De Anirban, Dhawan Naresh, Joshi Saajan, Dwivedi Deepak
Senior Resident (Anaesthesia & Critical Care), Command Hospital (Eastern Command), Kolkata, West Bengal, India.
Head & Consultant (Anaesthesia & Critical Care), Command Hospital (Eastern Command), Kolkata, West Bengal, India.
Med J Armed Forces India. 2024 Dec;80(Suppl 1):S329-S332. doi: 10.1016/j.mjafi.2023.10.002. Epub 2023 Nov 20.
Eisenmenger syndrome (ES) is a rare congenital heart disease (CHD) that occurs in persons with large intracardiac anatomical defects which may go undetected for many years, consequently results in reduced life expectancies and high mortality in the third and fourth decade of life. Pregnancy in patients with CHD and pulmonary arterial hypertension (PAH) is presaged with challenges, and perioperative management of such cases remains a topic of debate. Our case had both, atypical presentation, not diagnosed until her third trimester of third pregnancy and a quick progression to a severe stage with PAH and CorPulmonale. The atypical presentation being persistent polycythemia, raising clinical suspicion and thorough clinical examination revealing differential cyanosis and clubbing of hands and feet in pre-anesthesia checkup (PAC). A multidisciplinary approach clinched the diagnosis, and the use of a novel integrated method for perioperative management incorporating all available literature and modern interventions highlighted the critical role of anesthesiologists at a tertiary care setup.
艾森曼格综合征(ES)是一种罕见的先天性心脏病(CHD),发生于有较大心脏内解剖缺陷的人群,这些缺陷可能多年未被发现,从而导致预期寿命缩短,在生命的第三和第四个十年死亡率较高。患有先天性心脏病和肺动脉高压(PAH)的患者怀孕面临诸多挑战,此类病例的围手术期管理仍是一个有争议的话题。我们的病例有两个特点,一是表现不典型,直到第三次怀孕的孕晚期才被诊断出来;二是迅速发展到严重阶段,出现肺动脉高压和肺心病。不典型表现为持续性红细胞增多症,引起临床怀疑,全面的临床检查在麻醉前检查(PAC)中发现了差异性发绀以及手足杵状指。多学科方法确诊了病情,采用一种结合所有现有文献和现代干预措施的新型围手术期综合管理方法,凸显了三级医疗中心麻醉医生的关键作用。