Guha Anasuya, Antonova Petra, Zelinka Tomas, Vicha Ales, Pacak Karel, Chovanec Martin
Department of Otorhinolaryngology 3rd Faculty of Medicine and University Hospital Kralovske Vinohrady, Charles University Prague Czech Republic.
Department of Cardiology 2nd Faculty of Medicine and University Hospital Motol, Charles University Prague Czech Republic.
Clin Case Rep. 2024 Oct 16;12(10):e9448. doi: 10.1002/ccr3.9448. eCollection 2024 Oct.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, paragangliomas, and syringomyelia are uncommon diseases. Furthermore, in the absence of any genetic link and with less than five reported adult patients surviving unrepaired rare form of Tetralogy of Fallot, our case shows noteworthiness. The possibility of definitive treatment of these conditions is rendered unsafe due to this persistent defect. Thus, management and ongoing survival of this patient remains complex and challenging.
肺动脉闭锁合并室间隔缺损及主要体肺侧支动脉、副神经节瘤和脊髓空洞症是罕见疾病。此外,由于不存在任何遗传关联,且报道的未接受修复的法洛四联症罕见成年存活患者不到5例,我们的病例具有值得关注之处。由于这种持续性缺损,对这些病症进行确定性治疗的可能性变得不安全。因此,该患者的管理和持续生存仍然复杂且具有挑战性。