Sarcoid cardiomyopathy

Introduction to sarcoidosis cardiomyopathy Sarcoidosis is a granulomatous inflammatory disease, nodular myocardium, which is mainly caused by invasion of lung parenchyma and involves multiple organs including lymph nodes, skin, joints, liver, kidney and heart. Cardiacsarcoidosis (cardiacsarcoidosis) is a disease caused by progressive granulomatous infiltration of myocardial infarction. It is more insidious in the past, and patients may be killed by complete atrioventricular block and/or congestive heart failure. Even sudden death as the first symptom has caused great concern in the medical community. basic knowledge Sickness ratio: 0.0012% Susceptible people: no special people Mode of infection: non-infectious Complications: arrhythmia, heart failure, sudden death

Cause of sarcoidosis cardiomyopathy

It is believed that the blood source, lymphatic source and local spread invade the heart, non-case granuloma is its basic lesion, mainly composed of epithelial cells, occasionally multinucleated giant cells, surrounded by a small number of lymphocytes, advanced patients, myocardial The dense, transparent-like connective tissue is replaced by a heart, and the heart is often enlarged. The affected part is mainly the conduction system and the myocardium. When the lesion is extensive, the aorta, pulmonary artery, endocardium or pericardium can be involved, and the small coronary artery is more susceptible, while the large coronary artery The artery is not invaded.

Myocardial lesions (20%):

The affected part is the conduction system (sinus node, atrioventricular node, atrioventricular bundle), left ventricular free margin, ventricular septum, especially the basal part, right ventricle, papillary muscle and right atrium. The left atrium is the rarest, and the myocardium can be present. Diffuse lesions, after treatment and control, form extensive fibrosis, affecting the overall ventricular function, focal lesions with a small range of lesions due to local scar formation, resulting in abnormal wall motion and involving cardiac function, following the above scars Formation, ventricular wall thinning, can form ventricular aneurysm, confined to the left ventricular wall, or diffusely occupy most of the right ventricular wall; can also involve the bilateral ventricular wall, some patients can trace ECG changes similar to myocardial infarction, It is thought that it is related to myocardium extensive granuloma and subsequent progressive myocardial fibrosis, leading to transmural destruction of the ventricle.

Pericardial lesions (20%):

Pericardial involvement is rare, only 3%, granulomatous lesions involving the pericardium, clinical detectives are mostly a small amount of pericardial effusion, can be bloody, sometimes recurrent pericardial effusion, and even cardiac tamponade or constriction Pericarditis.

Valvular lesions (10%):

Clinical manifestations of mitral valve prolapse or regurgitation may directly affect the valve function (3%) due to granulomas, or impaired papillary muscles (68%). The latter is often temporary, and severe cases may occur. Cause pulmonary hypertension, if the granuloma block the mitral valve mouth, it can cause the mitral valve blood flow to be blocked.

Nodular vasculitis - small coronary artery disease (5%):

Cardiac sarcoidosis has coronary microvascular disease such as extracorporeal microvascular spasm and vasculitis. Granuloma can cause secondary hyperplasia of this type of endovascular membrane and narrow the lumen.

Sarcoidic cardiomyopathy prevention

Early diagnosis and effective treatment are important factors in improving the prognosis of cardiac sarcoidosis.

Sarcoidic cardiomyopathy complications Complications, arrhythmia, heart failure, sudden death

There may be complications such as arrhythmia, heart failure, sudden death.

Symptoms of sarcoidosis cardiomyopathy common symptoms dyspnea dry cough tachycardia syncope lymphadenopathy chest pain heart failure atrioventricular block right heart failure supraventricular arrhythmia

1. Systemic symptoms of sarcoidosis Sarcoidosis is a systemic disease. Except for the heart, other organs, especially the lungs, lymph nodes, and skin, can be affected and have corresponding symptoms and signs, including fever, discomfort, anorexia, Weight loss, dry cough, wheezing, difficulty breathing, spot or papule-like rash and joint pain, etc. In addition, eye involvement is quite common, mostly manifested as uveal inflammation, involving the conjunctiva, retina, lacrimal gland can cause visual impairment, when nodules Patients with endotracheal lymphadenopathy with some acute peripheral arthritis, uveitis and nodular erythema lesions called "acute sarcoidosis" or Laeffgren syndrome; and anterior uveitis with mumps and facial nerve Paralyzed is called Heerfordt syndrome.

2. Clinical symptoms of nodular heart disease The severity of cardiac symptoms depends on the location and extent of granulomatous and scar tissue invasion.

(1) Congestive heart failure: the detection rate of heart enlargement due to granuloma infiltration is less than 5%. Large granuloma infiltrating myocardium can cause congestive heart failure, and about 25% of patients with cardiac dysfunction can progress progressively. Death, in addition, can be manifested as chronic right heart failure due to extensive lung disease with fibrosis, or chronic left heart failure due to myocardial fibrosis caused by left ventricular contraction and/or diastolic dysfunction.

(2) arrhythmia: can be manifested as slow or rapid arrhythmia, the former is caused by multiple granulomas directly invading the sinus node, the atrioventricular connective tissue and the bundle branch and other parts of the conduction system, more reports show that conduction Obstruction is the most common clinical manifestation of nodular heart disease, the incidence rate is 40%, of which the detection rate of complete atrioventricular block is 23% to 30%, about 68% of patients have syncope episodes, and even because of repeated - In the case of seizures and death, the damaged part is almost always the ventricular septum. In severe cases, the atrioventricular bundle can be completely replaced by granuloma or scar tissue, followed by complete bundle branch block, the incidence rate is 12%. ~32%, right bundle branch block is especially common, clinically seen complete right bundle branch block is the only manifestation of sarcoidosis, the sinus node is completely granulomatous, and does not involve the heart If other parts of the tissue are transmitted, the electrocardiogram can describe the sinus static pattern. It is believed that the actual number of cases involving the sinus node and the atrioventricular connective tissue is much higher than that of the clinical reporter due to the limitations of the technical conditions of the examination. Arrhythmia Often with ventricular arrhythmia, the incidence rate is 22% to 40%, can be ventricular premature contraction, often multi-source or ventricular tachycardia, complete atrioventricular block and ventricular tachycardia Appearance, and finally can cause sudden death, supraventricular arrhythmia is less common, common cases have atrial premature contraction, paroxysmal atrial tachycardia, atrial flutter and atrial fibrillation.

(3) angina pectoris: angina pectoris can occur in patients with nodular heart disease, reported typical or atypical angina, half of 201 (201Tl) perfusion defect and normal coronary angiography, and chest pain can be mostly nitrate drugs Partial or complete remission, suggesting that chest pain may be related to microvascular vasospasm, and other scholars believe that angina pectoris and 201 (201Tl) perfusion defect are caused by adventitia or ventricular wall, coronary granuloma, fibrosis or vasculitis. For patients diagnosed with sarcoidosis, the possibility of sarcoidosis cardiomyopathy should be considered. X-ray chest X-ray and ECG are helpful for diagnosis. If there is difficulty in diagnosis, endocardial myocardial biopsy may be feasible, but the material should be comprehensive. False negative, young people with dilated cardiomyopathy or persistent ventricular tachycardia should consider whether there is a possibility of sarcoidosis cardiomyopathy and a comprehensive examination. According to the diagnostic criteria set by Fleming for cardiac sarcoidosis:

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

2 clinical diagnosis of sarcoidosis.

3 histology confirmed as sarcoidosis.

Examination of sarcoidosis cardiomyopathy

1. X-ray chest radiograph: can show mild to moderate heart enlargement or right heart enlargement, congestive heart failure, pericardial effusion and left ventricular aneurysm, in addition, hilar lymphadenopathy can still be detected, Pulmonary parenchymal infiltration and reticular nodular shadows, the latter is an important marker of lung tissue involvement.

2. Electrocardiogram : More than 50% of patients with sarcoidosis have abnormal electrocardiogram including repolarization abnormality, arrhythmia, and even transmural myocardial infarction. Patients with confirmed or suspected cardiac sarcoidosis should have regular 24h dynamic electrocardiogram and Exercise ECG examination.

3. Echocardiography: especially suitable for the diagnosis of ventricular aneurysm, valvular insufficiency, mitral valve dysfunction caused by mitral dysfunction, left ventricular or ventricular septum (limited or whole heart) dyskinesia or hypokinesia, and left ventricular enlargement Echocardiographic changes in lesions, granulomatous lesions, and scar formation were echo enhancement.

4. Radionuclide myocardial imaging: 201 (201Tl) imaging showed local myocardial perfusion defects and/or corresponding wall segmental motion abnormalities, which can assess the extent of myocardial damage, but the above changes are not caused by other causes. Invasion of heart and cardiomyopathy, and seen in patients with myocardial ischemia changes, it is not specific, and now the clinical application of 201 (201Tl) and 201 gallium (201Ga) myocardial imaging joint examination, especially using single photon emission computer The SPECT method, supplemented by 201 (201Tl) imaging, reveals a special change sign of gallium by the gallium (67Ga) defect area, which can significantly increase its specificity. In addition, 111 indium ( 111In) The labeled anti-myosin antibody Fab fragment (AMAAB) imaging and magnetic resonance imaging (MRI) and the like all contribute to the establishment of a diagnosis.

5. Cardiovascular angiography and endocardial biopsy : Coronary angiography is particularly important in the diagnosis of coronary heart disease. Ventricular angiography can show changes in vascular filling defects and wall motion abnormalities caused by granuloma, and histological diagnosis of cardiac sarcoidosis The standard is: a large number of inflammatory cell infiltration without typical cheese-like granuloma, endocardial biopsy has become the only valid method for the diagnosis of a viable cardiac sarcoidosis, but the granuloma is mostly damaged by patchy or diffuse forms. The left ventricle is at the bottom of the heart, and the distribution is uneven, so it is difficult to accurately sample. The detection rate is only 25% to 50% of the clinical diagnosis. 6. Electrophysiological examination can be used as a refractory ventricular arrhythmia caused by cardiac sarcoidosis. An important basis for the use of drug therapy, 7. Other Kveim test is a skin test of the suspension from the spleen or lymph node tissue of patients with sarcoidosis as an antigen. Clinical application shows that there are fewer false positives and higher specificity. And angiotensin-converting enzyme (ACE) assay, about 81% of patients with active sarcoidosis have significantly increased blood ACE levels, not only for diagnosis, but also for assessing cortical Flag alcohol treatment.

Diagnosis and diagnosis of sarcoidosis cardiomyopathy

The disease needs to be differentiated from idiopathic dilated cardiomyopathy. Yazaki reported that 15 cases of cardiac sarcoidosis and 30 cases of idiopathic dilated cardiomyopathy showed that patients with cardiac sarcoidosis have different clinical special manifestations, mostly female. The incidence of severely conductive lesions is high, the wall is abnormally thickened, the wall motion abnormalities are mixed, the ventricular septum and apex are obvious before perfusion defect, and the prognosis is very poor, which is different from idiopathic dilated cardiomyopathy.

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