Congenital Acyanotic Heart

Disease
• Prevalence: 8 per 1000 live births
• Aetiopathogenesis:
• Unknown mostly
• Chromosomal anomaly (Down Syndrome,
Turner Syndrome)
• Antenatal illness of mothers (DM, Rubella
infection, radiation)
 Acyanotic Congenital Heart Disease
Left-to-Right Shunt Lesions

• Atrial Septal Defect (ASD)

• Ventricular Septal Defect (VSD)
• Atrioventricular Septal Defect (AV Canal)
• Patent Ductus Arteriosus (PDA)
 Obstructive Heart Lesions
• Pulmonary Stenosis

• Aortic Stenosis

• Coarctation of the Aorta

 Clinical Presentation
A) Symptoms:
• Asymptomatic (ASD, small VSD)
• Effort intolerance
• Shortness of breath
• Failure to thrive
• Not growing well
• Bluish colouration of lip, skin, mouth
• Recurrent respiratory tract infections
 B) General Physical Examination:
• Appearance: Sick looking, often malnourished
(VSD)
• Respiratory rate: Increased (may be RTI, HF)
• Pulse rate: Increased (may be HF)
• BP: Normal
• JVP: Raised
• Pedal oedema: May be Ieft Heart failure
• Cyanosis: ±
• Anthropometry: Stunted & wasted
 Ventricular Septal Defect

• VSD – is an abnormal opening in the

ventricular septum, which allows free
communication between the Rt & Lt
ventricles. Accounts for 25% of CHD.
 Ventricular Septal Defect
• 4 Types
• Perimembranous (or membranous) – Most common (80%).

• Infundibular (subpulmonary or supracristal VSD) – involves

the RV outflow tract.

• Muscular VSD – can be single or multiple.

• AVSD – inlet VSD, almost always involves AV valvular

abnormalities.
 Cont’d..
• Depending on size:
• Small- <5mm (usually asymptomatic and 50%
will close spontaneously by age 2yrs)

• Moderate- 5-10mm Always symptomatic &

• Large- >10mm needs treatment
 Hemodynamics
• The left to right shunt occurs secondary to
Pulmonary Venous Resistance being < Systemic
Venous Resistance, not the higher pressure in the LV.

• This leads to elevated RV & pulmonary pressures &

volume hypertrophy of the LA & LV.

• Continuous exposure of pulmonary vascular bed to

high blood flow causes Pulmonary HTN and later
pulmonary vascular obstructive diseases with rt to lt
shunt (Eisenmenger Syndrome)
 Precordium
• Inspection: Hyperdynamic may be bulged
• Palpation:
Apex beat shifted to left
Thrill (may be palpable)
Left parasternal heave (may be palpable)
P₂(may be palpable)
• Auscultation:
 1st & 2nd Heart sounds are audible in all 4 areas
 A harsh pansystolic murmur heard along the lower
left sternal border, more prominent with small VSD,
maybe absent with a very Large VSD.
 Investigations

• Chest X-Ray: Cardiomegaly

Plethoric lung fields
• E.C.G: Normal/ LVH/Biventricular hypertrophy
• Echocardiography: Color doppler is diagnostic
 Treatment T

• Small VSD: -
No surgical intervention, no physical restrictions, just reassurance and periodic
follow-up and endocarditis prophylaxis.
•Moderate to Large VSD (Medical treatment):
•High calorie diet for proper nutrition
•Afterload reducers ( Captopril, Enalapril)
• Diuretics (Frusemide)
•Control of heart failure
• Digoxin if needed.

•
 Surgical Treatment
• Indications for Surgical Closure:
• Large VSD with medically uncontrolled
symptomatology & continued FTT.
• Ages 6-12 mo with large VSD & Pulm. HTN
• Age > 24 mo with Qp:Qs ratio > 2:1.
• Supracristal VSD of any size, secondary to risk of
developing AV insufficiency.
• Contraindication:
Severe pulmonary vascular disease.
 Prognosis

• 30-50% defects close spontaneously during

the first year of life.
• Maximum closes by 2 years , rest by 4 years.
• Children with small VSD have excellent long
term prognosis.
 Atrial Septal Defect

• ASD is an opening in the atrial septum

permitting free communication of blood
between the atria. Seen in 10% of all CHD.
 Atrial Septal Defect
• There are 3 major types:

• Secundum ASD – at the Fossa Ovalis, most common.

• Primum ASD – lower in position & is a form of ASVD,

MV cleft.

• Sinus Venosus ASD – high in the atrial septum,

associated w/partial anomalous venous return & the
least common.
 Atrial Septal Defect
Clinical Signs & Symptoms
• Rarely presents with signs of CHF or other cardiovascular
symptoms.

• Most are asymptomatic but may have easy fatigability or

mild growth failure.

• Cyanosis does not occur unless pulmonary HTN is

present.
 Precordium (ASD)
• Inspection: Usually normal
• Palpation: Apex beat shifted to left
Left parasternal heave (may be palpable)
P₂(may be palpable)
• Auscultation:
 1st Heart sound are audible in all 4 areas
 2nd heart sound widely splitted and fixed.
 A ejection systolic murmur best heard along
the upper left sternal border(pulmonary area).
 Atrial Septal Defect
• Question:
What causes the systolic & diastolic murmurs of ASD?

• Answer:
Systolic murmur is caused by increased flow across the
pulmonary valve, NOT THE ASD.

Diastolic murmur is caused by increased flow across the

tricupsid valve & this suggest high flow Qp:Qs is 2:1.
 Investigations

• Chest X-Ray: Cardiomegaly

Plethoric lung fields
• E.C.G: Normal/right axis deviation/ RVH
• Echocardiography: Color doppler is diagnostic
 Treatment
T

•Medical treatment:
•High calorie diet for proper nutrition
•Afterload reducers ( Captopril, Enalapril)
• Diuretics (Frusemide)
•Digoxin if needed.
•Surgical Treatment:
Closer of the defect.
 Atrial Septal Defect
• Question:
Is endocarditis prophylaxis required for
ASD?

• Answer:
NO
 Patent Ductus Arteriosus

• Persistence of normal fetal

vessel that joins the PA to
the Aorta (Ductus
arteriosus).

• Normally closes in the 1st

wk of life.

• Accounts for 10% of all

CHD.
 Patent Ductus Arteriosus
• Question:
What TORCH infection is PDA associated
with?

• Answer:
Rubella
 Patent Ductus Arteriosus
Hemodynamics
• As a result of higher aortic pressure, blood shunts
L to R through the ductus from Aorta to PA.

• Extent of the shunt depends on size of the ductus

& PVR:SVR.

• Small PDA, pressures in PA, RV, RA are normal.

 Patent Ductus Arteriosus
Hemodynamics
• Large PDA, PA pressures are equal to systemic
pressures. In extreme cases 70% of CO is
shunted through the ductus to pulmonary
circulation.

• Leads to increased pulmonary vascular

disease.
 Clinical Signs & Symptoms

• Small PDA’s are usually asymptomatic

• Large PDA’s can result in symptoms of CHF, growth
restriction, FTT.
• Bounding arterial pulses
• Widened pulse pressure
• Enlarged heart, prominent apical impulse
• Thrill in 2nd lt ICS
• Classic continuous machinary systolic murmur best
herad 2nd lt ICS.
• Mid-diastolic murmur at the apex
 Treatment
A. medical Treatment:
• Indomethacin, inhibitor of prostaglandin
synthesis can be used in premature infants.
• Ibuprofen: Orally
• Percutaneous catheter closure.
• Surgical treatment: Usually done by ligation &
division or intra vascular coil.
• Mortality is < 1%