Associated professor Masyuta D. I. The primary cardiomyopathies cardiomyopathy is defined as the dysfunction of myocardium icon

Associated professor Masyuta D. I. The primary cardiomyopathies cardiomyopathy is defined as the dysfunction of myocardium




НазваAssociated professor Masyuta D. I. The primary cardiomyopathies cardiomyopathy is defined as the dysfunction of myocardium
Дата19.09.2012
Розмір445 b.
ТипДокументи


M.Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A.V., Ph.D. CARDIOMYOPATHIES

  • Associated professor Masyuta D.I.


THE PRIMARY CARDIOMYOPATHIES

  • Cardiomyopathy is defined as the dysfunction of myocardium.

  • Heart muscle disease, in the absence of

    • congenital heart disease,
    • hypertension,
    • acquired valve processes,
    • abnormal coronary arteries,
    • infection, or
    • other systemic illnesses,
  • is classified as a primary cardiomyopathy.





THE PRIMARY CARDIOMYOPATHIES

  • They often manifest with an insidious onset of

    • congestive heart failure,
    • chest pain,
    • dyspnea,
    • arrhythmias, or
    • sudden death.
  • The prevalence of cardiomyopathy in the newborn period is 10 in 100,000 live births, whereas for all children the prevalence is 36.5 per 100,000 for dilated cardiomyopathy and 2.5 per 100,000 for hypertrophic cardiomyopathy.



^ THE PRIMARY CARDIOMYOPATHIES

  • The two constant features of any variety of cardiomyopathy are:

    • Cardiomegaly without any significant murmur.
    • ECG changes in the form of -
      • Changes in the T wave - invariably inversion.
      • Changes in the ST segment.
      • Conduction defects - prolongation or shortening of the PR interval/
      • Rarely ventricular arrhythmias.






Hypertrophic Cardiomyopathy

  • This condition is also known as idiopathic hypertrophic subaortic stenosis or asymmetric septal hypertrophy.

  • Massive ventricular hypertrophy with principal involvement of the ventricular septum characterizes the disease, but all portions of the left ventricle, and sometimes of the right ventricle, are affected.

  • Varying degrees of myocardial fibrosis are also present.













Pathophysiology

  • The mitral valve is displaced anteriorly by hypertrophy of the papillary muscles, and the left ventricular cavity is distorted by the massive generalized hypertrophy.

  • An excessive response to calcium or to excessive calcium ion channels may be responsible for myocyte hypertrophy in some patients.



Pathophysiology

  • The hypertrophic, fibrosed, stiff muscle has a decreased distensibility so that there is resistance to left ventricular filling, but systolic pumping function remains intact (or even hyperdynamic) until late in the course of the disease.

  • Obstruction to left ventricular outflow may develop owing to apposition of the abnormally placed anterior mitral leaflet against the hypertrophied septum.

  • Varying degrees of mitral valve insufficiency are common.



Epidemiology

  • Hypertrophic cardiomyopathy has been recognized in all age groups and may occur in many members of the same family.

  • In childhood, hypertrophic cardiomyopathy may be somewhat different from the adult disease.

  • There is a greater tendency for right ventricular outflow obstruction to occur

  • The left ventricular wall may be diffusely thickened, as opposed to only the septal portion, and a pure autosomal dominant inheritance pattern is less often seen.



Epidemiology

  • In infants of diabetic mothers, a transient form of hypertrophic cardiomyopathy may be encountered with or without left ventricular outflow tract obstruction. The increased left ventricular mass usually regresses within several months.

  • Premature infants who are receiving corticosteroids for chronic lung disease may also develop a transient hypertrophic cardiomyopathy, which resolves rapidly with cessation of steroid therapy.



Clinical manifestations

  • Many children are asymptomatic and are first evaluated only because of a heart murmur of mitral regurgitation.

  • In others the clinical pattern is dominated by

    • weakness,
    • fatigue,
    • dyspnea on effort,
    • palpitations,
    • angina pectoris,
    • dizziness, and
    • syncope.


Clinical manifestations

  • There is risk of sudden death even in asymptomatic children.

  • The pulse is brisk because of the early systolic ejection of blood from the ventricle.

  • There is a prominent left ventricular lift and double apical impulse.



Clinical manifestations

  • The 1st and 2nd heart sounds are usually normal.

  • The rarity of systolic ejection clicks helps to differentiate hypertrophic obstructive cardiomyopathy from valvular aortic stenosis.

  • The systolic murmur is ejection in type and of medium intensity.

  • It is heard maximally at the left sternal edge and apex.

  • The murmur may increase shortly after exercise is discontinued, during the Valsalva maneuver, or during assumption of the erect position.







Diagnosis

  • The electrocardiogram shows left ventricular hypertrophy with or without ST segment depression and T wave inversion.

  • Signs of the Wolff-Parkinson-White syndrome and other intraventricular conduction defects may be present.

  • Roentgenograms show mild cardiomegaly with prominence of the left ventricle.



Diagnosis

  • The echocardiogram shows

    • asymmetric left ventricular hypertrophy, predominantly affecting the interventricular septum;
    • systolic anterior motion of the anterior leaflet of the mitral valve; and
    • premature closure of the aortic valve.
  • Sometimes symmetric left ventricular hypertrophy occurs.

  • Doppler studies will demonstrate the presence of a left ventricular outflow tract gradient, which usually occurs in mid to late systole, when the muscular obstruction to the outflow is maximal.



Prognosis

  • The prognosis of hypertrophic cardiomyopathy is unpredictable, especially in the asymptomatic patient, who may remain stable for years.

  • Some patients will progress to chronic congestive heart failure, and others are at risk for sudden death caused by arrhythmia.



Treatment

  • There is no standardized therapy.

  • Competitive sports and strenuous physical activity should be discouraged.

  • Digitalis is contraindicated in most patients.

  • Aggressive diuresis or the infusion of isoproterenol or other inotropic agents should also be avoided.



Treatment

  • -Adrenergic blocking agents (propranolol) and calcium channel blocking agents (verapamil) have been used with some apparent success in decreasing the degree of outflow obstruction, but obliteration of a left ventricular outflow tract gradient does not necessarily affect the long-term prognosis.

  • Calcium channel blockers should not be used during infancy.

  • A pacemaker has been used in some older patients.



Treatment

  • Surgical ventricular septal myotomy or resection of the left ventricular outflow tract has been successfully accomplished in some patients, especially in those with disabling angina or syncope and in some with severe obstruction at rest (gradient exceeding 70 mm Hg).

  • Mitral valve replacement may be needed if obstruction cannot be alleviated.



Idiopathic Dilated Cardiomyopathy

  • This condition is characterized by massive cardiomegaly as a result of the extensive dilatation of the ventricles, most prominently the left.

  • Varying degrees of ventricular hypertrophy are also present.



Etiology

  • The etiology is unknown and is probably multifactorial.

  • A remote history of viral disease in some patients suggests that the disease may be a sequela of a previous myocarditis.

  • Patients with dilated cardiomyopathy may have carnitine deficiency; thus, urine and serum levels should be obtained.



Etiology

  • Genetic mitochondrial disease and other metabolic abnormalities affecting the myocardium may also result in dilated cardiomyopathy as a final common pathway.

  • X-linked dilated cardiomyopathy usually presents during adolescence in male patients and has been found to be associated with a mutation in the dystrophin gene.



Clinical manifestations

  • All age groups may be affected.

  • Usually the onset is insidious, but sometimes symptoms of congestive heart failure occur suddenly.

    • Irritability,
    • anorexia,
    • cough owing to pulmonary congestion, and
    • dyspnea with mild exertion
  • are common.



Clinical manifestations

  • When the disease is fully established,

    • the skin is cool and pale,
    • the blood pressure is decreased,
    • the pulse pressure is reduced, and
    • tachycardia is present.
  • The jugular venous pressure is increased, and

  • hepatomegaly and edema are common.



Clinical manifestations

  • The heart is enlarged, and

  • holosystolic murmurs of mitral and tricuspid insufficiency from severe dilatation of the valve annuluses may be present.

  • A summation gallop rhythm is usually audible.





Idiopathic Dilated Cardiomyopathy



Diagnosis

  • The electrocardiogram shows

    • a combination of atrial enlargement,
    • varying degrees of left ventricular hypertrophy, and
    • nonspecific T wave abnormalities.
  • The roentgenogram confirms the cardiomegaly.

  • Pulmonary congestion and pleural effusions may also be present.



Several cardiac abnormalities co-existing. Note enlarged heart







Diagnosis

  • The echocardiogram shows dilatation of the left atrium and ventricle, and poor contractility.

  • The right ventricle may also be affected.

  • Doppler studies show decreased flow velocity through the aortic valve and mitral regurgitation.

  • In long-standing cases, evidence of pulmonary hypertension may exist.



Prognosis

  • The course of the disease is usually progressively downhill, although some patients may remain stable for years.

  • Vigorous treatment for heart failure may result in a temporary remission, but relapses are common, and in time patients tend to become resistant to therapy.

  • At this point, the prognosis for survival beyond a year is poor.



Management

  • Inotropic agents are used to improve the left ventricular function.

  • Vasodilators are used to unload the left ventricle.

  • Diuretics are used to decrease preload.

  • Anti-arrhythmic medications are used for rhythm disturbances.



Management

  • Cardiac transplantation has been used very successfully in this group of patients as well as in patients with other forms of cardiomyopathy.

  • Serious complications include arrhythmias as well as pulmonary and/or systemic emboli from intracardiac thrombi.

  • Patients with severely depressed myocardial function should receive systemic anticoagulation with warfarin.



Restrictive cardiomyopathies

  • Poor ventricular compliance is the major abnormality, and inadequate filling of the ventricular cavities occurs during diastole.

  • It is a less common disorder than other types of cardiomyopathies.



Clinical Manifestations

  • They closely simulate those of constrictive pericarditis.

  • In its full-blown these form, restrictive cardiomyopathy results in

    • dyspnea,
    • edema,
    • ascites,
    • hepatomegaly,
    • increased venous pressure, and
    • pulmonary congestion.
  • The heart is mildly or moderately enlarged, and murmurs are nonspecific.



Clinical Manifestations

  • The electrocardiogram shows

    • prominent P waves,
    • often normal QRS voltage,
    • ST segment depression, and
    • T wave inversion.
  • Roentgenographic examination shows mild to moderate cardiomegaly.



Treatment

  • Treatment is directed toward relief of edema with diuretics.

  • Calcium channel blocking agents may be used to increase diastolic compliance.

  • Cardiac transplantation is the last management option.

  • Anticoagulant therapy may reduce the incidence of thromboembolism.



Treatment

  • Surgical dissection of affected endocardium and valvuli with their subsequent prosthetics is more effective, but prognosis even after successful operation often unfavorable.

  • The prognosis for restrictive cardiomyopathy is generally poor.





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