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
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CONTENT 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
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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.
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Evaluation of Criticlly Ill Neonate with Cyanosis
hyperoxiaABGECGCXRclinicalcause passlow po2 High pco2
normaldiagnosticdistressrespiratory failLow po2 Normal or low pco2
May be abnormal Abnormal heart size and vascular marking. Distress
less common Other cardiac fiding. cardiac passHigh pco2normal Slow
breathing. Cyanosis improve on stimulation neurologic
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Classification Cyanotic CHD Decreased pulmonary blood flow TOF
Pulmonary atresia with VSD Double-Outlet Right Ventricle Pulmonary
atresia without VSD Tricuspid atresia Ebstein anomaly Increased
pulmonary blood flow TGA Truncus arteriosus DORV without PS TAPVR
Truncus arteriosus Single ventricale
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Criteria of Cyanotic CHD with decrease pulmonary blood flow
Central cyanosis 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
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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,
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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
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Tetralogy of Fallot boot-shaped heart
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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),
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Pulmonary atresia with VSD
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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 E 2 is often required to keep the ductus arteriosus
open Most babies will need a 'Shunt' operation during infancy
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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.
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Double outlet right ventricle with PS
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Double outlet right ventricle with PS initial medical
management consists of increasing the fraction of inspired oxygen
(FIO 2 ), 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.
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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
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Pulmonary atresia.
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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
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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 single second sound giant A wave murmer of
VSD(+PDA) prostaglandin E 2 is often required to keep the ductus
arteriosus open in the early neonatal period until surgery can be
performed. Surgical treatment: The Fontan operation
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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.
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Ebstein's Anomaly =
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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
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Critaria CCHD with increased pulmonary blood flow Poor feeding
Recurrent chest infection Recurrent heart failure Increase P2 Chest
x ray show plethora
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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).
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()
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Investigation. Chest x-ray
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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)
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Double outlet right ventricle with out pulmonary stenosis
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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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. ;
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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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.
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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.
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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.
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Double inlet left ventricle
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hypoplastic left heart syndrome (HLHS), In hypoplastic left
heart syndrome (HLHS), the hearts left side including the aorta,
aortic valve, left ventricle and mitral valve is
underdeveloped.
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Pathophysiology
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Heterotaxy Syndrome Heterotaxy syndrome is a rare birth defect
that involves the positionof the heart and other organs. nt
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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.
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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.septal defects
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polysplenia Polysplenia or left atrial isomerism: Children with
this condition may have septal defects as well as problems with
heart valves and the hearts electrical system. The spleen may be
absent, or there may be several small spleens (polysplenia),
instead of one spleen.
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conclusions
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