The adult congenital heart diseases (ACHD) population is exceeding the pediatric congenital heart diseases (CHD) population and is progressively expanding each year, representing more than 90% of patients with CHD. Of these, about 75% have undergone surgical and/or percutaneous intervention for palliation or correction. Autopsy can be a very challenging procedure in ACHD patients. The approach and protocol to be used may vary depending on whether the pathologists are facing native disease without surgical or percutaneous interventions, but with various degrees of cardiac remodeling, or previously palliated or corrected CHD. Moreover, interventions for the same condition have evolved over the last decades, as has perioperative myocardial preservations and postoperative care, with different long-term sequelae depending on the era in which patients were operated on. Careful clinicopathological correlation is, thus, required to assist the pathologist in performing the autopsy and reaching a diagnosis regarding the cause of death. Due to the heterogeneity of the structural abnormalities, and the wide variety of surgical and interventional procedures, there are no standard methods for dissecting the heart at autopsy. In this paper, we describe the most common types of CHDs that a pathologist could encounter at autopsy, including the various types of surgical and percutaneous procedures and major pathological manifestations. We also propose a practical systematic approach to the autopsy of ACHD patients.

中文翻译：

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患有先天性心脏病（ACHD）的成人的尸检。

成人先天性心脏病（ACHD）人群已超过儿童先天性心脏病（CHD）人群，且逐年扩大，占CHD患者的90%以上。其中，约 75% 接受了手术和/或经皮干预以进行姑息或矫正。对于 ACHD 患者来说，尸检可能是一个非常具有挑战性的过程。所使用的方法和方案可能会有所不同，具体取决于病理学家是否面临未经手术或经皮干预的原生疾病，但有不同程度的心脏重塑，或先前已得到缓解或纠正的先心病。此外，在过去的几十年里，针对同一病症的干预措施不断发展，围手术期心肌保存和术后护理也不断发展，根据患者接受手术的时代不同，会产生不同的长期后遗症。因此，需要仔细的临床病理学相关性来协助病理学家进行尸检并就死因做出诊断。由于结构异常的异质性以及外科和介入手术的多样性，尸检时没有解剖心脏的标准方法。在本文中，我们描述了病理学家在尸检时可能遇到的最常见的先心病类型，包括各种类型的外科手术和经皮手术以及主要病理表现。我们还提出了一种实用的系统方法来对 ACHD 患者进行尸检。