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.

中文翻译：

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天然和手术矫正的先天性畸形心脏的尸检结果范围：10年的单中心经验

目标

我们回顾了在我们中心于1/2010年至4/2020年期间评估的一系列先天性心脏缺陷，评估了其总体解剖结构，评估了年龄分布，评估了所执行的手术程序，并关联了总体和超声检查结果。

方法

这项研究包括所有经过专门心脏尸检的尸检病例和移植心脏。回顾性评估尸检结果，包括先天性心脏缺陷的总体描述以及超声心动图检查结果。在经过手术矫正的心脏中，手术记录也包括在内。所有先天性心脏缺陷和外科手术均分为主要类别和附加类别。

结果

该研究包括92例活产儿尸体死亡，7例死产，2例年轻成年人，50例人工流产和5例离体心脏，中位年龄为36周。最常见的先天性先天性心脏病缺陷是左心发育不全，主动脉瓣狭窄，间隔缺损或持续性动脉干。51例患者接受了以瓣膜成形术，主动脉成形术和导致单心室循环的手术为主要特征的外科手术修复。在天然心脏中，有4例产后和16例流产/死产病例显示总体和超声检查结果不一致，这主要归因于室间隔缺损。与一致组相比，不一致组的妊娠年龄要低得多（P = .007）。

结论

尸检继续提供有关先天性心脏缺陷形态的基本信息。但是，遇到的先天性心脏缺陷通常很复杂，由于人工流产或死产，通常需要手术矫正或评估。因此，对于正确的总心脏评估，心脏解剖对病理学家提出了很高的要求。这也是产前心脏病学质量控制的重要组成部分。