Ventricular Septal Defects ICM-9CM Code 745.4 Ventricular Septal Defect 1. Definition: A.

VSD Refers To An Abnormal Communication Through The Septum Separating The Right Ventricle & Left Ventricle B. VSD: May Be: 1. Small Or Large 2. Single Or Multiple 2. Classification: A. Membranous: 75-80% Of Cases B. Muscular Or Trabecular: 5-20% Of Cases C. Canal Or Inlet: 8 % Of Cases 1. Defect Commonly Lies Beneath The Septal Leaflet Of The Tricuspid Valve 2. Often Associated With Down Syndrome D. Subarterial , Outlet, Infundibular or Cristal 5-7% 1. Least Common Type 2. Defect Usually Found Beneath Aortic Valve 3. May Lead To Aortic Regurgitation 3. Generalities: A. VSDs Are The Most Common Congenital Heart Defect At Birth. B. Many VSDs Close At Birth Or Spontaneously In Childhood

4. Epidemiology: A. Isolated VSD: Most Common Congenital Heart Abnormality At Birth : Accounts For 30% of All Congenital Heart Defects B. Prevalence: 1.17/1000Live Births 0.5 /1000 Adults C. VSD: 25% of All Congenital Heart Defects 10% of Heart Defects in Adults (Due to Spontaneous Closure of Many VSDs in Adulthood) D. May Be Associated With: 1. ASD: 35% 2. PDA: 22% 3. Coarctation of Aorta: 17% 4. Subvalvular Aortic Stenosis: 4 % 5. Subpulmonic Stenosis E. Multiple VSDs: More Prevalent In Patients With Tetralogy Of Fallot & Double Outlet RV Defects 5. Clinical Features: A. Small VSDs Are Asymptomatic B. Large Defects 1. Give Rise To: L To R Shunts 2. Thus Lead To Pulmonary Hypertension and CHF. 3. Then the Shunts Become R to L 4. Cyanosis Occurs Early Requiring Earlier Surgical Intervention C. Small or Medium Sized VSDs: 1. May Give Rise To Jet Lesions In The Right Ventricle With Superimposed Infective Endocarditis.

D. Infants 1. May Be Symptomatic At Birth Due to Elevated Pulmonary Artery Resistance 2. Over The First Few Weeks Of Life Pulmonary Arterial Resistance Decreases 3. Thus Allow More Left To Right Shunting Causing LV Volume Overload: A. Tachypnea B. Failure To Thrive C. Congestive Heart Failure E. Adults 1. Left To Right Shunt In the Absence Of Pulmonary Stenosis & Pulmonary Hypertension 2. Typical Symptoms: Heart Failure A. Shortness of Breath B. Orthopnea C. Dyspnea on Exertion 6. Physical Findings: Spectrum of Findings May Include: A. Holosytolic Murmur Heard Best Along Left Sternal Border B. Systolic Thrill C. Mid-Diastolic Rumble Heard at Apex D. S/3 E. Rales F. With Development Of Pulmonary Hypertension 1. Augmented Pulmonic Component Of S/2 2. Cyanosis 3. Clubbing 4. Right Ventricular Heave 5. Signs Of Right Sided Heart Failure (Seen In Eisenmengers Complex With Reversal of Shunt From Right To Left Direction)

7. Laboratory Tests: A. Non-Specific B. CBC: 1. Polycythemia Especially With Eisenmenger Complex C. ABG: 1. Hypoxemia 8. Imaging Studies A. Chest X-Ray: 1. Cardiomegaly: A. Due To Volume Overload Due To Magnitude of Shunt 2. Enlargement of Proximal Pulmonary Arteries 3. Redistribution & Pruning Of Distal Pulmonary Vessels Resulting From Sustained Pulmonary Hypertension B. EKG: 1. Varies According To Size of VSD & Presence of Pulmonary Hypertension 2. Large VSDs: A. Right Axis Deviation + B. Right Ventricular Hypertrophy C. Echocardiography: 1 2D Echo: & Color Doppler Echo A. Displays Size & Location Of The VSD 2. Continuous Wave Doppler: A. Only Approximates The Gradient Between the LV & RV 3. Magnitude of Shunt: A. Determined By Calculation Of Pulmonary To Systemic Flow Ratio D. Cardiac Catheterization A. May Confirm Right Heart Echo Pressures & Calculation of Pulmonary To Systemic Flow Ratio E. Ventriculography: A. May Help Locate VSD

9. Management: A. Non-Pharmacologic Rx: 1. Observation In Children If: A. Small Asymptomatic VSDs: 1. With Pulmonary/Systemic Flows Ratios <1.5 & 2. No Evidence of Pulmonary Hypertension 2. If CHF: A. Salt Restriction B. Oxygen B. Indications For Surgery: 1. Infants With CHF 2. Children Between Ages 1-6 Years With Persistent VSD & Pulmonary/Systemic Blood Flow Ratios 2.1 3. Adults: With VSD A. Pulmonary/ Systemic Flow Ratios : > 1.51 4. Percutaneous Transcatheter Closure By Amplatzer Occluder Devices A. Successful For Muscular VSD Closure In Small Studies of Congenital VSD & Anecdotally In Post Infarct VSDs

10. Disposition: A. Natural History of Isolated VSDs Depend On the Type Of Defect, Its Size & Associated Findings B. Approximately: 75-80% of Small VSDs Close Spontaneously By Age 10 Years C. Large VSDs: Only 10-15% Close Spontaneously D. Large VSDs Left Untreated: Lead To 1. Arrhythmias 2. CHF 3. Pulmonary Hypertension 4. Eisenmenger Complex E. Eisenmenger Complex: 1. Carries A Poor Prognosis 2. Most Patients Die Before Age 40 Years