Ventricular septal defect (VSD) is the most common congenital heart defect, accounting for 40% of cardiac anomalies with an incidence of 1 in 500 infants. The underlying causes are unclear, but genetic factors, including various chromosomal conditions, are implicated. Management includes medical and surgical interventions, with a focus on monitoring potential complications such as heart failure and pulmonary vascular disease.

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
F.Aljanadi

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
F.Aljanadi

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.
F.Aljanadi

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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9. F.Aljanadi

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11. F.Aljanadi

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.
F.Aljanadi

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
F.Aljanadi

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
F.Aljanadi

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
F.Aljanadi

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
F.Aljanadi

20. Medical Management
• initiation of diuretic therapy. ACEi
• CHF Mx
• nutritional supplementation,feeding
F.Aljanadi

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
F.Aljanadi

22. Techniques
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23. F.Aljanadi

24. Complications and post op care
• TR ,RVF
• AI
• Arrhythmias
• AV BLOCK
• RBBB
• LCOS
• PAH crisis
• Residual VSD (0.7-2%)
F.Aljanadi

25. Thank you
F.Aljanadi