OBSTRUCTIVE LESIONS

present with pallor, decreased urine output, cool extremities and poor pulses Coarctation of the Aorta narrowing of aor...

• present with pallor, decreased urine output, cool extremities and poor pulses

Coarctation of the Aorta

1. narrowing of aorta almost always at the level of the ductus arteriosus
2. commonly associated with bicuspid aortic valve (50%) '
3. if severe, presents with shock in the neonatal period when the ductus closes
4. often asymptomatic with upper extremity systolic pressures of 140-145 mm Hg
5. weak pulses, decreased blood pressure in lower extremities, radial-femoral delay
6. if associated with other lesions (e.g. PDA, VSD), can cause CHF
7. murmur: absent or systolic with late peak at apex, left axilla, left back
8. management: balloon arterioplasty or surgical correction
9. complications: essential hypertension
   

 

Aortic Stenosis

 

 

1. valvular (75%), subvalvular (20%), supravalvular and idiopathic hypertrophic subaortic stenosis (IHSS) (5%) 
2. often asymptomatic but may be associated with CHF, exertional chest pain, syncope or sudden death 
3. murmur:  SEM at URSB with aortic ejection click at the apex
4. management:  surgical or balloon valvuloplasty, repeated interventions and valve replacement may be necessary
5. SBE prophylaxis and exercise restriction required
   

 

Pulmonary Stenosis

 

1. valvular (90%), subvalvular or supravalvular
2. usually part of other congenital heart lesions (e.g. Tetralogy of Fallot) or in association with other syndromes (e.g. congenital rubella, Noonan syndrome)
3. critical pulmonic stenosis: inadequate pulmonary blood flow, dependent on ductus for oxygenation, progressive hypoxia and cyanosis
4. presentation varies from asymptomatic to CHF
5. murmur: wide split S2 maximal on expiration, SEM at ULSB, pulmonary ejection click
6. ECG: RVH 
7. CXR: dilated poststenotic pulmonary artery
8. management: balloon valvuloplasty
   

Hypoplastic Left Heart Syndrome

 

1. a spectrum of hypoplasia of left ventricle, atretic mitral and/or aortic valves, small ascending aorta, coarctation of the aorta with resultant systemic hypoperfusion
2. most common cause of death from congenital heart disease in first month of life
3. presents with circulatory shock and metabolic acidosis on closure of the ductus

 

Management

1. intubate and correct metabolic acidosis
2. IV infusion of PGE1 to keep ductus open
3. treatment options
• surgical correction (overall survival 50% to late childhood)
• transplantation
• no treatment