Antemortem and Post-Mortem Characteristics of Lethal Mitral Valve Prolapse Among All Countywide Sudden Deaths.

Antemortem and Post-Mortem Characteristics of Lethal Mitral Valve Prolapse Among All Countywide Sudden Deaths.
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DOI:
10.1016/j.jacep.2021.01.007
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发表时间:
2021-08
期刊:
JACC. Clinical electrophysiology
影响因子:
--
通讯作者:
Tseng ZH
Tseng ZH
中科院分区:
其他
文献类型:
--
作者:
Delling FN;Aung S;Vittinghoff E;Dave S;Lim LJ;Olgin JE;Connolly A;Moffatt E;Tseng ZH

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我们试图在一项对90岁以下连续心脏性猝死(SCD)的尸检研究中调查二尖瓣脱垂(MVP)的特征。多达2.3%的MVP患有SCD,但按照惯例,SCD很少被尸检证实。在一项对40年来年轻人的尸检研究中,7%的SCD是由MVP引起的;双叶受累、二尖瓣环分离(MAD)和替换性纤维化是常见的。在旧金山后SCD研究中,自2011年以来,对连续1000名年龄在18-90岁的WHO定义(推定)SCD进行了尸检,判定为603人。尸检定义的心律失常猝死(SAD)需要没有非心律失常的原因;MVP的诊断需要传单滚滚。我们回顾了100例尸检前的超声心动图,以确定尸检中遗漏的其他MVP。在603个推测的SCD中,339个(56%)是尸检定义的SADS,其中7个(1%)确定了MVP。通过回顾超声心动图,我们确定了另外6个MVP,在603个推定的SCD中至少有2%的患病率,在339个SADS中至少有4%的患病率(p=0.02比264个非SADS)。所有另外6个MVP均为单叶而不是双叶受累,且有轻微的二尖瓣返流,排除了血流动力学原因。不到一半的患者出现了替代性纤维化,但所有患者都有多部位间质纤维化。在一项对所有成人SCD的全县尸检研究中,MVP的患病率至少占SADS的4%,但尸检时遗漏了一半。单叶MVP往往在死后被漏诊。与年轻的SCD相比,老年SCD的致命性MVP并不总是有双叶解剖、替代性纤维化或MAD。高达2.3%的二尖瓣脱垂(MVP)患者发生心源性猝死(SCD),但按照惯例,SCD很少被尸检证实。虽然MVP的尸检研究仅限于较年轻的SCD,但SCD的尸检研究是对所有90岁以下的成年SCD进行的尸检。在这项研究中,MVP的患病率至少占尸检定义的心律失常猝死的4%,但尸检遗漏了一半的病例,全部为单肺。与年轻的SCD相比,老年SCD的致死性MVP并不总是存在双叶解剖、替代性纤维化或二尖瓣环分离;间质纤维化是普遍存在的。
We sought to investigate the characteristics of mitral valve prolapse (MVP) in a postmortem study of consecutive sudden cardiac deaths (SCDs) up to age 90. Up to 2.3% of MVPs suffer SCD, but by convention SCD is rarely confirmed by autopsies. In a postmortem study of young persons < 40 years, 7% of SCDs were caused by MVP; bileaflet involvement, mitral annular disjunction (MAD), and replacement fibrosis were common. In the San Francisco POST SCD Study, autopsies have been performed on >1000 consecutive WHO-defined (presumed) SCDs ages 18–90 since 2011, 603 adjudicated. Autopsy-defined sudden arrhythmic death (SAD) required absence of non-arrhythmic cause; MVP diagnosis required leaflet billowing. We reviewed 100 pre-mortem echocardiograms to identify additional MVPs missed on autopsy. Among 603 presumed SCDs, 339 (56%) were autopsy-defined SADs, with MVP identified in 7 (1%). We identified 6 additional MVPs by review of echocardiograms, for a prevalence of at least 2% among 603 presumed SCDs and 4% among 339 SADs (p = 0.02 vs 264 non-SADs). All 6 additional MVPs had monoleaflet rather than bileaflet involvement, and mild mitral regurgitation, ruling out hemodynamic cause. Less than half had MAD with replacement fibrosis, but all had multisite interstitial fibrosis. In a countywide postmortem study of all adult SCDs, MVP prevalence was at least 4% of SADs, but half were missed on autopsy. Monoleaflet MVP was often underdiagnosed postmortem. Compared to young SCDs, lethal MVP in older SCDs did not consistently have bileaflet anatomy, replacement fibrosis, or MAD. Up to 2.3% of individuals with mitral valve prolapse (MVP) suffer sudden cardiac death (SCD), but by convention SCD is rarely confirmed by autopsies. While postmortem studies of MVP are limited to younger SCDs, the POST SCD Study is a countywide postmortem investigation of all adult SCDs up to age 90. In this study, MVP prevalence was at least 4% of autopsy-defined sudden arrhythmic deaths, but half of cases, all monoleaflet, were missed by autopsy. Compared to young SCDs, lethal MVP in older SCDs did not consistently have bileaflet anatomy, replacement fibrosis, or mitral annular disjunction; interstitial fibrosis was universal.
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