VSD : Dr. Akif Baig

Ventricular septal defects occur either as an isolated defect or as a component of a more complex lesion

It occurs in 50 percent of all children with CHD and in 20 to 30 percent as an isolated lesion

Most common congenital cardiac anomaly in children

Second most common congenital abnormality in adults, second only to bicuspid aortic valves

They are more common in premature infants and those born with low weight

VSDs are slightly more common in females (56%)

1. Ventricular septal defects

2. Epidemiology Ventricular septal defects occur either as an isolated defect or as a component of a more complex lesion It occurs in 50 percent of all children with CHD and in 20 to 30 percent as an isolated lesion

3. Most common congenital cardiac anomaly in children Second most common congenital abnormality in adults , second only to bicuspid aortic valves

4. They are more common in premature infants and those born with low weight VSDs are slightly more common in females (56%)

5. Anatomy The IVS is a complex curvilinear, non-planar intracardiac partition The normal ventricular septum is mostly muscular with a small fibrous portion, the membranous septum

6. The four regions are the inlet septum, trabecular septum, outlet or infundibular septum (together making up the muscular septum ), and The Membranous Septum

7. The inlet septum is smooth walled and extends from the septal attachment of the tricuspid valve (TV) to the distal attachments of the tricuspid tensor apparatus The inlet septum separates the septal cusps of the mitral and tricuspid valves

8. The trabecular portion separates the body and apices of the two ventricles It extends from the attachments of the tricuspid leaflets outward to the apex and upwards to the crista supraventricularis

9. The smooth-walled outlet or infundibular septum, extends from the crista to the pulmonary valve The outlet septum separates the outlets of the ventricles.

10. The membranous septum is further divided by the septal leaflet of the TV into atrioventricular and interventricular components

11. ANATOMICAL CLASSIFICATION

12. Many classifications of VSDs have been proposed Soto et al classified VSDs depending on their location in the IVS as seen from the right ventricular side They are divided into four types of defects : Perimembranous Muscular Outlet Inlet

13. Perimembranous defects Infracristal , subaortic, membranous They are the most common and account for 80 percent of all VSDs These defects involve the membranous septum with extension into the adjacent inlet, outlet or muscular septum They lie in the outflow tract of the left ventricle (LV), immediately beneath the aortic valve

14. There is fibrous continuity between the aortic and tricuspid valves The conduction bundle is always found in the posteroinferior margin of the defect In perimembranous VSD, rarely LV to right atrium (RA) shunt ( Gerbode defect ) may be seen

15. Classification of Gerbode defect Gerbode defects can be classified according to location  : Supravalvular (direct): defect is superior to the septal leaflet of the tricuspid valve Infravalvular (indirect): defect is inferior to the septal leaflet of the tricuspid valve Intermediate : both inferior and superior defects are present in relation to the septal leaflet of the tricuspid valve

16. Muscular defects ( trabecular ) They account for 5 to 20 percent of all the VSDs They are entirely bounded by the muscular septum and are often multiple, when viewed from the right side Kirklin et al further subclassified them depending on their location in the muscular septum as: Anterior Midmuscular , Apical Posterior .

17. When multiple muscular defects are seen, it is often referred to as ‘Swiss cheese ’ type of VSDs Swiss cheese defects cannot close spontaneously

18. Subarterial or Outlet defects Supracristal , conal , infundibular , subpulmonary , doubly committed subarterial , doubly committed juxta -arterial 5 to 7 percent of VSDs Situated just beneath the pulmonary valve and communicate with the right ventricular outflow tract above the supraventricular crest

19. The incidence is as high as 30 percent in Asian populations This defect frequently leads to prolapse of the right coronary cusp or less likely the noncoronary cusp of the aortic valve causing aortic regurgitation (AR)

20. Inlet defects Canal type, endocardial cushion type , atrioventricular septum type, juxtatricuspid ) These VSDs account for about 8 percent of all the VSDs They are located posteriorly and inferior to the membranous septum

21. VSD Classification based on Hemodynamics

22. Small or restrictive Moderate or Moderately restrictive defects Large or Non restrictive VSD Size Less than one-third of the size of the aortic root 1/3–2/3 of the size of the aortic root >2/3 Pulmonary/Aortic systolic pressure ratio <0.3 < 0.66 >0.66 Qp /Qs < 1.4:1 > 1.4–2.2:1 >2.2:1 LV volume overload Minimal Present Present Tendency to develop increased PVR Nil Minimal Markedly Chances of Infective Endocarditis Yes Yes Yes

23. Small or restrictive Defects The small VSD is less than one-third of the size of the aortic root or the orifical area is <0.5 cm²/m² It is also called restrictive as the size of the defect limits the left to right shunt and there is a significant pressure gradient between the LV and RV The pulmonary/aortic systolic pressure ratio is <0.3 with a small shunt ( Qp /Qs < 1.4:1) The degree of LV volume overload is minimal There is no tendency to develop increased PVR But they can develop AR or bacterial endocarditis

24. Moderate or Moderately Restrictive Defects The VSD size is said to be moderately restrictive, when it is 1/3–2/3 of the size of the aortic root or the orifical area is > 0.5 to 1 cm2/m2 They are large enough to permit a moderate shunt, yet small enough to offer some resistance to flow The pulmonary/aortic systolic pressure ratio is < 0.66 with a moderate shunt ( Qp /Qs > 1.4–2.2:1) The peak systolic pressure difference is ≥ 20 mm Hg between the two ventricles

25. They develop moderate left to right shunt leading to volume overload of the left sided cardiac chambers causing LV and left atrium (LA) dilatation and hypertrophy The RV is not dilated and RV and pulmonary artery pressures may remain low or be moderately elevated The PVR is low , but variable and rarely progresses to PH

26. Large or Nonrestrictive VSD The VSD is large, when it measures more than 2/3 of the size of the aortic root or the orifical area is ≥1 cm2/m2 It is also called nonrestrictive VSD as there is no resistance to flow across the defect The pressures in the ventricles are equal and they function as a common pumping chamber with two outlets The degree of left to right shunt is dependent on the relationship between the pulmonary and systemic vascular resistance The pulmonary/aortic systolic pressure ratio is >0.66 with a large shunt ( Qp /Qs > 2.2:1)

27. In infants with moderate or large VSDs the decline in the PVR may be delayed for several months At about 4 to 6 weeks of life , the large left to right shunt causes increased PBF and subsequently increases pulmonary venous return into the LA and ultimately into the LV This leads to dilatation of LA and LV and increased LV end-diastolic pressure

28. The pulmonary overcirculation leads to increase in the pulmonary interstitial fluid and in severe forms may manifest as pulmonary edema The RV pressure increases and this causes RV dilatation and hypertrophy The increased pulmonary blood flow raises the pulmonary capillary pressure and there is elevated, but subsystemic PVR, which is variable Therefore, in a large VSD both pulmonary arterial and venous pressures are elevated

29. During the second year of life , the manifestations of congestive heart failure (CHF) decreases as the pulmonary artery pressure increases As PVR increases and exceeds SVR , right to left shunt occurs Initially it is mainly during exercise , due to the fall in SVR Later, the right to left shunt occurs at rest with persistent cyanosis There is marked fall in PBF with persistent hypoxemia RV failure finally supervenes

30. Eisenmenger VSD A large VSD, if left untreated, can result in irreversible damage to the pulmonary arterial tree with development of pulmonary vascular obstructive disease (PVOD) and Eisenmenger’s syndrome The systolic pressure ratio is 1 and Qp /Qs is less than 1 : 1, with a net right to left shunt There is identical RV and LV systolic pressures and suprasystemic PVR

31. Small or restrictive Moderate or Moderately restrictive defects Large or Non restrictive VSD Eisenmenger Size Less than one-third of the size of the aortic root 1/3–2/3 of the size of the aortic root >2/3 Pulmonary/Aortic systolic pressure ratio <0.3 < 0.66 >0.66 1.0 Qp /Qs < 1.4:1 > 1.4–2.2:1 >2.2:1 1:1 LV volume overload Minimal Present Present Tendency to develop increased PVR Nil Minimal Markedly Chances of Infective Endocarditis Yes Yes Yes

32. Natural History

33. Spontaneous Closure The incidence in perimembranous and muscular VSDs is high Low in outlet defects and inlet defects do not close Swiss cheese muscular defects do not close spontaneously Studies have documented that the spontaneous closure within the first year is significantly higher for muscular than for perimembranous defects

34. In patients with restrictive VSDs followed up from birth, there is a higher incidence of spontaneous closure (50-75 percent) The incidence of spontaneous closure in moderate and large VSDs is only 5 to 10 percent

35. Most defects which close do so in the first year of life and approximately 60 percent close before 3 years and 90 percent by 8 years of age

36. Therefore, blanket advice by the pediatrician that VSD will close should be avoided, unless the size and site of VSD is assessed properly

37. Small Peri -membranous VSD can close by various methods: The adherence of the septal leaflet of TV to the IVS causing an aneurysm-like pouch This can partially or completely close the defect, but this is at the cost of causing tricuspid regurgitation (TR)

38. The ingrowth of fibrous tissue with endocardial proliferation causing septal aneurysm

39. Prolapse of the aortic cusp especially the noncoronary or the right coronary cusp, through the defect can close the VSD at the cost of causing AR

40. Growth and hypertrophy of the muscular portion of the septum around the defect

41. The vegetation caused by bacterial endocarditis on the RV side of the VSD, but this is at the cost of infection

42. Right Ventricular Outflow Obstruction Gasul’s Effect 3 to 7 percent of cases Large VSD can over a variable period develop hypertrophy of the crista supraventricularis leading to significant infundibular obstruction This is seen particularly with perimembranous trabecular defects The left to right shunt may decrease with increasing stenosis and in severe stenosis may become right to left Cyanosis is initially seen with exercise and is intermittent and later becomes persistent

43. Aortic Regurgitation The incidence of aortic cuspal prolapse in outlet VSDs has been shown to be as high as 73% They can progress to AR in 52–78% of the patients In perimembranous VSDs , aortic cuspal prolapse has been shown to be 14% with progression to AR in 6%

44. In early systole , blood is ejected from the LV and is also shunted through the VSD The anatomically unsupported coronary cusp and aortic sinus are driven into the RV due to the Venturi effect

45. In diastole the intra-aortic pressure forces the aortic valve leaflet to close, but the unsupported cusp ( right or noncoronary ) is pushed down into the left ventricular outflow tract away from the opposed coronary cusp, resulting in AR

46. Infective Endocarditis Occurs in < 1 to 3 percent of patients with VSD A small perimembranous VSD that does not close spontaneously is generally associated with a good prognosis , but is at risk for development of IE The vegetation is usually located on the septal tricuspid leaflet at the site of impact of the jet

47. In muscular VSDs the incidence of IE is low, as the jet is dispersed in the RV cavity The site of the vegetation can occasionally be on the aneurysm of the ventricular septum Rarely, an acquired left ventricular to right atrial shunt, Gerbode defect , can occur due to the perforation of the septal tricuspid leaflet secondary to endocarditis

48. Pulmonary Vascular Obstructive Disease Pulmonary vascular obstructive disease may develop in 10 percent of the large VSDs In patients with pulmonary artery and RV systolic pressure < 50 percent of the systemic arterial systolic pressure there is moderate left to right shunt with possible CHF The PVR does not increase after the initial postnatal fall, but there is a small risk of increase, usually beyond 20 years of age

49. In patients with pulmonary artery systolic pressure >50 percent of the systemic arterial systolic pressure , there is significant risk for the development of pulmonary vascular changes Measured PVR falls to high normal levels in infancy and gradually rises in the ensuing years if the defect does not become smaller The risk of development of permanent pulmonary vascular disease is very rare before the first year of life

50. Hence, prompt diagnosis and closure of these defects at least prior to 18 months of age is likely to reduce the incidence of development of pulmonary vascular disease If untreated these large or non-restrictive VSDs will have a progressive rise in pulmonary artery pressure and a fall in left to right shunting In turn, eventually this leads to higher PVR and to Eisenmenger syndrome

51. Clinical features

52. The clinical manifestations of isolated VSDs have a wide spectrum Depends upon the size of the defect and the magnitude of the shunt It may range from being asymptomatic to severe heart failure The signs and symptoms begin to develop, when the fetal PH starts declining sufficiently to permit left to right shunting

53. Large VSD The infants with large VSDs present with symptoms due to CHF by 4 to 6 weeks , as the PVR decreases The symptoms are Increased respiratory rate ( tachypnea ) C hest retractions Feeding difficulties with suck-rest-suck cycle Excessive sweating of forehead Repeated respiratory infections and failure to thrive

54. Moderate VSD Parents may observe pulsations over the precordium or feel a thrill Child may have mild tachypnea , cough during feeding and fatigue Sweatin g especially during feeding is frequent in infants below 6 months They may also present with lack of adequate growth and with one or more episodes of pneumonia Older children may present with effort intolerance and fatigue

55. Small VSDs The children with small VSDs are asymptomatic Murmur on a routine health checkup. Older asymptomatic children may be detected during routine school health check-up.

56. Physical Examination The infants with large shunts with CHF are malnourished with poor growth and development These infants are tachypneic with chest retractions and there is precordial bulge with bilateral Harrison sulcus

57. If CHF is severe or if there is added pneumonia , there may be retractions and grunting Infants with nonrestrictive VSDs with balanced shunts may become cyanotic on crying or exercise Cyanosis and clubbing are seen in adolescent and adults with large VSD with high PVR/ Eisenmenger syndrome Peripheral edema is unusual in infants

58. Arterial Pulse Pulse is normal in small VSDs In moderate VSDs, the pulse is brisk due to the vigorous LV ejection In nonrestrictive defects with large left to right shunts and CHF there may be low volume pulse The pulse is normal in Eisenmenger syndrome , as the systemic stroke volume is maintained

59. Precordial Movement and Palpation In moderate to large VSDs, precordial pulsations are visible due to LV volume overload The apical impulse is LV type , hyperdynamic , displaced downward and outwards In small and moderate VSDs a precordial thrill is best felt in the third and fourth intercostal space (ICS) at the left sternal border (LSB)

60. In cases with subarterial VSD Thrill may be palpated in the second or first ICS and may radiate upwards to the left into the suprasternal notch and into left side of neck

61. Large VSDs with high PVR Left parasternal lift may be present In patients with severe PH, there is a left parasternal heave Palpable P2 in the left second ICS

62. Auscultation The first heart sound is normal The second heart sound (S2 ) is normal with normal split Sometimes A2 may be obscured by the long murmur in small VSDs Pulmonary component is normal or mildly increased in moderate VSDs

63. The murmur in small VSDs is grade 4/6, harsh long systolic, crescendo-decrescendo best heard along the lower LSB The murmur in moderately large defects are long, low pitched decrescendo murmur best heard in LSB The systolic murmurs in outlet defects are heard in the second ICS and may radiate upwards to the left into the suprasternal notch and into the left side of neck

64. When the shunt is large ( Qp /Qs > 2:1 ), a short mid-diastolic murmur is heard at the apex due to the increased flow across the mitral valve The soft blowing early diastolic decrescendo murmur in the left second and third ICS could be due to associated AR and peripheral signs are present if the AR is significant

65. ECG in VSD

66. ECG findings in ventricular septal defect (VSD) depend on the size of defect, magnitude of the left to right shunt and severity of pulmonary hypertension ECG is normal in small ventricular septal defects with small left to right shunts

67. Left atrial enlargement may be noted in moderately restrictive VSDs and in those with large left to right shunts Left axis deviation is common with inlet VSDs and AV septal defects. Left axis deviation may also be seen in about 5% of moderately restrictive VSDs Ventricular septal aneurysms and multiple VSDs (Swiss cheese ventricular septum) can be associated with left axis deviation

68. The  Katz- Wachtel phenomenon / sign  is tall diphasic RS complexes at least 50 mm in height in lead V2, V3 or V4 – mid precordial leads The sign has been described in  ventricular septal defect with biventricular hypertrophy  in children It can be seen with isolated ventricular septal defect as well as complex ventricular septal defect

69. In Eisenmenger complex , the ‘P’ waves are peaked with right sided axis There is a tall monophasic ‘R’ preceded by small ‘q’ or followed by small ‘s ’ wave in V1

70. In Gerbode defects there is both biatrial and biventricular enlargement The tall peaked right atrial P wave in Lead II may be present from infancy There is rSr in V1, and prominent left precordial q waves, tall R waves, upright T waves indicating biventricular volume overload The hallmark in the ECG is the combination of right atrial P waves with left ventricular hypertrophy

71. Chest Xray

72. Chest X-ray is practically normal in small VSDs Moderate VSDs show cardiac enlargement of varying severity and increased pulmonary vascular markings (PVM) or plethora Cardiomegaly with pulmonary plethora in moderate sized ventricular septal defect (VSD )

73. The downward and leftward displacement of the cardiac silhouette is due to LV enlargement

74. In large VSDs , there is generalized cardiac enlargement with increased PVM There is prominence of the MPA with RV enlargement LV apex is displaced posteriorly due to RVH

75. In large VSDs with PH, the heart size is normal There is RV enlargement with the cardiac apex rotated slightly upward and to left and posteriorly There is marked prominence of the MPA and its adjacent vessels with decreased pulmonary vascularity in the outer third of the lung fields or peripheral pruning C. shows peripheral pruning with no vascularity seen in lateral 1/3 of the lung fields (multiple arrows) in a case of large VSD with severe PH, with dilated right atrium and no cardiomegaly

76. The radiological finding in Gerbode defect is the disproprionate RA enlargement This huge RA enlargement on the right with RV infundibulum and LV enlargement on the left side, gives a ball shaped appearance to the cardiac silhouette

77. ECHO in VSD - 2020 Journal of the Indian Academy of Echocardiography & Cardiovascular Imaging | Published by Wolters Kluwer

78. A systematic echocardiographic assessment of VSD includes a detailed: Anatomic and Hemodynamic description

79. Anatomic description Exact location of the defect Number of defects Relationship to valve and valve attachments, Description of anatomic size of the defect, and Associated lesions (if any)

80. Detailed assessment of ventricular septum requires sweeping the entire ventricular septum in both 2D and color Doppler imaging from apex to base and from left to right Because of the curved nature of the ventricular septum, optimal imaging of a VSD needs to be done from subcostal , parasternal , apical, and right parasternal windows The best acoustic window is determined by the fact that shows the septum perpendicular to the ultrasound beam and the flow across the defect parallel to the beam

81. Perimembranous Ventricular Septal Defect Perimembranous defects are the most common type of VSDs and involve the membranous ventricular septum adjacent to aortic and tricuspid valves

82. Located adjacent to tricuspid valve, perimembranous VSD can be associated with tricuspid septal leaflet distortion with tricuspid regurgitation Accessory tissue or part of septal leaflet of tricuspid valve can partially or completely close the defect, referred to as “ventricular septal aneurysm”

83. Occasionally blood from VSD can traverse through aneurysmal tissue across tricuspid valve into the right atrium leading to LV to right atrial shunt Misinterpretation of this high‑ velocity flow as tricuspid regurgitation can lead to false overestimation of RV pressure

84. About 10% of perimembranous defects are associated with AV prolapse due to close proximity with AV It can be identified by right or noncoronary cusp protruding into the VSD best seen in parasternal long‑.and short‑ axis views Due to importance in the development of aortic regurgitation , the presence of aortic cusp prolapse should be carefully reported

85. INLET VSDs Inlet VSDs are located posteriorly adjacent to both atrioventricular valves These defects are commonly associated with atrioventricular septal defects (AVSDs) but can be isolated These are best imaged from apical four‑ chamber or parasternal short‑ axis views

86. AV valve involvement is common with inlet VSDs Tricuspid valve may be intrinsically abnormal with associated tricuspid regurgitation VSD associated with partial AVSD may have cleft in the anterior leaflet of mitral valve resulting in mitral regurgitation

87. Inlet VSD may rarely be associated with malalignment of atrial and ventricular septa , resulting in some degree of AV valve override, and rarely with straddling of AV valve chordal attachments

88. Subarterial Ventricular Septal Defect Subarterial or infundibular VSDs result from deficiency in conal or outlet septum beneath both semilunar valves These defects are best assessed from parasternal long‑. and short‑ axis views

89. About 60% of subarterial defects are associated with prolapse and distortion of the right coronary cusp of AV with half of these patients developing aortic regurgitation Prolapse can be demonstrated as diastolic bulging of the right coronary cusp into RV It can be mild to severe AV prolapse can lead to a reduction in size of VSD with associated risk of aortic regurgitation

90. Muscular Ventricular Septal Defect Muscular VSDs are defects appearing in trabecular or muscular septum entirely surrounded by muscular rim They can be further subdivided into anterior, mid‑ muscular , posterior, or apical defects according to their location The presence of multiple muscular defects especially in the mid‑ muscular or apical segment is referred to as “ swiss‑cheese ” septum

91. Assessment of the Size of the Ventricular Septal Defect Determination of the size of VSD is made on hemodynamic basis like Degree of left‑ to ‑ right shunt Presence of volume overload , and Pulmonary artery pressure

92. Historically, the size of VSD has been related to the size of aortic root VSD measuring <1/3 of aortic root diameter is classified as small 1/3–2/3 of aortic root diameter as moderate , and Lesion close to aortic root size is considered large

93. Hemodynamic classification uses pressure difference across LV to RV as a guide to size the defects When there is equalization of pressures in two ventricles in the presence of isolated VSD in the absence of pulmonary stenosis , it is called as a large or nonrestrictive defect

94. A restrictive defect has a pressure gradient across LV and RV as determined by Doppler technique , with pressure gradient of more than 60 mmHg as restrictive defect and pressure difference of 25–60 mmHg as moderately restrictive

95. Hemodynamic description Chamber size Estimation of right ventricular pressure and pulmonary artery pressure, and Estimation of overall shunt size

96. Hemodynamic assessment of VSD using echocardiography usually includes evaluation of right heart pressure and quantification of amount of shunt flow Velocity of blood flow across VSD as measured by Doppler is used to measure right ventricular systolic pressure using modified Bernoulli equation Right ventricular pressure = Systolic blood pressure − VSD jet peak gradient

97. Proper alignment of Doppler beam with VSD jet is necessary for accurate determination of right ventricular pressure that is equal to systolic PA pressure in the absence of right ventricular outflow tract obstruction In addition, tricuspid regurgitation peak velocity can be obtained to estimate RV pressure as follows : RV pressure = 4 × (TR jet velocity ) 2 + RA pressure. While determining TR jet velocity, proper alignment of Doppler beam to TR jet and complete TR signal must be obtained Any LV to RA shunt must be ruled out in the presence of perimembranous VSD to avoid false interpretation

98. VSD shunt volume is determined by size of VSD and PVR Left heart chamber dilatation is associated with pulmonary‑ to ‑ systemic flow ratio ( Qp /Qs) of > 1.5:1 Doppler‑ based methods for shunt quantification are not widely accepted due to variable results

99. Transesophageal and 3D Echocardiography Transesophageal echocardiography (TEE) is occasionally used In the pediatric age group, it is used most often intraoperatively to assess the completeness of the repair Three-dimensional echocardiography has proved accurate for quantifying shunt and can provide accurate visualization of defects that otherwise are difficult to evaluate by TTE

100. Cardiac Catheterization and Cineangiography Indications are : If there is uncertainty regarding either defect number, size , location and hemodynamic burden or additional lesions . The anatomy of multiple apical VSDs is delineated on angiogram even those defects which MRI and echocardiography sometimes cannot identify Interventional device closure of one or more defects To assess PVR and to study reactivity of the elevated PVR to different pulmonary vasodilators (100% O 2 and inhaled nitric oxide), especially in older patients

101. Management

102. MEDICAL MANAGEMENT The children with small VSDs are asymptomatic and have excellent long-term prognosis The parents need to be given reassurance , advise on subacute bacterial endocarditis prophylaxis and periodic clinical follow-up Medical therapy is required for patients with moderate to large VSDs till any intervention is done to close the defect

103. The drugs are usually a combination of diuretics (i.e. furosemide ), afterload -reducing agents angiotensin -converting enzyme (ACE) inhibitors and digoxin

104. Approaches To Ventricular Septal Defect Closure Surgical closure. Transcatheter techniques. Hybrid approach.

105. Surgery for Ventricular Septal Defect The indications for surgical closure of VSD in general are: Refractory heart failure and/or failure to thrive. Large defects that are unlikely to close, with or without symptoms Development of AR or aortic cusp prolapse especially in subpulmonic or outlet VSDs Asymptomatic older children with QP/QS greater than 2:1 The inlet and outlet VSDs which do not close spontaneously

106. Devices For Percutaneous Closure Of Ventricular Septal Defect

107. The transcatheter device closure of muscular VSDs have been in vogue for the past 15 years Although relatively common , perimembranous VSDs can be difficult to close percutaneously Previous devices (e.g. Rashkind or button devices ) have been unsuccessful in attempts to close these VSDs , because of the proximity of the defects to the aortic valve and the potential for aortic valve damage

108. Now many varieties of new devices like muscular septal occluder for muscular VSDs, asymmetrical and symmetrical perimembranous septal occluder and Amplatzer duct occluder II (ADO II) are available to close perimembranous VSDs and rarely Gerbode shunts

109. Midmuscular ventricular septal occluder

110. B. Asymmetric perimembranous VSD occluder with only 0.5 mm retention disc towards the aortic valve C . Symmetric perimembranous VSD occluder

111. D and E. Amplatzer ® membranous VSD occluder 2, has a dual layer waist to minimize radial pressure against the rims of the defect, and the waist length is increased to 3 mm to decrease the clamping effect on the ventricular septum

112. Recommendations For Device Closure Of Muscular Vsds

113. Class IIa Infants who weigh ≥ 5 kg Children and adolescents with hemodynamically significant (left ventricular or left atrial volume overload or pulmonary to systemic blood flow ratio ≥ 2:1) muscular ventricular septal defect (MVSD) to undergo percutaneous VSD device closure

114. Class IIb Neonates, infants who weigh < 5 kg Children with hemodynamically significant (left ventricular or left atrial volume overload or pulmonary to systemic blood flow ratio > 2 : 1) MVSD and associated cardiac defects requiring cardiopulmonary bypass may be considered for performance of hybrid perventricular closure of the VSD off bypass, followed by surgical repair of the remaining defects or device placement during cardiopulmonary bypass

115. Class III Neonates, infants and children with hemodynamically significant (left ventricular or left atrial volume overload or pulmonary to systemic blood flow ratio >2 : 1) inlet MVSDs with inadequate space between the defect and the atrioventricular or semilunar valves should not undergo device closure (hybrid or percutaneous ) Neonates, infants and children with a small to moderate sized MVSD (without symptoms or evidence of pulmonary hypertension) in whom there is a reasonable expectation that the defect will become smaller over time should be followed up expectantly and do not need closure of the VSD

116. Exclusion criteria Weight less than 3.0 kg ( unless the hybrid perventricular approach is used) Distance of less than 4 mm between the VSD and the aortic , pulmonic , mitral or tricuspid valves PVR greater than 7 indexed Wood units ; Sepsis and patients with conditions that would be expected to be exacerbated by the use of aspirin unless other antiplatelet agents could be used for 6 months

117. Guidelines

118. - AHA Guidelines 2018

119. - ESC 2020