Hypertensive and Heart related disorders

Question 1

The major cardiac complications of HTN result from what?

Answer 1

1. Pressure changes2. Ventricular Hypertrophy

Question 2

What are the criteria for diagnosing systemic hypertensive heart disease?

Answer 2

1. Left ventricular hypertrophy without any other CV pathology. AND 2. History or pathologic evidence of HTN

Question 3

Can mild hypertension (140/90) cause systemic hypertensive heart disease?

Answer 3

Yes

Question 4

What percentage of the U.S. population suffers from at least mild hypertension?

Answer 4

25%.

Question 5

Describe the morphology of systemic hypertensive heart disease.

Answer 5

1. Systemic HTN causes pressure overload on the heart, which will result in left ventricular hypertrophy. 2. As a result of increased left ventricular wall thickness, compliance decreases and diastolic filling will decrease. This can cause left atrial dilation. 3. Long standing systemic HTN will eventually cause left ventricular dilation too (in addition to already present hypertrophy).

Question 6

Is hypertensive heart disease symptomatic?

Answer 6

Usually not, but it is suspected by evidence of HTN, on EKG, or by doing an echocardiogram (which would show left ventricular hypertrophy). As disease progresses, atrial fibrillation (secondary to left atrial enlargement) and/or CHF will develop. KNOW features of progression.

Question 7

What are the 4 major types of hypertrophic heart disease?

Answer 7

1. Hypertensive heart disease 2. Hypertrophic cardiomyopathy 3. Aortic stenosis 4. Cor pulmonale

Question 8

Which is most common: Hypertensive heart disease, hypertrophic cardiomyopathy, aortic stenosis, or cor pulmonale?

Answer 8

Hypertensive heart disease > Aortic stenosis > hypertrophic cardiomyopathy. Cor pulmonale statistics unknown.

Question 9

T/F. All 4 types of hypertrophic heart disease present with dyspnea, angina, or sudden death?

Answer 9

False. Cor pulmonale does not present with angina

Question 10

T/F. All 4 types of hypertrophic heart disease are chronic conditions?

Answer 10

False. Cor pulmonale can be acute or chronic

Question 11

Epidemiology. Which of the 4 hypertrophic heart disease types presents in young patients?

Answer 11

ANS: Hypertrophic Obstructive Cardiomyopathy. Hypertensive heart disease, aortic stenosis, and chronic cor pulmonale present in OLDIES.

Question 12

Epidemiology. Describe how sex relates to 4 hypertrophic heart diseases?

Answer 12

Hypertensive Heart disease: Male=Female
Hypertrophic Cardiomyopathy: Male = Female
Aortic Stenosis: MALES!
Chronic cor pulmonale: Males (?)

Question 13

Epidemiology. Which of the 4 hypertrophic heart diseases is race related?

Answer 13

ANS: Hypertensive heart disease more common in blacks than whites. Other 3 types are not race specific

Question 14

Which of the 4 hypertrophic heart diseases does not cause left ventricular hypertrophy?

Answer 14

ANS: Chronic cor pulmonale.

Question 15

Which of the 4 hypertrophic heart diseases causes a murmur to develop?

Answer 15

Aortic Stenosis - YES
Hypertrophic cardiomyopathy - Sometimes
Hypertensive heart disease, Chronic for pulmonale - NO

Question 16

What is the thickness of a normal left ventricular wall?

Answer 16

Usually 1.2 to 1.4 cm. Hypertrophy can cause left ventricular wall to exceed 2 cm.

Question 17

Describe cardiac myocytes in hypertrophy?

Answer 17

Cardiac myocytes have expanded cytoplasm and enlarged nuclei. Nuclei are sometimes compared to boxcars

Question 18

Some patients with hypertrophic heart disease develop myocardial fibrosis. Name major contributing cytokine to this

Answer 18

TGF-Beta

Question 19

Describe why myocardialfibrosis is bad? Where does fibrosis develop (intracellularly, interstitially, etc)?

Answer 19

Fibrosis (which develops in the INTERSTITUM of the heart) will lead to increased wall thickness and stiffness, i.e.decreased compliance of the left ventricle. Decreased compliance causes blood to back up in atrium, causing atrial dilation. Atrial dilation eventually causes ventricular dilation. Again. Know progression.

Question 20

What is the relationship between kidney disease and hypertensive heart disease?

Answer 20

Kidney diseases causes HTN (and vice versa). Therefore, patients with chronic renal failure are highly prone to developing hypertensive heart disease.

Question 21

What exactly is hypertrophic cardiomyopathy?

Answer 21

Group of genetic conditions that is associated myocardial hypertrophy, defective diastolic filling, and ventricular outflow obstruction (in 1/3 of cases)

Question 22

Is systolic or diastolic function affected by hypertrophic cardiomyopathy?

Answer 22

Systolic function usually remains preserved. Diastolic dysfunction occurs in that there is HYPERcontraction.

Question 23

What is hypertrophic obstructive cardiomyopathy (HCOM)?

Answer 23

Condition that affects1/3 of patients with hypertrophic cardiomyopathy. Patients will haveasymmetric hypertrophy of upper septum, which will bulge out and block outflow tract.

Question 24

GENETICS:Name mode of inheritence of Hypertrophic cardiomyopathy?

Answer 24

Autosomal dominant with variable penetrance

Question 25

GENETICS: Mutations in which gene are most common in Hypertrophic cardiomyopathy (HCM)?

Answer 25

There are more than 400 different known mutations in 9 different genes, most being missense mutations. The most common mutation occurs in Beta-myosin heavy chains, then genes coding for cardiac Troponin T, alpha-tropomyosin, and myosin binding protein C. These 4 mentioned account for roughly 80% of all cases. 

Question 26

Morphology of hypertrophic cardiomyopathy

Answer 26

Massive myocardial hypertrophy, usually without ventricular dilation. Classically, have much thicker left ventricular septum than left ventricular free wall (ratio of 3:1 in size). Can have "banana like configuration" due to bulging of septum into left ventricle

Question 27

Describe what happens with mitral leaflets in hypertrophic cardiomyopathy?

Answer 27

Septal hypertrophy causes mitral leaflets to hit at body, rather than at the tips. Part of anterior leaflet sticking out is carried anteriorly and superiorly, due to blood flow hitting it, causing subaortic obstruction and eventual mitral regurgitation.  Fibrosis will eventually occur as a result of chronic damage and attempt to repair (Detail on another slide).

Question 28

Treatment for hypertrophic cardiomyopathy?

Answer 28

Besides open heart surgery, inject ethanol into a septal coronary artery and infarct the inner part of the hypertrophied upper septum

Question 29

Why is fibrosis seen in hypertrophic cardiomyopathy?

Answer 29

Contact between anterior leaflet of mitral valve and bulging septal wall causes a repair response, seen as fibrosis in both mitral valve and subaortic upper septum

Question 30

Valvular disease results in what 2 common occurrences?

Answer 30

1. Stenosis - failure of a valve to open completely, obstructing forward flow 2. Insuffiency - failure of a valve to close completely, thereby allowing regurgitation (backflow) of blood

Question 31

Aortic stenosis causes calcifications to occur where?

Answer 31

In the sinuses of Valsalva (the pockets formed between the cusps of the aortic valce and the aorta around them)

Question 32

Pathophysiology of Degenerative calcific aortic stenosis?

Answer 32

In elderly patients with degenerative calcific aortic stenosis, the formation of rocks in the sinsuses of Valsalva has gradually stenosed the valve orifice 

Question 33

Pathophysiology of Rheumatic aortic stenosis?

Answer 33

In aortic stenosis due to Rheumatic valvulitis, inflammation and fibrosis start at the commissure (the point where the valve opening touches the valve annulus) and it moves inward from there

Question 34

Pathophysiology of Bicuspid aortic stenosis?

Answer 34

In young and middle aged patients born with a bicuspid valve (1-2% of the US population) due to fusion of two cusps, aortic stenosis develops more rapidly

Question 35

Cor pulmonale consists of what two common features in the right ventricle?

Answer 35

right ventricular hypertrophy and dilation, frequently accompanied by right heart failure.

Question 36

Primary cause of cor pulmonale?

Answer 36

Pulmonary HTN from pulmonary disorders of lung parenchyma or pumonary vasculature

Question 37

Acute cor pulmonale is most commonly caused by? Do you see hypertrophy, dilation, or both?

Answer 37

Acute cor pulmonale most commonly caused by a pulmonary embolism. Usually only see dilation of the right ventricle. If an embolism causes sudden death, you often won't see dilation either. 

Question 38

Chronic cor pulmonale common cause? What morphologic changes do you observe

Answer 38

Due to chronic lung and pumonary vascular related diseases. Will see right ventricular (and often right atrial) hypertrophy. Size can often be comparable to left ventricle

Question 39

What is often present in pulmonary arteries of patients with chronic cor pulmonale?

Answer 39

 Because chronic cor pulmonale occurs in the setting of pulmonary HTN, the pulmonary arteries often contain atheromatous plaques and other lesions, reflecting long-standing pressure elevations.

Question 40

Which is more common, right heart failure due to left heart failure or cor pulmonale?

Answer 40

Right heart failure is much more common due to left heart failure, not cor pulmonale. Most patients with cor pulmonale will compensate instead of develop right heart failure.