Abnormal drainage of pulmonary veins – congenital pathology of heart which morphological basis is made by abnormal falling of pulmonary veins into the right auricle or the hollow veins entering it. The abnormal drainage of pulmonary veins is shown by fatigue, lag in physical development, heartaches, short wind, repeated pneumonia. For diagnostics of an abnormal drainage of pulmonary veins the X-ray analysis, the ECG, ultrasonography of heart, sounding of heart, an angiopulmonografiya, atrio-and a ventrikulografiya, MRT is carried out. Options of surgical correction of an abnormal drainage of pulmonary veins differ depending on defect type.
Abnormal drainage of pulmonary veins
Abnormal drainage of pulmonary veins – congenital heart disease at which falling of mouths of all or separate pulmonary veins into the right auricle, a coronal sine or hollow veins takes place. The abnormal drainage of pulmonary veins makes 1,5-3% of all VPS in cardiology with prevalence at males.
Most often the abnormal drainage of pulmonary veins is combined with defect of a mezhpredserdny partition and an open oval window; in 20% of cases - with the general arterial trunk, DMZhP, a transpoziyotion of the main vessels, Fallo's tetrad, a stenosis of a pulmonary artery, a hypoplasia of the left departments of heart, the only ventricle of heart, a dekstrokardiya. From ekstrakardialny pathology intestines diverticulums, umbilical hernias, kidneys, , a horseshoe kidney, and also various malformations of bone and endocrine systems occur at patients with an abnormal drainage of pulmonary veins.
Reasons of an abnormal drainage of pulmonary veins
The general reasons forming an abnormal drainage of pulmonary veins do not differ from those at other VPS.
Direct dissociation of pulmonary veins with the left auricle can be connected with two factors: lack of their connection or early atresia of the general pulmonary vein. In the first case under the influence of adverse conditions the levopredserdny outgrowth does not contact properly venous textures of a rudiment of a lung that leads to formation of an abnormal venous drainage. At an early atresia initial connection of the general pulmonary vein and a pulmonary vascular bed takes place, however further there is an obliteration of their gleam therefore venous pulmonary return begins to be carried out in other available collateral ways.
Classification of an abnormal drainage of pulmonary veins
At falling of mouths of all pulmonary veins into venous system of a big circle of blood circulation or the right auricle speak about a full (total) abnormal drainage of pulmonary veins. If in the right auricle or a big circle one or several pulmonary veins are drained, such form of defect is called partial. Most often (in 97% of cases) the veins departing from the right lung are abnormally drained.
On the basis of the level of a confluence of pulmonary veins the abnormal drainage is classified on 4 anatomic types (option).
- The I option - suprakardialny (nadserdechny). Pulmonary veins or are separately drained by the general collector in the top hollow vein or its branches (an unpaired vein, the left anonymous or additional top hollow vein).
- The II option – intrakardialny (warm). The abnormal drainage of pulmonary veins occurs in the right auricle or a coronal sine.
- The III option – subkardialny, infrakardialny (subwarm). Pulmonary veins fall into the lower hollow or vorotny vein (it is rare – to the lymphatic canal).
- The IV option – mixed. The abnormal drainage of pulmonary veins in venous system is carried out at various levels in different combinations.
Features of haemo dynamics at an abnormal drainage of pulmonary veins
In the antenatalny period of floggings usually has no haemo dynamic manifestations as intracardial blood circulation at a fruit means intake of blood from the right auricle in left (through an open oval window) and in botall a channel. After the birth expressiveness of haemo dynamic violations is defined a form (full or partial), by option of an abnormal drainage of pulmonary veins, and also a defect combination to other defects of heart.
From the point of view of haemo dynamics, the total abnormal drainage of pulmonary veins is characterized by the fact that all oxygenic blood from lungs gets to the right auricle where mixes up with blue blood. Further one part of blood comes to the right ventricle, another – through mezhpredserdny communication in the left auricle and a big circle of blood circulation. In this case the abnormal drainage of pulmonary veins is compatible to life only on condition of existence of the message between two circles of blood circulation which role is carried out by DMPP or an open oval window. Violation of haemo dynamics at a total abnormal drainage of pulmonary veins are followed by a hyperkinetic overload of the right departments of heart, pulmonary hypertensia and a gipoksemiya.
At a partial form of an abnormal drainage of pulmonary veins haemo dynamic violations are similar to that at mezhpredserdny defects. The defining role among them belongs to the pathological arterio-venous dumping of blood leading to increase in volume of blood in a pulmonary circle of blood circulation.
Symptoms of an abnormal drainage of pulmonary veins
Anatomo-haemo dynamic features are the cornerstone of clinical manifestations of an abnormal drainage of pulmonary veins: level of all-pulmonary resistance, extent of obstruction of venous return, amount of mezhpredserdny communication, functioning of a myocardium of the right ventricle. In the absence of defect in a mezhpredserdny partition or its extremely small amount of floggings it is incompatible with life – in this case only the emergency endovascular balloon atrioseptostomiya on Rashkinda allows to save the child.
At children with an abnormal drainage of pulmonary veins frequent repeated pneumonia and a SARS, a small increase in weight, short wind, slight cyanosis, cough, bystry fatigue, lag in physical development, a heartache, tachycardia are noted. At the expressed pulmonary hypertensia already in the early childhood the expressed cyanosis, a warm hump and heart failure develop.
Diagnostics of an abnormal drainage of pulmonary veins
The Auskultativny picture of an abnormal drainage of pulmonary veins reminds DMPP and is characterized by not rough sistoliyochesky noise in a projection of a pulmonary artery, splitting of the II tone. Data of the ECG confirm an overload of the right departments, an incomplete bloyokada of the right leg of a bunch of Gis; EOS is rejected to the right. The phonocardiography corresponds to all signs of DMPP.
In the analysis of results of a X-ray analysis of a thorax expansion of borders of heart to the right, vybukhany arches of a pulmonary artery, strengthening of the pulmonary drawing pays attention. A reliable sign of an abnormal drainage of pulmonary veins in the lower hollow vein is the symptom of "the Turkish saber".
According to the mouth EhoKG of pulmonary veins in the left auricle are not found; dilatation of the right ventricle, the reduced sizes of the left departments of heart and other indirect signs of an abnormal drainage of pulmonary veins comes to light. To the senior children and adults chrespishchevodny EhoKG is carried out.
When sounding cavities of heart the catheter from the right auricle or a hollow vein is carried out to a pulmonary vein. At this stage of diagnostics it is possible to establish localization of a confluence and the number of abnormally drained pulmonary veins. The right atriografiya, a ventrikulografiya, an angiopulmonografiya, a flebografiya of VPV allow to track the movement of contrast through a small circle of blood circulation in the right auricle or hollow veins.
At patients with an abnormal drainage of pulmonary veins in the differential and diagnostic plan it is necessary to exclude an atresia of the mitralny or aortal valve, the mitralny stenosis isolated by DMPP, trekhpredserdny heart, a stenosis of pulmonary veins, a transposition of magistralyyony vessels, a limfangioektaziya.
Treatment of an abnormal drainage of pulmonary veins
The way of surgical correction of a partial abnormal drainage of pulmonary veins is defined by a type of defect: at the same time the drainage level, the sizes and an arrangement of DMPP is considered. Depending on the called criteria numerous options of replantation of mouths of pulmonary veins or their general collectors in the left auricle are used. The ushivaniye or plastic DMPP is made for elimination of the mezhpredserdny message. To the children aged up to 3 months who are in critical condition the palliative operation of the closed atrioseptotomiya directed to increase in the mezhpredserdny message is performed.
Treat the general principles of radical correction of a total abnormal drainage of pulmonary veins: creation of an anastomoz between pulmonary veins and the left auricle, closing of DMPP, bandaging of the pathological message of pulmonary veins with venous vessels. After operation the syndrome of weakness of sinusovy knot, the increase in pulmonary hypertensia connected with inadequate providing ways of outflow from pulmonary veins can develop.
Forecast of an abnormal drainage of pulmonary veins
The natural course of a total abnormal drainage of pulmonary veins is adverse: 80% of children perish in the first year of life. Patients with a partial drainage of pulmonary veins can live to 20-30 years. Death of patients is connected with heavy heart failure or pulmonary infections.
Results of surgical correction of an abnormal drainage of pulmonary veins satisfactory, however among newborns intra-and the postoperative lethality remains high.