Left-ventricular geometry in young persons with connective tissue dysplasia

Assessment of left-ventricular geometry in slightly pronounced variants of connective tissue dysplasia (CTD) was done, in order to define a fine dividing line between unconditioned norm and first signs of pathology. Morphometric parameters of left ventricle in young persons with CTD were analyzed in compliance with modern echocardiographic concepts.

The study is based on data obtained from the examination of 214 patients with CTD from 18 to 29 years old (average age 20 (19; 22) years; 49.5% of men) and 100 healthy peers without signs of CTD (average age 20 (19; 21) years; 51.9% of men).

It is known that the asthenic type of physique with a narrow, conical shape of the chest often with a funnel-shaped or keel-like deformation, scoliosis, hyperkyphosis and hyperlordosis of the thoracic spine, «straight back» syndrome predisposes to changes in the heart and large vessels in accordance with the structural features of the spine , sternum, ribs and diaphragm. At the same time, the heart undergoes dynamics from a truly small (asthenic variant) to a thoracodiaphragmatic pulmonary heart (pseudoconstrictive, pseudodilation and false-stenotic variants), from the state of adaptation to the stage of decompensation.

It is indicated that when solving expert questions in adolescents and young people with CTD, a clinical interpretation of the results of a standard echocardiographic study by cardiologists and general practitioners should be carried out, paying attention not only to the development of the fibrous skeleton and associated valve and subvalvular structures, interstitial tissue disorders symmetries with the development of asymmetric left ventricular hypertrophy, intraventricular and intraatrial developmental abnormalities («extra» chords, etc.), but it is necessary to take into account the constitutional features of patients and the geometric pattern of the left ventricle, which is very important in terms of prognosis. 

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