Structural heart anomalies (SHA) are congenital abnormalities of cardiovascular system, characterized by various anatomical departures of heart and great vessels from normal conditions. SHA are a part of connective tissue dysplasia syndrome (CTDS), one of the most common congenital autosomal-dominant diseases in people of young and middle age. The most common SHA are a mitral valve prolapse, abnormal chords of left ventricle and their combinations. The clinical significance of these anomalies depends on a degree of severity and impact on intracardial hemodynamics, as described in the article. The most prognostically dangerous are multiple abnormal chords of left ventricle, which can be a sign of serious hereditary disease - a left ventricular non-compaction.