Congenital heart disease

Classification of congenital heart diseasesGroup I : Left to right shunts

Group II: Right to lefts shunts

Group III: Obstructive lesions

Left to right shunts

Atrial Septal Defect Ventricular Septal Defect Patent Ductus Arteriosus

Right to Left Shunts

1) Tetralogy of Fallot2) Tricuspid atresia

3) Ebstein’s anomaly

4) Transposition of Great Vessels

5) Truncus Arteriosus

6) Total Anomalous Pulmonary Venous Return (TAPVR)

Obstructive Lesions

Aortic stenosis Coarctation of the Aorta Pulmonic Stenosis

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%!

TOF

4 component!

Imagine this is a HEART!

TOF

1) Vetricular Septal Defect

TOF


2) Pulmonic Stenosis

TOF



3) Overriding of dextroposed aorta

TOF




4) Right Ventricular hypertrophy

TOF





Concentric R ventricular hypertrophy without cardiac enlargement

TOF





Concentric R ventricular hypertrophy without cardiac enlargement

Increase in right ventricular pressure*

RV and LV pressures becomes identical


There is little or no L to R shunt



Hence, VSD is silent



Hence, VSD is silent

Right ventricle into pulmonary artery across pulmonic stenosis producing 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




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 artery pressure reduce


Pulmonary artery pressure reduce

P2 become soft or inaudible

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 spellsPredominantly after waking upChild cryDyspneaBlueLose consciousConvulsionFrequency 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 – squattingCompensatory mechanismSquatting increases the peripheral vascular

resistance, which diminishes the

right-to-left shunt increases pulmonary

blood flow.

Cyanosis during feedingPoor feedingfussiness, 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 heardP2 soft & delayed: INAUDIBLE

MurmurShunt murmur (VSD) absentFlow murmur: Ejection systolic,

the smaller the flow the shorter the murmur

Ejection aortic click

Retinal engorgement Hemoptysis

ECG

ECG

ECG

ECGwiLLiammoRRow

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 Complication

1) Each anoxic spell is potentially fatal

2) Polycytemia1) Cerebrovascular thrombosis

3) Anoxic infaction of CNS1) 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 spell

1) Knee chest position

2) Humified O2

3) Be careful not to provoke the child 1) Especially you are bad at gaining IV access

2) Ask for help from someone more experience

3) Permit the baby to remain with mother

4) Morphine 0.1 -0.2 mg/Kg Subcutaneous

5) Correct acidosis – Sodium Bicarb IV

6) Propanolol1) 0.1mg/kg/IV during spells

2) 0.5 to 1.0 mg/kg/ 4-6hourly orally

7) Vasopressors: Methoxamine IM or IV drip

8) Correct anemia

9) 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:RBBBResidual VSDResidual Pulmonary stenosisPulmonary 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 surgeryRedirecting the pulmonary and systemic venous

return to result in a physiologically normal stateThe right ventricle remains the systemic ventricleRarely 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 S2Ejection click of the abnormal truncal valve Systolic murmur of truncal valve stenosis if

presentDiaastolic murmur of truncal valve

insufficiencyGallop

CXR : Cardiomegally , increased pulmonary circulation

Managment Acute management

No O2 to minimize pulmonary blood flow DiureticsAfterload 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 flowIf the PBF is decreased, the main presenting

symptom is cyanosisIf 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

Management

PBF

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

PBFAorta 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

Thank You

Congenital heart disease

Health & Medicine

Transcript of Congenital heart disease