From the Department of Cardiology, Boston Children's Hospital, Boston, MA; and Department of Pediatrics, Harvard Medical School, Boston, MA.
Circ Cardiovasc Imaging. 2014 May;7(3):502-9. doi: 10.1161/CIRCIMAGING.113.001473. Epub 2014 Mar 11.
Several clinical risk factors for death and heart transplantation have been identified in patients with Fontan circulation. It is unknown whether cardiac magnetic resonance (CMR) measurements of ventricular size and function are independently associated with these outcomes and further improve risk stratification.
Data on patients with Fontan circulation who had a CMR study from January 2002 to January 2011 were retrospectively reviewed. The end point was time to death or listing for heart transplantation after the CMR study. The median age of the 215 patients was 18.3 years (25th, 75th percentiles: 14, 26) with a median age at Fontan of 3.6 years (2.3, 7.1). During a median post-CMR follow-up period of 4.1 years (2.6, 6.2), 24 patients (11%) reached the end point: 20 deaths, 3 transplantations, and 1 transplantation listing. In a multivariable Cox regression model with clinical parameters only, protein-losing enteropathy was associated with death or listing for transplant. A multivariable model, including clinical and CMR parameters, showed that in addition to protein-losing enteropathy, ventricular indexed end-diastolic volume >125 mL/body surface area raised to the 1.3 power was associated with the endpoint. A likelihood-ratio test comparing the 2 models showed that the addition of indexed end-diastolic volume resulted in a significantly improved end point prediction (P<0.001)-C-index increased from 0.63 to 0.79.
CMR-derived ventricular indexed end-diastolic volume is an independent predictor of death or transplant in patients late after the Fontan operation and adds incremental value over clinical symptoms alone for risk stratification.
在 Fontan 循环患者中，已经确定了一些与死亡和心脏移植相关的临床风险因素。目前尚不清楚心脏磁共振（CMR）测量的心室大小和功能是否与这些结果独立相关，并进一步改善风险分层。
回顾性分析了 2002 年 1 月至 2011 年 1 月期间接受 CMR 检查的 Fontan 循环患者的数据。终点是 CMR 检查后死亡或心脏移植的时间。215 例患者的中位年龄为 18.3 岁（25%、75%：14、26），Fontan 年龄的中位数为 3.6 岁（2.3、7.1）。在 CMR 检查后中位随访时间为 4.1 年（2.6、6.2）期间，24 例患者（11%）达到终点：20 例死亡、3 例移植、1 例移植待排。在仅包含临床参数的多变量 Cox 回归模型中，蛋白丢失性肠病与死亡或移植待排相关。包含临床和 CMR 参数的多变量模型显示，除蛋白丢失性肠病外， indexed end-diastolic volume >125 ml/body surface area 升高到 1.3 次幂也与终点相关。比较 2 个模型的似然比检验显示， indexed end-diastolic volume 的增加显著改善了终点预测（P<0.001）-C 指数从 0.63 增加到 0.79。
CMR 衍生的 indexed end-diastolic volume 是 Fontan 手术后晚期患者死亡或移植的独立预测因素，并且在风险分层方面，与单纯临床症状相比，具有附加价值。
