Clinical- Cardiomyopathies- Lunderg Flashcards
What are pathologies that bring on changes in heart structure leading to changes in heart function?
cardiomyopathies
(diseases of the myocardium associated with cardiac dysfunction)
What is the name of the cardiomyopathy also known as stress-induced cardiomyopathy or broken-heart syndrome?
Takotsubo cardiomyopathy
Cardiomyopathies caused by acquired, genetic, and/or a mix between the two are:
primary cardiomyopathies
Cardiomyopathies involved with autoimmune/inflammatory, endocrine, infectious, infiltrative disorders, neuromuscular and storage diseases, nutritional deficiencies, and toxic substances are primary/secondary cardiomyopathies?
secondary
Clinical presentation of cardiomyopathies are similar to what other pathology?
CHF
Peripheral edema • Fatigue • Orthopnea • Dyspnea on exertion • Paroxysmal nocturnal dyspnea • Presyncope • Syncope • Cardiac ischemia
What is the first step to a clinical evaluation?
history and physical
What labs would be important to order for suspected cardiomyopathy?
CBC (anemia or infection can worsen HF)
Electrolytes (including calcium and magnesium)
BUN/creatinine
Liver Function Ts (affected by hepatic congestion)
Fasting blood glucose (underlying diabetes mellitus)
Fasting lipid profile
TSH (hyperthyroidism or hypothyroidism can affect the heart)
Concerning clinical eval for suspected cardiomyopathy, which test could tell us about electrical activity of heart?
ECG
Concerning clinical eval for suspected cardiomyopathy, which test could tell us about heart chamber size (dilation, restriction, hypertrophy)?
echocardiogram
Concerning clinical eval for suspected cardiomyopathy, what are some other tests that would be clinically relevant other than • History and physical • Electrocardiography • Echocardiography?
Cardiac magnetic resonance imaging (CMRI or CMR) • Coronary angiography • Genetic testing (with genetic counseling)
Which cardiomyopathy exhibits left ventricular hypertrophy without chamber dilation?
HYPERTROPHIC CARDIOMYOPATHY (HCM)
What is the inheritance pattern for HYPERTROPHIC CARDIOMYOPATHY (HCM), and the gene mutations affect what?
Autosomal dominant mutations of genes coding for sarcomere protein
Which cardiomyopathy characteristically presents with
- atypical chest pain (may be associated with meals, dehydration, or exertion)
- sudden cardiac death (or family members with sudden unexplained death)
- Systolic murmur that increases in intensity with Valsalva maneuvers
- May feel systolic thrill
- Normal S1 and S2, but may hear S4
HCM
HCM can present with atypical chest pain that may be associated with which 3 conditons?
after meals (GERD like pain), dehydration, or exertion
The systolic murmur in HCM is a harsh heard all over the precordium but usually is not transmitted to the carotids.
crescendo-decrescendo
What cardiomyopathy is suspected with the following common ECG findings?
- Prominent abnormal Q waves
- P wave abnormalities
- Left atrial enlargement
- Left axis deviation
LVH
• Deeply inverted T waves in V2 - V4
HCM
• Deeply inverted T waves in V2 - V4 in apical variant HCM
What are the two ways of diagnosing HCM with Sokolow-Lyon criteria on ECG:
Sum of S wave in V1 and R wave in V5 or V6 ≥3.5 mV (35 mm)
R wave in aVL ≥1.1 mV (11 mm; or sometimes ≥1.3 mV [13 mm]
What would be echocardiography findings in the case of hypertrophic cadiomyopathy?
Echocardiography shows hypertrophy of left ventricle with reduction in ventricular chamber volume
What criteria is met in this ECG (in absence of HTN) concerning apical variant HCM?
Deeply inverted T waves in precordial leads and also T-wave abnormalities in the limb leads.
(APICAL HYPERTROPHIC CARDIOMYOPATHY suspected in the absence of HTN along with ECG findings)
What is being demonstrated in this echo as concerning HCM?
asymmetric septal hypertrophy (white arrow) compared to the inferolateral wall of the LV
What is being demonstrated in this MRI as concerning HCM?
LV septal and wall hypertrophy
What is being demonstrated in this cardiac magnetic resonance as concerning HCM? Also what are the small round circles?
severe asymmetric left ventricular (LV) hypertrophy involving the septum (S). Multiple papillary muscle heads are also noted in the LV cavity. RV, right ventricle.
What are the main goals of therapy for hypertrophic cardiomyopathy?
Decrease exertional dyspnea and chest pain
Prevent sudden cardiac death
What is the first line therapy for symptomatic HCM?
Beta Blockers
What therapy can be used for symptomatic HCM if B Blockers are not tolerated?
Nondihydropyridine calcium channel blockers (like verapamil)
What are the 4 surgical options for treatment of symptomatic HCM?
surgical septal myectomy,
alcohol septal ablation,
and dual-chamber pacing,
heart transplantation (severe systolic symptoms)
All patients with HCM need risk stratification for and evaluation for placement of an implantable cardioverter-defibrillator
SCD (sudden cardiac death)
What is the most common cause of SCD in athletes (1/3 of cases)?
HCM
What populations groups is SCD more prevalent?
male athletes, nonwhites
What are some of the risk factors for sudden cardiac death?
Family history of sudden cardiac death in first degree relative
LV wall thickness at least 30mm
Recent unexplained syncope
Prior cardiac arrest
Prior sustained VT
Non-sustained VT
or abnormal BP response
Therapy for HCM involves 3 main components for the best outcomes:
- Symptom control (Bblockers, CCB, diuresis)
- SCD risk stratification (possible ICD implantation)
- Genetic counseling and screening
What is the cardiomyopathy disease of desmosomal proteins characterized by fibrofatty infiltration of healthy myocardium - leads to thinning and ballooning of ventricular wall, typically RV?
ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C)
ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C) is less common of the cardiomyopathies, about the cases are familial, and patients commonly present with symptoms in what age group?
1/2; 30s
What are 2 common symptoms of ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C)?
paplitations
syncope
What are some ECG abnormalities with ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C)?
inverted T waves and epsilon waves in right precordial leads
As concerning arrhthmogenic right ventricular dysplasia/cardiomyopathy, what are some possible cardiac imaging RV abnormatlities?
aneurysms,
segmental dilation,
reduced ejection fraction
Similar to pts with HCM, those with ARVD/C, what type of sports should be avoided due to increased risk of sudden cardiac death?
Should avoid competitive and endurance sports
What a 4 treatment options aimed at reducing arrhythmia and preventing sudden death in ARVD/C?
Beta blockers, antiarrhythmic drugs
- Catheter ablation
- Implantable cardioverter-defibrillator
- Heart transplantation
What is the most common cardiomyopathy in age 40 - 59 (but can occur at any age)?
DCM
A diagnosis of DCM is the leading indication for which treatment option?
heart transplantation
Which cardiomyopathy is associated with Enlarged ventricles, normal LV wall thickness, systolic dysfunction, and decreased ejection fraction?
DCM
What are the 2 characteristic symptoms of DCM?
arrhythmias and thromboembolic events
With which testing modality is DCM diagnosed?
echocardiography
Treatment guidlines is similar for what condition for the treatment of DCM?
CHF
Which cardiomyopathy is characterized by nondilated ventricles with impaired ventricular filling, often with moderate to marked biatrial enlargement?
Restrictive cardiomyopathy
In what place is restrictive cardiomyopathy most common?
tropics (Africa, India, South and Central America
What are 3 common presenting symptoms of restrictive cardiomyopathy?
dyspnea,
peripheral edema (often hepatomegaly and ascites),
fatigue (from reduced cardiac output from reduced preload)
Chest radiography often shows vascular congestion and a normal cardiac silhouette
pulmonary
What are 2 possible ECG findings with restrictive cardiomyopathy?
diffuse reduced voltage or prolonged PR interval
In which cardiomyopathy may echocardiography reveal biatrial enlargement and diastolic dysfunction, although left ventricular diastolic volume, wall thickness, and systolic function typically appear normal?
restrictive cardiomyopathy
The treatment for restictive cardiomyopathy should be aimed at:
addressing underlying cause
What are symptomatic interventions addressing restrictive cardiomyopathy?
managing volume overload with diuretics
or aldosterone antagonists
Its important to evaluate pts. with restrictive cardiomyopathy for insertion?
AV block/pacemaker
Concerning peripartum cardiomyopathy, pts. present with LV systolic dysfunction in last trimester to months postpartum (most patients present in the first postpartum month).
6
Peripartum cardiomyopathy is associated with increasing , black race, preeclampsia, HTN, gestations and history of peripartum cardiomyopathy in previous pregnancy
age; multiple
Symptoms of peripartum cardiomyopathy are consistent with heart failure (fatigue, edema and dyspnea on exertion), but can be confused with more common pregnancy complications like (can delay diagnosis of cardiomyopathy)
preeclampsia
Concerning peripartum cardiomyopathy, Echocardiography commonly shows left ventricular , left ventricular dysfunction, and hypertension
dilation; systolic; pulmonary
As concerning peripartum cardiomyopthy• Treatment follows standard heart failure therapy, but must consider pregnancy, which pharmocological treatments should be avoided?
ACE inhibitors/ARBs should be avoided in pregnancy
In postpartum cardiomyopathy, what should you consider if treating with diuresis?
Avoid hypotension and reduced uterine perfusion
Takotsubo Cardiomyopathy (Broken-Heart Syndrome)
Which cardiomyopathy is also called stress-induced cardiomyopathy or broken-heart syndrome?
TAKOTSUBO CARDIOMYOPATHY
Which cardiomyopathy presents with Abrupt onset of LV dysfunction in response to severe emotional or physiologic stress?
TAKOTSUBO CARDIOMYOPATHY
Which population is most commonly affected by TAKOTSUBO CARDIOMYOPATHY?
postmenopausal women
The presentation of Takotsubo cardiomyopathy is most similar to what other condition?
acute coronary syndrome
Treatment for Takotsubo cardiomyopathy should be consistent with what other condition?
acute coronary syndrome
As concerning Takotsubo cardiomyopathy, what is indicated when a loss of wall motion is noted in the left ventricular apex?
anticoagulation
As concerning Takotsubo cardiomyopathy, treatments usually can be withdrawn concurrently with reversal of symptoms within month/months
one
What is the diagnosis:
A 21-year-old man is evaluated during a routine medical examination prior to employment. He voices no active complaints and has no medical problems and takes no medications or illicit drugs. On review of symptoms (ROS) he tells you that 9 months ago he experienced an episode of syncope after playing volleyball with some friends. He attributed the episode to dehydration and did not seek medical attention for it. He has no family history of cardiovascular disease but reports that his father died at the age of 40 years for unclear reasons.
Hypertrophic Cardiomyopathy
What is the diagnosis:
Hypertrophic cardiomyopathy
What is the diagnosis:
A 45-year-old male comes to the emergency department complaining of chest pain and dyspnea. He has also noticed swelling in his legs and feet. His chest radiograph shows pulmonary vascular congestion with a normal cardiac silhouette, his electrocardiogram shows diffuse reduced voltage without signs of cardiac ischemia, and his echocardiogram reveals bi-atrial enlargement and diastolic dysfunction.
restrictive cardiomyopathy (RCM
What is the diagnosis:
A 64-year-old female comes to the emergency department complaining of sharp sub-sternal chest pain that comes with exertion and is associated with diaphoresis and dyspnea. Her symptoms started soon after hearing that her children had all been killed in a car accident.
Takotsubo cardiomyopathy
What is the diagnosis:
A 24-year-old male comes to your office for his pre-participation sports physical. He gets short of breath while playing sports occasionally, with associated transient mild chest pain that he attributes to the intensity of his exertion. You hear a harsh crescendo-decrescendo systolic murmur at the lower left sternal border on exam.
hypertrophic cardiomyopathy (HCM)
What is the diagnosis:
A 38-year-old female who is 39-weeks pregnant comes to your office complaining of fatigue and dyspnea that is worse with any exertion. She has also noticed swelling in her feet and ankles that has been worsening over the last week.
peripartum cardiomyopathy
