Download Management of VSD (Ventricular septal defect)

Management of VSD
(Ventricular septal defect)
Overview



The most common lesion seen in congenital heart
disease (about 2.5 per 1000 live births).
Ventricular defects may be located anywhere in
the ventricular septum, single or multiple, of
variable size and shape, with associated defects.
Ventricular defects are classified by their location
in the septum -> 4 types.
Anatomy
Anatomy
1. Membranous defects:
 Originally considered the most common type, are
now just more common in complicated lesions than
muscular defects.
 A small translucent structure located immediately
superior to the division of the septal band and
adjacent to the commissure between the anterior and
septal leaflets of the tricuspid valve. It lies directly
under the aortic valve on the left side and overlaps a
small segment of the right atrium.
 Associated aortic valvar abnormalities, ventricular
Aortic rim
Perimembranous VSD
Anatomy
2. Muscular defects:
 May be located anywhere in the apical,
mid, anterior or posterior muscular
septum and are often multiple.
 With echocardiography, now more
frequently identified & more common
than membranous defects when
uncomplicated.
Anatomy
3. Infundibular (subpulmonary) defects:
 Located under the pulmonary valve when
viewed from the right ventricle &
immediately beneath the aortic valve
viewed from the left ventricle.
 Associated prolapse of the adjacent right
coronary aortic valve cusp…
Anatomy
4. Endocardial cushion type of defects:
 Located beneath the tricuspid valve,
extending to the tricuspid valve ring.
 Occupy the area when an
atrioventricularis communis opening
would be found.
MANAGEMENT
Management
1. Influence of size of defect:
a) Small VSDs:



The child who has reached the age of 6 months without
evidence of congestive heart failure and pulmonary
hypertension can be managed conservatively.
After the 1st year of life, asymptomatic infants known to
have small, persistent patent defects should be examined
every 3 years or so to watch for aortic valve prolapse or
regurgitation, document any defect decrease in size.
Prophylactic antibiotics to prevent infective endocarditis.
Management
b) Large VSDs:
 Mortality rate with surgical closure < 1 %
 repair even in the first few months of life.
 Neonates & infants with poor growth, an single
unrestrictive defect, pulmonary artery
pressure (PAP) at or near systemic level:
surgical closure should be undertaken.
 Those with multiple defects, as long as they are
reachable through the tricuspid valve: also
should undergo surgical closure.
Management



Babies with restrictive defects, PAP < 60 %
systemic level, adequate growth: can be
followed medically.
In older patients, large defects with significant
shunts & left ventricular enlargement: should
be closed either surgically or catheterdelivered devices.
Catheter-delivered devices have been mostly
applied to patients > 1 year old.
Management
2. Influence of type of defect:
a) Membranous defects:
 Many cases often become smaller, with up to
27 %, including some large defects, closing
spontaneously -> patients with good growth &
low PAP should be followed medically.
 Malalignment membranous defects are rare,
usually large & associated with pulmonary
hypertension: should be surgically closed.
Management
b) Muscular defects: Very common, often multiple.
 Lesions may close spontaneously  follow infants
conservatively for some months as long as they are
doing well.
 If significant shunting persists with elevated PAP:
primary closure indicated.
 Apical and anterior defects are best managed with
catheter-delivered devices (in patients > 6
months), whereas those proximal to the moderator
band are accessible surgically at any age.
 Pulmonary artery banding is essentially no longer
A. At age 4 months, huge left-to-right shunt (Qp/Qs > 4/1) through multiple
muscular VSDs (arrows)
B. At age 14 months, spontaneous closure of defects with elimination of
shunt.
Management
c) Infundibular (Subpulmonary) defects: Rarely
small, not known to get smaller, aortic
regurgitation develops commonly  ALL
patients with significant anatomic defects are
referred to surgeons after the age of 6 months
or earlier if there is growth failure.
d) Endocardial cushion type of defect:
Uncommonly small, do not regress
spontaneously or get smaller  surgery is
ALMOST invariably needed.
COURSE
1. Spontaneous diminution in size: Frequencies
vary considerably, related to age, defect
location, follow-up duration, methods of
detection, particularly echocardiography.
 Inlet (endocardial cushion), typical
malalignment & subpulmonary defects are
usually large and remain so.
 Membranous and muscular defects often
decrease or close.
COURSE
2. Development of Pulmonary Vascular
Disease (Eisenmenger’s complex):
 The development of permanent Pulmonary
Vascular Disease is very rare before the
first birthday.
 More common with a large VSD, multiple
VSDs or associated patent ductus arteriosus
(PDA).
 Early surgery can minimalize this disease.
Development of Pulmonary Vascular Disease
(Eisenmenger’s complex)


Any susgestion of pulmonary hypertension
in patients > 12 months with VSD require
cardiac catheterization for evaluation.
Virtually all children with VSD & any
evidence of pulmonary vascular disease
undergo surgical correction in the first year
of life because it is highly likely that
pulmonary vascular disease must be of
recent onset.
Development of Pulmonary Vascular Disease
(Eisenmenger’s complex)


Beyond the age of 12 months, the decision to
operate depends on whether the vascular
change is minimal or advanced, is of recent
onset or show evidence of reversibility.
Reference for VSD closure: pulmonary
resistance estimated < 8 U/m2 & any
evidence of developing pulmonary vascular
disease, Qp/Qs > 2:1, clearly response to
dilator therapy (Oxygen, Nitric oxide).
Development of Pulmonary Vascular Disease
(Eisenmenger’s complex)


Contraindication for VSD closure: pulmonary
resistance > 8 U/m2, particularly those who have
no left-to-right shunt, those older than 2 years,
and those who show no response to pulmonary
arterial dilatation.
Women with pulmonary vascular disease
secondary to congenital heart problems must not
be pregnant & not use pills or mechanical devices
of contraception.
COURSE
3. Acquired aortic regurgitation:
Due to prolapse of one or more valve
cusps into the adjacent ventricular
defect (membranous or
subpulmonary), a bicuspid aortic
valve…
Acquired aortic regurgitation
a) Subpulmonary defects with aortic regurgitation:
 It is reasonable to recommend surgical closure of
any subpulmonary defects (other than rare tiny
ones) shortly after discovery (Stable patient > 15
years old, minimal shunt, mild regurgitation may
be continued medical observation).
 Mild regurgitation: closure alone is sufficient.
 Moderate or more regurgitation: valve plasty is
quite effective.
 Valve replacement is rarely needed, except in
older patients.
Acquired aortic regurgitation
b) Membranous defect with aortic regurgitation:
Obvious prolapse, and a tricuspid valve:
similar management indications as for
subpulmonary defects are reasonable.
Note: Strict endocarditis prophylaxis is vital in
these patients, before & after surgery.
COURSE
4. VSDs & aortic stenosis:
 VSDs may be membranous or muscular,
aortic stenosis may be valvar or subvalvar.
 VSDs may decrease in size or close
spontaneously and aortic stenosis lesions
may progress -> management varies
considerably & depends on the current
status.
VSDs & aortic stenosis
 Stable
small defects & mild or less
obstruction can be followed
medically indefinitely.
 Stable neonates with moderate
septal defects & minimal
obstruction can also be followed
medically, initially frequently.
VSDs & aortic stenosis




Symptomatic, large defect with high PAP, mild
stenosis: surgical closure alone is necessary.
Small defect with significant valvar outflow
obstruction: ballon dilation is the treatment of
choice.
Moderate or more subaortic obstruction:
surgical reresection is necessary but better
delayed ≥ 10 years because of common
recurrent obstruction.
Note: continued endocarditis prophylaxis is
essential.
COURSE
5. VSD & secundum atrial septal defect (ASD):
 Some ASDs are dilated patent foramen ovale
(PFO) because of the left atrial hypertension &
atrial left-to-right shunting which disappear
after closure of VSD.
 In patients with true secundum ASD, the left
atrial overload resulting from the VSD is
relieved by the ASD, and there is no atrial
hypertension.
 This distinction is not important because it has
the same operation route.
COURSE
6. VSD & PDA:
 When both defects are large and the
amount of left-to-right shunting is
determined by the pulmonary resistance,
even if it is large, may not affect the size of
shunt.
 From a practical standpoint, both defects
are closed at surgery in the infant without
any additional risk.
VSD & PDA



Restrictive VSD: PDA closure alone may be
benificial.
Significant lesions in very young patients:
surgical closure of both defects.
Normally growing infant with clearly
restrictive defects may be followed medically;
if PDA persists, it may be closed by coiloccluded catheterization.
VSD & PDA


Older patient with significant lesions and
low pulmonary resistance: close defects
surgically or by devices.
Elevated pulmonary resistance suspected:
catheterization to evaluate response to
oxygen & nitric oxide and temporary
occlusion and device closure of the ductus if
indicated.
COURSE
7. VSDs with Pulmonary stenosis:
a) Acyanotic Tetralogy of Fallot: differential
diagnosis by large VSD, infundibular pulmonary
stenosis, overriding aorta, the systemic level right
ventricular pressure.
b) Ventricular defect and valvar pulmonary stenosis:
Treatment is indicated or not depends on the
severity of the defects.
c) VSD with double-chambered right ventricle:
Muscle bundles traverse and obstruct the right
ventricular outflow tract, lesion is usually
COURSE
8. VSD & Mitral valve (MV) disease:
a) VSD & Mitral valve stenosis:
 Minimal MV stenosis, moderate or less
pulmonary hypertension: management is the
same as that for the usual VSD.
 Moderate MV stenosis, VSD of some size, there
will be systemic levels of pressure in the
pulmonary artery. The amount of left-to-right
shunting depends largely on the comparative
levels of pulmonary & systemic resistance.
VSD & Mitral valve stenosis


Severe MV stenosis, level of pulmonary
resistance may become so high ->
ventricular shunting reverses, cyanosis. The
ultimate success of management depends on
the success in treating MV stenosis.
Infant with severe MV stenosis, large VSD:
surgical defect closure + valvuloplasty. If
VSD is small: ballon dilation may apply to
MV stenosis.
VSD & Mitral valve stenosis

Older patient with large VSD, significant
MV stenosis: Catheterization for
hemodynamic evaluation. If there is high
pulmonary resistance, ballon dilation may
relieve stenosis, decrease resistance &
increase left-to-right shunting to close VSD
safely.
COURSE
b) VSD & mitral regurgitation: management
depends on clinical status, degree of
regurgitation, size of VSD.
 Both lesions are mild: medical management
indicated.
 Large left-to-right shunt at the ventricular
level & not severe regurgitation: VSD closure
alone may improve the regurgitation.
 Severe regurgitation: valvuloplasty is usually
effective in the short term.
Catheterization data in a 4-months-old infant with 2:1 left-to-right shunt,
membranous VSD (black arrow), and moderate mitral regurgitation through
anterior leaflet cleft (open arrow). Four years later, the VSD had
spontaneously closed, and at age 10 years, the mitral cleft was successfully
closed surgically.
SUMMARY
Type
Size
AP/PS RP/RS Qp/Qs
Ia
Resistive
<0.3
<0.3
1.0-1.5
Ib
Resistive
<0.3
<0.3
1.5-2
IIa
Resistive
0.3-0.7 <0.5
>2
IIb
Non
resistive
0.7-1
>2
<0.8
Hemodynamic Classification
Nom
III
VI
Size
Non
resistive
Non
resistive
AP/PS RP/RS Qp/Qs
>1
>1
<1
<0.7
<0.5
>2
VSD Ia and Ib: Malade de Roger
VSD IIa and IIb: VSD high debit
VSD III: Eissenmenger (large VSD)
VSD IV: VSD and Pulmonary stenosis
VSD management
VSD IA (Roger):Follow up
VSD IIa: Surgery if
Medical treatment failure
Pulmonary pressure increasing
LV too large
More than 1 year
VSD IIb: Surgery if single VSD, PA banding
multiple VSD patient <9month total
correction after
VSD+IA, VSD + RVOT obstruction…: Surgery
VSD type IB ????
+ 75% of VSD
+ Wait and see ?
+ Osler, AR, LV hemodynamic …
+ Surgery complication?
+ Intervention complication?
Reference
1.
2.
3.
4.
5.
6.
7.
8.
9.
Lillehei C.W., Cohen M., Warden H.E., Ziegler N.R., Varco R.L. The
results of direct vision closure of ventricular septal defects in eight patients
by means of controlled cross circulation. Surg Gynecol Obstet
1955;101:447-466.
Kumar K., Lock J.E., Geva T. Apical muscular ventricular septal defects
between the left ventricle and the right ventricular infundibulum.
Diagnostic and interventional considerations. Circulation 1997;95:12071213.[Abstract/Free Full Text]
Jonas R.A. Cerebral protection in infants. In: Yacoub M., Pepper J., eds.
Annual of cardiac surgery, 8th ed. London: Current Science, 1994:153-160.
Cooley D.A., Belmonte B.A., DeBakey M.E., Latson J.R. Temporary
extracorporeal circulation in the surgical treatment of cardiac and aortic
disease. Report of 98 cases. Ann Surg 1957;145:898-914.
Doty D.B., McGoon D.C. Closure of perimembranous ventricular septal
defect. J Thorac Cardiovasc Surg 1983;85:781-790.[Medline]
Stellin G., Milanesi O., Rubino M., et al. Repair of tetralogy of Fallot in the
first six months of life. Ann Thorac Surg 1995;60:S588-S591.
Hobbins S.M., Izukawa T., Radford D.J., Williams W.G., Trusler G.A.
Conduction disturbances after surgical correction of ventricular septal
defect by the atrial approach. Br Heart J 1979;41:289293.[Abstract/Free Full Text]
Nadal text book of Pediatric cardiology. 2009
Anderson R.H., Wilcox B.R. The surgical anatomy of ventricular septal
defect. J Card Surg 1992;7:17-35.[Medline]
1.
2.
3.
4.
5.
6.
7.
8.
9.
Pridjian A.K., Pearce F.B., Culpepper W.S., Williams L.C., Van Meter C.H., Ochsner
J.L. Atrioventricular valve competence after takedown to improve exposure during
ventricular septal defect repair. J Thorac Cardiovasc Surg 1993;106:1122-1125.[Abstract]
Griffiths S.P., Turk G.K., Ellis K., et al. Muscular ventricular septal defects repaired with
left ventriculotomy. Am J Cardiol 1981;48:877-886.[Medline]
Von Segesser L.K., Fasnacht M.S., Vogt P.R., Genoni M., Arbenz U., Turina M.I.
Prevention of residual ventricular septal defects with fibrin sealant. Ann Thorac Surg
1995;60:511-516.[Abstract/Free Full Text]
Fishberger S.B., Bridges N.D., Keane J.F., et al. Intraoperative device closure of
ventricular septal defects. Circulation 1993;88:205-209.
Breckenridge I.M., Stark J., Waterston D.J., Bonham-Carter R.E. Multiple ventricular
septal defects. Ann Thorac Surg 1972;13:128-136.[Medline]
Momma K., Toyama K., Takao A., et al. Natural history of subarterial infundibular
ventricular septal defect. Am Heart J 1984;108:1312-1317.[Medline]
Chauvaud S., Serraf A., Mihaileanu S., et al. Ventricular septal defect associated with
aortic valve incompetence. Ann Thorac Surg 1990;49:875-880.[Abstract]
Yacoub M.H., Khan H., Stavri G., Shinebourne E., Radley-Smith R. Anatomic
correction of the syndrome of prolapsing right coronary aortic cusp, dilatation of the
sinus of Valsalva and ventricular septal defect. J Thorac Cardiovasc Surg 1997;113:253261.[Abstract/Free Full Text]
Kirklin J.W. The movement of cardiac surgery to the very young. In: Crupi G., Parenzan
L., Anderson R.H., eds. . Perspectives in pediatric cardiology. Mt Kisco, New York:
Futura Publishing Co, 1989:3-20.