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"Child`s Health" 2 (61) 2015

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Total transposition of the great vessels: early neonatal and postnatal diagnostics, clinical manifestations, treatment and prognosis.

Authors: Kalashnikova K.A., Nikitina N.O. — Odessa National Medical University, Department of Propedeutics of Pediatrics, Odessa, Ukraine

Categories: Pediatrics/Neonatology

Sections: Clinical researches

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Summary

The article presents the published data on the prevalence, the main clinical manifestations, modern methods of early neonatal and postnatal diagnosis, treatment and prognosis by Transposition of the Great Vessels (Arteries) – the congenital malformation of cardiovascular system.
Transposition of the Great Vessels (Arteries) – is a group of the congenital heart malformations which is characterized by the anomaly of the position of the ascending aorta and the trank of the pulmonary arteria. There are two main kinds of this malformation: 1) Total Transposition of the Great Vessels, 2) Corrected Transposition of the Great Vessels.
According to International Statistical Classification of Diseases of the 10 revisition, the following is defined: Q20.3 Discondart ventricular- arterial connection (Dextroposition of the aorta. Transposition of the Great Vessels (total).

Frequency of the malformation. According  to  clinical data, frequency of the Total Transposition of the Great Vessels  among all the congenital heart malformations ranges from 4,2 to 9,9 %,  according  to  patholo-anatomical data – from 15,7 to 20,8 %.  Transposition of the Great Vessels  takes second place after Tetralogy of Fallot.
Interaction with other defects. Transposition of the Great Vessels can be observed under different chromosome aberrations and congenital syndromes.
Clinical manifestation of the malformation depends from the peculiarities of the hemodynamics. In transposition of great vessels one of the defects is the opening of the aorta from the right ventricle. The venous blood goes into the general circulation at once - the shunt is from the right to the left. Transposition of the Great Vessels (Arteries) – is a congenital heart malformation of the blue type – «blue  abnormality».  Oxygenation of the systemic blood may occur by mixing at any or all of three level: a ventricular septal defect, an atrial septal defect, a patent ductus arteriosus.
Early neonatal and postnatal diagnostics. In situations in which the ventricular septum is    infact, cyanosis and tachypnea are recognized in the first hours or days of life. Cardiac examination often is subtle. Cardiac examination is remarkable for a systolic ventricular septal defect – murmur.
Chest roentgenograms reveal a globular heart («egg on string» appearance), cardiomegaly.
The electrocardiogram typically shows peaked P waves and either right ventricular or biventricular hypertrophy.
Differential diagnostics should be led with the Corrected transposition of the great vessels with ussing  the echocardiography.   
Treatment. The early surgical correction is recommended to Patients with a transposition of the great vessels. Palliative and radical operations are worked out. Palliative treatment of these lesion cjnsist balloon atrial septostomy in effort to increase mixing. The infusion of the E1 prostaglandin are prescribed, until the surgical correction is done in newborn  with a patent ductus arteriosus. 
Prognosis with the Total Transposition of the Great Vessels with the natural course of the   malformation is extremely severe. Most children die during the first year of life. The prognosis is determined with the anatomical kind of this malformation and variant of the surgical correction.

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