Sullivan Rachel T, Frommelt Peter C, Hill Garick D
Pediatrics, Medical College of Wisconsin Affiliated Hospitals, 999 N 92nd Street, Suite 730, Wauwatosa, WI, 53226, USA.
Pediatric Cardiology, Children's Hospital of Wisconsin, 9000 W Wisconsin Ave, Milwaukee, WI, 53226, USA.
Pediatr Cardiol. 2017 Aug;38(6):1251-1256. doi: 10.1007/s00246-017-1653-2. Epub 2017 Jun 14.
The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis. Those with Down syndrome were compared to those without. The primary outcome of interest was time from repair to pulmonary valve replacement. Secondary outcomes included pulmonary regurgitation and indexed right ventricular volume on cardiac magnetic resonance imaging. The cohort of 284 patients included 35 (12%) with Down syndrome. Transannular patch repair was performed in 210 (74%). Down syndrome showed greater degree of pulmonary regurgitation (55 ± 14 vs. 37 ± 16%, p = 0.01) without a significantly greater rate of right ventricular dilation (p = 0.09). In multivariable analysis, Down syndrome (HR 2.3, 95% CI 1.2-4.5, p = 0.02) and transannular patch repair (HR 5.5, 95% CI 1.7-17.6, p = 0.004) were significant risk factors for valve replacement. Those with Down syndrome had significantly lower freedom from valve replacement (p = 0.03). Down syndrome is associated with an increased degree of pulmonary regurgitation and earlier pulmonary valve replacement after tetralogy of Fallot repair. These patients require earlier assessment by cardiac magnetic resonance imaging to determine timing of pulmonary valve replacement and evaluation for and treatment of preventable causes of pulmonary hypertension.
唐氏综合征与肺动脉高压之间的关联可能导致法洛四联症修复术后出现更严重的肺动脉反流，并可能使肺动脉瓣置换时间提前。我们比较了法洛四联症修复术后伴有和不伴有唐氏综合征患者的心脏磁共振测量的肺动脉反流和右心室扩张情况，以及肺动脉瓣置换时间。回顾我们2000年至2015年的手术数据库，确定了患有肺动脉狭窄的法洛四联症患者。将患有唐氏综合征的患者与不患有唐氏综合征的患者进行比较。感兴趣的主要结局是从修复到肺动脉瓣置换的时间。次要结局包括心脏磁共振成像上的肺动脉反流和右心室指数容积。284例患者队列中包括35例（12%）患有唐氏综合征。210例（74%）进行了跨环补片修复。唐氏综合征患者的肺动脉反流程度更高（55±14%对37±16%，p = 0.01），而右心室扩张率没有显著更高（p = 0.09）。在多变量分析中，唐氏综合征（HR 2.3，95%CI 1.2 - 4.5，p = 0.02）和跨环补片修复（HR 5.5，95%CI 1.7 - 17.6，p = 0.004）是瓣膜置换的显著危险因素。患有唐氏综合征的患者瓣膜置换的无事件生存率显著更低（p = 0.03）。唐氏综合征与法洛四联症修复术后肺动脉反流程度增加和肺动脉瓣置换时间提前有关。这些患者需要通过心脏磁共振成像进行更早的评估，以确定肺动脉瓣置换时间，并评估和治疗肺动脉高压的可预防病因。
