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Unexpected myocarditis in thalassaemia major patient screened for iron load cardiomyopathy

Alberto Roghi¹, Santo Dellegrottaglie², Patrizia Pedrotti², Stefano Pedretti², Elena Cassinerio³, Maria Domenica Cappellini³

¹ Niguarda Ca’Granda Hospital, Cardiology, Milan, 20100, Italy
² Cardiac MR Unit, A.De Gasperis Department of Cardiology, Milan, 20100, Italy
³ University of Milan, Centro Anemie Rare, Department of Medicine, Milan, 20100, Italy

Correspondence to:
alberto_roghi{at}fastwebnet.it

A 45-year-old white female with thalassaemia major, diabetes mellitus and hypogonadism underwent routine cardiac magnetic resonance (CMR) imaging to evaluate T2*, a myocardial and hepatic iron load indicator useful in the management of iron chelating therapy. At cardiac cine imaging, left ventricular antero-apical mild hypokinesia and pericardial effusion were evident. T2 weighted STIR image (fig 1) showed high signal intensity of the left ventricular lateral wall, a typical sign of myocardial oedema. Delayed enhancement imaging after paramagnetic contrast (fig 2) showed intramyocardial enhancement matching with the T2 weighted image. Myocardial T2* was within normal values, suggesting no evidence of myocardial iron overload.

View this figure (58K): in this window | in a new window | PowerPoint for Teaching   Figure 1 T2 weighted four chamber view showing high signal intensity of the lateral wall (white arrow).

 

View this figure (142K): in this window | in a new window | PowerPoint for Teaching   Figure 2 Delay enhancement short axis view showing lateral wall hyperenhancement (white arrow).

 

A flue-like syndrome a few days before the MR examination was the only significant symptom. The subsequent follow-up was negative for major events.

In thalassaemia major, transfusions and iron chelation therapy have significantly improved patient survival. However, congestive heart failure is still the leading cause of mortality. A direct iron toxic effect on myocardial cells is considered to be the main aetiologic factor of hypokinetic cardiomyopathy, but many other co-morbidities may precipitate cardiac failure. The incidence of infections among thalassaemic patients is higher than in normal controls and is related to the impairment of immune competence and to endocrinopathies such as hypothyroidism and diabetes mellitus.¹

Myocarditis is a serious complication related to iron load induced viral infection susceptibility and myocardial vulnerability.²

CMR imaging offers the opportunity to evaluate non-invasively myocardial morphology, function and iron load³ and to detect, as in the present case, an insidious presentation of acute myocarditis.

Competing interests: none.

Patient consent: Patient/guardian consent was obtained for publication.

1. Cunningham, MJ, Macklin, EA, Neufeld, EJ, et al. Complications of β-thalassemia major in North America. Blood 2004; 104: 34–9.[Abstract/Free Full Text]
2. Kremastinos, DT, Tiniakos, G, Theodorakis, GN, et al. Myocarditis in β-thalassemia major. Circulation 1995; 91: 66–71.[Abstract/Free Full Text]
3. Abdel-Aty, H, Boyè, P, Zagrosek, A, et al. Diagnostic performance of cardiovascular magnetic resonance in patients with suspected acute myocarditis. J Am Coll Cardiol 2005; 45: 1815–22.[Abstract/Free Full Text]

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