Department of Medico-Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy.
Cardiovasc Pathol. 2012 May-Jun;21(3):169-79. doi: 10.1016/j.carpath.2011.02.002. Epub 2011 Apr 12.
Juxtaposition of the atrial appendages is a rare congenital cardiac malformation, with the appendages both located on the left or right side of the great arteries. It is usually associated with cyanotic congenital heart disease. The aim of this report is to illustrate the anatomical features of normal and juxtaposed atrial appendages, with a review of the associated anomalies. In the Anatomical Collection of Congenital Heart Disease of the University of Padua, consisting in 1,526 specimens, we found 17 (1.1%) cases of atrial appendages juxtaposition with left juxtaposition in 15 (88%) and right juxtaposition in 2 (12%). Complete form was present in 11 cases and partial form in 6. In left juxtaposition, the situs was solitus in all, and the most frequent anomalies were complete transposition of great arteries in 9 (60%) and tricuspid atresia in 5 (33%); anomalies of position of the heart in the thorax (dextro-mesocardia) were present in 46% of cases, hypoplastic right ventricle in 73%, abnormal relation of the great arteries and subaortic or bilateral infundibulum in all. In right atrial juxtaposition, the atrial situs was solitus with mitral and pulmonary atresia in one case and left isomerism with aortic atresia and double-inlet right ventricle in the other. In describing this malformation, we propose to maintain the use of a positional definition using the terms right and left juxtaposition to describe the presence of both the appendages on the right or on the left side of the great arteries, respectively. The use of a morphological definition should be added in cases of situs inversus or isomerism, with description of the morphology of the appendage located in the wrong position.
心耳并置是一种罕见的先天性心脏畸形，两耳均位于大动脉的左侧或右侧。它通常与发绀型先天性心脏病有关。本报告的目的是说明正常和并置心耳的解剖特征，并复习相关的异常。在帕多瓦大学先天性心脏病解剖收藏中，有 1526 个标本，我们发现 17 例（1.1%）心耳并置，其中左并置 15 例（88%），右并置 2 例（12%）。完全型 11 例，部分型 6 例。在左并置中， situs 均为正位，最常见的异常是完全性大动脉转位 9 例（60%）和三尖瓣闭锁 5 例（33%）；心脏在胸腔内位置异常（右位心）占 46%，右心室发育不全占 73%，大动脉和主动脉下或双侧漏斗部关系异常均占 100%。在右心耳并置中，心房 situs 为正位，其中 1 例为二尖瓣和肺动脉闭锁，另 1 例为左同型异构伴主动脉闭锁和双入口右心室。在描述这种畸形时，我们建议使用位置定义来维持术语“右并置”和“左并置”，分别描述两个心耳分别位于大动脉的右侧或左侧。在 situs inversus 或同型异构的情况下，应增加形态学定义，描述位于错误位置的心耳形态。
