肺动脉高压对左心室射血分数正常的系统性轻链型淀粉样变性患者预后的影响

目的　评估初诊时肺动脉高压（PH）在左心室射血分数正常的系统性轻链型淀粉样变性（AL）患者预后中的价值。方法　回顾性分析2014年6月至2021年12月海军军医大学（第二军医大学）第二附属医院收治的初发AL患者134例。纳入标准为患者均经病理活检证实且左心室射血分数正常，并排除有先天性心脏病、中重度慢性阻塞性肺疾病等PH病因的患者。根据经胸超声心动图估测肺动脉压力值，将患者分为PH组和无PH组。使用Kaplan-Meier法计算总生存率和无进展生存率，并绘制生存曲线。采用单因素和多因素Cox比例风险回归模型分析PH对生存预后的影响。结果　最终纳入AL患者93例，其中PH组17例、无PH组76例。中位随访时间为19.4（1.0～87.0）个月，随访期间33例患者死亡。PH组和无PH组患者的中位总生存期分别为20.9（9.93，NA）个月、未达到，中位无进展生存期分别为10.5（6.93，NA）、47.0（35.87，NA）个月，差异均有统计学意义（P＜0.05）。校正基线资料后进行多因素Cox比例风险回归模型分析，结果显示PH是影响AL患者生存预后的独立危险因素，伴PH患者死亡的风险是无PH患者的4.93倍（95% CI 1.41～17.23，P=0.012 4），疾病进展的风险是无PH患者的3.53倍（95% CI 1.22～10.20，P=0.019 6）；在伴有心脏浸润的AL患者中，伴PH患者死亡和疾病进展的风险更高（HR=13.23，95% CI 2.35～74.93，P=0.003 4；HR=3.58，95% CI 1.09～11.71，P=0.035 3）。结论　超声心动图评估PH对于左心室射血分数正常的AL患者有重要的预后价值，在临床实践中需予以重视。

Effect of pulmonary hypertension on prognosis of patients with systemic immunoglobulin light-chain amyloidosis with normal left ventricular ejection fraction

Objective To evaluate the prognostic value of pulmonary hypertension (PH) in newly diagnosed patients with systemic immunoglobulin light-chain amyloidosis (AL) with normal left ventricular ejection fraction. Methods The data of the first-time AL patients admitted to The Second Affiliated Hospital of Naval Medical University (Second Military Medical University) from Jun. 2014 to Dec. 2021 were analyzed retrospectively. The inclusion criteria were:all patients were confirmed with AL by biopsy and pathology and had normal left ventricular ejection fraction; and patients with congenital heart disease, moderate and severe chronic obstructive pulmonary disease and other causes of PH were excluded. According to the estimation of pulmonary artery pressure by transthoracic echocardiography, the patients were divided into PH group and non-PH group. Kaplan-Meier method was used to calculate overall survival (OS) and progress-free survival (PFS) rates, and the survival curves were drawn. Univariate and multivariate Cox proportional hazards regression models were used to analyze the effects of PH on survival and prognosis. Results Finally, 93 AL patients were enrolled, including 17 patients in the PH group and 76 patients in the non-PH group. The median follow-up time was 19.4 (1.0-87.0) months, during which 33 patients died. The median OS time of patients was 20.9 months in the PH group and not available in the non-PH group, and the median PFS time was 10.5 months and 47.0 months, respectively, showing significant differences (both P<0.05). After adjusting the baseline data, the multivariate Cox regression analysis showed that PH was an independent risk factor affecting the survival and prognosis of AL patients. The risk of death in patients with PH was 4.93 times higher than that in patients without PH (hazard ratio[HR]=4.93, 95% confidence interval[CI]1.41-17.23, P=0.012 4); the risk of disease progression in patients with PH was 3.53 times higher than that in patients without PH (HR=3.53, 95% CI 1.22-10.20, P=0.019 6). Among AL patients with cardiac infiltration, patients with PH had a higher risk of death and disease progression (HR=13.23, 95% CI 2.35-74.93, P=0.003 4; HR=3.58, 95% CI 1.09-11.71, P=0.035 3). Conclusion Echocardiographic assessment of PH has important prognostic value for AL patients with normal left ventricular ejection fraction, which should be considered in clinical practice.