Ventricular Septal Defects

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)

Morphology The Ventricular Septum

Morphology The Ventricular Septum

1. 2. 3. 4. 5.

Membranous Outflow Trabecular septum Inflow Subarterial / Supracristal

VSD Types

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

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 > 2.2 : 1) Eisenmenger VSD irreversible pulmonary HTN and shunt may be zero or reversed (i.e. RL)

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

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

Echo Example 1

Echo Example 1

Echo Example 3

Echo Example 3

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

Clinical Scenarios & Recommendations

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

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

Interventions
Indications for Surgical Closure in adults: Evidence of LV volume overload (Class I if Qp/Qs >2, Class IIa if Qp/Qs > 1.5) History of bacterial endocarditis (Class I) Significant LR shunt with PA pressure < 2/3 systemic and PVR is < 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 < 2% in most centers

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

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

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