Louise Calder A
Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Private Bag 92024, Victoria Street West, Auckland 1142, New Zealand.
Pediatr Cardiol. 2011 Oct;32(7):875-84. doi: 10.1007/s00246-011-0003-z. Epub 2011 May 26.
Symmetry of bronchi, lung lobation, and atrial appendages is frequently found in patients with heterotaxy syndromes. To evaluate the reliability of thoracic situs as an indicator of atrial appendage morphology, thoracic situs was assessed in 306 postmortem cases: 250 with atrial situs solitus and 56 with heterotaxy syndromes. Five features that indicate thoracic situs were assessed: (1) lung lobation, (2) lengths of main bronchi, (3) ratio of left to right (L/R) bronchial lengths, (4) relationship of bronchi to ipsilateral pulmonary artery, and (5) number of cartilage rings in each main bronchus. In the group with heterotaxy, the expected symmetrical lung lobation, lengths of bronchi, number of cartilage rings, and relations to pulmonary arteries were found in 77, 77, 77, and 95% of cases, respectively. The ratios of L/R bronchial lengths were ≤ 1.5 in 90% of cases. The relations of the bronchi to the pulmonary arteries were the best predictors of symmetrical atrial appendages or splenic syndromes. Bronchial-atrial discordance occurred in ten cases: in situs solitus in one case and in heterotaxy in nine cases. Detection of heterotaxy syndromes is important because of the associated severe congenital cardiac defects. No single feature of thoracic situs is completely reliable. All available data should be used to make the diagnosis.
异心综合征患者常出现支气管、肺叶及心耳的对称性。为评估胸部位置作为心耳形态指标的可靠性，对306例尸检病例进行了胸部位置评估：250例心房位置正常，56例患有异心综合征。评估了五个指示胸部位置的特征：（1）肺叶划分，（2）主支气管长度，（3）左右支气管长度之比（L/R），（4）支气管与同侧肺动脉的关系，以及（5）每个主支气管中的软骨环数量。在异心组中，分别有77%、77%、77%和95%的病例出现了预期的对称肺叶划分、支气管长度、软骨环数量以及与肺动脉的关系。90%的病例中L/R支气管长度之比≤1.5。支气管与肺动脉的关系是对称心耳或脾脏综合征的最佳预测指标。支气管与心房不一致的情况发生在10例中：心房位置正常的有1例，异心的有9例。由于伴有严重的先天性心脏缺陷，检测异心综合征很重要。胸部位置的单一特征都不完全可靠。应使用所有可用数据进行诊断。
