ABSTRACT
AIM: To compare respiratory function in adults and children whose heart chordae were in anomalous position (AC) with reference to severity of heart connective tissue dysplasia syndrome. MATERIALS AND METHODS: The study included ninety one 6-14-year-olds and 125 adults with AC. The patients were divided into groups by the degree of atrioventricular valvular prolapse (AVVP). External respiration function was examined, and the loop flow-volume was registered by the pulmonological computer system. Inhalation bronchospasmolytic test with berotec was performed. RESULTS: Tracheobronchial dyskinesia related in degree to AVVP severity was found in 76 and 35% of the adults and children with AC and AVVP, respectively. The berotec test allowed more precise evaluation of tracheobronchial dyskinesia. CONCLUSION: Functional insufficiency of the connective tissue in patients with cardiac connective tissue dysplasia syndrome progresses with age.
Subject(s)
Chordae Tendineae/abnormalities , Heart Defects, Congenital/physiopathology , Mitral Valve Prolapse/physiopathology , Respiration , Adolescent , Adult , Bronchi/physiopathology , Child , Fenoterol , Heart Defects, Congenital/diagnosis , Humans , Mitral Valve Prolapse/diagnosis , Movement Disorders , Sympathomimetics , Trachea/physiopathologyABSTRACT
Phenotype was investigated in 86 patients with idiopathic prolapses of atrioventricular valves and in 21 patients with displaced chordae. 10 and 4 markers of connective tissue dysplasia were distinguished for patients with the prolapses and anomalous chordae, respectively. For patients with dysplasia of connective tissue of the heart a modified version of phenotypical length is proposed: norm-anomalous chordae--prolapse of the 1st degree--prolapse of the 2nd degree. For patients with 3 and more markers echo-CG registration is advisable to reject mitral and/or tricuspid valves.
