Chikkabyrappa Sathish, Mahadevaiah Guruprasad, Buddhe Sujatha, Alsaied Tarek, Tretter Justin
1 Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
2 Phoenix Children's Hospital, Phoenix, AZ, USA.
Semin Cardiothorac Vasc Anesth. 2019 Jun;23(2):225-236. doi: 10.1177/1089253218821382. Epub 2018 Dec 29.
Common arterial trunk (CAT), or truncus arteriosus, is a rare form of cyanotic congenital heart disease and is highly associated with DiGeorge syndrome (microdeletion 22q11.2). Prenatal diagnosis is highly feasible, allowing proper delivery planning and postnatal management. The clinical presentation is highly variable depending on the anatomical variation; however, most commonly presenting with mild cyanosis and significant tachypnea, although these patients can often go undetected in the immediate newborn period. Transthoracic echocardiography is adequate for diagnosis and detailed anatomical delineation in the majority. Additional imaging modalities such as cardiac catheterization, computed tomography angiography, or cardiac magnetic resonance imaging can be helpful in those with more complex pulmonary artery (PA) or aortic anatomy, or in the older repaired. The surgical management of CAT is complete repair in the neonatal period with resection of branch PAs from the CAT with placement of a right ventricular (RV)-to-PA conduit and patch closure of the ventricular septal defect. Overall surgical outcomes are excellent in most centers, with the expectation that the child will eventually outgrow the RV-to-PA conduit and require reoperation. Other potential reoperations or postsurgical interventions in addition to the RV-to-PA conduit may involve the truncal valve or branch PAs.
共同动脉干(CAT),即动脉干畸形,是一种罕见的青紫型先天性心脏病,与22q11.2微缺失的迪格奥尔格综合征高度相关。产前诊断非常可行,有助于进行恰当的分娩计划和产后管理。临床表现因解剖变异而差异很大;然而,最常见的表现是轻度青紫和显著呼吸急促,尽管这些患者在新生儿早期往往不易被发现。经胸超声心动图足以对大多数患者进行诊断和详细的解剖描绘。对于肺动脉(PA)或主动脉解剖结构更复杂的患者,或大龄已修复患者,心脏导管检查、计算机断层血管造影或心脏磁共振成像等其他成像方式可能会有所帮助。CAT的手术治疗是在新生儿期进行完全修复,从共同动脉干切除分支肺动脉,置入右心室(RV)至肺动脉导管,并修补室间隔缺损。大多数中心的总体手术效果良好,预计患儿最终会长大,不再需要RV至肺动脉导管并需要再次手术。除了RV至肺动脉导管外,其他潜在的再次手术或术后干预可能涉及动脉干瓣膜或分支肺动脉。