Sclerodegenerative aortic valve lesions and hereditary connective tissue disorders

Abstract

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)/MATERIAL 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. onclusion. Three or more signs of bone dysmorphogenesis and the Marfanoid habitus should be viewed as a predictor of SDAVL.

Keywords

• aortic valve
• dysplastic syndromes and phenotypes
• sclerodegenerative aortic valve lesions
• calcific aortic stenosis