Abnormal diastolic function and Global longitudinal strain in patients with Thalassemia Major on long term chelation the
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ORIGINAL PAPER
Abnormal diastolic function and Global longitudinal strain in patients with Thalassemia Major on long term chelation therapy Sunil K. Nadar1 · Shahina Daar2,3 · Wael A. Abdelmottaleb1 · Muhammad M. Shaikh1 · Hafsa Al Mahrouqi4 · Majida Al‑Raiisi4 · Moez Hassan5 · Badar Al Rawahi5 · Sarah Al Rahbi5 Received: 19 June 2020 / Accepted: 18 September 2020 © Springer Nature B.V. 2020
Abstract Cardiac complications are the major cause of mortality in patients with Thalassemia major (TM). Cardiac T2* MRI is currently the gold standard for assessing myocardial iron concentration. The aim of our study was to assess whether any echocardiographic parameter would correlate with these findings in patients well established on chelation therapy. This was a prospective study on patients with TM who are regularly followed in our clinic. Patients had a cardiac MRI and echocardiogram within 2 months of each other. Echo parameters included global longitudinal strain and diastolic function. We also compared these findings with those from a cohort of thalassemia intermedia (TI) and normal controls. A total of 84 patients (mean age 26.3 ± 6.1 years, 42.8% male) with TM were enrolled. All had normal left ventricular ejection fraction and only 8 patients had MRI T2* 20 ms features of diastolic dysfunction persist even in the presence of normal LV function and normal GLS. This suggests that diastolic function remains abnormal even when myocardial iron concentrations are normal and follow up therefore is essential. Keywords Thalassemia major · Thalassemia intermedia · Echocardiogram · Diastolic function · Global longitudinal strain
Introduction Beta-thalassemia is an inherited hemoglobinopathy that is prevalent in the Middle-east, North Africa, South-East Asia and mediterranean countries with the carrier rate ranging * Sunil K. Nadar [email protected] 1
Department of Medicine, Sultan Qaboos University Hospital, POBox 38, Muscat 123, Oman
2
Department of Hematology, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman
3
Stellenbosch Institute for Advanced Study (STIAS), Wallenberg Research Centre At Stellenbosch University, Stellenbosch 7600, South Africa
4
Department of Clinical Physiology, Sultan Qaboos University Hospital, POBox 38, Muscat 123, Oman
5
Department of Hematology, Sultan Qaboos University Hospital, POBox 38, Muscat 123, Oman
from around 1–11% [1, 2]. The severity of the disease depends on the genetic make up. Thalassemia major (TM), usually due to homozygous inheritance of mutated beta globin genes, is the most clinically severe type and requires regular blood transfusions from early childhood [3]. Thalassemia minor, on the other hand, is the heterozygous form, where only one of the beta-globin alleles is affected. Here, the patients usually only suffer from mild microcytic anemia and do not require blood transfusions [3]. Thalassemia intermedia (TI) patients on the other hand, have mild to moderate symptoms and patients may require infrequent transfusions
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