The cardiomyopathies are a group of diseases that primarily affect the heart muscle and are not the result of congenital, acquired valvular, hypertensive, coronary arterial, or pericardial abnormalities.
The diffuse myocardial fibrosis that accompanies multiple myocardial scars produced by myocardial infarctions can impair left ventricular function and is frequently referred to as ischemic cardiomyopathy.
Two fundamental forms of cardiomyopathy are recognized:
1. A primary type, consisting of heart muscle disease predominantly involving the myocardium and/or of unknown cause
2. A secondary type, consisting of myocardial disease of known cause or associated with a systemic disease such as amyloidosis or chronic alcohol use.
Classification of the cardiomyopathies into one of three morphologic types
on the basis of differences in their pathophysiology and clinical presentation
Classification of Cardiomyopathies
1. Primary Myocardial Involvement
a. Idiopathic (D,R,H)
b. Familial (D,R,H)
c. Eosinophilic endomyocardial disease (R)
d. Endomyocardial fibrosis (R)
2. Secondary Myocardial Invovlement
a. Viral myocarditis
b. Bacterial myocarditis
c. Fungal myocarditis
d. Protozoal myocarditis
e. Metazoal myocarditis
h. Familial storage disease (D,R)
i. Glycogen storage disease
l. Fabry's disease
Connective tissue disorders (D)
o. Systemic lupus erythematosus
p. Polyarteritis nodosa
q. Rheumatoid arthritis
r. Progressive systemic sclerosis
Infiltrations and granulomas (R,D)
w. Muscular dystrophy
x. Myotonic dystrophy
y. Friedreich's ataxia (H,D)
Sensitivity and toxic reactions (D)
Peripartum heart disease (D)
CLINICAL CLASSIFICATION OF CARDIOMYOPATHIES
Left and / or right ventricular enlargement, impaired systolic function, congestive heart failure, arrhythmias, emboli
Endomyocardial scarring or myocardial infiltration resulting in restriction to left and/or right ventricular filling
Disproportionate left ventricular hypertrophy, typically involving septum more than free wall, with or without an intraventricular systolic pressure gradient; usually of a nondilated left ventricular cavity
About one in three cases of congestive heart failure is due to dilated cardiomyopathy.
Left and right ventricular systolic pump function is impaired, leading to progressive cardiac dilatation.
Although no cause is apparent in many cases, dilated cardiomyopathy is either familial or the end result of myocardial damage produced by a variety of known or unknown infectious, metabolic, or toxic agents.
Dilated cardiomyopathy may be the late consequence of acute viral myocarditis, possibly mediated in part through an immunologic mechanism.
Although dilated cardiomyopathy may occur at any age, it most commonly becomes apparent clinically in the third or fourth decades.
A reversible form of Dilated cardiomyopahty may be found with alcohol abuse, pregnancy, thyroid disease, cocaine use, and chronic uncontrolled tachycardia.
One-fifth to one-third of patients have familial forms of dilated cardiomyopathy.
Mutations in > 20 genes that are transmitted in an autosomal dominant fashion have been described.
Most common are mutations in genes encoding sarcomeric proteins, such as -cardiac actin; - and -myosin; heavy chain -tropomyosin; and troponins T, I, and C.
It is believed that the abnormal proteins cause contractile dysfunction by impairing the production and transmission of force.
Ø Symptoms of left- and right-sided congenital heart failure usually develop gradually.
Ø Some patients have LV dilatation for months or even years before becoming symptomatic.
Ø Although vague chest pain may be present, typical angina pectoris is unusual and suggests the presence of ischemic heart disease.
Ø Syncope due to arrhythmias and systemic embolism may occur.
Variable degrees of cardiac enlargement and findings of congenital heart failure are noted.
In patients with advanced disease,
Ø The pulse pressure is narrow
Ø The jugular venous pressure is elevated
Ø Third and fourth heart sounds are common
Ø Mitral or tricuspid regurgitation may occur.
The chest roentgenogram demonstrates enlargement of the cardiac silhouette due to LV dilatation, although generalized cardiomegaly is often seen.
The lung fields may demonstrate pulmonary vascular redistribution and interstitial or, in advanced cases, alveolar edema.
The electrocardiogram often shows sinus tachycardia or atrial fibrillation, ventricular arrhythmias, left atrial abnormality, low voltage, diffuse nonspecific ST-T-wave abnormalities, and sometimes intraventricular and AV conduction defects.
Echocardiography, computed tomographic imaging and cardiac magnetic resonance imaging show LV dilatation, with normal, minimally thickened, or thinned walls, and systolic dysfunction. Circulating levels of brain natriuretic peptide are usually elevated.
Typical 2D echo of dilated cardiomyopathy showing dilated Left Ventricle and large left atrium
Cardiac catheterization and coronary angiography are often performed to exclude ischemic heart disease, and bedside hemodynamic monitoring may be helpful in the management of selected acutely decompensated patients.
Angiography reveals a dilated, diffusely hypokinetic left ventricle, often with some degree of mitral regurgitation.
Most patients pursue an inexorably downhill course, and the majority, particularly those > 55 years, die within 4 years of the onset of symptoms.
Death is due to either progressive Heart Failure or ventricular tachy or bradyarrhythmia, Systemic embolization is a concern, and patients should be considered for chronic anticoagulation.
Diuretics decrease preload and hence alleviate systemic and pulmonary venous congestion.
Vasodilators are probably the most useful addition to the treatment of dilated cardiomyopathy.
Traditional advice has been to avoid exercise and maintain a sedentary existence. Such restriction of exercise, however, leads to deconditioning which may adversely affect exercise tolerance.
This is reserved for those who are terminally sick and have a life expectancy of less than six months.
(1) Age above 55 years or less than 10 years
(2) Left ventricular conduction delay
(3) Absence of left ventricular hypertrophy
(4) Cardiothoracic ratio greater than 55 percent
(5) Cardiac index less than 2.6 litres/min/m2
Individuals who consume large quantities ( > 90 g/d) of alcohol over many years may develop a clinical picture resembling idiopathic or familial dilated cardiomyopathy.
The risk of developing cardiomyopathy is partially determined genetically.
A polymorphism of the gene encoding the alcohol metabolizing enzyme, alcohol dehydrogenase, as well as the form of the angiotensin-converting enzyme gene increase the predilection for the development of alcoholic cardiomyopathy.
Patients with advanced alcoholic cardiomyopathy and severe congestive heart failure have a poor prognosis, particularly if they continue to drink; fewer than one-quarter of such patients survive 3 years.
Management consists of abstention, which may halt the progression or even reverse the course of this disease.
A second presentation of alcoholic cardiotoxicity may be found in individuals without overt heart failure and consists of recurrent supraventricular or ventricular tachyarrhythmias.
Termed the holiday heart syndrome, it typically appears after a drinking binge; atrial fibrillation is seen most frequently, followed by atrial flutter and frequent ventricular premature depolarizations.
In contrast to the adverse cardiac effects of excessive alcohol consumption, moderate consumption (20–30 g/d) appears to be cardioprotective.It raises high-density lipoprotein cholesterol and is associated with a reduced incidence of ischemic heart disease, ischemic stroke, and metabolic syndrome.
Cardiac dilatation and congestive heart failure may develop during the last trimester of pregnancy or within 6 months of delivery.
The cause is unknown, although inflammatory myocarditis, immune activation, and gestational hypertension have all been incriminated.
The symptoms, signs, and treatment are similar to those in patients with idiopathic dilated cardiomyopathy, the latter including implantation of cardiac transplantation if the criteria for these therapies are satisfied.
The prognosis is related to whether the heart size returns to normal after the first episode of congestive heart failure.
Patients who recover from peripartum cardiomyopathy should be encouraged to avoid further pregnancies, particularly if left ventricular dysfunction persists.
Cardiac involvement is common in many of the muscular dystrophies.
In Duchenne's progressive muscular dystrophy, mutations in a gene that encodes a cardiac structural protein lead to myocyte death.
Myocardial involvement is most frequently indicated by a distinctive and unique ECG pattern consisting of tall R waves in the right precordial leads with an R/S ratio > 1.0, often associated with deep Q waves in the limb and lateral precordial leads.
A variety of supraventricular and ventricular arrhythmias is frequently found. Rapidly progressive heart failure may develop despite extended periods of apparent circulatory stability.
Myotonic dystrophy is characterized by a variety of ECG abnormalities, especially disorders of impulse formation and AV conduction, but other overt clinical evidence of heart disease is uncommon. Because of these abnormalities, syncope and sudden death are major hazards.
ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY
It is a familial cardiomyopathy characterized by progressive fibrofatty replacement of the right ventricle and, to a much lesser degree, of the left ventricular myocardium.
It is most commonly inherited in an autosomal dominant manner and is caused by multiple mutations of several genes encoding proteins that constitute desmosomes, structures that maintain normal contacts between cells.
It has been suggested that abnormalities in the desmosomes cause detachment of myocytes with consequent myocyte apoptosis and fibrofatty replacement. Among the desmosomal protein genes, the most common gene mutation occurs in plakophilin-2. Mutations in the cardiac ryanodine receptor gene and other genes have also been described.
On clinical examination, patients may manifest
Ø Right Ventricular failure with jugular venous distention,
Ø Ventricular tachyarrhythmias
The ECG typically shows QRS prolongation localized to the right precordial leads and left bundle branch block–type ventricular tachycardia.
CTI and CMRI typically show RV dilatation, RV aneurysm, and fatty replacement.
CMRI showing right ventricular enlargement and fatty replacement of the right ventricular myocardium of a patient with arrhythmogenic Right Ventricular dysplasia.
Restriction from competitive sports and antiarrhythmic therapy with beta blockers or amiodarone may be useful. If Right Ventricular failure becomes intractable, cardiac transplantation may be necessary.
TAKO-TSUBO (STRESS) CARDIOMYOPATHY
Also known as apical ballooning syndrome, this uncommon cardiac syndrome is characterized by the abrupt onset of severe chest discomfort preceded by a very stressful emotional or physical event.
It occurs most commonly in women > 50 years and is accompanied by ST-segment elevations and deep T-wave inversions in the precordial leads.
No obstruction in the epicardial coronary arteries is noted on angiography. There is severe akinesia of the distal portion of the left ventricle with reduction of the EF.
Troponins are usually mildly elevated. Cardiac imaging typically shows "ballooning" of the left ventricle in end-systole, especially of the LV apex.
All of these changes, which are often quite dramatic, are reversible within 3–7 days and do not cause long-term cardiac dysfunction or disability.
The mechanism responsible for Tako-tsubo cardiomyopathy is not clear, although it is likely that an adrenergic surge that includes circulating catecholamines, acting on the epicardial coronary vessels and coronary microcirculation, is involved. Although beta blockers are used in therapy, there is no definitive evidence that they are beneficial.
LEFT VENTRICULAR NONCOMPACTION
Left ventricular noncompaction is a recently characterized uncommon congenital cardiomyopathy that may present at any age with symptoms of
1. Congestive heart fialure
3. Ventricular arrhythmias.
It results from the arrest of normal embryogenesis, with the persistence of the deep recesses and sinusoids in the myocardium that characterize the embryonic heart.
The sinusoids and associated spongy network of myocardial fibers ordinarily undergo organization and "compaction" early in embryonic life; when this fails to occur, left ventricular noncompaction results.
This condition is diagnosed on echocardiography by the demonstration of multiple deep trabeculations into the myocardium, all of which communicate with the ventricular cavity, associated with left ventricular contractile dysfunction.
Hypertrophic cardiomyopathy is characterized by left ventricular hypertrophy, typically of a nondilated chamber, without obvious cause, such as hypertension or aortic stenosis.
It is found in about 1 in 500 of the general population.
Two features of hypertrophic cardiomyopathy
1. Asymmetric LV hypertrophy, often with preferential hypertrophy of the interventricular septum
2. A dynamic LV outflow tract pressure gradient, related to narrowing of the subaortic area.
About one-third of patients with hypertrophic cardiomyopathy demonstrate an outflow tract pressure gradient at rest and a similar fraction develop one with provocation.
The ubiquitous pathophysiologic abnormality is diastolic dysfunction, which can be detected by Doppler tissue imaging and results in elevated left ventricular end-diastolic pressures; the latter may be present despite a hyperdynamic, nondilated LV.
The pattern of hypertrophy is distinctive in hypertrophic cardiomyopathy and usually differs from that seen in secondary hypertrophy.
Most patients have striking regional variations in the extent of hypertrophy in different portions of the left ventricle, and the majority demonstrate a ventricular septum whose thickness is disproportionately increased when compared with the free wall.
In the disproportionately hypertrophied portions of the left ventricle, there is a bizarre and disorganized arrangement of myocytes, with disorganization of the myofibrillar architecture, along with a variable degree of myocardial fibrosis and thickening of the small intramural coronary arteries.
About half of all patients with hypertrophy cardiomyopathy have a positive family history compatible with autosomal dominant transmission.
More than 400 mutations of 11 different genes that encode sarcomeric proteins have been identified; these account for ~ 60% of cases. The most common are mutations of the cardiac -myosin heavy chain gene on chromosome 14.
Others involve -myosin heavy chains; cardiac troponins C, I, and T; cardiac myosin-binding protein C; actin; myosin light chains; and titin. Certain mutations are associated with more malignant prognoses.
Many sporadic cases of HCM probably represent spontaneous mutations.
Echocardiographic studies have confirmed that by the age of 20, when full expression has usually occurred, about one-half of the first-degree relatives of patients with familial hypertrophy cardiomyopathy have evidence of the disease.
However, in many of these relatives the extent of hypertrophy is mild, no outflow tract pressure gradient is present, and symptoms are not prominent.
Since the hypertrophic characteristics may not be apparent in childhood, a single normal echocardiogram in a child does not exclude the presence of the disease.
Screening by echocardiography of first-degree relatives between the ages of 12 and 20 should be carried out every 12–24 months, unless the diagnosis is established or excluded by genetic testing.
The clinical course of hypertrophic cardiomyopathy is highly variable.
Many patients are asymptomatic or mildly symptomatic and may be relatives of patients with known disease.
Unfortunately, the first clinical manifestation may be sudden death frequently occurring in children and young adults during or after physical exertion.
Indeed, hypertrophy cardiaomyopathy is the most common cause of sudden death in young competitive athletes.
In symptomatic patients, the most common complaint is
2. Diastolic ventricular dysfunction
3. Impairs ventricular filling
4. Elevated Left ventricular diastolic, Left atrial, and pulmonary capillary pressures.
6. Angina pectoris
Most patients demonstrate a double or triple apical precordial impulse and a fourth heart sound.
Those with intraventricular pressure gradients may have a rapidly rising arterial pulse.
The hallmark of obstructive hypertrophic cardiomyopathy 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 hypertrophic cardiomyopathy.
In contrast to the obstruction produced by a fixed narrowed orifice, such as valvular aortic stenosis, the pressure gradient in hypertrophic cardiomyopathy, when present, is dynamic and may change between examinations and even from beat to beat.
Obstruction appears to result from narrowing of the left ventricular outflow tract by systolic anterior movement of the mitral valve against the hypertrophied septum.
Three basic mechanisms are involved in the production and intensification of the dynamic intraventricular obstruction:
(1) Increased LV contractility,
(2)Decreased ventricular preload
(3)Decreased aortic impedance and pressure (afterload).
Interventions that increase myocardial contractility, such as exercise and sympathomimetic amines, and those that reduce ventricular preload, such as the strain phase of the Valsalva maneuver.
sudden standing, or nitroglycerin, reduce left ventricular end-diastolic volume and, thereby, may cause an increase in the gradient and the murmur.
Conversely, elevation of arterial pressure by squatting, sustained handgrip, augmentation of venous return by passive leg raising, and expansion of the blood volume all increase ventricular volume and ameliorate the gradient and murmur.
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Mild cardiac silhouette enlargement
Mild to moderate cardiac silhouette enlargement
ST-segment and T-wave abnormalities
Low voltage, conduction defects
Left ventricular dilatation and dysfunction
Left ventricular dilatation and dysfunction (RVG)
Normal or mildly reduced systolic function (RVG)
The ECG commonly shows LV hypertrophy and widespread deep, broad Q waves.
The latter suggest an old myocardial infarction but actually reflect severe septal hypertrophy
Echocardiogram in hypertrophic cardiomyopathy
Many patients demonstrate arrhythmias, both atrial and ventricular, during ambulatory monitoring.
Chest roentgenography may be normal, although a mild to moderate increase in the cardiac silhouette is common.
The mainstay of the diagnosis of hypertrophic cardiomyopathy is the echocardiogram, which demonstrates LV hypertrophy, often with the septum 1.3 times the thickness of the posterior LV free wall.
The septum may demonstrate an unusual "ground-glass" appearance, probably related to its myocardial fibrosis.
Systolic anterior motion 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.
An uncommon form of cardiomyopathy, characterized by apical hypertrophy, is associated with giant negative T waves on the ECG and a "spade-shaped" LV cavity; it usually has a benign clinical course.
CMRI is superior to echocardiography in providing accurate measurements of regional hypertrophy and in identifying sites of regional fibrosis.
1. Since sudden death often occurs during or just after physical exertion, competitive sports and very strenuous activities should be proscribed.
2. Dehydration should be avoided, and diuretics used with caution.
3. Adrenergic blockers ameliorate angina pectoris and syncope in one-third to one-half of patients. Although resting intraventricular pressure gradients are usually unchanged, these drugs may limit the increase in the gradient that occurs during exercise. It does not appear that adrenergic blockers offer any protection against sudden death.
4. Amiodarone appears to be effective in reducing the frequency of supraventricular as well as of life-threatening ventricular arrhythmias, and anecdotal data suggest that it may reduce the risk of SCD.
5. Nondihydropyridine calcium channel blockers (verapamil and diltiazem) may reduce the stiffness of the left ventricle, reduce the elevated diastolic pressures, increase exercise tolerance, and, in some instances, reduce the severity of outflow tract pressure gradients.
6. Disopyramide has been used in some patients to reduce LV contractility and the outflow pressure gradient; it may reduce symptoms as well.
The natural history of hypertrophy cardiomyopathy is variable, although many patients never exhibit any clinical manifestations.
Atrial fibrillation is common late in the course of the disease; its onset often leads to the development of or an increase in symptoms.
Infective endocarditis occurs in < 10% of patients.
However, endocarditis prophylaxis is currently recommended only in hypertrophy cardiomyopathy patients with a prior episode of infective endocarditis.
INHERITED METABOLIC CARDIOMYOPATHIES WITH LEFT VENTRICULAR HYPERTROPHY
CARDIAC DANON DISEASE
a. This condition is caused by mutations in an X-linked lysosome-associated membrane protein (LAMP2).
b. It is characterized by enlarged ventricular myocytes with periodic acid Schiff (PAS)–positive inclusions.
c. Patients present in childhood with CHF and serious arrhythmias.
d. The ECG shows severe LV hypertrophy and ventricular preexcitation.
GLYCOGEN STORAGE CARDIOMYOPATHY
1. This recently characterized condition is caused by a mutation in the 2 regulatory subunit (PRKAG2) of adenosine monophosphate–activated protein kinase (AMPK).
2. It is characterized by ventricular hypertrophy resembling that observed in hypertrophic cardiomyopathies and enlarged myocytes with vacuoles in the myocytes that stain for glycogen.
1. This X-linked autosomal recessive lysosomal storage disorder is caused by deficiency of lysosomal galactoside A and can lead to the accumulation of glycosphingolipids in the heart, with ventricular hypertrophy resembling hypertrophy cardiomyopathy .
2. Because of severe impairment in ventricular filling, it is sometimes classified as a restrictive cardiomyopathy.
3. It may be associated with AV conduction abnormalities and ventricular tachyarrhythmias.
4. CMRI is helpful in establishing the diagnosis.
5. Treatment consists of enzyme replacement therapy with agalsidase .
1. This is an autosomal recessive spinocerebellar degenerative disease caused by inadequate levels of frataxin, a protein involved in mitochondrial iron metabolism.
2. Approximately one-half of the patients develop cardiac symptoms.
3. The ECG most commonly demonstrates ST-segment and T-wave abnormalities.
4. Echocardiography and other imaging studies usually show symmetric left ventricular hypertrophy or asymmetric hypertrophy of the interventricular septum compared with the free wall.
5. Although the gross morphologic appearance of the heart in Friedreich's ataxia may be similar to that in hypertrophy cardiomyopathy, cellular disarray is lacking.
The hallmark of the restrictive cardiomyopathies is abnormal diastolic function; the ventricular walls are excessively rigid and impede ventricular filling.
In late stages systolic function is also impaired. Myocardial fibrosis, hypertrophy, or infiltration due to a variety of causes is responsible.
Myocardial involvement with amyloid is a common cause of secondary restrictive cardiomyopathy, although restriction is also seen in the transplanted heart, in hemochromatosis, glycogen deposition, endomyocardial fibrosis, sarcoidosis, hypereosinophilic disease, and scleroderma; following mediastinal irradiation; and in neoplastic infiltration and myocardial fibrosis of diverse causes.
In many of these conditions, particularly those with substantial concomitant endocardial involvement, partial obliteration of the ventricular cavity by fibrous tissue and thrombus contributes to the abnormally increased resistance to ventricular filling.
Thromboembolic complications are frequent in such patients.
The inability of the ventricles to fill limits cardiac output and raises filling pressures
a. Exercise intolerance
c. Elevated systemic venous pressure
f. Enlarged, tender, and often pulsatile liver.
g. Jugular venous pressure is elevated with inspiration (Kussmaul's sign).
h. The heart sounds may be distant, and third and fourth heart sounds are common.
i. Apex impulse is usually easily palpable
j. Mitral regurgitation is more common.
In patients with infiltrative cardiomyopathies, the ECG often shows low-voltage, nonspecific ST-T-wave abnormalities and various arrhythmias.
Pericardial calcification on x-ray, 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. Cardiac catheterization shows a reduced cardiac output, elevation of the RV and LV end-diastolic pressures, and a dip-and-plateau configuration of the diastolic portion of the ventricular pressure pulses resembling constrictive pericarditis.
Differentiation of restrictive cardiomyopathy from constrictive pericarditis is of importance because the latter is often curable by surgery.
Management is usually disappointing, except for hemochromatosis and Fabry's disease.
Chronic anticoagulation is often recommended to reduce the risk of embolization from the heart.
EOSINOPHILIC ENDOMYOCARDIAL DISEASE
Also called Loeffler's endocarditis and fibroplastic endocarditis, this condition occurs in temperate climates.
It appears to be a subcategory of the hypereosinophilic syndrome in which the heart is predominantly involved, with cardiac damage the apparent result of the toxic effects of eosinophilic proteins.
Typically, the endocardium of either or both ventricles is thickened markedly, with involvement of the underlying myocardium.
Cardiac imaging typically reveals ventricular thickening, especially of the posterobasal LV wall. Mitral regurgitation is frequently present on Doppler echocardiography.
Large mural thrombi may develop in either ventricle, thereby compromising the size of the ventricular cavity and serving as a source of pulmonary and systemic emboli.
Hepatosplenomegaly and localized eosinophilic infiltration of other organs are usually present.
Management usually includes diuretics, afterload-reducing agents, and anticoagulation.
The use of glucocorticoids and hydroxyurea appears to improve survival.
Surgical treatment with resection of fibrotic tissue and mitral valve repair or replacement may be helpful in selected patients.
Involvement of the heart is the most frequent cause of death in primary amyloidosis (AL) and hereditary amyloidosis (ATTR), with deposition of amyloid in the cardiac interstitium.
On gross pathologic examination, the heart is firm, rubbery, and noncompliant and has a waxy appearance.
Clinically significant cardiac involvement is uncommon in the secondary form. Focal deposits of amyloid in the hearts of elderly persons, although common, are usually clinically insignificant.
Four clinical presentations are seen:
(1) Diastolic dysfunction,
(3)Arrhythmias and conduction disturbances
The two-dimensional echocardiogram may be helpful in establishing the diagnosis of amyloidosis and may show a thickened myocardial wall with a diffuse, hyperrefractile "speckled" appearance.
CMRI typically shows late gadolinium enhancement of the subendocardium.
Aspiration of abdominal fat or biopsy of the myocardium or other organs permits the ante mortem diagnosis to be established in over three-quarters of cases.
Chemotherapy, often with alkylating agents such as melphalan, together with glucocorticoids, appears to have improved survival in individual cases.
Heart transplantation may help selected patients. However, the overall prognosis is poor, especially in the primary form with advanced cardiac involvement.
OTHER RESTRICTIVE CARDIOMYOPATHIES
Iron-overload cardiomyopathy is often the result of multiple transfusions or a hemoglobinopathy, most frequently -thalassemia; the familial form should be suspected if cardiomyopathy occurs in the presence of diabetes mellitus, hepatic cirrhosis, and increased skin pigmentation.
The diagnosis may be confirmed by endomyocardial biopsy.
CMRI shows a reduced signal as iron levels rise.
Phlebotomy may be of some benefit if employed early in the course of the disease.
Continuous subcutaneous administration of deferoxamine or other iron chelators may reduce body iron stores and result in clinical improvement.
Is generally associated with other manifestations of systemic disease.
It may cause restrictive as well as congestive features, since cardiac infiltration by sarcoid granulomas results not only in increased stiffness of the myocardium but also in diminished systolic contractile function.
A variety of arrhythmias, including high-grade AV block, have been noted.
A common cardiac manifestation of systemic sarcoidosis is RV overload due to
pulmonary hypertension as a result of parenchymal pulmonary involvement.
Many patients are treated empirically with glucocorticoids.
The carcinoid syndrome
It results in endocardial fibrosis and stenosis and regurgitation of the tricuspid and pulmonary valve; morphologically similar lesions have been seen with the use of the anorexic agents fenfluramine and phentermine
1. In Psora cardiac and emotional symptoms alternate
2. Heart affection may occur from fear, disappointment
3. Anxiety about death
4. The hammering sensation in the region of the heart, and many other uncomfortable sensations
5. The psoric patient may experience the rush of blood to the head
6. The psoric patient always think the heart action is about to stop and that he will die soon
7. He keeps his mind on his heart and constantly taking his own pulse
8. Bradycardia is psoric
9. Psoric dysponea is painful with features of cyanosis.
1. The heart is affected as a result of suppression of the rheumatic complaint
2. Sycosis has incoordination, dilatation and abnormality of the cardiac valves
3. Sycotic patient are generally fleshy and puffy and their dysponea is caused by obesity.
4. Sycotic cardiac pains are like an electric shock, which comes and goes suddenly
5. Sycotic patient suffers form fluttering, throbbing, with oppression and difficulty in breathing at intervals
6. Marked anascara and dropsical manifestation such as cardiac dropsy occur.
7. Tachycardia is sycotic
8. Thrombosis, embolism, cardiac attack in the circulation associated with the formation of embolus.
1. The patient is liable to suffer from the ulcerative bacterial endocarditis and heart affection with valvular degeneration
2. Congenital abnormalities
3. Sensation of heaviness in the percordium with lack of expression
4. Syphilitic cardiac problems are aggravated at night, from sunset, perspiration and extremes of temperature movement and from the warmth of the bed
5. Palpation which are tuberculo- syphilitic
6. Irregularity of pulse, irregularity of the rhythm
7. Lack of expression and realization and syphilitic do not convey their troubles to their friends, family or doctor
1. Palpitations and a rush of blood to the head and chest with redness of the face and flushed cheeks is charactersctic of the tubercular maism
2. Violent palpitations with beating and shaking of the whole body is representative of the tubercular miasm
3. The tubercular patient suffering from a heart condition wants to keep still
4. Tubercular condition are ameliorated by lying down and in open air
5. There is constant but gradual” falling away of the chest” and rush of blood in the chest and face in tubercular
6. Tubercular dysponea is painful
7. Persistent emaciation occurs in tubercular cardiac patient
HOMOEOPATHIC MEDICAL REPERTORY
Chapter : Heart
Rubric : Hypertrophy, heart
3 marks : ACON, AUR, AUR-I, BROM, CACT, CRAT, DIG, KALI-C, KALM, LITH, RHUS-T, SPIG, SPONG
2 marks : aml-n, arn, ars, aur-m, caust, conv, ferr, glon, graph, hep, iber, iod, lycps, naja, nat-m, phos, puls, verat-v.
1 mark : apis, aspar, bell, cer-b, chol, kali-bi, kali-f, lil-t, nux-v, phyt, plb, prun, stap, stroph, visc,
angina pectoris, chronic, with – NAJA
athletes, of uncomplicated - arn, brom, caust, rhus-t
dilation with - cact, chlol, kali-f, rhus-t
eccentric - apis
oedema pedum - cact, prun
exercise with - rhus-t.
A CONSCISE REPERTORY OF HOMOEOPATHIC MEDICINE
Chapter : Heart
Rubric : Hypertrophy
3 marks : LYC
2 marks : Acon, Aur, Bro, Cact, Iber, Kali-c, Kalm, Lith, Naj, Pru-sp, Thyr,
Compensatory : Adon
Gymnastics from : Bro, Rhus-t, Thyr.
Palpation, with : Bro.
Chapter : Chest
Rubric : Hypertrophy
Subrubric : Heart
3 marks : Acon, Aur, Aur-I, Cact, Kali-c, Kalm, Spong.
2 marks : Aml-ns, Arn, ARs, Aur-m, Brom, Caust, Conv, Ferr, Glon, Graph, Hep, Iber, Iod, Lil-t, Lyc, Nat-m, Phos, Rhus-t, Spig, Verat- v
1 mark : aethyl-n, aspar, aur-br, bell, brom, chlol, cimic, coffin, kali- bi , plb, prun, staph, stroph, stry-ar, thyr
Accompanied with hypertension : crat
Liver complication of the : mag
Numbness and tingling of left arm : Acon, Cimic, Puls, rhus-t
Overexertin from : arn, brom, calc, calc-p, chim
REPERTORY OF THE HOMOEOPATHIC MATERIA MEDICA
Chapter : Chest
Rubric : Hypertrophy of heart
3 marks : Acon, Cur, Aur-I, Cact, Kali-c, Kalm, Lilth, Spong
2 marks : amnl-n, arn, ars, aur-m, brom, dig, ferr, glon, graph, hep, iber, iod, lach, lyc, lycps, bnaja, nat-m, puls, rhus-t, spig.
1 mark : aspar, kali-bi, nux-v, plb, staph.
Numbness and tingling of left arm : Acon, Rhus-t
Over-exertion from : calc, caust, kali-c, rhus-t
BOGER BOENNINGHAUSENS CHARACTERISTICS AND REPERTORY
Chapter : chest
Sub section : heart and region of
Rubric : hypertrophy
2 marks : ars, bro, iod, kali-bi, pho, spo.,
· Affections of the heart with pain in left shoulder.
· Stitching pain in chest.
· Palpitation, with anxiety, fainting, and tingling in fingers.
· Pulse full, hard; tense and bounding; sometimes intermits.
· Temporal and carotid arteries felt when sitting.
· Sensation as if the heart stopped beating for two or three second immediately followed by a tumultuous rebound, with sinking at the epigastrium.
· Pulse rapid, feeble, irregular.
· High Blood Pressure - Valvular lesions of arterio-sclerotic nature (Aurum 30)
· Endocarditis with mitral insufficiency together with violent and rapid action.
· Acts best in the incipiency of cardiac incompetence.
· Heart weakness of arterio-sclerosis. Tobacco heart.
· Violent palpitation; worse lying on left side, at approach of menses.
· Angina pectoris, with suffocation, cold sweat, and ever-present iron band feeling.
· Pain in apex, shooting down left arm.
· Palpitation, with vertigo; dyspnoea, flatulence.
· Constriction; very acute pains and stitches in heart; pulse feeble, irregular, quick, without strength.
· Endocardial murmurs, excessive impulse, increased praecordial dullness, enlarged ventricle.
· Low blood pressure.
· Sensation as if heart were suspended.
· Palpitation and burning in heart region. Weak, rapid pulse; intermits, due to digestive disturbance.
· Threatened heart failure.
· Heart muscles seem flabby, worn out.
· Heart weakness with oppression, stitches and insomnia.
· Cardiac dyspnea. Cardiac dropsy.
· Extreme dyspnea on least exertion without much increase of pulse.
· Pain in region of heart and under left clavicle.
· Heart dilated, first sound weak. Valvular murmurs, angina pectoris.
· Sustains the heart in infectious diseases.
· Cardiac dilatation. Angina Pectoris. Pain under left scapula and under left clavicle.
· Attacks of angina worse raising arms.
· The least movement causes violent palpitations.
· Sudden sensation as if Heart stood still.
· Sensation as if heart would cease beating, if he moves, must hold the breath and keep still.
· Palpitations with depression from grief.
· Frequent stitches in heart. Irregular Heart especially with mitral valve disease.
· Cardiac failure following fevers.
· Heart tired after sprains.
· Dilated heart. Hypertrophy with dilatation.
· Cardiac dropsy. Pericarditis, copious serous exudation.
· Heart's action tumultuous, rapid and visible.
· Paroxysms of anguish around heart.
· Fluttering of heart with anxiety.
· Pains, sharp, burning shooting, stabbing, radiating to left scapula and arm, take away the breath.
· Palpitations, worse leaning forward.
· Tachycardia with pain.
· Sharp pains take away the breath. Shooting through chest above heart into shoulder-blades.
· Pulse slow, weak, tremulous. (Dig., Apoc.)
· Trembling and fluttering of the heart, extending to back, worse vexation.
· Sudden shock in heart.
· Throbbing, dull stitch in cardiac region.
· Pains in heart before menses and associated with pains in bladder and before urinating, better after.
· Pain in heart, extends to head.
· Hypertrophy from overexertion.
· Heart feels tired, pains go down left arm.
· Palpitations, worse when sitting still, better from walking.
· Trembling and palpitations when sitting still.
· Left arm aches with heart disease.
· violent palpitations, audible, attending other symptoms.
· Throbbing of carotids and subclavian arteries.
· Violent sticking or compressive pains, radiating to throat, arms, scapula,
· worse least motion or bending double.
· Frequent attacks of palpitations, especially with foul odor from mouth.
· Craving for hot water which relieves.
· The dry, chronic sympathetic cough or organic heart disease is relieved by Spongia.
· Rapid and violent palpitations with dyspnea, cannot lie down, also feels best resting in horizontal position.
· Awakened suddenly after midnight with pain and suffocation is flushed, hot and frightened to death.
· Blood surging up to neck, head and face with closure of eyelids and tears.
· Surging of heart into chest, as if it would force out upward.
· Hypertrophy of heart, especially right with asthmatic symptoms.
· Pulse frequent, hard, full or feeble.
· Aching pain and constriction around the heart.
· Tumultuous action of the heart.
· Fluttering of heart on least excitement.
· Full soft pulse.
· Violent beating of the heart and carotids with sense of constriction.
· The heart has an irregular, rumbling sort of sound.
· Strain of heart from violent running.
· Weakened heart muscles.
· Palpitations after any exertion.
· Beats shake the whole body.
· Stitches in heart.
· Heart pains, left to right.
· Horror of constant death with cardiac distress at night.
· Cardiac dropsy with distressing dyspnea.
· Heart pains into neck and occiput with anxiety, difficult breathing, fainting spells.
· Palpitations with anguish, worse lying on back, ascending stairs with slight causes.
· Palpitation, pain, dyspnea, faintness. Palpitation with tremulous weakness after stools. Beats audible.
· Visible pulsations. Weak heart, trembles.
· Irritable heart in smokers and tobacco chewers. Dilatation. Cyanosis.
· Heart feels loose on walking. Sensation as if heart stopped beating for two or three seconds, immediately followed by tumultuous rebound with sinking at the epigastrium.
· Oppression at the heart.
· Heart bruised, sore worse suppressed foot sweat.
· Palpitations compels him to stop. Violent palpitation, at puberty.
· Pulse rapid, feeble, irregular.
· It is useful for heart disorders.
· Feeling as if heart beat throughout the chest.
· Sensation as if heart ceased beating, then starting very suddenly.
· Palpitation from the least exertion.
· Tobacco heart, especially when due to cigarettes.
· Angina pectoris. Extremely rapid and irregular pulse.
· Palpitation worse least motion, better walking slowly of masturbators after loss of fluids.
· Sense of oppression. Anemic murmur.
· Heart suddenly bleeds into the Blood vessels and as suddenly draws a reflux, leaving pallor of surface.
· Pulse full, but soft and yielding, also, small and weak.
· Throbbing in all blood vessels.
· Fluttering. Palpitations with dyspnea.
· Violent palpitations, throbbing carotids.
· Heart seems full, quivers. Laborious action. Cannot go uphill. Pulsations in the whole body to the finger tips.
· Purring noise at the region of the heart. Cardiac pain radiates to all parts.
· Full tense pulse. Venous pulse. Boiling sensation in the region.
· As of an electric shock from heart to neck.
· Strong pulsations in the whole body.
· Palpitation of heart with anxiety with nosebleeds.
· Cold feeling in precordium. Pulse slows during day, fast in the morning
· Conscious of heart's action. On turning on left side, stitching pain as of needles through ventricles felt at each systole.
· Palpitations with vertigo and choking in throat. Stitching pains in cardiac region.
· PULSE full, irregular intermittent. Worse least motion and in warm room.
· Sensation of weight and pressure with occasional sharp, stinging pains.
· Dropsy with enlarged heart.
· Violent palpitations induced by slightest exertion or by laughing or coughing.
· Violent heart action.
· Feels as if squeezed by an iron hand (Cact.) followed by great weakness and faintness.
· Tachycardia. Palpitations, worse least exertion.
· Feeling of vibration or purring over heart. Tachycardia.
1. A CONCISE REPERTORY OF HOMOEOPATHIC MEDICINE
2. API TESXTBBOOK OF MEDICINE
3. BOGER BOENNINGHAUSENS CHARACTERISTIC REPERTORY
4. CHRONIC DISEASE BY SAMUEL HAHNEMANN
5. HOMOEOPATHIC MATERIA MEDICA – MURPHY
6. HOMOEOPATHIC MEDICAL REPERTORY- MURPHY
7. KUMAR AND CLARKE TEXT BOOK OF MEDICINE
8. MATERIA MEDICA OF HOMOEOPATHIC MEDICINE- S.R.PATHAK
9. ORGANON OF MEDICINE – B.K.SARKAR
10. OXFORD HAND BOOK ACUTE MEDICINE
11. PRINCIPLES AND PRACTICE OF MEDICINE – DAVIDSONS
12. PRINCIPLES OF INTERNAL MEDICINE – HARRISION
13. SYNTHESIS – FREDRICK SCHROYNES
14. THE CHRONIC MIASM