Cardiomyopathy and Cardiac Tumors Flashcards
What is the most common form of cardiomoypathy? What type of dysfunction does it result in?
DILATED CARDIOMYOPATHY
Results in SYSTOLIC DYSFUNCTION after failed compensatory eccentric hypertrophy
What are the 3 complications of DILATED CARDIOMYOPATHY?
- BIVENTRICULAR CHF - Due to systolic dysfunction
- MITRAL/TRICUSPID REGURG - Due to stretching of the chambers and associated valves
- ARRHYTHMIA - Due to stretching of the conduction system
What is the most common cause of DILATED CARDIOMYOPATHY? What are some other causes (ABCCCDH)
MOST COMMON CAUSE = Idiopathic
Other causes: ABCCCD
A- alcohol abuse**, B-Beriberi (wet) syndrome [thiamine deficiency]
C-chagas (from T. cruzi infection, also causing achalasia)
C- cocaine chronic usage
C- Coxsackie A/B viral myocarditis late complication
D-doxorubicin toxicity [generates free radicals, intercalates in DNA to create DNA breaks and decrease replication]
H- Hemochromatosis
Which cardiomyopathy tends to present peri-partumly (Last month of pregnancy - first 6mo after childbirth)?
DCM
What is the inheritance pattern of the genetic mutation resulting in DCM?
AD
What is the most common cause of HCM?
FAMILIAL - AD Mutation of beta-myosin heavy chain protein of SARCOMERE
What is the most common form of cardiomoypathy? What type of dysfunction does it result in?
DILATED CARDIOMYOPATHY
Results in SYSTOLIC DYSFUNCTION after failed compensatory eccentric hypertrophy
What are the 3 complications of DILATED CARDIOMYOPATHY?
- BIVENTRICULAR CHF - Due to systolic dysfunction
- MITRAL/TRICUSPID REGURG - Due to stretching of the chambers and associated valves
- ARRHYTHMIA - Due to stretching of the conduction system
What is the most common cause of DILATED CARDIOMYOPATHY? What are some other causes (ABCCCDH)
MOST COMMON CAUSE = Idiopathic
Other causes: ABCCCD
A- alcohol abuse**, B-Beriberi (wet) syndrome [thiamine deficiency]
C-chagas (from T. cruzi infection, also causing achalasia)
C- cocaine chronic usage
C- Coxsackie A/B viral myocarditis late complication
D-doxorubicin toxicity [generates free radicals, intercalates in DNA to create DNA breaks and decrease replication]
H- Hemochromatosis
Which cardiomyopathy tends to present peri-partumly (Last month of pregnancy - first 6mo after childbirth)?
DCM
What is the inheritance pattern of the genetic mutation resulting in DCM?
AD
What is the most common cause of HCM?
FAMILIAL - AD Mutation of beta-myosin heavy chain protein of SARCOMERE
What physical exam finding do you hear with DCM? What do you see on Echo? What do you see on CXR?
PE: S3
ECHO: Dilated heart
CXR: Balloon appearance
What are 2 feared complications of HCM?
1) SUDDEN DEATH - Due to ventricular arrhythmia = most common cause of sudden death in young athletes
2) DIASTOLIC DYSFUNCTION: Decrease in LV compliance -> Compensatory LV concentric hypertrophy -> Increase LVP -> Increase LAH -> Increase atrial systole (>20%) to maintain SV, EDV. When this benign compensatory mechanism fails, you get diastolic dysfunction
Which 2 valvular pathologies can HCM cause/mimic? BOTH SYSTOLIC MURMURS
**UWORLD 1) CAUSES MITRAL REGURG: Asymmetric septal hypertrophy -> Impaired mitral valve closure -> Mitral regurgitation
2) MIMICS AORTIC STENOSIS (HARSH SYSTOLIC EJECTION MURMUR best heard along lower left sternal border + apex): Hypertrophy of the ventricular septum below the aortic valve -> Decreased outflow into aorta -> EXERTIONAL SYNCOPE** + Angina + CHF
(Decreased TPR due to vasodilation of vascular beds during exercise, Decreased CO -> Decreased cerebral perfusion = SYNCOPE)
What is the classic finding of HCM on biopsy?
DISORGANIZED MYOFIBER ARRAY
Which neurological disorder is commonly associated with HCM?
FRIEDREICH ATAXIA
What is RESTRICTIVE CARDIOMYOPATHY? Which dysfunction ensues?
Decreased compliance of ventricular endomyocardium -> Restricts DIASTOLIC FILLING -> DIASTOLIC DYSFUNCTION
Which 2 systemic conditions can cause RESTRICTIVE CARDIOMYOPATHY? Which rare condition of massive fibrosis in CHILDREN causes RESTRICTIVE C.? Which rare condition of massive fibrosis in ADULTS cause RESTRICTIVE C?
- SYSTEMIC - SARCOIDOSIS (granuloma deposits in heart) + AMYLOIDOSIS (amyloid deposits in heart)
- ENDOCARDIAL FIBROELASTOSIS - Fibrosis of ENDOcardium (children)
- LOEFFLER SYNDROME - Fibrosis of ENDO/MYOcardium (adults)
Which immune cell infiltrate characterizes LOEFFLER SYNDROME, as a cause of RESTRICTIVE CARDIOMYOPATHY?
EOSINOPHILS
What is the classic EKG finding of RCM? Why?
LOW-VOLTAGE EKG + Diminished QRS due to amyloid or granuloma deposits/fibrosis
What is the most common primary cardiac tumor in ADULTS?
MYXOMA = BENIGN MESENCHYMAL tumor with gelatinous appearance
What is the classic histology appearance of a MYXOMA?
Abundant ground substance = Gelatinous appearance
Where is the classic location of a MYXOMA? What valvular pathology can it mimic?
MYXOMA - Classically arises as a pedunculated mass in LEFT ATRIUM -> Obstructs the mitral valve
Mimics MITRAL STENOSIS
