Congenital heart disease Flashcards
What is the Ross operation?
For those with aortic valve issues
Replaces aortic valve with patients own pulmonary valve
Allograft for pulmonary valve
Increased longevity when compared to bioprosthetic valve
Where does the aorta come off in transposition of the great arteries?
RV
What are the causes of congenital heart disease?
Genetic
Toxins
Viral infections
Idiopathic (majority of cases)
What are some genetic causes of congenital heart disease?
Trisomy 21 - VSD / AVSD 22q deletion (Di George Syndrome) - tetralogy of fallot
Cyanotic heart disease definition
Due to cardiac lesion (usually VSD or ASD) oxygen saturations low (75-80%)
What CHDs cause shunting?
ASD
VSD
PDA
Anomolous pulmonary veins
What are the lesions in Tetralogy of Fallot?
VSD
RV hypertrophy
Overriding aorta
PA stenosis
What is Ebsteins anomaly?
Anomaly of TV and RV:
TV leaflet adheres to wall of underlying myocardium, downward displacement of TV annulus, dilatation of the atrialised portion of RV, fenestrations in the valve
What are some complications following repair of tetralogy of fallot?
Pulmonary regurgitation / stenosis
Arrythmias (esp. with trans-ventricular approach)
RV/LV dysfunction
Aortic root dilatation
What are inherited causes of aortic aneurysm? (9)
Bicuspid AV Ehler's Danlos Turner syndrome Famillial aortic aneurysm Loey's Dietz Marfan syndrome Non-specific connective tissue disorder TOF / Truncus arteriosus Idiopathic
What vasculidities are associated with aortic aneurysm?
Giant cell
Takayasu
Kawasaki
Behcet
What percentage of first degree relatives are affected by aortic aneurysm?
15-20%
What percentage of births have bicuspid AV?
What is the mode of transmission?
~2%
Autosomal dominant
What is D-TGA and L-TGA?
D-TGA; aorta comes of right sided RV
L-TGA: aorta comes off left sided RV
What diseases are associated with pulmonary valve stenosis? (2)
Noonan syndrome
William syndrome
