Contents 1 Overview 2 Disease name 3 English name 4 Alias ??of cardiac sarcoidosis 5 Classification 6 ICD number 7 Epidemiology 8 Causes of sarcoid cardiomyopathy 9 Pathogenesis 9.1 Myocardial lesions 9.2 Pericardial lesions 9.3 Valvular disease 9.4 Nodular vasculitis small coronary artery disease 10 Clinical manifestations of sarcoid cardiomyopathy 10.1 Systemic symptoms of sarcoidosis 10.2 Clinical symptoms of sarcoid heart disease 10.2.1 Congestive heart failure 10.2.2 Heart rhythm Disorders 10.2.3 Angina pectoris 11 Complications of sarcoid cardiomyopathy 12 Auxiliary examinations 12.1 Chest X-ray 12.2 Electrocardiogram 12.3 Echocardiography 12.4 Radionuclide myocardial imaging 12.5 Cardiovascular angiography and endocardial biopsy 12.6 Electrophysiological examination 12.7 Others 13 Diagnosis of sarcoid cardiomyopathy 14 Differential diagnosis 15 Treatment of sarcoid cardiomyopathy 15.1 Corticosteroids 15.2 Correction of arrhythmia 15.3 Pacemaker 15.4 Surgery 16 Prognosis 17 Prevention of sarcoid cardiomyopathy 18 Related Drug attachments: 1 Cardiac sarcoidosis-related drugs This is a redirect entry that shares content about sarcoidosis cardiomyopathy. For the convenience of reading, sarcoid cardiomyopathy in the following text has been automatically replaced by cardiac sarcoidosis. You can click here to restore the original appearance, or use notes to display 1 Overview

Sarcoidosis (sarcoidosis) is the cause It is an unknown, granulomatous inflammatory disease that mainly invades the lung parenchyma and involves multiple organs throughout the body, including lymph nodes, skin, joints, liver, kidneys, and heart. Nodular cardiomyopathy (cardiac sarcoidosis) is a disease of cardiac function caused by progressive granulomatous infiltration of the myocardium. The clinical course during life is relatively insidious. Patients may suffer from complete atrioventricular block and/or congestive heart failure. Sudden death due to heart failure, or even sudden death as the first symptom, has attracted great attention from the medical community.

Corticosteroids are the first-choice drugs for the treatment of nodular heart disease. They help the absorption of granulomas and prevent their progression into irreversible fibrotic lesions. Sarcoidosis is often associated with hypercalcemia, and corticosteroid treatment can cause hypokalemia, which can aggravate the original arrhythmia. At this time, it is necessary to select corresponding effective antiarrhythmic drugs according to the patient's condition and type of arrhythmia to alleviate symptoms and reduce mortality. Patients with complete atrioventricular block and syncope or Aspen syndrome attacks who are ineffective after drug treatment should be implanted with a permanent pacemaker. Surgical removal of ventricular aneurysms eliminates ventricular arrhythmias. Patients with systemic sarcoidosis are not suitable for organ transplantation. Young patients with simple sarcoid cardiomyopathy who are seriously ill and have fallen into end-stage irreversible heart failure can be treated with heart transplantation.

Early diagnosis and effective treatment are important factors in improving the prognosis of sarcoid cardiomyopathy. 2 Disease name

Cardiac sarcoidosis 3 English name

cardiac sarcoidosis

4 Alias ??of cardiac sarcoidosis

Nodular Cardiomyopathy; sarcoid cardiomyopathy 5 categories

Cardiovascular Medicine> Cardiomyopathy 6 ICD number

I43.8﹡ 7 Epidemiology

Prevalence Pathology shows that the incidence of lesions involving the heart is 20% to 25%. This disease mostly occurs in people aged 20 to 40 years old, and is slightly more common in women. 8 The cause of cardiac sarcoidosis

The cause of sarcoidosis is currently unknown.

9 Pathogenesis

It is now believed that the disease originates from the menstrual blood source, lymphatic source and local diffusion and invades the heart. Non-caseating granuloma is the basic lesion of cardiac sarcoidosis. Mainly composed of epithelial-like cells, occasionally multinucleated giant cells, surrounded by a small number of lymphocytes. In advanced patients, the myocardium may be replaced by dense, hyaline-like connective tissue. The heart often enlarges, and the affected parts are mainly the conduction system and myocardium. When the disease is extensive, it can involve the aorta, pulmonary artery, endocardium, or pericardium. Small coronary arteries are more likely to be affected, while large coronary arteries are not affected. 9.1 Myocardial lesions

The affected parts are the conduction system (sinoatrial node, atrioventricular node, atrioventricular bundle), left ventricular free edge, interventricular septum, especially the base, right ventricle, and *** muscle. and the right atrium, with the left atrium being the least common. The myocardium can be diffusely damaged, and after treatment and control, extensive fibrosis can occur, affecting overall ventricular function. Focal damage with a smaller range of lesions may cause abnormal ventricular wall segmental motion and affect cardiac function due to local scarring. Following the above-mentioned scarring, the ventricular wall becomes thinner and aneurysms may form. It is limited to the left ventricular wall, or diffusely occupies most of the right ventricular wall; it can also involve both ventricular walls at the same time. Some patients with cardiac sarcoidosis may have ECG changes similar to those seen in myocardial infarction, which are thought to be related to extensive myocardial granuloma and subsequent progressive myocardial fibrosis, leading to transmural destruction of the ventricles. 9.2 Pericardial lesions

Pericardial involvement is rare, only 3%, and is caused by granulomatous lesions involving the pericardium. Most of the clinical findings are a small amount of pericardial effusion, which may be bloody, and sometimes present with recurrent pericardial effusion. Even cardiac tamponade or constrictive pericarditis occurs. 9.3 Valvular disease

The clinical manifestation is mitral valve prolapse or insufficiency, which can directly damage the valve due to granuloma (3%), or indirectly affect valve function after damage to the vaginal muscles (68%) . The latter is often temporary, and in severe cases can occasionally cause pulmonary hypertension. If the granuloma blocks the mitral valve orifice, it may cause obstruction of mitral valve blood flow. 9.4 Nodular vasculitis and small coronary artery lesions

Cardiac sarcoidosis involves coronary microvascular lesions such as extraventricular microvascular spasm and vasculitis. Granuloma can cause secondary proliferation of the intima of such blood vessels and narrow the lumen. 10 Clinical manifestations of cardiac sarcoidosis 10.1 Systemic symptoms of sarcoidosis

Sarcoidosis is a systemic disease. In addition to the heart, other organs, especially the lungs, lymph nodes, skin, etc., can be affected and manifested. There are corresponding symptoms and signs. These include fever, malaise, anorexia, weight loss, dry cough, wheezing, dyspnea, spotted or papular rash, and joint pain. In addition, eye involvement is quite common, often manifesting as uveal inflammation. Those involving the conjunctiva, retina, and lacrimal glands can cause visual impairment. When paratracheal lymphadenopathy is detected in patients with sarcoidosis and is accompanied by some acute peripheral arthritis, uveitis, and erythema nodosum, it is called "acute sarcoidosis" or Laeffgren's syndrome; while anterior uveitis with parotid gland People with inflammation and facial nerve paralysis are called Heerfordt syndrome. 10.2 Clinical symptoms of nodular heart disease

The severity of cardiac symptoms depends on the location and extent of granuloma and scar tissue invasion. 10.2.1 (1) Congestive heart failure

The detection rate of cardiac enlargement due to granulomatous infiltration is less than 5%. Large granulomas infiltrating the myocardium can cause congestive heart failure. About 25% of patients with cardiac insufficiency may progress to death. In addition, it can still manifest as chronic right heart failure caused by extensive lung disease accompanied by fibrosis, or chronic left heart failure caused by left ventricular systolic and/or diastolic dysfunction caused by myocarditis and myocardial fibrosis. 10.2.2 (2) Arrhythmia

Arrhythmia can manifest as bradycardia or tachyarrhythmia. The former is mostly caused by granulomas directly invading the conduction system of the sinoatrial node, atrioventricular connecting tissue, and bundle branches. Many reports show that conduction disorder is the most common clinical manifestation of nodular heart disease, with an incidence rate of 40%. Among them, the detection rate of complete atrioventricular block is 23% to 30%, and about 68% of patients have it. Syncope and even death due to repeated attacks of Aspen syndrome. The damaged part is almost always the base of the interventricular septum. In severe cases, the atrioventricular bundle may be completely replaced by granuloma or scar tissue. The second is complete bundle branch block, with an incidence rate of 12% to 32%, and right bundle branch block is particularly common.

Clinical reports have shown that complete right bundle branch block is the only manifestation of cardiac damage in sarcoidosis. When the sinus node is completely involved by granuloma and does not involve other parts of the heart's conductive tissue, the electrocardiogram can depict a sinus resting pattern. It is now believed that infants and young children are subject to inspection technical conditions. Therefore, the actual number of cases of sinus node and atrioventricular connecting tissue involvement is much higher than clinical reports.

The majority of tachyarrhythmias are ventricular arrhythmias, with an incidence rate of 22% to 40%. It can be premature ventricular contractions, often in the form of polygenic or ventricular tachycardia. Complete atrioventricular block and ventricular tachycardia occur alternately, and can eventually lead to sudden death. Supraventricular arrhythmias are less common, and common ones include premature atrial contractions, paroxysmal atrial tachycardia, atrial flutter, and atrial fibrillation. 10.2.3 (3) Angina pectoris

Angina pectoris may occur in patients with cardiac sarcoidosis. It has been reported that half of the patients with typical or atypical angina pectoris have 201thallium (201Tl) perfusion defect but normal coronary angiography, and Chest pain is often partially or completely relieved by nitrates, suggesting that chest pain may be related to microvascular spasmodic ischemia. Other scholars believe that angina pectoris and 201 thallium (201Tl) perfusion defect are caused by adventitia or ventricular wall, coronary granuloma, fibrosis or vasculitis. 11 Complications of cardiac sarcoidosis

Cardiac sarcoidosis can cause complications such as arrhythmia, heart failure, and sudden death. 12 Auxiliary examinations 12.1 Chest X-ray

It can show mild to moderate heart enlargement or right heart enlargement, congestive heart failure, pericardial effusion, left ventricular aneurysm and other changes. In addition, hilar lymphadenopathy, lung parenchymal infiltration, and reticular nodule shadows can still be detected, the latter being important signs of lung tissue involvement. 12.2 Electrocardiogram

More than 50% of patients with sarcoidosis have abnormal electrocardiograms including repolarization abnormalities, arrhythmias, and occasionally transmural myocardial infarction-like changes. Patients with confirmed or suspected sarcoid cardiomyopathy should routinely undergo 24-hour dynamic electrocardiography and exercise electrocardiography. 12.3 Echocardiography

It is particularly suitable for diagnosing ventricular aneurysms, valvular insufficiency, mitral valve prolapse caused by *** muscle dysfunction, left ventricular or ventricular septal (limited or global) dyskinesia, or Hypomotility, left ventricular enlargement and other lesions. The echocardiographic changes of patients with granulomatous lesions and scarring are echogenicity. 12.4 Radionuclide myocardial imaging

201 thallium (201Tl) imaging shows local myocardial perfusion defects and/or corresponding abnormal wall segment motion, which can evaluate the degree of myocardial damage. However, the above changes are not only seen in cardiac infiltration and cardiomyopathy caused by other causes, but also in patients with myocardial ischemia changes, so they are not specific. Nowadays, 201 thallium (201Tl) and 201 gallium (201Ga) combined myocardial imaging are commonly used in clinical examination, especially the single photon emission computed tomography (SPECT) method, supplemented by 201 thallium (201Tl) imaging, through 67 gallium ( 67Ga) Gallium photography in the defect area shows increased special signs of change, which can significantly improve its specificity. In addition, 111In (111In)-labeled anti-myosin antibody Fab fragment (AMAAB) imaging and magnetic resonance imaging (MRI) are helpful in establishing the diagnosis. 12.5 Cardiovascular angiography and endocardial biopsy

Coronary angiography is particularly important in excluding the diagnosis of coronary heart disease. Ventriculography can show changes such as vascular filling defects and abnormal ventricular wall motion caused by granulomas. The histological diagnostic criteria for cardiac sarcoidosis are massive inflammatory cell infiltrates without typical caseating granulomas. Endocardial biopsy has become the only feasible diagnostic method for sarcoid cardiomyopathy in vivo. However, because granulomas mostly damage the bottom of the left ventricle in patchy or diffuse forms, and are unevenly distributed, accurate sampling is difficult, and the detection rate is only 25% to 50% of clinical diagnosis. 12.6 Electrophysiological examination

It can be clinically used as an important basis for drug treatment in patients with refractory ventricular arrhythmias caused by cardiac sarcoidosis. 12.7 Others

Kveim test is a skin test using a suspension extracted from the spleen or lymph node tissue of sarcoidosis patients as an antigen. Clinical application shows that there are few false positives and high specificity.

Angiotensin-converting enzyme (ACE) is also measured. About 81% of patients with active sarcoidosis have significantly elevated blood ACE levels, which not only helps in diagnosis, but can also be used as a marker to evaluate corticosteroid treatment. 13 Diagnosis of cardiac sarcoidosis

The possibility of cardiac sarcoidosis should be considered in patients diagnosed with sarcoidosis. Chest X-ray and electrocardiogram are helpful in diagnosis. When diagnosis is difficult, endocardial myocardial biopsy can be performed, but the sample should be comprehensive to prevent false negatives. Young people with dilated cardiomyopathy or sustained ventricular tachycardia should consider the possibility of sarcoid cardiomyopathy and undergo a comprehensive examination.

According to the diagnostic criteria set by Fleming, cardiac sarcoidosis is:

① Symptoms of cardiac sarcoidosis: conduction block; paroxysmal arrhythmia; heart failure; ST segment and T wave abnormalities.

②Clinically diagnosed as sarcoidosis.

③ Histologically confirmed sarcoidosis. 14 Differential diagnosis

Cardiac sarcoidosis needs to be differentiated from idiopathic dilated cardiomyopathy. Yazaki reported the results of 15 cases of sarcoid cardiomyopathy and 30 cases of idiopathic dilated cardiomyopathy. Patients with arthropathic cardiomyopathy have different clinical manifestations, most of whom are women. They have a high incidence of severe conduction lesions, abnormal ventricular wall thickening, varying abnormality of ventricular wall activity, perfusion defects that are obvious in the ventricular septum and apex, and poor prognosis. Extremely poor, all different from those with idiopathic dilated cardiomyopathy. 15 Treatment of cardiac sarcoidosis 15.1 Corticosteroids

Corticosteroids are the drugs of choice for the treatment of nodular heart disease. They help the absorption of granulomas and prevent their progression into irreversible fibrotic lesions. It has been reported that patients treated with this drug have improved abnormal electrocardiograms, improved myocardial diastolic function, alleviated myocardial damage, returned viable myocardial function to normal, slowed or eliminated abnormal myocardial perfusion, and gradually normalized ACE levels. The initial dose of prednisone should be 60 mg/d, and the dose should be gradually reduced after a few weeks. The final maintenance dose should be the lowest effective therapeutic dose. The medication should be taken for a long time and cannot be withdrawn at will. 15.2 Correcting arrhythmia

Sarcoidosis is often complicated by hypercalcemia, and corticosteroid treatment can cause hypokalemia, which can aggravate the original arrhythmia. At this time, it is necessary to select corresponding effective antiarrhythmic drugs according to the condition and type of arrhythmia of patients with cardiac sarcoidosis to alleviate symptoms and reduce mortality. 15.3 Cardiac pacemaker

Patients with complete atrioventricular block and syncope or Aspen syndrome attack who are ineffective after drug treatment should be implanted with a permanent pacemaker. At that time, corticosteroids and other related drugs should still be given according to the needs of the condition. If the patient has refractory ventricular tachyarrhythmia and is at risk of sudden death, an implantable automatic cardioverter defibrillator (AICD) should be installed. 15.4 Surgery

Surgical removal of ventricular aneurysms can eliminate ventricular arrhythmias. Patients with systemic sarcoidosis are not suitable for organ transplantation. Young patients with simple sarcoid cardiomyopathy who are seriously ill and have fallen into end-stage irreversible heart failure can be treated with heart transplantation. 16 Prognosis

Roberts believes that once cardiac symptoms and signs appear, the survival period of nodular heart disease is only 2 years. However, Fleming disputed this. Among the 270 patients with sarcoid cardiomyopathy reported by him, more than 100 cases had a survival period of more than 5 years, 36 cases had a survival period of more than 10 years, and 7 cases had a survival period of more than 20 years. Ventricular tachyarrhythmia or conduction block and congestive heart failure are the main causes of death. Early diagnosis and effective treatment are important factors in improving the prognosis of sarcoid cardiomyopathy. 17 Prevention of cardiac sarcoidosis

Treat sarcoidosis early to prevent heart disease. 18 Related drugs

Prednisone Cardiac sarcoidosis related drugs Cortisone acetate injection

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