Cardiomyopathy and Cardiac Tumors Flashcards

1
Q

What is the most common form of cardiomoypathy? What type of dysfunction does it result in?

A

DILATED CARDIOMYOPATHY

Results in SYSTOLIC DYSFUNCTION after failed compensatory eccentric hypertrophy

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2
Q

What are the 3 complications of DILATED CARDIOMYOPATHY?

A
  1. BIVENTRICULAR CHF - Due to systolic dysfunction
  2. MITRAL/TRICUSPID REGURG - Due to stretching of the chambers and associated valves
  3. ARRHYTHMIA - Due to stretching of the conduction system
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3
Q

What is the most common cause of DILATED CARDIOMYOPATHY? What are some other causes (ABCCCDH)

A

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

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4
Q

Which cardiomyopathy tends to present peri-partumly (Last month of pregnancy - first 6mo after childbirth)?

A

DCM

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5
Q

What is the inheritance pattern of the genetic mutation resulting in DCM?

A

AD

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6
Q

What is the most common cause of HCM?

A

FAMILIAL - AD Mutation of beta-myosin heavy chain protein of SARCOMERE

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7
Q

What is the most common form of cardiomoypathy? What type of dysfunction does it result in?

A

DILATED CARDIOMYOPATHY

Results in SYSTOLIC DYSFUNCTION after failed compensatory eccentric hypertrophy

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8
Q

What are the 3 complications of DILATED CARDIOMYOPATHY?

A
  1. BIVENTRICULAR CHF - Due to systolic dysfunction
  2. MITRAL/TRICUSPID REGURG - Due to stretching of the chambers and associated valves
  3. ARRHYTHMIA - Due to stretching of the conduction system
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9
Q

What is the most common cause of DILATED CARDIOMYOPATHY? What are some other causes (ABCCCDH)

A

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

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10
Q

Which cardiomyopathy tends to present peri-partumly (Last month of pregnancy - first 6mo after childbirth)?

A

DCM

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11
Q

What is the inheritance pattern of the genetic mutation resulting in DCM?

A

AD

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12
Q

What is the most common cause of HCM?

A

FAMILIAL - AD Mutation of beta-myosin heavy chain protein of SARCOMERE

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13
Q

What physical exam finding do you hear with DCM? What do you see on Echo? What do you see on CXR?

A

PE: S3
ECHO: Dilated heart
CXR: Balloon appearance

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14
Q

What are 2 feared complications of HCM?

A

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

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15
Q

Which 2 valvular pathologies can HCM cause/mimic? BOTH SYSTOLIC MURMURS

A

**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)

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16
Q

What is the classic finding of HCM on biopsy?

A

DISORGANIZED MYOFIBER ARRAY

17
Q

Which neurological disorder is commonly associated with HCM?

A

FRIEDREICH ATAXIA

18
Q

What is RESTRICTIVE CARDIOMYOPATHY? Which dysfunction ensues?

A

Decreased compliance of ventricular endomyocardium -> Restricts DIASTOLIC FILLING -> DIASTOLIC DYSFUNCTION

19
Q

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?

A
  1. SYSTEMIC - SARCOIDOSIS (granuloma deposits in heart) + AMYLOIDOSIS (amyloid deposits in heart)
  2. ENDOCARDIAL FIBROELASTOSIS - Fibrosis of ENDOcardium (children)
  3. LOEFFLER SYNDROME - Fibrosis of ENDO/MYOcardium (adults)
20
Q

Which immune cell infiltrate characterizes LOEFFLER SYNDROME, as a cause of RESTRICTIVE CARDIOMYOPATHY?

A

EOSINOPHILS

21
Q

What is the classic EKG finding of RCM? Why?

A

LOW-VOLTAGE EKG + Diminished QRS due to amyloid or granuloma deposits/fibrosis

22
Q

What is the most common primary cardiac tumor in ADULTS?

A

MYXOMA = BENIGN MESENCHYMAL tumor with gelatinous appearance

23
Q

What is the classic histology appearance of a MYXOMA?

A

Abundant ground substance = Gelatinous appearance

24
Q

Where is the classic location of a MYXOMA? What valvular pathology can it mimic?

A

MYXOMA - Classically arises as a pedunculated mass in LEFT ATRIUM -> Obstructs the mitral valve

Mimics MITRAL STENOSIS

25
Q

What is the most common primary tumor in CHILDREN? What is it classically associated with?

A

RHABDOMYOMA - Benign HAMARTOMA of cardiac muscle

TUBEROUS SCLEROSIS

26
Q

Where is the classic location of a RHABDOMYOMA?

A

VENTRICLE

27
Q

What are the most common origins of metastatic cardiac tumors?

A

LUNG CARCINOMA
BREAST CARCINOMA
MELANOMA
LYMPHOMA

28
Q

Where is the classic location of a metastatic cardiac tumor? What is the most common clinical manifestation of this tumor?

A

PERICARDIUM

Results in PERICARDIAL EFFUSION

29
Q

What are the 2 classical characteristics of HCM? (Think: 1. Where does it preferentially hypertrophy? and 2. Leaflets of valves)

A
  1. ASYMMETRIC SEPTAL HYPERTROPHY

2. SYSTOLIC ANTERIOR MOTION OF MITRAL VALVE