Objective Elevated levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are associated with heart failure-related death in hypertrophic cardiomyopathy (HCM), but the relationship between NT-proBNP level and sudden cardiac death (SCD) in HCM remains undefined. Methods The study prospectively enrolled 977 unrelated patients with HCM with available NT-proBNP results who were prospectively enrolled and followed for 3.0±2.1 years. The Harrell’s C-statistic under the receiver operating characteristic curve was calculated to evaluate discrimination performance. A combination model was constructed by adding NT-proBNP tertiles to the HCM Risk-SCD model. The correlation between log NT-proBNP level and cardiac fibrosis as measured by late gadolinium enhancement (LGE) or Masson’s staining was analysed. Results During follow-up, 29 patients had SCD. Increased log NT-proBNP levels were associated with an increased risk of SCD events (adjusted HR 22.27, 95% CI 10.93 to 65.63, p<0.001). The C-statistic of NT-proBNP in predicting SCD events was 0.80 (p<0.001). The combined model significantly improved the predictive efficiency of the HCM Risk-SCD model from 0.72 to 0.81 (p<0.05), with a relative integrated discrimination improvement of 0.002 (p<0.001) and net reclassification improvement of 0.67 (p<0.001). Furthermore, log NT-proBNP levels were significantly correlated with cardiac fibrosis as detected either by LGE (r=0.257, p<0.001) or by Masson’s trichrome staining in the myocardium (r=0.198, p<0.05). Conclusion NT-proBNP is an independent predictor of SCD in patients with HCM and may help with risk stratification of this disease. Data are available upon reasonable request.

中文翻译：

---

N端脑钠肽前体与肥厚型心肌病心源性猝死

目的 N-末端脑钠肽前体（NT-proBNP）水平升高与肥厚型心肌病（HCM）心力衰竭相关死亡有关，但 NT-proBNP 水平与 HCM 心源性猝死（SCD）的关系仍然存在不明确的。方法 该研究前瞻性纳入了 977 名具有可用 NT-proBNP 结果的 HCM 无关患者，这些患者被前瞻性纳入并随访了 3.0±2.1 年。计算接受者操作特征曲线下的 Harrell C 统计量以评估辨别性能。通过将 NT-proBNP 三分位数添加到 HCM Risk-SCD 模型来构建组合模型。分析了通过晚期钆增强 (LGE) 或马森染色测量的 log NT-proBNP 水平与心脏纤维化之间的相关性。结果随访期间，29例患者出现SCD。log NT-proBNP 水平增加与 SCD 事件风险增加相关（调整后的 HR 22.27，95% CI 10.93 至 65.63，p<0.001）。NT-proBNP 在预测 SCD 事件中的 C 统计量为 0.80 (p<0.001)。组合模型将 HCM Risk-SCD 模型的预测效率从 0.72 显着提高到 0.81 (p<0.05)，相对综合区分改进为 0.002 (p<0.001)，净重分类改进为 0.67 (p<0.001)。此外，通过 LGE (r=0.257, p<0.001) 或通过心肌中的马森三色染色 (r=0.198, p<0.05) 检测到的 log NT-proBNP 水平与心脏纤维化显着相关。结论 NT-proBNP 是 HCM 患者 SCD 的独立预测因子，可能有助于该疾病的风险分层。