Download Ventricular Septal Defects

Ventricular Septal Defect in adults
Dr. Mohamed Sofi MD; FRCP
(London); FRCPEdin; FRCSEdin
Ventricular Septal Defect (VSD)
A ventricular septal defect is • Inlet defects (type 3, also
a defect in the septum
known as atrioventricular
resulting in
canal type)
communication between
• Muscular (type 4) most
the ventricular cavities.
common type in young
Morphology: 4 types
children
• Infundibular (type 1, also • Complete AV septal
referred to as supracristal,
(endocardial cushion)
more commomn in Asia
defects
• Membranous (Type 2)
most common type in
adults (80%)
VENTRICULAR SEPTAL DEFECT IN ADULTS
• VSD accounts for only
10 % of congenital heart
defects in adults.
• Many close
spontaneously
• About 5 % of patients
with VSDs have
chromosomal
abnormalities including
trisomy 13, 18, and 21
syndromes
• VSDs are of various
sizes and locations, can
be single or multiple
• VSDs are associated with
other congenital heart
defects including:
– ASD (35 %)
– PDA (22 %)
– Right aortic arch (13 %)
– Tetralogy of Fallot
• Majority of congenital
VSDs in adults present as
an isolated defect
Pathophysiology
• Defect size is often compared to aortic annulus
– Large: > 50% of annulus size
– Medium: 25-50% of annulus size
– Small: <25% of annulus size
• Restrictive VSD is typically small, such that a significant
pressure gradient exists between the LV and RV (high
velocity), with small shunt
• Moderately restrictive VSD  moderate shunt
• Large / non-restrictive VSD  large shunt
• Eisenmenger VSD  irreversible pulmonary HTN and
shunt may be zero or reversed
Types of ventricular septal defects
Anatomic diagram illustrates the positions of different
ventricular septal defects: infundibular (1),
membranous (2), inlet or canal (3), muscular or
trabecular (4).
Membranous VSD
Parasternal long axis view with Color Flow Doppler demonstrating
membranous VSD (arrow) underneath the aortic valve with Color Flow
Doppler demonstrating a left-to-right shunt from the left ventricular
outflow tract into the right ventricle (double arrow).
Ventricular septal defect (Mid-muscular part)
Inlet ventricular septal defect
This apical four chamber image shows the inlet VSD
(arrow) at the juncture of the atrioventricular valves. Inlet
ventricular septal defect
Membranous VSD long axis color still frame
Long axis still frame from a two-dimensional transthoracic echocardiogram with
and without color flow mapping. In the left hand panel, color flow mapping
demonstrates the jet of left-to-right flow crossing the septum just apical to the aortic
annulus. The ventricular septal defect (VSD) is shown without color flow mapping
in the right hand panel (arrow).
Muscular VSD multiple color still frame
Paired apical images with and without color flow mapping show a large posterior muscular
VSD and two smaller apical muscular VSDs (arrows). Though the larger posterior defect
near the crux is easily seen without color flow mapping, the apical defects are more easily
missed without color flow Doppler interrogation of the septum.
Natural History
The natural history of isolated
ventricular VSDs depends on
the type, size of defect, and
associated hemodynamic
abnormalities.
• Spontaneous closure —
Spontaneous closure occurs in
40 to 60 % of individuals with
VSDs, during early childhood
• Endocarditis — Endocarditis
risk persists in those with
unrepaired VSD The risk of
endocarditis is higher in those
with unrepaired VSD
compared to those who have
undergone repair.
• Arrhythmias — Arrhythmias
including atrial and ventricular
PMB and tachycardias have
been observed in adult patients
with VSD. The incidence of
VT and sudden death in
natural history studies of
VSDs are 5.7 and 4.0 percent,
respectively
• Heart failure — Heart failure
due to chronic volume
overload of the left ventricle
occurs in medium or large
VSDs
CLINICAL MANIFESTATIONS
• Isolated small restrictive defect The following scenarios may
with small left to right shunt
occur with large VSDs:
(often referred to as “maladie
• Have early large left to right
de Roger”) generally remain
shunting with development of
asymptomatic in adulthood.
heart failure during infancy.
• Patients with moderate sized
• In rare cases, Eisenmenger
VSDs may remain
syndrome occurs sometime
asymptomatic or develop
during late childhood to
symptoms of mild heart
early adulthood. The right to
failure in childhood.
left shunt causes cyanosis.
• Heart failure usually resolves
with medical therapy and with
time as the child grows and the
VSD gets smaller in absolute
and/or relative terms.
CLINICAL MANIFESTATIONS
Dyspnea and fatigue in patients
with VSDs result from:
• Progressive left ventricular
overload due to the VSD
• Significant aortic regurgitation
• Pulmonary hypertension
(PHTN)
• Double chambered right
ventricle (DCRV).
• Syncope is a rare symptom in
patients with VSD
• Patients with VSDs may also
present with arrhythmias or
sudden death
• Membranous VSD may
close spontaneously via
adherence of the septal
leaflet of the tricuspid valve,
which forms an aneurysm of
the membranous septum
• Septal aneurysm may
cause a new midsystolic
click and may lead to
syncope due to RV outflow
obstruction.
Exams and Tests
Listening with a stethoscope usually reveals a heart
murmur. The loudness of the murmur is related to the
size of the defect and amount of blood crossing the
defect.
Tests may include:
• Chest x-ray -- looks to see if there is a large heart
with fluid in the lungs
• ECG -- shows signs of an enlarged left ventricle
• Echocardiogram -- used to make a definite diagnosis
• MRI of the heart -- used to find out how much blood
is getting to the lungs
• Cardiac catheterization (rarely needed, unless
there are concerns of high blood pressure in the
lungs)
Treatment
• Small VSD: no treatment may be needed. But closely
monitored to make sure that the hole eventually closes.
• Large VSD: who have symptoms related to heart failure
may need medicine to control the symptoms and surgery
to close the hole.
• If symptoms continue, even with medication, surgery to
close the defect with a patch is needed.
• Some VSDs can be closed with a special device during a
cardiac catheterization, which avoids the need for
surgery, but only certain types of defects can successfully
be treated this way.
INDICATIONS FOR INTERVENTION
VSD closure indications include:
• There is a clinical evidence of
left ventricular volume
overload.
• History of infective
endocarditis.
• Left to right shunting with
pulmonary artery pressure
less than two thirds of
systemic pressure and
pulmonary vascular resistance
is less than two-thirds of
systemic vascular resistance.
• Closure of a VSD is
reasonable when there is net
left to right shunting in the
presence of left ventricular
systolic or diastolic
dysfunction or failure.
• Closure of a VSD is not
recommended in patients
with severe irreversible
pulmonary artery
hypertension