Mitral valve repair in a patient with mitral regurgitation and osteogenesis imperfecta tarda

Cardiovascular Surgery Department, Virgen Macarena Hospital, Seville, Spain.
Interactive Cardiovascular and Thoracic Surgery (Impact Factor: 1.16). 12/2011; 13(6):655-6. DOI: 10.1510/icvts.2011.277103
Source: PubMed


We present the case of a 62-year-old female with a diagnosis of osteogenesis imperfecta and mitral valve regurgitation. The patient underwent a mitral valve repair without complications. We describe the case and our surgical technique.

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    ABSTRACT: Osteogenesis imperfecta (OI) or "brittle bone disease" is a rare connective tissue hereditary disorder. The most common clinical presentation of OI is bone fractures. OI also involves extraskeletal structures; however, cardiovascular manifestations are rare. In this report, we describe the cardiovascular anomalies of patients with OI who underwent valve surgery and review the literature on this subject.
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    ABSTRACT: A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was in osteogenesis imperfecta (OI) patients with valve disease undergoing valve replacement which type of valve (bioprosthetic or mechanical) is most appropriate in terms of safety, complications and survival. Altogether more than 77 papers were found as a result of the reported search, of which 43 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Previous review articles have presented case reports up to 2009. As all published data are based on case reports, we conducted a more detailed analysis that included the aforementioned series, reports that were missed prior to 2009 and all published data from 2009 to October 2013. Our analysis identified 43 OI patients. Mechanical valves were used in the majority of cases (31 patients), bioprosthetic valves in 10 patients and homografts in 2 patients. We conclude that based on the best available evidence, it appears that bioprosthetic valves have had better outcomes (mortality rate 10%) and a lower valve-related complication rate (0%) compared with mechanical valves (mortality rate 16.1%, complication rate 16.1%), even though differences were not statistically significant. Although the existing evidence is solely based on case reports of a relatively small number, we would suggest the use of bioprosthetic valves in OI patients with valve disease, as they appear to be safer according to our analysis. Moreover, considering the surgical difficulties related to the friability and weakness of the tissues in terms of suture lines and implantation of the valve as well as the high risk of perioperative bleeding which can be related to tissue friability, capillary fragility and platelet dysfunction followed by the risk of major traumatic fractures and a possible risk of aortic dissection in the future, the bioprosthetic valves seem to be safer taking into account the avoidance of lifelong anticoagulation and its secondary bleeding complications.
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