Defect of a mezhpredserdny partition – the congenital anomaly of heart which is characterized by existence of the open message between the right and left auricles. Defect of a mezhpredserdny partition is shown by short wind, increased fatigue, lag in physical development, heartbeat, pallor of skin, noise in heart, existence of "a warm hump", frequent respiratory diseases. Defect of a mezhpredserdny partition is diagnosed taking into account data of the ECG, FKG, EhoKS, kateterization of cavities of heart, an atriografiya, angiopulmonografiya. At defect of a mezhpredserdny partition its ushivaniye, plasticity or rentgenendovaskulyarny occlusion is made.
Defect of a mezhpredserdny partition
Defect of a mezhpredserdny partition (DMPP) – one or several openings in the partition dividing the left and right predserdny cavities which existence causes pathological dumping of blood, violation of intracardial and system haemo dynamics. In cardiology defect of a mezhpredserdny partition occurs at 5-15% of persons with congenital heart diseases, at the same time is twice more often diagnosed for women.
Along with defects of an interventricular partition, a koarktatsiy aorta, an open arterial channel, defect of a mezhpredserdny partition is among the most often found congenital heart diseases. Defect of a mezhpredserdny partition can be the isolated congenital heart disease or be combined with other intracardial anomalies: defect of an interventricular partition, abnormal drenazhy pulmonary veins, mitralny or trikuspidalny insufficiency.
Reasons of defect of a mezhpredserdny partition
Formation of defect is connected with an underdevelopment of primary or secondary mezhpredserdny partition and endokardialny rollers in the embryonic period. Can lead genetic, physical, ecological and infectious factors to violation of an organogenesis.
The risk of development of defect of a mezhpredserdny partition in future child is significantly higher in those families where there are relatives with congenital heart diseases. Cases of family defects of a mezhpredserdny partition in combination with atrioventricular blockade or with an underdevelopment of bones of a brush (Holt Oram syndrome) are described.
Except hereditary conditionality, to emergence of defect of a mezhpredserdny partition, can give viral diseases of the pregnant woman (a rubella, chicken pox, herpes, syphilis, etc.), diabetes and other endokrinopatiya, reception of some medicines and alcohol during pregnancy, production harm, the ionizing radiation, gestational complications (toxicoses, threat of an abortion, etc.).
Features of haemo dynamics at defect of a mezhpredserdny partition
Owing to pressure difference in the left and right auricles, at defect of a mezhpredserdny partition there is an arteriovenozny shunting of blood from left to right. The size of dumping of blood depends on the sizes of the mezhpredserdny message, a ratio of resistance of atrioventricular openings, plastic resistance and volume of filling of ventricles of heart.
The left-right shunting of blood is followed by increase in fullness of a small circle of blood circulation, increase of volume loading of the right auricle and strengthening of work of the right ventricle. In view of discrepancy between the area of a valvate opening of a pulmonary artery and volume of emission from the right ventricle, there is a development of a relative stenosis of a pulmonary artery.
The long gipervolemiya of a small circle of blood circulation gradually leads to development of pulmonary hypertensia. It is observed only when the pulmonary blood-groove exceeds norm in 3 and more times. Pulmonary hypertensia at defect of a mezhpredserdny partition usually develops at children 15 years are more senior, at late sclerous changes of pulmonary vessels. Heavy pulmonary hypertensia causes right ventricular insufficiency and change of the shunt that is shown in the beginning by tranzitorny venoarterialny dumping (at physical activity, a natuzhivaniye, cough, etc.), and then and the persistent, followed constant cyanosis at rest (Eyzenmenger's syndrome).
Classification of defects of a mezhpredserdny partition
Defects of a mezhpredserdny partition differ by quantity, the sizes and an arrangement of openings.
Taking into account degree and character of an underdevelopment of primary and secondary mezhpredserdny partitions respectively mark out primary, secondary defects, and also the total absence of a mezhpredserdny partition causing the general, only auricle (three-chambered heart).
Carry cases of an underdevelopment of primary mezhpredserdny partition with saving primary mezhpredserdny message to primary DMPP. In most cases they are combined with splitting of shutters of two - and three-leaved valves, the open atrioventricular channel. Primary defect of a mezhpredserdny partition is, as a rule, characterized by the big size (3-5 cm) and localized in the lower part of a partition over predserdno-ventricular valves and has no bottom edge.
Secondary DMPP are formed by an underdevelopment of a secondary partition. Usually they have the small sizes (1-2 cm) and settle down in the center of a mezhpredserdny partition or in the field of mouths of hollow veins. Secondary defects of a mezhpredserdny partition are often combined with abnormal falling of pulmonary veins into the right auricle. At this type of defect the mezhpredserdny partition is kept in the lower part.
The combined defects of a mezhpredserdny partition meet (primary and secondary, DMPP in combination with defect of a venous sine). Also defect of a mezhpredserdny partition can enter into structure of difficult VPS (a triad and Fallo's pentade) or to be combined with heavy warm defects - Ebstein's anomaly, a hypoplasia of cameras of heart, a transposition of the main vessels.
Formation of the general (only) auricle is connected with an underdevelopment or total absence of primary and secondary partitions and existence of the big defect occupying the space of all mezhpredserdny partition. The structure of walls and both predserdny ears at the same time are kept. Three-chambered heart can be combined with anomalies of atrioventricular valves, and also with an aspleniya.
One of options of mezhpredserdny communication is the open oval window caused by an underdevelopment of own valve of an oval opening or its defect. However in view of the fact that the open oval opening is not the true defect of a partition connected with insufficiency of its fabric, this anomaly cannot be ranked as defects of a mezhpredserdny partition.
Symptoms of defect of a mezhpredserdny partition
Defects of a mezhpredserdny partition can proceed with long haemo dynamic compensation, and their clinic differs in a considerable variety. Expressiveness of symptomatology is defined by the size and localization of defect, duration of existence of VPS and development of secondary complications. On the first month of life as the only manifestation of defect of a mezhpredserdny partition usually serves tranzitorny cyanosis during the crying and concern that usually contacts perinatal encephalopathy.
At defects of a mezhpredserdny partition of the average and big sizes the symptomatology is shown in the first 3-4 months or by the end of the first year of life and characterized by resistant pallor of integuments, tachycardia, moderate lag in physical development, an insufficient gain of body weight. For children with defects of a mezhpredserdny partition typically frequent developing of respiratory diseases - recurrent bronchitis, pneumonia proceeding with long damp cough, persistent short wind, the plentiful damp rattles, etc. caused by a gipervolemiya of a small circle of blood circulation. At children of the first decade of life frequent dizzinesses, tendency to faints, bystry fatigue and short wind at physical activity are noted.
Small defects of a mezhpredserdny partition (to 10-15 mm) do not cause violation of physical development of children and characteristic complaints therefore the first clinical signs of defect can develop only on the second or third decade of life. Pulmonary hypertensia and heart failure at defect of a mezhpredserdny partition are formed approximately by 20 years when there is cyanosis, arrhythmias, is rare – a blood spitting.
Diagnostics of defect of a mezhpredserdny partition
At objective inspection of the patient with defect of a mezhpredserdny partition pallor of integuments, "a warm hump", moderate lag in growth and weight comes to light. Perkutorno is defined increase in borders of heart to the left and to the right; at an auskultation to the left of a breast in the II-III mezhreberye moderately intensive systolic noise which unlike defect of an interventricular partition or a stenosis of a pulmonary artery, never happens rough is listened. Over a pulmonary artery splitting of the II tone and emphasis of its pulmonary component comes to light. Auskultativny data are confirmed when carrying out a phonocardiography.
At secondary defects of a mezhpredserdny partition of ECG change reflect an overload of the right departments of heart. Incomplete blockade of the right leg of a bunch of Gis, AV-blockade, a syndrome of weakness of sinusovy knot can be registered. The X-ray analysis of bodies of a thorax allows to see strengthening of the pulmonary drawing, a vybukhaniye of a trunk of a pulmonary artery, increase in a shadow of heart owing to a hypertrophy of the right auricle and a ventricle. At roentgenoscopy the specific sign of defect of a mezhpredserdny partition - the strengthened pulsation of roots of lungs is found.
EhoKG-issledovaniye with color Doppler mapping reveals the left-right dumping of blood, existence of defect of a mezhpredserdny partition, allows to determine its size and localization. When sounding cavities of heart increase in pressure and saturation of blood oxygen in the right departments of heart and pulmonary artery is found. In case of diagnostic difficulties inspection is supplemented atriografiy, ventrikulografiy, flebografiy jugular veins, an angiopulmonografiya, heart MRT.
Defect of a mezhpredserdny partition should be differentiated from defect of an interventricular partition, an open arterial channel, mitralny insufficiency, the isolated stenosis of a pulmonary artery, Fallo's triad, abnormal falling of pulmonary veins into the right auricle.
Treatment of defect of a mezhpredserdny partition
Treatment of defects of a mezhpredserdny partition only surgical. As indications to cardiac operation serves identification of a gemodinamicheska of significant arteriovenozny dumping of blood. Optimum age for correction of defect at children - from 1 to 12 years. Surgical treatment is contraindicated at high pulmonary hypertensia with venoarterialny dumping of the blood caused by sclerous changes of pulmonary vessels.
At defects of a mezhpredserdny partition resort to various ways of their closing: to an ushivaniye, plasticity by means of a pericardiac rag or a synthetic patch in the conditions of a hypothermia and IK. Rentgenendovaskulyarny occlusion of defect of a mezhpredserdny partition allows to close openings no more than 20 mm.
Surgical correction of defects of a mezhpredserdny partition is followed by the good remote results: at 80-90% of patients it is noted normalization of haemo dynamics and lack of complaints.
Forecast of defect of a mezhpredserdny partition
Small defects of a mezhpredserdny partition are compatible to life and can be found even at advanced age. At some patients with small defect of a mezhpredserdny partition perhaps spontaneous closing of an opening within the first 5 years of life. Life expectancy of persons with big defects of a mezhpredserdny partition at the natural course of defect averages 35-40 years. Death of patients can occur from right ventricular heart failure, violations of a rhythm and conductivity of heart (paroksizmalny tachycardia, vibrating arrhythmia, etc.), is more rare — from pulmonary hypertensia of high degree.