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CONGENITAL HD Overview BY RAGAB ABDELSALAM (MD) Prof. of Cardiology. Causes: Inheritance : A multifactorial etiology has been assumed in which interaction between hereditary predisposition - PowerPoint PPT Presentation
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CONGENITAL HD
Overview BY
RAGAB ABDELSALAM (MD)
Prof. of Cardiology
Causes: Inheritance:
A multifactorial etiology has been assumed in which interaction
between hereditary predisposition
and environmental influences
resultsin the defects .
Maternal factors
- Maternal diabetes
- Maternal phenylketonuria.
- Maternal alcohol
consumption and fetal alcohol
syndrome
- Genetic risk factors .
-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
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
Classification of congenital heart
diseases
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
b) Acyanotic diseases
-Core triatriatum
-Severe coarctation
-Coronary artery originating from pulmonary Artery.
-Endocardial fibroelastosis
c) Others
- Single ventricle without obstruction.
-Hypoplastic left heart syndrome
*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
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-
B) Acyanotic lesions
Aortic atresia.-
Critical aortic stenosis-
Iterrupted aortic arch- -Severe pre-or juxtaductal
Coarctation -
The radiographic classification of CHD relies on:
1. Clinical information (cyanosis)
2- Plain film information (increased pulmonary vascularity, decreased pulmonary vascularity (
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;
The shunt can be located in:
1- Ventricular septal defect.
2- Atrial septal defect.
3- Patent ductus arteriosus
4- Uncommon lesions:
- Aorticopulmonary window.
-Ruptured sinus of Valsalva aneurysm, coronary artery fistula
5- Other
- Endocardial cushion defect
- PAPVC
II) Acyanotic CHD with normal pulmonary vascularity
Normal pulmonary vascularity is associated with either outflow
obstruction or valvular insufficiency:
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
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.
* Types:Normal heart size- Tetralogy of Fallot
- Fallot variants ( trilogy, pulmonary atresia / pseudotruncus I/II)
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
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.
-
- Clinical symtoms: CHF, cyanosis, recurrent pneumonia, growth retardation.
- The admixture of venous blood may occur at the level of:
* Large veins:- Total anomalous pulmonary
venous connection (ASD is also present)
* Large arteries:- Truncus arteriosus
Atrium- Transposition of great arteries (VSD
is also present).
Ventricle- Single ventricle- DORV (types I, II = Taussig-
Bing.
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%)
2- Pentalogy of Fallot: Tetralogy + ASD
3- Trilogy of Fallot: PA stenosis + RV
hypertrophy +Patent foramen ovale , or (ASD)
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
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.
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).
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.
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
c) Thoracic situs refers to position of the tracheobronchial tree:
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
Dxtrocardia
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
Type II: ( Isolated dextrocardia)
1 -Mirror-like cardiac malposition.
1 -Normal position of other viscera.
3 -serious cardiac anomalies.
Type III ( Dextroversion with):
1 -Heart is merely displaced to right.
2 -RV remains to right & LV remains to left.
3 -Serious cardiac anomalies.
Type IV (Dextroposition) : Aquired dextrocardia:
The heart is displaced by external factors: Pulmonary , pleural or diaphragmatic
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
The three common cynotic Heart Diseases
1 (Falot tetralogy
2 (fallot Trilogy. 3 (Eisenmenger”s syndrome
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
Fallot Tetralogy
Fallot Triology
Eisenmenger”s
RVHMild or moderate
Marked Marked
Systolic thrill
Absent or mild
Markedabsent
PA-pulsation
absentabsentPresent &
diastolic shock
S2singleSplit with weak P2
Closely split&
accentuatred P2
ClickAbsent PresentPresent
Syst. murmur
Mild or moderate
HarshMild
Diast. murmure
absentabsentPR ( usually)
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
Echo-PS ,- VSD -- aortic overriding
Valvular PS
-- ASD
-- RVH
-Determine the site of defect & PH+
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
Diagnostic Work-up
1- Clinical assessment.2- ECG.3- X-ray4- Echo-Doppler assessment.5-Cardiac catheterizatiopn.
6- Others: - MRI. -CT. - Radionucleide assessment.
Special X-Ray Cotour
*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“
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.
Boot-shaped heart ( Coeur-en)
Scimitar” syndrome ( figure of- 8)
Rib-notching
Aneurysmally Dilated Pulm. Artery )special left(
Egg-en situ.
Box-shaped heart.
Echocardiography
VSD
VSD
Endocardial Cashion Defect
Fallot” Tetralogy
TGA
TGA
Double –outlet RV
Problems with Congenital
HD
1- Infective Endocarditis:
All congenital HD are prone to IE except ASD.
2- Heart failure.
3- sudden Death.
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.
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.
7- Rheumatologic complications:
8- Gallstones:It is composed of calcium bilirubinate and
consequent cholecystitis .
9- Arrhythmias
Patients with Eisenmenger syndrome are at risk for sudden cardiac death, the etiology of which remains poorly defined .
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.
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.
•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.
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.