Cardiomyopathy is heart muscle disease, often of unknown cause. There are three types: dilated cardiomyopathy, Hypertropic obstructive cardiomyopathy and Restrictive cardiomyopathy.
Cardiomyopathy is heart muscle disease, often of unknown cause. There are three types: dilated cardiomyopathy, Hypertropic obstructive cardiomyopathy and Restrictive cardiomyopathy.
Cardiomyopathy is heart muscle disease, often of unknown cause. There are three types: dilated cardiomyopathy, Hypertropic obstructive cardiomyopathy and Restrictive cardiomyopathy.
Departemen Kardiologi & Kedokteran Vaskular FK USU RSUP H.Adam Malik
Definition of cardiomyopathy Cardiomyopathy is heart muscle disease, often of unknown cause. There are three types 1. Dilated cardiomyopathy 2. Hypertropic obstructive cardiomyopathy (HOCM) 3. Restrictive cardiomyopathy Dilated Cardiomyopathy The heart is dilated and has impaired function. The coronary arteries are normal. Probable causes of dilated cardiomyopathy include : Alcohol Viral infection (echovirus, coxsackievirus, and enteroviruses most likely) Untreated hypertension Autoimmune disease Thyrotoxicosis Drugs (cocaine, doxorubicin, cyclophosphamide, lead) Haemochromatosis Acquired immune deficiency syndrome (AIDS) Clinical features Progressive biventricular cardiac failure leads to : Fatigue Dyspnoea Peripheral oedema Ascites
Other complications secondary to the progressive dilatation of the ventricles include : Mural thrombi with systemic or pulmonary embolization Dilatation of the tricuspid and mitral valve rings leading to functional valve regurgitation Atrial fibrillation Ventricular tachyarrhthmias and sudden death Investigation Chest radiography This may show : Enlarged cardiac shadow Signs of pulmonary oedema (upper lobe blood diversion, interstitial shadowing at the bases) Pleural effusions
Electrocardiography Electrocardiography may highlight : Tachycardia Poor R wave progression across the chest leads Echocardiography Points to consider with echocardigraphy include : Can the dilated ventricles be easily visualized? Can the regurgitant valves be seen? Occasionally, intracardiac thrombus may be seen. Transthoracic echocardiography is not a reliable method for diagnosing this but it can be accurately diagnosed by transoesophageal echocardiography.
Cardiac catheterization This is important to exclude coronary artery disease (the most common cause of ventricular dysfunction).
Blood tests Viral titres may be useful and also thyroid function tests. Management The management plan follows four basic steps (the same applies for any other case of cardiac failure): 1. Search for and treat any underlying cause (e.g.stop alcohol) 2. Treat cardiac failure (diuretics, ACE inhibitors, nitrates) 3. Treat any arrhythmias (digoxin or amiodarone for atrial fibrillation, amiodarone for ventricular arrhythmias) 4. Anticoagulate with warfarin to prevent mural thrombi.
If the cardiac failure does not respond to the above steps and the patient is suitable candidate then cardiac transplantation is a possible option. Hypertrophic obstructive cardiomyopathy This disorder is characterized by asymmetrical hypertrophy of the cardiac septum-the cardiac septum is hypertrophied compared to the free wall of the left ventricle. Hypertrophic obstructive cardiomyopathy is inherited as an aotusomally dominant trait with equal sex incidence. The myocytes of the left ventricle are abnormally thick when examined microscopically.
65% 35% 10% www.kanter.com/hcm Clinical features
There are four main symptoms : 1. Angina (even in the absence of coronary artery disease)-due to the increased oxygen demands of the hypertrophied muscle 2. Palpitations-there is an increased incidence of atrial fibrillation and ventricular arrhythmias in this condition 3. Syncope and sudden death-which may be due to left ventricular outflow tract obstruction by the hypertrophied septum or to a ventricular arrhythmia 4. Dyspnoea-due to the stiff left ventricle, which leads to a high end-diastolic pressure and can therefore lead to pulmonary oedema.
It can be difficult to differentiate between HOCM and aortic stenosis on examination. Use the following features to help : Pulse slow rising in aortic stenosis, jerky or with a normal upstroke in HOCM. Thrill and murmur both found in the second right intercostal space in aortic stenosis and at the left lower sternal edge in HOCM. Variation of the murmur with Valsava manoeuvre this does not occur in aortic stenosis, but the murmur of HOCM is increased because the volume of the left ventricle is reduced by the manoeuvre and therefore the outflow obstruction worsens.
Diagnosis and investigations
Electrocardiography This is usually abnormal in HOCM. The most common abnormalities are T wave and ST segment abnormalities; the signs of left ventricular hypertrophy may also be present.
Continuous ambulatory electrocardiography
The presence of ventricular arrhythmias is common in patients who have HOCM and is a cause of sudden death. It is thought that the presence of ventricular arrhythmias on an ambulatory ECG monitor is a risk factor for sudden death and that an antiarrhythmic agent should be commenced. These tests are usually performed as part of a yearly screening programme for these patients.
Echocardiography This is the most useful investigation because it confirms the diagnosis and can be used to assess the degree of outflow tract obstruction. Characteristic echocardiography findings include : Asymmetrical hypertrophy of the septum. Abnormal systolic anterior motion of the anterior leaflet of the mitral valve. Left ventricular outflow tract obstrustion.
Prognosis
Children who are diagnosed when they are under 14 years of age have a poor prognosis and a high incidence of sudden death. Adults have a better prognosis, but they also have a higher mortality rate than the general population. Another outcome is progressive cardiac failure with cardiac dilatation.
Management Drug management As with aortic stenosis, vasodilators should be avoided because they worsen the gradient across the obstruction.Therefore patients who have HOCM should not receive nitrates. -blockers (-adrenoceptor antagonists) are used because their negative inotropic effect acts to decrease the contractility of the hypertrophied septum and reduce the outflow tract obstruction. Antiarrhythmic agents are important in patients who have ventricular and atrial arrhythmias. Dual-chamber pacing
This reduces the outflow tract gradient by pacing the heart from the right ventricular apex and therefore altering the pattern of septal motion. New devices incorporate both a dual-chamber pacemaker and a cardioverter defibrillator to treat malignant ventricular arrhythmias in patients with HOCM.
Surgery
Surgery is used only when all other treatments have failed. A myomectomy is performed on the abnormal septum. There is a new catheter technique to infarct the septum by occluding the septal artery.
Restrictive cardiomyopathy This is the least common of the cardiomyopathies in developed countries. The ventricular walls are excessively stiff and impede ventricular filling, therefore end-diastolic pressure is increased. The systolic function of the ventricle is increased. Possible causes of restrictive cardiomyopathy include : Storage diseases (e.g.glycogen storage diseases) Infiltrative diseases (e.g.amyloidosis, sarcoidosis) Scleroderma Endomyocardial diseases (e.g.endomyocardial fibrosis, hypereosinophilic syndrome, carcinoid)
Clinical features
The main features are : Dyspnoea and fatigue due to poor cardiac output Peripheral oedema and ascites Elevated jagular venous pressure with a positive Kussmauls sign (increase in jugular venous pressure during inspiration).
Management There is no specific treatment and the condition usually progresses towards death relatively quickly, most patients do not survive beyond 10 years after diagnosis.