Assessment of the Cardiac Abnormalities in Patients with Beta Thalassemia: Prospective Observational Study

Abstract

Aim: To evaluate the cardiac abnormalities in patients with beta thalassemia.
Methods: The prospective observational study which was carried in the Department of
Paediatric, Darbhanga medical college and Hospital, Laheriasarai, Darbhanga, Bihar, India. A
total of 50 transfusion dependent thalassemic patients who were registered at the outdoor
thalassemia transfusion center at the same institute were enrolled in the present study. Informed
consent was taken from patients or their parents and Institutional Ethical Committee approval
was obtained. Thalassemic children >7 years of age who were on regular blood transfusions,
irrespective of chelation were included in the study. A detailed physical examination was done
in each case and the vital parameters, signs of congestive cardiac failure and size of liver and
spleen were noted. Chest X- ray, electrocardiogram (ECG) and 2-dimensional and Doppler
echocardiography were done.
Results: A total of 50 multiply transfused thalassemic patients were enrolled for the study. The
youngest patient was 9 years old and the oldest was 24 years old. A total of 33 (66%) males
and 17 (34%) females. Mean pretransfusion hemoglobin of the enrolled patients was 8.5gm%.
Out of 50 patients, 42 were taking deferiprone, 6 were on desferrioxamine and 2 patient was
taking combination chelation therapy. 8 (16%) patients had symptoms related to cardiac disease
and all of them were more than 13 years of age. Cardiomegaly on chest radiograph was present
in 36 (72%) patients. Only 4 (8%) patients had electrocardiographic (ECG) abnormalities. All
4 patients having ECG abnormalities had signs of congestive cardiac failure on clinical
examination and cardiomegaly on chest radiograph. All the 50 patients had normal left
ventricular ejection fraction i.e. LVEF (LVEF ≥60%). diastolic dysfunction/ restrictive
physiology on Doppler echocardiography was present in 30 (60%) and absent in 20 (40%)
patients. Diastolic dysfunction was present in all 8 patients having signs of congestive cardiac failure as well as all 3 patients with ECG abnormalities. Out of 30 patients having diastolic
dysfunction, cardiomegaly on chest radiograph was present in 27 patients. (p value 0.006).
Conclusion: Diastolic dysfunction on Doppler echocardiography is an early sign of myocardial
dysfunction in patients with beta thalassemia. ECG abnormalities detect heart failure due to
iron overload late in the course of the disease, and therefore ECG is not very sensitive for early
detection of cardiac dysfunction.