Download 2-Acyanotic CHD

Dr. Talaat Ali Sabeeh
Al-Jarrah
Pediatric Cardiologist
Acyanotic Congenital Heart Disease
(Left-to-Right Shunt Lesions)
1-Ventricular septal defects
2-Atrial septal defects
3-Atrio-ventricular septal defects ( ECD)
5-Patent ductus arteriosus
6-PS.:mild,moderate.
7-Aortic stenosis
8-Mitral stenosis/incompetence
9-Coarctation of aorta
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Common Cyanotic Lesions
Decreased flow:
Tetralogy of Fallot
Tricuspid Atresia
Sever PS
Ebstein’s anamoly
Increased Flow:
Transposition of great vessles
Truncus Arteriosus
Hypoplastic left heart
Single ventricle
TAPVR .
 Prevalence
 I-Cyanotic: 22%
 II-Acyanotic: 68%
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VSD
ASD
PDA
TOF
PS
AS
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30%
7-8%
7-8%
7-8%
7-8%
5%
 Question:How you can differentiate cyanosis due to
cardiac cause from pulmonary (lung) cause?
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A: Hyperoxia Test
 Neonates with cyanotic congenital heart disease
usually do not have significantly raised arterial PaO2
during administration of 100% oxygen.
Atrial Septal Defect
 Atrial Septal Defect
 ASD is an defect in the atrial septum permitting free
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communication of blood between the atria. Seen in 78 % of all CHD.
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 with partial anomalous venous return & the
least common.
 Clinical Signs & Symptoms
 Rarely : signs of CHF or other CV symptoms.
 Most are asymptomatic but may have easy fatigability
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or mild growth failure.
Cyanosis does not occur unless pulmonary HTN .
Hyperactive precordium,RV heave,fixed widely splitS2.
II-III/VI systolic ejection murmur @ LSB.
Mid-diastolic murmur heard over LLSB.
 4-Once pulmonary HTN w/ shunt reversal occurs this
is considered too late.
 Mortality is < 1%.
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Question. Is endocarditis prophylaxis required for
ASD?
 Answer: NO
Ventricular Septal Defect
Ventricular Septal Defect
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VSD abnormal opening in IVS,so communication
between the RV& LV. Accounts for 30 % of CHD.
Types
1-Perimembranous (or membranous) – Most common.
2-Infundibular (subpulmonary or supracristal VSD) –
involves the RV outflow tract.
3-Muscular VSD – can be single or multiple.
4-AVSD – inlet VSD, almost always involves AV valvular
abnormalities.
 Hemodynamics
 The left to right shunt ,leads to elevated RV & pulmonary
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pressures & volume hypertrophy of the LA & LV.
Clinical Signs & Symptoms
1- Small - moderate VSD, 3-6mm, are usually asymptomatic and
50% will close spontaneously by age 2yrs.
2- Moderate – large VSD, almost always have symptoms and will
require surgical repair
II-IV /VI harsh HSH murmur LSB, more prominent with small
VSD, maybe absent with a very Large VSD.
• Prominent P2, Diastolic murmur.
• CHF, FTT, Respiratory infections, exercise intolerance
hyperactive Precordium
Symptoms develop between 1 – 6 months
 Treatment
 • Small VSD - no surgical intervention, no physical
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restrictions, just reassurance and periodic follow-up
and IE prophylaxis.
• Symptomatic VSD - Medical treatment initially.
Indications for Surgical Closure:
1-Large VSD with medically uncontrolled
symptomatology & continued FTT.
2-Ages 6-12 mo with large VSD & pulmonary
hypertension
3-Age > 24 mo with Qp:Qs ratio > 2:1.
4-Supracristal VSD of any size, secondary to risk of
developing AV insufficiency.
Atrioventricular Septal Defect
Atrioventricular Septal Defect
 Incomplete fusion the the ECD, which help to form the
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lower portion of the atrial septum, the membranous
portion of the ventricular septum and the septal
leaflets of the triscupid and mitral valves. They
account for 4% OF ALL CHD.
Question. What genetic disease is AVSD more
commonly seen in?
Answer: Down’s Syndrome (Trisomy 21), Seen in 2025% of cases.
Types
Complete Form
Incomplete Form
Clinical Signs & Symptoms
 Complete type : same large VSD.
 Incomplete type: same ASD.
 Treatment
 Surgery is always required.
 Treat congestive symptoms.
 Pulmonary banding may be required in premature infants
or infants < 5 kg.
 Correction is done during infancy to avoid irreversible
pulmonary vascular disease (Eisenmenger `s syndrome).
 Mortality low with incomplete 1-2% & as high as 5% with
complete AVSD.
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Patent Ductus arteriosus
Patent Ductus arteriosus
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PDA – Persistence of the normal fetal vessel that joins the PA to the Aorta.
Normally closes in the 1st few wk of life.
Accounts for 7-8% of all CHD
Female : Male ratio of 2:1 . Often associated with coarctation & VSD.
Question. What TORCH infection is PDA associated with?
Answer: Rubella
 Hemodynamics
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Higher aortic pressure, blood shunts L to R through ductus from Aorta to PA.
Extent of the shunt depends on size of the ductus & PVR.
Small PDA, pressures in PA, RV, RA are normal.
Large PDA, PA pressures are increasing.
 Clinical Signs & Symptoms
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Small PDA’s are usually asymptomatic
Large PDA’s : catabolic state
Widened pulse pressure
Enlarged heart, prominent apical impulse
Classic continuous machinary systolic murmur
PDA Treatment
 Indomethacin, inhibitor of prostaglandin synthesis
can be used in premature infants.
 PDA requires Surgical or Transcatheter closure.
 Closure is required treatment heart failure & to
prevent pulmonary vascular disease.
 Usually done by surgical ligation or division and
suturing
Mortality is < 1%. 
Obstructive lesions
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 1-Pulmonary Stenosis
 2-Aortic Stenosis
 3-Coarctation of the Aorta
 Pulmonary Stenosis
 Pulmonary Stenosis is obstruction in the region of either the
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pulmonary valve or the subpulmonary ventricular outflow tract.
Accounts for 7-8% of all CHD.
Most cases are isolated lesions
Can present w/or w/o an intact ventricular septum.
Question. What syndrome is PS associated with?
Answer: Noonan’s Syndrome, secondary to valve dysplasia.
 Hemodynamics
 RV pressure hypertrophy  RV failure.
 RV pressures maybe > systemic pressure(LV pressure).
 Cyanosis is indicative of Critical PS.
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Clinical Signs & Symptoms
 Depends on the severity of obstruction.
 Asymptomatic w/ mild PS< 40mmHg,but in Moderate:40-
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70mmHg & Sever : > 70mmHg.
Prominent jugular a-wave, RV lift(heave)
Split 2nd hrt sound w/ a delay
Ejection click, and Ejection systolic murmur.
Heart failure & cyanosis seen in severe cases
 Treatment
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Mild PS no intervention required, follow-up.
Mod-severe : require relieve of stenosis.
Balloon valvuloplasty, treatment of choice.
Surgical valvotomy is also a consideration, but rarely
needed.
Aortic Stenosis
Aortic Stenosis
obstruction to the outflow from the left ventricle at or near
the aortic valve that causes a systolic pressure gradient of
more than 10mmHg. Accounts for 5% of CHD.
 Types
 1-Valvular – Most common.
 2-Subvalvular(subaortic) – involves the left outflow tract.
 3-Supravalvular – involves the ascending aorta is the least
common.
 Question. Which syndrome with Supravalvular AS?
 Answer: Williams Syndrome
 Hemodynamic: Pressure hypertrophy of the LV and LA .
 Mild AS
20-40mmHG
 Moderate AS
40-60mmHg
 Sever AS
> 60mmHg
Clinical Signs & Symptoms
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Mild AS : asymptomatic.
Moderate AS :chest pain, dypsnea on exertion, dizziness & syncope.
Severe AS : weak pulses, left sided heart failure, Sudden Death.
LV thrust at the Apex.
Systolic thrill on Rt.base/suprasternal notch.
Ejection click ,and Ejection systolic murmur ,radiation to the carotids.
 Treatment
 Because surgery does not offer a cure ,it is reserved for patients with
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symptoms and a resting gradient of 60-80mmHg.
For subaortic stenosis it is reserved for gradients of 40-50mmHg
because of it’s rapidly progressive nature.
Balloon valvuloplasty is the treatment of choice.
Treatment Aortic insufficiency & re-stenosis is likely after surgery and
may require valve replacement.
Activity should not be restricted in Mild AS.
Mod-severe AS, no competitive sports.
Coarcotation Of Aorta
Coarctation- is narrowing of the aorta at varying points
anywhere from the transverse arch to the iliac bifurcation.
 98% of coarctations are juxtaductal
 Male: Female ratio 3:1. Accounts for 7 % of all CHD.
 Question: What other heart anomaly is coarctation
associated with?
 Answer: Bicuspid aortic valve, seen in > 70% of cases.
 Question: What genetic syndrome is coarctation seen in?
 Answer: Turner’s Syndrome.
 Hemodynamics
 Obstruction of left ventricular outflow  pressure
hypertrophy of the LV.
Coarcotation Of Aorta
Clinical Signs & Symptoms
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Classic signs: absence,weakness or delayed femoral pulses.
Higher BP in the upper extremities than lower extremities.
90% have systolic hypertension of the upper extremities.
Pulse discrepancy between Rt. & Lt arms.
With severe coarc. hypoperfusion, acidosis, HF and shock.
II/VI systolic ejection murmur @ LSB.
Cardiomegaly, rib notching on X-ray.
Treatment
With severe coarctation maintaining the ductus with
prostaglandin E is essential.
 Native:Surgical intervention, to prevent LV dysfunction.
 Angioplasty is used by some centers.
Re-coarctation can occur, balloon angioplasty is the 
procedure of choise.