先天性心脏畸形患者死后尸检结果的范围：10 年单中心经验。

Spectrum of postmortem autopsy findings in native and surgically corrected hearts with congenital malformations: a 10-year single-center experience.

机构信息

Clinical and Transplant Pathology Centre, Institute for Clinical and Experimental Medicine, Videnska 1958/9, Prague 4, 140 21, Czech Republic; Department of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, Prague 5, 150 06, Czech Republic; Department of Pathology and Molecular Medicine, 3rd Faculty of Medicine, Charles University and Thomayer Hospital, Videnska 800, Prague 4, 140 59, Czech Republic.

Children's Heart Centre, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, Prague 5, 150 06, Czech Republic.

出版信息

Cardiovasc Pathol. 2021 Mar-Apr;51:107309. doi: 10.1016/j.carpath.2020.107309. Epub 2020 Nov 12.

DOI:10.1016/j.carpath.2020.107309

PMID:33189923

Abstract

OBJECTIVES

We reviewed a spectrum of congenital heart defects assessed in our center between 1/2010 and 4/2020, evaluated their gross anatomy, assessed the age distribution, evaluated performed surgical procedures, and correlated gross and ultrasound findings.

METHODS

All necroptic cases and explanted hearts that underwent specialized cardiac autopsy were included in this study. Autopsy findings including gross description of congenital heart defects together with echocardiographic findings were retrospectively assessed. In surgically corrected hearts, the operation records were included as well. All congenital heart defects and surgical procedures were subclassified into main and additional category.

RESULTS

The study included 92 necroptic cases of live-born children, 7 stillbirths, 2 cases of young adults, 50 induced abortions, and 5 explanted hearts, with median age 36 weeks. The most frequently encountered leading congenital heart defects were hypoplastic left heart syndrome, aortic stenosis, septal defects, or persistent arterial trunk. Fifty-one patients underwent surgical repair represented mainly by valvuloplasties, aortoplasty, and procedures leading to univentricular circulation. In the native hearts, 4 postnatal and 16 abortion/stillbirth cases showed discordance between gross and sonographic findings, mainly attributed to missed ventricular septal defect. Gestational age of the discordant group was significantly lower compared to the concordant group (P= .007).

CONCLUSIONS

Autopsy continues to provide essential information about the morphology of congenital heart defects. However, the encountered congenital heart defects were usually complex, often surgically corrected or evaluated as a result of induced abortion or still birth. Cardiac autopsy therefore places high demands on pathologists with regards to proper gross heart assessment. It is also an invaluable part of quality control in prenatal cardiology.

摘要

目的

我们回顾了 2010 年 1 月至 2020 年 4 月期间在我们中心评估的一系列先天性心脏缺陷，评估了其大体解剖结构，评估了年龄分布，评估了所进行的手术程序，并将大体和超声检查结果进行了相关性分析。

方法

所有尸检病例和接受专门心脏解剖的植入心脏均包括在本研究中。回顾性评估尸检结果，包括先天性心脏缺陷的大体描述以及超声心动图检查结果。在接受手术矫正的心脏中，还包括手术记录。所有先天性心脏缺陷和手术程序均分为主要和辅助类别。

结果

该研究包括 92 例活产儿尸检病例、7 例死产儿、2 例年轻成人、50 例人工流产和 5 例植入心脏，中位胎龄为 36 周。最常见的主要先天性心脏缺陷是左心发育不全综合征、主动脉瓣狭窄、室间隔缺损或永存动脉干。51 例患者接受了手术修复，主要包括瓣膜成形术、主动脉成形术和导致单心室循环的手术。在原生心脏中，4 例新生儿和 16 例流产/死产病例的大体和超声检查结果存在差异，主要归因于室间隔缺损漏诊。差异组的胎龄明显低于一致组（P =.007）。