Congenital Heart Disease Flashcards
Acyanosis presentation
Cyanotic presentation
Eisenmenger syndrome aka
Pink lips/oral mucosa
Blue baby
Tardive/Late onset cyanosis
L to R Shunt
Acyanotic, inc pulm blood flow
Recirc of oxygenated blood
No dec O2 sats
ASD/VSD/PDA/AV canal
L to R shunt pathway
Complications
Late cyanosis- overcirc of lungs, PHTN, PVR inc, RVH, shunt reverses and get cyanosis
Sudden death
ASD Flow murmur
Excess flow across RVOT
Higher pressure in LA
Mostly due to secundum
VSD
Large
Small
Types
higher pressure in LV
At birth with HF
After 2-6 weeks, murmur
Perimembranous»Infundibular or muscular
AV Septal defect
Associated with
Failure of fusion of endocardial cushions
Large septal defect involving lower atrial septum and membranous ventricular septum
Down syndrome
PDA murmur
Treatment
Harsh, continuous
1 surgical ligation
Trascatheter coil occlusion
Medically (indomethacin PGEI)
ToF
May also have
Autosuperior mal-alignment of indfundibular septum
Valvular PS, RAA, ASD, absent ductus
(D) TGA
Anatomy
Repair options
Conotruncal error during organogenesis and looping of heart
Ventriculoarterial discordance with RV connecting to aorta, LV with PA
Arterial swith or atrial/venous switch (mustard)
(L) Congenitally corrected TGA
Results in
Ventricular inversion (corrects) transposition
Ventriculoarterial and atrioventricular discordance
RA to LV to PA to Lungs
LA to RV to Ao
Truncus arteriosus
Failure to divide Truncus into aorta and PA
TAPVR
Pulmonary veins train into the L innominate V, coronary sinus, portal venous system
Coarctation
Association
With PDA
W/o PDA
Narrowing of aorta
Infantile- proximal to PDA
Adult- aortic lumen, after SCA
Turner’s syndrome
Early sx, require surgery
Asx, UE HTN and weak LE pulses later
Turner syndrome presentation
Female, short webbed neck streak ovaries coarctation BAV
Congenital AS with Atresia in Neonate
Neonatal critical aortic Valve stenosis
Subvalvular (subaortic) stenosis
Small/dysplastic obstructive cusp tissue with diminutive valve ring
If severe, can lead to HLHS (LV and AA hypoplasia and LV endocardial fibroelastosis
Ring/collar
Williams Syndrome (Supravalvular AS)
Elfin facies (lateral upper eyelid fullness, small nose and full lips)
Hypercalcemia and irritability
Cocktail party personality
Mental retardation
May have thickening/constriction of aortic wall
Noonan
Short, webbed neck Epicanthal folds Ptosis Hypertelorism Ear malformation Hypoplastic nipples Shield Chest/Pectus Exc Cryptochidism
PS/ASD
Dysplastic valves
HCM (RV and LV)
MVP
