Download 天 津 医 科 大 学 授 课 教 案

天 津 医 科 大 学 授 课 教 案
（共 6 页、第 1 页）
课程名称：内科学
课程内容：心肌病
教师姓名： 李永乐 职称： 副主任医师 授课日期： 2013 年 4 月 25 日 10 时— 12 时
授课对象：国际学院系 10 年级
班（硕 本 专科）教材版本：内科学第二版
授课方式： 面授
学时数：2
听课人数：50
本单元或章节的教学目的与要求：
1. Familiar with definition and classifacation
2.Grasp pathophysiology, clinical features and treatment of different type of cardiomyopathy
授课主要内容及学时分配：
Etiologic classification and dilated cardiomyopathies
Restrictive, hypertrophic cardiomyopathies
50’
50’
重点、难点及对学生要求（包括掌握、熟悉、了解、自学）
pathophysiology ，clinical manifestation and treatment
外语词汇：
辅助教学情况：
Powerpoint, VCD
复习思考题：
1.
The pathophysiology and clinical features of restrictive ，hypertrophic cardiomyopathies?
2.
Clinical features of dilated cardiomyopathies？
参考资料：
Braunwald.Heart.Disease.8th
主任签字：
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天 津 医 科 大 学 授 课 教 案
（共 6 页、第 2 页）
CARDIOMYOPATHY
The cardiomyopathies are diseases that involve the myocardium primarily and are not the
result of hypertension or congenital, valvular, coronary, arterial, or pericardial abnormalities.
(l) a primary type: consisting of heart muscle disease of unknown cause;
(2) a secondary type: known cause, or associated with a disease involving other organ
systems.
Clinical classification of cardiomyopathies
l Dilated (congestive): Left and/or right ventricular enlargement, impaired systolic
function, congestive heart failure, arrhythmias, emboli
2 Restrictive: Endomyocardial scarring or myocardial infiltration resulting in restriction to
left and/or right ventricular filling.
3 Hypertrophic: Disproportionate left ventricular hypertrophy, typically involving septum
more than free wall, with or without an interentricular systolic pressure gradient; usually of a
nondilated left ventricular cavity
DILATED (CONGESTIVE) CARDIOMYOPATHY
Left and/or right ventricular systolic pump function is impaired,--- cardiac enlargement and
often producing symptoms of congestive heart failure. Mural thrombi are often present
Histologic examination : interstitial and perivascular fibrosis, minimal necrosis and cellular
infiltration.
.
Etiology: toxic, metabolic, or infectious agents. the late sequel of acute viral myocarditis,
mediated through an immunologic mechanism.
A small number of patients have familial forms of the disease; evidence of X-linked
inheritance.
Clinical manifestations:
(1)Symptoms of left- and right-sided congestive failure, dyspnea on exertion, fatigue,
orthopnea, paroxysmal nocturnal dyspnea, peripheral edema, and palpitations,
(2)left ventricular dilatation before becoming symptomatic.
(3)vague chest pain , angina pectoris unusual --- concomitant coronary artery disease.
Physical examination:
Variable degrees of cardiac enlargement and findings of congestive heart failure .
Advanced disease, the pulse pressure↓, and the jugular venous pressure↑. Third and
fourth heart sounds are common, and mitral or tricuspid regurgitation.
Laboratory examinations:
The chest roentgenogram: LV enlargement, cardiomegaly sometimes--concomitant
pericardial effusion. pulmonary venous hypertension and interstitial or alveolar edema.
.ECG: sinus tachycardia or atrial fibrillation, ventricular arrhythmias, left atrial enlargement,
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ST-T-wave abnormalities, interventricular conduction defects.
Echocardiography and radionuclide ventriculography: LV enlargement, with normal or
minimally thickened or thinned walls, and systolic dysfunction (EF↓); pericardial effusion.
Hemodynamic : LV end-diastolic,LA, and pulmonary capillary wedge pressures↑; RV
failure, RV end-diastolic, RV, and central venous pressures↑. dilated, diffusely hypokinetic LV,
with mitral regurgitation; the coronary arteries normal, excluding ischemic cardiomyopathy.
Endomyocardial biopsy: cell inflammation, --- inflammatory etiology ---previous viral
myocarditis.
Treatment:
downhill course, >55 years of age, die within 2 years. due to either CHF or ventricalar
arrhythmia; sudden death.
(1)exertion should be prevented.
(2)Systemic embolization is common,--- receive anticoagulants.
(3) Standard therapy of heart failure with salt restriction, diuretics, digitalis, and
vasodilators may produce symptomatic improvement, increased risk of digitalis toxicity.
(4) myocardial inflammation ---treated with corticosteroids, often in association with
azathioprine;
(5) treated cautiously with gradually increasing doses of beta-adrenergic blockers
Antiarrhythmic agents --- resistant / surgical interruption of the arrhythmic circuit /
implantation of an automatic internal defibrillator / cardiac transplantation
Alcoholic cardiomyopathy consume large quantities of alcohol over many years may
develop. stopping alcohol consumption--- may halt the progression, ---even reverse the course of
this disease, unlike the idiopathic variety. Treatment: total and permanent abstinence.
Peripartum cardiomyopathy Cardiac dilatation and congestive heart failure of unexplained
cause may develop during the last month of pregnancy or within the first few months after
delivery. The etiology unknown / preexisting cardiomyopathy not apparent prior to pregnancy.
Cardiac enlargement, mural thrombi,myocardial degeneration and fibrosis. The patient
typically multiparous, black, and over the age of 30. Symptoms, signs, and treatment are similar
to idiopathic dilated cardiomyopathy; pulmonary and systemic emboli are particularly common.
The mortality rate 25 to 50%. Prognosis related to whether the heart size returns to normal.
Avoid further pregnancies, particularly if cardiomegaly persists.
Drugs A variety of pharmacologic agents may damage the myocardium acutely, producing
a pattern of inflammation (myocarditis), or they may lead to chronic damage of the type seen
with idiopathic dilated cardiomyopathy.
RESTRICTIVE CARDIOMYOPATHY
The infiltrative diseases, also show some impairment of systolic function. Myocardial
天 津 医 科 大 学 授 课 教 案
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involvement with amyloid /hemochromatosis. glycogen deposition, endomyocardial
fibrosis, fibroelastosis, the eosinophilias, neoplastic infiltration, and myocardial fibrosis of
diverse causes.
Manifestation: elevated venous pressure,dependent edema, ascites, and an enlarged, tender
liver. jugular venous pressure, or it may rise with inspiration (Kussmaul's sign). heart sounds
may be distant, and third and fourth heart sounds are common. In contrast to constrictive
pericarditis, the apex impulse is usually easily palpable.
ECG: low-voltage, nonspecific ST-T-wave changes and various arrhythmias.
X-RAY: Pericardial calcification is absent ,which would suggest constrictive pericarditis,.
Echocardiography: symmetrically thickened LVwalls and normal or slightly reduced
systolic function.
Cardiac catheterization: decreased cardiac output, elevation of
RV,LV
end-diastolic pressures, and a dip-and-plateau configuration of the diastolic portion of the
ventricular pressure pulse resembling that seen in constrictive pericarditis.
Differentiation : constrictive pericarditis,
This distinction is of importance because the
latter curable by operation. right ventricular transvenous endomyocardial biopsy (by revealing
interstitial infiltration or fibrosisin restrictive cardiomyopathy) and computed tomography or
magnetic resonance imaging (by demonstrating a thickened pericardium in constrictive
pericarditis).
Endomyocardial fibrosis a progressive disease of unknown etiology that occurs
in
children and young adults residing in tropical and subtropical Africa, particularly Uganda and
Nigeria.
characterized by fibrous endocardial lesions of the inflow portion of the right or left
ventricle (or both)and often involves the artioventricular valves, producing valvular regurgitation.
The apex --- a mass of thrombus and fibrous tissue.
a frequent cause of heart failure in Africa,
accounting for up to one-quarter of deaths due to heart disease.
left-sided atrioventricular valve---symptoms of pulmonary congestion, while predominant
right-sided disease presents features of a restrictive cardiomyopathy.
Treatment: surgical excision of the fibrotic endocardium, replacement of the involved
atrioventricular valve
Eosinophilie endomyocardial disease Also called Loeffer's endocarditis and fibroplastic
endocarditis, a subcategory of the hypereosinophilic syndrome. the endocardium / underlying
myocardium.of either or both ventricles thickens markedly, Large mural thrombi.
Hepatosplenomegaly and localized eosinophilic infiltration of other organs are usually present.
Routine
management
with
digitalis,
diuretics,
afterload-reducing
agents,
anticoagulation as indicated, glucoconicoids and cytotoxic drugs .
HYPERTROPHIC CARDIOMYOPATHY: Left ventricular hypertrophy, nondilated
and
天 津 医 科 大 学 授 课 教 案
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chamber, without obvious antecedent cause.
(l) heterogeneous left ventricular (LV) hypertrophy, often with asymmetric septal
hypertrophy (ASH), the upper portion
(2) a dynamic left ventricular outflow tract pressure gradient, systolic anterior motion of
the mitral valve (SAM).
idiopathic
hypertrophic
subaortic
stenosis
(IHSS),
cardiomyopathy (HOCM), and muscular subaortic stenosis.
hypertrophic
obstructive
--- increased stiffness of the
hypertrophied muscle.
A ventricular septum whose thickness is disproportionately increased when compared with
the free wall./ disproportionate involvement of the apex or left ventricular free wall.
50% be transmitted as autosomal dominants with a variable degree of expression and
penetrance in close relatives. Echocardiogaphic studies have confirmed that about one-third of
the first-degree relatives occour.
SAM--- dynamic pressure gradient: (l) ventricular contractility↑, ventricular systolic
volume ↓and
ejection velocity of the blood ↑,SAM. (2) decreased ventricular volume
(preload), outflow tract↓ and (3) aortic impedance and pressure (afterload)↓, velocity of
flow through the subaortic area↑ -- ventricular systolic volume↓.
myocardial contractility↑--- gradient and the murmur.↑
Clinical features:
(1)asymptomatic --sudden death, frequently occurring in children and young adults, often
during or after physical exertion.
(2) dyspnea--stiffness of the left ventricular walls--impairs ventricular filling and leads to
elevated left ventricular diastolic and left atrial pressures.
(3) angina pectoris, fatigue, syncope, and near syncope ("graying-out spells").
a double or triple apical impulse, a rapidly rising carotid arterial pulse, and a fourth
heart sound.a systolic murmur, 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,
holosystolic and blowing in quality, the mitral regurgitation that usually accompanies obstructive
hypertrophic cardiomyopathy.
Laboratory evaluation:
(1)ECG: left ventricular hypertrophy and widespread, deep, broad Q waves that suggest an
old myocardial infarction but apparently ---abnormal electrophysiologic properties of the
hypertrophied septum. arrhythmias,
(2)Chest X-RAY: may be normal,
(3) echocardiogram: which demonstrates left ventricular hypertrophy , often with the septum
1.3 or more times the thickness of the high posterior left ventricular free wall. Unusual
天 津 医 科 大 学 授 课 教 案
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ground-glass appearance. The left ventricular cavity is typically small.
(4)Radionuclide scintigraphy with thallium 201 frequently reveals evidence of myocardial
perfusion defects even in symptomatic patients.
Treatment :
(1)Beta-adrenergic blockers:
(2) antiarrhtythmic agent: amiodarone appears to be effective in reducing the frequency
of supraventricular as well as life-threatening ventricular arrhythmias.
(3) Surgical myotomy/myectomy of the hypertrophied septum
(4) percutaneous transluminal myocardial ablation(PTSMA)
Digitalis, diuretics, nitrates, and beta-adrenergic agonists are best avoided if possible,
particularly in patients with known left ventricular outflow tract pressure gradiants.
Prognosis :variable . Atrial fibrillation. 10%Infective endocarditis , sudden death, are due
to ventricular arrhythmias . strenuous exercise should be avoided in all patients,
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