More Pedia Cardio slides

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