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常染色体显性遗传性多囊肾病的超声心动图特征

Echocardiographic characteristics of autosomal dominant polycystic kidney disease.

作者信息

Koska-Ścigała Agata, Jankowska Hanna, Jankowska Magdalena, Dudziak Maria, Hellmann Marcin, Dębska-Ślizień Alicja

机构信息

Department of Neurology, Regional Hospital in Elbląg, Elbląg, Poland.

Division of Cardiac Diagnostics, Faculty of Medicine, Medical University of Gdańsk, Gdańsk, Poland.

出版信息

Sci Rep. 2024 Dec 2;14(1):29867. doi: 10.1038/s41598-024-81536-2.

Abstract

Cardiovascular complications in patients with autosomal dominant polycystic kidney disease (ADPKD) are frequently investigated extrarenal manifestations with contradictory outcomes. The primary goal of this study is to explore the prevalence of cardiovascular abnormalities using echocardiography and analyze their associations with clinical characteristics at different stages of chronic kidney disease (CKD) progression in ADPKD patients. We included sixty-eight patients in the study. All patients underwent transthoracic echocardiography using GE Vingmed Ultrasound (GE Norway Health Tech, Oslo, Norway). Demographic information, prior medical history, and antihypertensive medication use were recorded. To diagnose the rapid progression of CKD, creatinine levels were measured twice, with a one-year interval. Analysis revealed left ventricular hypertrophy (LVH) in over 40% of ADPKD patients, as indicated by various LVH parameters. Notably, a decline in estimated glomerular filtration rate (eGFR) after one year of observation was associated with increased left ventricular mass. Other prevalent findings included asymptomatic left ventricular diastolic dysfunction (ALVDD) in 39% of patients, left atrium (LA) enlargement in 39%, and mild valvular regurgitations in 80%. Ejection fraction, aortic root dimension, and the prevalence of mitral valve prolapse were not significantly increased. Cardiac indices did not differ substantially across the different eGFR stages. LVH, LA enlargement, ALVDD and valvular regurgitations are characteristics of cardiac phenotype in ADPKD. Cardiac indices were not different across different stages of CKD pointing towards the diagnosis of ADPKD being the main drive of their occurrence.

摘要

常染色体显性多囊肾病(ADPKD)患者的心血管并发症是经常被研究的肾外表现,但结果相互矛盾。本研究的主要目的是使用超声心动图探讨心血管异常的患病率,并分析其与ADPKD患者慢性肾脏病(CKD)进展不同阶段临床特征的相关性。我们纳入了68例患者进行研究。所有患者均使用GE Vivid超声(GE挪威医疗科技公司,奥斯陆,挪威)进行经胸超声心动图检查。记录人口统计学信息、既往病史和抗高血压药物使用情况。为诊断CKD的快速进展,肌酐水平间隔一年测量两次。分析显示,各种左心室肥厚(LVH)参数表明,超过40%的ADPKD患者存在LVH。值得注意的是,观察一年后估计肾小球滤过率(eGFR)下降与左心室质量增加有关。其他常见发现包括39%的患者存在无症状左心室舒张功能障碍(ALVDD)、39%的患者左心房(LA)扩大以及80%的患者存在轻度瓣膜反流。射血分数、主动脉根部内径和二尖瓣脱垂的患病率没有显著增加。不同eGFR阶段的心脏指数没有实质性差异。LVH、LA扩大、ALVDD和瓣膜反流是ADPKD心脏表型的特征。不同CKD阶段的心脏指数没有差异,表明ADPKD的诊断是其发生的主要驱动因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d1c0/11612295/6c62bc4391db/41598_2024_81536_Fig1_HTML.jpg

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