classificationandcyanoticheartdisease-131231035553-phpapp01

8/12/2019 classificationandcyanoticheartdisease-131231035553-phpapp01

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Cyanotic congenitalheart disease


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Classification of congenital heartdiseases

Group I : Left to right shuntsGroup II: Right to lefts shunts

Group III: Obstructive lesions


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Left to right shunts Atrial Septal Defect Ventricular Septal Defect

Patent Ductus Arteriosus


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Right to Left Shunts1) Tetralogy of Fallot2) Tricuspid atresia3) Ebsteins anomaly

4) Transposition of Great Vessels5) Truncus Arteriosus6) Total Anomalous Pulmonary Venous

Return (TAPVR)


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Obstructive Lesions Aortic stenosis Coarctation of the Aorta

Pulmonic Stenosis


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Cyanotic heart disease

Right to Left Shunt


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Who is this guy?


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TIENNE-LOUIS ARTHUR FALLOT !

a French physician,

1888 Fallotaccurately describedin detail the fouranatomicalcharacteristics oftetralogy of Fallot.


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Tetralogy OF Fallot Most common cyanotic heart disease!

75%!


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TOF4 component!

Imagine this is a HEART!


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TOF1) Vetricular Septal Defect


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TOF1) Vetricular Septal Defect2) Pulmonic Stenosis


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3) Overriding of dextroposed aorta


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3) Overriding of dextroposed aorta4) Right Ventricular hypertrophy


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Concentric R ventricularhypertrophy withoutcardiac enlargement


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Concentric R ventricularhypertrophy withoutcardiac enlargement

Increase in right ventricular pressure*


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RV and LV pressuresbecomes identical


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There is little orno L to R shunt


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Hence, VSD is silent


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Hence, VSD is silent

Right ventricleinto pulmonaryartery acrosspulmonic stenosisproducing ejectionsystolic murmur


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Hence, themore severethe pulmonarystenosis


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The BIGGERthe Left toRIGHT shunt


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Less flow intothe pulmonaryartery


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Shorter theejectionsystolic murmur


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Shorter theejectionsystolic murmur

More cynosis because of less flow to the lung!


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Hence, Severity of cyanosis is directly

proportional to the severity of pulmonicstenosis

Intensity of the systolic murmur isinversely related to the severity ofpulmonic stenosis


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Congestive failure never occur* because

Right ventricle is effectively decompressed because of the ventricular septal defect.

* exception


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Congestive failure never occur* because

Right ventricle is effectively decompressed because of the ventricular septal defect.

* exception

1)Anemia2)Infective Endocarditis3)Systemic hypertension4)Unrelated myocarditis

complicating TOF5)Aortic or pulmonary valve

regurgitation


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Pulmonary obstruction results in delayed P2


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Pulmonary artery pressurereduce


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P2 become soft orinaudible


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P2 become soft orinaudible

(Second Sound) S2= A2 + P2Since P2 is inaudible, hence S2 = A2 + P2[S2 is single sound]

Aorta is displace anteriorly too, A2 becomeLOUD!


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Ascending aorta in TOF is large, resultsaortic ejection click


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Diastolic interval is clear

No S3 No S4


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Concentric rightventricular hypertrophyreduce thedistensibility of theright ventricle duringdiastole


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Concentric rightventricular hypertrophyreduce thedistensibility of theright ventricle duringdiastole

a waves become

prominent in JVP*

*but not too tall


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Clinical Picture Symptomatic any time after birth Paroxysmal attacks of dyspnea

Anoxic spells Predominantly after waking up

Child cry Dyspnea Blue Lose conscious

Convulsion Frequency varies fromonce a few days to manyattack everyday


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tet spell lethal, unpredictable episodes

The mechanism spasm of theinfundibular septum, which acutely worsens the RV outletobstruction.


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Dyspnea on exertion Exercise intolerance


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Sitting posture squatting Compensatory mechanism Squatting increases the peripheral vascular

resistance, which diminishes the

right-to-left shunt

increases pulmonary blood flow.


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Cyanosis during feeding Poor feeding fussiness, tachypnea, and agitation. Birth weight is low. Growth is retarded. Development and puberty may be delayed.


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Rarely, patient remain asymptomatic into

adult life.


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Physical examination Clubbing + Cyanosis (Variable) Squatting position

Scoliosis Common bulging left hemithorax


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Prominent a waves JVP Normal heart size

Mild parasternal impulse Systolic trill (30%)


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Retinal engorgement Hemoptysis


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ECG ECG


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ECG ECG


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ECG ECG wiLLiammoRRow


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ECG Right axis deviation (+120 to +150) Right or combined ventricular

hypertrophy Right atrial hypertrophy Partial or complete right bundle branch

block (especially true of patients aftersurgical repair)


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Coeur en sabot(boot-shaped heart)

secondary to upliftingof the cardiac apexfrom RVH

and the absence of anormal main pulmonaryartery segment


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Normal heart size dueto the lack ofpulmonary blood flowand congestive heartfailure


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Decreased pulmonaryvascularity


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Right atrialenlargement

Right-sided aortic arch(20-25% of patients)with indentation ofleftward-positionedtracheobronchialshadow


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Echocardiography Reveals a large VSD overriding aorta variable degrees of right ventricular

outflow tract (RVOT) obstruction


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Course and Complication

1) Each anoxic spell is potentially fatal2) Polycytemia

1) Cerebrovascular thrombosis3) Anoxic infaction of CNS

1) Neurological complication


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4) LUNG is an awesome filter.1) Bypassing it may not be a good idea!2) TOF, venous blood from gut, peripheral

system by pass the lung and re-entercirculation

3) Hence TOF can cause:

1) Brain Abcess2) Infective endocarditis3) Paradoxical embolism


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Management of anoxic spell

1) Knee chest position2) Humified O23) Be careful not to provoke the child

1) Especially you are bad at gaining IV access2) Ask for help from someone more experience3) Permit the baby to remain with mother

4) Morphine 0.1 -0.2 mg/Kg Subcutaneous5) Correct acidosis Sodium Bicarb IV


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6) Propanolol1) 0.1mg/kg/IV during spells2) 0.5 to 1.0 mg/kg/ 4-6hourly orally

7) Vasopressors: Methoxamine IM or IVdrip

8) Correct anemia9) GA is the last resort


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Palliative Surgery Blalock-Taussig shunt Pott procedure Waterston shunt


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Blalock Taussig Shunt Subclavian artery Pulmonary artery

anastomosis


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Modified Blalock Taussig Shunt Goretex graft


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Surgical Palliation


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Palliative operation prolong life Increase exercise tolerance


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Definitive operation

Closing the VSD Resecting infundibular 90% can return almost normal life after operation

Complication: RBBB Residual VSD Residual Pulmonary stenosis

Pulmonary regurgitation (pulmonary valve excised) Risk 5%

f


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Transposition of Great Areries(TGA)

Aorta originatingfrom the right ventricle, andpulmonary arteryoriginating fromthe left ventricle

Accounts for 5-7%of all congenitalheart disease


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TGA Survival is dependent on the presence of mixing

between the pulmonary and systemic circulation Atrial septal defect is essential for survival

50% of patients have a VSD Usually presents in the first day of life with

profound cyanosis

More common in boys


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TGA Exam :

cyanosis in anotherwise healthy

looking baby Loud S2 ( aorta is

anterior ) CXR :

Egg on side Narrow

mediastinum


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TGA .. Acute Management PGE-1 with no supplemental O2

Maintain ductus arteriosus patency, this willincrease the effective pulmonary blood flow,and thence increase the left atrial pressure,therefore inhance the left to right shunt at theatrial level

Balloon atrial septostomy

Life saving procedure in the presence ofinadequate atrial septal defect


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TGA .. Surgical Management

Arterial switch with re-implantation of the coronary artery to the

new aortic site. Atrial switch :

the old style surgery Redirecting the pulmonary and systemic venous

return to result in a physiologically normal state The right ventricle remains the systemic ventricle Rarely needed


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Truncus Arteriosus The presence of a

common trunk thatsupply the systemic,pulmonary andcoronary circulation

Almost alwaysassociated with VSD

1.2-2.5% of allcongenital heartdisease


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Truncus Arteriosus

There are

differentanatomical tupesof truncusarteriosus

This is relevant forsurgical repair


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Truncus Arteriosus Generally patients have increased

pulmonary blood flow Degree of cyanosis is mild and may not be

evident clinically until late stage withpulmonary vascular disease

Presenting feature is congestive heartfailure (tachypnia, hepatomegally)


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Truncus Arteriosus Exam is significant for

Single S2 Ejection click of the abnormal truncal valve

Systolic murmur of truncal valve stenosis ifpresent Diaastolic murmur of truncal valve

insufficiency

Gallop CXR : Cardiomegally , increased

pulmonary circulation


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Managment Acute management

No O2 to minimize pulmonary blood flow Diuretics Afterload reduction to inhance systemic blood flow

Surgical management: completerepair with VSD closure andconduit placement between theright ventricle and pulmonaryarteries Long term problems :

truncal valve dysfunction RV conduit obstruction


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Trcuspid Atresia Complete absence of

communication between the rightatrium and right ventricle

About 3 % of

congenital heartdisease


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Tricuspid Atresia There is an obligate interatrial communication Usually associated with VSD The pulmonary blood flow is dependent on the

size of the VSD Pulmonary blood flow can be increased or

decreased causing variable presenting symptoms If there is no VSD ( also called Hypoplastic right

ventricle) the pulmonary blood flow isdependent on the PDA

T i id A i i


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Tricuspid Atresia- presentation The presentation will depend on the

amount of pulmonary blood flow If the PBF is decreased, the main presenting

symptom is cyanosis If the PBF is increased the presentation is that

of congestive heart failure

CXR will also reflect the amount ofpulmonary blood flow


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Tricuspid Atresia- EKG

Very characterestic : Left axis deviation


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ManagementPBF

Decreased Increased

PGE-1, and minimalsupplemental O2 tomaintain ductal patency

No O2 Afterload reductionDiuretics


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Total Anomalous Pulmonary VenousReturn (TAPVR)


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TAPVR- Infracardiac


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Radiography


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Infracardiac type


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Thank You

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