Cyanotic congenital heart disease

Cyanotic congenital heart disease

Classification of congenital heart diseases
• Group 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

• Tetralogy of Fallot
• Tricuspid atresia
• Ebstein’s anomaly
• Transposition of Great Vessels
• Truncus Arteriosus
• Total Anomalous Pulmonary Venous Return (TAPVR)

Obstructive Lesions

Aortic stenosis
Coarctation of the Aorta
Pulmonic Stenosis

Cyanotic heart disease

Right to Left Shunt

Cyanotic heart disease

bluish or purplish tinge to the skin and mucous membranes.
5 g/dL of unoxygenated hemoglobin in the capillaries
This usually correspond to oxygen saturation of 70-80%

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
• Vetricular Septal Defect

TOF
• Vetricular Septal Defect
• Pulmonic Stenosis

TOF
• Vetricular Septal Defect
• Pulmonic Stenosis
• Overriding of dextroposed aorta

TOF
• Vetricular Septal Defect
• Pulmonic Stenosis
• Overriding of dextroposed aorta
• Right Ventricular hypertrophy

TOF
• Vetricular Septal Defect
• Pulmonic Stenosis
• Overriding of dextroposed aorta
• Right Ventricular hypertrophy


Concentric R ventricular hypertrophy without cardiac enlargement

TOF
• Vetricular Septal Defect
• Pulmonic Stenosis
• Overriding of dextroposed aorta
• 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

• Anemia
• Infective Endocarditis
• Systemic hypertension
• Unrelated myocarditis complicating TOF
• 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 Complication
• Each anoxic spell is potentially fatal
• Polycytemia
• Cerebrovascular thrombosis
• Anoxic infaction of CNS
• Neurological complication
•

• 4) LUNG is an awesome filter.

• Bypassing it may not be a good idea!
• TOF, venous blood from gut, peripheral system by pass the lung and re-enter circulation
• Hence TOF can cause:
• Brain Abcess
• Infective endocarditis
• Paradoxical embolism





Management of anoxic spell
• Knee chest position
• Humified O2
• Be careful not to provoke the child
• Especially you are bad at gaining IV access
• Ask for help from someone more experience
• Permit the baby to remain with mother
• Morphine 0.1 -0.2 mg/Kg Subcutaneous
• Correct acidosis – Sodium Bicarb IV

• Propanolol

• 0.1mg/kg/IV during spells
• 0.5 to 1.0 mg/kg/ 4-6hourly orally
• Vasopressors: Methoxamine IM or IV drip
• Correct anemia
• 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 septostomy
Life 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

Management
PBF
Decreased
Increased
PGE-1, and minimal supplemental O2 to maintain ductal patency
No O2
Afterload reduction
Diuretics

Surgical Management

Single 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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