Classification of Congential Heart Diseases and cyanotic heart disease

Cyanotic congenital heart disease

Classification of congenital heart diseases

Group I : Left to right shuntsGroup II: Right to lefts shuntsGroup III: Obstructive lesions

Left to right shunts• Atrial Septal Defect• Ventricular Septal Defect• Patent Ductus Arteriosus

Right to Left Shunts1) Tetralogy of Fallot2) Tricuspid atresia3) Ebstein’s anomaly

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

Return (TAPVR)

Obstructive Lesions• Aortic stenosis• Coarctation of the Aorta• Pulmonic Stenosis

Cyanotic heart disease

Right to Left Shunt

Who is this guy?

ÉTIENNE-LOUIS ARTHUR FALLOT!

•  a French physician, 1888 Fallot accurately described in detail the four anatomical characteristics of tetralogy of Fallot.

Tetralogy OF Fallot• Most common cyanotic heart

disease!

75%!

TOF4 component!

Imagine this is a HEART!

TOF1) Vetricular Septal Defect

TOF1) Vetricular Septal Defect2) Pulmonic Stenosis

TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta

TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta4) Right Ventricular hypertrophy

TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta4) Right Ventricular hypertrophy

Concentric R ventricular hypertrophy without cardiac enlargement

TOF1) Vetricular Septal Defect2) Pulmonic Stenosis3) Overriding of dextroposed aorta4) Right Ventricular hypertrophy

Concentric R ventricular hypertrophy without cardiac enlargement

Increase in right ventricular pressure*

RV and LV pressures becomes identical

RV and LV pressures becomes identical

There is little or no L to R shunt

RV and LV pressures becomes identical

There is little or no L to R shunt

Hence, VSD is silent

RV and LV pressures becomes identical

There is little or no L to R shunt

Hence, VSD is silent

Right ventricle into pulmonary artery across pulmonic stenosis producing ejection systolic murmur

Hence, the more severe the pulmonary stenosis

Hence, the more severe the pulmonary stenosis

The BIGGER the Left to RIGHT shunt

Hence, the more severe the pulmonary stenosis

The BIGGER the Left to RIGHT shunt

Less flow into the pulmonary artery

Hence, the more severe the pulmonary stenosis

The BIGGER the Left to RIGHT shunt

Less flow into the pulmonary artery

Shorter the ejection systolic murmur

Hence, the more severe the pulmonary stenosis

The BIGGER the Left to RIGHT shunt

Less flow into the pulmonary artery

Shorter the ejection systolic murmur

More cynosis because of less flow to the lung!

Hence,• Severity of cyanosis is directly

proportional to the severity of pulmonic stenosis

• Intensity of the systolic murmur is inversely related to the severity of pulmonic stenosis

Congestive failure never occur* because…

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

* exception

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

Pulmonary obstruction results in delayed P2

Pulmonary obstruction results in delayed P2

Pulmonary artery pressure reduce

Pulmonary obstruction results in delayed P2

Pulmonary artery pressure reduce

P2 become soft or inaudible

Pulmonary obstruction results in delayed P2

Pulmonary artery pressure reduce

P2 become soft or inaudible

(Second Sound) S2= A2 + P2

Since P2 is inaudible, hence S2 = A2 + P2[S2 is single sound]

Aorta is displace anteriorly too, A2 become LOUD!

• Ascending aorta in TOF is large, results aortic ejection click

• Diastolic interval is clear• No S3• No S4

Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole

Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole

“a” waves become prominent in JVP*

*but not too tall

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 from

once a few days to many attack everyday

“tet spell”• lethal, • unpredictable episodes • The mechanism

– spasm of the infundibular septum,which acutely worsens the RV outlet obstruction.

• Dyspnea on exertion• Exercise intolerance

• Sitting posture – squatting– Compensatory mechanism– Squatting increases the peripheral

vascular resistance, – which diminishes the

right-to-left shunt – increases pulmonary

blood flow.

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

delayed.

• Rarely, patient remain asymptomatic into adult life.

Physical examination• Clubbing + Cyanosis (Variable)• Squatting position• Scoliosis – Common• bulging left hemithorax

• Prominent “a” waves JVP• Normal heart size

– Mild parasternal impulse• Systolic trill (30%)

• S1 normal• S2 single

– only A2 heard– P2 soft & delayed: INAUDIBLE

• Murmur– Shunt murmur (VSD) absent– Flow murmur: Ejection systolic,

the smaller the flow the shorter the murmur

• Ejection aortic click

• Retinal engorgement• Hemoptysis

ECG• ECG

ECG• ECG

ECG• ECG wiLLiam

moRRow

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 after surgical repair)

• Coeur en sabot (boot-shaped heart)

secondary to uplifting of the cardiac apex from RVH

and the absence of a normal main pulmonary artery segment

• Normal heart size due to the lack of pulmonary blood flow and congestive heart failure

• Decreased pulmonary vascularity

• Right atrial enlargement

• Right-sided aortic arch (20-25% of patients) with indentation of leftward-positioned tracheobronchial shadow

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

outflow tract (RVOT) obstruction

Course and Complication1) Each anoxic spell is potentially fatal2) Polycytemia

1) Cerebrovascular thrombosis3) Anoxic infaction of CNS

1) Neurological complication

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-enter circulation

3) Hence TOF can cause:1) Brain Abcess2) Infective endocarditis3) Paradoxical embolism

Management of anoxic spell1) 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

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

7) Vasopressors: Methoxamine IM or IV drip

8) Correct anemia9) GA is the last resort

Palliative Surgery• Blalock-Taussig shunt• Pott procedure• Waterston shunt

Blalock Taussig Shunt• Subclavian artery – Pulmonary

artery anastomosis

Modified Blalock Taussig Shunt

• Goretex graft

Surgical Palliation

• Palliative operation prolong life• Increase exercise tolerance

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%

Transposition of Great Areries (TGA)

• Aorta originating from the right ventricle, and pulmonary artery originating from the left ventricle

• Accounts for 5-7% of all congenital heart disease

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

TGA• Exam :

• cyanosis in an otherwise healthy looking baby

• Loud S2 ( aorta is anterior )

• CXR : • Egg on side• Narrow

mediastinum

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

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

• Balloon atrial septostomyLife saving procedure in the presence of

inadequate atrial septal defect

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

Truncus Arteriosus• The presence of a

common trunk that supply the systemic, pulmonary and coronary circulation

• Almost always associated with VSD

• 1.2-2.5% of all congenital heart disease

Truncus Arteriosus

• There are different anatomical tupes of truncus arteriosus

• This is relevant for surgical repair

Truncus Arteriosus• Generally patients have increased

pulmonary blood flow• Degree of cyanosis is mild and may

not be evident clinically until late stage with pulmonary vascular disease

• Presenting feature is congestive heart failure (tachypnia, hepatomegally)

Truncus Arteriosus• Exam is significant for

– Single S2– Ejection click of the abnormal truncal

valve – Systolic murmur of truncal valve

stenosis if present– Diaastolic murmur of truncal valve

insufficiency– Gallop

• CXR : Cardiomegally , increased pulmonary circulation

Managment• Acute management

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

blood flow•Surgical management: complete repair with VSD closure and conduit placement between the right ventricle and pulmonary arteries•Long term problems :

– truncal valve dysfunction– RV conduit obstruction

Trcuspid Atresia• Complete absence

of communication between the right atrium and right ventricle

• About 3 % of congenital heart disease

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 is dependent on the PDA

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 of

pulmonary blood flow

Tricuspid Atresia- EKG

Very characterestic : Left axis deviation

ManagementPBF

Decreased Increased

PGE-1, and minimal supplemental O2 to maintain ductal patency

No O2Afterload reductionDiuretics

Surgical ManagementSingle ventricle paliation• First stage : to establish a reliable

source of PBF– Aorta to pulmonary artery shunt ( BT

shunt)– Pulmonary arterial banding in cases of

increased PBF• Second stage: Glenn Anastomosis

( superior vena cava to pulmonary artery

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

Total Anomalous Pulmonary Venous Return (TAPVR)

TAPVR- Infracardiac

Radiography

Infracardiac type

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