Rao Sruti, Najm Hani K, Stewart Robert D, Ahmad Munir, Erenberg Francine, Yaman Malek
Department of Pediatric Cardiology, Cleveland Clinic Children's Hospital, Cleveland, Ohio.
Department of Pediatric Cardiothoracic and Vascular Surgery, Cleveland Clinic Children's Hospital, Cleveland, Ohio.
Echocardiography. 2019 May;36(5):996-1000. doi: 10.1111/echo.14334. Epub 2019 Apr 22.
Tetralogy of Fallot/Absent Pulmonary Valve (TOF/APV) has been classically associated with the absence of a patent ductus arteriosus (PDA). We present a rare case of APV in TOF with a discontinuous left pulmonary artery (LPA) that was suspected during fetal echocardiogram. Postnatal echocardiogram confirmed the origin of a hypoplastic LPA from the PDA. Despite an aneurysmal (right pulmonary artery) (RPA), axial imaging demonstrated widely patent tracheobronchial system with no evidence of bronchial compression. Clinically, the child required only minimal respiratory support. Genetic testing was positive for 22 q11deletion, commonly associated with this lesion. Surgery consisted of unifocalization of the discontinuous LPA with placement of a valved pulmonary homograft during complete repair of this lesion. Our case highlights the importance of prenatal detection, to aid in the prompt initiation of prostaglandins so as to ensure early rehabilitation of the left lung. Inability to visualize one of the branch pulmonary arteries (PA's) and a PDA on fetal echocardiogram in TOF/APV must raise suspicion for an eccentric branch PA with ductal origin.
法洛四联症/肺动脉瓣缺如（TOF/APV）传统上与动脉导管未闭（PDA）的缺失有关。我们报告了一例罕见的TOF合并APV病例，胎儿超声心动图检查怀疑存在左肺动脉（LPA）中断。产后超声心动图证实发育不良的LPA起源于PDA。尽管（右肺动脉）（RPA）呈瘤样扩张，但轴向成像显示气管支气管系统广泛通畅，无支气管受压迹象。临床上，患儿仅需极少的呼吸支持。基因检测显示22q11缺失呈阳性，此病变通常与之相关。手术包括在该病变的完全修复过程中，对中断的LPA进行单灶化处理并植入带瓣同种异体肺动脉。我们的病例强调了产前检测的重要性，有助于及时启动前列腺素治疗，以确保左肺早期恢复。在TOF/APV的胎儿超声心动图检查中，若无法显示一支肺分支动脉（PA）和PDA，则必须怀疑存在起源于导管的偏心分支PA。
