Cardiomyopathy and Cardiac tumors

Question 1

What are cardiomyopathies?

Answer 1

Cardiomyopathy is a chronic disease of the heart muscle (myocardium), in which the muscle is abnormally enlarged, thickened, and/or stiffened. The weakened heart muscle loses the ability to pump blood effectively, resulting in irregular heartbeats (arrhythmias) and possibly even heart failure.

Question 2

What is dilated cardiomyopathy?

Answer 2

Dilation of all four chambers of the heart; most common form of cardiomyopathy

Question 3

What are the clinical features of dilated cardiomyopathy?

Answer 3

Results in systolic dysfunction (ventricles cannot pump), leading to biventricularc CHF; complications include mitral and tricuspid valve regurgitation and arrhythmia.

Question 4

What is the most common cause of dilated cardiomyopath?

Answer 4

Most commonly idiopathic

Question 5

What are other causes of dilated cardiomyopathy besides idopathic?

Answer 5

1. Genetic mutation (usually autosomal dominant)
2. Myocarditis (usually due to coxsackie A or B) characterized by a lymphocytic infiltrate in the myocardium; results in chest pain, arrhythmia with
sudden death, or heart failure. Dilated cardiomyopathy is a late complication.
3. Alcohol abuse
4. Drugs (e.g., doxorubicin)
5. Pregnancy—seen during late pregnancy or soon (weeks to months) after childbirth

Question 6

What is the treatment in dilated cardiomyopathy?

Answer 6

Treatment is heart transplant.

Question 7

What is hypertrophic myocardiopathy?

Answer 7

Massive hypertrophy of the left ventricle

Question 8

What is the usual cause of hypertrophic cardiomyopathy?

Answer 8

Usually due to genetic mutations in sarcomere proteins; most common form is autosomal dominant,

Question 9

.What are the clinical features in hypertrophic cardiomyopathy?

Answer 9

1. Decreased cardiac output—Left ventricular hypertrophy leads to diastolic dysfunction (ventricle cannot fill).
2. Sudden death due to ventricular arrhythmias; hypertrophic cardiomyopathy is a common cause of sudden death in young athletes.
3. Syncope with exercise—Subaortic hypertrophy of the ventricular septum resultsin functional aortic stenosis

Question 10

What can we find in hypertrophic cardiomyopathy biopsy?

Answer 10

Biopsy shows myofiber hypertrophy with disarray

Question 11

What is Restrictive Cardiomyopathy?

Answer 11

Decreased compliance of the ventricular endomyocardium that restricts filling during diastole

Question 12

What are the causes of Restrictive Cardiomyopathy?

Answer 12

Causes include amyloidosis, sarcoidosis, hemochromatosis, endocardial fibroelastosis (children), and Loefller syndrome (endomyocardial fibrosis with an eosinophilic infiltrate and eosinophilia).

Question 13

What are the EKG findings in restrictive cardiomyopathy?

Answer 13

Presents as congestive heart failure; classic finding is low-voltage EKG with diminished QRS amplitude.

Question 14

What is a Myxoma?

Answer 14

Benign mesenchymal tumor with a gelatinous appearance and abundant ground substance on histology

Question 15

What is the most common primary cardiac tumor in adults?

Answer 15

Myxoma

Question 16

How does Myxoma usually prensent itself?

Answer 16

Usually forms a pedunculated mass in the left atrium that causes syncope due to obstruction of the mitral valve

Question 17

What is Rhabdomyoma?

Answer 17

A benign tumor derived from striated muscle; the cardiac form is considered to be a hamartoma and is often associated with tuberous sclerosis.

Question 18

How common is Rhabdomyoma?

Answer 18

Most common primary cardiac tumor in children

Question 19

What structure does Rhabdomyoma usually affect?

Answer 19

Usually arises in the ventricle

Question 20

What is the common metastases to the heart?

Answer 20

Common metastases to the heart include breast and lung carcinoma, melanoma, and lymphoma

Question 21

What structure does Metastasis of the heart usually affect?