Cardiotoxicity is a rare but serious complication of hydroxychloroquine, a 4-aminoquinoline increasingly used in the treatment of rheumatological disorders. We describe typical clinical, echocardiographic, and histological features of this rare condition according to the currently available literature, illustrated with a recent new biopsy-proven case of hydroxychloroquine cardiotoxicity in a 52-year-old female with rheumatoid arthritis. Presentation in this case was of a rapidly progressive decompensated biventricular cardiomyopathy associated with recurrent biomarker elevations, conduction system disease, and possibly neuromyotoxicity. Death occurred suddenly 2 months after diagnosis despite drug discontinuation and clinical improvement. The potential role of cardiac magnetic resonance delayed gadolinium enhancement imaging in the prognosis of this toxic cardiomyopathy is also introduced. This case-based literature review highlights that, although rare, hydroxychloroquine cardiotoxicity can be fatal, particularly if irreversible histopathological changes have occurred prior to drug discontinuation. Given this, regular screening with 12-lead electrocardiography and transthoracic echocardiography to detect conduction system disease and/or biventricular morphological or functional changes should be considered in hydroxychloroquine-treated patients in addition to recommended ophthalmological screening.

Background— The value of left atrial (LA) diameter, volume, and strain to risk stratify hypertrophic cardiomyopathy patients for new-onset atrial fibrillation (AF) was explored. Methods and Results— A total of 242 hypertrophic cardiomyopathy patients without AF history were evaluated by (speckle-tracking) echocardiography. During mean follow-up of 4.8±3.7 years, 41 patients (17%) developed new-onset AF. Multivariable analysis showed LA volume (≥37 mL/m 2 ; hazard ratio, 2.68; 95% confidence interval, 1.30–5.54; P =0.008) and LA strain (≤23.4%; hazard ratio, 3.22; 95% confidence interval, 1.50–6.88; P =0.003), but not LA diameter (≥45 mm; hazard ratio, 1.67; 95% confidence interval, 0.84–3.32; P =0.145), as independent AF correlates. Importantly, 59% (n=24) of AF events occurred despite a baseline LA diameter <45 mm, observed in 185 patients. In this patient subset, LA strain (area under the curve 0.73) and LA volume (area under the curve 0.83) showed good predictive value for new-onset AF. Furthermore, patients with LA volume <37 versus ≥37 mL/m 2 and LA strain >23.4% versus ≤23.4% had superior 5-year AF-free survival of 93% versus 80% ( P =0.003) and 98% versus 74% ( P =0.002), respectively. Importantly, LA volume <37 mL/m 2 and strain >23.4% yielded high negative predictive value (93% and 98%, respectively) for new-onset AF. Likelihood ratio test indicated incremental value of LA volume assessment ( P =0.011) on top of LA diameter to predict new-onset AF in hypertrophic cardiomyopathy patients with LA diameter <45 mm, which tended to increase further by addition of LA strain ( P =0.126). Conclusions— LA diameter, volume, and strain all relate to new-onset AF in hypertrophic cardiomyopathy patients. In patients with normal LA size, however, both LA volume and strain further refine risk stratification for new-onset AF.

Aims Limited data exist on the risk of developing cardiac sarcoidosis (CS) and/or adverse events in sarcoidosis patients. Using LV global longitudinal strain (GLS), an emerging sensitive parameter of LV function, we evaluated the prevalence of subclinical cardiac dysfunction in sarcoidosis and investigated whether LVGLS predicts adverse outcomes in this population. Methods and results A total of 130 patients with proven sarcoidosis undergoing echocardiography at our referral centre were identified. Following exclusion of those with evidence of CS (n = 14) or other pre‐existing structural heart disease (n = 16), 100 patients (55 ± 13 years, 48% male, 90% pulmonary involvement) and 100 age‐ and gender‐matched controls were included. LVGLS was measured by speckle‐tracking analysis. The primary endpoint was a composite of all‐cause mortality, heart failure hospitalization, device implantation, new arrhythmias, or future development of CS on advanced cardiac imaging modalities. LVGLS was significantly impaired in sarcoidosis patients compared with controls (–17.3 ± 2.5 vs. –20.0 ± 1.6%, P < 0.001). Overall, 27 patients (27%) reached the endpoint during a median follow‐up of 35 months. On Cox proportional hazards model analysis, abnormal 24‐h Holter, larger LV end‐diastolic diameters, and more impaired LVGLS were significantly associated with the endpoint; however, only LVGLS remained independently associated on multivariate analysis [hazard ratio (HR) 1.4, 95% confidence interval (CI) 1.1–1.7, P = 0.006]. Patients with LVGLS less than –17.3% were significantly more likely to be free of the primary endpoint (log‐rank P = 0.01). Conclusion LVGLS is impaired in sarcoidosis patients, suggesting subclinical cardiac dysfunction despite the absence of conventional evidence of cardiac disease, and is independently associated with occurrence of cardiac events and/or development of CS.