CONGENITAL HEART

DISEASES
Practical approach in
children with heart murmur
MIHAI CRAIU MD PhD
IMCC

NORMAL HEART SOUNDS

NORMAL HEART SOUNDS

A normal heartbeat makes two sounds

like "lubb-dupp" (sometimes described
as tumm-taah"), which are the sounds
of heart valves closing .

NORMAL HEART SOUNDS

The normal sounds are:
the first heart sound (S1) produced by
the closing of the AV valves and
second heart sound (S2), produced by
the closing of the semilunar valves.

NORMAL HEART

CARDIAC CYCLE

HEART MURMURS

Heart murmurs are abnormal sounds

during cardiac cycle generated by
turbulent blood in or near the heart.

HEART MURMURS

Murmurs may be physiological

(benign) or pathological (abnormal).

HEART MURMURS
Abnormal murmurs can be VALVULAR
caused by:
stenosis restricting the opening of a
heart valve,
or with valvular insufficiency which
allows backflow of blood when the
incompetent valve closes

MITRAL VALVE INSUFFICIENCY

HEART MURMURS
Abnormal murmurs can be NONVALVULAR caused by:
Septal defects
ASD, VSD

Endocardial cushion defect

Patent ductus arteriosus
Out-flow tract stenosis
Vascular stenosis (Ao, AP)

CONGENITAL HEART DISEASE

There are three different hemodinamic
CHDs with abnormal murmurs

Left to right shunt (NO cyanosis)

Right to left shunt (WITH cyanosis)
No shunt (vascular)

CHD with L/R shunt

NO CYANOSIS!!
Most frequent group of CHDs
Most of them are completely
curable with cath-lab procedures or
surgery

CHD with L/R shunt

ASD (atrial septal defect)
VSD (ventricular septal defect)
PDA (patent ductus arteriosus)

VSD
VSDs are the most common congenital
cardiac anomalies.
They are found in 30-60% of all newborns
with a congenital heart defect, or about 2-6
per 1000 births.
Congenital VSDs are frequently associated
with other congenital conditions, such as
Down syndrome

VSD

A ventricular septal defect (VSD) is a

defect in the ventricular septum, the
wall dividing the left and right
ventricles of the heart.

VSD
The ventricular septum consists of
an inferior muscular and superior
membranous portion and is
extensively innervated with
conducting cardiomyocytes.

BLOOD FLOW
During ventricular contraction some of
the blood from the left ventricle leaks
into the right ventricle (SHUNT),
passes through the lungs and reenters
the left ventricle via the pulmonary
veins and left atrium.

PATHOPHYSIOLOGY
L/R SHUNTING produces:

volume overload on the left ventricle

(shunt + venous return from lungs)
high right ventricular pressure and
volume, causing PAH (pulmonary
artery hypertension)

VSD
In severe cases, the pulmonary arterial
pressure can reach levels that equal the
systemic pressure.
This reverses the shunt, so that blood
then flows from the right ventricle into
the left ventricle, resulting in cyanosis,
as blood is by-passing the lungs for
oxygenation (Eisenmenger syndrome =
fixed PAH)

SIGNS & SIMPTOMS

VSD is an acyanotic congenital heart
defect
Pansystolic (Holosystolic) murmur
(depending upon the size of the defect)
+/- palpable thrill (palpable turbulence
of blood flow).
An infant with a large VSD will fail to
thrive and become sweaty, pale and
tachypnoeic with feeds

LAB
ECG( PREDOM DE R mai mari decat
S)
Chest Rx(cardiomegalie)
Ultrasound(confirma rx)

ECG of VSD

Rx in VSD

Echo of VSD

TREATMENT
Most cases do not need treatment and
heal at the first years of life.
Treatment is either conservative or
surgical.
Smaller congenital VSDs often close on
their own, as the heart grows, and may
be treated conservatively.

SURGERY
Should be done in selected cases:
Failure of congestive cardiac failure to
respond to medications
VSD with pulmonic stenosis
Large VSD with pulmonary hypertension
VSD with aortic regurgitation

OCCLUDERS / AMPLATZER

CHD with CYANOSIS

Fallot tetralogy (trilogy or pentalogy)

TGA
Truncus arteriosus
Persistent fetal circulation
Endocardial cushion defect (AV def)
Univentricular heart

TETRALOGY of FALLOT

A congenital cardiac anomaly with

cyanosis that consists of four defects:
pulmonic stenosis,
ventricular septal defect,
malposition of the aorta so that it
arises from the septal defect or the
right ventricle (Overriding aorta),
right ventricular hypertrophy.

TETRALOGY of FALLOT
Occurs in less than 1 in 2000 live
births.
Tetralogy of Fallot is the most
common cyanotic congenital heart
defect.

TETRALOGY of FALLOT

SIGNS & SIMPTOMS

SQUATTING

LAB
CBC
Incresed Hb level
Polycytemia( dam aspirina!!)
Incresed platelet count
Sa nu aibe trombocitoza!!)

CHEST Rx in FALLOT

ECHO in FALLOT

TREATMENT

DIFFERENTIALS FOR FALLOT