Atrial fibrillation in β-thalassemia Major Patients: Diagnosis, Management and Therapeutic Options

Abstract

The prevalence of atrial fibrillation (AF) in β-thalassemia major (β-TM) patients has increased in the last few years, reaching up to 33.0%. Several factors may drive this value to even more in the next years. We summarized the main challenges in the management and therapy of AF in this very specific group of patients. KEYWORD Arrhythmia; atrial fibrillation (AF); β-thalassemia (β-thal); iron overload β-Thalassemia major (β-TM) is a hereditary hemoglobin (Hb) disorder caused by reduced synthesis of β-globin chain and resulting in a chronic hemolytic anemia that typically requires lifelong transfusion therapy. If left untreated, this could result in growth retardation, bone marrow expansion, extramedullary hematopoiesis, splenomegaly, greater intestinal iron absorption, hypercoagulability and higher susceptibility to infections till death occurs [1-3]. Although traditionally prevalent in the Mediterranean Basin, Middle East, North India, and Southeast Asia, migration of those populations to North America and Western Europe has rendered β-thalassemia (β-thal) a global health problem. According to the Thalassemia International Federation, (Nicosia, Cyprus), about 200,000 people are affected by β-TM and registered as receiving regular treatment around the world [4]. The highest carrier frequency is reported in Cyprus (14.0%), Sardinia (10.3%) and Southeast Asia [5].

Twelve‐lead and signal‐averaged electrocardiographic parameters among beta‐thalassemia major patients

“…The main finding in our study is that β‐TM patients have a higher prevalence of a prolonged PR interval (17.02%), atrial fibrillation (10.64%), and LPs (38.3%). These findings are in agreement with previous reports 9‐13,18 . However, it is the first study that quantifies the prevalence of PR interval prolongation and LPs in a β‐TM population of advanced age (mean value 40.91 years) compared with previous studies 11‐13 .…”

“…These findings are in agreement with previous reports. [9][10][11][12][13]18 However, it is the first study that quantifies the prevalence of PR interval prolongation and LPs in a β-TM population of advanced age (mean value 40.91 years) compared with previous studies. [11][12][13] Additionally, our study is unique in terms of combining data from patient comorbidities, electrocardiography, echocardiography, and CMR.…”

“…8 Apart from end-stage heart failure, a significant proportion of β-TM patients die suddenly. 5,9 Indeed, according to a recent National Registry in Greece, 10.18% of the β-TM patients suffered sudden cardiac death. 5 Atrial fibrillation prevalence among β-TM patients varies in literature, ranging from 10% to 33.8%.…”

“…5 Atrial fibrillation prevalence among β-TM patients varies in literature, ranging from 10% to 33.8%. 10 Signal-averaged electrocardiography (SAECG) is an attractive method for the arrhythmiological assessment of β-TM patients, by detecting slow conducting areas in the ventricular myocardium, the so-called late potentials (LPs). Such abnormal signals have been reported among young β-TM patients from Lebanon and Italy, ranging from 3 to 31.5%.…”

Thalassemia in the emergency department: special considerations for a rare disease

“…Young, anemic patients with atrial fibrillation are more likely to be symptomatic given their relative state of hyperdynamic circulation. Atrial fibrillation needs to be managed by rate controlling agents, iron chelation in the case of iron overload, anticoagulation (after patient is screened for bleeding risk), and rhythm control agents [43]. In the case of unstable patients with supraventricular arrhythmias (patients with hypotension, chest pain, altered mental status, signs of acute heart failure), immediate cardioversion should be performed.…”