Pulmonary hypertension in well-transfused thalassemia major patients

Meloni, Antonella; Detterich, Jon; Pepe, Alessia; Harmatz, Paul; Coates, Thomas D.; Wood, John C.

doi:10.1016/j.bcmd.2014.11.003

Cited by 30 publications

(18 citation statements)

References 60 publications

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“…This notion may be at the center of the argument of the need to transfuse patients with non-transfusion dependent thalassemia (NTDT) more regularly to avoid or delay PH [28]. Female predominance, though insignificant, among patients with PH in the current study is shared by some reports [29,30], but disputed by others [17][18][19]. Strong female predominance in many forms of PH is well recognized [31].…”

Section: Plos Onementioning

confidence: 60%

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Doppler-defined pulmonary hypertension in β-thalassemia major in Kurdistan, Iraq

Mohammad

Dawad²,

Kashmoola³

et al. 2020

PLoS ONE

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Cardiopulmonary complications are among the most important complications of thalassemia major. Pulmonary hypertension is among these complications and studies addressing its frequency and associations in the latter disorder are sparse from Iraq. For this purpose a total 100 thalassemia major patients (≥ 8 years old) were enrolled from a main thalassemia center in Kurdistan, Northern Iraq. All patients had a full history and clinical examination. Full blood count, biochemical tests and viral screen including hepatitis B surface antigen and hepatitis C virus antibody, in addition to transthoracic Doppler echocardiography for tricuspid regurgitation jet velocity (TRV). The enrolled patients had a mean (SD) age of 17.6 (5.5) years, and included 52 males and 48 females. Pulmonary hypertension as defined by TRV> 2.8 m/s coupled with both exertional dyspnea and an absence of left sided heart failure, was identified in nine patients (9%). The latter subgroup of patients had significantly higher reticulocyte counts, S. LDH, S. ferritin, and hepatitis C sero-positivity compared to those without this complication by univariate analysis. While by multivariate logistic regression only reticulocytes and hepatitis C sero-positivity remained significant. Furthermore, TRV as a continuous variable was positively correlated with reticulocytes, S. bilirubin and LDH (p<0.001, p = 0.002 and p<0.001 respectively), but not with age or S. ferritin (p = 0.77, and p = 0.93 respectively). In conclusion, pulmonary hypertension is not uncommon in Iraqi patients with thalassemia major, and it appears to be linked to chronic hemolysis rather than iron overload.

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Section: Plos Onementioning

confidence: 60%

“…While PH is a well-recognized complication of TM, its doppler-defined frequencies in various TM series showed remarkable variability ranging from 1.7% to 79% [13][14][15][16][17][18][19]. The most important cause of such variability is the choice of the TVR cut-off point.…”

Section: Discussionmentioning

confidence: 99%

Doppler-defined pulmonary hypertension in β-thalassemia major in Kurdistan, Iraq

Mohammad

Dawad²,

Kashmoola³

et al. 2020

PLoS ONE

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“…Meloni et al [62] reported echocardiography results from 60 TM patients and evaluated the association between TRV, iron stores, and serologic markers of hemolysis. They concluded that well-transfused TM patients have a lower risk for PH.…”

Section: Discussionmentioning

confidence: 99%

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

et al. 2017

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Background: Ischemia-modified albumin (IMA) is an altered type of serum albumin that forms under conditions of oxidative stress and an independent predictor of major adverse cardiovascular events. Objectives: To measure the levels of IMA in 45 children and adolescents with β-thalassemia major (β-TM) compared with 30 healthy controls and assess its relation to lipid peroxidation, vascular complications and subclinical atherosclerosis. Methods: β-TM patients without symptoms of heart disease were studied focusing on transfusion history, chelation therapy, serum ferritin, malondialdehyde (MDA) and IMA levels. Echocardiography was performed and carotid intima media thickness (CIMT) was assessed. Results: IMA and MDA levels were significantly higher in β-TM patients compared with controls (p < 0.001). IMA was higher among patients with heart disease, pulmonary hypertension risk and serum ferritin ≥2500 µg/l than those without. TM patients compliant to chelation had significantly lower IMA levels. IMA levels were positively correlated to MDA and CIMT while negatively correlated to ejection fraction and fractional shortening. Conclusion: Our results highlight the role of oxidative stress in the pathophysiology of vascular complications in thalassemia. IMA could be useful for screening of β-TM patients at risk of cardiopulmonary complications and atherosclerosis because its alteration occurs in early subclinical disease.

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“…We have previously reported low GAB in thalassaemia compared to normal control subjects (Morris et al , ), however patients were not differentiated by TRV or cardiopulmonary dysfunction. Of interest, Meloni et al () recently published an association of TRV elevation with reduced GAB, together with increased anaemia, cardiac index and diastolic dysfunction in 60 TM patients. We describe, however, the first report to identify excess arginase activity as a key mechanism associated with low GAB in thalassaemia.…”

Section: Discussionmentioning

confidence: 99%

Dysregulated arginine metabolism and cardiopulmonary dysfunction in patients with thalassaemia

et al. 2015

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Pulmonary hypertension (PH) commonly develops in thalassaemia syndromes, but is poorly characterized. The goal of this study was to provide a comprehensive description of the cardiopulmonary and biological profile of patients with thalassaemia at risk for PH. A case-control study of thalassaemia patients at high versus low PH-risk was performed. A single cross-sectional measurement for variables reflecting cardiopulmonary status and biological pathophysiology were obtained, including Doppler-echocardiography, 6-minute-walk-test, Borg Dyspnea Score, New York Heart Association functional class, cardiac magnetic resonance imaging (MRI), chest-computerized tomography, pulmonary function testing and laboratory analyses targeting mechanism of coagulation, inflammation, haemolysis, adhesion and the arginine-nitric oxide pathway. Twenty-seven thalassaemia patients were evaluated, 14 with an elevated tricuspid-regurgitant-jet-velocity (TRV) ≥2.5m/s. Patients with increased TRV had a higher frequency of splenectomy, and significantly larger right atrial size, left atrial volume and left septal-wall thickness on echocardiography and/or MRI, with elevated biomarkers of abnormal coagulation, lactate dehydrogenase levels and arginase concentration, and lower arginine-bioavailability compared to low-risk patients. Arginase concentration correlated significantly to several echocardiography/MRI parameters of cardiovascular function in addition to global-arginine-bioavailability and biomarkers of haemolytic rate, including lactate dehydrogenase, haemoglobin and bilirubin. Thalassaemia patients with a TRV ≥2.5m/s have additional echocardiography and cardiac-MRI parameters suggestive of right and left-sided cardiac dysfunction. In addition, low arginine bioavailability may contribute to cardiopulmonary dysfunction in β-thalassaemia.

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Pulmonary hypertension in well-transfused thalassemia major patients

Cited by 30 publications

References 60 publications

Doppler-defined pulmonary hypertension in β-thalassemia major in Kurdistan, Iraq

Doppler-defined pulmonary hypertension in β-thalassemia major in Kurdistan, Iraq

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

Dysregulated arginine metabolism and cardiopulmonary dysfunction in patients with thalassaemia

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