Tate Gisslen, MDMentor: Bradley S. Marino, MD, MPP,

MSCEMay 6, 2011

Ventricular Septal Defects

Anatomy4 morphological components of septum

MembranousInletOutlet/InfundibularMuscular/Trabecular

AnatomyMembranous-70-80%

SmallLocated at base, between inlet and outletPerimembranous - Extends to adjacent septum

Membranous Membranous

AnatomyInlet

Inlet 5-8%, AV valve to chordae attachments

Inlet

AnatomyOutlet/Infundibular

5-7% Separates L and R outflow tracts

Infundibular

AnatomyMuscular/Trabecular (5-20%)

Anterior/Marginal (anterior to septal band)Midmuscular/Central (posterior to septal band)Apical (inferior to moderator band)Posterior (beneath septal leaflet)

Muscular

PhysiologyBlood flow (which way and how

much) dependent on multiple factorsSmall and restrictive

Lesion sizeLarge and non-restrictive

Balance between pulmonary and systemic vascular resistance

Lesion Size• Restrictive VSD–< 0.5 cm2 (Smaller than Ao valve orifice area)–Small L to R shunt–Normal RV output–75% spontaneously close < 2yrs

• Non-restrictive VSD–> 1.0 cm2 (Equal to or greater than to Ao valve

orifice area)–Equal RV and LV pressures– Large hemodynamically significant L to R shunt–Rarely close spontaneously

Vascular Resistance• Pulmonary resistance may remain high longer in

infants with large VSD– Minimal L to R shunt

• Decreasing pulmonary resistance leads to significant L to R shunt – Clinical symptoms of CHF

• Persistent L to R shunt leads to hypertrophy of the medial smooth muscle layer of the pulmonary arteries which increases PVR and potential R to L shunting

• Long-standing L to R shunting that results in chronically increased PVR may lead to persistent R to L shunting described as “Eisenmenger Physiology”

Clinical Features-Small LesionsMurmur

4 to 10 days, early with rapid decrease in PVR

Asymptomatic normal feeding, growth and development

MurmursRestrictive VSD - Holosystolic murmurcorrelates with continuous pressure gradient

Non-restrictive large VSD – no murmur (no turbulence if no gradient)

Clinical Features-Large LesionsAccentuated precordial activity

More prominent as LV volume increasesSigns/symptoms of CHF

DiaphoresisTachypneaFatigue with feedingHepatomegalyRalesDuskiness with crying

May develop as early as 2 weeksSeverity increases as PVR decreases

Evaluation

Chest RadiographyCardiomegalyIncreased pulmonary vasculature

Pulmonary edema

CXR of VSD

Evaluation

EKGSmall: normal or LVH

Prominent Q, R, and T waves in II, III, aVF and V6

Large: Biventricular hypertrophyRVH- rsR’ in V1, S wave in V6

EchocardiographyAssess indication in consultation with Cardiology

Assess location, size, and multiplicity

RV and PA pressureAssess for LA and LV dilationAssess LV functionNote relation to great vessels, AV valves

Cardiac CatheterizationAble to document

Number of defectsPresence of associated defectsMagnitude of shunt Estimate PVR

Not used if information apparent by other meansMost information available through Echocardiography

Prevalence

Most common congenital heart lesion

Occurs in 50% of children with heart lesions

15-20% in isolation5-50 per 1000 live births56% female

Associated DefectsLeft Heart Defects

Aortic stenosisCoarctation of the aorta

Right Heart DefectsTetrology of FallotDouble Outlet Right Ventricle

Truncus ArteriosusSome single ventricle (e.g. Tricuspid

atresia, double inlet left ventricle)

Chromosomal Disorders associated with VSD

Trisomy 21: 40% of T21 will have VSDTrisomy 13, 18: 18% of T13, 31% of T18 will

have VSD22q11 deletion:

Tetrology of Fallot is most common anomaly VSD with or without aortic arch anomaly is second most

common Holt-Oram (Hand-heart syndrome): TBX5 gene

found on Chromosome 12Recurrence risk for VSD based on parental VSD

Paternal 2%Maternal 6-10%

Treatment for Small VSDNo medication or surgery if asymptomatic75-80% close by 2 years

Observation

No antibiotic prophylaxis for procedures

Treating a Moderate to Large VSD

Treatment of CHF

Determining when to repair

CHF TreatmentHigh-calorie formulaMedication

DiureticsFurosemide with or without

spironolactoneAfterload reduction

Enalapril or Captopril Digoxin (maybe) Symptoms of CHF improve as L to R shunt decreases

Indications for InterventionDecompensated CHFCompensated CHF with:

Large hemodynamically significant VSD - L to R shunting with Qp/Qs > 2:1, even if asymptomatic, ideally before 1 year

Growth failure, unresponsive to medical therapy is an indication for surgery

Not Indicated

Small VSDs - 6 months without CHF or ↑PVR

Small hemodynamically insignificant VSD – L to R shunting with Qp/Qs < 1.5:1

Eisenmenger physiology

Post-InterventionMost infants have normal growth

and developmentEarly closure (< 1 year) associated

with better LV function and regression of hypertrophy

Residual VSD is not commonRBBB is common following surgery

Rare complete heart block

Surgery

Typically dacron patch closure

Sometimes primary closure

Surgical mortality < 1%

Catheter ClosureLocation

MuscularPerimembranous

ComplicationsHeart blockValve insufficiency

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