Unidade de Doenças Autoimunes, Medicina 7.2, Hospital Curry Cabral - Centro Hospitalar Lisboa Central (CHLC), Lisboa, Portugal; Serviço de Medicina Interna, Hospital de Abrantes - Centro Hospitalar Médio Tejo (CHMT), Abrantes, Portugal.
Unidade de Hipertensão Pulmonar, Serviço de Cardiologia - Hospital Garcia de Orta (HGO), Almada, Portugal.
Rev Port Cardiol (Engl Ed). 2020 Jan;39(1):47.e1-47.e5. doi: 10.1016/j.repc.2018.01.015. Epub 2020 Mar 12.
Pulmonary embolism due to primary antiphospholipid syndrome is rarely associated with chronic thromboembolic pulmonary hypertension, and therefore according to the latest guidelines on pulmonary hypertension, routine screening is not recommended. We describe a young patient with a late diagnosis of chronic thromboembolic pulmonary hypertension in the context of pulmonary embolism, primary antiphospholipid syndrome and suboptimal anticoagulation. Of note, mild cardiopulmonary symptoms were consistently misattributed to a depressive disorder because physical examination was normal, serial Doppler echocardiography failed to show pulmonary hypertension, and all other diagnostic tests were normal. Once symptoms became severe, positive screening tests led to the correct diagnosis and surgical referral, and bilateral pulmonary endarterectomy was successfully performed. This case demonstrates the need for extra awareness in patients with antiphospholipid syndrome and pulmonary embolism.
由于原发性抗磷脂综合征引起的肺栓塞很少与慢性血栓栓塞性肺动脉高压相关,因此根据肺动脉高压的最新指南,不建议常规筛查。我们描述了一例年轻患者,在肺栓塞、原发性抗磷脂综合征和抗凝不充分的背景下,出现慢性血栓栓塞性肺动脉高压的迟发性诊断。值得注意的是,由于体格检查正常、连续多普勒超声心动图未能显示肺动脉高压且所有其他诊断检查均正常,因此一直将轻微心肺症状错误归因于抑郁症。一旦症状变得严重,阳性筛查试验导致了正确的诊断和手术转诊,并成功进行了双侧肺动脉内膜切除术。该病例表明,对于抗磷脂综合征和肺栓塞患者,需要有额外的认识。