TAKAYASU ARTERITIS MASQUERADING AS IDIOPATHIC DILATED CARDIOMYOPATHY: DEMONSTRATING DIAGNOSTIC PITFALLS AND SUCCESSFUL ENDOVASCULAR MANAGEMENT

Abstract

Takayasu arteritis (TA) is a chronic granulomatous vasculitis affecting large vessels, with cardiovascular complications occurring in approximately 40% of patients¹. Dilated cardiomyopathy (DCM) represents an uncommon but serious manifestation, occurring in 6% of TA cases². The nonspecific presentation often leads to misdiagnosis as idiopathic DCM, particularly when blood pressure appears normal due to cardiac dysfunction³. Early recognition is crucial as endovascular intervention can significantly improve cardiac function⁴. This study examines cases initially misdiagnosed as idiopathic DCM, subsequently identified as TA, highlighting diagnostic challenges and demonstrating successful endovascular management strategies in this vulnerable population.

Methods : We conducted a retrospective analysis of our institutional databases (January 2018-December 2023) identifying patients initially diagnosed with idiopathic DCM who were subsequently confirmed to have TA. Data collection included demographics, clinical presentation, laboratory markers (ESR, CRP), multimodality imaging (echocardiography, CT, MRI, angiography), intervention details, and follow-up outcomes. Primary endpoints included correct diagnosis establishment and procedural success. Secondary endpoints assessed left ventricular ejection fraction (LVEF) improvement and symptom resolution at follow-up.

Results: Eight patients (6 females, 2 males; mean age 15 years, range 11-20) were identified. All presented with NYHA class II-III symptoms and LVEF 15-35%. Diagnostic workup revealed elevated inflammatory markers in all cases. Vascular involvement included descending thoracic aortic stenosis (n=5), bilateral renal artery stenosis (n=2), and diffuse aortitis with myocarditis (n=1). All patients underwent endovascular intervention with technical success achieved in all procedures. Mean LVEF improved from 25% to 45% at 6-month follow-up. NYHA class improved to I-II in all patients. One patient required repeat intervention for restenosis, ultimately requiring surgical planning. No procedure-related complications occurred.

Conclusions: TA should be considered in young patients presenting with DCM, particularly in endemic regions. Key diagnostic clues include elevated inflammatory markers, abnormal pulse examination, and increased carotid intima-media thickness. Multimodality imaging is essential for diagnosis. Endovascular intervention provides effective management with significant cardiac function improvement. A multidisciplinary approach involving cardiology, rheumatology, and interventional radiology optimizes outcomes. Systematic screening protocols could prevent diagnostic delays and improve long-term prognosis in this population.