INTRODUCTION: Hydroxychloroquine-induced cardiomyopathy is a rare and life-threatening condition characterized by restrictive hemodynamics. Drug cessation and aggressive afterload reduction halts disease allowing for clinical recovery. We present a 50-year-old woman with hydroxychloroquine-induced restrictive cardiomyopathy and correlate clinical, echocardiographic, and right heart catheterization (RHC) findings at initial presentation and following treatment.
CASE PRESENTATION: A 50 year-old female with systemic lupus erythematosus (SLE) was referred to the pulmonary hypertension (PH) clinic for evaluation of worsening shortness of breath. She described progressive exertional dyspnea, lower extremity swelling, and increasing abdominal girth for 6 months. The patient’s medical history was remarkable for complications of SLE including serositis with pericardial and pleural effusions; ascites; mesangiopathic nephritis; Raynaud's phenomenon; leucopenia; and anemia. Medications consisted of hydroxychloroquine (200 mg twice daily for over 10 years) and corticosteroids. Physical examination revealed resting tachycardia at 100 bpm, blood pressure 123/58 mmHg, respiratory rate 12, and oxygen saturation of 96% on room air. Pertinent findings included increased jugular venous pressure, diminished breath sounds at the base of the right lung with dullness to percussion, an accentuated P2 component of the second heart sound despite muffled heart sounds, a protuberant abdomen, and 2+ pitting edema to the knees. Chest X-ray demonstrated a right basilar pleural effusion. EKG was normal voltage, sinus rhythm with normal intervals and no evidence of ischemia or infarction. Transthoracic echocardiography (2D echo) revealed mild left ventricular dilatation, mild pericardial effusion, moderate mitral valve regurgitation, moderate tricuspid valve regurgitation and an estimated pulmonary artery systolic pressure (sPAP) of 52 mmHg. Pulmonary function tests demonstrated combined obstruction and restriction with normal diffusing capacity. A ventilation/perfusion scan demonstrated no evidence of chronic thromboembolic disease. Given the longstanding use of hydroxychloroquine as well as pericardial involvement of SLE, and after evaluation for pulmonary etiology of symptoms, our differential included both restrictive cardiomyopathy and constrictive pericarditis. A cardiac MRI was pursued and was non-revealing. A left/right heart cardiac catheterization (L/RHC) was performed demonstrating mean right atrial pressure (mRAP) of 16 mmHg with pronounced “x” and “y” descents; right ventricular end diastolic pressure (RVEDP) was 23 mmHg with square root sign; mean pulmonary artery pressure (MPAP) was 29 mmHg; pulmonary capillary wedge pressure (PCWP) was 22 mmHg; left ventricular end diastolic pressure (LVEDP) was 24 mmHg. Cardiac output (CO) was 5.61 L/min by Fick. Cardiac index (CI) was 3.13 L/min/m2 by Fick. Pulmonary vascular resistance (PVR) was 1.25 Woods units. The equalization of pressures of the right ventricle, pulmonary artery, PCWP and left ventricle during diastole strongly suggested restrictive physiology. Hydroxychloroquine therapy was discontinued. Aggressive afterload reduction was instituted. The patient’s symptoms resolved. Repeat 2D echo three months following discontinuation of hydroxychloroquine also suggested resolution, demonstrating sPAP of 36 mmHg. A repeat L/RHC one year after discontinuation of hyroxychloroquine demonstrated: mRAP 6 mmHg; RVEDP 13 mmHg; mPAP 23 mmHg; PCWP 11 mmHg. CO/CI were 4.07 L/min and 2.35 L/min/m2 by Fick respectively.
DISCUSSION: Hydroxycholoquine therapy is indicated to treat connective tissue disorders including SLE. Cardiovascular complications include myocardial thickening, restrictive cardiomyopathy, and conduction disorders. The time interval between initiation of therapy and development of complications has been documented to vary from 7 months to 25 years . We present a case of prolonged hydroxychloroquine use resulting in clinical heart failure and restrictive cardiomyopathy as demonstrated by RHC. Discontinuation of hydroxychloroquine therapy and aggressive afterload reduction resulted in dramatic improvement in clinical symptoms and hemodynamics.
CONCLUSIONS: It is important to consider restrictive cardiomyopathy as an etiology of heart failure in patients treated with hydroxychloroquine.
Reference #1 Cotroneo J, Sleik K, Rodriguez E, Klein A. Hydroxychloroquine-induced restrictive cardiomyopathy. Eur J Electrocardiology 2007;8,247-251
DISCLOSURE: The following authors have nothing to disclose: Daniel Urbine, Rana Awdish
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