CONGENITAL HEART CONGENITAL HEART DISEASESDISEASES

ETSEGEGENET GEDLUETSEGEGENET GEDLUDepartmentDepartment of Pediatrics and of Pediatrics and Child HeathChild Heath

AAU , Collage of Heath AAU , Collage of Heath sciences sciences

January 2011January 2011

ObjectiveObjective To revise the fetal circulation in summeryTo revise the fetal circulation in summery

To discuss about the Classification of congenital To discuss about the Classification of congenital heart diseaseheart disease

Discuses the common acyanotic and cyanotic Discuses the common acyanotic and cyanotic congenital heart diseases congenital heart diseases epidemiology, epidemiology, pathophysiology pathophysiology ,clinical presentation,,clinical presentation, Investigation and principles of management. of Investigation and principles of management. of common congenital Heart disease common congenital Heart disease

Cont ..Cont ..At the end of the lecture a student should be able At the end of the lecture a student should be able

to discus to discus Basic fetal circulation Basic fetal circulation classification of CHDclassification of CHD The epidemiologyThe epidemiology pathophysiologypathophysiology clinical presentation and physical findings of clinical presentation and physical findings of

common CHDcommon CHD The principles of management including The principles of management including

relevant investigations for respective lesions .relevant investigations for respective lesions .

Fetal circulationFetal circulationThe right and left ventricles exist in a parallel The right and left ventricles exist in a parallel circuit.circuit.

In the fetus gas and metabolite exchange In the fetus gas and metabolite exchange provided by the provided by the placentaplacenta

Parallel circuit maintainedParallel circuit maintained Foramen ovaleForamen ovale Ductus arteriosus Ductus arteriosus Ductus venosus Ductus venosus

Foetal CirculationFoetal Circulation Arterial blood leaves the Arterial blood leaves the

placenta via the umbilical placenta via the umbilical veinvein

This branches and delivers This branches and delivers blood to the IVC by way of blood to the IVC by way of the ductus venosus the ductus venosus

Blood then goes into the right Blood then goes into the right atrium, 30% goes across the atrium, 30% goes across the foramen ovale, the rest to the foramen ovale, the rest to the RV then to PARV then to PA

Instead of going to the lungs, Instead of going to the lungs, 85% goes through the PDA 85% goes through the PDA to the aortato the aorta

Transitional circulationTransitional circulationInterruption of Umbilical cordInterruption of Umbilical cord

Removal of the low resistance placental circulation result in Removal of the low resistance placental circulation result in an an Increased systemic vascular resistanceIncreased systemic vascular resistance

lack of blood flow through the placenta leads to closure of lack of blood flow through the placenta leads to closure of Ductus venosus Ductus venosus ((the ligamentum venosumthe ligamentum venosum))

Expansion of lungs :Expansion of lungs :

Mechanical expansion of the lungs and increased arterial Mechanical expansion of the lungs and increased arterial P02 result in a rapid P02 result in a rapid decrease in pulmonary vascular decrease in pulmonary vascular resistance. resistance.

The increased blood volume from the pulmonary circulation The increased blood volume from the pulmonary circulation increased the LA volume and pressure sufficiently to increased the LA volume and pressure sufficiently to close the foramen ovalclose the foramen ovalee ((fossa ovalisfossa ovalis))

PPDA closureDA closure : The ductus flow become left to right and later the : The ductus flow become left to right and later the ductus will obliterated.(ductus will obliterated.(ligamentum arteriosusligamentum arteriosus) )

Transitional circulationTransitional circulationThe right ventricle is coupled with low The right ventricle is coupled with low resistance pulmonary circulation and its wall resistance pulmonary circulation and its wall thickness and mass decreased thickness and mass decreased

The left ventricle coupled to high resistance The left ventricle coupled to high resistance systemic circulation and its wall thickness and systemic circulation and its wall thickness and mass increased and deliver the entire mass increased and deliver the entire systemic cardiac out put.systemic cardiac out put. There is a change from fetal haemoglobin to There is a change from fetal haemoglobin to adult haemoglobinadult haemoglobin

Congenital Heart diseaseCongenital Heart diseaseIntroduction:Introduction:Def: Def: Structural or functional heart disease Structural or functional heart disease

that present at birth.that present at birth. It is not static ,there is always a continuous It is not static ,there is always a continuous

anatomical or physiological changeanatomical or physiological change

The incidence is higher in abortus and still The incidence is higher in abortus and still births births

Estimate in live birth range from 4-10.2 per Estimate in live birth range from 4-10.2 per 1000live birth1000live birth. .

Cont….Cont….

The incidence of specific type of CHD The incidence of specific type of CHD varies from one country to another varies from one country to another

Specific aetiology only known 10% Specific aetiology only known 10% 8% genetic 8% genetic 2% environmental (rubella, foetal-alcohol 2% environmental (rubella, foetal-alcohol

syndromesyndrome 90% Multifactorial inheritance 90% Multifactorial inheritance

Prevalence of Congenital heart Prevalence of Congenital heart Disease (chart review)Disease (chart review)

DevelopedDeveloped

countriescountries

Ethio-Swedish Ethio-Swedish 19861986

TAHTAH

2003-082003-08

VSDVSD 28.3%28.3% 4141 28.4%28.4%

PDAPDA 12%12% 13%13% 16.5%16.5%

ASDASD 10.7%10.7% 13.6%13.6% 13%13%

COACOA 8.8%8.8% <2%<2% 1.8%1.8%

TOFTOF 7%7% 9%9% 5.7%5.7%

PSPS 6%6% 9.9%9.9% 8.8%8.8%

ASAS 2.3%2.3% 3.5%3.5% 3.4%3.4%

cont.….cont.….

The risk of recurrence in siblings varies The risk of recurrence in siblings varies from 1-4%from 1-4%

Third Pregnancy 20-30% Third Pregnancy 20-30% Parents with CHD 4-6%Parents with CHD 4-6% Varies with type of inheritanceVaries with type of inheritance Except PDA and ASD males are more Except PDA and ASD males are more

affected than females.affected than females.

Congenital Heart Disease Congenital Heart Disease TypesTypes

I.I. Lesions with Increased Pulmonary Lesions with Increased Pulmonary Blood Flow Blood Flow Heart failure or Heart failure or Pulmonary vascular resistance Pulmonary vascular resistance

II.II. Lesions with Decreased Pulmonary Lesions with Decreased Pulmonary Blood FlowBlood Flow

III.III. Lesions with Inadequate Systemic FlowLesions with Inadequate Systemic Flow

Common Congenital Heart Common Congenital Heart DiseasesDiseases

Acynotic Acynotic Shunts ( L to R) :Shunts ( L to R) :• ASDASD• VSDVSD• PDAPDA• AVSDAVSDStenosis:Stenosis:• ASAS• PSPS• CoarctationCoarctation

• cyanosiscyanosis• TOFTOF• TGATGA• Tricuspid atresiaTricuspid atresia• TruncusTruncus• TAPVRTAPVR• Ebstein’sEbstein’s• Single ventricle eg. Single ventricle eg.

HLHSHLHS

Clinical manifestation of CHDClinical manifestation of CHD

CHD suspected in any child with:CHD suspected in any child with:

Feeding difficultyFeeding difficulty Recurrent attack of respiratory tract infectionRecurrent attack of respiratory tract infection Growth failureGrowth failure Cyanosis unresponsive to 100% oxygenCyanosis unresponsive to 100% oxygen TachycardiaTachycardia Respiratory distressRespiratory distress Rhythm disturbanceRhythm disturbance Murmur ( Murmur ( absence of murmur doesn't rule out or in CHD)absence of murmur doesn't rule out or in CHD)

ManagementManagement of CHD of CHDGeneral principle General principle 1) Treatment of Congestive Heart Failure1) Treatment of Congestive Heart Failure DiureticsDiuretics Inotropic supportInotropic support After load reduction After load reduction 2) Correction of underlying defect (timing depend on the type 2) Correction of underlying defect (timing depend on the type

and severityand severity

3)Prevention and treatment of complication3)Prevention and treatment of complication

Pulmonary HPT : early surgical correctionPulmonary HPT : early surgical correction Infective endocarditis: Administration of antibiotic Infective endocarditis: Administration of antibiotic

chemoprophylaxis as indicated.chemoprophylaxis as indicated. Non infective thromboembolism : prevent polycythemiaNon infective thromboembolism : prevent polycythemia polycythemia :partial exchange transfusionpolycythemia :partial exchange transfusion

Counselling of parents on the risk of recurrenceCounselling of parents on the risk of recurrence

VentricularVentricular Septal Defect Septal Defect

Most common CHDMost common CHD Both sexes are equally Both sexes are equally

affectedaffected Incidence of 1/3000 Incidence of 1/3000 Can be single or Can be single or

multiplemultiple Can be associated with Can be associated with

other congenital heart other congenital heart diseases diseases

VSD cont.…VSD cont.…

Types of VSDTypes of VSD 70% membranous close to pulmonary 70% membranous close to pulmonary

valve and Pulmonary arteryvalve and Pulmonary artery 20% muscular20% muscular 5% Aortic valve (sub aortic)5% Aortic valve (sub aortic) 5% near junction of Mitral and tricuspid 5% near junction of Mitral and tricuspid

valve (A-V canal defect)valve (A-V canal defect)

ClinicalClinical manifestations manifestations

I) I) Asymptomatic:Asymptomatic: Small VSD , trivial shunt ,the pulmonary Small VSD , trivial shunt ,the pulmonary

pressure is normalpressure is normal Loud harsh Holosystolic murmur at LLSB, Loud harsh Holosystolic murmur at LLSB,

with thrillwith thrill X-ray is normalX-ray is normal EKG normalEKG normal

VSD CONT….VSD CONT….II ) II ) Large defects:Large defects: Excessive pulmonary blood flow lead to Excessive pulmonary blood flow lead to

pulmonary hypertensionpulmonary hypertension Dyspnea, feeding difficultyDyspnea, feeding difficulty Poor growthPoor growth Profuse perspirationProfuse perspiration Recurrent pulmonary infectionRecurrent pulmonary infection

Physical ExaminationPhysical Examination:: Prominence of the precordiumProminence of the precordium Palpable parasternal liftPalpable parasternal lift Apical trust with systolic thrillApical trust with systolic thrill Holosystolic murmur at LLSB less harsh Holosystolic murmur at LLSB less harsh

and more blowingand more blowing Diastolic murmur at the apex Diastolic murmur at the apex Increased P2 indicate pulmonary HPT.Increased P2 indicate pulmonary HPT.

VSD cont….VSD cont….

Severity depend on :Severity depend on : The The sizesize of the defect of the defect Level of pulmonary resistance to systemic Level of pulmonary resistance to systemic

resistance.resistance. Defects < 0.5 restrictiveDefects < 0.5 restrictive > 1cm sq non restrictive> 1cm sq non restrictive

Majority closes spontaneouslyMajority closes spontaneously Large defects lead to CHF at early age Large defects lead to CHF at early age

Chest X-Ray (VSD)Chest X-Ray (VSD)

Cardiomegally Cardiomegally

(LA, LV,RV,PA)(LA, LV,RV,PA)

Increased vascular Increased vascular markingmarking

Diagnostics cont…Diagnostics cont…

ECG:ECG: P wave peaked P wave peaked

and notchedand notched Left ventricular Left ventricular

hypertrophy hypertrophy (biventricular)(biventricular)

Echocardiography (VSD)Echocardiography (VSD)

Position and size Position and size of VSDof VSD

Chamber sizeChamber size Pressure Pressure

gradient across gradient across the defectthe defect

Direction of Direction of shuntshunt

CLINICAL COURSE:CLINICAL COURSE:

Small sized defectsSmall sized defects are closed spontaneously in the first year of lifeare closed spontaneously in the first year of life The risk of endocarditis is independent of the sizeThe risk of endocarditis is independent of the size

Moderate to large defects:Moderate to large defects: decreased in size but not closeddecreased in size but not closed Heart failure and growth failure is common at the early Heart failure and growth failure is common at the early

age age Risk of pulmonary hypertension lead to pulmonary Risk of pulmonary hypertension lead to pulmonary

vascular diseases.vascular diseases. Eisenmengers syndrome due to reversal of shunt which Eisenmengers syndrome due to reversal of shunt which

presented with absence of thrill and cyanosis, decreased presented with absence of thrill and cyanosis, decreased heart size. heart size.

TreatmentTreatment

Small size Small size Reassurance Reassurance No surgical treatmentNo surgical treatment Maintain integrity of primary and permanent teethMaintain integrity of primary and permanent teeth Give anti- infective endocarditis prophylaxisGive anti- infective endocarditis prophylaxis

Antibiotic prophylaxis beforeAntibiotic prophylaxis before dental visit dental visit TonsillectomyTonsillectomy instrumentation of GUT,GITinstrumentation of GUT,GIT

Treatment cont….Treatment cont….

Large VSD:Large VSD: Control CHFControl CHF Prevent development of Pulmonary vascular Prevent development of Pulmonary vascular

disease.disease. Surgical closure in the first year of life (6M-Surgical closure in the first year of life (6M-

12M).12M). Device closure of the VSD with Amplatzer device Device closure of the VSD with Amplatzer device

Umbrella)Umbrella) Palliative : pulmonary banding if surgery is not Palliative : pulmonary banding if surgery is not

possible for the time beingpossible for the time being

Patent Ductus Arteriosus(PDA)Patent Ductus Arteriosus(PDA) During foetal life blood from During foetal life blood from

PA shunted through the DA in PA shunted through the DA in to the Aorta.to the Aorta.

After birth closed functionallyAfter birth closed functionally Prematurity and hypoxia Prematurity and hypoxia

predispose for patenceypredispose for patencey Female are more affected Female are more affected

than males Commonly than males Commonly associated with rubella of the associated with rubella of the mother mother

Isolated PDAs are common in Isolated PDAs are common in high altitudehigh altitude

PathophysiologyPathophysiology

Blood flow from the aorta to the pulmonary.Blood flow from the aorta to the pulmonary.

Extent of the shunt depend on Extent of the shunt depend on size of the ductus size of the ductus ratio of pulmonary and systemic ratio of pulmonary and systemic

vascular resistancevascular resistance

Clinical manifestationClinical manifestationDepend on the :Depend on the : Size of the defect and direction of flowSize of the defect and direction of flow Small defects no symptomSmall defects no symptom Large defect result in Large left to right shunt Large defect result in Large left to right shunt

CHFCHFGrowth FailureGrowth FailureRepeated ARIRepeated ARIReversal of shunt ,(Eisenmengers ) result in Reversal of shunt ,(Eisenmengers ) result in

dyspnoea and cyanosisdyspnoea and cyanosis

Physical EXAMINATIONPhysical EXAMINATION Bounding pulseBounding pulse Wide pulse pressureWide pulse pressure Heave , thrill in the 2nd icsHeave , thrill in the 2nd ics Continuous machinery murmur 2nd ics Continuous machinery murmur 2nd ics EKGEKG: bi-ventricular hyperthrophy: bi-ventricular hyperthrophy X-rayX-ray: prominent PA, increases PA marking : prominent PA, increases PA marking

enlarged chambers(LA,LV),enlarged chambers(LA,LV), ECHOECHO: size of the PDA, direction of flow, : size of the PDA, direction of flow,

chamber sizechamber size CatheterizationCatheterization: a step up oxygen saturation, : a step up oxygen saturation,

PDA anatomy in angiographyPDA anatomy in angiography

Clinical course:Clinical course:

Small defects : few or no cardiac symptomsSmall defects : few or no cardiac symptoms Large defects:Large defects:

CHFCHF Infective endocarditisInfective endocarditis Systemic emboliSystemic emboli Calcification of the ductusCalcification of the ductus Non infective thrombosis with embolizationNon infective thrombosis with embolization Paradoxical emboliParadoxical emboli

Eisenmenger syndrome if left untreatedEisenmenger syndrome if left untreated

TreatmentTreatment

Medical therapy:Medical therapy: Congestive heart failure treatmentCongestive heart failure treatment infective endocarditis prophylaxisinfective endocarditis prophylaxis Surgical closure of the PDA (banding)Surgical closure of the PDA (banding) Closure of the PDA coil embolization or Closure of the PDA coil embolization or

device closure without thoracotomydevice closure without thoracotomy

Coarctation of the AortaCoarctation of the Aorta Occurs at any point from Occurs at any point from

transverse arch to iliac bifurcationtransverse arch to iliac bifurcation

98% below the origin of left sub 98% below the origin of left sub clavian at the origin of Ductus clavian at the origin of Ductus

Male to female ratio: 2:1Male to female ratio: 2:1Associated with:Associated with: Turner syndrome Turner syndrome Bi cuspid aortic valve (70%)Bi cuspid aortic valve (70%) Left sided obstructive lesions Left sided obstructive lesions

( Shone complex)( Shone complex) Mitral valve abnormality Mitral valve abnormality Sub aortic stenosisSub aortic stenosis

PathophysiologyPathophysiology Collaterals develop to bypass the Collaterals develop to bypass the

obstruction.obstruction. Hypertension of the aortic branch proximal Hypertension of the aortic branch proximal

to coarctationto coarctation In Pre ductal type the RV blood ejected In Pre ductal type the RV blood ejected

through the ductus to supply the through the ductus to supply the descending aorta lead to differential descending aorta lead to differential cyanosis. cyanosis.

Clinical ManifestationsClinical Manifestations Severe critical stenosis the neonate Severe critical stenosis the neonate

present with evidence of CHF if not present with evidence of CHF if not corrected surgically result in death.corrected surgically result in death.

Past the neonatal period Usually Past the neonatal period Usually asymptomaticasymptomatic

Older children:Older children:HeadachesHeadachesEpistaxisEpistaxisClaudication, cold feetClaudication, cold feet

Physical ExaminationPhysical Examination::

Weak or absent femoral pulsesWeak or absent femoral pulses Increased B/P in the upper extremitiesIncreased B/P in the upper extremities B/P difference between upper and lower B/P difference between upper and lower

extremitiesextremities Radio Femoral pulse delay ( Collaterals)Radio Femoral pulse delay ( Collaterals) A2 is loud , systolic murmur 3rd and 4th A2 is loud , systolic murmur 3rd and 4th

ULSBULSB

Cont….Cont….

Chest x_ray:Chest x_ray: Dilated descending Dilated descending

Aorta, enlarged LV.Aorta, enlarged LV. Rib notchingRib notching

ECG: normal in ECG: normal in childhood, later LV childhood, later LV hypertrophyhypertrophy..

ECHOECHO

Measure the stenotic Measure the stenotic areaarea

gradientgradient

TreatmentTreatment

Neonatal: closure of the ductus lead to hypo Neonatal: closure of the ductus lead to hypo perfussion and acidosis, thus give infusion of perfussion and acidosis, thus give infusion of prostaglandin to reopen the ductus, after prostaglandin to reopen the ductus, after stabilization stabilization surgical treatmentsurgical treatment..

Older children with CHF and no hypertension Older children with CHF and no hypertension medical treatment followed by medical treatment followed by surgery or surgery or angioplasty.angioplasty.

Re -stenosis balloon angioplasty is safe.Re -stenosis balloon angioplasty is safe.

Tetralogy of FallotTetralogy of Fallot

Common cyanotic Common cyanotic congenital cardiac congenital cardiac anomalyanomaly

Four anatomical Four anatomical components of TOF:components of TOF:

VSDVSD Overriding AortaOverriding Aorta Right ventricular outflow Right ventricular outflow

obstructionobstruction Right ventricular Right ventricular

hypertrophyhypertrophy

Pathophysiology:Pathophysiology:

• Severity directly proportional to the degree Severity directly proportional to the degree of RVOT obstruction.of RVOT obstruction.

• Change in pulmonary and systemic Change in pulmonary and systemic vascular resistance and the degree of vascular resistance and the degree of RVOT obstruction affect degree of R-L RVOT obstruction affect degree of R-L shunt.shunt.

• Infundibular stenosis is progressive.Infundibular stenosis is progressive.

Clinical ManifestationsClinical ManifestationsVariable depend on RVOT obstructionVariable depend on RVOT obstruction pink to cyanosispink to cyanosis

CHF is not a usual manifestation of TOFCHF is not a usual manifestation of TOF

SquattingSquatting

Dyspnoea on exertionDyspnoea on exertion

Hypoxic spellsHypoxic spells

Growth failureGrowth failure

Physical Examination:Physical Examination:

Cyanosis variableCyanosis variableClubbingClubbingUsually S2 is single,Usually S2 is single,Quite precordiumQuite precordiumThrill at the pulmonary area(-+)Thrill at the pulmonary area(-+)Systolic ejection murmur at the Systolic ejection murmur at the pulmonary area(LUSB)pulmonary area(LUSB)

Clinical pictures cont….Clinical pictures cont….

Electrocardiography (TOF)Electrocardiography (TOF)

Right axis deviationRight axis deviation

Right atrial and right Right atrial and right ventricular ventricular enlargementenlargement

Chest x ray (TOF)Chest x ray (TOF)

Normal sized, boot Normal sized, boot shaped heart.shaped heart.

Reduced pulmonary Reduced pulmonary vascular marking vascular marking (oligemic depend on (oligemic depend on the degree of RVOT the degree of RVOT obstruction.obstruction.

Echocardiography (TOF)Echocardiography (TOF) Location and size of the VSDLocation and size of the VSD The aortic overrideThe aortic override The degree of RVOT The degree of RVOT

obstructionobstruction The size of pulmonary valve The size of pulmonary valve

annulusannulus Look for additional pulmonary Look for additional pulmonary

artery branch stenosis.artery branch stenosis. Look for other associated Look for other associated

anomaliesanomalies Right aortic archRight aortic arch Coronary arteries anatomic Coronary arteries anatomic

variationsvariations

Cardiac catheterization Cardiac catheterization and Angiographyand Angiography

HemodynanmicHemodynanmic

Anatomical informationAnatomical information

TherapeuticTherapeutic

ComplicationsComplications

Cardiovascular accidents : occurs in 4-5% of Cardiovascular accidents : occurs in 4-5% of cases is due to cerebral embolism.cases is due to cerebral embolism.

Brain abscess: rare in the first two years of Brain abscess: rare in the first two years of life may be due to small cerebral infract which life may be due to small cerebral infract which is super infected due to bacteraemia.is super infected due to bacteraemia.

Infective endocarditisInfective endocarditis

PolycythemiaPolycythemia

ManagementManagementMedical managementMedical management::

AA) Neonatal period if pulmonary flow is dependent of DA, ) Neonatal period if pulmonary flow is dependent of DA, give prostaglandin to prevent ductal closure and followed by give prostaglandin to prevent ductal closure and followed by palliative shunt (B-T Shunt)palliative shunt (B-T Shunt)

B)Recognition and treatment of hyper cyanotic spell.B)Recognition and treatment of hyper cyanotic spell.Knee chest positioning of the patientKnee chest positioning of the patientAdministration of oxygenAdministration of oxygenVolume expansionVolume expansionCorrection of acidosisCorrection of acidosisSedation with morphineSedation with morphinePropranolol Propranolol C) Treat infective endocarditis C) Treat infective endocarditis D) Anaemia should be correctedD) Anaemia should be correctedE) Severe polycythemia correction with phlebotomy should E) Severe polycythemia correction with phlebotomy should be done.be done.

ManagementManagement

Surgical Management:Surgical Management:

A) Palliative: Modified Blalock-Taussig shunt A) Palliative: Modified Blalock-Taussig shunt between pulmonary artery branch and between pulmonary artery branch and subclavian artery subclavian artery

B) Corrective surgery : closing of the VSD B) Corrective surgery : closing of the VSD and reliving all possible source of RVOT and reliving all possible source of RVOT obstruction.obstruction.

CompleteTranspostion of the CompleteTranspostion of the Great ArteriesGreat Arteries

The great arteries arise The great arteries arise from morphologically from morphologically wrong ventricles. (The wrong ventricles. (The aorta arise from the RV aorta arise from the RV and Pulmonary arises and Pulmonary arises from LV in the setting of a from LV in the setting of a concordant concordant atrioventricular atrioventricular connection.connection.

Transposition of the Great ArteriesTransposition of the Great Arteries

TGA occurs in 8-9% TGA occurs in 8-9% of CHDof CHD

Male are affected Male are affected than Femalesthan Females

Different variety existDifferent variety exist

HemodynamicsHemodynamicsThe RV pressure is systemicThe RV pressure is systemic

The blood returning from the lung pass to the lung via The blood returning from the lung pass to the lung via PAPA

Systemic venous return passes back to the systemic Systemic venous return passes back to the systemic circulation via the aorta.circulation via the aorta.

Both ventricles are volume overloaded.Both ventricles are volume overloaded.

The right ventricle also pressure overloads and result The right ventricle also pressure overloads and result in CHFin CHF

Survival depend on associated Large ASD, VSD,PDA.Survival depend on associated Large ASD, VSD,PDA.

Clinical features:Clinical features:Cyanosis detected 87% of the neonates Cyanosis detected 87% of the neonates immediately ; rest later at the age of 1 to 3 immediately ; rest later at the age of 1 to 3 month and is progressive.month and is progressive.ClubbingClubbingSquatting only 20% of patientsSquatting only 20% of patientsDyspnoeaDyspnoeaCardiac failure Cardiac failure CoughCough

Physical findingsPhysical findings1)TGA with no VSD1)TGA with no VSDCyanosis intense Cyanosis intense Precordial liftPrecordial liftLoud first soundLoud first soundSplitted S2Splitted S2Murmur may not be heardMurmur may not be heard

Radiology (TGA)Radiology (TGA)

Increased pulmonary Increased pulmonary vascular markingvascular marking

Large heart with egg - Large heart with egg - on side appearance.on side appearance.

Electrocardiography (TGA)Electrocardiography (TGA)

TGA with intact ventricular septum:TGA with intact ventricular septum: P wave tallP wave tall P upright T wave in lead V1 and V3RP upright T wave in lead V1 and V3R Right ventricular hypertrophyRight ventricular hypertrophy Right axis deviationRight axis deviation

Echocardiography(TGA)Echocardiography(TGA)

Shows the origion of :Shows the origion of : pulmonary artery from pulmonary artery from

the LVthe LV Aorta from RVAorta from RV Associated lesions Associated lesions

like VSD and ASD be like VSD and ASD be identified.identified.

Coronary artery Coronary artery anatomyanatomy

PrognosisPrognosis

Patient die of anoxia and or CHF in the Patient die of anoxia and or CHF in the first 6 month of life if there is little first 6 month of life if there is little communication.communication.

ManagementManagementMedical managementMedical management:: Prostaglandin to keep the DA open until Prostaglandin to keep the DA open until

palliation donepalliation done Give oxygenGive oxygen Treat metabolic acidosis with bicarbonate.Treat metabolic acidosis with bicarbonate.SurgicalSurgical:: Palliative balloon septostomy creating Palliative balloon septostomy creating

/increasing ASD Rashkind procedure./increasing ASD Rashkind procedure. Arterial Switch (Jaten Procedure)Arterial Switch (Jaten Procedure) Atrial switch ( Mustard procedure)Atrial switch ( Mustard procedure)