CONGENITAL HD Overview BY
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CONGENITAL HD
Overview BY
RAGAB ABDELSALAM (MD)
Prof. of Cardiology
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Causes: Inheritance:
A multifactorial etiology has been assumed in which interaction
between hereditary predisposition
and environmental influences
resultsin the defects .
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Maternal factors
- Maternal diabetes
- Maternal phenylketonuria.
- Maternal alcohol
consumption and fetal alcohol
syndrome
- Genetic risk factors .
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-A family history of a cardiac or noncardiac defect in either a parent or a preceding sibling is a major risk factor
-Familial congenital heart
defects are often concordant by phenotype and developmental
mechanism
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Genotype-phenotype correlation:
-Careful family history of first- and second-degree relatives, including detailed analysis of pregnancy loss, racial origin, and consanguinity
-A search for risk factors such as gestational diabetes mellitus
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Classification of congenital heart
diseases
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Murmurless Congenital Heart
Diseases a) Cyanotic diseases
- - Transposition of great arteries
- Pulmonary atresia with intact septum - total anomalies of pulmonary
venous drainage
- Tricuspid atresia with or without pulmonary atresia
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b) Acyanotic diseases
-Core triatriatum
-Severe coarctation
-Coronary artery originating from pulmonary Artery.
-Endocardial fibroelastosis
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c) Others
- Single ventricle without obstruction.
-Hypoplastic left heart syndrome
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*Duct dependent lesions
-These are lesions presenting very early in life that are dependent on ductal patency for survival
These lesions fall into two main categories
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A) Cyanotic lesions in which pulmonary blood flow is almost or totally dependent on the duct
• Severe tetrallogy of fallot-
-Pulmonary atresia with intact septum
pulmonary atresia with VSD-
Critical pulmonary stenosis-
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B) Acyanotic lesions
Aortic atresia.-
Critical aortic stenosis-
Iterrupted aortic arch- -Severe pre-or juxtaductal
Coarctation -
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The radiographic classification of CHD relies on:
1. Clinical information (cyanosis)
2- Plain film information (increased pulmonary vascularity, decreased pulmonary vascularity (
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I ) Acyanotic CHD with increased pulmonary vascularity.
Common denominator of these lesions is that there is a L-R shunt where pulmonary flow is greater than true aortic flow;
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The shunt can be located in:
1- Ventricular septal defect.
2- Atrial septal defect.
3- Patent ductus arteriosus
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4- Uncommon lesions:
- Aorticopulmonary window.
-Ruptured sinus of Valsalva aneurysm, coronary artery fistula
5- Other
- Endocardial cushion defect
- PAPVC
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II) Acyanotic CHD with normal pulmonary vascularity
Normal pulmonary vascularity is associated with either outflow
obstruction or valvular insufficiency:
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A) Outflow obstruction:1. - Coarctation of aorta
2. - Interruption of aortic arch
3. - Aortic stenosis
4. - Pulmonary stenosis / insufficiency
B) Valvular insufficiency: - Aortic insufficiency - Pulmonary insufficiency
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III) Cyanotic CHD with decreased pulmonary vascularity
- Common denominator of these lesions is that there is a decreased pulmonary vascularity due to obstruction of pulmonary blood flow.
- In addition, there is an intracardiac defect through which blood is shunted away from the lungs causing cyanosis.
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* Types:Normal heart size- Tetralogy of Fallot
- Fallot variants ( trilogy, pulmonary atresia / pseudotruncus I/II)
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Increased heart size- Ebstein's anomaly (largest heart in
CHD)
- Tricuspid atresia
ddx: 1 - Uhl's disease (RV myocardium absent with tricuspid atresia)
2-Pulmonary stenosis with ASD
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IV) Cyanotic CHD with increased pulmonary vascularity (admixture lesions)
- Common denominator of these lesions is that there is an "admixture" of systemic and pulmonary venous blood (bidirectional shunting.
-
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- Clinical symtoms: CHF, cyanosis, recurrent pneumonia, growth retardation.
- The admixture of venous blood may occur at the level of:
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* Large veins:- Total anomalous pulmonary
venous connection (ASD is also present)
* Large arteries:- Truncus arteriosus
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Atrium- Transposition of great arteries (VSD
is also present).
Ventricle- Single ventricle- DORV (types I, II = Taussig-
Bing.
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TETRALOGY VARIANTS
1- Pink tetralogy: - 1/3 have mild pulmonary valvular
obstruction with large VSD, allowing sufficient pulmonary flow.
- Pulmonary atresia and VSD = pseudotruncus, extreme end of the spectrum.
- Infundibular hypertrophy in VSD (3%)
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2- Pentalogy of Fallot: Tetralogy + ASD
3- Trilogy of Fallot: PA stenosis + RV
hypertrophy +Patent foramen ovale , or (ASD)
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TRANSPOSITION OF GREAT ARTERIES (TGA)
Types: Complete transposition of great arteries (D-TGA)
AORTA ORIGINATES FROM RV
- PA originates from LV
- Normal position of atria and ventricles
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Corrected transposition of great arteries (L-TGA):
- Transposition of great arteries
- Inversion of ventricles
- The relative position of aorta and pulmonary artery can be derived from the diagram on the right.
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D-TGA (COMPLETE TRANSPOSITION):
Two independent circulations exist:
• Blood returning from body RV blood delivered to body (aberrant aorta)
• Blood returning from lung LV blood delivered to lung (via ASD,etc).
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This circulatory pattern is incompatible with life unless there are associated anomalies that permit mixing of the two circulations, e.g., through ASD, VSD, or PDA.
• Hemodynamics: depends on the level of admixture and R->L shunting.
• RA and RV enlarged.
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GENERAL:
Situs stuff: - Abdominal situs refers to position of
liver and stomach:
a) Abdominal situs solitus: liver on right, stomach on left (normal)
b) Abdominal situs inversus: liver on left, stomach on right
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c) Thoracic situs refers to position of the tracheobronchial tree:
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Thoracic situs solitus (normal)
1- Left main bronchus longer than right main bronchus
2- Left main bronchus inferior to left PA
3- Right main bronchus superior to right PA
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Dxtrocardia
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Type I ( situs inversus totalis) with
1 -Mirror-like malposition of the heart
2 - Mirror-like malposition of other viscera.
3 - Kartegner suyndrome: Situs inversus , sinusitis & bronciectasis
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Type II: ( Isolated dextrocardia)
1 -Mirror-like cardiac malposition.
1 -Normal position of other viscera.
3 -serious cardiac anomalies.
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Type III ( Dextroversion with):
1 -Heart is merely displaced to right.
2 -RV remains to right & LV remains to left.
3 -Serious cardiac anomalies.
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Type IV (Dextroposition) : Aquired dextrocardia:
The heart is displaced by external factors: Pulmonary , pleural or diaphragmatic
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Congenital dextrocardia
Acquired dextrocardia
1 -No apparent cause 1 -Apparent cause ( pushing
or pulling) the heart
2 -Trachea is central2 -Trachea is displaced to the right
3 -Apex is localized (LV)3 -Diffuse ( as it is formed by RV)
4 -Situs inversus may be presnt
4 -Absent
5 -Associated congenital anomalies
5 -Absent
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The three common cynotic Heart Diseases
1 (Falot tetralogy
2 (fallot Trilogy. 3 (Eisenmenger”s syndrome
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Fallot Tetralogy
Fallot Triology
Eisenmenger”s
Cyanosis
)Onset(
Usually since birth
Late ( First on excersion
Late ( First on excersion
Cyanotic spells
Present AbsentAbsent
Squatting Present AbsentAbsent
Cyanosis & clubbing
Marked Moderate Moderate
Neck veins Normal Prominent A & systolic expansion
Prominent A & systolic expansion
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Fallot Tetralogy
Fallot Triology
Eisenmenger”s
RVHMild or moderate
Marked Marked
Systolic thrill
Absent or mild
Markedabsent
PA-pulsation
absentabsentPresent &
diastolic shock
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S2singleSplit with weak P2
Closely split&
accentuatred P2
ClickAbsent PresentPresent
Syst. murmur
Mild or moderate
HarshMild
Diast. murmure
absentabsentPR ( usually)
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GallopNever present
May be present
May be present
ECGRVH ,but no strain
RVH with stran
RVH with stran
X- Ray -Lung oligemia ,
small PA ,coeur en Sabot & Right aortic
arch
Lung oligemia, enlarged PA, , Big heart & normal
aortic arch
Peripheral lung
oligemia,enlarged
main PA & its two
branches+ Big heart
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Echo-PS ,- VSD -- aortic overriding
Valvular PS
-- ASD
-- RVH
-Determine the site of defect & PH+
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Catheteri- zation
-Low PA pressure ,
- RV & aortic pressures are equal ,
- Catheter may pass from RV to
aorta-RV Angio
Anatomy
-Low PA pressure ,-RV pressure may exceed aortic pressure ,-Catheter cannot pass from RV to aorta-RV angio anatomy r
-High PA pressure-- RV pressure may exceed
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Diagnostic Work-up
1- Clinical assessment.2- ECG.3- X-ray4- Echo-Doppler assessment.5-Cardiac catheterizatiopn.
6- Others: - MRI. -CT. - Radionucleide assessment.
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Special X-Ray Cotour
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*Examples:1 -Boot-shaped heart (Coeur-en
Sabot ) Fallot Tetralogt
2 -Scimitar” syndrome ( figure of- 8) Total anomalies of the pulmonary venous drainage
3 -Box-shaped heart Ebstein“
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4 -Goose-neck appearance Trunchus arteriosus.
5 -Figure of (3) Coarctation of aorta.
6 -Reverse appearance on barium swallow (E-sign) Coarctation of aorta.
7 -Rib-notching Coarctation of aorta.
8 -Egg-en situ TGA.
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Boot-shaped heart ( Coeur-en)
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Scimitar” syndrome ( figure of- 8)
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Rib-notching
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Aneurysmally Dilated Pulm. Artery )special left(
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Egg-en situ.
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Box-shaped heart.
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Echocardiography
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VSD
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VSD
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Endocardial Cashion Defect
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Fallot” Tetralogy
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TGA
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TGA
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Double –outlet RV
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Problems with Congenital
HD
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1- Infective Endocarditis:
All congenital HD are prone to IE except ASD.
2- Heart failure.
3- sudden Death.
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4- Hematologic complications of chronic hypoxemia include:
- Erythrocytosis; iron deficiency and
bleeding diathesis
- Hemostatic abnormalities have been documented in cyanotic patients with erythrocytosis and can occur in up to 20% of patients.
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5 - Neurologic complications: - Cerebral hemorrhage.
- Paradoxical cerebral emboli.
- Brain abscess.
6 - Renal dysfunction: - It can manifest itself as proteinuria,
hyperuricemia, or renal failure .
- Urate nephropathy, uric acid nephrolithiasis and gouty arthritis are rare but may occur.
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7- Rheumatologic complications:
8- Gallstones:It is composed of calcium bilirubinate and
consequent cholecystitis .
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9- Arrhythmias
Patients with Eisenmenger syndrome are at risk for sudden cardiac death, the etiology of which remains poorly defined .
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The choice of antiarrhythmic drugs are complicated by:
-The presence of ventricular dysfunction and lung disease.
- The pro-arrhythmic effects.- The use of pacemakers to treat
bradyarrhythmias, which are primary or secondary to antiarrhythmic therapy can be complicated by Inadequate venous access.
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10 -The decision to use anticoagulants:
It is complicated by the presence of: - Bleeding diathesis. - Difficulty obtaining a true
measure of INR due to reduced plasma volume.
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•Interventional Options 1- Percutaneous closure of intracardiac shunts.
A variety of devices can be used to close ASDs, PDAs and occasionally VSDs
2- Palliative surgical interventions : It is performed in patients with cyanotic lesions. They are defined as those operations which
serve to either increase or decrease pulmonary blood flow while allowing a mixed circulation and cyanosis to persist.
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3- Physiologic repair:• It is a term which can be applied to
procedures which result in total or near-total anatomic and physiologic separation of the pulmonary and systemic circulations in complex cyanotic lesions thereby resulting in relief of cyanosis.
4- Heart & Lung Transplantation :• One or both lungs with surgical shunt
closure and heart-lung transplantation have been performed in cyanotic patients with or without palliation who are no longer candidates for other forms of intervention.