2018 Board Review Pre-Test:
Cardiomyopathies III: HCM, Amyloid and Infiltrative

Cardiomyopathies III: HCM, Amyloid and Infiltrative - Akshay Desai, MD, MPH, FHFSA

Please complete this brief Pre-Test to give a baseline for learning .

Question Title

* 1. A 64-year-old female with HTN presents for evaluation of new onset dyspnea on exertion. She has NYHA Class 2 symptoms. BP =118/70, HR=64. She is taking amlodipine 10 mg daily, metoprolol 25 mg twice daily, and aspirin 325 mg daily. An echocardiogram reveals HCM, septal thickness of 22 mm with systolic anterior motion of the mitral valve, moderate mitral regurgitation, and a left ventricular outflow tract gradient of 50 mmHg at rest, increasing to 80 with Valsalva maneuver.

Which of the following should you recommend as the first step?

A. Discontinue amlodipine

B. Increase metoprolol

C. Septal myectomy

D. Septal ablation

Question Title

* 2. ICD implantation is considered appropriate for which of the following indications?

A. Syncope during blood draw 10 days ago

B. Left ventricular outflow tract gradient > 90 mmHg

C. Maximal left ventricular wall thickness > 30 mm

D. Late gadolinium enhancement > 5 % of LV mass on cardiac MRI

Question Title

* 3. A 48-year-old male HCM patient received an ICD after experiencing an out of hospital cardiac arrest. His maximum wall thickness is 25 mm and he has dynamic left ventricular outflow tract obstruction with a peak gradient of 64 mmHg at rest. He is active and asymptomatic.
What should you advise regarding screening of his relatives?

A. All adult first degree relatives should have an echocardiogram once after age 25 to exclude HCM.

B. Echocardiography is recommended every 2 years in all first-degree relatives

C. First degree relatives engaged in competitive athletics should be screened annually

D. A normal electrocardiogram is sufficient to exclude HCM in family members

Question Title

* 4. 61-year-old man who presents with h/o dyspnea on exertion and lightheadedness over the past 6 months.
Echo shows LVEF 40% with global hypokinesis, mildly decreased Rv function
Cardiac MRI shows extensive late gadolinium enhancement - most prominent in the basal and mid anteroseptal and inferoseptal RV insertion sites. Late gadolinium enhancement was also present in the basal and mid anteroseptal, anterior, inferoseptal and inferior segments in a mid-wall/sub-epicardial pattern.
Cardiac PET with no regional perfusion defects seen on the stress or rest images. On the glucose metabolic images, there was focal, intense FDG uptake involving the basal anterior and anteroseptal walls.

Which of the following is indicated in this patient?

A. Permanent pacemaker implantation

B. ICD implantation

C. EP study to see if inducible VT and then decide about PPM vs. ICD

D. Steroid therapy and then decide about device implantation based on response

Question Title

* 5. Please provide your first and last name.
Pre-test questions will not be scored, your name is required solely to confirm completion.

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