弯刀综合征,一种罕见的先天性心脏异常,涉及肺静脉异常引流到全身静脉,导致独特的成像特征,类似于弯曲的剑。此病例报告提供了巴基斯坦弯刀综合征的独特实例,强调其临床重要性和管理的挑战。一名26岁的女性,从小就有反复的肺部感染和呼吸道症状,被诊断患有弯刀综合征。放射学评估,包括胸部X光片,计算机断层扫描肺血管造影(CTPA),经胸超声心动图,确认存在源自右半膈并与下腔静脉(IVC)连接的弯曲血管。病人和她的医疗团队选择了保守的管理,涉及多学科护理,为感染量身定制的治疗方法,定期监测。弯刀综合征的罕见性需要仔细的诊断和管理决策。虽然经常推荐手术干预,该病例证明了根据患者偏好和不断变化的临床病程选择保守治疗的复杂性.文献综述揭示了手术和保守方法的不同结果,强调个性化战略的必要性。放射技术,比如CTPA和MRI,在诊断和监测中起着举足轻重的作用。此病例报告强调了弯刀综合征的临床意义,特别是在报告病例有限的地区,就像巴基斯坦。多学科管理方法,关于保守治疗的决策过程,独特的放射学发现有助于医学界对这种罕见疾病的理解。
Scimitar syndrome, a rare congenital cardiac anomaly, involves abnormal pulmonary vein drainage into systemic veins, leading to distinct imaging features resembling a curved-blade sword. This
case report presents a unique instance of scimitar syndrome in Pakistan, emphasizing its clinical importance and the challenges of management. A 26-year-old female with a history of recurrent pulmonary infections and respiratory symptoms since childhood was diagnosed with scimitar syndrome. Radiological assessments, including chest X-rays, computed tomography pulmonary angiograms (CTPA), and transthoracic echocardiography, confirmed the presence of a curved vessel originating from the right hemidiaphragm and connecting with the inferior vena cava (IVC). The patient and her medical team opted for conservative management, involving multidisciplinary care, tailored treatment for infections, and regular monitoring. The rarity of Scimitar syndrome necessitates careful diagnosis and management decisions. While surgical intervention is often recommended, this
case demonstrates the complexities of choosing conservative management based on patient preferences and the evolving clinical course. A literature review reveals varied outcomes of surgical and conservative approaches, emphasizing the need for personalized strategies. Radiological techniques, such as CTPA and MRI, play pivotal roles in diagnosis and monitoring. This
case report underscores the clinical significance of scimitar syndrome, particularly in regions with limited reported cases, like Pakistan. The multidisciplinary management approach, the decision-making process regarding conservative treatment, and the unique radiological findings contribute to the medical community\'s understanding of this rare condition.