Takayasu Arteritis Unmasked During Device Infection Evaluation in Non-Ischemic Cardiomyopathy

Background

Takayasu arteritis is a rare large-vessel vasculitis affecting approximately 1–3 individuals per million annually, predominantly women aged 20–40 years. Clinically apparent myocardial involvement is uncommon, although heart failure may result from hypertension, valvular disease, coronary vasculitis, or inflammatory myocardial dysfunction.

Case Presentation

A 29-year-old woman presented with severe nonischemic cardiomyopathy (LVEF 13%) and acute renal failure requiring temporary hemodialysis. Coronary angiography showed no obstructive disease. Positive ANA and low complement prompted empiric corticosteroids and mycophenolate. She was subsequently transferred in cardiogenic shock requiring milrinone and intravenous diuresis. Cardiac MRI demonstrated myocardial fibrosis, while endomyocardial biopsy showed no active myocarditis. Further evaluation was unrevealing, and cryptogenic nonischemic cardiomyopathy was diagnosed.

After one year of maximally tolerated guideline-directed medical therapy, LVEF improved to 28%, and an extravascular ICD was implanted for primary prevention. Three weeks later, a pocket infection required system explantation. CT imaging unexpectedly demonstrated diffuse aortic and branch-vessel wall thickening with carotid, subclavian, and mesenteric involvement. Examination revealed an absent radial pulse, carotid bruits, and an inter-arm blood pressure discrepancy.

Findings

Inflammatory markers were elevated. Intraoperative cultures grew MSSA, while blood cultures remained negative. Retrospective review of CT imaging from one year earlier demonstrated similar large-vessel wall thickening with skip lesions, supporting a longstanding vasculitic process consistent with Takayasu arteritis.

Repeat cardiac MRI demonstrated LVEF improvement to 48%. Given ventricular recovery and absence of sustained ventricular arrhythmias or prior ICD therapies, device reimplantation was deferred.

Discussion & Conclusion

Takayasu arteritis may rarely present with severe unexplained cardiomyopathy before classic vascular manifestations become clinically apparent. In this case, large-vessel abnormalities were recognized incidentally during evaluation of an ICD-related infection, while retrospective imaging confirmed that the vasculitic process preceded the diagnosis by at least one year.

The absence of obstructive coronary disease or active myocarditis does not exclude cardiovascular involvement from Takayasu arteritis. Careful vascular examination and review of prior cross-sectional imaging were critical to diagnosis. This case also highlights the importance of reassessing ICD candidacy when ventricular dysfunction may be reversible and substantial LVEF recovery occurs.