Журнал «Здоровье ребенка» 7 (58) 2014

The article presents the published date on the prevalence, the main clinical    manifestations, modern methods of early neonatal and postnatal  diagnosis, treatment and prognosis by tricuspid atresia – the congenital malformation of cardiovascular system.

Tricuspid atresia – is a congenital heart malformation of the blue type, which is characterized with the absence of the direct communication between the right atrium and the right ventricle, right ventricle hypoplasia and the presence of the different accompanying cardiac anomalies (ventricular septal defect, atrial septal defect, patent ductus arteriosus, transposition of great vessels, etc).

According to the International  Disease Classification-10, the following is defined: Q22.4 The congenital tricuspidal valve stenosis. Tricuspidal valve atresia.

Frequency of the malformation among all the congenital heart anomalies is 0,3-0,7 %,  due to the clinical data – 2,4 %, due to the postmortem data – 2,6 – 5,3 % and holds the third place in the frequency rate among the defects of the cyanotic type after Fallot’s tetrad and mainstream vessels transposition.

There exist many different anatomic variants of the tricuspidal valve atresia.

Clinical picture. Clinically, tricuspidal valve atresia signs itself with chronical hypoxia – marked cyanosis, which is noted right after the birth or in the period of infancy, with the dyspnea at rest, dyspnocyanotic attacks, or traits of the congestive heart failure (with the absence of the pulmonary artery stenosis).

Early neonatal and postnatal diagnostics. A new-born reveals diffuse cyanosis which becomes more expressed with the exertion and baby’s crying, quick tiredness during feeding, frequent possetings, dyspnea. At the examination there is cyanosis from the blue to violet colour, the apical beat is diffuse. The auscultation picture may be different. Polycythemia is present in the blood counts.

The electrocardiographical data are specific with this anomaly. This is the only defect of the blue type, under which deviation of the electric axis is found along with the left ventricle hypertrophy. The echocardiography data: there can be found the absence of the echo signal from the tricuspidal valve, small measures of the right ventricle and dilatation of the left ventricle’s cavity.

The X-ray data show that associated with the frequently decreased pulmonary circulation, the enlargement of the left atrium and left heart chambers are found along with the hypoplasia of the right ventricle.

Differential diagnostics should be led with the only ventricle, pulmonary atresia with the hypoplasia of the tricuspidal valve and the right ventricle, transposition of the great vessels.     Treatment. The surgical correction is recommended. Palliative and radical operations are worked out.

Prognosis with the tricuspidal valve atresia is extremely severe. Most children die during the first year of life. Closing of the oval window or functioning of the ductus arteriosus contribute to it.

The surgery treatment leads to the decrease or disappearance of the cyanosis, polycythemia, reduction of the tension on the left ventricle. All this determines the increase of survival among patients of this group.