Heart Failure and Cardiomyopathies Flashcards
What is the diagnosis:
- Heart failure with reasonably preserved EF ~ 50%
- Normal coronaries
- LV hypertrophy
- Renal dysfunction
- FH: brother and maternal grandfather also affected by HF
Fabry’s disease
- X-linked disorder
- Alpha-galactosidase A deficiency
Describe the findings
Restrictive Cardiomyopathy (Doppler)
- classic findings
What is recommended treatment course for LVNC?
What maneuvers would increase HCM murmur?
- Valsalva (decreased preload)
- Squat-to-stand (decreased preload)
- Exercise (decreased preload + increase afterload)
*****Dramatically different with maneuvers
What is the diagnosis?
RCM - ARVD
What are the main treatment options for HOCM?
- Decrease contractility + Preserve DFP and preload
- BB
- Verapamil
- Diltiazem
- Disopyramide
What is the best measure of treatment success with HOCM?
symptomatic improvement
What is the differential diagnosis of thickened LV?
- LVH
- HCM
- Renal failure
- Cardiac Amyloid
- Glycogen storage disease
- Anderson-Fabry’s disease
- Freidreich’s ataxia
When can genetic testing be utilized to screen family members in HCM?
Positive genetic testing in a family member
- specific genetic mutation identified
- can be utilized to screen family members for this mutation
Define stroke work of the ventricle
What influences stroke work?
- Represented by the area enclosed by the pressure-volume loop
- Changes in stroke work are influenced by:
- Preload
- Afterload
- Intrinsic contractility
Describe the classic hemodynamic findings of RCM:
- LVEDP > 5 mmHg + RVEDP
- Elevated filling pressures
- Ventricular concordance
- RVEDP < ⅓ of RVSP
- RVSP > 50 mmHg
- Dip and plateau morphology
Describe details of HCM:
- Prevalence
- Overall prognosis
- SCD rate
- Family screening
- Exercise
- Medication
- Prevalence –> 1:500
- Overall prognosis –> Good
- SCD rate –> 1% year
- Family screening –> Needs screening
- Exercise –> Healthy lifestyle
- Medication –> Treat symptoms
What is the diagnosis?
RCM - Cardiac Amyloidosis
What is the most common symptom associated with LV noncompaction?
Dyspnea
When are patiets considered to have obstructive HCM?
PG > 30 mmHg (at rest)
- PG > 50 mmHg –> sufficient to result in symptoms
What patient’s with LVNC would benefit from ICD placement?
- FH SCD
- NSVT
- Reduced EF
- Syncope (unexplained) / Symptoms
- LGE on cMRI
*****Class IIb recommendations
What are the indications for surgical myectomy or ETOH septal ablation in HOCM?
- LVOT obstruction
- Angina, dyspnea, and/or syncope resulting in significant impairment in quality of life
- Symptoms persist despite appropriate medical therapy
Describe key findings of ARVD
- fatty replacement of RV free wall
- EKG
- Repolarization abnormality
- Conduction delay
- Arrhythmia
- VT with LBBB morphology
- 30% with FH
- No competitive athletics
What is the most likely intervention? (A –> B)
Dobutamine (primarily B1-adrenergic agonist)
- Shift in the end-systolic pressure-volume relationship (ESPVR) –>
- consistent with an increase in contractility
- Increase in stroke volume
- difference between EDV and ESV
- EDV is reduced, because the increased contractility of the LV has resulted in a lower ESV before onset of diastolic filling
- difference between EDV and ESV
What is the diagnosis?
RCM - Hypereosinophilic syndrome (Loeffler’s syndrome)
Describe the diagram
- The end-systolic and end-diastolic pressure-volume relationships (ESPVR, EDPVR) are the boundaries of the PV loop
- End-systolic elastance (Ees): surrogate for cardiac contractility and is represented by the slope of the ESPVR
Define the pressure-volume loop
- depict instantaneous recordings of ventricular pressure against ventricular volume during a single cardiac cycle
- Loop area, which represents stroke work, changes based on varying:
- preload
- afterload
- intrinsic properties of the myocardium
- ESPVR and EDPVR define these properties - remain constant in spite of changing loading conditions of the heart
How can you estimate stroke work?
SV x mean LV or aortic pressure (during ejection) = stroke work
Define ESPVR
End-systolic PV relationship
- linear relationship
- represents the contractile properties of the chamber
- when the myocardium is maximally contracted
What is the preferred screening methodology in HCM family members?
- Positive genetic mutation (in family member)
- genetic testing
- Negative genetic testing (in family member)
- imaging
What is the inheritance pattern of HCM?
Autosomal dominant
- each offspring will have 50% chance of inheriting
What is the monitoring frequency for HCM family members?
- < 18 years of age –> every 12-18 months
- Adults –> every 5 years
When should family members be screened for HCM?
No later than onset of puberty
or
At any consideration of competitive athletics
What condition would “Bifid impulse” be heard?
HOCM or mixed aortic disease with severe AR
- apex - palpable S4
- single central pulse wave with two peaks separated by a distinct mid-systolic dip.
- early component percussion wave results from rapid left ventricular ejection
- late component tidal wave represents a reflected wave from the periphery due to an artery’s recoil effect.
Define EDPVR
End-diastolic PV relationship
- nonlinear relationship
- represents the stiffness properties of the ventricular chamber
- when the myocardium is maximally relaxed and undergoing filling
Define end-systolic elastance
- slope of the ESPVR
- surrogate for cardiac contractility
- leftward shift (increased steepness of the slope) of ESPVR
- positively inotropic drugs
- increased HR (pacing, physiologic stimuli)
- “force-frequency relationship”
- rightward shift (decreased steepness of the slope) of ESPVR
- negatively inotropic drugs
How is the EDPVR affected in regards to volume?
Nonlinear
- Low chamber volumes –> increases in volume are associated with minimal changes in pressure
- high LV chamber compliance at low volumes
- High chamber volumes –> increases in volume are associated steep changes in pressure
- chamber compliance has decreased as a result of stretch of elastic elements
Describe the findings
Positive inotropy - Increased HR (pacing, physiologic stimuli)
- ESPVR is shifted to the left without change in preload or afterload
Describe the findings
Positive Inotropy and Positive Lusitropy
- ESPVR is shifted to the left
- EDPVR is displaced down and to the right
- Inotropes: Epinephrine, Isoproterenol
- Lusitropy: rate of myocardial relaxation
Describe the findings
Increased afterload - Phenylephrine
- Afterload is elevated without change in contractility or stiffness –> reduce stroke volume
- Phenylephrine
Describe the findings
Decreased afterload
- Afterload is reduced without change in contractility or stiffness, –> increased SV
- Sodium nitroprusside, Hydralazine, ACE
Describe the findings
Increased preload - IV fluids
- Preload is elevated without a change in contractility or stiffness –> increased SV
Describe the findings
Negative inotropy - BB’s or CCB’s
- ESPVR is shifted to the right without change in afterload or preload
- BB’s or CCB’s
Describe the findings
Frank-Starling Relationship
- “Length-Tension” Relationship
- increases in EDV –> stretch of ventricular myocytes –> increased tension generation –> stronger contraction
- allows the heart to increase SV when there is increased venous return
- Increasing chamber volume beyond a certain point –> decreases tension generation
What are the Class I recommendations for ICD implantation in patients with Hypertrophic Cardiomyopathy?
- SCD
- VF
- VT (hemodynamically significant)
What are the Class IIa recommendations for ICD implantation in patients with Hypertrophic Cardiomyopathy?
Primary Prevention
-
Family History HCM - SCD
- > 1 first degree relative
- Unexplained syncope (non-neurocardiogenic)
- Massive LVH > 30 mm
Class IIb - (Require additional risk factors)
- Multiple-repetitive NSVT (on Holter)
- Abnormal exercise BP response
*****Require additional risk factors
- End-stage (LVEF < 50%)
- LV apical aneurysm
- LGE > 15% LV mass**
- Marked LVOTO ( > 30mm Hg) at rest
- Modifiable (intense competitive sports, CAD)
- Age > 60 years
- SCD uncommon in this age group
- Alcohol septal ablation (?)
Describe the risk stratification groups in Hypertrophic cardiomyopathy
What causes changes to EDPVR?
Changes in intrinsic properties or composition of the myocardium
- ischemia
- fibrosis
- hypertrophy
- infiltrative disease
Describe the features of GCM
- Pathophysiology
- Histologically
- Pathophysiology:
- believed to be mediated by T lymphocytes
- can be transferred by T lymphocytes in animal models
- Histologically
- characterized by a diffuse, nongranulomatous infiltrate of T lymphocytes, histiocytes, and eosinophils with myocyte necrosis and little fibrosis
What are the discharge criteria for HF patients?
What are the discharge criteria for HF patients?
Should be considered for patients with advanced or refractory HF?
What is one way to confirm HFpEF in patients with normal resting hemodynamics?
PASP > 45 mmHg with exercise
- high sensitivity
Describe excitation-contraction coupling
- Mechanism by which small amounts of extracellular calcium enter the myocyte (first step**) during the plateau phase of the action potential
- lead to larger intracellular calcium release from the SR to initiate myocardial contraction
What is the diagnosis?
- commonly seen in younger individuals
- rapildly progressive (decline in cardiac function - EF 23%)
- often associated with ventricular arrhythmias
- high frequency of autoimmune disorders
Giant cell myocarditis
Describe the features of Giant Cell Myocarditis
- fulminant, rapidly progressive disease that is usually fatal and affects young, otherwise healthy individuals
- associated with autoimmune conditions (but specific cause not known)
- Presentation: (63 GCM patients diagnosed with biopsy)
- fulminant disease that presents within days to weeks
- new heart failure symptoms (75%)
- ventricular arrhythmias (14%)
- heart block (5%)
- fulminant disease that presents within days to weeks
Describe the finding
Severe TR
- monophasic “CV” wave
- CV wave lifted completely off the baseline
- monophasic event in systole, occurring within the RA
- ventricularization of RA waveform
What constitutes a pathologic or abnormal “v” wave in PCWP tracings?
What are causes?
- “v” wave more than 10 mmHg than PCWP
- PCWP “v” waves
- MR
- VSD
- Noncompliant LA
- previous A-fib ablation procedures
Describe the findings
Pericardial Tamponade
- Rapid x only
- Blunted ‘y’ descent (no early diastolic RV filling)
Describe the findings
Pericardial constriction
- Rapid x and y descents
- y = early rapid diastolic RV filling
Describe when step-up O2 saturations are significant?
What does this imply?
Intra or Extra cardiac shunt may be present
What is the differential diagnosis in a patient with rapidly progressive heart failure and high-grade heart block?
- GCM
- Sarcoidosis
- Lyme disease
- Chagas disease
Diagnose GCM
EMB (RV)
- sensitivity (85%)
- due to diffuse endocardial pattern of inflammation
- If results inconclusive or discordant –>
- re-biopsy of RV or LV EMB should be considered
Describe the findings
- a = atrial systole
- x = atrial relaxation, decrease of pressure
- c = closure of the TV
- v = ventricular systole, atrial diastole
- y = passive filling of the RV
What is the treatment for GCM?
- GDMT for heart failure
- avoidance of Digoxin (risk of heart block and proarrhythmia)
- Mechanical support (IABP, VADs) –> bridge to recovery or transplant
- 78% of patients on GCM registry with VADs had successful bridging to transplantation
- Immunosuppression
- can see histopathologic improvement, but replacement with fibrosis is common
- cessation –> recurrence (as far as 8 years after diagnosis)
What are the most important predictors of hospital morbidity and mortality in ADHF patients?
- Elevated BUN ( ►43 mg/dL)
- SBP ( < 100-115 mmHg)
- Elevated Cr. ( ► 2.0-2.75 mg/dL)
******ADHERE and OPTIMIZE-HF Trials
What is the recommendation for anticoagulation in PPCM?
Therapeutic consideration
- due to high incidence of thrombembolism
- especially during pregnancy and in the first 6-8 weeks postpartum due to hypercoaguability during these time peiords
What are the indications for invasive hemodynamic monitoring / RHC in heart failure patients?
Decompensated HF who:
- not responding as expected when decision making based on non-invasive methods
- persistent symptoms
- hypotension
- renal failure despite apparent volume overload
- therapies with significant risks are being considered
- Inotropes
- LVADs
- Transplant
In addition to guideline-directed medical therapy, what intervention for HF patients has also been proven to help reduce rehospitalizations?
Standardized disease education
- 1-hour nurse educator-delivered teaching session at the time of discharge resulted in:
- improved clinical outcomes
- increased self-care and treatment adherence
- reduced cost of care
What is the 5-year mortality rate among individuals newly diagnosed with Heart Failure?
50%
What conditions represent the highest population attributable risk for developing HF?
Hypertension
and
CAD
What is the treatment for lymphocytic myocarditis?
GDMT for heart failure
- BB, ACE, diuretics
- No role for immunosuppresive therapies
Describe the findings
- A-fib
- LVH
- ST and/or T wave abnormalities secondary to hypertrophy
- I, V5 and V6
- downsloping ST-segement depression and TWI in leads
- V1-V2
- subtle ST-segment elevation ( < 1 mm)
- I, V5 and V6
What is the main reason to initiate chronic inotropic infusion therapy?
Palliation
- mortality is very high in this patient cohort
What is the diagnosis and next best step?
- 54 year old male with HFpEF presents with volume overload
- PE: JVP, clear lungs, S4 gallop and pitting edema
- EKG: low voltage and nonspecific ST-T wave changes
- Echo: LVEF 50%, severe biatrial enlargement, PASP 50 mmHg
- RHC: equalization of diastolic pressures, prominent Y descent, “square-root” sign
-
Restrictive CMP vs. Constrictive pericarditis
- suspected Cardiac Amyloidosis
-
Endomyocardial biopsy
- gold standard
What are the absolute contraindications to these mechanical support devices?
- Impella
- IABP
- TandemHeart
- VA-ECMO
- Impella –> LV thrombus
- IABP –> moderate-severe AR
- TandemHeart –> LA thrombus
- VA-ECMO –> severe PAD
What is the screening/monitorig for cardiotoxicity in patients undergoing chemotherapy treatment?
- Detailed CV history and exam
- Medical management of CV risk factors
- Imaging Assessment of cardiac function
- prior to and during therapy
- Consideration of Biomarker assessment
- Troponin, NT-proBNP
- prior to and during therapy
Describe the findings
HOCM - Brockenbrough sign
- PVC –> ventricular contraction will be more forceful, and the pressure generated in the LV will be higher
- Pressure gradient –> increased
- Pulse pressure –> decreased
Describe the findings
- NSR
- 1st degree AV block
- 2:1 AV block
- LAD
- LVH
- RBBB, complete
- ST and/or T wave abnormalities secondary to hypertrophy
What medications can caused myocardial dysfunction in cancer treatment (chemotherapy)?
- Anthracyclines (doxorubicin)
- Trastuzuamab (Herceptin)
-
Sunitinib (Sutent)
- tyrosine-kinase inhibitor
- used for renal cell and gastric stromal tumors
Which functions in the heart is tropinin necessary for?
Cardiomyocyte contraction and relaxation
- myosin, actin, tropomyosin and troponins are sarcomeric proteins that interact with calcium and ATP to produce myocardial contraction and relaxation
Which patient populations are aldosterone antagonists indicated for mortality benefit in HF patients?
post-MI patients
and
chronic HF (LVEF < 35%) + NYHA II-IV functional class
What are typical echocardiographic findings of HFpEF?
- concentric LV remodeling
- diastolic dysfunction
- LA dilatation
- PH