ADULT CONGENITAL HEART DISEASE Flashcards
intro
spectrum of disorder= diff to those in kids
adutls likelier to have more complex lesions
commonest in order of freq
- bicuspid aortic valve
- asd
- vsd
- tof
bicuspid aortic valve
no probs at birth - works fine
eventually develop stenosis + regurg
predisposed to IE/SBE/dissection
intense exercise may exacerbate problems
asd- what are the 2 types of holes and which is more common
ostium secundum- high in septum- commonest
- oft asympto till adulthood
ostium primum- present early, associated w. AV valve abnormalities
asd s/s
embolism may pass from venous system to left side of heart causing a stroke
symptoms:
- dyspnoea
- phtn
- arrhythmias
- chest pain
signs:
- AF
- high JVP
- pulm ESM
- PHTN may cause TR/PR
- increased freq migraines
- wide fixed split S2
asd: ix
Ostium secundum (70% of ASDs)
- associated with Holt-Oram syndrome (tri-phalangeal thumbs)
- ECG: RBBB with RAD
Ostium primum
- present earlier than ostium secundum defects
- associated with abnormal AV valves
- ECG: RBBB with LAD, prolonged PR interval
asd: rx
This is by surgical repair of the defect, which has a good result if pulmonary hypertension has not developed.
An excess pulmonary blood flow of 1.5 times the systemic flow is generally regarded as an indication for surgery in adults.
treatment of secundum defect
- A defect that is moderate or large should be sutured or covered with a patch of pericardium.
rx of primary defect
- Early surgery is recommended.
- The defect is closed and incompetent valves are repaired.
- The operation has a higher morbidity and mortality than that for an ostium secundum.
vsd define
normally septum separates 2 ventricles
now a hole connects them
vsd: causes
congential - 2 in 1000 births
acquired- post MI
vsd vsd: s/s
depends on size + site of holes
small VSD- hemodynamically les significant and give louder murmmurs
- harsm PSM @ L sternal edge
- systolic thrill
- L parasternal heave
large VSD: pulm htn
vsd: complications
IE/ SBE
PHTN
eisenmenger’s complex [long standing L to R cardiac shunt]
vsd ix
ecg
- small vsd: normal ecg
- large vsd: LVH, RVH
cxr:
- small: mild pulm plethora
- large: cardiomegaly + marked pulm plethora
rx
rx: surgical closure if symptomatic VSD w/ large shunt
tetrology of fallot define
etralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease*.
It typically presents at around 1-2 months, although may not be picked up until the baby is 6 months old
TOF is a result of anterior malalignment of the aorticopulmonary septum. The four characteristic features are:
- ventricular septal defect (VSD)
- right ventricular hypertrophy
- right ventricular outflow tract obstruction, pulmonary stenosis
- overriding aorta
tof s/s
The severity of the right ventricular outflow tract obstruction determines the degree of cyanosis and clinical severity
infants:
- Acyanotic at birth- only pulm mumur is initial finding
- gradually becomes cyanotic- due to less blood flow to lungs and R to L shunt across VSD
- during hypoxic spell:
- restless agitation
- inconsolable crying
- toddlers squat [increased peripheral vasc resistance]
- difficulty feeding
- failure to thrive
- clubbing
ADULTS:
pts = oft asympto
cyanosis - common
late symptoms:
- exertional dyspnoea
- palpies
- rv failire
- syncope
- even sudden death
- ESM of pulm stenosis
tof: ix
ecg- rvh + rbbb
cxr- normal/boot shaped heart
echo- shows anatomuy + degree of stenosis
cardiac ct/mri- gives valuable info for planning surgery
