Hypereosinophilic syndromes (HES) have heterogenous clinical presentations and can cause significant morbidity, including cardiac complications and thromboembolic events. Characterizing cardiac manifestations in HES will aid the evaluation and management of these patients.
A retrospective review of electronic medical records (January 2002 to June 2017) was performed for adult patients with a diagnosis of idiopathic HES. Each chart review detailed symptoms at clinical presentation, eosinophil counts, troponin levels, and echocardiographic findings. The study was approved by the institutional review board.
Twenty-one patients were identified for review, consisting of 10 males and 11 females. Symptom duration at time of initial presentation ranged from 1 to 156 months (average 29.3 months). Eosinophil counts ranged from 1090 to 62,160 eosinophils per microliter at the time of initial presentation. Thirteen (62%) patients presented with cardiovascular symptoms, including chest pain, dyspnea on exertion, or lower extremity edema. Eight (38%) patients had elevated troponin levels and two additional patients had elevated LDH with no troponin levels drawn. Eleven (52%) patients demonstrated valve abnormalities on echocardiogram including one patient with thrombus formation in the left ventricular endocardium. Four (19%) patients had ejection fractions less than 45%. Maintenance therapies included the following: 7 patients (33%) prednisone and hydroxyurea, 5 patients (24%) prednisone and interferon, 5 patients (24%) prednisone only, and 4 patients (19%) mepolizumab.
Cardiac symptoms and abnormalities are frequently seen with idiopathic HES. Prompt, comprehensive cardiovascular evaluation is essential to reduce HES-associated cardiac morbidities.