Cardiac T2* MR in patients with thalassemia major: a 10-year long-term follow-up
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ORIGINAL ARTICLE
Cardiac T2* MR in patients with thalassemia major: a 10-year long-term follow-up Shahina Daar 1,2 & Murtadha Al Khabori 3
3
3
& Sarah Al Rahbi & Moez Hassan &
AbuBakr El Tigani 3 & Dudley J Pennell 4,5
Received: 11 January 2020 / Accepted: 1 June 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract The consequence of regular blood transfusion in patients with thalassemia major (TM) is iron overload. Herein, we report the long-term impact of chelation on liver iron concentration (LIC) and cardiac T2* MR in patients with TM. This is a retrospective cohort study over 10 years of adolescents and adults with TM aged at least 10 years who had their first cardiac T2* MR between September 2006 and February 2007. One-year chelation therapy was considered the unit of analysis. A total of 99 patients were included in this study with a median age of 18 years. The median cardiac T2* MR and LIC at baseline were 19 ms and 11.6 mg/g dw, respectively. During follow-up, 18 patients died and six underwent successful bone marrow transplantation. Factors associated with decreased survival were older age (HR 1.12, p = 0.014) and high risk cardiac T2* (HR 8.04, p = 0.004). The median cardiac T2* and LIC significantly improved over the 10-year follow-up period (p = 0.000011 and 0.00072, respectively). In conclusion, this long-term “real-life” study confirms that low cardiac T2* adversely impacts the overall survival in patients with TM. Higher baseline LIC predicts a larger reduction in LIC, and lower baseline cardiac T2* predicts a larger improvement in T2*. Keywords Thalassemia . Oman . Iron overload . MRI T2* . Heart
Introduction Thalassemia major (TM) is an inherited blood disorder resulting in severe anemia requiring blood transfusions to sustain life. As humans have a limited capacity for getting rid of excess iron, the consequence of this treatment is iron overload of the tissues, including the heart, liver, and endocrine organs. Before the advent of iron chelators, the majority of patients with TM died in their second decade from cardiac siderosis [1]. Although iron chelation therapy has increased life expectancy in TM, cardiac disease remains the commonest cause of death in most populations [2–4]. Mortality was due to both
* Murtadha Al Khabori [email protected]; [email protected] 1
Sultan Qaboos University, Muscat, Sultanate of Oman
2
Stellenbosch Institute for Advanced Study (STIAS), Wallenberg Research Centre at Stellenbosch University, Stellenbosch 7600, South Africa
3
Sultan Qaboos University Hospital, Muscat, Sultanate of Oman
4
Royal Brompton Hospital, London, UK
5
Imperial College, London, UK
non-compliance in some patients and also the difficulty in early identification of patients at risk to make medical intervention effective. This latter problem was addressed by the development of imaging studies that allowed for accurate evaluation of cardiac iron load by magnetic resonance imaging (MRI) using the highly sensitive relaxation parameter T2* [5]. Further studi
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