Restrictive Cardiomyopathy

Question 1

In restrictive cardiomyopathy, what are the main LV findings?

Answer 1

• Non-dilated LV (or small cavity).
• Normal systolic function (in early disease; impairs later on).
• Impaired diastolic dysfunction (with or without LVH).

Question 2

RCM can be classified as primary/idiopathic or secondary; which has normal wall thickness and which has LVH?

Answer 2

Primary/idiopathic = normal wall thickness; Secondary = LVH.

Question 3

Which classification of RCM (primary/idiopathic or secondary) are due to infiltrative disorders?

Answer 3

Secondary.

Question 4

Impaired diastolic dysfunction results in an increase in what three pressures?

Answer 4

LVEDp and LAp (and as a result elevated right sided pressures).

Question 5

True or false; a common finding in RCM is biatrial dilatation.

Answer 5

True.

Question 6

True or false; a common finding in RCM is biventricular dilatation.

Answer 6

False; usually the ventricles are normal size and systolic function.

Question 7

True or false; a common finding in RCM is a dilated IVC.

Answer 7

True.

Question 8

In restrictive physiology (grade III diastolic dysfunction) what are the “normal” findings?

Answer 8

E/A >2, DT <130ms, reduced e’ velocities, dilated LA, S/D<1 and E/e’ >13.

Question 9

What is the most common cause of secondary RCM?

Answer 9

Cardiac Amyloidosis.

Question 10

LV wall thickness of >12mm (with concentric thickening) may suggest an infiltrative disease in the absence of what?

Answer 10

Other pathology, such as HTN, HCM or significant AS.

Question 11

True or false; a common finding in RCM is a small pericardial/pleural effusion.

Answer 11

True.

Question 12

With regards to the pulmonary vein flow, a PV and a dur-A dur of what is indicative of restrictive filling?

Answer 12

PVa ≥ 0.35m/s ; a dur - A dur ≥ 20ms.

Question 13

IVRT is the time interval between the end of LVOT ejection and the onset of mitral inflow; a short or long IVRT is in keeping with severe restrictive filling?

Answer 13

Short.

Question 14

What is considered short for IVRT?

Answer 14

<50ms.

Question 15

“A” dur refers to what?

Answer 15

Duration of atrial filling wave on PW Doppler (mitral inflow).

Question 16

“a” dur refers to what?

Answer 16

Duration of atrial reversal from PW Doppler of pulmonary vein flow.

Question 17

With the a dur - A dur calculation, which is taken away from which (between mitral inflow and pulmonary vein flow).

Answer 17

Pulmonary Vein Flow - Mitral Inflow.