Supravalvular Aortic Stenosis

Question 1

Supravalvular AS is associated with

Answer 1

William syndrome

Hypercalcemia
Growth failure
Mental retardation

Question 2

Subbalvular AS Is associated with

Answer 2

Hypertrophic cardiomyopathy

Question 3

Pathology

Answer 3

Concenric left venticular hypertrophy (systole maintained)
+ increased flow gradient
+increased velocity

LV filling pressure is high
Diastolic dysfunction

As disease progree contracctile function is lost

(Systolic failure) Cardiac output lost

Gradient lost

Question 4

Symptomatology of AS

Answer 4

ASD 532
ANGINA SYNCOPE DYSPNEA

anypoint he can have unexplained sudden cardiac death <1%

Question 5

Why angina in AS

Answer 5

Left ventricular hypertrophy
Decreased diastolic perfusipn time
Decreased capillary density with respect tobwall thickness
Associated atherosclerosis

Question 6

Syncope reason

Answer 6

Fixed cardiac output state

Question 7

Dyspnea reason

Answer 7

Increased LV filling pressure
Decreased cardiac output state ( during failure)

Question 8

HEYES sx

Answer 8

Aortic stenosis with angiodysplasia of the ascending colon

Question 9

Pulse

Answer 9

Severe–pulsus tardus pattern (slow rising pulse)

Very severe–pulsus parvus tardus(amplitufe low)

Question 10

Apex in AS

Answer 10

Sustained heaving apex
Laterally displaced