Ventricular Septal Defects

1. Ventricular Septal Defects Tate Gisslen, MD Mentor: Bradley S. Marino, MD, MPP, MSCE May 6, 2011

2. Anatomy • 4 morphological components of septum • Membranous • Inlet • Outlet/Infundibular • Muscular/Trabecular

3. Anatomy • Membranous-70-80% • Small • Located at base, between inlet and outlet • Perimembranous - Extends to adjacent septum Membranous Membranous

4. Anatomy • Inlet • Inlet 5-8%, • AV valve to chordae attachments Inlet

5. Anatomy • Outlet/Infundibular • 5-7% • Separates L and R outflow tracts Infundibular

6. Anatomy • Muscular/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

7. Physiology • Blood flow (which way and how much) dependent on multiple factors • Small and restrictive • Lesion size • Large and non-restrictive • Balance between pulmonary and systemic vascular resistance

8. 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

9. 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”

10. Clinical Features-Small Lesions • Murmur • 4 to 10 days, early with rapid decrease in PVR • Asymptomatic • normal feeding, growth and development

11. Murmurs • Restrictive VSD - Holosystolic murmur • correlates with continuous pressure gradient • Non-restrictive large VSD – no murmur (no turbulence if no gradient)

12. Clinical Features-Large Lesions • Accentuated precordial activity • More prominent as LV volume increases • Signs/symptoms of CHF • Diaphoresis • Tachypnea • Fatigue with feeding • Hepatomegaly • Rales • Duskiness with crying • May develop as early as 2 weeks • Severity increases as PVR decreases

13. Evaluation • Chest Radiography • Cardiomegaly • Increased pulmonary vasculature • Pulmonary edema

14. CXR of VSD

15. Evaluation • EKG • Small: normal or LVH • Prominent Q, R, and T waves in II, III, aVF and V6 • Large: Biventricular hypertrophy • RVH- rsR’ in V1, S wave in V6

16. Echocardiography • Assess indication in consultation with Cardiology • Assess location, size, and multiplicity • RV and PA pressure • Assess for LA and LV dilation • Assess LV function • Note relation to great vessels, AV valves

17. Cardiac Catheterization • Able to document • Number of defects • Presence of associated defects • Magnitude of shunt • Estimate PVR • Not used if information apparent by other means • Most information available through Echocardiography

18. Prevalence • Most common congenital heart lesion • Occurs in 50% of children with heart lesions • 15-20% in isolation • 5-50 per 1000 live births • 56% female

19. Associated Defects • Left Heart Defects • Aortic stenosis • Coarctation of the aorta • Right Heart Defects • Tetrology of Fallot • Double Outlet Right Ventricle • Truncus Arteriosus • Some single ventricle (e.g. Tricuspid atresia, double inlet left ventricle)

20. Chromosomal Disorders associated with VSD • Trisomy 21: 40% of T21 will have VSD • Trisomy 13, 18: 18% of T13, 31% of T18 will have VSD • 22q11 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 12 • Recurrence risk for VSD based on parental VSD • Paternal 2% • Maternal 6-10%

21. Treatment for Small VSD • No medication or surgery if asymptomatic • 75-80% close by 2 years • Observation • No antibiotic prophylaxis for procedures

22. Treating a Moderate to Large VSD • Treatment of CHF • Determining when to repair

23. CHF Treatment • High-calorie formula • Medication • Diuretics • Furosemide with or without spironolactone • Afterload reduction • Enalapril or Captopril • Digoxin (maybe) • Symptoms of CHF improve as L to R shunt decreases

24. Indications for Intervention • Decompensated CHF • Compensated 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

25. 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

26. Post-Intervention • Most infants have normal growth and development • Early closure (< 1 year) associated with better LV function and regression of hypertrophy • Residual VSD is not common • RBBB is common following surgery • Rare complete heart block

27. Surgery • Typically dacron patch closure • Sometimes primary closure • Surgical mortality < 1%

28. Catheter Closure • Location • Muscular • Perimembranous • Complications • Heart block • Valve insufficiency

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