Hyperertensive and Related Heart Disease Flashcards
Name the 4 types of hypertrophic heart disease.
1) Hypertensive heart disease
2) Hypertrophic cardiomyopathy
3) Aortic stenosis
4) Cor pulmonale
How common are hypertrophic heart diseases?
very common
Most hypertrophic heart diseases present with what symptoms? What is the exception?
dyspnea, angina or sudden death
Exception: no angina with cor pulmonale
What is the only exception to the rule that hypertrophic heart diseases are chronic?
cor pulmonale can be acute or chronic
What is the only hypertrophic heart disease that is more common in young people than the elderly?
hypertrophic cardiomyopathy
What is the only hypertrophic heart disease that is more common in blacks than whites?
hypertensive heart disease
60% of blacks, 30% of whites with hypertension in US [40% of blacks, 25% of whites have hypertension in US]
What is the only hypertrophic heart disease that does NOT present with left ventricular hypertrophy?
chronic cor pulmonale
In which hypertrophic heart disease do you ALWAYS hear a murmur? In which one might you sometimes hear a murmur?
Always hear a murmur in aortic stenosis
Sometimes hear a murmur in hypertrophic cardiomyopathy
What is the hypertrophic heart disease that requires surgery (stenting)?
aortic stenosis
Hypertrophied cardiac myocytes have what features?
expanded cytoplasm and enlarged nuclei (boxcars)
What drives the progressive myocardial
fibrosis seen in some patients with hypertrophic heart disease?
cytokine response (TGF-beta)
People in what kind of organ failure are highly prone to getting hypertensive heart disease?
chronic renal failure
The myocardial fibrosis in hypertensive heart disease and other hypertrophic heart diseases tends to be where?
INTERSTITIAL
True or false: Hypertrophic cardiomyopathy (the group of genetic conditions) is associated with symmetric hypertrophy of the septum.
FALSE: associated with Asymmetric hypertrophy of the septum
Why do 1/3 of patients with hypertrophic cardiomyopathy have obstruction of outflow from the left ventricle? How can you tell?
asymmetrically hypertrophied upper septum bulges into the outflow tract
If you see endocardial plaque in the left ventricle outflow track and a thickened anterior mitral leaflet
The subset of hypertrophic cardiomyopathy that involves obstructed left ventricular outflow is called what?
hypertrophic obstructive cardiomyopathy (HCOM)
Does every patient with HCM have HCOM?
NO! only 1/3
Which mitral leaflet causes the problems in HCOM? What does it do?
anterior mitral leaflet swings in and “hits” the hypertrophied, bulging septum and causes a subaortic obstruction
The motion of the anterior mitral leaflet in HCOM has what effect on the mitral valve?
results in a failure of coaptation of the mitral leaflets and mitral regurgitation
Chronic hitting of the anterior mitral leaflet and the bulging septum result in what?
repair response: fibrous thickening of the mitral valve
Other than surgical resection of the bulging septum in HCM, how can you fix this problem?
injecting alcohol into a septal coronary artery and infarcting the inner part of the hypertrophied upper septum
What is the characteristic histology of HCM?
marked myocyte hypertrophy, myocyte disarray, interstitial fibrosis
What does a stenotic aortic valve look like?
nodular calcifications in the sinuses of Valsalva (the pockets formed between the cusps of the aortic valve and the aorta around them)–these are on the outer side
What does a stenotic aortic valve have trouble doing?
opening (but he says it doesn’t close completely either)
Where is the heart valve commissure?
where the valve opening meets the valve annulus
What is the morphology of degenerative calcific aortic stenosis in the elderly?
formation of rocks in the sinuses of Valsalva
gradually stenoses the valve orifice.
What is the morphology of rheumatic aortic stenosis in young and middle aged adults?
inflammation and fibrosis start at the commissure and it moves inward from there
What congenital valve defect can lead to a more rapid development of aortic stenosis (presentation in 40s-50s?
bicuspid aortic stenosis
The risk factors for calcific aortic stenosis are nearly identical to the risk factors for what disease?
atherosclerosis
What is the normal ratio of right ventricular wall thickness to left ventricular wall thickness?
the thickness of the right ventricular wall should be less than a third of the left ventricular wall
What does chronic cor pulmonale mean?
right ventricular hypertrophy
What does acute cor pulmonale mean?
right ventricular dilatation
What is acute-on-chronic cor pulmonale?
right ventricular dilatation + right ventricular hypertrophy
What is the difference between stenosis and insufficiency?
Stenosis is the failure of a valve to open completely–obstructing forward flow. It is almost always a chronic process.
Insufficiency is the failure of a valve to close completely–allowing regurgitation of flow. It can be abrupt or insiduous.
Where is the most common site of valvular disease?
mitral valve
What is the difference between a murmur and a thrill?
thrills are severe lesions that can be palpated, murmurs can only be heard
What is the most common congenital valvular lesion? What causes it?
Bicuspid aortic valve, caused by problems with the “notch signaling pathway”
List the 4 changes that affect the integrity of the valvular ECM?
1) calcifications
2) decreased numbers of valve fibroblasts and myofibroblasts
3) Alterations of the ECM (increased proteoglycan, decreased collagen/elastin, etc.)
4) Changes in production of MMPs or inhibitors
What is the most common cause of aortic stenosis?
Calcifications- either related to normal aging OR a counterpart to atherosclerosis in elderly
What changes in the heart of someone with aortic stenosis to maintain CO?
left ventricular hypertrophy (because the outflow obstruction builds up pressure in the LV)
What are the common presentations of someone with aortic stenosis?
dyspnea, angina, or sudden death
What is the most common cause of HCM?
gain-of-funciton missense mutations (AD inheritance or de novo mutation) in one of several genes responsible for controlling the contractile apparatus- 70-80% of the time this is a sarcomeric protein
What is the most common sarcomeric protein to be mutated in HCM?
beta-myosin heavy chain
The ventricle in HCM looks like what fruit?
“banana like configuration”
When do you typically see HCM in patients?
during postpubertal growth spurt
Is the SV increased or decreased in HCM?
decreased due to impaired diastolic filling and smaller chamber size
Why do people with HCM get exertional dyspnea?
increased pulmonary venous pressure
What type of murmur is heard with HCM?
harsh, crescendo-decrescendo systolic ejection murmur
with S4 heart sound
What is the leading cause of sudden cardiac death in athletes under 35?
MI from HCM
Why do athletes die from HCM AFTER big games?
asymmetric hypertrophy of the septum causes obstruction that is relieved when the ventricle dilated during exercise. However, after that, dilation subsides while myocardial oxygen demand is still high and obstruction impedes the coronary flow and leads to MI and arrhythmia.
Why is hypertrophic cardiomyopathy autosomal dominant?
It is characteristic of genetic diseases of structural proteins to be autosomal dominant because they are “building something with half-bad materials yielding a faulty structure”
What is a sign of hypertensive heart disease on echocardiography? Why does this happen?
left atrial dilation–occurs becuase left atrium fills with the blood that it cannot squeeze into the smaller left ventricular cavity due to reduced left ventricular compliance. This is diastolic dysfunction.
Previous rheumatic valvulitis will lead to what type of aortic valve?
Stenotic- fibrous adhesion extending from the commissure from 3 sides (triangular), closing more than half of the valve opening with NO nodular calcification
Aortic valve stenosis due to pervious rheumatic valvulitis commonly presents with what?
95% have simultaneous mitral stenosis
Describe a pulmonary artery in pulmonary HTN.
Severe thickening of the wall and narrowing of the lumen with increased SM cells and fibrous tissue
What is the most common cause of chronic cor pulmonale?
COPD (pulmonary emphysemia)
How does emphysema lead to pulmonary HTN?
destroys vascular beds when it destroys alveolar structure. Right heart has to pump the same amount of blood through it, so the pressure goes up!
Will a pulmonary thromboembolus cause pulmonary HTN?
Getting a few hundred little thromboemboli (like in sickle cell patients or Factor V Leiden patients) will lead to pulmonary HTN
What can also cause pulmonary HTN that is an “underrecognized cause” according to Nichols?
foreign material getting stuck in pulmonary arteries from IV drug use. Tumor embolus can also be an underrecognized cause.
What is characteristic of some forms of pumonary HTN, especially seen in primary pulmonary HTN in young women?
plexiform lesions
