What Is Junctional Tachycardia?

Sarcoidosis is an inflammatory disease of granulomatous disease. The etiology is unknown. It mainly invades the lung parenchyma and involves multiple organs throughout the body, such as lymph nodes, skin, joints, liver, kidney, and heart. Patients may have sudden death due to complete atrioventricular block and / or congestive heart failure, and even sudden death is the first symptom.

Basic Information

English name: sarcoid heart disease
Visiting department: Cardiology

Common symptoms: Congestive heart failure, arrhythmia, angina pectoris, etc.
Contagious: no

Etiology of sarcoid cardiomyopathy

Non caseinous granulomas are mainly composed of epithelioid cells, occasionally multinucleated giant cells, and a small number of lymphocytes around them. It is caused by blood, lymphatic and local spread invasion to the heart. Myocardium in advanced patients can be replaced by dense hyaline connective tissue. The heart is enlarged. The affected area is mainly the conductive system and the myocardium. When the lesion is extensive, it can involve the aorta, pulmonary artery, endocardium, or pericardium.

Clinical manifestations of sarcoid cardiomyopathy

Systemic manifestations of sarcoidosis

Sarcoidosis is a systemic disease. Except for the heart, other organs, especially the lungs, lymph nodes, and skin can be affected. May have fever, malaise, anorexia, weight loss, dry cough, wheezing, dyspnea, spots or pimples-like rash, and joint pain. In addition, the eyes are mostly uveal inflammation; those involving the conjunctiva, retina, and lacrimal glands can cause visual impairment. When patients with sarcoidosis have paratracheal lymphadenopathy with some acute peripheral arthritis, uveitis, and nodular erythema, they are called acute sarcoidosis or Laeffgren syndrome; and anterior uveitis with mumps People with facial paralysis are called Heerfordt syndrome.

2. Nodular heart disease

The severity of cardiac symptoms depends on the location and extent of granuloma and scar tissue invasion.

(1) Congestive heart failure Massive granuloma infiltrates the myocardium can cause congestive heart failure, and a few patients with progressive worsening of heart dysfunction can cause death. In addition, it can still be manifested as chronic right heart failure due to extensive lung disease with fibrosis, or left ventricular contraction and / or diastolic dysfunction caused by myocarditis and myocardial fibrosis.

(2) Arrhythmia can be manifested as bradycardia or tachyarrhythmia. The former multiple-line granuloma is caused by direct invasion of the sinoatrial node, atrioventricular connecting tissue, and the conduction system of the bundle branch. Tachyarrhythmias are mostly ventricular arrhythmias, which can be premature ventricular contractions, often multi-source or ventricular tachycardia. Complete atrioventricular block and ventricular tachycardia appear alternately, which can eventually lead to sudden death. Supraventricular arrhythmias are rare. Common cases include preatrial contraction, paroxysmal atrial tachycardia, atrial flutter, and atrial fibrillation.

(3) Angina pectoris may occur in patients with nodular heart disease. Authors have reported typical or atypical angina pectoris, and chest pain can mostly be partially or completely relieved by nitrate drugs. .

Sarcoid cardiomyopathy examination

1.X-ray chest radiograph

Can show the heart showed mild to moderate enlargement or right heart enlargement, congestive heart failure, pericardial effusion, and left ventricular wall tumor. In addition, hilar lymphadenopathy, pulmonary parenchymal infiltration, and reticular nodule shadows can be detected, the latter being an important sign of lung tissue involvement.

2. ECG

More than half of sarcoidosis patients have abnormal electrocardiograms including repolarization abnormalities, arrhythmias, and occasionally transmural myocardial infarction-like changes. Patients with confirmed or suspected sarcoidosis should undergo routine 24-hour ambulatory electrocardiogram and exercise electrocardiogram.

3. Echocardiography

Particularly suitable for diagnosis of wall tumors, valvular insufficiency, mitral valve prolapse due to papillary muscle dysfunction, left ventricular or ventricular septal (limited or whole heart) dyskinesia or hypodymia, and left ventricular enlargement. Echocardiography of patients with granulomatous lesions and scarring changed to echo enhancement.

4. Radionuclide myocardial imaging

201 ( ²⁰¹ Tl) imaging shows local myocardial perfusion defect and / or abnormal movement of the corresponding wall segment, which can assess the degree of myocardial damage. The clinical application of 201 ( ²⁰¹ Tl) and 201 gallium ( ²⁰¹ Ga) myocardial imaging is used in clinical practice, especially using single-photon emission computed tomography (SPECT) method, supplemented by 201 ( ²⁰¹ Tl) imaging. The gallium ( ⁶⁷ Ga) defect area has a special sign of increased gallium reflection, which can significantly increase its specificity. In addition, 111 In ( ¹¹¹ In) labeled anti-myosin antibody Fab fragments (AMAAB) imaging and magnetic resonance imaging (MRI) can help establish the diagnosis.

5. Cardiovascular angiography

Coronary angiography is particularly important in the diagnosis of coronary heart disease. Ventricular angiography can show changes in vascular filling defects and abnormal wall motion caused by granulomas.

6. Endocardial biopsy

Endocardial biopsy has become the only viable method for the diagnosis of sarcoidosis in vivo. However, because granuloma mostly involves the patchy or diffuse form of the left ventricular heart, and the distribution is uneven, it is difficult to accurately sample.

7. Electrophysiological examination

Clinical can be an important basis for the treatment of refractory ventricular arrhythmias caused by sarcoidosis.

8. Other

The Kveim test is a skin test that uses a suspension extracted from the spleen or lymph node tissue of a sarcoidosis patient as an antigen. Clinical application shows that there are fewer false positives and higher specificity. In addition, the angiotensin-converting enzyme (ACE) measurement showed that the blood ACE level of approximately 81% of patients with active sarcoidosis was significantly increased, which was not only helpful for diagnosis, but also can be used as a marker for evaluating corticosteroid treatment.

Diagnosis of sarcoid cardiomyopathy

X-ray chest and ECG are helpful for diagnosis. When diagnosis is difficult, endocardial myocardial biopsy is feasible, but the material should be comprehensive. Young people with dilated cardiomyopathy or persistent ventricular tachycardia should be considered for sarcoid cardiomyopathy. The diagnosis criteria for sarcoidosis according to Fleming are: manifestations of sarcoidosis: conduction block; paroxysmal arrhythmia; heart failure; ST segment and T wave abnormalities. Clinically diagnosed as sarcoidosis. histology confirmed sarcoidosis.

Differential diagnosis of sarcoid cardiomyopathy

This disease needs to be distinguished from idiopathic dilated cardiomyopathy. Cardiac sarcoidosis patients have different clinical special manifestations. They are mostly females, with a high incidence of severe conductive lesions, abnormal thickening of the wall, and abnormal wall activity. Before the perfusion defect, the ventricular septum and the apex were obvious, and the prognosis was very poor, which were different from those with idiopathic dilated cardiomyopathy.

Sarcoid cardiomyopathy complications

Complications such as arrhythmia, heart failure, and sudden death can occur.

Sarcoid cardiomyopathy treatment

Corticosteroid

The drug of choice is to help the absorption of granulomas and prevent their progression to irreversible fibrotic lesions. It has been reported that patients receiving this drug have improved abnormal electrocardiogram, improved myocardial diastolic function, relieved myocardial damage, restored myocardial function to normal, slowed or eliminated abnormal myocardial perfusion, and ACE levels gradually became normal. The initial dose of prednisone should be 60 mg / day, which should be gradually reduced after a few weeks, and the final maintenance amount should be the minimum effective therapeutic dose. The medicine should be taken for a long time, and the medicine cannot be withdrawn at will.

2. Antiarrhythmic treatment

Sarcoidosis is often associated with hypercalcemia, and hypokalemia can be caused by corticosteroids, which can exacerbate the original arrhythmia. According to the patient's condition and type of arrhythmia, the corresponding effective antiarrhythmic drugs should be selected to reduce symptoms and reduce mortality.

3. Pacemaker

Patients with complete atrioventricular block and syncope or Alzheimer's syndrome onset should be implanted with a permanent pacemaker if they fail to respond to medication. At the same time, corticosteroids and other related drugs should still be combined. For patients with refractory ventricular tachyarrhythmias and at risk of sudden death, an implantable automatic cardioversion defibrillator (ICD) should be placed.

4. Surgery

Surgical removal of ventricular aneurysm can eliminate ventricular arrhythmias. Patients with systemic sarcoidosis should not receive organ transplants. Heart transplantation is feasible for young patients with simple sarcoidosis who are severely ill and have been trapped in end stage irreversible heart failure.

Prognosis of sarcoid cardiomyopathy

Ventricular tachyarrhythmia or conduction block and congestive heart failure are the main causes of death. Early diagnosis and effective treatment are important factors to improve the prognosis of sarcoidosis.