Stein D, Kloster F E
Am Heart J. 1977 Nov;94(5):637-41. doi: 10.1016/s0002-8703(77)80135-x.
Aortic and mitral valve abnormalities have been reported which clearly appear to be related to the underlying connective tissue disorder in two patients, a father and daughter, with osteogenesis imperfecta. Although this appears to occur with a much lower prevalence and lesser severity than in the Marfan syndrome, the true prevalence of cardiac connective tissue involvement is not known, and the orthopedic complications of osteogenesis imperfecta may have overshadowed attention to cardiovascular abnormalities. In evaluating patients with osteogenesis imperfecta, careful attention should be paid to cardiovascular findings and if valvular lesions are noted, patients should be instructed regarding the need for antibiotic prophylaxis for dental and surgical procedures. The valvular lesions can progress, and regular follow-up cardiovascular evaluation should be planned. Finally, despite potential problems with tissue friability and healing and a possible tendency for increased bleeding, successful valve replacement can be carried out if necessitated by cardiac disability.
据报道，在一名患有成骨不全症的父亲及其女儿这两名患者中，出现了主动脉瓣和二尖瓣异常，这些异常显然与潜在的结缔组织疾病有关。尽管这种情况的发生率和严重程度似乎比马凡综合征低得多，但心脏结缔组织受累的真实发生率尚不清楚，而成骨不全症的骨科并发症可能使人们对心血管异常的关注有所忽视。在评估成骨不全症患者时，应仔细关注心血管检查结果，如果发现瓣膜病变，应告知患者在进行牙科和外科手术时需要使用抗生素预防感染。瓣膜病变可能会进展，因此应计划定期进行心血管评估随访。最后，尽管存在组织易碎、愈合困难以及出血可能增加的潜在问题，但如果心脏功能障碍需要，仍可成功进行瓣膜置换手术。
