DOI: https://dx.doi.org/10.18203/2349-3291.ijcp20222114
Published: 2022-08-24

Prevalence of pulmonary hypertension in pediatric patients with beta-thalassemia major in Central India: a cross sectional study

Ruchi A. Gedam, Saira Merchant

Abstract


Background: The objective of the study was to determine the prevalence of pulmonary hypertension in pediatric patients with beta thalassemia major in a tertiary care centre in Central India.

Methods: A cross sectional study was conducted in department of pediatrics, Government Medical College and Hospital, Nagpur, India over a period of 2 years. Total 100 subjects of thalassemia major diagnosed by high performance liquid chromatography (HPLC) attending day care in this department were enrolled for study. Measurement of pulmonary pressure was done by using 2D echocardiography with Doppler studies and tricuspid regurgitant jet velocity was measured. This was done by experienced cardiologist. Variables predictive of pulmonary hypertension studied were age, sex, serum ferritin levels, age of onset of chelation therapy, compliance to chelation therapy and number of blood transfusion per year.

Results: Out of 100 subjects, 18 subjects had pulmonary hypertension. Univariate analysis proved that age, serum ferritin levels, age of onset of chelation therapy, compliance to chelation therapy and number of blood transfusion per year were significant predictors of pulmonary hypertension. While, multiple logistic regression analysis revealed that only 3 variables that is age of onset of chelation therapy, number of blood transfusion per year and compliance to chelation therapy retained their significance in this patient population.

Conclusions: Prevalence of pulmonary hypertension was found out to be 18%. 2 subjects in the pulmonary hypertension group died. High morbidity and mortality of pulmonary hypertension clearly indicates that much work is needed in disease detection and development of more effective therapies. For thalassemic children, early detection and prevention of pulmonary hypertension in childhood would improve survival, reduce morbidity and mortality.


Keywords


Pulmonary hypertension, Beta thalassemia, TR jet velocity

Full Text:

PDF

References


Grow K, Vashist M, Abrol P, Sharma S, Yadav R. Innovare Academic Sciences beta thalassemia in India. Current Status Challenges Ahead. 2014;6(4):2-7.

Vlahos AP, Koutsouka FP, Papamichael ND, Makis A, Baltogiannis GG, et al. Determinants of pulmonary hypertension in patients with Beta-thalassemia major and normal ventricular function. Acta Haematol. 2012;128(2):124-9.

McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006;114(13):1417-31.

Aessopos A, Farmakis D, Deftereos S, Tsironi M, Tassiopoulos S, Moyssakis I, et al. Thalassemia heart disease: A comparative evaluation of thalassemia major and thalassemia intermedia. Chest. 2005;127(5):1523-30.

Meloni A, Detterich J, Pepe A, Harmatz P, Coates TD, Wood JC. Pulmonary hypertension in well-transfused thalassemia major patients. Blood Cells Mol Dis. 2015;54(2):189-94.

Walker M, Wood J. Cardiac complications in thalassaemia major. In: Cappellini MD, Cohen A, Porter J, eds. Guidelines for the Management of Transfusion Dependent Thalassaemia (TDT). 3rd ed. Nicosia (CY): Thalassaemia International Federation; 2014.

Aessopos A, Farmakis D. Pulmonary hypertension in beta-thalassemia. Ann N Y Acad Sci. 2005;1054:342-9.

Beshlawy A, Youssry I, El-Saidi S, El Accaoui R, Mansi Y, Makhlouf A, et al. Pulmonary hypertension in beta-thalassemia major and the role of L-carnitine therapy. Pediatr Hematol Oncol. 2008;25(8):734-43.

Grisaru D, Rachmilewitz EA, Mosseri M, Gotsman M, Lafair JS, Okon E, Goldfarb A, Hasin Y. Cardiopulmonary assessment in beta-thalassemia major. Chest. 1990;98(5):1138-42.

Du ZD, Roguin N, Milgram E, Saab K, Koren A. Pulmonary hypertension in patients with thalassemia major. Am Heart J. 1997;134(3):532-7.

Kiter G, Balci YI, Ates A, Hacioglu S, Sari I. Frequency of pulmonary hypertension in asymptomatic beta-thalassemia major patients and the role of physiological parameters in evaluation. Pediatr Hematol Oncol. 2010;27(8):597-607.

Alkholy UM, Mohamed SA, Elhady M, Attar SE, Abdalmonem N, Zaki A. Vascular endothelial growth factor and pulmonary hypertension in children with beta thalassemia major. J Pediatr (Rio J). 2019;95(5):593-9.

Elalfy MS, Youssef OI, Deghedy MMR, Abdel Naby MM. Left Ventricular Structural and Functional Changes in Children With β-Thalassemia and Sickle Cell Disease: Relationship to Sleep-disordered Breathing. J Pediatr Hematol Oncol. 2018;40(3):171-7.

Aessopos A, Farmakis D, Hatziliami A, Fragodimitri C, Karabatsos F, Joussef J, et al. Cardiac status in well-treated patients with thalassemia major. Eur J Haematol. 2004;73(5):359-66.