Congenital Cardiac Disease Flashcards
Pulmunary stenosis
Associated with:
Alagille (peripheral pulmunary stenosis)
Williams (peripheral pulmunary stenosis)
Noonan (vavlular pulmunary stenosis)
Congenital rubella
Presentation:
Usually pink and asymptomatic
If severe: RVH as right ventricle must contract against obstruction
Newborns with critical PS - same as pulmunary atresia: R–> L shunt, cyanosis, poor feeding, tachypnoea
Murmur- ESM at LUSB radiating to back, may have associated thrill at LUSB or suprasternal notch
Systolic ejection click LUSB only if valvular (eg Noonan)- louder on expiration
Loudness of murmur= increased severity
ECG: RAD + RVH if mod
+ RAH + strain pattern (T wave inversion V1-V4, ST depression)
CXR: usually normal heart size (unless CHF), may have dilated main pulmunary artery (post stenotic dilatation)
Pulmunary vascular markings usually normal - decreased only if severe
Progression: mild not progressive, mod- severe worsens with age
Rx: balloon valvuloplasty
Aortic stenosis
Most commonly valvular- due to bicuspid valve - most common isolated congenital cardiac defect
If subvalvular, usually associated w VSD, PDA, CoA
Supravalvular associated w Williams
Symptoms: if mild- mod - ayptomtic w mild exercise intolorence
Classic triad: dyspnoea, chest pain, exertional syncope
Neonates with severe AS- poor perfusion w weak pulse, pale cool skin, slow cap refill, or resp distress caused by pulmunary oedema
O/E:Ejection systolic murmur heard loudest over the aortic area (RUSB)
Radiates to the carotid arteries, can also radiate to back
Loudest on expiration and when the patient is sitting forwards
Bicuspid aortic valve may have AR
Other clinical features of aortic stenosis may include:
Slow rising pulse with narrow pulse pressure if severe AS (but usually normal BP)
Non-displaced, heaving apex beat (if present indicates left ventricular hypertrophy)
Reduced or absent S2 (a sign of moderate-severe aortic stenosis)
Reverse splitting of S2: aortic valve closes after pulmonary valve (due to the longer time required for blood to exit the left ventricle)
Supravalvular: associated thrill
Valvuar: ejection click
Subvalvular: no thrill or click
Look for signs William syndrome (supravalvular AS)- elfin facies, II, friendly personality, big mouth, widely spaced teeth
ECG: normal if mild AS
LVH if mod-severe AS
CXR:usually normal, may have prominant aortic knob
Rx: balloon valvuloplasty or surgical repair
