Suryono Suryono, Wulandari Pipiet, Ariyanti Dwi, Maulana Aditha Satria, Sembodo R Handi, Junior Narendra Wahyu, Saputra Antonius Dwi
Departement of Cardiology, Faculty of Medicine, University of Jember - Dr. Soebandi General Hospital, Jl. Kalimantan No. 37, Jember, East Java, 68121, Indonesia.
Departement of Radiology, Faculty of Medicine, University of Jember - Dr. Soebandi General Hospital, Jl. Kalimantan No. 37, Jember, East Java, 68121, Indonesia.
Egypt Heart J. 2022 May 21;74(1):41. doi: 10.1186/s43044-022-00279-5.
Takayasu arteritis (TA) is included in large vessel vasculitis with unknown aetiopathogenesis. TA is one of the rare diseases with a predilection for young women. The diagnosis of TA is difficult due to variation in clinical presentations and non-specific initial symptoms. This case demonstrates rare TA in a young male with congestive heart failure as the predominant manifestation.
We report a 26-year-old male presented with severe dyspnea, palpitation, orthopnea, paroxysmal nocturnal dyspnea, and claudication in the left arm. Four limbs blood pressure discrepancy was present. Chest X-ray showed cardiomegaly with calcification aortic arch and pulmonary edema. Echocardiography revealed that left ventricular ejection fraction decreased with severe aortic and mitral valve regurgitation. Computed tomography angiography showed stenosis of the left common carotid artery and total occlusion of the left subclavian artery with collateral artery. There was vascular thickness and calcification from the peri-aortic valve, ascending aorta, aortic arch, and thoracic descending aorta until abdominal aorta with high-grade stenosis on the inferior side of the renal artery branching accompanied by a post-stenotic dilatation.
This patient's heart failure was precipitated by secondary hypertension and aortic regurgitation caused by vasculitis of TA. In general, there is no difference in the management of congestive heart failure in patients with TA. Optimized pharmacology therapy with combination steroid and methotrexate successfully inducing remission of TA after 3-months follow-up.
大动脉炎(TA)属于病因发病机制不明的大血管血管炎。TA是一种好发于年轻女性的罕见疾病。由于临床表现多样且初始症状不具特异性,TA的诊断较为困难。本病例展示了一名以充血性心力衰竭为主要表现的年轻男性患者的罕见TA病例。
我们报告一名26岁男性,出现严重呼吸困难、心悸、端坐呼吸、阵发性夜间呼吸困难以及左臂间歇性跛行。存在四肢血压差异。胸部X线显示心脏增大,主动脉弓钙化及肺水肿。超声心动图显示左心室射血分数降低,伴有严重的主动脉瓣和二尖瓣反流。计算机断层血管造影显示左颈总动脉狭窄,左锁骨下动脉完全闭塞并伴有侧支动脉。从主动脉瓣周围、升主动脉、主动脉弓及胸降主动脉直至腹主动脉存在血管增厚及钙化,在肾动脉分支下方有高度狭窄并伴有狭窄后扩张。
该患者的心力衰竭是由TA血管炎引起的继发性高血压和主动脉反流所致。一般而言,TA患者充血性心力衰竭的治疗并无差异。优化的药物治疗,联合使用类固醇和甲氨蝶呤,在3个月的随访后成功诱导TA缓解。