Aim. To investigate the role of hereditary mechanisms in the development of sclerotic degenerative disorders of aortic valve. Material and methods. Clinical, phenotypical, and echocardiographic examination was performed in 180 middle-aged and elderly people (mean age 63,8±9 years; 102 men, 78 women), including 86 patients with calcific aortic stenosis (55 after aortic valve surgery) and 61 controls. The diagnostic criteria of the National Guidelines on hereditary connective tissue disorders (2009) were used. The valve biopsy samples were examined using histological, morphological, and immunohistochemical (TGF-

AIM To estimate the role of different dysplastic syndromes and phenotypes in the development of sclerodegenerative lesions and calcific stenosis of the aortic valve (AV). SUBJECTS AND METHODS One hundred and fifty patients (82 men and 68 women) aged 42 to 83 years were examined. A study group consisted of 89 patients (mean age 60.7 +/- 8.9 years) with clinical and echocardiographic signs of sclerodegenerative AV lesions (SDAVL). A control group included 61 patients (mean age 61.3 +/- 7.6 years) who had no clinical, physical, or echocardiographic signs of SDAVL. Twenty-five patients with critical calcific aortic stenosis (CAS) underwent histological examination of removed aortic semilunar valves after prosthetic AV replacement. RESULTS Persons with a Marfanoid habitus (18%) and mitral valve prolapse (10.7%) are most common in older age groups. An association has been found between the bone signs of dysmorphogenesis and the development of SDAVL and CAS. CONCLUSION Three or more signs of bone dysmorphogenesis and the Marfanoid habitus should be viewed as a predictor of SDAVL.

The paper attempts to revise the working classification of minor heart anomalies (MHA). Ills proposed to exclude some syndromes and abnormalities which clearly have an independent clinical significance. The need for exclusion of prevalent normal variants, as well as anatomical and physiological characteristics of child's heart, is justified. The inverse dynamics of selected MHA in older vs. younger age groups is demonstrated. The authors suggest that the current working classification should be modified.

The paper attempts to revise the working classification of minor heart anomalies (MHA). It is proposed to exclude some syndromes and abnormalities which clearly have an independent clinical significance. The need for exclusion of prevalent normal variants, as well as anatomical and physiological characteristics of child’s heart, is justified. The inverse dynamics of selected MHA in older vs. younger age groups is demonstrated. The authors suggest that the current working classification should be modified.

Calcific aortic stenosis (CAS) stands on top among the acquired heart diseases nowadays. Of all the known risk factors for CAS there is no evidence of correlation with the inherited connective tiss...

The heritable connective tissue disorders with common general phenotypical and clinical features should be called dysplasia of the connective tissue. Classification of the basic dysplastical syndromes and phenotypes is offered. Article considers the algorithm of diagnostics of the basic syndromes and phenotypes and the practical questions arising at attempt of their classification.

The heritable connective tissue disorders with common general phenotypical and clinical features should be called dysplasia of the connective tissue. Classification of the basic dysplastical syndromes and phenotypes is offered. Article considers the algorithm of diagnostics of the basic syndromes and phenotypes and the practical questions arising at attempt of their classification.