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Lev's Disease
Lev's disease is an acquired complete heart block due to idiopathic fibrosis and calcification of the electrical conduction system of the heart. Lev's disease is most commonly seen in the elderly, and is often described as senile degeneration of the conduction system. One form has been associated with SCN5A. Presentation Associated conditions Stokes–Adams attacks can be precipitated by this condition. These involve a temporary loss of consciousness resulting from marked slowing of the heart when the atrial impulse is no longer conducted to the ventricles. This should not be confused with the catastrophic loss of heartbeat seen with ventricular fibrillation or asystole Asystole (New Latin, from Greek privative a "not, without" + ''systolē'' "contraction") is the absence of ventricular contractions in the context of a lethal heart arrhythmia (in contrast to an induced asystole on a cooled patient on a heart-lu .... History It was described independently by Maurice Lev an ...
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Complete Heart Block
Third-degree atrioventricular block (AV block) is a medical condition in which the electrical impulse generated in the sinoatrial node (SA node) in the atrium of the heart can not propagate to the ventricles. Because the impulse is blocked, an accessory pacemaker in the lower chambers will typically activate the ventricles. This is known as an '' escape rhythm''. Since this accessory pacemaker also activates independently of the impulse generated at the SA node, two independent rhythms can be noted on the electrocardiogram (ECG). * The P waves with a regular P-to-P interval (in other words, a sinus rhythm) represent the first rhythm. * The QRS complexes with a regular R-to-R interval represent the second rhythm. The PR interval will be variable, as the hallmark of complete heart block is the lack of any apparent relationship between P waves and QRS complexes. Presentation People with third-degree AV block typically experience severe bradycardia (an abnormally low measured hea ...
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Electrical Conduction System Of The Heart
The cardiac conduction system (CCS) (also called the electrical conduction system of the heart) transmits the signals generated by the sinoatrial node – the heart's pacemaker, to cause the heart muscle to contract, and pump blood through the body's circulatory system. The pacemaking signal travels through the right atrium to the atrioventricular node, along the bundle of His, and through the bundle branches to Purkinje fibers in the walls of the ventricles. The Purkinje fibers transmit the signals more rapidly to stimulate contraction of the ventricles. The conduction system consists of specialized heart muscle cells, situated within the myocardium. There is a skeleton of fibrous tissue that surrounds the conduction system which can be seen on an ECG. Dysfunction of the conduction system can cause irregular heart rhythms including rhythms that are too fast or too slow. Structure Electrical signals arising in the SA node (located in the right atrium) stimulat ...
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SCN5A
Sodium channel protein type 5 subunit alpha, also known as NaV1.5 is an integral membrane protein and tetrodotoxin-resistant voltage-gated sodium channel subunit. NaV1.5 is found primarily in cardiac muscle, where it mediates the fast influx of Na+-ions (INa) across the cell membrane, resulting in the fast depolarization phase of the cardiac action potential. As such, it plays a major role in impulse propagation through the heart. A vast number of cardiac diseases is associated with mutations in NaV1.5 (see paragraph genetics). ''SCN5A'' is the gene that encodes the cardiac sodium channel NaV1.5. Gene structure SCN5A is a highly conserved gene located on human chromosome 3, where it spans more than 100 kb. The gene consists of 28 exons, of which exon 1 and in part exon 2 form the 5' untranslated region ( 5’UTR) and exon 28 the 3' untranslated region ( 3’UTR) of the RNA. SCN5A is part of a family of 10 genes that encode different types of sodium channels, i.e. brain-type ( ...
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Ventricular Fibrillation
Ventricular fibrillation (V-fib or VF) is an abnormal heart rhythm in which the ventricles of the heart quiver. It is due to disorganized electrical activity. Ventricular fibrillation results in cardiac arrest with loss of consciousness and no pulse. This is followed by sudden cardiac death in the absence of treatment. Ventricular fibrillation is initially found in about 10% of people with cardiac arrest. Ventricular fibrillation can occur due to coronary heart disease, valvular heart disease, cardiomyopathy, Brugada syndrome, long QT syndrome, electric shock, or intracranial hemorrhage. Diagnosis is by an electrocardiogram (ECG) showing irregular unformed QRS complexes without any clear P waves. An important differential diagnosis is torsades de pointes. Treatment is with cardiopulmonary resuscitation (CPR) and defibrillation. Biphasic defibrillation may be better than monophasic. The medication epinephrine or amiodarone may be given if initial treatments are no ...
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Asystole
Asystole (New Latin, from Greek privative a "not, without" + ''systolē'' "contraction") is the absence of ventricular contractions in the context of a lethal heart arrhythmia (in contrast to an induced asystole on a cooled patient on a heart-lung machine and general anesthesia during surgery necessitating stopping the heart). Asystole is the most serious form of cardiac arrest and is usually irreversible. Also referred to as cardiac flatline, asystole is the state of total cessation of electrical activity from the heart, which means no tissue contraction from the heart muscle and therefore no blood flow to the rest of the body. Asystole should not be confused with very brief pauses in the heart's electrical activity—even those that produce a temporary flatline—that can occur in certain less severe abnormal rhythms. Asystole is different from very fine occurrences of ventricular fibrillation, though both have a poor prognosis, and untreated fine VF will lead to asystole. Faul ...
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Heart Block
Heart block (HB) is a disorder in the heart's rhythm due to a fault in the natural pacemaker. This is caused by an obstruction – a block – in the electrical conduction system of the heart. Sometimes a disorder can be inherited. Despite the severe-sounding name, heart block may cause no symptoms at all in some cases, or occasional missed heartbeats in other cases (which can cause light-headedness, syncope (fainting), and palpitations), or may require the implantation of an artificial pacemaker, depending upon exactly where in the heart conduction is being impaired and how significantly it is affected. Heart block should not be confused with other conditions, which may or may not be co-occurring, relating to the heart and/or other nearby organs that are or can be serious, including angina (heart-related chest pain), heart attack (myocardial infarction), any type of heart failure, cardiogenic shock or other types of shock, different types of abnormal heart rhythms ( arrhythmias ...
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