Sieweke Jan Thorben, Haghikia Arash, Riehle Christian, Klages Carolin, Akin Muharrem, König Tobias, Zwadlo Carolin, Treptau Jens, Schäfer Andreas, Bauersachs Johann, Westhoff-Bleck Mechthild
Department of Cardiology and Angiology,Hannover Medical School,Hannover,Germany.
Department of Cardiology,Charité Universitätsmedizin Berlin,Campus Benjamin Franklin, Berlin,Germany.
Cardiol Young. 2019 May;29(5):602-609. doi: 10.1017/S1047951119000258. Epub 2019 Apr 30.
Late Fontan survivors are at high risk to experience heart failure and death. Therefore, the current study sought to investigate the role of non-invasive diagnostics as prognostic markers for failure of the systemic ventricle following Fontan procedure.
This monocentric, longitudinal observational study included 60 patients with a median age of 24.5 (19-29) years, who were subjected to cardiac magnetic resonance imaging, echocardiography, cardiopulmonary exercise testing, and blood analysis. The primary endpoint of this study was decompensated heart failure with symptoms at rest, peripheral and/or pulmonary edema, and/or death.
During a follow-up of 24 months, 5 patients died and 5 patients suffered from decompensated heart failure. Clinical (NYHA class, initial surgery), functional (VO2 peak, ejection fraction, cardiac index), circulating biomarkers (N-terminal pro brain natriuretic peptide), and imaging parameters (end diastolic volume index, end systolic volume index, mass-index, contractility, afterload) were significantly related to the primary endpoint. Multi-variate regression analysis identified afterload as assessed by cardiac magnetic resonance imaging as an independent predictor of the primary endpoint (hazard ratio 1.98, 95% confidence interval 1.19-3.29, p = 0.009).
We identified distinct parameters of cardiopulmonary exercise testing, cardiac magnetic resonance imaging, and blood testing as markers for future decompensated heart failure and death in patients with Fontan circulation. Importantly, our data also identify increased afterload as an independent predictor for increased morbidity and mortality. This parameter is easy to assess by non-invasive cardiac magnetic resonance imaging. Its modulation may represent a potential therapeutic approach target in these high-risk patients.
法洛四联症晚期幸存者发生心力衰竭和死亡的风险很高。因此，本研究旨在探讨非侵入性诊断作为法洛四联症术后体循环心室功能衰竭预后标志物的作用。
这项单中心纵向观察性研究纳入了60例患者，中位年龄为24.5（19 - 29）岁，这些患者接受了心脏磁共振成像、超声心动图、心肺运动试验和血液分析。本研究的主要终点是失代偿性心力衰竭，伴有静息症状、外周和/或肺水肿和/或死亡。
在24个月的随访期间，5例患者死亡，5例患者发生失代偿性心力衰竭。临床指标（纽约心脏协会分级、初次手术情况）、功能指标（峰值摄氧量、射血分数、心脏指数）、循环生物标志物（N末端脑钠肽前体）和影像学参数（舒张末期容积指数、收缩末期容积指数、质量指数、收缩性、后负荷）与主要终点显著相关。多变量回归分析确定，通过心脏磁共振成像评估的后负荷是主要终点的独立预测因子（风险比1.98，95%置信区间1.19 - 3.29，p = 0.009）。
我们确定了心肺运动试验、心脏磁共振成像和血液检测的不同参数，作为法洛四联症循环患者未来发生失代偿性心力衰竭和死亡的标志物。重要的是，我们的数据还确定后负荷增加是发病率和死亡率增加的独立预测因子。该参数易于通过非侵入性心脏磁共振成像进行评估。对其进行调节可能代表这些高危患者潜在的治疗方法靶点。
