FREQUENCY OF PULMONARY HYPERTENSION IN NON-TRANSFUSION DEPENDENT THALASSEMIAS
DOI:
https://doi.org/10.71000/07h3zf02Keywords:
Children, Non-transfusion dependent thalassemia, pulmonary hypertensionAbstract
Introduction: One of the main cardiac complications in non-transfusion-dependent thalassemia (NTDT) is pulmonary hypertension. It has been demonstrated that the occurrence of pulmonary hypertension positively correlates with a number of clinical and laboratory indicators, including iron overload.
Objective: The objective of this study was to determine the frequency of pulmonary hypertension in non-transfusion dependent thalassemia (NTDT).
Study Design: Cross-sectional study as carried out in Thalassemia centre of sir Ganga Ram Hospital Lahore.
Methodology: Following the ethical committee's acceptance letter, 170 patients who met the inclusion and exclusion criteria and visited the thalassemia center at Sir Ganga Ram Hospital Lahore (SGRH) were added to the study. Every enrolled patient had an echocardiogram performed in the hospital to check for TRV and ejection fraction. According to the operational definition, pulmonary hypertension was identified. In accordance with hospital practice, treatment was provided. Age is displayed as the mean standard deviation..
Results: Of the 170 cases in our study, 61.18% (n=104) were between the ages of 5 and 9 and 38.82% (n=66) were between the ages of 10 and 12. The mean age was 5.82+2.30 years, and 71.76% (n=122) of the cases were male and 28.24% (n=48) were female. Of the cases with non-transfusion dependent thalassemia (NTDT), 6.47% (n=11) had pulmonary hypertension, while 93.53% (n=159) did not show any signs of this morbidity.
CONCLUSION: We came to the conclusion that pulmonary hypertension is not significantly more common in non-transfusion dependent thalassemia (NTDT). However, as it is the major data, we believe that smoother multicenter local trials could validate it.
References
Colah R, Gorakshakar A, Nadkarni A. Global burden, distribution and prevention of beta-thalassemias and hemoglobin E disorders. Expert Rev Hematol 2010;3(1):103-117.
Ahmed S, Saleem M, Sultana N, Raashid Y. Prenatal diagnosis of β-thalassemia in Pakistan: Experience in a Muslim country. Prenatdiagn. 2000;20:378-83.
Musallam KM, Rivella S, Vichinsky E, Rachmilewitz EA. Non-transfusion-dependent thalassemias. Haematologica 2013;98(1): 833-844.
Derchi G, Galanello R, Bina P, Cappellini MD, Piga A, Lai ME, Quarta A, Casu G, Perrotta S, Musallam KM, Forni GL. Estimation of the prevalence of pulmonary artery hypertension in a large group of β-thalassemia patients using right heart catheterization. Blood 2012;120(21):3262.
Taher A, Isma’eel H, Cappellini MD. Thalassemia intermedia: revisited. Blood Cells Mol Dis 2006;37(1):12-20.
Amoozgar H, Farhani N, Karimi M. Early echocardiographic findings in beta-thalassemia intermedia patients usingstandard and tissue Doppler methods. PediatrCardiol 2011;32(2):154-159.
Yeager ME, Nguyen CM, Belchenko DD. Circulating fibrocytes are increased in children and young adults with pulmonary hypertension. Eur Respir J. 2012;39(1):104–111.
Chueamuangphan N, Wongtheptien W, Nawarawong W, Sukornthasarn A, Chuncharunee S, Tawichasri C, Patumanond J. Clinical indicators for pulmonary arterial hypertension in thalassemia. J Med Assoc Thai 2012;95(1):16-21.
Farmakis D, Aessopos A. Pulmonary hypertension associated with hemoglobinopathies: prevalent but overlooked. Circulation. 2011;123(11):1227–32
Levin C, Koren A. Healing of refractory leg ulcer in a patient with thalassemia intermedia and hypercoagulability after 14 years of unresponsive therapy. Isr Med Assoc J. 2011;13(5):316–8
Badesch DB, Champion HC, Sanchez MA. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(1, Suppl):S55–S66.
Beghetti M, Galiè N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;53(9):733–740.
Downloads
Published
Issue
Section
License
Copyright (c) 2025 Abnas Batool (Author)
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
