The Heart in Vasculitis

Any cardiac tissue can be affected in most primary vasculitides. However, certain entities such as eosinophilic granulomatosis with polyangiitis (or Churg-Strauss syndrome) and Takayasu arteritis involve the heart in up to 60% of patients. Vasculitic cardiac complications are important to recognize because they have been linked to increased mortality and impact the treatment decision-making process. This article reviews the spectrum of cardiac manifestations in adults with large-, medium-, and small-vessel primary systemic vasculitides as well as their diagnosis and treatment.

Key points

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Pericarditis, myocarditis, coronary arteritis, and valvular heart disease have variable incidence across all different forms of primary vasculitis.
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Arteritis of the epicardial coronary arteries is more frequent in Takayasu’s arteritis, polyarteritis nodosa, and Behcet’s disease; however, angiitis of the main coronaries and vasculitis of the myocardial coronary microcirculation have been observed in most primary vasculitides.
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Pathology and cardiac imaging studies have found that cardiac involvement in vasculitis is often subclinical (eg, Takayasu’s arteritis, polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis, and Behcet’s disease); however, the morbidity and mortality impact of this phenomenon remains unknown.
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With the exception of pericarditis, most cardiac manifestations require aggressive immunosuppression, whereas coronary and valvular disease may also need surgical intervention with revascularization or valve replacement.
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When surgery is indicated, perioperative and long-term control of the inflammation is of paramount importance to assure surgical success and prevent future complications, such as graft and stent restenosis or valvular dehiscence.

Introduction

Heart involvement in primary systemic vasculitides is important to recognize, because it has been linked to increased mortality and requires prompt diagnosis and treatment. Almost all primary vasculitides can target the heart. Although cardiac compromise is a rare manifestation in vasculitis, with less than 10% of patients affected overall, certain entities, such as eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) and Takayasu’s arteritis (TAK) can cause heart complications in up to 60% of patients. Any cardiac tissue can be involved, and patients present with varied clinicopathologic syndromes including pericarditis, myocarditis, coronary arteritis, valvulopathy, and intracavitary cardiac thrombosis ( Table 1 ). A high index of suspicion is required, because electrocardiography (ECG), echocardiography, cardiac magnetic resonance imaging (cMRI), and coronary angiography are not completely sensitive or specific for diagnosing vasculitic injury, and histologic sampling is not always possible. Therapy depends on the specific manifestation and whether it represents active disease or prior damage (healed or “burnt out” disease). Both medical and surgical management approaches have been described, although controlled studies of treatment outcomes have not yet been carried out in most vasculitides. We herein describe cardiac manifestations of large-, medium-, and small-vessel primary systemic vasculitides in adults as well as their diagnosis and treatment.

Table 1

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