Term
| What are the 3 functional classes of cardiomyopathy? |
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Definition
1) Dilated (MOST COMMON)
- Impaired contractility and systolic function with increased LV volume and NO HYPERTROPHY
**2 and 3 can become 1 following decompensation**
2) Hypertrophic
- LVH, preserved systolic function and diastolic dysfunction
3) Restrictive
- Stiffened/fibrotic myocardium leading to diastolic dysfunction |
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Term
| Why does the technical definition of "cardiomyopathy" exclude ischemic (CAD) and hypertensive causes of dilated cardiomyopathy? |
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Definition
| They are cardiac diseases, in themselves! |
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Term
A 45 year old patient presents with DOE, fatigue, weakness, PND and orthopnea.
They also are tachycardic, with leftward displaced diffuse MI, an S3 and a pan-systolic murmor.
On pathology, you see mural thrombi, myocyte degeneration/atrophy and interstitial/perivascular fibrosis.
What is the ddx? |
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Definition
This could be HF or Idiopathic Dilated Cardiomyopathy. Remember, IDC is diagnosis of exclusion!
20-60 with LV systolic dysfunction suggests Idiopathic dilated cardiomyopathy (IDC).
Pathology of IDC:
1) Enlarged soft heart with 4-chamber dilation and mural thrombi
2) Myocyte degeneration, atrophy.
3) Interstitial and perivascular fibrosis |
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Term
True or False:
Severe aortic stenosis can cause hypertrophic cardiomyopathy. |
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Definition
False!
The definition of HCM dictates that it must be "unexplained LVH associated with non-dilated ventricular chambers in the absense of another cardiac or systemic disease." |
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Term
A young athlete suddenly drops dead on the basketball court.
What is the pathophysiology and pathology of the condition that killed him? |
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Definition
familial HCM (50% AD and 50% new mutations)
1) Pathophysiology
- Decreased compliance causes increased LV/LA/Pulmonary pressures
- Rapid flow causes outflow obstruction, with venturi forces drawing the anterior leaflet of the MV toward the bulging upper septum (MR and A fib)
2) Pathology
- Wall thickening and asymmetric hypertrophy of upper septum.
- Microscopic myocyte disarray, hypertrophy and interstitial replacement fibrosis |
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Term
| Why might you hear a murmor of mitral regurgitation along with an absent "A" wave in HCM? |
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Definition
Rapid flow causes outflow obstruction that, via venturri forces, pulls the anterior leafet of the MV toward the bulging upper septum.
When this happens, you can get mitral regurgitation and A fib. |
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Term
A young patient presents with dyspnea, angine, syncope and lightheadedness on standing.
Upon ausculation, S4 is present and you ear a crescendo-decrescendo systolic murmur at the LSB. You also feel a double apical impulse.
How do you treat? |
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Definition
This is HCM
Treat the symptoms and prevent SCD.
Beta blockers, non-dihyropyridine calcium channel blockers or other arrythmic drugs. |
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Term
| What is the diagnosis/treatment for Cardiac amyloidosis |
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Definition
This is rare and is an example of restrictive cardiomyopathy. The issue is that the myocardium is stiffened (+/- hypertrophy).
Can lead to HF, heart block and arrhythmia
- Diagnose with biopsy and then TRANSPLANT (make sure to get rid of myeloma or liver producing protein is dealt with!). |
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Term
A young African American patient presents with fever, malaise, fatigue and dyspnea. On X-ray you notice bilateral pulmonary hilar lymphadenopathy.
What would you expect to see on pathology? How could you treat? |
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Definition
Age, race and symptoms suggest Cardiac Sarcoidosis.
Pathology
1) Noncaseating granulomas in lungs, LN, spleen, skin, eyes and heart).
Treat
2) Immunosuppression and Steroid |
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Term
What is the pathology/signs and symptoms/complications/diagnosis and treatment for a
cardiac myxoma? |
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Definition
Benign gelatenous mesenchymal neoplasm of endocardium. Most common primary tumor or heart.
1) Pathology
- 90% in atria (72% in LA)
- Mucoid matrix with scattered cells.
2) S & S
- Dyspnea, cough, transient neuro, fatigue and fever
- Loud S1, diastolic rumble, holosystolic murmor (may look like mitral stenosis).
3) Complications
- Intermittent mitral obstruction, embolization, MI and SCD
4) Diagnosis/Treat
- Echo, CT or MRI
- Surgical excision |
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Term
What is the pathology/signs and symptoms/complications/diagnosis and treatment for a
cardiac sarcoidosis? |
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Definition
Multi-organ autoimmune inflammatory disease common in AA.
1) Pathology
- Noncaseating granulomas in lungs, LN, liver, spleen, skin, eyes and heart.
2) S & S
- Young african americans with arrhythmias, fever, malaise, fatigue, weight loss and dyspnea.
3) Complications
- Syncope and SCD from arrhythmias
4) Diagnosis/Treat
- Clinical/radiology/histological
- Steroids & immunosuppression |
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Term
| How can you approach an accurate diagnosis for a patient who presents with HF (e.g. what do you ask?)? |
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Definition
1) What is LV systolic function (EF)?/ get Echo
2a) IF LV EF is reduced, is it due to CAD?
2b) Provided CAD is excluded, what other clues do you have? |
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Term
Which Cardiomyopathy has each of the following features?
1) Unexplained hypertrophy due to genetic diseases of contractile proteins
2) Abnormal protein deposition; Congo red stain positive
3) Noncaseating granulomas involving the base of the heart and the conduction system
4) Dilated LV with no evident cause |
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Definition
1) HCM (diastolic)
2) Amyloid CM (restrictive) (diastolic)
3) Sarcoid (other)
4) Idiopathic Dilated CM (systolic) |
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Term
How do you treat each of the following cardiomyopathies?
1) IDC
2) HCM
3) Amyloid
3) Sarcoid |
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Definition
1) Heart failure drugs; Transplant
2) NO competitive sports; medications and if refractory, surgical myectomy or alcohol septal ablation.
3) Transplant
4) Steroids + Immunosuppression |
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