The Missed Culprit for A Complex Case of Non-ischemic Cardiomyopathy

Background Hydroxychloroquine (HCQ) is one of the immunomodulatory medications used in autoimmune diseases. Usually, HCQ is well tolerated with minimal side effects. However, cardiotoxicity is a rare and serious complication of HCQ. We present a rare case of HCQ-induced cardiomyopathy due to chronic HCQ use. Case 60-year-old female patient with medical history of systemic lupus erythematosus on chronic HCQ therapy for 28 years, non-ischemic cardiomyopathy with LVEF 30% (for more than 10 years), and complete heart block status post pacemaker insertion presented to the hospital with acute chest pain, progressive shortness of breath, and severe weight loss. Physical examination was unremarkable. EKG showed no concerning signs of acute myocardial ischemia. Lab values showed NT-proBNP >35,000 pg/ml and elevated High Sensitivity Cardiac Troponin with 0-hour and 1-hour values of 186 pg/mL and 336 pg/ml respectively. Due to her symptoms, patient underwent a coronary angiogram that showed normal coronaries and right-sided heart catheterization that showed elevated biventricular filling pressures (right atrium pressure of 10 mmHg, RV systolic pressure of 28 mmHg and diastolic pressure of 8 mmHg, mean pulmonary artery pressure of 21 mmHg, and wedge pressure of 15 mmHg) with severely reduced cardiac index of 2.0 L/min/m2. Repeated echocardiogram showed LVEF of 30% with global hypokinesis. Patient was started on dobutamine with significant improvement in her symptoms. Decision-making HCQ-induced cardiomyopathy was suspected. Patient underwent an endomyocardial biopsy that revealed a pathognomonic finding of myocyte vacuolization, consistent with HCQ-induced cardiomyopathy (Figure A). HCQ was discontinued immediately. However, patient was severely deconditioned and malnourished secondary to the end-stage heart failure and was a poor candidate for direct orthoptic heart transplantation or durable mechanical circulatory support. Patient accepted hospice care and passed away peacefully. Conclusion HCQ can rarely cause non-ischemic cardiomyopathy. Main risk factors include longer duration of therapy and higher cumulative dose of HCQ. Patients remain asymptomatic for a long time and then present with symptoms of acute heart failure. The main diagnostic test is endomyocardial biopsy and histopathological examination showing myocyte vacuolization. Medication reconciliation is crucial in patients with non-ischemic cardiomyopathy as some commonly used medications can be the cause of the cardiomyopathy. Management includes immediate discontinuation of HCQ and guideline-directed medical therapy for heart failure. The prognosis of HCQ-induced cardiomyopathy varies from complete recovery if HCQ is stopped early enough, to partial or no recovery, if irreversible cell injury happens. Hydroxychloroquine (HCQ) is one of the immunomodulatory medications used in autoimmune diseases. Usually, HCQ is well tolerated with minimal side effects. However, cardiotoxicity is a rare and serious complication of HCQ. We present a rare case of HCQ-induced cardiomyopathy due to chronic HCQ use. 60-year-old female patient with medical history of systemic lupus erythematosus on chronic HCQ therapy for 28 years, non-ischemic cardiomyopathy with LVEF 30% (for more than 10 years), and complete heart block status post pacemaker insertion presented to the hospital with acute chest pain, progressive shortness of breath, and severe weight loss. Physical examination was unremarkable. EKG showed no concerning signs of acute myocardial ischemia. Lab values showed NT-proBNP >35,000 pg/ml and elevated High Sensitivity Cardiac Troponin with 0-hour and 1-hour values of 186 pg/mL and 336 pg/ml respectively. Due to her symptoms, patient underwent a coronary angiogram that showed normal coronaries and right-sided heart catheterization that showed elevated biventricular filling pressures (right atrium pressure of 10 mmHg, RV systolic pressure of 28 mmHg and diastolic pressure of 8 mmHg, mean pulmonary artery pressure of 21 mmHg, and wedge pressure of 15 mmHg) with severely reduced cardiac index of 2.0 L/min/m2. Repeated echocardiogram showed LVEF of 30% with global hypokinesis. Patient was started on dobutamine with significant improvement in her symptoms. HCQ-induced cardiomyopathy was suspected. Patient underwent an endomyocardial biopsy that revealed a pathognomonic finding of myocyte vacuolization, consistent with HCQ-induced cardiomyopathy (Figure A). HCQ was discontinued immediately. However, patient was severely deconditioned and malnourished secondary to the end-stage heart failure and was a poor candidate for direct orthoptic heart transplantation or durable mechanical circulatory support. Patient accepted hospice care and passed away peacefully. HCQ can rarely cause non-ischemic cardiomyopathy. Main risk factors include longer duration of therapy and higher cumulative dose of HCQ. Patients remain asymptomatic for a long time and then present with symptoms of acute heart failure. The main diagnostic test is endomyocardial biopsy and histopathological examination showing myocyte vacuolization. Medication reconciliation is crucial in patients with non-ischemic cardiomyopathy as some commonly used medications can be the cause of the cardiomyopathy. Management includes immediate discontinuation of HCQ and guideline-directed medical therapy for heart failure. The prognosis of HCQ-induced cardiomyopathy varies from complete recovery if HCQ is stopped early enough, to partial or no recovery, if irreversible cell injury happens.