1. VSD SHABNAM.MOHAMMADZADEH, MD FELLOW OF ADULT CONGENITAL HEART DISEASE

2. A: STRAIGHT HEART TUBE B:BULGING C: C- LOOP a b c d e RV LV O RV LV A O RV LV A O RV LV A 3. WHICH ONE IS INCORRECT 1. Prevalence of VSD s is not race dependent 2. A restrictive VSD may close spontaneously during adult life 3. Late development of subaortic stenosis are well described 4. A moderately restrictive VSD leads to left atrial and ventricular dilation and dysfunction as well as a variable increase in pulmonary vascular resistance 4. PREVALENCE, ETIOLOGY Most common congenital heart defect. accounting for up to 40 % of cardiac anomalies . Chromosomal disorders associated with an increased incidence of VSD , (Down syndrome), (Di George syndrome), (Turner syndrome). Familial forms , TBX5, GATA4, and NKX2.5 mutations . Children from an adult with a VSD that is not associated with a genetic disorder may have a risk of VSD as high as 3 % if the father is affected and a 6 % risk if the mother is affected 5. TYPES OF VENTRICULAR SEPTAL DEFECTS TYPE 1 :( 1 ) subarterial valve; defect: in the left ventricular outflow tract just below the aortic valve (conal,subpul, infundibular, supracristal, doubly commited) ( 4 ) outlet TYPE 2 :( 2 ) perimembranous defect, membranous septum ( yellow , white , and blue dashed circumference for outlet, trabecular and inlet subtype, ) TYPE3 :( 3 ) inlet or atrioventricular defect, which lies inferior to the septal leaflet of the tricuspid valve; and ( 5 ) inlet and apical TYPE 4 : ( 6 ) muscular defects, which are entirely bounded by the muscular septum and are often multiple 6 5 2 1 3 4 6. TYPE 1 Conal,subpul, infundibular, supracristal, doubly commited ( 4 ) outlet Aortic regurgitation (87% IN 20Y) Prolapse of the anterior aortic valve leaflet. ( LCC,RCC 6 % of defects 30% in Asian Spontaneous closure of this type of defect is uncommon Doubly committed subarterial : More common in Asian patients, In the outlet septum, Bordered by fibrous continuity of the aortic and pulmonary valves. 7. TYPE 2 Synonyms: perimembranous, paramembranous,conoventricular SUBTYPES : Inlet, trabecular, outlet, and confluent. (multiple areas of the septum) Most common VSD, (80 % of defects) Bordered by fibrous continuity between the leaflets of an AV valve and an arterial valve. AI (Prolapse of,RCC,NCC) 8. TYPE 3 Synonyms: inlet, AV canal type, endocardial cushion May be associated with AV canal defect. Trisomy 21 syndrome. 58 % of VSDs . 9. TYPE 4(MUSCULAR) Rim totally composed of septal muscle Subclassified as inlet, trabecular outlet, or confluent . 20 % of VSDs in infants Spontaneous closure is common,. Frequently multiple. Swiss-cheese septum 10. GERBODE-TYPE VSD Left ventricle to right atrium. Extremely rare 11. OTHER ANOMALIES Ven septum psudoaneurysm BAV Sub AO stenosis Caoarctation Pulmonary Valve stenosis DCRV AV valve malformations 12. CASE 1 22 y/o male DOE FCII Harsh systolic murmur ,LSB Load P2 Diastolic rumble on apex 13. PATHOPHYSIOLOGY A restrictive VSD 1. Produces a significant pressure gradient between the left ventricle and the right ventricle 2. Pulmonary-to-aortic systolic pressure ratio < 0.3 3. Small (1.4 : 1) shunt. 4. Less than 5mm, or defect size 25% 2.2 2. pulmonary-to-aortic systolic pressure ratio greater than 0.66. 3. Defect diameter >75% of aortic diameter 4. PH in less than 2years 16. PATHOPHYSIOLOGY An Eisenmenger VSD PAP/systolic pressure ratio of 1 Qp/Qs less than 1 : 1 Net right-to-left shunt. 17. NATURAL HISTORY 1. A restrictive VSD may close spontaneously during childhood and sometimes in adult life. 2. A perimembranous defect ,doubly committed VSD, 1. Progressive AR. 2. Subaortic and subpulmonary stenosis 3. Left ventricular to right atrial shunt 3. A moderately restrictive VSD 1. Left atrial and ventricular dilation and dysfunction 2. Variable increase in pulmonary vascular resistance. 4. A large or nonrestrictive VSD 1. Ventricular volume overload early in life 2. Progressive rise in pulmonary artery pressure 3. A fall in left-to-right shunting. 18. WHICH ONE IS INCORRECT? 1. Infants with large nonrestrictive defects tend to present later than restrictive ones 2. Diastolic murmur of MV in neonates with large VSD may occur without any other anatomical disorder 3. Cyanosis is rare in early childhood, 4. Medical management of the symptomatic infant is directed at improving symptoms before surgery 5. Most adult patients with a small restrictive VSD are asymptomatic 19. CLINICAL FEATURE IN NEONATE Restrictive defect, the murmur becomes apparent only as the pulmonary vascular resistance falls. Large nonrestrictive defects tend to present later. breathlessness, congestive heart failure, and failure to thrive in the second and third months a pulmonary ejection murmur and a mitral rumble Cyanosis is rare in early childhood, and if it is present, other causes of a raised pulmonary vascular resistance should be excluded (e.g., mitral stenosis or coexisting lung disease). Medical management of the symptomatic infant : diuretics , ACE inhibition 20. Harrisons grooves caused by the thoracic retractions of chronic dyspnea 21. CLINICAL FEATURE IN ADULT Small restrictive VSD : Asymptomatic. Harsh or high-frequency pansystolic murmur, usually grade 3 to 4/6, maximal intensity at the left sternal border in the third or fourth intercostal space. Moderately restrictive VSD Dyspnea in adult life, perhaps triggered by atrial brillation Displaced cardiac apex with a similar pansystolic murmur as well An apical diastolic rumble Third heart sound at the apex from the increased ow through the mitral valve. Large nonrestrictive Eisenmenger VSDs Central cyanosis and clubbing of the nailbeds Right ventricular heave, a palpable and loud P2, and a right-sided S4. A Pulmonary ejection click, a soft and scratchy systolic ejection murmur, High-pitched decrescendo diastolic murmur of pulmonary regurgitation (Graham Steell). Peripheral edema usually reects right-sided heart failure. 22. MODERATELY RESTRICTIVE 1. LA enlargement 2. LV volume overload 23. ECG Mirrors the size of the shunt and the degree of pulmonary hypertension. Small, restrictive VSDs: normal tracing. Moderate-sized VSDs :left atrial overload ,left ventricular volume overload, deep Q and tall R waves with tall T waves in leads V5 and V6 and perhaps eventually atrial fibrillation. After repair, : usually normal with right bundle branch block. 24. NONRESTRICTIVE,PH 1. RVH 2. LV volume overload 25. NONRESTRECTIVE ,EISENMENGER RVH 26. 1 2 3 27. R LN AVS MS TS DC 28. QP/QS Diameter of RVOT:23 RVOT VTI: 24 Diameter of LVOT: 20 LVOT VTI: 21 29. SUBPULMONIC VSD 30. INLET TYPE VSD 31. AVSD 32. PERIMEMBRANOUS VSD 33. MUSCULAR VSD 34. CARDIAC CATHETERIZATION. 1. Hemodynamic signicance of a VSD is questioned 2. Assessment of pulmonary artery pressures and resistances. 35. CHAMBER SATURATION% PRESSURE(mmH g) IVC 78 HSVC 65 LSVC 67 HRA 67 6 MRA 70 LRA 71 P RV 80 100 DRV 83 100 MPA 84 100 PCWP 12 LV 98 120 AO 98 120 36. INDICATIONS FOR INTERVENTION 1. The presence of a significantVSD 1. The symptomatic patient shows a Qp/Qs > 1.5 : 1, 2. Pulmonary artery systolic pressure > 50 mm Hg, 3. Increased left ventricular and left atrial size, or deteriorating left ventricular function in the absence of irreversible pulmonary hypertension. 2. Presence of a perimembranous or outlet VSD with more than mild aortic regurgitation 3. History of recurrent endocarditis. 4. In children, a nonrestrictive VSD and a smaller VSD with significant symptoms failing to respond to medication. 37. Elective surgery is usually performed between 3 and 9 months of age. PVR< 7 Wood units, closure :safely undertaken if there is a net left-to-right shunt of at least 1.5 : 1 or strong evidence of pulmonary reactivity on challenge with a pulmonary vasodilator (oxygen, nitric oxide). 38. REPRODUCTIVE ISSUES well tolerated :small or moderate VSDs and in women with repaired VSDs. Pregnancy is contraindicated in Eisenmenger syndrome ,high maternal (!50%) and fetal (!60%) mortality. 39. FOLLOW-UP Good to excellent functional class and good left ventricular function before surgical closure, life expectancy after surgical correction is close to normal. The risk of progressive aortic regurgitation is reduced after surgery, as is the risk of endocarditis, unless a residual VSD persists. Yearly cardiac evaluation : with right ventricular outflow tract obstruction, left ventricular outflow tract obstruction, aortic regurgitation not undergoing surgical repair; patients with Eisenmenger syndrome adults with significant atrial or ventricular arrhythmias. late repair of moderate or large defects, which are often associated with left ventricular impairment and elevated pulmonary artery pressure at the time of surgery.