Pulmonary hypertension in patients with thalassemia major

Du, Zhenhong; Roguin, Nathan; Milgram, Elliot; Saab, Kamal; Koren, Ariel

doi:10.1016/s0002-8703(97)70091-7

Cited by 123 publications

(106 citation statements)

References 26 publications

Supporting

Mentioning

102

Contrasting

Order By: Relevance

“…4 The presence of a persistent hypercoagulable state combined with the infrequent occurrence of significant thrombotic events suggests that thrombosis is largely a subclinical process in thalassemia and has been associated with autopsy findings of platelet and fibrin thrombi in the microvasculature in the lungs and the brain. 20,21 These thrombi could contribute to the pulmonary hypertension, low lung capacity, hypoxemia, and diffusion defects associated with right heart failure (cor pulmonale) [22][23][24][25][26][27] and to the high frequency of ischemic brain lesions associated with asymptomatic brain damage as detected by MRI. 14 Several etiologic factors may play a role in the pathogenesis of the hypercoagulable state in thalassemia.…”

Section: Discussionmentioning

confidence: 99%

“…24,25 In support of this idea, a Doppler echocardiography study in which pulmonary artery pressure was measured in 33 patients with ␤-TM (aged 2-24 years) showed that 28 patients had evidence of pulmonary hypertension. 26 Pulmonary artery hypertension was also detected by M-mode and Doppler echocardiography in 15 of 16 children, aged 5 to 14 years, with homozygous ␤ thalassemia and ␤ thalassemia/hemoglobin E disease. 27 Right ventricular dysfunction was detected earlier than left ventricular dysfunction in these children, suggesting that the right heart failure and pulmonary hypertension seen in thalassemia could result from microembolization in the lungs.…”

Section: Deep Venous Thrombosis and Pulmonary Embolismmentioning

confidence: 94%

See 1 more Smart Citation

The hypercoagulable state in thalassemia

Eldor

Rachmilewitz

2002

Blood

436

376

View full text Add to dashboard Cite

Thalassemia is a congenital hemolytic disorder caused by a partial or complete deficiency of ␣-or ␤-globin chain synthesis. Homozygous carriers of ␤-globin gene defects suffer from severe anemia and other serious complications from early childhood. The disease is treated by chronic blood transfusion. However, this can cause severe iron overload resulting in progressive organ failure. Some forms of ␣ thalassemia are also associated with a similar clinical picture. Despite the difficulties associated with treatment, standards of care for thalassemic patients have improved in recent years, resulting in almost doubling of the average life expectancy. As a consequence, additional previously undescribed, complications are now being recognized. In particular, profound hemostatic changes have been observed in patients with ␤-thalassemia major (␤-TM) and ␤-thalassemia intermedia (␤-TI) and also in patients with ␣ thalassemia (hemoglobin H disease). The presence of a higher than normal incidence of thromboembolic events, mainly in ␤-TI, and the existence of prothrombotic hemostatic anomalies in the majority of the patients, even from a very young age, have led to the recognition of the existence of a chronic hypercoagulable state in thalassemic patients. Despite the appearance of numerous publications on the frequent occurrence of thromboembolic complications in thalassemia, this complication has not been emphasized or comprehensively reviewed. This review summarizes the current literature and discusses possible mechanisms of the lifelong hypercoagulable state that exists in thalassemia. (Blood. 2002;99:36-43)

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Deep Venous Thrombosis and Pulmonary Embolismmentioning

confidence: 94%

The hypercoagulable state in thalassemia

Eldor

Rachmilewitz

2002

Blood

436

376

View full text Add to dashboard Cite

show abstract

“…Although right heart failure was suggested to be secondary to pulmonary hypertension in previous studies (33,34), results of recently published reports support that right heart failure develops primarily (26,32). In postmortem examination of cardiac hemochromatosis, both ventricles were shown to be involved equally (35).…”

Section: In 2001mentioning

confidence: 92%

Beta talasemi majorlu çocuk ve ergenlerde doku Doppler yöntemiyle diyastolik işlevlerin değerlendirilmesi

Yavuz¹,

Nişli²,

Ertuğrul³

et al. 2011

tpa

View full text Add to dashboard Cite

A As ss se es ss sm me en nt t o of f d di ia as st to ol li ic c f fu un nc ct ti io on n i in n c ch hi il ld dr re en n a an nd d a ad do ol le es sc ce en nt ts s w wi it th h b be et ta a--t th ha al la as ss se em mi ia a m ma aj jo or r b by y t ti is ss su ue e D Do op pp pl le er r i im ma ag gi in ng g T Ta an ne er r Y Ya av vu uz z, , K Ke em ma al l N Ni ifl fll li i* *, , N Na ac ci i Ö Ön ne er r* ** *, , T Tü ür rk ka an n E Er rt tu u¤ ¤r ru ul l* *, , Z Za af fe er r fi fia al lc c› ›o o¤ ¤l lu u * ** ** *, , G Gö ön nü ül l A Ay yd do o¤ ¤a an n* ** ** *, , Z Ze ey yn ne ep p K Ka ar ra ak ka afl fl* ** ** ** *, , Ü Üm mr ra ah h A Ay yd do o¤ ¤a an n* *, , A Ay yg gü ün n D Di in nd da ar r* *, , R Ru uk ki iy ye e E Ek ke er r Ö Öm me er ro o¤ ¤l lu u* * Material and Method:The study included 61 beta-thalassemia major subjects (age 4 to 20 years; mean age, 10.7±4.1 years; 32 females and 29 males) with normal left ventricular function and 52 healthy control subjects matched for age and sex. All participants underwent M-mode echocardiography and left ventricular systolic function was measured; diastolic function of the right and left ventricle was analysed using tissue Doppler imaging and conventional Doppler echocardiography. SPSS for Windows 13.0 sofware program were used for statistical analysis, and student's t-test were used to compare data. This study was approved by the ethics committee of the Istanbul Medical Faculty. Results: Body surface area was significantly smaller in the patient group than in the control group (1.0±0.2 vs. 1.2±0.3 m 2 , p<0.01). Transmitral early peak-E velocity (E) and E/A ratio was found to be significantly increased in patients in comparison to the controls (p<0.01 and p=0.039, respectively). Late peak-A velocity (A) of mitral valve and all Doppler velocities across the tricuspid valve did not differ between groups (p>0.05). In patients with beta-thalassemia major, the early diastolic (Em) velocities of the myocardium at the base of the left and right ventricle, at the middle segment of the left and right ventricle, and the interventricular septum were found to be higher than controls (p<0.05). We also found significantly higher late diastolic (Am) velocities at the base and middle segments of RV and higher Em/Am ratio at the base of the right ventricle and at the middle segments of the left ventricle, right ventricle and the interventricular septum compared with controls (p<0.05). Conclusions: These findings obtained from young aged beta-thalassemia major patients with normal ventricular systolic function were believed to arise from high preload and hyperdynamic response to chronic anemia rather than true ventricular restriction. We suggest that long-term follow-up studies should be carried out in patients with beta-thalassemia major using tissue Doppler in order to evaluate the diagnostic accuracy of this imaging technique for the diagnosis of early stages of cardiac involvement. (Turk Arch Ped 2011; 46: 27-32)

show abstract

“…Recent studies suggest that patients with thalassemia major (TM) and, even more so, those with thalassemia intermedia (TI), have a unique hemodynamic pattern consistent with right ventricular cardiomyopathy and PH, in addition to the better characterized left ventricular abnormalities. [4][5][6][7][8][9][10] Advancing age and a history of splenectomy are major risk factors for PH in this population. 3,[10][11][12][13] Other risk factors include transfusion-naivety and an elevated nucleated red blood cell count.…”

Section: Introductionmentioning

confidence: 99%

“…TM patients for some time, 1,2,4,5,16,17,[23][24][25][26][27] specific guidelines for the management of these patients have not been established and clinical trials are lacking. The phosphodiesterase-5 (PDE-5) inhibitor sildenafil has been shown to reduce pulmonary vascular resistance and improve functional capacity in PH.…”

Section: Introductionmentioning

confidence: 99%