congenital heart disease in SA Flashcards
pressure -overload
what are the causes of outflow obstruction
congenital outflow tract obstruction
- pulmonic stenosis
- sub-aortic stenosis
high afterload
combo of both
what is pressure overload
- consequence of outflow tract obstruction: (sub-) valvular stenosis
- increased reistance to the ventricular systolic outflow
- proportionate increase in ventricular pressure if flow remains constant
- pressure = flow x resistance
- increased wall stress leads to increased ventricular muscle mass (hypertrophy) - eccentric
what are the clinical signs due to outflow tract obstruction
- secondary to ventricular hypertrophy leads to reduced ventricular filling capacity
- during exercise when heart rate is high, the filling is even more impaired
- when cardiac output drops to a critical amount, cerebral hypoperfusion ensues (weakness or fainting)
- ventricular or atrial arrhythmias due to ischemic scars in myocardium (fainting, sudden death)
what is pulmonic stenosis
a dynamic or fixed anatomic obstruction to flow from the right ventricle to the pulmonary arterial vasculature
what breeds are predisposed to pulmonic stenosis
- bullmastif
- beagle
- bulldog
- spaniel
- keeshond
- schnauzer
- chihuahua
- terrier breed
what can pulmonic stenosis be due to
- isolated valvular (majority) obstruction
- subvalvular obstruction
- suprevalvular obstruction
- may be found in association with more complicated congenital heart disorders
what is type A valvular stenosis
lack of division of valve leaflets (fusion)
normal dimension of annulus
what is type B valvular stenosis
hypoplastic valve annulus and thickened leaflets
what is coronary malformation
- engl. bulldog
- aberrant left coronary artery encircles PA-causing narrowing
what are the clinical signs of PS
- none: diagnosis incidental during puppy well visit
- exercise intolerance
- syncope
what are the physical exam findings of patient with PS
- systolic murmur 4-6/6 loudest over left 3rd ICS
- arterial pulses: normal
- mucous membranes: pink
- ascites (rare)
what are the 3 diseases that can be associated with a systolic ejection murmur that is diamond shaped
- aortic stenosis
- pulmonic stenosis
- ventricular septal defect
what are the characteristics of PS on chest x-ray
- “reverse D” due to enlargement of the right ventricle (hypertrophy from chronic pressure overload)
- enlarge PA (post stenotic bulge)
what diagnostic plan provides a definitive diagnosis for PS
echocardiography
what are characteristics of PS on echo
- RV hypertrophy and dilation
- fused or thickened and relatively immobile pulmonic valve cusps
- turbulent blood flow across the stenosis
- post stenotic pulmonary artery dilation
PS
what does doppler allow us to estimate
- intra-cardiac pressure
- calculation of pressure difference (gradient) between the right ventricle and pulmonary artery
what is the Bernoulli equation
pressure gradient: 4 x velocity^2
how is the velocity of blood flow across the stenosis measured
by echo and used to asses the severity of disease
what are characteristics of PS using angiography
contrast
- hypertrophy of RV walls
- post-stenotic dilation of the main pulmonary artery
- filling defect at level of pulmonary valves
what is the prognosis of PS
- mild (0-50mmHg) - may need no intervention, good long term prognosis
- moderate (50-85mmHg) - may or may not need valvuloplasty
- severe (>85mmHg) - baloon dilation valvuloplasty -or for moderate with more than mild TR or R-L atrial shunt
how to tx PS
- beta blocker therapy (atenolol) to decrease HR during excercise and reduce RV hypertrophy; reduce myocardial O2 consumption
- baloon valvuloplasty
what is pulmonic stenosis coronary anomaly
- engl. bulldogs and some boxers may have an anamolous left main coronary artery arising from a single right coronary artery associated with the stenosis
- associated with an increased risk of balloon valvuloplasty b/c of the risk of avulsion of the left coronary artery during insufflation of the balloon and subsequent rupture of the left coronary
what is breeding advice for PS
- affected individuals and their parents should not be used for breeding
- siblings should only be used after careful screening
- if any affected offspring are born, breeding of the parents should be discontinued
key points
PS most commonly occurs due to:
- commissural fusion
- dysplasia of valve leaflets