Acyanotic Congenital Heart

Disease
Left to right shunts Obstructive Lesions
• Ventricular Septal Defects • Pulmonary Valvular
(VSD) Stenosis (PS)
• Atrial Septal Defects • Aortic Stenosis (AS)
(ASD) • Coarctation of Aorta
• Patent Ductus Arteriosus (COA)
(PDA)
 Atrial Septal Defect (ASD)
• Isolated anomaly in 5% to 10% M:F::1:2
• 30-50% of children have ASD as part of
cardiac defects.
 Pathology

• Three types - secundum , primum and sinus

venosus.
• The Patent Formen Oval (PFO ) does not
ordinarily produce intracardiac shunts.
• Ostium secundum is the most common type of
ASD (50 -70 % ). The defect is present in fossa
ovalis, allowing shunting of blood from LA to RA.
ASD - Types
 Pathology – Contd..
• Ostium primum occurs in 30% of all ASDs including
those as a part of complete atrioventricular defects.
• Isolated Ostium Primum ASD occurs in 15% of all
ASD
• Sinus venosus occurs in 10% of all defects. It is
commonly located at the entry of SVC into RA and
rarely at the entry of IVC into the RA.
 ASD Types

Primum ASD Secundum ASD Sinus Venosus

ASD
ASD - Hemodynamics
 Clinical Manifestations
• Children and infants usually asymptomatic
• A widely split S2 and a grade 2-3/6 ejection systolic
murmur are characteristic
• Large shunts have a mid diastolic rumble due to
relative TS audible at LLSB.
• Typical findings may be absent in children with
large defects.
 Investigations
• ECG
– RAD of +90 to +180 degrees and mid RVH or RBBB
with an rsR’ pattern in V1
• X ray chest
– Cardiomegaly with RA and RV enlargement is visible.
– A prominent MPA segment , RPA and increased PVMs
can be seen
 Investigations - Contd..
Echocardiography
• Indirect signs of left to right shunts
• RVE and RAE and dilated PA
• Doppler estimates pressures in RV & PA
• M mode – increased RV dimension & paradoxical motion
of IVS - signals of RV volume overload
 Patent Foramen Ovale
Diagnostic Criterion
There is discontinuity
between upper margin
of septum primum and
limbus of the foramen
ovale,the septum
primum is redundent
not deficient as seen in
secundum
 Atrial Septal Defect Primum

Diagnostic Criterion
Deficiency of the lower atrial septum above the atrioventricular
valves

Problems
Dilated coronary Sinus may give false impression
 Lesions associated with ASD Primum
• Inlet VSD
• Cleft mitral valve
• Presence and severity of Atrio Ventricular valve
regurgitation.
• Partial attachment of septal leaflet of mitral valve to
IV septum.
 Secundum Type ASD

Diagnostic criterion
Deficiency of septum primum from central portion of
IAS.
ASD OS Echo Measurement
 Sinus Venousus ASD
Diagnostic Criteria
Deficiency of portion of
atrial septum between
orifice of SVC and
superior limbic band or
IVC and inferior limbic
band
 Natural History of ASD
• Spontaneous closure 87%.
• ASD <3mm by 18months 100% closure.
• ASD between 3-8mm by 18m. 80%closure.
• ASD > 8mm rarely closes spontaneously.
• If untreated CHF and PAH develops in adults
• SBE prophylaxis is not required unless associated
defects present.
MANAGEMENT: DEFINITVE
Example of Closure Device

Amplatzer Septal Occluder

 Ventricular Septal Defect
(VSD)
• Membranous
apical- just under the aortic
valve.
• Muscular
inlet - beneath AV valves
• Trabecular - central, apical,
marginal.
• Outlet (infundibular) - beneath
aortic valve e.g. TOF.
 Ventricular Septal Defect

An opening or hole in the interventricular septum

VSD - Hemodynamics
 Clinical Presentations
 With small VSD patient is asymptomatic.
 With large VSD, delayed growth and
development, repeated pulmonary infections
and CHF.
 With long standing pulmonary hypertension, a
history of cyanosis and a decreased activity.
 Investigations
ECG
• Small VSD, ECG is normal.
• With moderate VSD, LVH and occasional LAH may be
seen.
• Large defect, ECG shows combined ventricular
hypertrophy (CVH)
• If PVOD develops, ECG shows RVH only.
 Investigations – Contd..
X ray
• Cardiomegaly (LA, LV increase).
• MPA and hilar PA enlarge in PVOD
 Ventricular Septal defects
Objectives
• Confirm (VSD)
• Determine size and site of VSD
• Rule out associated lesions
• Estimate right ventricular and
pulmonary arterial pressure
• In a large shunt there may be
enlargement of LV, LA.
VSD Perimembranous - Echo
VSD Muscular Multiple - Echo
 Interventricular Gradient
• Doppler cursor in the RV side of VSD will give a positive
jet from the base line in left to right shunt.
• Right arm systolic BP – VSD gradient = RVSP =PA
pressure
• Normal PA pressure = 25-30mm Hg
Mild PAH - 30-50mm Hg
Moderate PAH - 50-75mmHg
Severe PAH - > 75mm Hg
 VSD
Diagnostic Criteria
• These defects may be seen as drop out echoes.
• From interventricular septum, to be more definitive,
they are seen in more than one view.
• Additionally, left atrial enlargement, Left ventricular
enlargement
• Doppler reveals turbulence across the VSD when
the RV pressure is normal
Complications associated with VSD
• Ventricular Septal Aneurysm
• Aortic Regurgitation ( common with outlet defects )
Implication – surgical closure is indicated in absence
of large shunt to reduce risk of progressive AV
dysfunction
• Vegetation on RV side.
 Natural History of VSD
• Spontaneous closure in 30 to 40% with
membraneous & muscular VSD in first 6m.
• CHF develops in infants with large VSD not before
4-8 weeks
• PVOD develops as early as 6-12 mo. With large
VSD, for which Surgical closure or pulmonary artery
banding has to be done
 Common Atrioventricular Canal
Diagnostic criteria
There is
• VSD atrioventricular canal
type
• ASD Ostium primum
• Common Atrio-Ventricular
Valve
 Patent Ductus Arterious (PDA)
Diagnostic criteria
• Lumen of the vessel
visualized along the
entire length.
• LAE
• LV dilatation
PDA - Hemodynamics
 PDA - Features
• Measure LA & LV reflecting the volume of left to right
shunt.
• Look for associated defects like coarctation of aorta
aorto pulmonary window.
 PDA - Diagnostic Criteria

• Pulmonary arterial end is left of pulmonary trunk and

adjacent to LPA. Aortic insertion opposite and just beyond
origin of subclavian artery.
• Doppler performed on PA proximal to ductal opening The
peak velocity will give the pressure difference between
aorta & PA
PDA - Echo

Note: The
Shunt going
from below
upwards in
the
pulmonary
artery
 Natural History of PDA
• Unlike PDA of prematures spontaneous closure of
PDA does not occur.PDA of term are due to
structural abnormality of ductal smooth muscle.
• PVOD, CHF or recurrent pneumonia.
• SBE, more frequent with small PDA.
 Coarctation of Aorta (COA)
• 8- 10 % of all CHD.
• M:F = 2:1. 30% of Turners Syndrome.
• 85% of COA have bicuspid valve.
• Poor feeding, dyspnea & poor weight gain, & acute
circulatory shock in first 6 weeks .
• 20-30% of COA develop CHF by 3 months
 Coarctation of the aorta
Diagnostic finding
Aortic lumen is narrowed,
typically distal to the left
subclavian artery.
Hypoplastic aortic arch
Post stenotic dilatation of the
aorta.
Bicuspid aortic valve.
Doppler will show the severity of
obstruction.
Coarctation of the aorta -
Hemodynamics
Coarctation of the aorta - Types
 Natural History of COA
• Bicuspid valve may cause stenosis or regurgitation
with age.
• SBE may occur on either aortic valve or on
coarctation.
• LV failure, rupture of aorta, ICH, hypertensive
encephalopathy may develop during childhood.
Thank you