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TRICUSPID ATRESIA
1. Atretic (missing) tricuspid valve
2. Hypoplastic right ventricle
3. Ventricular septal defect
4. Atrial septal defect
5. Pulmonary Stenosis
Truncus Arteriosus1. Pulmonary arteries
arise from aorta2. Truncal valve,
occasionally quadracuspid,
stenotic and/or insufficient;
overrides the ventricular septal
defect3. Ventricular septal
defect, large
Truncus Arteriosus
• Systolic thrill along the LSB• Normal S1 followed by a loud ejection
click while S2 is loud and single• Apical diastolic low-pitched murmur due
to increased flow across the normal mitral valve is audible
• Mortality by heart failure
Total Anomalous PulmonaryVenous Return
> All 4 pulmonary veins drain
to the RA
> RV volume
overload
Pulmonary Stenosis
• 5 – 8% of CHD• Associated with congenital rubella,
Noonan & William syndrome• Types: Valvar, subvalvular
(infundibular), supravalvular or peripheral
• Manifestations: asymptomatic unless severe
Pulmonary Stenosis
• Hemodynamics: RV pressure overload
• Physical examination– RV tap– Ejection click– Systolic thrill– Systolic ejection murmur at the LUSB
with radiation to the back; soft P2
Pulmonary Stenosis• ECG
– RAD– RBBB if mild– RVH (Pure R & upright T in V1)
• CXR– Normal or RV cardiomegaly– Normal or dilated MPA (post-stenotic
dilatation)
Pulmonary Stenosis
• Natural History:Asymptomatic; progression unlikelyEasy fatigability & CHF if severe
> Chest pain, syncope, sudden death
> Arrhythmias
> Infective endocarditis
Pulmonary Stenosis• Management:Interventional
catheterization– Balloon valvuloplasty
> Surgical– Valvotomy
(Brock’s procedure)
Aortic Stenosis• Valve is usually thickened and bicuspid
with fused commissures and eccentric orifice
• Rise in LV pressure due to LVOT obstruction
• LVH and high intracavitary pressure may lead to inadequate coronary artery filling
• Reduced compliance of LV – diastolic dysfunction
Aortic Stenosis• Usually asymptomatic until the LV fails• Syncope and sudden death may occur
with exercise• Harsh systolic ejection murmur at the
RUSB• Systolic thrill (suprasternal notch)• ECG may show ischemia in severe
stenosis
Aortic Stenosis
• Management:– SBE prophylaxis– Avoidance of competitive sports in all
except mild case– Balloon valvoplasty – Surgical open valvotomy– Aortic valve replacement
Coarctation of the Aorta• More common in boys• Obstruction in the descending aorta just
opposite the ligamentum arteriosum (after left subclavian artery)
• Aortic valve is bicuspid in more than 50%• Pressure build-up in the proximal aorta
and LV --- hypertension in the upper extremity
Coarctation of the Aorta• CHF in infancy if severe • Most children are asymptomatic• Weak, delayed or absent femoral pulses • Blood pressure higher in the arms than legs• LVH may be seen in CXR or ECG• Rib notching may be seen on CXR if
collaterals have formed (usually children > 5y)
Coarctation of the Aorta
• SBE prophylaxis
• Anti-hypertensive tx
• Balloon angioplasty/stent placement in selected cases (usually recurrent CoA and adolescent/adult)
• Surgical repair – treatment of choice
Systolic Ejection MurmursAtrial septal defect 2nd LICS with a widely
split S2
Pulmonic stenosis 2nd LICS with radiation to the upper back
Aortic stenosis 2nd RICS
Coarctation of the aorta
2nd LICS with radiation to the interscapular area
Systolic Regurgitant Murmurs
Ventricular septal defect
LLSB
Mitral regurgitation LLSB with radiation to the L ant. axillary line
Chest x ray findings in CHD:Tetralogy of Fallot Boot-shaped heart or
couer en sabot
Transposition of the great arteries
Egg-shaped heart
Total anomalous pulmonary venous return
Snowman sign or figure of 8
Coarctation of the aorta
Rib notching