Echo Conference 5/11/11

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Ventricular Septal Defects

• Echo Conference 5/11/11
• Darryn Appleton

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Outline

• Morphology, Types Pathophysiology
• Natural History and Clinical Presentation
• Some Echo examples
• Clinical Scenarios and Recommendations
• Interventions Indications, Surgery, Percutaneous
• Pregnancy and Endocarditis Prophylaxis
• Review Questions

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Introduction

• The most common form of CHD, accounting for up to
  20-40 of patients diagnosed with CHD
• Impact may range from asymptomatic to pulmonary
  HTN, LV volume overload and RVH
• Morphology 4 types
• Membranous most common type in adults (80)
• Muscular most common type in young children
• Complete AV septal (endocardial cushion) defects
• Supracristal (subarterial)

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Morphology The Ventricular Septum
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Morphology The Ventricular Septum

• Membranous
• Outflow
• Trabecular septum
• Inflow
• Subarterial / Supracristal

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VSD Types
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VSD Types
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VSD Types
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Pathophysiology

• Defect size is often compared to aortic annulus
• Large gt 50 of annulus size
• Medium 25-50 of annulus size
• Small lt25 of annulus size

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Pathophysiology

• Restrictive VSD is typically small, such that a
  significant pressure gradient exists between the
  LV and RV (high velocity), with small shunt
  (Qp/Qs 1.4 1)
• Moderately restrictive VSD ? moderate shunt
  (Qp/Qs 1.4 to 2.2 1)
• Large / non-restrictive VSD ? large shunt (Qp/Qs
  gt 2.2 1)
• Eisenmenger VSD ? irreversible pulmonary HTN and
  shunt may be zero or reversed (i.e. R?L)

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Natural History

• Restrictive typically does not have hemodynamic
  impact and may close spontaneously
• Location Location Location Subaortic may result
  in progressive AI
• Moderately restrictive does create LV overload
  and dysfunction along with variable increase in
  PVR
• Large / non-restrictive LV volume overload
  earlier in life with progressive pulm HTN and
  ultimately Eisenmenger syndrome

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Clinical Features

• Peds
• Murmur
• Dyspnea, CHF, Failure to thrive
• Adults
• Asymptomatic murmur harsh, pansystolic, left
  sternal border
• Mod restrictive dyspnea, a.fib, displaced apex,
  murmur, S3
• Non-restrictive Eisenmenger VSD central
  cyanosis, clubbing, RV heave, loud P2

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Echo Example 1
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Echo Example 1
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t
Outlet VSD Para long axis
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Echo Example 2
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Echo Example 2
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Echo Example 2
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Supracristal VSD, with pulm outflow tract
obstruction
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Echo Example 3
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Echo Example 3
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Echo Example 3
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Echo Example 3
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Echo Example 3
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Echo Example 3

• Type
• Size

Membranous
Restrictive
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Echo Example 4
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Echo Example 4
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Echo Example 3
Type Size Shunt
Muscular
Large / Non-restrictive

• R?L (inc RH pressures)
• RV dilated
• Eisenmengers

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Clinical Scenarios Recommendations

• Symptomatic young infant with Pulm HTN
• Early surgery within 3 months.
• Medical therapy with diuretics /- ACEI pre-op
• Asymptomatic pt without Pulm HTN but with LV
  overload
• Closure usually recommended to avoid late LV
  dysfunction
• Asymptomatic pt, small VSD, no LV dilation
• Conservative
• Asymptomatic pt, small VSD but with AI/prolapse
• Peri-membranous VSD with more than trivial AI
  should have surgery

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Clinical Scenarios Recommendations

• Eisenmenger Syndrome
• Supportive
• Bosentan (Endothelin receptor antagonist)
  improves functional capacity, QOL
• Sildenafil

Penny DJ, Vick GW. Lancet 2011 377 1103-12
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Interventions

• Indications for Surgical Closure in adults
• Evidence of LV volume overload (Class I if Qp/Qs
  gt2, Class IIa if Qp/Qs gt 1.5)
• History of bacterial endocarditis (Class I)
• Significant L?R shunt with PA pressure lt 2/3
  systemic and PVR is lt 2/3 SVR
• Surgical Closure
• Considered the first-line choice of therapy for
  those with indications
• Usually involves direct patch closure w
  cardio-pulm bypass
• Operative mortality lt 2 in most centers

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Long Term Surgical Outcomes

• Retrospective review of 46 pts with surgical VSD
  repair at Mayo Clinic

Mongeon et al. JACC Int 2010 3 290-7
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Interventional Options

• Percutaneous Device Closure
• Muscular VSDs can typically be closed
  percutaneously
• Class IIb recommendation in Guidelines (i.e.
  surgery still preferred)
• No FDA approved devices for perimembranous VSDs,
  although there are specific devices for this
  purpose
• Concern re proximity of defect to AV node and
  high risk of complete AV block requiring pacemaker

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Pregnancy and VSDs

• Pregnancy well tolerated in women with small to
  moderate sized VSDs as long as there is no
  pulmonary vascular involvement
• Eisenmenger syndrome Pregnancy contraindicated
  due to exceptionally high risk of maternal and
  fetal death

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Endocarditis Prophylaxis for VSD

• Uncomplicated VSD no Abx for dental or other
  procedures required
• Post repair
• Abx for 6 months following surgical or
  percutaneous repair
• Indefinite Abx if there is residual shunt
• Risk of bacteremia from daily life usually
  exceeds that of procedure ? Abx for procedures
  only prevent small of cases
• Focus should be on optimal dental hygiene for
  those with CHD

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Question 1

• An isolated VSD will generally cause enlargement
  of which chamber(s)
• A Left atrium, left ventricle
• B Right ventricle
• C Right ventricle, pulmonary artery
• D Aorta
• E Right ventricle, right atrium

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Question 1

• An isolated VSD will generally cause enlargement
  of which chamber(s)
• A Left atrium, left ventricle
• B Right ventricle
• C Right ventricle, pulmonary artery
• D Aorta
• E Right ventricle, right atrium

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Question 2
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Question 2

• The defect shown on the previous slide is a
• A Muscular VSD
• B Sinus venosus VSD
• C Perimembranous VSD
• D Inlet VSD
• E Supracristal VSD

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Question 2

• The defect shown on the previous slide is a
• A Muscular VSD
• B Sinus venosus VSD
• C Perimembranous VSD
• D Inlet VSD
• E Supracristal VSD

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Question 3

• A common complication of this defect is
• A Pulmonary valve endocarditis
• B Aortic regurgitation
• C Aortic dissection
• D Tricuspid regurgitation
• E Right ventricular enlargement

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Question 3

• A common complication of this defect is
• A Pulmonary valve endocarditis
• B Aortic regurgitation
• C Aortic dissection
• D Tricuspid regurgitation
• E Right ventricular enlargement

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Question 4

• There is no diastolic flow in this perimembranous
  VSD
• A True
• B False

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Question 4

• There is no diastolic flow in this perimembranous
  VSD
• A True
• B False

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Question 5

• A restrictive VSD is a simple lesion with a good
  long term prognosis. However, complications can
  occur. All of the following are possible
  complications of a VSD except
• A Endocarditis
• B Aortic regurgitation
• C Aortic valve prolapse
• D Eisenmenger Syndrome
• E Right sided volume overload

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Question 5

• A restrictive VSD is a simple lesion with a good
  long term prognosis. However, complications can
  occur. All of the following are possible
  complications of a VSD except
• A Endocarditis
• B Aortic regurgitation
• C Aortic valve prolapse
• D Eisenmenger Syndrome
• E Right sided volume overload

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Question 6
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Question 6

• The pulmonary artery systolic pressure in this
  patient with a VSD is
• A Normal
• B Moderately elevated
• C Systemic / Supra-systemic

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Question 6

• The pulmonary artery systolic pressure in this
  patient with a VSD is
• A Normal
• B Moderately elevated
• C Systemic / Supra-systemic

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Question 7

• A patient with a VSD undergoes TTE. BP measured
  at the time of the study is 125/75 (right arm),
  MAP 92. CW doppler across the VSD gives a peak
  velocity of 5 m/s. Assuming RA pressure of 5,
  what is the estimated PASP?
• A 20mmHg
• B 25 mmHg
• C 30 mmHg
• D 72 mmHg
• E 105 mmHg

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Question 7

• A patient with a VSD undergoes TTE. BP measured
  at the time of the study is 125/75 (right arm),
  MAP 92. CW doppler across the VSD gives a peak
  velocity of 5 m/s. Assuming RA pressure of 5,
  what is the estimated PASP?
• A 20mmHg
• B 25 mmHg
• C 30 mmHg
• D 72 mmHg
• E 105 mmHg

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VSD Hemodynamics

• Peak gradient 4 x v2 (Simplied Bernoulli
  equation)
• VSD gradient LV systolic pressure RV systolic
  pressure
• RVSP LVSP - VSD gradient
• RVSP cuff systolic BP - VSD gradient (or 4 x
  v2)
• Assuming no aortic outflow tract obstruction
• PASP RVSP
• Assuming no pulmonary outflow tract obstruction