Periodic Familial Disease

Article about Periodic Familial Disease

Periodic disease (Pacemaker disease) is a rare congenital heart defect characterized by a hemodynamically ineffective atrioventricular conduction defect due to sinus node disease. In this case, a blister forms in the interatrial septum; its size may be insignificant and not cause a significant hemodynamic disturbance, and sometimes it can increase due to the expansion of the intermuscular part of the septum.

The basis of periodic disease is arrhythmogenic dysplasia of the cardiac conduction system. Reciprocal excitation between the sinus and atrioventricular nodes, gradually losing the ability to stimulate contractions of the ventricles of the heart and the tone of the vagus nerve fibers decreases. Thus, there is an uneven contraction of the ventricular cavities while maintaining the normal rhythm of the sinus impulse. In this case, an attack of long-lasting bradycardia or asystole occurs, which only requires the administration of oxygen in cases of prolonged bradycardic oxygen deficiency, but does not cause negative consequences for the body as a whole. Increased tone of the vagus nerves under the influence of the attack itself of periodic conduction disturbances. Along with this, there is a tendency to delay the increase in lactic acid in the blood, mainly amino acid, and with prolonged manifestation of cardiac asthma and pyruvatemic acid. Paroxysm