Limits...
Effects of combined deferiprone with deferoxamine on right ventricular function in thalassaemia major.

Alpendurada F, Smith GC, Carpenter JP, Nair SV, Tanner MA, Banya W, Dessi C, Galanello R, Walker JM, Pennell DJ - J Cardiovasc Magn Reson (2012)

Bottom Line: The right ventricle (RV) is also affected by the toxic effects of iron and may cause additional cardiovascular perturbation.The increase in RVEF was greater with lower baseline T2* 8-12 ms (4.7 vs 0.5%, p = 0.01) than with T2* 12-20 ms (2.2 vs 0.8%, p = 0.47).Therefore adding deferiprone to deferoxamine has beneficial effects on both RV and LV function in TM patients with cardiac siderosis.

View Article: PubMed Central - HTML - PubMed

Affiliation: Royal Brompton & Harefield NHS Foundation Trust, London, UK.

ABSTRACT

Background: Combination therapy with deferoxamine and oral deferiprone is superior to deferoxamine alone in removing cardiac iron and improving left ventricular ejection fraction (LVEF). The right ventricle (RV) is also affected by the toxic effects of iron and may cause additional cardiovascular perturbation. We assessed the effects of combination therapy on the RV in thalassaemia major (TM) using cardiovascular magnetic resonance (CMR).

Methods: We retrieved imaging data from 2 treatment trials and re-analyzed the data for the RV responses: Trial 1 was a randomized controlled trial (RCT) of 65 TM patients with mild-moderate cardiac siderosis receiving combination therapy or deferoxamine with placebo; Trial 2 was an open label longitudinal trial assessing combination therapy in 15 TM patients with severe iron loading.

Results: In the RCT, combination therapy with deferoxamine and deferiprone was superior to deferoxamine alone for improving RVEF (3.6 vs 0.7%, p = 0.02). The increase in RVEF was greater with lower baseline T2* 8-12 ms (4.7 vs 0.5%, p = 0.01) than with T2* 12-20 ms (2.2 vs 0.8%, p = 0.47). In patients with severe cardiac siderosis, substantial improvement in RVEF was seen with open-label combination therapy (10.5% ± 5.6%, p < 0.01).

Conclusions: In the RCT of mild to moderate cardiac iron loading, combination treatment improved RV function significantly more than deferoxamine alone. Combination treatment also improved RV function in severe cardiac siderosis. Therefore adding deferiprone to deferoxamine has beneficial effects on both RV and LV function in TM patients with cardiac siderosis.

Show MeSH

Related in: MedlinePlus

Breakdown of improvement in RVEF (%) for different groups according to chelation therapy and myocardial T2* baseline. Vertical lines represent standard error.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3278357&req=5

Figure 4: Breakdown of improvement in RVEF (%) for different groups according to chelation therapy and myocardial T2* baseline. Vertical lines represent standard error.

Mentions: When grouping the patients on combination therapy (the 32 patients from the combination arm in the RCT plus the 15 unblinded patients on open-label combined therapy), we found an inverse relation between myocardial T2* at baseline and improvement in RVEF over one year (Figure 4). Patients with severe iron loading had a greater improvement in RVEF than patients with moderate iron loading (10.5% vs. 4.7%, p < 0.01).


Effects of combined deferiprone with deferoxamine on right ventricular function in thalassaemia major.

Alpendurada F, Smith GC, Carpenter JP, Nair SV, Tanner MA, Banya W, Dessi C, Galanello R, Walker JM, Pennell DJ - J Cardiovasc Magn Reson (2012)

Breakdown of improvement in RVEF (%) for different groups according to chelation therapy and myocardial T2* baseline. Vertical lines represent standard error.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3278357&req=5

Figure 4: Breakdown of improvement in RVEF (%) for different groups according to chelation therapy and myocardial T2* baseline. Vertical lines represent standard error.
Mentions: When grouping the patients on combination therapy (the 32 patients from the combination arm in the RCT plus the 15 unblinded patients on open-label combined therapy), we found an inverse relation between myocardial T2* at baseline and improvement in RVEF over one year (Figure 4). Patients with severe iron loading had a greater improvement in RVEF than patients with moderate iron loading (10.5% vs. 4.7%, p < 0.01).

Bottom Line: The right ventricle (RV) is also affected by the toxic effects of iron and may cause additional cardiovascular perturbation.The increase in RVEF was greater with lower baseline T2* 8-12 ms (4.7 vs 0.5%, p = 0.01) than with T2* 12-20 ms (2.2 vs 0.8%, p = 0.47).Therefore adding deferiprone to deferoxamine has beneficial effects on both RV and LV function in TM patients with cardiac siderosis.

View Article: PubMed Central - HTML - PubMed

Affiliation: Royal Brompton & Harefield NHS Foundation Trust, London, UK.

ABSTRACT

Background: Combination therapy with deferoxamine and oral deferiprone is superior to deferoxamine alone in removing cardiac iron and improving left ventricular ejection fraction (LVEF). The right ventricle (RV) is also affected by the toxic effects of iron and may cause additional cardiovascular perturbation. We assessed the effects of combination therapy on the RV in thalassaemia major (TM) using cardiovascular magnetic resonance (CMR).

Methods: We retrieved imaging data from 2 treatment trials and re-analyzed the data for the RV responses: Trial 1 was a randomized controlled trial (RCT) of 65 TM patients with mild-moderate cardiac siderosis receiving combination therapy or deferoxamine with placebo; Trial 2 was an open label longitudinal trial assessing combination therapy in 15 TM patients with severe iron loading.

Results: In the RCT, combination therapy with deferoxamine and deferiprone was superior to deferoxamine alone for improving RVEF (3.6 vs 0.7%, p = 0.02). The increase in RVEF was greater with lower baseline T2* 8-12 ms (4.7 vs 0.5%, p = 0.01) than with T2* 12-20 ms (2.2 vs 0.8%, p = 0.47). In patients with severe cardiac siderosis, substantial improvement in RVEF was seen with open-label combination therapy (10.5% ± 5.6%, p < 0.01).

Conclusions: In the RCT of mild to moderate cardiac iron loading, combination treatment improved RV function significantly more than deferoxamine alone. Combination treatment also improved RV function in severe cardiac siderosis. Therefore adding deferiprone to deferoxamine has beneficial effects on both RV and LV function in TM patients with cardiac siderosis.

Show MeSH
Related in: MedlinePlus