Risk factors for pulmonary hypertension in patients with β thalassemia intermedia

Karimi, Mehran; Musallam, Khaled M.; Cappellini, Maria Domenica; Daar, Shahina; El‐Beshlawy, Amal; Belhoul, Khawla; Saned, Mohamed Salaheldin; Temraz, Sally; Koussa, Suzanne; Taher, Ali T.

doi:10.1016/j.ejim.2011.05.013

Cited by 53 publications

(50 citation statements)

References 40 publications

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“…Nonetheless, and similar to patients with Class 4 pulmonary hypertension attributed to chronic thromboembolic disease, hypercoagulability can play a major role in the cause of PAH, where thrombi may be present in both the small distal pulmonary arteries and the proximal elastic pulmonary arteries. 7 The association of splenectomy and older age with increased PAH risk in our study echoes previous reports 18,19,22,24,25,33,35,[39][40][41] and further supports the latter hypothesis. Abnormalities of platelets and hemolyzed red blood cells are believed to be the key factors causing hypercoagulability and subsequent thrombotic events in β-thalassemia; especially older, splenectomized, β-thalassemia intermedia patients.…”

Section: Discussionsupporting

confidence: 81%

“…7,8,36 Although pulmonary hypertension can occur in β-thalassemia patients in the setting of siderotic leftventricular dysfunction, pulmonary hemosiderosis and subsequent fibrosis, 37 and chronic thromboembolic disease, 22,38,39 these remain less commonly reported pulmonary hypertension settings (classes) and such patients would have been excluded from our study which exclusively aimed to evaluate PAH. Nonetheless, and similar to patients with Class 4 pulmonary hypertension attributed to chronic thromboembolic disease, hypercoagulability can play a major role in the cause of PAH, where thrombi may be present in both the small distal pulmonary arteries and the proximal elastic pulmonary arteries.…”

Section: Discussionmentioning

confidence: 99%

“…42 The association of thrombocytosis, increased platelet activation, increased levels of nucleated red blood cells, and other markers of hypercoagulability with PAH in patients with β-thalassemia (mostly intermedia) have been reported, even in patients with no evidence or history of pulmonary embolic disease. 19,24,25,34,39,43 Other factors are also suggested to cause PAH in β-thalassemia. Associations between lower hemoglobin levels and increased markers of hemolysis with this morbidity have been reported.…”

Section: Discussionmentioning

confidence: 99%

“…46 An association between iron overload (or absence of chelation therapy) and PAH has also been observed in patients with β-thalass emia. 17,22,31,39,[47][48][49] Iron is implicated in the pathogenesis of several vascular disorders including atherosclerosis, arterial thrombosis, microangiopathic hemolytic anemia, vasculitis, and reperfusion injury in nonthalassemic populations. 50 The presence of non-transferrin-bound iron in the sera of β-thalassemia patients can cause oxidative vessel injury and endothelial activation.…”

Section: Discussionmentioning

confidence: 99%

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Prevalence and Risk Factors for Pulmonary Arterial Hypertension in a Large Group of β-Thalassemia Patients Using Right Heart Catheterization

et al. 2014

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Distinction of the various forms of β-thalassemia relies on clinical grounds. Patients with β-thalassemia major present to medical attention in early childhood with severe anemia requiring life-long transfusion and iron chelation therapy for survival.2 Conversely, patients with β-thalassemia intermedia present later in childhood with milder anemia and remain largely transfusion-independent.3 Such variation in phenotype has been attributed to several environmental and genetic factors that alter the α/β-chain imbalance and subsequent ineffective erythropoiesis and peripheral hemolysis, the hallmarks of disease process in β-thalassemia. 4 Clinical Perspective on p 345Among the sequelae associated with a diagnosis of β-thalassemia, pulmonary arterial hypertension (PAH) has received great attention in recent years.5 PrevalenceBackground-Pulmonary arterial hypertension (PAH) remains a concern in patients with β-thalassemia major (TM) and intermedia (TI); however, studies evaluating its prevalence and risk factors using systematic confirmation on right heart catheterization are lacking. Methods and Results-This was a multicenter cross-sectional study of 1309 Italian β-thalassemia patients (mean age 36.4±9.3 years; 46% men; 74.6% TM, 25.4% TI). Patients with a tricuspid-valve regurgitant jet velocity ≥3.2 m/s (3.6%) on transthoracic echocardiography further underwent right heart catheterization to confirm the diagnosis of PAH (mean pulmonary arterial pressure ≥25 mm Hg and pulmonary capillary wedge pressure ≤15mm Hg). The confirmed PAH prevalence on right heart catheterization was 2.1% (95% confidence interval [CI], 1.4-3.0) and was higher in TI (4.8%; 95% CI, 3.0-7.7) than TM (1.1%; 95% CI, 0.6-2.0). The positive predictive value for the tricuspid-valve regurgitant jet velocity ≥3.2 m/s threshold for the diagnosis of pulmonary hypertension was 93.9%. Considerable functional limitation and decrease in the 6-minute walk distance were noted in patients with confirmed PAH. On multivariate logistic regression analysis, independent risk factors for confirmed PAH were age (odds ratio, 1.102 per 1-year increase; 95% CI, 1.06-1.15) and splenectomy (odds ratio, 9.31; 95% CI, 2.57-33.7). Conclusions-The prevalence of PAH in β-thalassemia patients as confirmed on right heart catheterization was 2.1%, with an ≈5-fold higher prevalence in TI than TM. Advanced age and splenectomy are risk factors for PAH in this patient population. Clinical Trial Registration-URL: http://www.ClinicalTrials.gov. Unique identifier: NCT01496963.(Circulation. 2014;129:338-345.)

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Section: Discussionsupporting

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Prevalence and Risk Factors for Pulmonary Arterial Hypertension in a Large Group of β-Thalassemia Patients Using Right Heart Catheterization

et al. 2014

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“…24,83,84 Observational studies continue to confirm that NTDT patients who receive transfusions experience fewer leg ulcers, thrombotic events, pulmonary hypertension, and silent brain infarcts compared with transfusion-naïve patients. 30,64,80,85,86 Successful management of the hematopoietic compensatory extramedullary pseudotumors has also been reported using transfusion therapy with and without radiation or surgery, especially in the most debilitating cases with paraspinal involvement. 31 It is absolutely essential to assess the patient carefully over the first few months after the diagnosis is established and not to embark on any treatment modality, especially transfusion therapy, too hastily.…”

Section: Transfusion Therapymentioning

confidence: 99%

Non-transfusion-dependent thalassemias

Musallam

Rivella

Vichinsky³

et al. 2013

Haematologica

249

268

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A prospective analysis for prevalence of complications in Thai nontransfusion‐dependent Hb E/β‐thalassemia and α‐thalassemia (Hb H disease)

2018

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Recently, complications in patients with nontransfusion-dependent thalassemia (NTDT), in particular those with β-thalassemia intermedia (β-TI), were found to be significantly different from those in patients with transfusion dependent thalassemia (TDT), mainly β-thalassemia major (β-TM). However, this information is rather limited in other forms of NTDT. In this prospective study, adult Thai NTDT patients were interviewed and clinically evaluated for thalassemia related complications. Fifty-seven NTDT patients (age 18-74 years), 59.6% Hb E/β-thalassemia and 40.4% Hb H disease, were recruited; 26.4% were splenectomized. The most common complications were gallstones (68.4%), osteoporosis (26.3%), and pulmonary hypertension (15.8%). Splenectomy was associated with higher rate of gallstones and serious infection (P = .001 and .052, respectively), consistent with a multivariate analysis (RR = 9.5, P = .044, and RR = 15.1, P = .043, respectively). In addition, a higher hemoglobin level was inversely associated with gallstones in both univariate and multivariate analyses (P = .01 and .022, respectively). Serum ferritin was associated with abnormal liver function (P = .002). In contrast to the previous study, the prevalence of thrombosis was less common in our population (1.7%), probably due to differences in transfusion therapy, ethnicity, and underlying genotypes. For the first time, this prospective study provided the current prevalence of NTDT related complications in a Southeast Asian population with a different underlying genetic basis compared with previous studies. Although individual prevalence of each complication might differ from other studies, several important clinical factors such as splenectomy, degree of anemia, and iron overload seem to be determining risks of developing these complications consistently across different ethnicities.

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Risk factors for pulmonary hypertension in patients with β thalassemia intermedia

Cited by 53 publications

References 40 publications

Prevalence and Risk Factors for Pulmonary Arterial Hypertension in a Large Group of β-Thalassemia Patients Using Right Heart Catheterization

Prevalence and Risk Factors for Pulmonary Arterial Hypertension in a Large Group of β-Thalassemia Patients Using Right Heart Catheterization

Non-transfusion-dependent thalassemias

A prospective analysis for prevalence of complications in Thai nontransfusion‐dependent Hb E/β‐thalassemia and α‐thalassemia (Hb H disease)

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