This presentation talks about the ventricular septal defect definition, incidence rate, Genetics, morphology, physiology, classification, investigations and management

1. Ventricular Septal Defect
VSD
Firas Aljanadi
RVH
Oct 2019
F.Aljanadi

2. Background
• Most common CHD
• 40% of cardiac anomalies
• incidence of 1/500 infants,
• 3–3.5 per 1,000 live-born infants .
• In the adult population, the incidence is lower (0.3per
1,000).
• No sex preference
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3. Genetics
• Underlying etiology of VSD is unclear. Chromosomal , Familial ,
Geographic, Environmental
• Trisomy 21 (Down syndrome), 22q11 deletion (Di George syndrome),
and 45X deletion (Turner syndrome).
• Familial forms linked to TBX5, GATA4, and NKX2.5 mutations .
• Children from an adult: a risk of VSD as high as 3 % if father
• a 6 % risk if the mother is affected
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4. Historical Background
• Vesale (sixteenth century), Morgagni,Senac (eighteenth century).
• Roger (nineteenth century) 1897 the correlation with cardiac murmur
• In France, the term “maladie de Roger” (Roger’s disease) : a very small
VSD without any clinical consequence but with a loud murmur
• PA banding : Muller and Damman in 1952 .
• 1954, Lillehei, first to surgically close a VSD with the aid of cross
circulation,
• Heart-lung machine : series of 20 patients treated by surgical closure
at the Mayo clinic was reported in 1957 .
• DHCA, initially described in 1969 by Okamoto et al. Sir B. Barratt-
Boyes repair of VSD in small infants.
• percutaneous closure of a VSD by a device introduced by
catheterization and secondly the intraoperative perventricular closure
with a device, without the use of Cardiopulmonary bypassF.Aljanadi

5. Embryology
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6. Embryology
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7. ClassificationAnatomic Types of VSD
Cono-Ventricular Defects (70-80%)
 often beyond the membranous septum
:perimembranous or paramembranous.
• hypoplasia or displacement of the conal septum,
the VSD may be called a “malalignment defect.”
• Can extend to the infundibular
(outlet) or AV canal (inlet) septum.
• often covered partially with accessory fibrotic
tricuspid tissue.
AV Canal or Inlet VSD(5-8%)
The cephalad border of these defects
is the tricuspid valve annulus; Both the cono-ventricular and
the inlet VSDs
Muscular VSD(5-20%)
• entire circumference is muscular tissue.
• mid-muscular, posterior, or apical portion
of the interventricular septum. Singular or multiple.
• +/- other VSD types.
multiple VSDs from the right ventricular, often only a singular
large defect as viewed from the left ventricular
aspect. Multiple defects are sometimes described
as “Swiss cheese”-like septum.
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8. Classification
Conal Septal or Supracristal VSD (5-7%)
• In the infundibular portion of the septum and
• usually produce near continuity between pulmonary
and aortic valves annuli :Doubly committed or juxta-
arterial VSDs.
Important Anatomic Relations of
VSD
 Perimembranous VSDs :aortic valve annulus,
partially obscured by septal leaflet tissue of the
tricuspid valve.
 The atrioventricular node and bundle of His
-Membranous and inlet-type VSDs: posteroinferior rim
of the VSD.
-Conduction tissue usually far from muscular or
supracristal-type VSDs,
-Some large mid-muscular defects---superior rim
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12. Physiology and
Pathophysiology
• No PA restriction , no increased PVR
significant left-to right shunt 
increased pulmonary blood flow
a volume load on the left ventricle.
• magnitude of the shunt is determined by: Size ,PVR,SVR,LVOT,RVOT
• Small restrictive defects :a QP/QS ratio lower than 1.5/1 not have hemodynamic
consequences, 85–95 % will spontaneously close in the first year after birth .
• Large no restriction symptoms are usually present in the absence of increased
pulmonary vascular resistanceunlikely to close spontaneously
• without treatment pulmonary vascular occlusive disease with eventual reversal right-
to-left shunt and Eisenmenger syndrome F.Aljanadi

13. Diagnosis ,Clinical
• Pansystolic murmur-smaller-Louder, thrill
• Impulse may be displaced laterally, and an apical mid-diastolic
• murmur across the mitral valve and/or a third heart sound can be heard.
• neonatal period, usually asymptomatic. As the PVR falls, left-to-right shunt
increases and symptoms may be exhibited.
• Tachypnea, tachycardia diaphoresis and dyspnea ,while feeding, poor weight
gainFTT
• Several months or years of significantly increased pulmonary blood flow from a
nonrestrictive VSD, reversal of flow across the VSD may occur resulting in no
heart failure symptoms but relative desaturation with right-to-left shunt or
Eisenmenger syndrome.
• Occasionally, the usual fall in neonatal pulmonary vascular resistances does not
occur. These children may not exhibit signs of heart failure, despite a large VSD.
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14. Diagnosis
• ECG :normal ,tachy, (inlet) LAD , chambers enlargement
• CXR :normal in small, increased pulmonary vascularity ,LA,LV
dilated ,cardiomegaly, perihilar oedema , PA prominent
• ECHO :Mainstay of diagnosis,TOE, 3D
• Cath:is only indicated in complicated cases: to assess the
pulmonary vascular resistance in individuals with suspected
pulmonary vascular occlusive disease and in suspected multiple
VSD or to close the malformation by transcatheter approach
• cMRI :limited , sedation,in poor echo ,complex anatomy,VSD
impact,vol overload,Qp/Qs
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15. En face view of the left ventricular septal surface
showing midmuscular VSD (arrow)
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16. Small perimembranous VSD
with ventricular septal aneurysm on
transesophageal echocardiographic
examination.
Small apical muscular VSD on 3-
dimensional echocardiographic
image
A 3-dimensional
echocardiographic image
shows flow
from small muscular VSD
(arrow) tunneling through the
septum
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17. . Oblique short-axis view of perimembranous VSD
shows resulting color Doppler jet flows of VSD and
tricuspid regurgitation.
Subaortic high-membranous VSD with flow into the
right ventricular outflow tract (RVOT) on intraoperative
transesophageal echocardiogram
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18. Ultrasound color Doppler 4-chamber view
of midmuscular VSD
MRI image of
midmuscular VSDF.Aljanadi

19. Natural History
• Cardiac failure: size
• Spontaneous diminution in size and closure:69%
perimembranous+ 60% muscular , less likely after 1 yr .
• LVOTO ,RVOTO(3-7%)
• AR (10%),rare <2yr, RCC 60-70%, NCC 10-15%
(perimembranous),
• PVOD(5-22%), earlier in Down’s
• IE 18.7 per 10,000 p years in non operated , 7.3 operated ,
smaller higher , in TV vegetations ,AV or PV vegetations rule out
VSD
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20. Medical Management
• initiation of diuretic therapy. ACEi
• CHF Mx
• nutritional supplementation,feeding
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21. Surgical Management
• Pulmonary Artery Banding (PAB),
• Rarely indicated nowadays ,: Swiss-cheese variety, some VSDs
and coarctation ,VSDs with straddling of one AV valve,infants
with very low body weight and those who are not fit for CPB of
an intercurrent illness a contraindication to CPB and significant
CHF that necessitates some intervention
Median sternotomy, (PTFE) strip,Silastic material, or Teflon strip
umbilical tape
PAP (30-50% of systemic BP), sBP(rise 10-15mmHg),SaO2 (85-
90%,Fio2 50%),Trusler’s rule :wt+20 (noncyanotic non mixing )
• Surgical VSD Closure :is recommended for failure of medical
management,presence of supracristal VSD, moderate to large
VSDs with persistent increased PVR.
• Interventional and Hybrid Management
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22. Techniques
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24. Complications and post op care
• TR ,RVF
• AI
• Arrhythmias
• AV BLOCK
• RBBB
• LCOS
• PAH crisis
• Residual VSD (0.7-2%)
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25. Thank you
F.Aljanadi