Transcript Tetralogy of Fallot

Congenital cyanotic heart disease
Under supervision of
Prof.Dr/ Mariam Abu-Shady
Professor of pediatrics and neonatology
Faculty of medicine for girls
Al-Azhar University
Dr.Marwa El-Hady Abd Elmoneim
Assisted lecturer of pediatric
Faculty of medicine for girls
Al-Azhar University
By:
Amira Mohammad Ahmed
Amira farag biomy
Omnia shams godaa
CONTENT
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Definition of Congenital cyanotic heart disease
Evaluation of Criticlly Ill Neonate with Cyanosis
Classification of Congenital cyanotic heart disease
Tetralogy of Fallot
Pulmonary atresia with VSD
Double outlet right ventricle with PS
Pulmonary atresia
Tricuspid atresia
Ebstein's Anomaly
Congenital cyanotic heart
disease
It is abnormality in cardio circulatory structure that
present at birth.
CHD occurs in about 8/1000 live births, and those with
critical CHD are 2/1000 live births.
Prompt diagnosis and treatment of CHD significantly
decrease mortality and prevent secondary damage to
other organ systems.
Evaluation of Criticlly Ill Neonate with Cyanosis
cause
clinical
CXR
ECG
ABG
hyperoxia
respiratory
distress
diagnostic
normal
low po2
High pco2
pass
cardiac
Distress less
common
Other cardiac
fiding.
Abnormal
heart size
and
vascular
marking.
May be
abnormal
Low po2
Normal or
low pco2
fail
neurologic
Slow
breathing .
Cyanosis
improve on
stimulation
normal
normal
High pco2
pass
Classification
Cyanotic CHD
Decreased pulmonary blood
flow
Increased pulmonary blood flow
TOF
TGA
Pulmonary atresia with VSD
Truncus arteriosus
Double-Outlet Right Ventricle
DORV without PS
Pulmonary atresia without VSD
TAPVR
Tricuspid atresia
Truncus arteriosus
Ebstein anomaly
Single ventricale
Criteria of Cyanotic CHD with decrease
pulmonary blood flow
• Central cyanosis
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clubbing
Growth retardation
Decreased Pulmonary component of S2
Chest x ray show lung oligaemia
ECG show right ventricular hypertrophy or left
ventricular hypertrophy
• Echocardiography : Diagnostic
• cardiac catheterization; Diagnostic, done
preoperative
Tetralogy of Fallot
• It is a condition caused by a combination of
four heart defects that are present at birth.
These defects are:
• Pulmonary Infundibular Stenosis
• Overriding aorta
• ventricular septal defect (VSD)
• Right ventricular hypertrophy,
Tetralogy of Fallot
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Tetralogy of Fallot
• hypercyanotic spells:
Sometimes, babies with tetralogy of Fallot will suddenly
develop worsen of central cyanosis after crying,
feeding or having a bowel movement. It is treated by
placing child in the knee-to-chest position,
oxygen,morphine and propranol.
• systolic thrill over left sternal border
• Single second sound
• Ejection systolic murmur(PS)
• Treated by:
Total correction or The Black-Taussig operation
Tetralogy of Fallot
boot-shaped
heart
Pulmonary atresia with VSD
Pulmonary atresia with ventricular septal
defect (PA-VSD) is a cyanotic congenital
heart disease characterized by
underdevelopment of the right ventricular
(RV) outflow tract with atresia of the
pulmonary valve and a large ventricular
septal defect (VSD),
Pulmonary atresia with VSD
Pulmonary atresia with VSD
• A prominent a wave in the jugular pulse may be found.
• auscultation:
• systolic murmur usually is audible along the lower left
sternal border.
• A continuous murmur is best heard over the upper chest in
the presence of a PDA.
• If systemic-to-pulmonary collateral arteries are present,
continuous murmurs may be diffusely audible over the
entire chest and back.
• Treatment:
prostaglandin E2 is often required to keep the ductus arteriosus
open
Most babies will need a 'Shunt' operation during infancy
Double outlet right ventricle with PS
Double outlet right ventricle (DORV) is a
congenital heart disease in which the aorta rises
from the right ventricle instead of from the left
ventricle
Both the pulmonary artery and aorta come from
the same pumping chamber. No arteries arise
from the left ventricle .
Double outlet right ventricle with PS
Double outlet right ventricle with PS
• initial medical management consists of increasing
the fraction of inspired oxygen (FIO2), which may be
up to 100%. This decreases pulmonary vascular
resistance, thereby increasing the amount of blood
flow in the lungs
• Surgical treatment
create a tunnel through the VSD. The tunnel connects
to the aorta.
Pulmonary atresia
Pulmonary atresia with intact ventricular
septum (PA/IVS) is a condition in which the
valve that allows blood to flow from right
ventricle of heart to lungs (pulmonary valve)
hasn't formed properly or is closed (atresia).
PA/IVS is a rare congenital heart disease
present from the early stages of a baby's
heart development
Pulmonary atresia.
Pulmonary atresia
• systolic thrill over pulmonary area
• prostaglandin E1 is usually used to help the blood
move into the lungs.
• Open heart surgery to repair or replace the valve
Tricuspid atresia
Tricuspid atresia is the third most common
form of cyanotic congenital heart disease,
The deformity consists of a complete lack of
formation of the tricuspid valve with absence
of direct connection between the right
atrium and right ventricle.
Tricuspid atresia
1) Atrial Septal Defect
2) Missing Tricuspid Valve
3) Hypoplastic (very small)
Right Ventricle
4) Pulmonary Stenosis
(narrowing of pulmonary
valve)
5) Ventricular Septal Defect
6) Patent Ductus Arteriosus
Tricuspid atresia
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single second sound
giant A wave
murmer of VSD(+PDA)
prostaglandin E2 is often required to keep the
ductus arteriosus open in the early neonatal
period until surgery can be performed.
• Surgical treatment:
The Fontan operation
Ebstein's Anomaly
Ebstein's anomaly is a condition in which the
tricuspid valve is malformed and sits lower than
normal in the right ventricle. This condition is
associated with right ventricle dysfunction, and
commonly atrial septal defect or patent foramen
ovale.
Ebstein's Anomaly
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Ebstein's Anomaly
• Jugular venous pulse
• Large a and v waves late in the course of the disease,
• First heart sound is widely split with loud tricuspid component
• Third and fourthheart sounds are commonly present
• Medical Care
Antibiotic prophylaxis for infective endocarditis
Medical therapy for heart failure - Angiotensin-converting enzyme
(ACE) inhibitors, diuretics, and digoxin
• Surgical Care
Surgical care includes correction of the underlying tricuspid valve and
right ventricular abnormalities heart sounds are commonly
present
Critaria CCHD with increased
pulmonary blood flow
Poor feeding •
Recurrent chest infection •
Recurrent heart failure •
Increase P2
Chest x ray show plethora
Transposition of the great vessels
Def:Transposition of the great vessels is •
acongenital heart defect in which The two
major vessels that carry blood away from the
heart -- the aorta and the pulmonary artery -are switched (transposed).
()
Investigation
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Chest x-ray
Total anomalous pulmonary venous
return
Def:Total anomalous pulmonary venous •
return is acongenital heart disease) in which
none of the four veins that take blood from
the lungs to the heart is attached to the left
atrium (left upper chamber of the heart)
Double outlet right ventricle with out
pulmonary stenosis
Investigation
Chest x-rays :no pulmonary stenosis will •
cause increase pulmonary blood flow
resulting in cardiomegaly with increase
pulmonary vascular markings. The
mediastinum may be narrow due to
malposed great vessels
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Truncus arteriosus (TA)
is an uncommon congenital cardiovascular •
anomaly that is characterized by a single
arterial trunk arising from the normally
formed ventricles by means of a single
.semilunar valve (ie, truncal valve)
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Classification
 .Truncus arteriosus type I is characterized by origin of a
single pulmonary trunk from the left lateral aspect of the
common trunk, with branching of the left and right
pulmonary arteries from the pulmonary trunk .
 .Truncus arteriosus type II is characterized by separate but
proximate origins of the left and right pulmonary arterial
branches from the posterolateral aspect of the common
arterial trunk .
Type3,4
 .In truncus arteriosus type III, the branch pulmonary arteries
originate independently from the common arterial trunk or aortic
arch, most often from the left and right lateral aspects of the trunk.
This occasionally occurs with origin of one pulmonary artery from
the underside of the aortic arch, usually from a ductus arteriosus .
 .Type IV truncus arteriosus, originally proposed by Collett and
Edwards as a form of the lesion with neither pulmonary arterial
branch arising from the common trunk, is now recognized to be a
form of pulmonary atresia with ventricular septal defect rather than
truncus arteriosus .
Double inlet left ventricle
hypoplastic left heart syndrome
(HLHS),
In hypoplastic left heart syndrome (HLHS), •
the heart’s left side — including the aorta,
aortic valve, left ventricle and mitral valve —
is underdeveloped.
Pathophysiology
Heterotaxy Syndrome
Heterotaxy syndrome is a rare birth defect •
that involves the positionof the heart and
other organs. nt
“right atrial isomerism” and “left atrial
isomerism”
In a normal heart, the left atrial appendage
looks different than the right. In heterotaxy
syndrome, the two appendages look similar.
Asplenia
 Asplenia or right atrial isomerism: Children with this
condition have multiple heart defects. They may have septal
defects and problems with heart valves, particularly the
pulmonary valve. The spleen may be absent (asplenia), and
the liver and other organs may be on the wrong side of the
body.
polysplenia
 Polysplenia or left atrial isomerism: Children with this
condition may have septal defects as well as problems with
heart valves and the heart’s electrical system. The spleen
may be absent, or there may be several small spleens
(polysplenia), instead of one spleen.
conclusions
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