单尖主动脉瓣在年轻人中的现有证据：系统性回顾和 Metanalysis。

IF 3 3区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Current Problems in Cardiology Pub Date : 2024-10-18 DOI:10.1016/j.cpcardiol.2024.102884

Andreina Carbone MD , Francesco Ferrara MD, PhD , Roberta Bottino MD , Valentina Russo MD , Salvatore Rega MD , Giuseppe Limongelli MD, PhD , Eduardo Bossone MD, PhD , Arturo Evangelista MD, PhD

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引用次数: 0

摘要

主动脉瓣单尖（UAV）是一种罕见的先天性瓣膜异常，常被误诊为更常见的主动脉瓣双尖（BAV）。本研究旨在探讨患有 UAV 的年轻成人的人口统计学、临床特征、诊断、手术方案、短期和长期疗效。研究人员对1971-2024年期间诊断为UAV的患者（≥14岁）进行了系统回顾和荟萃分析。在检索到的 2953 项研究中，有 67 项病例报告、6 项系列病例（n=130）和 13 项回顾性研究（n=918）被纳入分析。采用随机效应模型对回顾性研究的数据进行汇总，以估算汇总风险比和平均差异。UAV 多为成人单发（诊断时平均年龄为 36 岁，76-79% 为男性）。UAV患者最常见的相关疾病是主动脉瓣狭窄（3.8%至12%）、室间隔缺损（3%）和特纳综合征（3%）。一般来说，诊断是通过 TTE 进行的，并通过 TEE（+/- 3D-TEE ）确诊。最常见的手术类型是主动脉瓣置换术。在回顾性研究和病例报告中，分别有 44% 和 35% 的病例描述了升主动脉扩张。在回顾性研究和病例报告中，分别有 38% 和 27% 的报告称同时进行了升主动脉置换/修复手术。有 3 项研究报告了 10 年的总生存率，从 95% 到 98% 不等。UAV 应被视为不同于 BAV 的独立个体。关于家族发病的可能性、主动脉的相关组织病理学变化以及理想的随访和干预措施，还需要进一步的研究。

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Current evidence of unicuspid aortic valve in young adults: A systematic review and metanalysis

Unicuspid aortic valve (UAV) is a rare congenital valvular anomaly, often misdiagnosed as the more prevalent bicuspid aortic valve (BAV). The aim of this study was to explore demographic, clinical characteristics, diagnosis, surgical options, short and long term outcomes of young adults with UAV.

A systematic review and meta-analysis of literature were conducted for studies (1971-2024) including patients (≥ 14 years old) with diagnosis of UAV. Among the 2953 studies retrieved, 67 case reports, 6 case series (n = 130) and 13 retrospective studies (n = 918), were included in the analysis. Data from retrospective studies were aggregated using a random effects model for estimating the pooled risk ratio and mean difference.

UAV is mostly unicommissural in adults (mean age 36 years old at diagnosis, 76–79 % males). The most common conditions associated in UAV patients were aortic coarctation (from 3.8 to 12 %), ventricular septal defect (3 %) and Turner syndrome (3 %). In general, the diagnosis was performed with TTE and confirmed with TEE (+/- 3D-TEE). The most common types of surgery were AVR. Dilated ascending aorta was described in 44 % and 35 % of retrospective studies and case reports, respectively. Concomitant ascending aorta replacement/repair was reported 38 % and 27 % of retrospective studies and case reports, respectively. Overall survival was reported in 3 studies, ranging from 95 to 98 % at 10 years.

UAV should be considered a separate entity from BAV. Further investigations with regards to the possibility of a familial incidence, associated histopathological changes in the aorta, and ideal follow up and intervention are needed.

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来源期刊

Current Problems in Cardiology 医学-心血管系统

CiteScore

4.80

自引率

2.40%

发文量

392

审稿时长

6 days

期刊介绍： Under the editorial leadership of noted cardiologist Dr. Hector O. Ventura, Current Problems in Cardiology provides focused, comprehensive coverage of important clinical topics in cardiology. Each monthly issues, addresses a selected clinical problem or condition, including pathophysiology, invasive and noninvasive diagnosis, drug therapy, surgical management, and rehabilitation; or explores the clinical applications of a diagnostic modality or a particular category of drugs. Critical commentary from the distinguished editorial board accompanies each monograph, providing readers with additional insights. An extensive bibliography in each issue saves hours of library research.