Severity of heart failure and health-related quality of life in beta-thalassemia patients: a cross-sectional study

Cardiovascular complications account for a substantial increase in morbidity and mortality in beta-thalassemia patients. Many patients have structural heart disease, and some of them present with symptomatic heart failure (HF). Quality of life (QOL) of beta-thalassemia patients is lower than that of the general population. The aim of our study was to explore the relationship between HF stages and QOL in beta-thalassemia patients. Seventy-three consecutive adult beta-thalassemia patients took part in this cross-sectional study. Stages of HF, classified with increasing severity as A, B, and C, were determined based on ACC/AHA guidelines. QOL was assessed using the SF-36 questionnaire. Fifteen patients had stage C HF, twenty-eight had stage B HF, and the remaining were considered stage A patients, as beta thalassemia is a predisposing factor for HF. All QOL domains except for bodily pain were significantly lower in stage C patients than in stage A patients. Stage C patients had significantly lower QOL scores for physical functioning, role physical, and social functioning domains than stage B patients. Stage B patients’ QOL differed from stage A patients only in the vitality domain. In the multiple regression analysis which took several demographic and clinical factors into account, stage of HF was the most important factor associated with QOL, and negatively and significantly related to five QOL domains, namely physical functioning, role physical, general health, social functioning, and vitality. In conclusion, QOL is negatively affected by the severity of heart failure in beta-thalassemia patients. © 2020, Springer-Verlag GmbH Germany, part of Springer Nature

Pregnancy in patients with hemoglobinopathies: 30-year results of a Greek Thalassemia and Sickle Cell Department

The aim of the current study is the longitudinal epidemiological study of pregnancies, their outcome and the changes in their treatment, in patients with hemoglobinopathies, during 30 years at a Thalassemia and Sickle Cell Department. The data of a total of 47 pregnancies of 40 women with hemoglobinopathies monitored in the Thalassemia and Sickle Cell Department of Hippokrateio General Hospital of Athens were retrospectively collected. The data were divided and evaluated in two time periods, the first before 2000 and the second between 2000 and 2017. There were four miscarriages and 43 completed pregnancies. The mean pregnancy duration was 34.92 weeks. Thalassemia major and thalassemia intermedia patients had higher percentages of in vitro fertilization (IVF) pregnancies and IVF attempts, with the majority of IVF attempts and pregnancies in the time period after 2000. During the period 2000-2017, more women received transfusions and iron chelation therapy both before and during pregnancy compared to the period before 2000. During the period 2000-2017, three women presented hemorrhagic complications. Placental abruption occurred in two cases, while one woman suffered a stroke. Six women had liver disease and two cardiac problems. The rate of pregnancies in women with hemoglobinopathies has increased after the year 2000 due to the increased use of IVF technique. Pregnancy planning, close collaboration between gynecologists and hematologists and appropriate pregnancy monitoring are essential for an optimal pregnancy outcome. © 2020 2020 Walter de Gruyter GmbH, Berlin/Boston