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Torsades de pointes

Torsades de pointes is a distinctive polymorphic ventricular tachycardia in which the QRS amplitude varies and the QRS complexes appear to twist around the baseline. Torsades de pointes is associated with a prolonged QT interval, which may be congenital or acquired. It is usually not sustained and terminates spontaneously but frequently recurs unless the underlying cause is corrected. Torsades de pointes may degenerate into sustained ventricular tachycardia or ventricular fibrillation.1 Torsades is a life-threatening arrhythmia and may present as sudden cardiac death in patients with structurally normal hearts.

Epidemiology

The corrected QT interval is longer in the white population than in the black population, and longer in females than males. Therefore, torsades de pointes is more common in white races and in females. Torsades occurs at any age. If it occurs at an early age, the cause is usually due to congenital long QT syndrome. In later years, the cause is usually due to acquired long QT syndrome.

Risk factors2

Congenital long QT syndromes, e.g. Jervell and Lange-Nielsen syndrome, Romano-Ward syndrome. Acquired long QT syndromes: o Acute myocardial infarction. o Drugs, e.g. antiarrhythmic agents of classes Ia and III, erythromycin, ketoconazole, tricyclic antidepressants, antipsychotics. o Electrolyte disturbances; hypokalaemia, hypomagnesaemia, hypocalcaemia. o Renal failure, liver failure. o Metabolic; hypothyroidism, anorexia nervosa, starvation. o Bradycardia; sinoatrial disease, atrioventricular (AV) block. o Toxins; heavy metals, insecticides.

Presentation

Episodes of torsades in patients with congenital long QT syndromes may be triggered by stress, fear or physical exertion. Patients with torsades usually present with recurrent episodes of palpitations, dizziness, and syncope.3 Sudden cardiac death can occur with the first episode. Nausea, pallor, cold sweats, shortness of breath and chest pain may occur. A history of congenital deafness or a family history of sudden death may indicate a long QT syndrome.1 Physical findings depend on the rate and duration of tachycardia and the degree of cerebral hypoperfusion. Findings include rapid pulse, low or normal blood pressure, and transient or prolonged loss of consciousness. Other physical signs depend on the cause, e.g. features of a congenital disorder.

Differential diagnosis

Ventricular tachycardia. Supraventricular tachycardia with aberrant conduction. Other causes of syncope or sudden cardiac death.

Investigations

ECG:4 Paroxysms of 5-20 beats, with a heart rate faster than 200 beats per minute. Sustained episodes are occasionally seen. o Progressive change in polarity of QRS about the isoelectric line occurs with complete 180 twist of QRS complexes in 10-12 beats. o Usually, a prolonged QT interval and pathological U waves are present. The most consistent indicator of QT prolongation is a QT of 0.60 seconds or longer or a QTc (corrected for heart rate) of 0.45 seconds or longer. QTc = QT interval divided by the square root of the interval (in seconds) between the onset of each QRS complex (Bazett's formula). o A short-long-short sequence between the R-R interval occurs before the trigger response. Electrolytes; hypokalaemia, hypomagnesaemia and hypocalcaemia. Cardiac enzymes; assessment for myocardial ischaemia.2 CXR and echocardiography, to rule out structural heart disease.
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Phases of the cardiac action potential


The standard model used to understand the cardiac action potential is the action potential of the ventricular myocyte. The action potential has 5 phases (numbered 0-4). Phase 4 is the resting membrane potential, and describes the membrane potential when the cell is not being stimulated. Once the cell is electrically stimulated (typically by an electric current from an adjacent cell), it begins a sequence of actions involving the influx and efflux of multiple cations and anions that together produce the action potential of the cell, propagating the electrical stimulation to the cells that lie adjacent to it. In this fashion, an electrical stimulation is conducted from one cell to all the cells that are adjacent to it, to all the cells of the heart.

Phase 4
Phase 4 is the resting membrane potential. This is the period that the cell remains in until it is stimulated by an external electrical stimulus (typically an adjacent cell). This phase of the action potential is associated with diastole of the chamber of the heart. In addition to stimulus from adjacent cells, certain cells of the heart have the ability to undergo spontaneous depolarization, in which an action potential is generated without any influence from nearby cells. This is known as cardiac muscle automaticity.

Phase 0
Phase 0 is the rapid depolarization phase. The slope of phase 0 represents the maximum rate of depolarization of the cell and is known as dV/dtmax. This phase is due to the opening of the fast Na + channels causing a rapid increase in the membrane conductance to Na+ (GNa) and thus a rapid influx of Na+ ions (INa) into the cell; a Na+ current. The ability of the cell to open the fast Na+ channels during phase 0 is related to the membrane potential at the moment of excitation. If the membrane potential is at its baseline (about -85 mV), all the fast Na+ channels are closed, and excitation will open them all, causing a large influx of Na + ions. If, however, the membrane potential is less negative, some of the fast Na + channels will be in an inactivated state insensitive to opening, thus causing a lesser response to excitation of the cell membrane and a lower V max. For this reason, if the resting membrane potential becomes too positive, the cell may not be excitable, and conduction through the heart may be delayed, increasing the risk for arrhythmias. The fast Na+ channel The fast sodium channel can be modeled as being controlled by a number of gates. Each gate (or gating variable) can attain a value between 1 (fully open) and 0 (fully closed). The product of all the gates denotes the percentage of channels available to conduct Na+. Following the model of Hodgkin and Huxley, the sodium channel contains three gates: m, h, and j. In the resting state, the m gate is closed (zero) and the h and j gates are open (one). Hence, the product denoting the percentage of conducting channels is also zero. Upon electrical stimulation of the cell, the m gate opens quickly while simultaneously the h and j gates close more slowly. For a brief period of time, all gates are open (i.e. non-zero) and Na+ can enter the cell

following its electrochemical gradient. If, as above, the resting membrane potential is too positive, the h or j gates may be considerably less than one, such that the product of m, h and j becomes too small upon depolarization.

Phase 1
Phase 1 of the action potential occurs with the inactivation of the fast Na+ channels. The transient net outward current causing the small downward deflection of the action potential is due to the movement of K + and Cl- ions, carried by the Ito1 and Ito2 currents, respectively. Particularly the Ito1 contributes to the "notch" of some ventricular cardiomyocyte action potentials. It has been suggested that Cl- ions movement across the cell membrane during Phase I is as a result of the change in membrane potential, from K+ efflux, and is not a contributory factor to the initial repolarization ("notch").

Phase 2
This "plateau" phase of the cardiac action potential is sustained by a balance between inward movement of Ca2+ (ICa) through L-type calcium channels and outward movement of K+ through the slow delayed rectifier potassium channels, IKs. The sodium-calcium exchanger current, INa,Ca and the sodium/potassium pump current, INa,K also play minor roles during phase 2.

Phase 3
During phase 3 (the "rapid repolarization" phase) of the action potential, the L-type Ca2+ channels close, while the slow delayed rectifier (IKs) K+ channels are still open. This ensures a net outward current, corresponding to negative change in membrane potential, thus allowing more types of K+ channels to open. These are primarily the rapid delayed rectifier K+ channels (IKr) and the inwardly rectifying K+ current, IK1. This net outward, positive current (equal to loss of positive charge from the cell) causes the cell to repolarize. The delayed rectifier K+ channels close when the membrane potential is restored to about -80 to -85 mV, while IK1 remains conducting throughout phase 4, contributing to set the resting membrane potential.