Download right atrium right ventricle

Non cyanotic
congenital heart diseases
Krzysztof Narebski
Torun
Problems to discuss
I. Transition from fetal to newborn circulation
II. Non cyanotic congenital heart diseases :
• Atrial septal defect
• Ventricular septal defect
• Atrioventricular septal defect
(A)
Radiographic position of normal
right heart structures.
RA, right atrium;
TV, tricuspid valve;
RV, right ventricle;
MPA, main pulmonary artery;
RPA right pulmonary artery.
(B)
Radiographic position of normal
left heart structures.
LA, left atrium;
MV, mitral valve;
LV, left ventricle.
Intracardiac pressures
Estimated pressures
1st week of life
(term baby):
RV ~ 40 >> 30 >> 25
LV ~ 40 >> 50 >> 60
Intracardiac oxygen saturation
Abbreviations
•
•
•
•
•
•
•
•
•
•
•
•
CHD –
FO –
DA –
RA, RV –
LA, LV –
CHF –
PVR –
PBF –
PGE –
SVR –
CVP –
RAAS –
congenital heart disease
foramen ovale
ductus arteriosus
right atrium, right ventricle
left atrium, left ventricle
congestive heart failure
pulmonary vascular resistance
pulmonary blood flow
prostaglandin
systemic vascular resistance
central venous pressure
renin-angiotensin-aldosterone system
Maximal fetal
blood oxygen
saturation
up to 85 %
in umbilical
vein
Cardiac output
RV 55 %
LV 45 %
Neonatal ECG
(RV domination)
Right to left shunt
b/o
-RA vol overload
-Low placental
resistance
Increase
oxygen
blood saturation
during first
10 minutes
after birth
up to
95 – 100 %
PGE
Events after birth:
-PVR falls (8 weeks)
-PBF increase
-LA pressure
exceed RA pressure
-FO closure
-DA closure
(oxygen and lack of
prostaglandin)
Foramen
ovale
Ductus
arteriosus
Changes in the circulation from fetus to newborn.
Atrial septal defect
(ASD)
ASD - types
• ASD II (ostium secundum) up to 80 %
• SV-ASD
(sinus venosus) 10 %
• ASD I (ostium primum)
10 % or AVSD
Atrial septal
defect
(a) Ostium secundum
ASD - deficiency of
FO and surrounding
atrial septum.
(b) AVSD - deficiency
of the atrioventricular
septum.
(c) Murmur.
(d) Chest x-ray.
(e,f) ECG.
(g) Examples of an
occlusion device used
to close secundum
ASD
ASD
Physiology :
• FO closure in the first day of life
• Anatomic FO closure in 2 – 3 months
• FO can be opened in some circumstances in up
to 20 % of adults without any symptoms
Characteristic of ASD:
• Up to 14 % of CHD
• Most often as isolated CHD
ASD – signs and symptoms
• No signs if small ASD (Qp/Qs < 1.5)
• If Qp/Qs > 3 :
– dyspnoea, heart failure
in small children (after infancy)
– split second heart sound
– ejection murmur in II left intercostal space
radiating into the lung (pulmonary valve too
small for enlarged right ventricular output)
ASD - ECG
• Right axis deviation with right ventricular hypertrophy
– tall R in V1-3 and deep S in V4-6
• Partial right bundle branch block – RsR’ pattern in V1
ASD – chest x-ray
Enlargement of :
PA
RA
• right atrium
• right ventricle
(normal left atrium)
• pulmonary artery
Increased
pulmonary flow
ASD II - Echocardiography
ASD I - Echocardiography
ASD
color
doppler
ASD - treatment
• Small ASD :
- No treatment or diuretics
• Large ASD :
- Percutaneous transcatheter closure
(Amplatzer)
- Surgery (rarely)
Ventricular septal
defect (VSD)
VSD
Characteristics :
• Interventricular septum defect
• Left to right shunt between ventricles
• The most common (20 - 40 % congenital
cardiac anomalies)
• Occur in other cardiovascular associations
(e.g.: TOF, CoA, pulmonary stenosis)
VSD showing a
left-to-right shunt.
(b) Murmur.
(c) Chest x-ray.
(d) ECG.
VSD - types
Many classifications !
• A – subarterial
• B – perimembranous
(most common)
• C – AVSD
• D – muscular
VSD – pathophysiology
• Left to right shunt (according to pressure gradient)
Addition of extra blood to normal pulmonary flow :
• Increased PBF and increased pulmonary interstitial fluids up
to oedema
• Increased pulmonary venous return into LA
• Increased LA volume, pressure & dilatation
• LV hypertrophy
• Finally biventricular overload & hypertrophy
Decreased cardiac output >> heart failure
VSD – signs and symptoms
Symptoms (after neonatal period) depend on the size of defect
and magnitude of the left to right shunt !
• Small VSD: mild or no symptoms, murmur !
• Moderate: excessive sweating (increased sympathetic tone
- RAAS) during feeds. Fatigue with feeding, failure to thrive,
frequent respiratory infections
(pulmonary congestion)
• Large: more severe with tachypnoea,
tachycardia, hepatomegaly
VSD – murmur
• Harsh, holosystolic murmur loudest along the
lower left sternal border
• The murmur detected after
PVR decreased (usually
after newborn period)
• Physiologic splitting of
S2 usually retained
VSD - ECG
• !!! Voltage scale is half
• Biventricular hypertrophy (left > right)
VSD chest x-ray
Cardiomegaly :
Both left
chambers
(up to both
ventricles)
enlargement
Increased PBF
LA
LV
VSD - echo
Systole
Early diastole
(a) medium muscular VSD (arrow).
(b) color Doppler shows a left-to-right shunt (blue) during systole.
(c) Also a small right-to-left shunt (red) during early diastole (LV
relaxes more quickly than RV, resulting in a transient pressure
gradient favoring shunting from the right to left ventricle).
VSD - treatment
• Small especially muscular VSD – no treatment
• Amplazer or surgery as definitive treatment
• PA banding in 2-stage procedure
• Before surgery according to symptoms :
– Supplemental oxygen
– Additional calories or tube feeds
– Diuretics (relieves pulmonary congestion)
– Angiotensin-converting enzyme (ACE) inhibitors (reduce
both systemic and pulmonary pressures)
– Digoxin (increases contractility)
Atrioventricular septal
defect (AVSD)
or
Common atrioventricular canal (CAVC)
or
Endocardial cushion defect
AVSD
Characteristics :
• Broad spectrum of malformations characterized
by a deficiency of atrioventricular septum
• Atrioventricular valves always abnormal
• Clinical presentation depends on the degree of
intracardiac left to right shunt, atrioventricular
regurgitation and pulmonary vascular resistance
• Congestive heart failure
• Several method of classification
AVSD - types
I.
II.
-
Partial AVSD consists of:
mitral and tricuspid annuli are separated
primum ASD and inlet VSD
cleft of anterior mitral valve leaflet (insufficiency
of valve with mitral regurgitation towards RA)
more common in trisomy 21 (Down syndrome).
Complete AVSD consists of:
single valve annulus
ASD (up to common atrium)
VSD
ECG superior mean axis
- aVF negative
(due to primary abnormality
of conductive system).
AVSD
mitral cleft
Partial AVSD
(separated
annuli)
Complete AVSD
(single valve
annulus)
AVSD – hemodynamic problems
Heart failure and cardiomegaly in newborn period !!!
• The most important is regurgitation from LV to RA
via mitral cleft (shunt from the highest to the
lowest pressure chamber). RA > LA.
Consequence of left to right shunt and
atrioventricular regurgitation is blood
Intracardiac overload, which leads to
biventricular hypertrophy and
finally cardiac insufficiency up to
pulmonary hypertension.
AVSD – signs and symptoms of
congestive heart failure
Infant and children (severe AVSD) :
• Poor feeding with inadequate weight gain
• Tachypnea and labored breathing up to
respiratory insufficiency
• Shock like symptoms
Teenagers and young adult (mild AVSD) :
• Exercise intolerance
• Arrhythmias
AVSD – physical examination
Depends of the severity of left to right shunt!
• Fixed splittng of the second heart sound (S2)
• Pulmonary ejection (systolic) murmur audible at the
left upper sternal border
• Diastolic murmur and third heart
sound (S3) as a result of high flow
across the tricuspid component
of the atrioventricular valve
AVSD – physical examination cont.
• Apical murmur (blowing quality) of MR
(systolic)
(presence of this apical murmur + fixed S2
differentiates partial AVSD from ASD II)
• Fine crepitations in severe MR (rare in
infants)
AVSD –
chest xray
AVSD - echocardiography
Complete AVSD
AVSD - echocardiography
Common valve in complete AVSD
Partial AVSD – ECG in 3 year old child
aVF
• aVF is negative so the axis is superior (abnormal
septal depolarization)
• P-wave enlargement concordant with RA, LA, or
biatrial enlargement
• V1 rSR' – partial RBBB
AVSD - treatment
• Surgery as definitive treatment in the first 6
months (closure of intra-chambers connections)
• Before surgery according to symptoms :
- Supplemental oxygen
- Additional
calories
- Diuretics
40-60%
- ACE
inhibitors
ASD – primary RV overload and
hypertrophy
VSD – primary LV overload and
hypertrophy
AVSD – primary biventricular overload and
hypertrophy
Thank you for your attention
ECG
essentials
in pediatrics
(hypertrophy)
Right ventricular hypertrophy
R in V1
S in V6
all population
0 – 7 days
8 – 30 days
1 – 3 months
3 ms – 16 years
R/S in V1 0 – 3 months
3 – 6 months
6 ms – 3 years
3 – 5 years
6 – 15 years
T in V1
< 12 years
qR in V1 all population
> 20 mm
> 14 mm
> 10 mm
> 7 mm
> 5 mm
> 6.5
>4
> 2.4
> 1.6
> 0.8
positive & R/S > 1
Left ventricular hypertrophy
All pediatric population :
-
S in V1
> 20 mm
R in V6
> 20 mm
T in V5 or V6
inversion
Q in V5 or V6
> 4 mm
(S in V2) + (R in V5) > 60 mm
(Sokoloff index)
Both ventricular hypertrophy
Signs of right ventricular hypertrophy and :
-
Q in V5 or V6
T in V6
> 2 mm
inversion
Atrial enlargement
Right atrial enlargement :
-
P in any leads > 3 mm (peaked shaped)
Left atrial enlargement :
-
P in any leads (dual or biphasic shaped)
P > 0.09 sec
Remember
Electrical axis of ECG leads
Extreme
axis deviation
is always
pathology
Remember
Axis deviation and QRS complex
Remember
P wave should be positive in I, II, aVF
Newborn – normal ECG
Child of 8 years – normal ECG
Thank you for your attention