Heart Failure and Transplant Flashcards
Echo findings for HCM over athletes heart
- Irregular hypertrophy
- Normal sized LV diastolic dimensions
- Left atrial enlargement
- Abnormal ECG
- Abnormal LV diastolic function
- Female gender
Infectious cause of DCM from Latin America
Chaga’s disease
- Higher risk of stroke d/t apical aneurysms
Echo findings for restrictive cardiomyopathy
Enlarged atria (Mickey mouse ears) - won’t have this in constrictive pericarditis
EKG, echo and CXR findings for myocarditis
- Low voltage QRS and sinus tachycardia
- CXR with no cardiomegaly (acute process)
- Echo with thick myocardium (not thinned out like DCM)
Gold standard for diagnosis of myocarditis
Biopsy but not typically done (and low yield with patchy disease)
Biopsy will show lymphocytes and edema
Two peas
:)
MRI findings for myocarditis
Focal subepicardial delayed enhancement
Treatment for lyme myocarditis
IV ceftriaxone x 3 weeks
Treatment and biopsy findings for giant cell myocarditis
- Tx with steroids (only actual indication)
- Biopsy with multinucleated giant cell
EKG, echo and CXR findings for dilated CM
- EKG with increased LV forces, sometimes can see atrial enlargement
- Echo with thinned out myocardium
- CXR with cardiomegaly
Genetic mutations in DCM
- Dystrophin (X-linked)
- Sarcomere (TTN, MYH7)
- LAMIN (conduction problems)
MRI findings for DCM
Mid wall LGE
Outpatient treatment for DCM
- Asymptomatic: ACE, B-blocker
- Symptomatic: lasix, aldactone, digoxin
Aspirin
DCM and low ANC
Barth syndrome
Barth syndrome genetic mutation and enzyme problem
- Elevated 3-methylglutaconic acid
- TAZ gene mutation, X-linked (also linked to LVNC)
Arrhythmogenic RV cardiomyopathy has what type of mutations
Desmosome mutations
ITALIAN descent
ALCAPA ECG findings
Q waves in leads I and aVL
Exam findings for HCM murmur
- Crescendo decrescendo murmur that increases with standing or Valsalva and decreases with squatting and hand grip
Type of genetic mutations for HCM
Sarcomere mutations (MYBPC1, MYH7)
Autosomal dominant
MRI findings for HCM
LGE at LV and RV junctions
Pathology for DCM vs HCM
- DCM has fibrosis between cells
- HCM has chaotic/disarrayhypertrophy
Treatment for HCM
- B-blockers (DO NOT DECREASE RISK OF SUDDEN DEATH)
- CCB
- Exercise restriction
- ICD if 2 or more risk factors
HCM, hypotonia, big tongue, short PR disease and enzyme
Pompe disease
Alpha glucosidase deficiency
If alpha glucosidase is normal then it’s Dannon disease
HCM, coarse facial features, corneal clouding, HSM genetic disease and enzyme
- Alpha-L-iduronidase deficiency (Hurler syndrome)
If presents later without eye involvement then hunter syndrome
Survival of RCM
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
Contra-indications to transplant
- PVR > 8 and not reactive
- Irreversible end organ damage
- PA/PV severely hypoplastic
- Re-transplant within 6 months of prior transplant
Survival of transplant in infants vs adolescents
Infants: 1 yr 75%, 5 yr 68%
Adolescents: 1 yr 88%, 5 yr 68%
Why is ABO incompatible transplant acceptable in infants
- Organ transplant across blood types typically leads to hyper-acute rejection
- Infants don’t have blood group antibodies so it’s safe to do
What is induction immunosuppression post transplant
- 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
Mechanism of action of calcineurin inhibitors
Block T cell activation and IL2 production
Side effects of calcineurin inhibitors
- Tacro: nephrotoxic, diabetes
- Cyclosporine: HTN, gingival hyperplasia, hirsutism
Mycophenolate (cellcept) mechanism of action
Adjunct to calcineurin inhibitors
Prevents both T and B cell proliferation
What is sirolimus used for in post transplant
Used to prevent renal dysfunction and coronary artery disease (less potent rejection med)
Inhibits mTOR and T cell proliferation
Side effects of sirolimus
Wound healing (switch off if having surgery)
Mouth ulcers
Treatment for acute cellular rejection vs. antibody mediated rejection
- Cellular treat with pulse steroids
- AMR treat with IVIG or PLEX
Symptoms of CMV infection in transplant patient
Fever
Pneumonitis or colitis
Tx: ganciclovir
Really high risk for CAV with CMV
Most common form of PTLD
Abdominal
Most are EBV driven
Need tissue for diagnosis
Treatment for PTLD
Decrease immunosuppresion
Rarely need surgery or chemo
Leading cause of late graft loss or re-transplant
CAV – neo-intimal proliferation
Treatment for CAV
- Change from calcineurin inhibitor to sirolimus
- Statin
- ASA
- Re-transplant
Stage and class of heart failure
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
What heart failure meds do you have to dose adjust if you start amio
- Dig
- Warfarin