Heart Failure and Transplant

Question 1

Echo findings for HCM over athletes heart

Answer 1

• Irregular hypertrophy
• Normal sized LV diastolic dimensions
• Left atrial enlargement
• Abnormal ECG
• Abnormal LV diastolic function
• Female gender

Question 2

Infectious cause of DCM from Latin America

Answer 2

Chaga’s disease
- Higher risk of stroke d/t apical aneurysms

Question 3

Echo findings for restrictive cardiomyopathy

Answer 3

Enlarged atria (Mickey mouse ears) - won’t have this in constrictive pericarditis

Question 4

EKG, echo and CXR findings for myocarditis

Answer 4

• Low voltage QRS and sinus tachycardia
• CXR with no cardiomegaly (acute process)
• Echo with thick myocardium (not thinned out like DCM)

Question 5

Gold standard for diagnosis of myocarditis

Answer 5

Biopsy but not typically done (and low yield with patchy disease)

Biopsy will show lymphocytes and edema

Question 6

Two peas

Answer 6

:)

Question 7

MRI findings for myocarditis

Answer 7

Focal subepicardial delayed enhancement

Question 8

Treatment for lyme myocarditis

Answer 8

IV ceftriaxone x 3 weeks

Question 9

Treatment and biopsy findings for giant cell myocarditis

Answer 9

• Tx with steroids (only actual indication)
• Biopsy with multinucleated giant cell

Question 10

EKG, echo and CXR findings for dilated CM

Answer 10

• EKG with increased LV forces, sometimes can see atrial enlargement
• Echo with thinned out myocardium
• CXR with cardiomegaly

Question 11

Genetic mutations in DCM

Answer 11

• Dystrophin (X-linked)
• Sarcomere (TTN, MYH7)
• LAMIN (conduction problems)

Question 12

MRI findings for DCM

Answer 12

Mid wall LGE

Question 13

Outpatient treatment for DCM

Answer 13

• Asymptomatic: ACE, B-blocker
• Symptomatic: lasix, aldactone, digoxin
  Aspirin

Question 14

DCM and low ANC

Answer 14

Barth syndrome

Question 15

Barth syndrome genetic mutation and enzyme problem

Answer 15

• Elevated 3-methylglutaconic acid
• TAZ gene mutation, X-linked (also linked to LVNC)

Question 16

Arrhythmogenic RV cardiomyopathy has what type of mutations

Answer 16

Desmosome mutations

ITALIAN descent

Question 17

ALCAPA ECG findings

Answer 17

Q waves in leads I and aVL

Question 18

Exam findings for HCM murmur

Answer 18

• Crescendo decrescendo murmur that increases with standing or Valsalva and decreases with squatting and hand grip

Question 19

Type of genetic mutations for HCM

Answer 19

Sarcomere mutations (MYBPC1, MYH7)
Autosomal dominant

Question 20

MRI findings for HCM

Answer 20

LGE at LV and RV junctions

Question 21

Pathology for DCM vs HCM

Answer 21

• DCM has fibrosis between cells
• HCM has chaotic/disarrayhypertrophy

Question 22

Treatment for HCM

Answer 22

• B-blockers (DO NOT DECREASE RISK OF SUDDEN DEATH)
• CCB
• Exercise restriction
• ICD if 2 or more risk factors

Question 23

HCM, hypotonia, big tongue, short PR disease and enzyme

Answer 23

Pompe disease
Alpha glucosidase deficiency

If alpha glucosidase is normal then it’s Dannon disease

Question 24

HCM, coarse facial features, corneal clouding, HSM genetic disease and enzyme

Answer 24

• Alpha-L-iduronidase deficiency (Hurler syndrome)

If presents later without eye involvement then hunter syndrome

Question 25

Survival of RCM

Answer 25

2 year is 50% - very poor Often transplant early but need to monitor PVR closely pre-transplant as at high risk of developing irreversible PH

Question 26

Contra-indications to transplant

Answer 26

- PVR > 8 and not reactive - Irreversible end organ damage - PA/PV severely hypoplastic - Re-transplant within 6 months of prior transplant

Question 27

Survival of transplant in infants vs adolescents

Answer 27

Infants: 1 yr 75%, 5 yr 68% Adolescents: 1 yr 88%, 5 yr 68%

Question 28

Why is ABO incompatible transplant acceptable in infants

Answer 28

- Organ transplant across blood types typically leads to hyper-acute rejection - Infants don't have blood group antibodies so it's safe to do

Question 29

What is induction immunosuppression post transplant

Answer 29

- 5-7 days post transplant - Give ATG/thymoglobulin which eradicates T cells - Goal is to delay starting calcineurin inhibitors (d/t renal toxicity) - Anti-CD3 rabbit monoclonal antibody

Question 30

Mechanism of action of calcineurin inhibitors

Answer 30

Block T cell activation and IL2 production

Question 31

Side effects of calcineurin inhibitors

Answer 31

- Tacro: nephrotoxic, diabetes - Cyclosporine: HTN, gingival hyperplasia, hirsutism

Question 32

Mycophenolate (cellcept) mechanism of action

Answer 32

Adjunct to calcineurin inhibitors Prevents both T and B cell proliferation

Question 33

What is sirolimus used for in post transplant

Answer 33

Used to prevent renal dysfunction and coronary artery disease (less potent rejection med) Inhibits mTOR and T cell proliferation

Question 34

Side effects of sirolimus

Answer 34

Wound healing (switch off if having surgery) Mouth ulcers

Question 35

Treatment for acute cellular rejection vs. antibody mediated rejection

Answer 35

- Cellular treat with pulse steroids - AMR treat with IVIG or PLEX

Question 36

Symptoms of CMV infection in transplant patient

Answer 36

Fever Pneumonitis or colitis Tx: ganciclovir Really high risk for CAV with CMV

Question 37

Most common form of PTLD

Answer 37

Abdominal Most are EBV driven Need tissue for diagnosis

Question 38

Treatment for PTLD

Answer 38

Decrease immunosuppresion Rarely need surgery or chemo

Question 39

Leading cause of late graft loss or re-transplant

Answer 39

CAV -- neo-intimal proliferation

Question 40

Treatment for CAV

Answer 40

- Change from calcineurin inhibitor to sirolimus - Statin - ASA - Re-transplant

Question 41

Stage and class of heart failure

Answer 41

Stage is LV dysfunction - A: at risk, B: mild/mod and asymptomatic, C: severe/symptomatic, D: refractory Class is symptoms - 1: asymptomatic, 2: symptoms with moderate exertion, 3: symptoms with mild exertion, 4: symptoms at rest

Question 42

What heart failure meds do you have to dose adjust if you start amio

Answer 42

- Dig - Warfarin