

1. rightward QRS axis

2. RVH is usually present afterthe first few days of life

3. Biventricular hypertrophy

(BVH)

ECHOCARDIOGRAPHY

In the parasternal short-axis view,

1. The great arteries appear asdoublecircles

2. The PA is in the centre of the heart

3. The aorta is usually anterior and

slightly to the right of the PA.

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Congenitally Corrected Transposition of the Great Arteries

y

In L-transposition, the atrioventricular relationships are discordant, withthe right atrium connected to the left ventricle and the left atrium to the

right ventricle (ventricular inversion).

y Rare less than 1%

y Asymptomatic when L-TGA is not associated with other defects.

y Most patients with associated defects become symptomatic with cyanosis

resulting from VSD and PS or CHF resulting from a large VSD.

y Exertional dyspnea and easy fatigability may develop with regurgitation of

the systemic AV valve

y Hyperactive precordium occurs in the presence of a large VSD

y The S2 is loud and single at the upper left or right sternal border.

y A grade 2 to 4/6 harsh, holosystolic murmur along the lower left sternal

border indicates the presence of VSD or systemic AV valve regurgitation.

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y CXR

1. A straight, left upper cardiac border,

formed by the ascending aorta

2. Cardiomegaly and increased pulmonary

vascular markings are present with VSD.

3.RT pulmonary hilum often prominent &

elevated------rt sided water fall

appearance

y ECHOCARDIOGRAPHY

1.The morphological left ventricle is

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TGA .. Acute Management :

y PGE-1 with no supplemental O2

Maintain ductus arteriosus patency, this will increase the effectivepulmonary blood flow, and thence increase the left atrial pressure,therefore inhance the left to right shunt at the atrial level

y Rashkind balloon atrial septostomy

Life saving procedure in the presence of inadequate atrial septal defect

y The arterial switch (Jantene) procedurey with re-implantation of the coronary artery to the new

aortic site.

y Atrial switch (Mustard orSenning operation) :y

the old style surgeryy Redirecting the pulmonary and systemic venous return to

result in a physiologically normal statey The right ventricle remains the systemic ventricley Rarely needed

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DOUBLE-OUTLET RIGHT VENTRICLE

y 50 percent of each semilunar valve arises from the morphological right ventricle.

y Clinical physiological responds depends on size & location of VSD and presence or absenceof PS

Major clinical patterns

1. with a subaortic VSD with PSPS---------- commonest

clinical scenario and management algorithm are similar or identical to that oftetralogy ofFallot

2. with a subaortic VSD ,no PS with lowpulmonaryvascularresistanceno PS with lowpulmonaryvascularresistance----resembles :nonrestrictiveperimembranousVSD

3. with a subaortic VSD ,no PS withhighpulmonaryno PS withhighpulmonaryvascularresistancevascularresistance -----

resembles: Eisenmenger syndrome

4. with a subpulmonary VSD with no PS(no PS(TaussigTaussig--Binganomaly)Binganomaly)----resembles :

TGA with nonrestrictiveVSD.

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TOTALANOMALOUS PULMONARY VENOUS CONNECTION

y All of the systemic and pulmonary venous return drains to the right atrium

y Most have symptoms during the first year of life,80 percent die before 1year of age if not treated

y Infradiaphragmaticcyanotic since birth

y Supradiaphragmaticcyanosis & CHF at 4-6wk

y Feature similar to ASD with

increased rt sided flow.

RV type impulse

S1-loud(T1)

Wide fixed splitting of s2

Accentuated P2

Midsystolic murmur at left 2nd ics

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y CXRfigure-of-8 or

snowman heart

due to enlargement ofthe heart and thepresence of a dilatedright superior vena cava,

innominate vein, and leftvertical vein.

ECG

1. Rt axis deviation

2. RAH &

3. RVH

ECHOCARDIOGRAPHY

1. Marked enlargement of the right ventricle and a small

left atrium.

2. An echo-free space representing the pulmonary

venous confluence can usually be seen behind the left

atrium.

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Treatment.

y Surgical correction of TAPVR is indicated during infancy.

y Before surgery, infants may be stabilized with prostaglandin E1 to dilate

the ductus venosus and the ductus arteriosus.

y Some may require balloon atrial septostomy, but it is of little or no

benefit in the presence of pulmonary venous obstruction.

y Surgically, the commonpulmonary venous trunk is anastomosed

directly to the left atrium, the ASD is closed, and the connection to the

systemic venous circuit is interrupted.

y Results have been generally good, even for critically ill neonates.

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Truncus Arteriosus

y In truncus arteriosus, a single arterial trunk (truncus arteriosus) arises from the

heart and supplies the systemic, pulmonary, and coronary circulations.y A VSD is always present, with the truncus overriding the defect and receiving

blood from both the right and left ventricles . Generally patients have increasedpulmonary blood flow

y Degree of cyanosis is mild and may not be evident clinically until late stage with

pulmonary vascular diseasey Presenting feature is congestive heart failure (tachypnia, hepatomegally)

y Exam is significant fory Single S2y Ejection click of the abnormal truncal valve

y Systolic murmur of truncal valve stenosis if presenty Diaastolic murmur of truncal valve insufficiencyy Gallop

y CXR : Cardiomegally , increased pulmonary circulation

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Types:

y pulmonary arteries(P.A) may arise together

from the posterior left side of the persistent

truncus arteriosus and then divide into left

and right P.A(type Itruncus arteriosus).

y In types II and III truncus arteriosus, no main

P.A is present, and the right and left P.As

arise from separate orifices in theposterior(type II)orlateral(type III)aspects of the

truncus arteriosus.

y Type IVtruncus has no identifiable

connection between the heart and P.A, andpulmonary blood flow is derived from major

aortopulmonary collateral arteries arising from

the transverse or descending aorta; also been

calledpseudotruncusbut is essentially a form of

pulmonary atresia with a VSD

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y Therapeutic Management:

a. Medical Management: aimed at reducing the effects of CHF and

preventing polycythemiaCHF is treated with digoxin and diuretics.

b. Surgical Management:

1. Pulmonary artery banding for NBs who do not respond to early medicalmanagement.

2. Rastelli repair- Total corrective repair includes closing the VSD andplacement of a conduit from the RV to the pulmonary artery.

3. Valvuloplasty of the truncal valve which is the new aortic valve may be

performed to improve valvular competence. Blood flow postop isnormal.

4. Conduit replacement is necessary as the child grows and a future truncalvalve repair or replacement may be needed.

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Single Ventricle (Double-Inlet Ventricle,

Univentricular Heart)

y With a single ventricle, both atria empty through a common atrioventricular

valve or via two separate valves into a single ventricular chamber, with total

mixing ofsystemic and pulmonary venous return.

y The aorta and pulmonary artery both arise from this single chamber.

y Variable Cl. features and depends on the associated intracardiac anomalies.y If pulmonary outflow is obstructed, the findings may be similar to those of

TOF: marked cyanosis without heart failure.

y If pulmonary outflow is unobstructed, the findings are similar to those of

transposition with VSD: minimal cyanosis with marked heart failure.

y The eventual development of pulmonary vascular disease reduces pulmonary

blood flow so that the cyanosis increases and signs of cardiac failure appear to

improve (Eisenmenger physiology

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y Findings on the ECG are nonspecific.

y CXR confirms the degree of cardiomegaly. In the absence of pulmonary stenosis,

pulmonary vasculature is increased, whereas in the presence of pulmonarystenosis, pulmonary vasculature is diminished.

y Absence or near absence of the ventricular septum is the principal echocardiographic sign.

y Treatment :

y If pulmonary stenosis is severe, an aortopulmonary shunt is indicated.

y If pulmonary blood flow is unrestricted, pulmonary arterial banding is used tocontrol heart failure and prevent progressive pulmonary vascular disease. TheGlenn shunt followed by a modified Fontan operation (cavopulmonary isolationprocedure, is the ultimate treatment of choice.

y If subaortic stenosis is present because of a restrictive connection to a

rudimentary outflow chamber, surgical relief can be provided by anastomosingthe proximal pulmonary artery to the side of the ascending aorta (Damus-Stansyl-Kaye operation).

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Hypoplastic Left Heart Syndromey Accounts for 1% of all CHDs. It is seen more frequently in males than in females.

Approximately 95% of all affected infants who are untreated will die within the 1st months

of life.

y Inadequate development of the left side of the heart results in only one effective ventricle.

The syndrome may include aortic valve atresia, hypoplasia of the LV, atresia or hypoplasia

of the ascending aorta, and mitral valve stenosis or atresia. Most infants have intact

ventricular septum.

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y Manifestations:

y Most infants present (within the first few days of life) with tachypnea and early CHF from

increased pulmonary blood flow and as the ductus arteriosus begins to close, systemic

hypoperfusion and shock.

y The infant appears grayish blue in color with dyspnea and hypotension.

y Therapeutic Management:

Die within the first month of life without surgical intervention.

a.MedicalManagement: correction of the acid-base and electrolyte imbalances and

reestablishment of ductal patency with PGE1.b.SurgicalManagement

y Norwood Procedure a three-step palliative repair.

y An alternative therapy is cardiac transplantation, either in the immediate neonatal period,

thereby obviating stage I of the Norwood procedure, or after a successful stage I Norwood

procedure is performed as a bridge to transplantation.

Cardiac transplantation as a single, definitive correction has been successful with 85%

operative survival rate and 81% 5-year survival rate.

The scarcity of neonatal donor heart, however, greatly limits the number of infants who may

receive transplant.

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Eisenmenger syndromey DEFINITION: it is a pulmonary vascular obstructive disease that develops as a consequence of

a large preexisting left-to-right shunt such that pulmonary artery pressures approach systemic levels

and the direction of the flow becomes bidirectional or right to left.y Causes 1.Simple--ASD, VSD, and PDA

2.Complex--AV septal defect, T.arteriosus, aortopulmonary window, and univentricular heart.

y The high pulmonary vascular resistance is usually established in infancy (by age 2 years, except in ASD)and is sometimes present from birth.

SYMPTOMS

y Cyanosis --- central1st decadeVSD

2nd decade---PDA

3rd decadeASD

y Exercise intolerance (dyspnea and fatigue) is proportional to the degree of hypoxemia or cyanosis.

y Palpitations in nearly half the patients (atrial fibrillation/flutter in 35 percent,

Ventricular tachycardia in up to 10 percent);

y Hemoptysis in about 20 percent; due to bleeding bronchial vessels or pulmonary infarction.

y Pulmonary thromboembolism, angina, syncope, and endocarditis in about 10 percent each; and

y congestive heart failure after 40 year

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PhysicalexaminationCentral cyanosis and clubbing of the nail beds.

Pink nail beds on the right (>left) hand and cyanosis and clubbing of both feet,so-called differential cyanosis.

JVP normal or elevated.

Signs of pulmonary hypertension1. a right ventricular heave, palpable and loud P2, and a right-sided S42. pulmonary ejection click and a soft and scratchy systolic ejection murmur,attributable to dilation of the pulmonary trunk, and

3. high-pitched decrescendo diastolic murmur of pulmonary regurgitation

(Graham Steell) are audible.Peripheral edema is absent until right-sided heart failure ensues.

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CXR

1. Dilated central pulmonary arteries with rapid tapering of the peripheral pulmonary vasculature

are the radiographic hallmarks of Eisenmenger syndrome(E.S).

2. Pulmonary artery calcification in long-standing PAH

3. E.S due to VSD or PDA usually has a normal or slightly increased cardiothoracic ratio.

4. E.S due to an ASD typically has a large cardiothoracic ratio due to right atrial and ventricular

dilation, along with an inconspicuous aorta.

5. Calcification of the duct & prominent aortic knuckle -----Eisenmenger PDA.

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CARDIACCATHETERIZATION.

1. provides direct measurement of the pulmonary artery pressure, documenting the

existence of severe pulmonary hypertension,

2. Administration of pulmonary arterial vasodilators (O2, nitric oxide, prostaglandin I2[epoprostenol]) can discriminate among patients in whom surgical repair is

contraindicated and those with reversible pulmonary hypertension who may benefit fromsurgical repair.

OPEN-LUNG BIOPSY.

when reversibility of the pulmonary hypertension is uncertain from the hemodynamic

data.

ECG.

1. Peaked P waves consistent with right atrial

overload2. right ventricular hypertrophy with right axis

deviation

3. Atrial arrhythmias can be present.

ECHOCARDIOGRAPHY.

1. The intracardiac defect should be seen readily

along with bidirectional shunting.

2. A pulmonary hypertensive PDA is not easilyseen.

3. Evidence of pulmonary hypertension is found.

4. Assessment of pulmonary right ventricular

function adds prognostic value.

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Summary

Cyanotic heart disease commonly presents in the neonatal period.

Rapid diagnosis and referral are mandatory because patients can becomeunstable very quickly.

Prostaglandin E1 promotes blood flow through the ductus arteriosus andis a useful stabilizing maneuver in many, but not all, of these conditions.

Echocardiography and cardiac catheterization are the diagnostic tools ofchoice

Early surgical intervention is often required, either for palliation or fordefinitive correction.

Current surgical therapy for most lesions has evolved from earlypalliation and delayed repair to complete correction in early infancywith improved morbidity and mortality.

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