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New-Onset Heart Failure in a Middle-aged Man

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his mid-60s with a history significant for hereditary spherocytosis, polycythemia vera, hemochromatosis, hypereosinophilia, and asymptomatic paroxysmal supraventricular tachycardia presented to the emergency department because of dyspnea. Over a 4-week period, he developed dyspnea with moderate levels of physical activity that were occasionally associated with brief episodes of lightheadedness. He also reported orthopnea but no paroxysmal nocturnal dyspnea or leg edema. There was no chest pain, palpitations, cough, fever, or hemoptysis. He had tachypnea with a respiratory rate of 24 breaths per minute with a normal oxygen saturation, temperature, heart rate, and blood pressure. A soft (I/VI) systolic murmur with a brief diastolic component was appreciated over the apex. Jugular venous distension was present; there were no pulmonary rales, abdominal distention, or leg edema. Workup results demonstrated leukocytosis (white blood cell count, 39 800/μL [to convert to ×109 L, multiply by 0.001]; 15920/μL eosinophils [44%]), with a hemoglobin level of 12.4 g/dL (to convert to grams per liter, multiply by 10) and a platelet count of 153 ×103/μL (to convert to ×109 L, multiply by 1). A basic metabolic panel yielded normal results and troponin was undetectable. His chest radiography results were suggestive of pulmonary vascular congestion. The echocardiogram results demonstrated a normal left ventricular ejection fraction with no substantial wall motion abnormalities. There was an echogenic band traversing the apical portion of the left ventricle with increased echogenicity compared with the adjacent myocardium (Video). Representative frames of the echocardiogram are shown in Figure 1.

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Loeffler syndrome with a left ventricular thrombus

B. Cardiac magnetic resonance imaging (MRI)

Loeffler syndrome is a progressive, restrictive heart failure syndrome with inflammatory endocardial fibrosis in the setting of hypereosinophilia. In hypereosinophilic syndromes (HES), eosinophils infiltrate, disrupt, and progressively destroy tissues through the deposition of enzymatic, granular, cationic, and proinflammatory proteins.1,2 Hypereosinophilic syndromes are rare with an incidence of approximately 0.036 per 100 000.1 Cardiac involvement is present in approximately 50% of patients with HES; the resultant fibroplastic endocarditis and eventual endomyocardial fibrosis may cause a restrictive cardiomyopathy. The damaged endocardium and prothrombotic milieu provide a nidus for mural thrombus formation.13 A new cardiac mass in the setting of hypereosinophilia should immediately cause concern for a thrombus.

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Article Information

Corresponding Author: Stephen E. Wilkinson, MD, Spectrum Health, c/o GME, 100 Michigan St NE, Grand Rapids, MI 49503 (stephen.wilkinson@spectrumhealth.org).

Published Online: December 4, 2019. doi:10.1001/jamacardio.2019.4520

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Gotlib  J.  World Health Organization-defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management.  Am J Hematol. 2017;92(11):1243-1259. doi:10.1002/ajh.24880PubMedGoogle ScholarCrossref
2.
Benezet-Mazuecos  J, de la Fuente  A, Marcos-Alberca  P, Farre  J.  Loeffler endocarditis: what have we learned?  Am J Hematol. 2007;82(10):861-862, 3. doi:10.1002/ajh.20957PubMedGoogle ScholarCrossref
3.
Séguéla  PE, Iriart  X, Acar  P, Montaudon  M, Roudaut  R, Thambo  JB.  Eosinophilic cardiac disease: molecular, clinical and imaging aspects.  Arch Cardiovasc Dis. 2015;108(4):258-268. doi:10.1016/j.acvd.2015.01.006PubMedGoogle ScholarCrossref
4.
Habash  F, Vallurupalli  S.  Challenges in management of left ventricular thrombus.  Ther Adv Cardiovasc Dis. 2017;11(8):203-213. doi:10.1177/1753944717711139PubMedGoogle ScholarCrossref
5.
Kajy  M, Shokr  M, Ramappa  P.  Use of direct oral anticoagulants in the treatment of left ventricular thrombus: systemic review of the current literature [published online January 29, 2019].  Am J Ther. PubMedGoogle Scholar
6.
Ogbogu  PU, Bochner  BS, Butterfield  JH,  et al.  Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy.  J Allergy Clin Immunol. 2009;124(6):1319-25.e3. doi:10.1016/j.jaci.2009.09.022PubMedGoogle ScholarCrossref
7.
Chao  BH, Cline-Parhamovich  K, Grizzard  JD, Smith  TJ.  Fatal Loeffler’s endocarditis due to hypereosinophilic syndrome.  Am J Hematol. 2007;82(10):920-923. doi:10.1002/ajh.20933PubMedGoogle ScholarCrossref
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