Simplified mitral valve repair in pediatric patients with connective tissue disorders

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• 作者：Luca A. Vricella ; MD ; FACS^a ; ^{lvricella@jhmi.edu} ; William A. Ravekes ; MD^a ; Eloisa Arbustini ; MD^b ; Robert D.B. Jaquiss ; MD^c ; Constantine Mavroudis ; MD^a ; Harry C. Dietz ; MD^a ; Marshall L. Jacobs ; MD^a ; Narutoshi Hibino ; MD ; PhD^a ; Duke E. Cameron ; MD^a
• 关键词：mitral valve repair ; connective tissue disorders
• 刊名：The Journal of Thoracic and Cardiovascular Surgery
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：153
• 期：2
• 页码：399-403
• 全文大小：580 K
• 卷排序：153

文摘

In pediatric patients with connective tissue disorders (CTDs), early cardiac presentation often involves severe mitral regurgitation (MR) associated with severe bileaflet prolapse and, less frequently, aortic root enlargement. We adopted a simplified repair to address MR and prevent systolic anterior motion (SAM) in this unique group of patients.Materials and MethodsRetrospective review of clinical and echocardiographic data of all pediatric patients (age < 18 years) with CTD and MR undergoing simplified repair at 3 institutions (2000-2014).ResultsEighteen children who underwent surgery for severe MR and bileaflet prolapse were identified. All were treated with ring annuloplasty and Alfieri edge-to-edge repair. Median age and weight were 8.2 years (range, 0.4-17.2 years) and 24.9 kg (5.6-63.3 kg), respectively. Median left ventricular end diastolic dimension median z score was 4.9 (2.1-11.9). One patient died (5.6%), and there were no other major complications. Among survivors, 94.4% had mild regurgitation or less, with no stenosis or SAM at median clinical follow-up of 2.4 years (range, 0-13.9 years). Median left ventricular end-diastolic dimension z score regressed to 1.3 (&minus;0.5 to 4.3).ConclusionsIn pediatric patients with CTD and severe MR, a simplified approach is associated with intermediate-term competence, absence of SAM or significant stenosis, and regression of left ventricular enlargement.