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Disease
Introduction
CHD are the structural cardiac defects at birth
Incidence – 7-8‰ and 40 – 50% are
diagnosed at birth
Etiology: genetic and environmental factors
Intrauterine circulation
ETIOLOGY
environment factors 2%
Mother’s illnesses (Systemic lupus
erythematosus, Diabetes, Phenylketonuria)
Drugs :cocaine, barbituratece, metronidazole,
fluconazole, alcohol, tabaco etc
Maternal infections : rubella, cytomegalovirus
Extreme pregnancy obesity
ETIOLOGY
Genetic factors 8%
Trisomies 18, 13, 21
(Edwards syndrome, Patau syndrome, Down
syndrome )
Source: Hoffman JIE, Kaplan S: The incidence of congenital heart disease. J Am Coll Cardiol 39:1890, 2002
Diagnostic
Clinical examination
– Inspection: cyanosis, clubbing fingers
– Palpation: thrill, femoral, radial pulse, liver dimensions
– Auscultation: cardiac, extracardiac
– BP measuring
Investigations
– Radiology
Cardiomegaly: CTI > 0.6
Particular shapes
Pulmonary vascular system
– ECG: RVH, LVH, BVH
– Ecocardiography
Anatomy of the heart
Classification 1
Without cyanosis Cyanosis
1. ASD 1. TGA
2. VSD 2. HLHS
3. AVSD 3. Tetralogy of Fallot
4. PDA 4. Abnormal pulmonary
5. CoAo connections
6. Cong Mi St 5. Pulmonary atresia
7. Ao St 6. Tricuspid atresia
8. P St 7. Arterial Trunk
9. Endomiocardial 8. Ebstein anomaly
fibroelastosis 9. Univentricular heart
Classification 2
A physiopathological classification:
– Intra/extracardiac shunts: ASD, VSD, AVSD,
PDA
– Conotrunkal anomalies:
Increased pulmonary flow: TGA, PAT
Decreased pulmonary flow: TOF, TA, PA
– Obstructive lessions:
Left: Mi St, HLHS, Ao S, Co Ao
R: TA, PA
Cardiac and vascular shunts
ASD Anatomical classification:
blue, ostium
secundum defect;
green, ostium primum
defect;
red, sinus venosus
defects;
yellow, defect of the
coronary sinus.
ASD HEMODINAMICS
Left to right shunt
– with PHT with
increased vascular
resistance
– Right cavities
dilation
ASD – clinical manifestations
Asymptomatic
– moderated
cardiomegaly
(dilatation of the RA,
RV, and PA)
ASD- eco
- The position and size of the defect
- Evaluation of the ratio Systemic/pulmonary flow
- Right ventricular dilatation
- Abnormal ventricular septum movement.
ASD - paraclinic
ECG
- Right ax deviation + 90/+ 180
- RVH or RBBB with pattern rSR‘ in V1
ASD - management
Spontaneous closure in 40% of cases in the first 4
years of life
Types of VSD
• perimembranous - 80%
• musclular - 5-20%
• subarterial (doubly committed)
Clasification VSD
Perimembranous
80%
Outlet (infundibular)
5-7%
Inlet (posteroinferior)
5-8%
specific CAVC
Muscular
5-20%
Swiss cheese
VSD
caracteristic of some genetic syndromes
(trisomies 13, 18, 21)
13 Trisomy = Patau syndrome (VSD,PDA)
18 Trisomiy = Edwards syndrome
(ASD,VSD,PDA)
21 Trisomy =Down syndrome
(AVSD,VSD,ASD,PDA,TOF)
Family forms
- risk value: 3% if the father has VSD
and 6% if the mother has VSD
VSD - hemodynamics
Shunt left - right which in time
will increase vascular
resistance
with pulmonary hypertension
Hemodynamic consequences
depend on the size of the
defect and pulmonary vascular
resistance and systemic ratio
ECG:
- small VSD: normal
- Moderate VSD: LVH, LAD
- Large VSD: biventricular hypertrophy, LAD
- Pulmonary vascular obstructive disease: RVH
Eco
– The position and size of VSD
– Size of the LA, LV - indirect indicators of shunting
– Doppler signal at the PA level, and tricuspid regurgitation
VSD
-Small VSD: normal
-Moderate VSD: LVH, LAD
-Large VSD: biventricular hypertrophy, LAD
-Pulmonary vascular obstructive disease: RVH
Chest x-ray
- Cardiomegaly
Increased pulmonary flow
eco
The position and size of VSD
Size of the LA, LV - indirect indicators of shunting
Doppler signal at the PA level, and tricuspid regurgitation
VSD management
Spontaneous closure in 30-40% of VSD, most frequently
those small, muscular, and early in life
The large defects tend to become smaller with age
The inlet or infundibular defects do not close
spontaneously nor diminish
CHF occurs in children with large VSD but not before 6-8
weeks of age
Pulmonary vascular obstructive disease start to emerge
at 6 to 12 months of life in patients with large VSD but
reversal of the shunt appears in the second decade of life
Infective endocarditis is rare
VSD -treatment
Medical
Treatment with digitalis and diuretics for CHF
No effort limitation is necessary in the absence
of PHT
Maintaining good dental hygiene and
prevention of endocarditis
VSD treatment
Surgical
Procedures
Palliative - PA banding, rarely – for associated lessions
Direct closure –cardiopulmonary bypass with atrial approach, preferable, than right
ventriculotomy
– Indications and timing
Qp/Qs > 2,
– At 12-18 months in those who respond to medical treatment
– At 2-4 years in asymptomatics
– Having HF or growth deficiency – nonresponsive to medical
treatment even as infants
partial (without
VSD)
Hemodinamic AVSD
• the right side of the heart contains venous blood with a low oxygen content
•the left side of the heart contains arterial blood with a high oxygen content
• if there is a defect in the septum, it is possible for blood to travel from the
left side of the heart => and the blood from the two sides of the heart mixing
with each other
AVSD paraclinic
ECG: ax qrs – superior (-40→-150),
characteristic, RVH, LVH, BAV gr I
Chest X-Rays: cardiomegaly+increased
vasculary flow
Echo - dgn
ECG ; Rx AVSD
CoAo
Also called aortic
narrowing
Is a congenital
condition whereby the
Ao is narrow, usually in
the area where the
ductus arteriosus
(ligamentum arteriosum
after regression) inserts
Coarctations are most
common in the aortic
arch
Coarctation of the Aorta
Coarctation may be preductal
or ductal
In the preductal type there is
a tubular hypoplasia of the
aortic arch. Symptoms
become manifest early in
infancy.
The ductal type consists of a
localized constriction of the
aorta in the region of the
closure of the ductus
arteriosus.
Symptoms in this type
usually are delayed until
adolescence or later.
clinic
Fatigue
Breathing
difficulties
Chest pain
Poor apetite /
trouble feeding
Failure to thrive
CoAo Clinic- SPECIFIC!!
Arterial hypertension in the arms with low
blood presure in the lower extremities
(Blood presure ½ SUP body– Blood presure ½
INF a corpului (right arm) = 20mmHg )
To be compatible with life there must be an exchange of blood between the
two circulations, through either a patent foramen ovale, an ASD, a VSD, or a
PDA.
TGA
TGA - clinically
Cyanosis
HF signs (dyspnea, fatigability
Severe hypoxemia non-responsive to
oxygen
TGA - paraclinic
ECG
– Right ax deviation, RVH.
– If there is a large VSD - signs of LVH
Chest X -Rays
– Cardiomegaly, increased pulmonary flow
– Appearance of "egg lying on the diaphragm"
with a narrowed mediastinum
– Echo – diagnostic – aspect of “two circles”
TGA
TGA - treatment
Medical
- treatment of hypoglicemia/acidosis/hypocalcemia
- PGE1 IV- reopening duct
- oxygen
Interventional
Surgical
Switch
– atrial - Senning - many post-op complications
– Ventricular - Rastelli