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 Arteriosus 1. Pulmonary arteries arise from aorta 2. Truncal valve, occasionally quadracuspid, stenotic and/or insufficient; overrides the ventricular septal defect 3. 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 Pulmonary Venous 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 P 2

Pulmonary Stenosis ECG –RAD –RBBB if mild –RVH (Pure R & upright T in V 1 ) CXR –Normal or RV cardiomegaly –Normal or dilated MPA (post-stenotic dilatation)

Pulmonary Stenosis Natural History:  Asymptomatic; progression unlikely  Easy 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 Murmurs Atrial septal defect2nd LICS with a widely split S2 Pulmonic stenosis2nd LICS with radiation to the upper back Aortic stenosis2nd RICS Coarctation of the aorta 2nd LICS with radiation to the interscapular area

Systolic Regurgitant Murmurs Ventricular septal defect LLSB Mitral regurgitationLLSB with radiation to the L ant. axillary line

Chest x ray findings in CHD: Tetralogy of FallotBoot-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