左心室心肌致密化不全患者的临床风险预测。

Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction.

机构信息

Cardiology Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Departament de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Inherited Cardiovascular Disease Unit and Cardiovascular Genetics. Hospital Universitari Vall d'Hebron, Barcelona, Spain.

出版信息

J Am Coll Cardiol. 2021 Aug 17;78(7):643-662. doi: 10.1016/j.jacc.2021.06.016.

DOI:10.1016/j.jacc.2021.06.016

PMID:34384546

Abstract

BACKGROUND

Left ventricular noncompaction (LVNC) is a heterogeneous entity with uncertain prognosis.

OBJECTIVES

This study sought to develop and validate a prediction model of major adverse cardiovascular events (MACE) and to identify LVNC cases without events during long-term follow-up.

METHODS

This is a retrospective longitudinal multicenter cohort study of consecutive patients fulfilling LVNC criteria by echocardiography or cardiovascular magnetic resonance. MACE were defined as heart failure (HF), ventricular arrhythmias (VAs), systemic embolisms, or all-cause mortality.

RESULTS

A total of 585 patients were included (45 ± 20 years of age, 57% male). LV ejection fraction (LVEF) was 48% ± 17%, and 18% presented late gadolinium enhancement (LGE). After a median follow-up of 5.1 years, MACE occurred in 223 (38%) patients: HF in 110 (19%), VAs in 87 (15%), systemic embolisms in 18 (3%), and 34 (6%) died. LVEF was the main variable independently associated with MACE (P < 0.05). LGE was associated with HF and VAs in patients with LVEF >35% (P < 0.05). A prediction model of MACE was developed using Cox regression, composed by age, sex, electrocardiography, cardiovascular risk factors, LVEF, and family aggregation. C-index was 0.72 (95% confidence interval: 0.67-0.75) in the derivation cohort and 0.72 (95% confidence interval: 0.71-0.73) in an external validation cohort. Patients with no electrocardiogram abnormalities, LVEF ≥50%, no LGE, and negative family screening presented no MACE at follow-up.

CONCLUSIONS

LVNC is associated with an increased risk of heart failure and ventricular arrhythmias. LVEF is the variable most strongly associated with MACE; however, LGE confers additional risk in patients without severe systolic dysfunction. A risk prediction model is developed and validated to guide management.

摘要

背景

左心室心肌致密化不全（LVNC）是一种预后不确定的异质性疾病。

目的

本研究旨在建立和验证主要不良心血管事件（MACE）的预测模型，并确定在长期随访中无事件发生的 LVNC 病例。

方法

这是一项回顾性纵向多中心队列研究，连续纳入符合超声心动图或心血管磁共振 LVNC 标准的患者。MACE 定义为心力衰竭（HF）、室性心律失常（VA）、系统性栓塞或全因死亡率。

结果

共纳入 585 例患者（年龄 45±20 岁，57%为男性）。左心室射血分数（LVEF）为 48%±17%，18%的患者存在延迟钆增强（LGE）。中位随访 5.1 年后，223 例（38%）患者发生 MACE：HF 110 例（19%），VA 87 例（15%），系统性栓塞 18 例（3%），34 例（6%）死亡。LVEF 是与 MACE 独立相关的主要变量（P<0.05）。在 LVEF>35%的患者中，LGE 与 HF 和 VA 相关（P<0.05）。使用 Cox 回归建立了 MACE 的预测模型，该模型由年龄、性别、心电图、心血管危险因素、LVEF 和家族聚集组成。在推导队列中的 C 指数为 0.72（95%置信区间：0.67-0.75），在外部验证队列中的 C 指数为 0.72（95%置信区间：0.71-0.73）。无心电图异常、LVEF≥50%、无 LGE 和阴性家族筛查的患者在随访期间未发生 MACE。