Henri roger French paediatrician Maladie de Roger Rogers disease or defect small muscular VSD bruit de Roger ID: 1014175
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1. Ventricular septal defect
2. Henri roger – French paediatrician Maladie de Roger (Roger's disease or defect – small muscular VSD bruit de Roger (Roger's murmur)- loud pansystolic murmur of VSD
3. Most common congenital heart disease excluding bicuspid aortic valve 20%of VSD occur as a part of complex CHD80% occurs as an isolated defect
4. Chromosomal disorders associated with vsd Trisomy 21- 40% will have inlet VSD Trismy 13 – patau’sTrismy 18 – EdwardsDigeorge syndrome – 22q11 deletionHolt oram syndrome
5. Parts of ventricular septum
6. Mostly muscular VSD refers to trabecular VSD , although muscular part includes both inlet and outlet
7. Types of vsd
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10. Membranous vsdMost common VSD – 80%Perimembranous VSD with outlet extension – TOF typePerimembraneous VSD with inlet extension – ECD typePerimembranous VSD with muscular extensionMembraneous VSD can be partially closed by septal leaflet of tricuspid valve. The conduction pathway passes adjacent to the membranous VSD It directly does not cause any conduction defects , but during surgery , suturing can hamper the conduction pathway leading to a post op RBBB
11. TREATMENT 1/3rd – resolve spontaneouslySurgical closure – standard treatment Percutaneous device- off label
12. Outlet vsd Outlet septum separates the pulmonary arteries from aorta and also seperates the RVOT from LVOTIn outlet VSD, there is a communication between RVOT and LVOTLV directly ejects blood through the pulmonary artery and aorta , doubly commitedIncidence – rare
13. Mechanism of ar in outlet vsd – venutri effect On either side of a high velocity jet , a low pressure zone is created
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17. AR in VSD is independent of size , it’s location matters.Oulet VSD , rarely perimembranous can cause AR . VSD+AR cause Additional LV volume overload .Eisenmenger is rare.Occurs around 3-8 years of age .Cuspal prolapse can partially close VSD
18. Treatment Never closeSurgical closureMild AR – surgical VSD closure onlyModerate AR – repair of aortic valve is considered along with surgical VSD closure.Severe AR – aortic valve repair or replacement with surgical VSD closure is mandatory .
19. Inlet VSD AV canal defects / endocardial cushion defects Down syndrome, Ellisvancrevald syndrome Never close
20. Muscular vsdTrabecular VSDSingle or multiple (swiss cheese septum)
21. May spontaneously close due to septal contractions.2/3 rd of muscular VSD can closeDevice closure –Amplatzer muscular VSD cccluderSurgery
22. Classification by size of the defect
23. hemodynamicsDepends on 3 factorsSize of the VSD RV and LV pressurePVR and SVR
24. Size of the vsd In small VSD , the large pressure difference between LV and RV during a systole , a high velocity get is created.This creates a loud pansystolic murmur with thrillIn case of large VSD , LV and RV behave as a common chamber due to pressure equalization , so murmur will not that much audible
25. When does L to r shunt establishes? Around 4-6 weeks of age This is the time taken for fall in RV pressure below LV pressure.At birth , VSD shunt murmurs are not present
26. Hemodynamic classificationRestrictive- LVSP > > RVSP Pulmo /Aortic systolic pressure ratio < 0.3Qp / Qs < 1.4 : 1Moderately restrictive RVSP high, but less than LVSPQp/Qs 1.4 - 2.2 : 1Pulmo/aortic systolic pressure ratio<0.66
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28. Non restrictive/ large - >1.0 cm2 ( equal to or greater than to the size of aortic orificeQp/Qs > 2.2. Pulmo/ aortic systolic pressure ratio>0.66Flow determined by PVRLarge hemodynamically significant L to R shunt Equal LV and RV pressure- act as single chamber during systole .SBP of LV and RV are identicalPresent as LA, LV volume overload + RV pressure overload.
29. As the size of the VSD increasesMore left to right shuntMore blood flow through pulmonary arteriesHyperkinetic pulmonary artery hypertension
30. Ra/rv in vsd with l to r shuntBlood is ejected into RV during systole , when it is contracting and ejecting blood into pulmonary artery .No volume overload / no dilatation of RVSome times VSD may be directed towards pulmonary artery , pumping into it directly .
31. Large / non restrictive vsdPulmonary artery , LA, LV volume overloadedRV pressure overload leads to increased pulmonary vascular resistanceSigns – PSM -mitral MDM + LVS3 -loud P2 - Narrowed S2 splitEisenmenger – single sound s2.
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33. Hyperkinetic PH develops by 2 months later develops into PVOD (pulmonary vascular obliterative disease)PAH Eisenmenger syndromeRV failureInitially – hyperkinetic PH , later due to PVR
34. Clinical featuresRetarded growth and development Exercise intoleranceRecurrent respiratory tract infectionCHF
35. small/restrictive vsd <0.5 cm2( smaller than aortic valve orifice area)Small L to R shuntNormal RV output75% close spontaneously < 2 years Present as loud PSM LLSB or early systolic murmur in a small muscular VSD .Thrill +Increased Infective endocarditis risk – high pressure jet ,on RV side
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37. SPONTANEOUS CLOSURE OF vsd
38. TIME OF CLOSUREMost destined to close by 1 year60% by 3 year90% by 8 yearsMax age – 46 years
39. Common in smaller VSDAsymptomatic VSD (45% vs 7% in CHF)Partial closure occurs equally in small and largeBut total differs (71% in small and 54% in large )Even large VSD can spontaneously close
40. Spontaneous closure is rare in…Inlet VSDOutlet VSDMalaligned VSD
41. complicationsCHF ( seen beyond 1 month upto 6 months of age )AR (outlet VSD)SOVAInfective endocarditis(usually > 1-2 years of age , but can occur at any age )Eisenmenger syndrome (30-50% of large VSDs)Acquired PS – gasula defectJet of VSD . If hits pulmonary artery , causes pulmonary artery hypertrophy and leads to development of acquired PS – Arrythemias – VT , SVT , AF , sudden death can also occur.
42. Infective endocardiritisMembranous VSD - vegetation on the tricuspid valveInfundibular defects - aortic or pulmonary valve vegetation.Musclar VSD – RV side of the defect. ACC/AHA -- no antibiotic prophylaxis for the acyanotic uncomplicated VSD with no prior history of endocarditis.Patients with a proven episode of endocariditis- increased risk of recurrent infection – surgical closure may be recommended.Patients with a proven episodpate of endocarditis increased risk for recurrent infection so surgical closure may be recommended
43. Ie prophylaxisThe 2007 guidelines of the AHA recommend no antibiotic prophylaxis in children with an isolated VSD except :During the first six months after the repair with prosthetic material or deviceRepaired VSD with a residual defect at the site or adjacent to the site of a prosthetic deviceProphylactic antibiotics is recommended for dental and respiratory tract procedures
44. Improvement of symptoms in VSD.Closing defect - soft S2, high frequency & shorter murmurIncreasing PVR : increased RV pulsations ,S2 loud & narrow splitInfundibular hypertrophy & resulting decreased L to R shunt : S2 decreases in intensity ,crescendo-decrescendo systolic murmur in the ULSBCyanosis (shunt reversal ).
45. investigationLV volume overload and and RV pressure overloadSmall VSD – normal ECGLarge VSD-BVH+LAERAD
46. Katz-Wachtel phenomenon:Tall diphasic QRS complexes (>50 mm in height) in the mid-precordial leads (leads V2, V3 or V4) typically associated with Biventricular Hypertrophy (BVH).
47. Katz- watchel phempmenon
48. Chest x rayLA, LV VOLUME OVERLOADINCREASE IN PULMONARY BLOOD FLOW
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50. echocardiographyTTE+TEESize of defectLocation of defectShunt directionRims of defectQP/QSChambers enlargedFunction of chamberPA pressureAssociated abnormalities
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53. CATHERISATION STUDY To find out the operabilityTo measure pulmonary artery and aortic saturation.To measure pulmonary artery and aortic pressure
54. TREATMENT INDICATIONS FOR CLOSURE
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56. Device closureUncomplicated muscular VSDSelected perimembraneous VSDDevelopment of complete heart block is the most significant procedural complication – 6 %
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58. Surgical closureDirect suturePatch closure
59. When to closeLarge VSD become inoperable at 2 yrsEisenmenrger establishes by 2 yrs of age
60. Small vsdAnnual follow up till 10 yrs of ageThen every 2-3 yrsComplications – AR, IE , Acquired PS
61. MODERATE VSDAsymptomatic (normal pulmonary artery pressure with left heart dilation – closure of VSD by 2-5 yrs of ageSymptomatic – if controlled with medication , closure by 1-2 yrs of age
62. Large vsdPoor growth / congestive heart failure not controlled with medication – as soon as possibleControlled heart failure – by 6 months of age
63. Medical management small VSDs are asymptomatic and have excellent long-term prognosis.Neither medical therapy nor surgery is indicated.Children with moderate or large VSDs develop symptomatic congestive heart failure (the clinical manifestation of pulmonary overcirculation and left-sided volume overload)In this patients , a trial of medical therapy is indicated
64. Furosemide-1to 3mg/kg/day divided into two or three doses.Chronic furosemide can result in hypercalcemia, renal damage and electrolyte disturbances.Addition of spironolactone can be helpful to minimize potassium loss.
65. Additional initial therapy includes increasing the caloric density of the feedings.Systemic afterload reduction with enalapril (initial dosage of 0.1mg/kg/24 hours divided into twice daily, gradually increasing to 0.5mg/kg/24 hours divided into twice daily dose.
66. Digoxin may be indicated if diuresis and afterload reduction do not provide adequate symptom relief and surgery is not advisable.The usual dose of digoxin is 10µg/kg/day that can be given once daily or in divided doses twice a day
67. Eisenmenger syndromeEisenmsenger syndrome is not a congenital heart diseaseIt is an entirely preventable disorder , which arise as a complication of CHD
68. GERBODES DEFECTHigh ventricular septal defect associated with a defect of the septal leaflet of the tricuspid valve , which allows left ventricular blood to enter the right atrium .Membranous VSD
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70. Vsd and pregnancyMother - cardiac complications were rare in isolated VSD No events of arrhythmias, heart failureCardiovascular events (MI, stroke, CV mortality) in 1%FetusPreterm delivery – 11.7%Fetal mortality - 1.3%Perinatal mortality - 0Recurrent congenital heart disease of any type – 2.7%Irrespective of the underlying lesion , eisenmenger syndrome poses a high risk to the mother and the fetus .
71. ACUTE VSDTraumaticAs a complication of MI – ventricular septal rupture.
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