This document provides an overview of cardiomyopathy, focusing on its three main types: dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy. It defines cardiomyopathy as a disease of the heart muscle not caused by other structural heart issues. For each type, it discusses presentation, causes, morphology, clinical features, investigations and management. Dilated cardiomyopathy involves an enlarged heart with reduced function, while hypertrophic cardiomyopathy features abnormal thickening and restrictive cardiomyopathy impaired diastolic function. The document presents detailed information on evaluating and treating each form of cardiomyopathy.
Cardiomyopathy is a group of disease that affect the heart muscle. Early on there may be few or no symptoms. As the disease worsens, shortness of breath, feeling tired, and swelling of legs may occur, due to the onset of heart failure. An irregular heart beat and fainting may occur.
Cardiomyopathy is a disease of the heart muscles that makes it harder for your heart to pump blood to the rest of your body. Cardiomyopathy can lead to heart failure.
According to the structural and functional abnormalities of the heart muscle
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
Arrhythmogenic right ventricular cardiomyopathy
Unclassified cardiomyopathy
Cardiomyopathy is a group of disease that affect the heart muscle. Early on there may be few or no symptoms. As the disease worsens, shortness of breath, feeling tired, and swelling of legs may occur, due to the onset of heart failure. An irregular heart beat and fainting may occur.
Cardiomyopathy is a disease of the heart muscles that makes it harder for your heart to pump blood to the rest of your body. Cardiomyopathy can lead to heart failure.
According to the structural and functional abnormalities of the heart muscle
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
Arrhythmogenic right ventricular cardiomyopathy
Unclassified cardiomyopathy
Pathology of Cardiomyopathies
Literally means “disease of the heart muscle”.
Term “cardiomyopathy” is used to describe heart disease resulting from an abnormality in the myocardium.
Diseases of the myocardium usually produce:
>abnormalities in cardiac wall thickness and chamber size.
>mechanical or electrical dysfunction
>associated with significant morbidity and mortality.
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
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Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
Anti ulcer drugs and their Advance pharmacology ||
Anti-ulcer drugs are medications used to prevent and treat ulcers in the stomach and upper part of the small intestine (duodenal ulcers). These ulcers are often caused by an imbalance between stomach acid and the mucosal lining, which protects the stomach lining.
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Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
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MANAGEMENT OF ATRIOVENTRICULAR CONDUCTION BLOCK.pdfJim Jacob Roy
Cardiac conduction defects can occur due to various causes.
Atrioventricular conduction blocks ( AV blocks ) are classified into 3 types.
This document describes the acute management of AV block.
The prostate is an exocrine gland of the male mammalian reproductive system
It is a walnut-sized gland that forms part of the male reproductive system and is located in front of the rectum and just below the urinary bladder
Function is to store and secrete a clear, slightly alkaline fluid that constitutes 10-30% of the volume of the seminal fluid that along with the spermatozoa, constitutes semen
A healthy human prostate measures (4cm-vertical, by 3cm-horizontal, 2cm ant-post ).
It surrounds the urethra just below the urinary bladder. It has anterior, median, posterior and two lateral lobes
It’s work is regulated by androgens which are responsible for male sex characteristics
Generalised disease of the prostate due to hormonal derangement which leads to non malignant enlargement of the gland (increase in the number of epithelial cells and stromal tissue)to cause compression of the urethra leading to symptoms (LUTS
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
2. INTRODUCTION
Cardiomyopathy is disease of the heart muscle.
This term is intended to exclude cardiac dysfunction that results from other
structural heart disease, such as coronary artery disease, primary valve
disease, or severe hypertension.
As of 2006, cardiomyopathies are defined as “a heterogeneous group of
diseases of the myocardium associated with mechanical and/or electrical
dysfunction that usually exhibit inappropriate ventricular hypertrophy or
dilatation and are due to a variety of causes that frequently are genetic”
3. GENERAL PRESENTATION
For all cardiomyopathies, the early symptoms often relate to exertional intolerance with
breathlessness or fatigue, usually from inadequate cardiac reserve during exercise.
Shortness of breath may occur during routine daily activity such as dressing and may
manifest as dyspnea or cough when lying down at night.
Peripheral edema
Particularly in younger patients in whom ascites and abdominal discomfort may dominate.
All three types of cardiomyopathy can be associated with atrioventricular valve
regurgitation, typical and atypical chest pain, atrial and ventricular tachyarrhythmias, and
embolic events.
5. DILATED CARDIOMYOPATHY
About one in three cases of congestive heart failure is due to dilated cardiomyopathy.
An enlarged left ventricle with decreased systolic function as measured by left ventricular
ejection fraction characterizes dilated cardiomyopathy.
The dilated cardiomyopathy phenotype is often viewed as a “final common pathway” of
numerous types of cardiac injuries.
LV and/or RV systolic pump function is impaired, leading to progressive cardiac dilatation
(remodelling).
Symptoms of HF typically appear only after remodelling has been ongoing for some
time (months to years).
6. DILATED CARDIOMYOPATHY
DCM is either familial or the end result of myocardial damage produced by a variety
of known or unknown infectious, metabolic, or toxic agents.
One-fifth to one-third of patients have familial forms of DCM.
A reversible form of DCM may be found with alcohol abuse, pregnancy, thyroid
disease, cocaine use, chemotherapy drugs, nutritional deficiencies and chronic
uncontrolled tachycardia.
Although DCM may occur at any age, it most commonly becomes apparent
clinically in the 3rd or 4th decades.
7. CAUSES
Genetic influences
20-50% are familial
Autosomal dominant –predominant pattern
Mutations in genes encoding dystrophin,δ sarcoglycan,troponin T, β MHC
Myocarditis
Alcohol and other toxins
Childbirth (peripartum cardiomyopathy)
9. MORPHOLOGY
Heart enlarged,heavy,flabby
Mural thrombi common
Dilatation of all chambers,both
ventricular hypertrophy
Microscopically- atrophic and
hypertrophic myocardial fibres, cardiac
myocytes show degenerative changes
Interstitial and endocardial fibrosis
10. CLINICAL FEATURES
Symptoms may be gradual in onset
Acute presentation
Misdiagnosed as viral URI in young adults
Symptoms/Signs of heart failure
Pulmonary congestion (left HF) dyspnea (rest, exertional, nocturnal), orthopnea
Systemic congestion (right HF) edema, nausea, abdominal pain, nocturia
Low cardiac output
Hypotension, tachycardia, tachypnea
Fatigue and weakness
Arrhythmia
Atrial fibrillation, conduction delays, sudden death
11. INVESTIGATIONS
Chest radiogram:
Enlargement of the cardiac silhouette due to LV dilatation, although
generalized cardiomegaly is often seen.
Electrocardiogram:
sinus tachycardia or atrial fibrillation,
ventricular arrhythmias,
left atrial abnormality,
low voltage,
diffuse nonspecific ST-T-wave abnormalities,
intraventricular and/or AV conduction defects.
13. INVESTIGATIONS
Echocardiography, CTI, and CMRI
will show LV dilatation, with normal, minimally thickened, or thinned walls, and
systolic dysfunction.
Circulating levels of brain natriuretic peptide(BNP) are usually elevated.
Cardiac catheterization and coronary angiography are often performed to
exclude ischemic heart disease.
16. MANAGEMENT
Limit activity based on functional
status
Salt restriction of 5g NaCl diet
Fluid restriction for significant low
Na+
Initiate medical therapy
ACE inhibitors, diuretics
digoxin, carvedilol
ß-blocking agents
Anticoagulation for EF <30%, or if
patient has a history of
thromboembolism, presence of mural
thrombi
Intravenous dopamine, dobutamine
and/or phosphodiesterase inhibitors
Cardiac transplantation
18. HYPERTROPHIC CARDIOMYOPATHY
Hypertrophic cardiomyopathy is defined as left ventricular hypertrophy that develops in
the absence of causative hemodynamic factors, such as hypertension, aortic valve disease,
or systemic infiltrative or storage diseases.
It is the leading cause of sudden death in the young and is an important cause of heart
failure.
A sarcomere mutation is present in 60% of patients with hypertrophic cardiomyopathy
and is more common in those with familial disease and characteristic asymmetric septal
hypertrophy.
19. HYPERTROPHIC CARDIOMYOPATHY
Hypertrophic cardiomyopathy is characterized by age-dependent and incomplete
penetrance.
More than nine different sarcomere genes with over 1400 mutations have been implicated,
although 80% of patients have a mutation in either MYH7 or MYBPC3
In MYBPC3 mutation carriers, the average age of disease development is 40 years, while
30% remain free from hypertrophy after 70 years.
LV hypertrophy is asymmetric, often with preferential hypertrophy of the interventricular
septum.
It is found in about 1 in 500 of the general population
20. PATHOGENESIS
The major abnormality of the heart in HCM -- excessive
thickening of the muscle.
Thickening usually begins during early adolescence and stops
when growth has finished.
Hypertrophy is usually greatest in the septum, associated
with obstruction to the flow of blood into the aorta.
Asymmetric septal hypertrophy with obstruction to the
outflow of blood from the heart may occur.
The mitral valve touches the septum, blocking the outflow
tract.
Some blood is leaking back through the mitral valve causing
mitral regurgitation
21. PATHOGENESIS
Systolic obstruction is initiated by drag forces, which push an anteriorly displaced and
enlarged anterior mitral leaflet into contact with the hypertrophied ventricular septum.
Mitral leaflet coaptation may ensue, leading to posteriorly directed mitral regurgitation.
In order to maintain stroke volume across outflow tract obstruction, the ventricle generates
higher pressures, leading to higher wall stress and myocardial oxygen demand.
Smaller chamber size and increased contractility exacerbate the severity of obstruction.
23. CLINICAL FEATURES
About half of all patients with HCM have a positive family history compatible
with autosomal dominant transmission.
Many patients are asymptomatic - echocardiographic finding
Symptomatic
dyspnea in 90%
angina pectoris in 75%
fatigue, pre-syncope, syncope.
↑ risk of SCD
palpitation, PND, CHF, dizziness less frequent
Atrial fibrillation and thromboembolism
24. PHYSICAL EXAMINATION
Most patients demonstrate a double or triple apical precordial impulse and S4 heart
sound.
A rapidly rising arterial pulse: Those with intraventricular pressure gradients.
Systolic murmur:
The hallmark of obstructive HCM is a systolic murmur, which is typically harsh, diamond-
shaped, and usually begins well after the first heart sound.
The murmur is best heard at the lower left sternal border as well as at the apex, where it is
often more holosystolic and blowing in quality, no doubt due to the mitral regurgitation
that usually accompanies obstructive HCM
26. INVESTIGATIONS
ECG: LV hypertrophy and widespread deep, broad Q waves, T wave inversions.
Chest X-ray: may be normal, although a mild to moderate increase in the cardiac silhouette is
common.
Echocardiogram:
The mainstay of the diagnosis of HCM
LV hypertrophy, often with the septum 1.3 times the thickness of the posterior LV free wall.
SAM of the mitral valve, often accompanied by mitral regurgitation, is found in patients with pressure
gradients.
The LV cavity typically is small in HCM, with vigorous motion of the posterior wall but with reduced septal
excursion.
CMRI: is superior to echocardiography in providing accurate measurements of regional hypertrophy
and in identifying sites of regional fibrosis
29. MANAGEMENT
Since SCD often occurs during or just after physical exertion, competitive
sports and very strenuous activities should be proscribed.
Beta-adrenergic blockers
Calcium antagonist
Disopyramide
Amiodarone, sotalol
Pacemaking
Myotomy-myectomy
30. RESTRICTIVE CARDIOMYOPATHY
The least common of the physiologic triad of cardiomyopathies.
restrictive cardiomyopathy, which is dominated by abnormal diastolic function, often with mildly
decreased contractility and ejection fraction (usually >30-50%).
Both atria are enlarged, sometimes massively.
Modest left ventricu1ar dilation can be present, usually with an end diastolic dimension <6 cm.
End-diastolic pressures are elevated in both ventricles, with preservation of cardiac output until
late in the disease.
Much less common than DCM and HCM
32. CLINICAL MANIFESTATIONS
Symptoms of right and left heart failure.
Exercise intolerance and dyspnea are usually prominent.
Commonly have dependent edema, ascites, and an enlarged, tender, and often pulsatile liver.
Kusmaul’s sign is positive.
Heart sounds may be distant, and third and fourth heart sounds are common.
Unlike constrictive pericarditis the apex impulse is usually easily palpable, and mitral regurgitation
is more common.
Thromboembolic complications are frequent in such patients.
33. AMYLOIDOSIS
Amyloidosis is the major cause of restrictive cardiomyopathy.
Several proteins can self-assemble to form the beta-sheets of amyloid proteins, which deposit with
different consequences depending on the type of protein.
Cardiac amy10id is classically suspected from thickened ventricular walls with an ECG that shows 10w
voltage.
A characteristic refractile brightness in the septum on echocardiography is suggestive of the diagnosis,
but neither sensitive nor specific.
Both atria are dilated, often dramatically, and diastolic dysfunction may be more obvious than in left
ventricular hypertrophy from other causes.
Therapy for all types of amyloid is predominantly for symptoms of fluid retention, which often requires
high doses of loop diuretics.
34. MORPHOLOGY
The heart is firm and rubbery with a
waxy cut surface. The atria are
markedly dilated and the left
endocardium, normally smooth has a
yellow – brown amyloid deposit that
gives texture to the surface.
Echo shows thickened walls of both ventricle
without major chamber dilation. The atria are
markedly dilated consistent with chronically
elevated ventricular pressure.
35. FIBROTIC RCM
Progressive fibrosis can cause restrictive myocardial disease without
ventricular dilation. Thoracic radiation, common for breast and lung cancer
or mediastinal lymphoma, can produce early or late restrictive
cardiomyopathy.
Scleroderma causes small vessel spasm and ischemia that can lead to a
small, stiff heart with reduced ejection fraction without dilation.
The pulmonary hypertension associated with scleroderma may lead to
more clinical right heart failure because of concomitant fibrotic disease of
the right ventricle.
36. INVESTIGATIONS
ECG: low-voltage, nonspecific ST-T-wave abnormalities and various
arrhythmias.
Chest x-ray: Pericardial calcification which occurs in constrictive
pericarditis, is absent.
Echocardiography, CTI, and CMRI typically reveal symmetrically thickened
LV walls and normal or slightly reduced ventricular volumes and systolic
function; the atria are usually dilated.
Doppler echocardiography typically shows diastolic dysfunction
37. MANAGEMENT
No satisfactory medical therapy.
Chronic anticoagulation is often recommended to reduce the risk of embolization
from the heart.
Drug therapy must be used with caution.
Diuretics for extremely high filling pressures.
Vasodilators may decrease filling pressure.
Calcium channel blockers to improve diastolic compliance.
digitalis and other inotropic agents are not indicated.
38. Reference
Harrisons
Maron BJ, Moller JH, Seidman C. et al. Impact of laboratory molecular
diagnosis on contemporary diagnostic criteria for genetically transmitted
cardiovascular disease: hypertrophic cardiomyopathy, long-QT syndrome,
and Marfan Syndrome. Circulation. 1998;98
Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, Dubourg
O, Kühl U, Maisch B, McKenna WJ, et al. Classification of the
cardiomyopathies: a position statement from the European Society Of
Cardiology Working Group on Myocardial and Pericardial Diseases. Eur
Heart J. 2008