Noel Cory V, Kovalchin John P, Adler Brent, Yates Andrew R
Division of Cardiology, Department of Pediatrics, Nationwide Children's Hospital, Columbus, Ohio, USA.
Congenit Heart Dis. 2014 Jul-Aug;9(4):294-9. doi: 10.1111/chd.12138. Epub 2013 Sep 19.
Tracheobronchial anomalies are rare congenital malformations that are typically managed conservatively. Several reports have documented their increased incidence in patients with congenital heart disease. However, none of these reports have detailed the incidence found among patients with hypoplastic left heart syndrome (HLHS). Airway obstruction, whether by extrinsic compression or an undiagnosed tracheobronchial anomaly, in the perioperative period may have significant morbidity in this tenuous population.
From June 2003 to August 2011, 164 consecutive patients with HLHS underwent a palliative surgical procedure for their cardiac disease. Sixty-three of these patients received either multidetector computed tomography (CT) of the chest or cardiac magnetic resonance imaging (MRI). A total of 124 studies (106 CT, 18 MRI) were performed during this time span. The studies were reviewed independently by a pediatric cardiologist and a pediatric radiologist. Length of intubation, intensive care unit (ICU) stay, and hospital stay were also reviewed for all patients.
Three of the 63 patients had a congenital abnormality of the tracheobronchial tree (4.8%), which is higher than the incidence that has been reported in the general population. Two of the patients had bilateral left-sided bronchus with an absence of the right upper lobe bronchus. The third patient was found to have a very rudimentary right upper lobe bronchus with absence of the right upper lobe of the lung. The mean intubation time was not significantly different between the groups (P = .615). There was no significant difference of either the total ICU or hospital stay between the two groups.
Our study demonstrates a higher incidence of tracheobronchial anomalies among patients with HLHS, a severe form of cyanotic congenital heart disease. Patients with a tracheobronchial abnormality did not show a difference in morbidity during the postoperative time period.
气管支气管异常是罕见的先天性畸形，通常采用保守治疗。有几份报告记录了先天性心脏病患者中其发病率的增加。然而，这些报告均未详细说明左心发育不全综合征（HLHS）患者中的发病率。围手术期气道阻塞，无论是外在压迫还是未被诊断出的气管支气管异常，在这群脆弱的患者中都可能导致严重的发病情况。
从2003年6月至2011年8月，164例连续的HLHS患者因心脏病接受了姑息性外科手术。其中63例患者接受了胸部多排螺旋计算机断层扫描（CT）或心脏磁共振成像（MRI）。在此时间段内共进行了124项检查（106例CT，18例MRI）。这些检查由一名儿科心脏病专家和一名儿科放射科医生独立进行评估。还对所有患者的插管时间、重症监护病房（ICU）住院时间和住院时间进行了评估。
63例患者中有3例存在气管支气管树先天性异常（4.8%），高于一般人群中报告的发病率。其中2例患者双侧左支气管，右上叶支气管缺如。第3例患者被发现右上叶支气管非常发育不全，右肺上叶缺如。两组之间的平均插管时间无显著差异（P = 0.615）。两组之间的总ICU住院时间或住院时间均无显著差异。
我们的研究表明，在HLHS（一种严重的青紫型先天性心脏病）患者中，气管支气管异常的发病率更高。气管支气管异常的患者在术后期间的发病率没有差异。
