Congenital heart anomaly «single ventricle»: structural and functional diagnosis in everyday practice

Abstract

Diagnosis and description of rare congenital heart anomalies are great challenges for the ultrasonographer. The biggest difficulties arise in hospitals, which do not have specialization in pediatrics or cardiac surgery. A rare form of congenital heart defects is a group of anomalies, unitedly defined as «single ventricle». Severe hemodynamic disturbances and unfavorable course of the disease reduce the possibility to detect this defect in adolescence or adulthood. Single ventricle, as the anomaly main feature, can occur in different forms of pathology. The most commonly single ventricle syndrome includes left heart hypoplasia, tricuspid atresia, and double inlet single ventricle syndromes. The most favorable course have cases when a single ventricle anatomically is left ventricle along with separate and properly formed atrioventricular valves, in the absence of main arteries significant stenosis and their normal location. This variant of a double inlet single ventricle is known as the «Holmes heart». Echocardiographic diagnosis allows to detail the anatomical and functional defects that are present in each case. Detailed identification of diagnostic features allows to specify the diagnosis and avoid inconsistencies in the obtained data interpretation. We present a clinical case of one of the most rare forms of these disease. The described case feature, in addition to its rarity, is long compensated course of defect. The lack of obvious signs of heart failure in this case may be due to a number of factors, such as a balance between the pulmonary and systemic circulation, absence of progressive changes in the pulmonary circulation blood vessels, atrioventricular valves functional adequacy, the absence of stenosis and valves changes of the main arteries, anatomically left ventricle with sufficient contractility.