Pulmonary hypertension in patients with thalassemia major☆,☆☆
Section snippets
Subjects
Thirty-three consecutive patients (18 male, 15 female) with β-thalassemia major were studied from December 1994 to December 1995. All patients were regularly monitored in our pediatric hematology clinic. Age varied from 2 to 24 years (mean ± SD 12.1 ± 6.6 years). All but two patients had no cardiac symptoms or clinically apparent heart failure, and none of them were taking cardioactive medications at the time of examination. Each of the patients received blood transfusions approximately every 3
Clinical features
Of 33 patients, 25 had a serum ferritin level >1000 ng/ml, eight had a level <1000 ng/ml; 21 patients had undergone splenectomy; 27 of the 30 patients (three cases did not have liver function test results) had abnormal liver function test results (aspartate aminotransferase >40 U/L, total bilirubin >1 mg/dl, and direct bilirubin >0.2 mg/dl). Only two patients had cardiac symptoms at the time of cardiac evaluation. Cardiac failure developed in three patients 4 months after the examination. Two
Discussion
Impaired left ventricular systolic and diastolic function has been well investigated in patients with thalassemia major. 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12 A few studies have also noticed right ventricular dysfunction in these patients, which has been found to be more prevalent and present earlier than left ventricular dysfunction. 8, 25 Therefore it has been speculated that pulmonary hypertension may be the cause, although without direct evidence. Recently Aessopos et al. 14 measured
Acknowledgements
We thank Drs. Elizabeth Shternberg, Larisa Lukina, and Manhal A. Hanna for their help in collecting clinical data.
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Cited by (110)
Evaluation of ECG and echocardiographic findings in patients aged 2 to 18 years affected with thalassemia major
2021, Progress in Pediatric CardiologyCitation Excerpt :In other words, when LVEF falls below 45% and the heart progresses toward severe congestive heart failure, because of a more dramatic increase in left ventricular end-diastolic pressure compared to the left ventricular dilatation, SPAP rises again to 52 mm Hg and therefore in thalassemia major, in addition to hemolysis which causes the release of arginase from erythrocytes and disruption of arginine metabolism and finally, reduction of nitric oxide [33–35], histological and structural changes of myocardium also bear a critical effect on pulmonary blood pressure and affect pulmonary hypertension resulting from hemolysis. Further, in a study by Zhong et al. [36], in all patients over 10 years and also in two-thirds of the patients under 10, the pulmonary artery systolic pressure proved to be above 30 mm Hg. The result of their study was that pulmonary hypertension occurs in the early stages of heart involvement in thalassemia major.
Study of platelet activation, hypercoagulable state, and the association with pulmonary hypertension in children with β-thalassemia
2018, Hematology/ Oncology and Stem Cell TherapyCitation Excerpt :Several studies reported a similar prevalence of pH in thalassemic patients [33,34]. By contrast, a higher prevalence (79%) was reported in children by Du et al. [35]. The difference between studies could be attributed to the different characteristics of the studied populations and treatment modalities they were receiving, in addition to discrepancies in the methods used to define the PH.
A global view of pulmonary hypertension
2016, The Lancet Respiratory Medicine
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Reprint requests: Zhong-Dong Du, MD, Division of Cardiology, Capital Institute of Pediatrics & The Children’s Hospital, No. 2, Ya Bao Rd., 100020 Beijing, China. E-mail: [email protected]
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