PrintThis PatientPlus article is written for healthcare professionals so the language may be more technical than the condition leaflets. You may find the abbreviations list helpful.
The heart failure is due to restrictive cardiomyopathy. It is associated with various eosinophilic states. Eosinophilic arteritis and myocarditis is rapidly progressive so that the diagnosis is usually made post mortem. It was first described in 1936 when Löffler reported a very rare syndrome of eosinophilia, active carditis and multi-organ involvement.[1]
NB: Löffler's syndrome is different. It is a transient respiratory illness with eosinophilia and shadowing on chest X-ray, usually due to parasitic infestation.
Aetiology
Most cases are of idiopathic origin.
However other causes include:
- Hypereosinophilic syndrome
- Eosinophilic leukaemia
- Carcinoma
- Lymphoma
- Drug reactions
- Parasites
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Presentation
- The usual presentation is with weight loss, fever, cough, a rash (possibly pruritic) and symptoms of congestive heart failure.
- Early cardiac involvement occurs in 20 to 50% of cases.
- The physical findings are those of heart failure:
- Peripheral oedema and elevated jugular venous pressure
- Tachycardia
- Cardiomegaly may precede signs of heart failure
- There may be a murmur of mitral regurgitation as described in mitral valve disease
- The gallop of a 3rd heart sound may be heard and possibly even a 4th heart sound
- Systemic emboli may cause renal or neurological problems.
- Signs of a restrictive cardiomyopathy may be very similar to those of constrictive pericarditis but the presence of a palpable apex beat and mitral regurgitation suggest cardiomyopathy.
Differential diagnosis
- Other causes of restrictive cardiomyopathy include amyloidosis, sarcoidosis and myeloma.
- In children rhabdomyosarcoma may be found. It is commoner with tuberose sclerosis.
Investigations
- Full blood count will show marked eosinophilia - at least 0.44 x 109/l
- Electrocardiogram, echocardiogram and possibly CT scan and biopsy - useful to diagnose cardiomyopathy (Löffler's cardiomyopathy is characterised by restrictive filling but quite good left ventricular systolic function)
- Cardiac catheterisation
Management
- Congestive failure is treated in the usual way with diuretics, digoxin, ACE inhibitors and other drugs to reduce afterload.
- In early disease, immune suppression and cytotoxic drugs have had variable success.[2]
- Steroids appear of benefit in acute myocarditis.
- Prednisolone and hydroxycarbamide have been used to suppress eosinophilia.
- Once fibrosis has occurred, surgery may be of benefit:
- Removing fibrosed endocardium may improve elasticity.
- Mitral and even tricuspid valves may need replacement.
- Operative mortality is 15-30% and complete atrioventricular (AV) block is a common complication.
Prognosis
- Prognosis is poor and depends upon the degree of involvement of the heart. Onset is usually slow but with accelerating right and left heart failure.
- Syncope and sudden death are less common than with other constrictive cardiomyopathies.
- The mean survival time is 18 months.[3]
Further reading & references
- Hasan SA; Loeffler eosinophilic endocarditis. eMedicine. October 2008.
- Loffler W: Endocarditis parietalis fibroplastica mit Blut-eosinophilie, ein eigenartiges Krankheitsbild. Schweiz Med Wochenschr; 1936; 66: 817-820.
- Solley GO, Maldonado JE, Gleich GJ, et al; Endomyocardiopathy with eosinophilia. Mayo Clin Proc. 1976 Nov;51(11):697-708.
- Adler CP; Obstructive-restrictive cardiomyopathy. On the clinical aspects and pathology of Loffler endocarditis. Versicherungsmedizin. 1989 Sep 1;41(5):151-4.
| Original Author: Dr Hayley Willacy | Current Version: Dr Hayley Willacy | |
| Last Checked: 15/10/2009 | Document ID: 2395 Version: 22 | © EMIS |
Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.