Abstract

Introduction: Hydroxychloroquine (HCQ) is a commonly used drug for autoimmune diseases. However, it can in rare instances result in life-threatening cardiac consequences. We present a case of HCQ-induced cardiomyopathy in a 61-year-old female. Case presentation: A 61-year-old female with a 15-year history of SLE (Systemic Lupus Erythematosus) presented with new onset heart failure and atrial fibrillation. The patient reported slowly progressive dyspnea on exertion for the last 6 months with worsening bilateral lower extremity swelling. A TTE revealed a moderately dilated left ventricle with an ejection fraction of 20%, with biatrial enlargement. An admission EKG showed new-onset atrial fibrillation. Subsequently, left and right heart catheterization showed normal coronaries, elevated filling pressures with equalization of the wedge, and right atrial pressure suggestive of a restrictive etiology. cMRI was deferred as the patient had multiple abdominal pellets from a prior gunshot wound. An endomyocardial biopsy was obtained which revealed myocardial vacuolization and lipofuscin deposition consistent with HCQ-induced cardiomyopathy. HCQ was discontinued and the patient was discharged on Guideline-directed medical therapy with repeat TTE showing an improved ejection fraction of 40% without the persistence of any symptoms. Discussion: Myocardial toxicity associated with HCQ remains a difficult diagnosis, particularly due to the multiplicity of clinical presentations, echocardiographic and EKG features. The onset of cardiomyopathy in these patients is seen after exposure to a high cumulative dose. Presently there are no guidelines regarding the need for cardiac surveillance in patients using HCQ, unlike the need for Ophthalmic exams. Therefore, it is paramount that patients exposed to high cumulative doses of HCQ be screened in a cost-effective way. Especially since a delayed diagnosis is associated with a poorer prognosis.

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