Transcript Lecture 10. The mostly spread congenital heart diseases in children

Congenital Heart Disease
Prof. Pavlyshyn H.A.
Recognition of Cyanosis
Differential cyanosis
1. pink upper, blue lower
CoA (Coarctation of the
aorta), IAA (Interrupted aortic
arch), Pulm Htn
2. blue upper, pink lower
Transposition of the great vessels
d-TGA with pulm Htn
dextro-Transposition of the great
arteries
*indicates serious underlying
cardiac or lung disease*
Patent Ductus Arteriosus
Hemodynamics
• As a result of
higher aortic
pressure, blood
shunts L to R
through the
ductus from
Aorta to PA.
Patent Ductus Arteriosus
Clinical Signs & Symptoms
• tachycardia
• respiratory problems shortness of breath
• Poor growth
• Differential cyanosis cyanosis of the lower extremities
but not of the upper body.
Patent Ductus Arteriosus
Clinical Signs & Symptoms
• Classic continuous
machine-like murmur
• It begins soon after onset of the 1st sound,
reaches maximal intensity at the end of
systole, and wanes in late diastole.
• prominent apical impulse
enlarged heart,
• Left subclavicular thrill
• Bounding pulse
• Widened pulse pressure
Ventricular Septal
Defect
• During systole some of the blood from the LV leaks into the RV,
passes through the lungs and reenters the LV via the pulmonary veins
and LA.
• Such circuitous route of blood causes volume overload on the LV.
• The LV normally has a much higher systolic pressure (~100 mm Hg)
than the RV (~85 mm Hg) and through VSD blood leaks into the RV
and elevates RV pressure and volume, causing Pulm HTN.
• These changes lead to elevated RV & pulmonary pressures & volume
hypertrophy of the LA & LV.
Ventricular Septal Defect
Clinical Signs & Symptoms
• Small - moderate VSD, 3-6mm, are usually
asymptomatic.
Small defects located predominantly in the muscular
septum with slight hemodynamic impairment
(Tolochinov-Roge disease)
• Moderate – large VSD, almost always have
symptoms and will require surgical repair.
Ventricular Septal
Defect
Listen at the back for radiation of murmurs
• Pansystolic/holosystolic murmur - loud, harsh, blowing
heard best over the LLSB, frequently is accompanied by thrill
(depending upon the size of the defect) +/• more prominent with small VSD, may be absent with a very large
VSD.
ECG:
overload of LV and RV
І
ІІ
ІІІ
V1
V2
V3
AVR
V4
AVL
V5
AVF
V6
LA, LV or biventricular hypertrophy. RV hypertrophy predominates
when pulmonary vascular resistance is high.
Atrial Septal Defect - ASD
• is a form of CHD that enables blood flow between the left and
right atria via the interatrial septum (it is possible for blood to
travel from the left side to the right side of the heart).
• Seen in 10% of all CHD.
There are 3 major types:
Sinus
Venosus
• Secundum ASD
• Primum ASD – low in the septum
• Sinus Venosus ASD
ASD with left-to-right
shunt
• In the case of a large ASD (>9mm), may result in left-to-right shunt,
blood will shunt from the LA to the RA.
• This extra blood may cause a volume overload of both the right
atrium and the right ventricle.
• Ultimately the RV must push out more blood than the LV due to the
L-to-R shunt. This condition can result in eventually RV-failure
(dilatation and decreased systolic function) and Pulm Htn.
Listen carefully
Systolic ejection murmur – its medium pitched, seldom
accompanied by a thrill, and best heard at the LSB (left middle and
upper sternal border);
Short, rumbling mid-diastolic murmur produced by the
increased volume of blood flow across the tricuspid valve is often
audible at the LLSB (lower left sternal border) .
Atrial Septal Defect
Diagnosis
• X-ray chest:
pulmonary
vascularity is
increased
• ECG: right-axis
deviation;
• Echo-CG: RV is
enlarged, defect is
visualized;
Coarctation of the Aorta
• Coarctation- is
narrowing of the
aorta at varying
points anywhere
from the transverse
arch to the iliac
bifurcation.
• Male: Female ratio
3:1.
• Accounts for 7 %
of all CHD.
Coarctation of the Aorta
Hemodynamics
• Obstruction of left
ventricular outflow
 LV afterload
increases 
pressure
hypertrophy of the
LV.
Coarctation of the Aorta
Clinical Signs &
Symptoms
• Sings of low cardiac
output, poor peripheral
perfusion - LE
hypoperfusion, acidosis,
HF and shock.
• Decreased and delayed
pulses in lower
extremities.
• Systolic ejection
murmur @ LSB.
• Cardiomegaly, rib
notching on X-ray.
Pulmonary Stenosis
• Pulmonary Stenosis is
obstruction in the region
of either the pulmonary
valve or the
subpulmonary
ventricular outflow
tract.
• Accounts for 7-10% of
all CHD.
• Most cases are
Pulmonary
Stenosis
Hemodynamics
RV pressure hypertrophy  RV failure.
RV pressures maybe > systemic pressure.
Post-stenotic dilation of main PA.
W/intact septum & severe stenosis  R-L shunt through
FO  cyanosis.
• Cyanosis is indicative of Critical PS.
•
•
•
•
Pulmonary Stenosis
Clinical Signs & Symptoms
• Depends on the severity of obstruction.
• Asymptomatic w/ mild PS <
30mmHg.
• Mod-severe: 30-60mmHg, > 60mmHg
• Prominent jugular a-wave
• RV lift, RV heave
• Split 2nd hrt sound
• Ejection click, followed by systolic
murmur.
• Heart failure & cyanosis not relieved
by inhaled oxygen seen in severe cases.
Right sided obstruction
1. Obstruction of RV
outflow (Pulmonary
stenosis);
2. VSD;
3. Dextroposition of
the aorta with
override of the
ventricular septum;
4. RV hypertrophy
Tetralogy of Fallot
Assessment Findings with
Tetralogy of Fallot
Symptoms are variable depending
of degree of obstruction
• Cyanosis – is variable (isn’t
present at the birth, occurs later
in the 1st yr of life)
• Digital clubbing and hyperpnea
at rest are directly related to the
degree of cyanosis
• Tachycardia
• Mental retardation
• Retarded growth and
development
• RV heave
• Systolic ejection murmur is
heard along the left sternal border
Assessment Findings with
Tetralogy of Fallot
• Paroxymal dyspnea
• Severe dyspnea on exertion
• Squatting position for the relief
of dyspnea caused physical
effort,
• “Blue” spells, “tet” spells,
paroxysmal hypercyanotic
attacks – infant becomes
hyperpnea, restless, cyanosis
increases, gasping respirations,
syncope
Hypercyanotic Spells/Blue Spells/Tet Spells
Clinical Manifestations
‫ ٭‬Most often occurs in morning after feedings,
defecation, or crying
‫ ٭‬Acute cyanosis
‫ ٭‬Hyperpnea
‫ ٭‬Inconsolable crying
‫ ٭‬Hypoxia which leads to acidosis
Chest X-Ray
• Decreased
pulmonary vascular
marking
• “Boot-shaped
heart”
Treatment of the Child with TOF
•
•
•
•
Decrease cardiac workload
Prevention of intercurrent infection
Prevention of hemoconcentration
Surgical repair – palliative or corrective
surgery
d-Transposition of the Great Arteries
• Pathophysiology
– Cyanosis due to failure of
delivery of pulmonary venous
blood to the systemic
circulation
– Two parallel circulations with
no mixing
– Open atrial septum (fossa
ovalis) allows some left-toright shunt, enhanced by a
left-to-right ductus arteriosus
shunt
– Presence of ventricular septal
defect facilitates mixing
•
Transposition of the Great
Arteries
Aorta from right
ventricle, pulmonary
artery from left
ventricle.
• Cyanosis from birth,
hypoxic spells
sometimes present.
• Heart failure often
present.
• Cardiac enlargement
and diminished
pulmonary artery
segment on x-ray.
Transposition of the Great
Arteries
• Anatomic communication
must exist between
pulmonary and systemic
circulation, VSD, ASD, or
PDA.
• Untreated, the vast majority
of these infants would not
survive the neonatal period.
Transposition of the Great Arteries
Clinical Manifestations
• Cyanosis, tachypnea are most
often recognized within the 1st hrs
or days of life.
• Hypoxemia is usually moderate to
severe, depending on the degree of
atrial level shunting and whether
the ductus is partially open or
totally closed.
• Physical findings, other than
cyanosis, may be remarkably
nonspecific.
• Murmurs may be absent, or a
soft systolic ejection murmur may
be noted at the midleft sternal
border.