Nicholson George T, Kelleman Michael S, De la Uz Caridad M, Pignatelli Ricardo H, Ayres Nancy A, Petit Christopher J
1Department of Pediatrics,Division of Pediatric Cardiology,Children's Healthcare of Atlanta,Emory University School of Medicine,Atlanta,Georgia,United States of America.
2Department of Pediatrics,Division of Biostatistics,Children's Healthcare of Atlanta,Atlanta,Georgia,United States of America.
Cardiol Young. 2017 May;27(4):697-705. doi: 10.1017/S1047951116001104. Epub 2016 Jul 26.
Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone's syndrome and the factors associated with left heart growth.
All patients diagnosed with Shone's syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed.
A total of 121 patients with Shone's syndrome presented at a median age of 28 days (0-17.3 years) and were followed-up for 7.2 years (0.01-35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time.
Shone's syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.
肖恩综合征是一种除左心室流出道梗阻外还伴有二尖瓣狭窄的复杂病症。评估该人群长期预后的研究较少。我们试图确定我们儿科肖恩综合征患者的长期预后以及与左心生长相关的因素。
回顾了1978年至2010年间所有诊断为肖恩综合征且双心室循环的患者。还回顾了基线超声心动图和心导管检查数据。追踪干预次数（手术+经导管）、二尖瓣置换发生率和心脏移植发生率。也评估了该人群的生存率和左心结构生长情况。
共有121例肖恩综合征患者，中位年龄28天（0 - 17.3岁），随访7.2年（0.01 - 35.5年）。这些患者在研究期间接受了258次干预，主动脉缩窄的存在与再次进行左心干预相关。10年无移植生存率为86%。肺动脉高压的存在与死亡率相关。随着时间推移，二尖瓣和主动脉瓣环以及左心室舒张末期内径出现左心结构生长。
肖恩综合征患者需接受多次左心干预。主动脉缩窄与再次干预的可能性增加相关。生存率似乎比预期更乐观。该人群会出现显著的左心生长。肺动脉高压与死亡风险增加相关。
