Cardiomyopathy / cardiac patho Test 2/2 Flashcards
Cardiomyopathy Damages the muscle
tone of the heart
Cardiomyopathy
heart, muscle, disease
disease of the heart
Cardiomyopathies = Hetergeneous group of diseases which have _________.
intrinsic myocardial dysfunction as their common feature.
Cardiomyopathies by definition excludes what?
myocardial disease caused by: ischemia, rheumatic disease, congenitial disease, cor pulmonale or HTN
How does cardiomyopathy differ from other heart disorders?
fairly uncommon, affecting only about 50,000 Americans.
leading cause of heart transplantation.
It often occurs in the young.
It tends to be progressive and sometimes worsens fairly quickly
Non-ischemic cardiomyopathy is due to
specific causes, and is often associated with diseases involving other organs as well as the heart.
Primary Cardiomyopathy
unknown cause (idiopathic)
Secondary Cardiomyopathy
may have a recognized extrinsic cause:
drug toxicity
amyloidosis
Non-ischemic Cardiomyopathy
Pathophysiologic Groups
- Dilated
- Hypertrophic
- Restrictive
Most Common Cardiomyopathy
Dilated
all ages, but middle aged men most common
What are the symptoms of cardiomyopathy? Although symptoms may not appear until late in the progression of the disease.
Symptoms are that of Heart failure: SOB, weakness, edema, arrythmias
Etiology of dilated Cardiomyopathy ?
alcohol, genetic, postviral, pregnancy,
Most often; idiopathic
Pathology of dilated Cardiomyopathy ?
4-chamber dilatation
hypertrophy
thrombi
Once diagnosed, this disease has often reached an advanced stage and heart failure has occurred.
Dilated Cardiomyopathy
5-year survival rate once heart failure is diagnosed
50%
10-year survival rate for Dilated Cardiomyopathy ?
25%
2nd most common form of cardiomyopathy, (but still rare)
0.2% of US population have it (inherited)
Hypertrophic Cardiomyopathy
Although many people have no symptoms; what are some of the symptoms we might see?
- SOB,
- Chest discomfort,
- Sudden death caused by chaotic heartbeat
Clinical symptoms of Hypertrophic Cardiomyopathy ?
angina
syncope
systolic murmur
dyspnea
Pathology of Hypertrophic Cardiomyopathy ?
- Myocardial hypertrophy L > R - Septum > free wall - Disorganized myofibrils - Abnormal coronary arteries thick-walled branches
Hypertrophic Cardiomyopathy AKA ?
(IHSS)
idiopathic hypertrophic sub- aortic stenosis
LV outflow tract obstruction
Severe hypertension
that was untreated for many years can lead to what?
Hypertrophic Cardiomyopathy
left ventricular hypertrophy, with asymmetric bulging of a very large interventricular septum into the left ventricular chamber describes what heart disease?
Hypertrophic Cardiomyopathy
Hypertrophic Carrdiomyopathy carries the risk of sudden death due to cardiac arrest brought on by arrythmias, what is the percentage of patients that die each year?
2 - 3%
What is the rarest form of cardiomyopathy?
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy results from what?
Another disease which occurs elsewhere in the body
Typical symptoms of CHF?
weakness,
SOB,
edema
With Restrictive Cardiomyopathy the heart remains ?
small unlike all other forms
Clinical symptoms of Restrictive Cardiomyopathy ?
- Rarest Kind
- Inhibition of diastolic filling (Diastolic Dysfunction)
- Heart failure
- R > L
Etiology of Restrictive Cardiomyopathy ?
unknown: possibly genetic
Pathology of Restrictive Cardiomyopathy is associated with what “myocardial” diseases ?
infiltrative
amyloidosis
sarcoidosis
At what temperature do we feel comfortable removing the cross clamp?
34 degrees Celsius
Deposition of abnormal substances that cause the ventricular walls to become progressively rigid, thereby impeding ventricular filling.
Some infiltrative cardiac diseases increase ventricular wall thickness,
while others cause chamber enlargement with secondary wall thinning.
Infiltrative cardiomyopathies
Increased wall thickness, small ventricular volume, and occasional dynamic left ventricular outflow obstruction describes what disease?
amyloidosis
A poorly understood disease that most commonly affects the lungs but can also involve almost every part of the body including the heart. Sarcoidosis is characterized by the presence of granulomas, which are ball-like collections of white blood cells that cluster around and react to a foreign substance. The inflammation associated with granulomas can damage virtually every part of the heart, including the electrical system, muscle, valves, arteries, and surrounding tissue called the pericardium.
Sarcoidosis
Pathology of Restrictive Cardiomyopathy that is associated with what “endocardial” diseases ?
- Loffler’s eosinophilic endocarditis
- Primary endocardial fibroelastosis
A form of restrictive cardiomyopathy which affects the endocardium and occurs with white blood cell proliferation, specifically of eosinophils.
This type of restrictive cardiomyopathy describes what disease?
Loffler’s eosinophilic endocarditis
Restrictive cardiomyopathy is defined as a disease of the heart muscle which results in
impaired filling of the heart ventricles during diastole.
Restrictive cardiomyopathy characterized by a thickening of the innermost lining of the heart chambers (the endocardium) due to an increase in the amount of supporting connective tissue and elastic fibers. It is an uncommon cause of unexplained heart failure in infants and children.
Primary endocardial fibroelastosis
EFE
Restrictive Cardiomyopathy Generally gets progressivily worse. What is the 5 year survival rate?
30%
What are 6 pharmacologic treatments for Cardiomyopathy ?
Inotropes Diuretics Vasodilators ACE-inhibitors/Beta blockers Others: Angiotensin II inhibitors, Anticoagulants
Nitropruside =
Vasodilator
What are 5 surgical treatments for Cardiomyopathy ?
- Pacemaker
- LaPlace Surgery
- LVAD
- Transplantation
- TAH (
LaPlace surgical treatments for Cardiomyopathy ?
Cardiomyoplasty (historic)
Batista
Acorn
Batista Procedure
Developed by Dr. Randas Batista (Brazil) in 1996
(PLV) Partial Left Ventriculectomy between both papillary muscles and extends from the appex to the mitral annulus.
Mitral valve and subvalvular apparatus were either preserved, repaired, or replaced,
“LV Reduction”
LVAD surgical treatments for Cardiomyopathy ?
Bridge to Recovery
Bridge to Transplant
Destination Therapy
Surgical remodeling for Ischemic heart Failure?
- Batista
- Left ventricular aneurysmectomy
Surgical remodeling for Non-Iscemic heart Failure?
Cardiomyoplasty
Current and Future LV Reconstructive procedure for Ischemic heart Failure?
Dor procedure
Current and Future LV Reconstructive procedures for Non-Ischemic heart Failure?
- Acorn (CorCap Cardiac Support Device)
- Myosplint
Cardiomyoplasty AKA
dynamic cardiomylasty
Surgical therapy for dilated cardiomyopathy in which the latissimus dorsi muscle is wrapped around the heart and paced during ventricular systole.
Cardiomyoplasty
“It appears that those who can survive the operation do not need it and those who need it, cannot survive it”
Initial experience with the Batista procedure demonstrated what?
↑ LVEF
↑ Clinical Function Status
↓ Heart Size
Of 120 patients Batista reported a 22% operative mortality and 2 year survival of what ?
55%.
Late fatal arrhythmias plagued the Batista procedure, forcing the use of concomitant implantable defibrillators.
Therefore the Batista procedure
has fallen out of favor and is no longer considered to be an appropriate option.
Dor procedure AKA
Endoventricular circular patch pasty (EVCPP),
Shortens the Long axis
Unchanged Short axis
.
An approach to surgical LV reconstruction in the setting of postinfarction aneurysm formation
Dor procedure AKA (EVCPP)
The Dor procedure for ischemic heart failure can be considered for patients with ?
- Symptomatic Aneurysms
- Angina Pectoris
- Systemic Embolization
- Malignant ventricular tachyarrhythmias
A net that is placed around and attached to the heart to support the damaged heart muscle and limit further enlargement. It provides passive support that
reduces the stress on the ventricular wall.
The CorCap Cardiac Support Device by Accorn
Treats Non-Ischemic Cardiomyopathy
Accorn Device Preliminary data suggest what?
Improves HF symptoms
↑ LVEF
↑ LVEDD
↑ Quality of life
Device that is composed of: -Two pads on the surface of the heart - Wire that passes through the ventricle - Placed under tension to create a bilobular shape
Myosplint
What is the ultimate treatment for heart failure?
Heart transplantation
What part of the recipient heart
remains with the recipient during heart transplantation?
Partial Left Atrium
HEART TRANSPLANTATION
ACTUARIAL SURVIVAL (1982-2000)
Half Life =
Conditional Half Life =
Half Life = 9.1 yrs
Conditional Half Life = 11.6 yrs