Cardiomyopathy (Dilated, Restrictive, ARVC, Stress) Flashcards
What is Dilated Cardiomyopathy (DCM)?
A condition characterized by dilation and impaired systolic function of one or both ventricles, often resulting in heart failure. The ejection fraction is less than 40% due to dilation of the left ventricle. Either the left or the right ventricle can be dilated.
What are the etiologies of Dilated Cardiomyopathy (DCM)?
Ischemia, genetic mutations, alcohol, cocaine, viral myocarditis, peripartum (usually the end of pregnancy), chemotherapy (e.g., doxorubicin), and tachycardia-induced cardiomyopathy.
How is dilated cardiomyopathy diagnosed?
TTE
At what stages of pregnancy are patients most at risk for developing dilated cardiomyopathy?
Third trimester. Sometimes after childbirth.
What is the advice to patients of childbearing who have a history of dilated cardiomyopathy?
Avoid pregnancy with contraceptives until 6 months and the ejection fraction is above 50%.
What breast cancer medication is known to cause cardiomyopathy?
Trastuzumab is a monoclonal antibody used in the treatment of HER2-positive breast cancer. This is reversible.
Is cardiomyopathy secondary to doxorubicin reversible or irreversible?
irreversible
What medication serves to prevent or mitigate cardiomyopathy secondary to doxorubicin?
Dexrazoxane (iron chelator) prevents anthracycline-induced cardiotoxicity
What arrhythmia is associated with the development of dilated cardiomyopathy?
Chronic atrial fibrillation can lead to tachycardia mediated cardiomyopathy.
Other than viral, what infections can lead to dilated cardiomyopathy?
Lyme disease and Chagas.
What are the clinical features of Dilated Cardiomyopathy (DCM)?
Symptoms of heart failure (dyspnea, fatigue, orthopnea), arrhythmias, and thromboembolic events.
What are the characteristic ECG findings in Dilated Cardiomyopathy (DCM)?
Non-specific ST-T wave changes, left bundle branch block (LBBB), or atrial fibrillation.
What imaging findings are seen in Dilated Cardiomyopathy (DCM)?
Echocardiogram: Dilated ventricles with reduced ejection fraction.
MRI: Late gadolinium enhancement in non-ischemic patterns.
What is the management of Dilated Cardiomyopathy (DCM)?
HFrEF management with ACE inhibitors, beta-blockers, diuretics, aldosterone antagonists, anticoagulation if atrial fibrillation or thrombi present. Consider ICD for severe cases.
What is Hypertrophic Cardiomyopathy (HCM)?
A genetic condition characterized by asymmetric left ventricular hypertrophy, often involving the interventricular septum, leading to diastolic dysfunction.
What are the etiologies of Hypertrophic Cardiomyopathy (HCM)?
Autosomal dominant mutations in sarcomere proteins (e.g., MYH7, MYBPC3); associated with sudden cardiac death in young athletes.
What are the clinical features of Hypertrophic Cardiomyopathy (HCM)?
Dyspnea, chest pain, syncope, palpitations; increased risk of sudden cardiac death during exertion.
What are the characteristic ECG findings in Hypertrophic Cardiomyopathy (HCM)?
Left ventricular hypertrophy (LVH), deep narrow Q waves in lateral leads, or T-wave inversions.
What imaging findings are seen in Hypertrophic Cardiomyopathy (HCM)?
Echocardiogram: Asymmetric septal hypertrophy. MRI: Myocardial fibrosis (late gadolinium enhancement).
What is the management of Hypertrophic Cardiomyopathy (HCM)?
Beta-blockers, calcium channel blockers (e.g., verapamil) for symptom relief. Avoid dehydration. ICD for high-risk patients.
What is Restrictive Cardiomyopathy (RCM)?
A group of disorders characterized by impaired ventricular filling due to reduced myocardial compliance, often resulting in diastolic heart failure (HFpEF).
What are the etiologies of Restrictive Cardiomyopathy (RCM)?
Infiltrative diseases (e.g., amyloidosis, sarcoidosis), storage diseases (e.g., hemochromatosis), or Fabry disease.
What is the type of restrictive cardiomyopathy associated with this type of white blood cell?
Eosinophilic myocarditis.
What condition causes diffuse thickening of the endocardium due to excessive fibroelastic tissue deposition in infants?
Endocardial fibroelastosis
What are the clinical features seen with all Restrictive Cardiomyopathies (RCM)?
Fatigue, dyspnea, right-sided heart failure symptoms (e.g., ascites, peripheral edema); preserved systolic function.
What symptoms are most prominent in patients with restrictive cardiomyopathy?
right sided heart failure symptoms.
How is restrictive cardiomyopathy diagnosed?
TTE
What is unique about the cardiomyopathy associated with hemochromatosis?
This can lead to both restrictive and dilated cardiomyopathy.
What is the most common form of restrictive cardiomyopathy?
The most common form of restrictive cardiomyopathy (RCM) is amyloidosis.
What is subtype of heart failure in patients with restrictive cardiomyopathy due to amyloidosis?
Amyloidosis causes a specific type of restrictive cardiomyopathy caused by amyloid protein deposition in the myocardium, leading to stiffness and reduced compliance and HFpEF.
What characteristic finding is seen on ECHO with amyloidosis leading to restrictive cardiomyopathy?
The echocardiogram will show a thickened myocardium with a ‘speckled’ appearance. Also seen on cardiac MRI will be a diffuse late gadolinium enhancement.
What is observed on EKG with amyloidosis induced restrictive cardiomyopathy?
Low voltage ECG, and ventricular wall thickening on echocardiogram.
What atrial changes are seen on ECHO with restrictive cardiomyopathy?
Bilateral atrial enlargement, normal or reduced ventricular size and a restrictive filling pattern.
What are the characteristic ECG findings with most restrictive cardiomyopathies?
Atrial fibrillation due to bilateral atrial enlargement, or nonspecific ST-T wave changes.
Which two restrictive cardiomyopathies are associated with Low voltage QRS complexes?
1) Amyloidosis: The deposition of amyloid fibrils within the myocardial tissue leads to electrical dissociation, meaning the thickened myocardium does not generate high voltage signals, resulting in a paradoxically low QRS voltage despite ventricular wall thickening seen on echocardiography. Also seen are pseudoinfarction patterns (Q waves), and conduction abnormalities (e.g., AV block).
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2) Sarcoidosis (sometimes): If extensive fibrosis infiltrates the myocardium, it can cause conduction abnormalities and occasionally low voltage.
What is the management of Restrictive Cardiomyopathy (RCM)?
Use the treatment approach for HFpEF (diuretics for volume overload and SGLT-2 inhibitors), treat underlying cause (e.g., amyloidosis, hemochromatosis), or heart transplantation in severe cases.
What are the etiologies of Restrictive Cardiomyopathy due to Amyloidosis?
Caused by systemic amyloidosis (e.g., AL amyloidosis, transthyretin [ATTR] amyloidosis).
What is the management of Restrictive Cardiomyopathy due to Amyloidosis?
Treat underlying amyloidosis (e.g., chemotherapy for AL amyloidosis or tafamidis for ATTR amyloidosis).
Tafamidis inding to the thyroxine-binding sites of transthyretin (TTR) in the serum and work by stabilizing the tetrameric structure of TTR, preventing its dissociation into monomers. With treatment they work by reducing amyloid fibril deposition, which is responsible for organ damage in ATTR amyloidosis. Tafamidis is the only FDA-approved therapy for Transthyretin Amyloid Cardiomyopathy.
What is Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
A genetic cardiomyopathy affecting the right ventricle, characterized by fibrofatty replacement of the myocardium and risk of arrhythmias.
What are the etiologies of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
Mutations in desmosomal proteins (e.g., plakoglobin, desmoplakin), typically inherited in an autosomal dominant pattern.
What are the clinical features of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
Palpitations, syncope, ventricular tachycardia, and risk of sudden cardiac death; right-sided heart failure in advanced cases.
What are the characteristic ECG findings in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
T-wave inversions in right precordial leads (V1-V3), epsilon waves, or ventricular arrhythmias.
What imaging findings are seen in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
Echocardiogram: RV dilation, reduced function, and regional wall motion abnormalities. MRI: Fibrofatty infiltration.
What is the management of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
Implantable cardioverter-defibrillator (ICD) for arrhythmia prevention, beta-blockers, and antiarrhythmic drugs (e.g., sotalol).
What is Stress-Induced (Takotsubo) Cardiomyopathy?
A transient form of cardiomyopathy triggered by acute stress, often presenting with apical ballooning on imaging and reversible left ventricular dysfunction.
What are the risk factors for Stress-Induced (Takotsubo) Cardiomyopathy?
Emotional or physical stress, hypoglycemia, catecholamine surge, or postmenopausal status.
Stress-Induced (Takotsubo) Cardiomyopathy affects the right or left heart?
Stress-induced (takotsubo) cardiomyopathy, which most commonly affects middle-aged or older women and believed to result from a catecholamine surge brought on by acute physical or emotional stress (eg, traumatic event, illness of a loved one), results in left ventricular dysfunction due to microvascular spasms with consequent ischemia and myocardial stunning that leading to impaired contraction. This condition characteristically has segmental, mid and apical hypokinesis and basilar hyperkinesis. These wall motion abnormalities create a distinctive balloon shape on echocardiography that resembles an octopus trap (takotsubo means “octopus trap” in Japanese). Stress-induced cardiomyopathy typically presents with chest pain that can mimic myocardial infarction. Some patients also have signs and symptoms of decompensated heart failure. ECG commonly shows evidence of ischemia (eg, ST-segment elevation, T-wave inversions) in the anterior precordial leads, and QTc prolongation may also be present. Serum troponin levels can be moderately elevated; however, coronary angiography reveals an absence of obstructive coronary artery disease. Treatment of stress-induced cardiomyopathy is supportive, and the condition usually resolves on its own within several weeks.
What are the clinical features of Stress-Induced (Takotsubo) Cardiomyopathy?
Acute chest pain, dyspnea, ST-elevation on ECG mimicking myocardial infarction; no obstructive coronary disease.
What are the characteristic ECG findings in Stress-Induced (Takotsubo) Cardiomyopathy?
ST-elevation in anterior leads, prolonged QT interval; resolves as function improves.
What imaging findings are seen in Stress-Induced (Takotsubo) Cardiomyopathy?
Echocardiogram: Apical ballooning and ventricular dyskinesia, with normal coronary arteries.
MRI: No late gadolinium enhancement.
What is the management of Stress-Induced (Takotsubo) Cardiomyopathy?
Supportive care with beta-blockers, ACE inhibitors, and resolution of stressors. Typically resolves within weeks.
