Skip to main content
Top
Published in: Pediatric Cardiology 3/2015

01-03-2015 | Original Article

Early Detection of Right Ventricular Diastolic Dysfunction by Pulsed Tissue Doppler Echocardiography in Iron Loaded Beta Thalassemia Patients

Authors: Hala Mounir Agha, Amal Beshlawy, Mona Hamdy, Alae Sobeih, Fatma El Zahrae, Inas Abd El Satar, Antoine AbdelMassih, Fadwa Said, Ossama Abd El Aziz, Mona El Tagui, Dudley J. Pennell

Published in: Pediatric Cardiology | Issue 3/2015

Login to get access

Abstract

Early heart iron overload in beta thalassemia major patients can be quantified through T2* cardiovascular magnetic resonance (CMR). To clarify the value of tissue Doppler imaging (TDI) in early detection of myocardial dysfunction in iron loaded thalassemia patients diagnosed by CMR. Two groups were included in the study; Group I: 69 asymptomatic thalassemia patients (28 females, 41 males), mean age 18.1 ± 7.03 years (range 6–39 years); Group II (n = 41) healthy normal controls matched for age and sex. Serum ferritin and CMR were performed to assess the cardiac siderosis (T2* < 20 ms). Group I was subdivided into two subgroups; Group Ia (n = 26) T2* < 20 ms and Group Ib (n = 43) T2* > 20 ms. Conventional and Doppler echocardiography of LV, RV dimensions and functions and pulmonary artery pressure were evaluated. Right ventricular diastolic function assessed by tricuspid annular E′/A′ was positively correlated with T2* value; lower tricuspid E′/A′ ratios were correlated with lower T2* values (r = 0.366, P = 0.002). Tricuspid annular A′ was significantly higher in group Ia compared to group Ib (16.7 ± 5.2 vs 12.1 ± 4.0 cm/s, P < 0.001). Tricuspid E′/A′ < 1 was common in group Ia compared to group Ib (19/26 (73.0) vs 3/43 (6.97%), P < 0.001). By multivariate analysis, right ventricular diastolic dysfunction (tricuspid E′/A′ < 1) was associated with serum ferritin and T2* level of the thalassemia patients. TDI is a promising tool for quantitative assessment of myocardial function and early detection of right ventricular diastolic dysfunction in iron loaded beta thalassemia major patients.
Literature
1.
go back to reference Akpinar O, Acartürk E, Kanadaşi M, Unsal C, Başlamişli F (2007) Tissue doppler imaging and NT-proBNP levels show the early impairment of ventricular function in patients with beta-thalassaemia major. Acta Cardiol 62:225–231CrossRefPubMed Akpinar O, Acartürk E, Kanadaşi M, Unsal C, Başlamişli F (2007) Tissue doppler imaging and NT-proBNP levels show the early impairment of ventricular function in patients with beta-thalassaemia major. Acta Cardiol 62:225–231CrossRefPubMed
2.
go back to reference Alpendurada F, Carpenter JP, Deac M, Kirk P, Walker JM, Porter JB et al (2010) Relation of myocardial T2* to right ventricular function in thalassaemia major. Eur Heart J 31:1648–1654CrossRefPubMed Alpendurada F, Carpenter JP, Deac M, Kirk P, Walker JM, Porter JB et al (2010) Relation of myocardial T2* to right ventricular function in thalassaemia major. Eur Heart J 31:1648–1654CrossRefPubMed
3.
go back to reference Anderson LJ, Holden S, Davis B et al (2001) Cardiovascular T2 star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J 22:2171–2179CrossRefPubMed Anderson LJ, Holden S, Davis B et al (2001) Cardiovascular T2 star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J 22:2171–2179CrossRefPubMed
4.
go back to reference Bland JM, Altman DG (1986) Statistical methods for assessing agreement between two methods of clinical measurement. Lancet 8476(1):307–310CrossRef Bland JM, Altman DG (1986) Statistical methods for assessing agreement between two methods of clinical measurement. Lancet 8476(1):307–310CrossRef
5.
go back to reference Borgna-Pignatti C, Rugolotto S, De Stefano P, Piga A, Di Gregorio F, Gamberini MR, Sabato V, Melevendi C, Cappellini MD, Verlato G (1998) Survival and disease complications in thalassemia major. Ann N Y Acad Sci 850:227–231CrossRefPubMed Borgna-Pignatti C, Rugolotto S, De Stefano P, Piga A, Di Gregorio F, Gamberini MR, Sabato V, Melevendi C, Cappellini MD, Verlato G (1998) Survival and disease complications in thalassemia major. Ann N Y Acad Sci 850:227–231CrossRefPubMed
6.
go back to reference Borgna-Pignatti C, Rugolotto S, De Stefano P et al (2004) Survival and complication in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica 89:1187–1193PubMed Borgna-Pignatti C, Rugolotto S, De Stefano P et al (2004) Survival and complication in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica 89:1187–1193PubMed
7.
go back to reference Carpenter JP, Alpendurada F, Deac M, Maceira A, Garbowski M, Kirk P, Walker JM, Porter JB, Shah F, Banya W, He T, Smith GC, Pennell DJ (2010) Right ventricular volumes and function in thalassemia major patients in the absence of myocardial iron overload. J Cardiovasc Magn Reson 23(12):24CrossRef Carpenter JP, Alpendurada F, Deac M, Maceira A, Garbowski M, Kirk P, Walker JM, Porter JB, Shah F, Banya W, He T, Smith GC, Pennell DJ (2010) Right ventricular volumes and function in thalassemia major patients in the absence of myocardial iron overload. J Cardiovasc Magn Reson 23(12):24CrossRef
8.
go back to reference Cheitlin MD, Armstrong WF, Aurigemma GP, Beller GA, Bierman FZ, Davis JL et al (2003) ACC/AHA/ASE 2003 Guideline Update for the Clinical Application of Echocardiography. J Am Soc Echocardiogr 16:1091–1110PubMed Cheitlin MD, Armstrong WF, Aurigemma GP, Beller GA, Bierman FZ, Davis JL et al (2003) ACC/AHA/ASE 2003 Guideline Update for the Clinical Application of Echocardiography. J Am Soc Echocardiogr 16:1091–1110PubMed
9.
go back to reference Ehlers KH, Levin AR, Markenson AL, Marcus JR, Klein AA, Hilgartner MW, Engle MA (1980) Longitudinal study of cardiac function in thalassemia major. Ann N Y Acad Sci 344:397–404CrossRefPubMed Ehlers KH, Levin AR, Markenson AL, Marcus JR, Klein AA, Hilgartner MW, Engle MA (1980) Longitudinal study of cardiac function in thalassemia major. Ann N Y Acad Sci 344:397–404CrossRefPubMed
10.
11.
go back to reference Fernandes JL, Silveira MA, Fertrin K, Lauar S, Fattori A, Coelho O, Junqueira FP, da Cunha GM, Coutinho AC Jr, Pereira FB, Verissimo M, Saad ST (2012) Left and right ventricular function and volume assessment in young thalassemia major patients with no related myocardial iron overload. Ann Hematol 91:1839–1844CrossRefPubMed Fernandes JL, Silveira MA, Fertrin K, Lauar S, Fattori A, Coelho O, Junqueira FP, da Cunha GM, Coutinho AC Jr, Pereira FB, Verissimo M, Saad ST (2012) Left and right ventricular function and volume assessment in young thalassemia major patients with no related myocardial iron overload. Ann Hematol 91:1839–1844CrossRefPubMed
12.
go back to reference García-Fernández MA, Azevedo J, Moreno M, Bermejo J, Pérez- Castellano N, Puerta P et al (1999) Regional diastolic function in ischaemic heart disease using pulsed wave Doppler tissue imaging. Eur Heart J 20:496–505CrossRefPubMed García-Fernández MA, Azevedo J, Moreno M, Bermejo J, Pérez- Castellano N, Puerta P et al (1999) Regional diastolic function in ischaemic heart disease using pulsed wave Doppler tissue imaging. Eur Heart J 20:496–505CrossRefPubMed
13.
go back to reference Hahalis G, Manolis AS, Gerasimidou I, Alexopoulos D, Sitafidis G, Kourakli A, Körfer R, Koerner MM, Vagenakis AG, Zoumbos NC (2001) Right ventricular diastolic function in beta-thalassemia major: echocardiographic and clinical correlates. Am Heart J 141:428–434CrossRefPubMed Hahalis G, Manolis AS, Gerasimidou I, Alexopoulos D, Sitafidis G, Kourakli A, Körfer R, Koerner MM, Vagenakis AG, Zoumbos NC (2001) Right ventricular diastolic function in beta-thalassemia major: echocardiographic and clinical correlates. Am Heart J 141:428–434CrossRefPubMed
14.
go back to reference Hahalis G, Manolis AS, Apostolopoulos D, Alexopoulos D, Vagenakis AG, Zoumbos NC (2002) Right ventricular cardiomyopathy in beta-thalassaemia major. Eur Heart J 23:147–156CrossRefPubMed Hahalis G, Manolis AS, Apostolopoulos D, Alexopoulos D, Vagenakis AG, Zoumbos NC (2002) Right ventricular cardiomyopathy in beta-thalassaemia major. Eur Heart J 23:147–156CrossRefPubMed
15.
go back to reference Hamdy AM, Zein El-Abdin MY, Abdel-Hafez MA (2007) Right ventricular function in patients with beta thalassemia: relation to serum ferritin level. Echocardiography 24(8):795–801CrossRefPubMed Hamdy AM, Zein El-Abdin MY, Abdel-Hafez MA (2007) Right ventricular function in patients with beta thalassemia: relation to serum ferritin level. Echocardiography 24(8):795–801CrossRefPubMed
17.
go back to reference Hu Z, Metaxas D, Axel L (2003) In vivo strain and stress estimation of the heart left and right ventricles by MRI images. Med Image Anal 7:435–444CrossRefPubMed Hu Z, Metaxas D, Axel L (2003) In vivo strain and stress estimation of the heart left and right ventricles by MRI images. Med Image Anal 7:435–444CrossRefPubMed
18.
go back to reference Kremastinos DT, Tsiapras DP, Tsetsos GA, Rentoukas EI, Vretou HP, Toutouzas PK (1993) Left ventricular diastolic Doppler characteristics in beta-thalassemia major. Circulation 88:1127–1135CrossRefPubMed Kremastinos DT, Tsiapras DP, Tsetsos GA, Rentoukas EI, Vretou HP, Toutouzas PK (1993) Left ventricular diastolic Doppler characteristics in beta-thalassemia major. Circulation 88:1127–1135CrossRefPubMed
19.
go back to reference Kukulski T, Hubbert L, Arnold M, Wranne B, Hatle L, Sutherland GR (2000) Normal regional right ventricular function and its change with age: a doppler myocardial imaging study. J Am Soc Echocardiogr 13:194–204CrossRefPubMed Kukulski T, Hubbert L, Arnold M, Wranne B, Hatle L, Sutherland GR (2000) Normal regional right ventricular function and its change with age: a doppler myocardial imaging study. J Am Soc Echocardiogr 13:194–204CrossRefPubMed
20.
go back to reference Magrì D, Sciomer S, Fedele F, Gualdi G, Casciani E, Pugliese P, Losardo A, Ferrazza G, Pasquazzi E, Schifano E, Mussino E, Quaglione R, Piccirillo G (2008) Early impairment of myocardial function in young patients with beta-thalassemia major. Eur J Haematol 80(6):515–522CrossRefPubMed Magrì D, Sciomer S, Fedele F, Gualdi G, Casciani E, Pugliese P, Losardo A, Ferrazza G, Pasquazzi E, Schifano E, Mussino E, Quaglione R, Piccirillo G (2008) Early impairment of myocardial function in young patients with beta-thalassemia major. Eur J Haematol 80(6):515–522CrossRefPubMed
21.
go back to reference Nikitin NP, Witte KK (2004) Application of tissue Doppler imaging in cardiology. Cardiology 101:170–184CrossRefPubMed Nikitin NP, Witte KK (2004) Application of tissue Doppler imaging in cardiology. Cardiology 101:170–184CrossRefPubMed
22.
go back to reference Olivieri NF, Nathan DG, MacMillan JH, Wayne AS, Liu PP, McGee A et al (1994) Survival in medically treated patients with homozygous B-thalassemia. N Engl J Med 331:574–578CrossRefPubMed Olivieri NF, Nathan DG, MacMillan JH, Wayne AS, Liu PP, McGee A et al (1994) Survival in medically treated patients with homozygous B-thalassemia. N Engl J Med 331:574–578CrossRefPubMed
23.
go back to reference Pela G, Regolisti G, Coghi P, Cabassi A, Basile A, Cavatorta A et al (2004) Effects of the reduction of preload on left and right ventricular myocardial velocities analyzed by Doppler tissue echocardiography in healthy subjects. Eur J Echocardiogr 5:262–271CrossRefPubMed Pela G, Regolisti G, Coghi P, Cabassi A, Basile A, Cavatorta A et al (2004) Effects of the reduction of preload on left and right ventricular myocardial velocities analyzed by Doppler tissue echocardiography in healthy subjects. Eur J Echocardiogr 5:262–271CrossRefPubMed
24.
go back to reference Pennell DJ, Berdoukas V, Karagiorga M et al (2006) Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood 107:3738–3744CrossRefPubMed Pennell DJ, Berdoukas V, Karagiorga M et al (2006) Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood 107:3738–3744CrossRefPubMed
25.
go back to reference Pennell DJ, Udelson JE, Arai AE, Bozkurt B, Cohen AR, Galanello R, Hoffman TM, Kiernan MS, Lerakis S, Piga A, Porter JB, Walker JM, Wood J (2013) Cardiovascular function and treatment in β-Thalassemia major: a consensus statement from the american heart association. Circulation 128(3):281–308CrossRefPubMed Pennell DJ, Udelson JE, Arai AE, Bozkurt B, Cohen AR, Galanello R, Hoffman TM, Kiernan MS, Lerakis S, Piga A, Porter JB, Walker JM, Wood J (2013) Cardiovascular function and treatment in β-Thalassemia major: a consensus statement from the american heart association. Circulation 128(3):281–308CrossRefPubMed
26.
go back to reference Shekerdemian LS, Bush A, Lincoln C, Shore DF, Petros AJ, Redington AN (1997) Cardiopulmonary interactions in healthy children and children after simple cardiac surgery: the effects of positive and negative pressure ventilation. Heart 78:587–593CrossRefPubMedCentralPubMed Shekerdemian LS, Bush A, Lincoln C, Shore DF, Petros AJ, Redington AN (1997) Cardiopulmonary interactions in healthy children and children after simple cardiac surgery: the effects of positive and negative pressure ventilation. Heart 78:587–593CrossRefPubMedCentralPubMed
27.
go back to reference Sohn DW, Chai IH, Lee DJ, Kim HC, Oh Bh, Lee MM et al (1997) Assessment of mitral annulus velocity by Doppler tissue imaging in evaluation of left ventricular diastolic function. J Am Coll Cardiol 30:474–480CrossRefPubMed Sohn DW, Chai IH, Lee DJ, Kim HC, Oh Bh, Lee MM et al (1997) Assessment of mitral annulus velocity by Doppler tissue imaging in evaluation of left ventricular diastolic function. J Am Coll Cardiol 30:474–480CrossRefPubMed
28.
go back to reference Abott AXSYM System (1995) Eur J Clin Chem Clin Biochem 33:105–111 Abott AXSYM System (1995) Eur J Clin Chem Clin Biochem 33:105–111
29.
go back to reference Tei C, Ling LH, Hodge DO, Bailey KR, Oh JK, Rodeheffer RJ, Tajik AJ, Seward JB (1995) New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function–a study in normals and dilated cardiomyopathy. J Cardiol 26:357–366PubMed Tei C, Ling LH, Hodge DO, Bailey KR, Oh JK, Rodeheffer RJ, Tajik AJ, Seward JB (1995) New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function–a study in normals and dilated cardiomyopathy. J Cardiol 26:357–366PubMed
30.
go back to reference Teichholz LE, Kreulen T, Herman MV, Gorlin R (1976) Problems in echocardiographic volume determinations: echocardiography angiographic correlations in the presence or absence of asynergy. Am J Cardiol 37:7e11 Teichholz LE, Kreulen T, Herman MV, Gorlin R (1976) Problems in echocardiographic volume determinations: echocardiography angiographic correlations in the presence or absence of asynergy. Am J Cardiol 37:7e11
31.
go back to reference Telfer PT, Prestcott E, Holden S, Walker M, Hoffbrand AV, Wonke B (2000) Hepatic iron concentration combined with long-term monitoring of serum ferritin to predict complications of iron overload in thalassaemia major. Br J Haematol 110:971–977CrossRefPubMed Telfer PT, Prestcott E, Holden S, Walker M, Hoffbrand AV, Wonke B (2000) Hepatic iron concentration combined with long-term monitoring of serum ferritin to predict complications of iron overload in thalassaemia major. Br J Haematol 110:971–977CrossRefPubMed
32.
go back to reference Uçar T, Ileri T, Atalay S, Uysal Z, Tutar E, Ertem M (2009) Early detection of myocardial dysfunction in children with beta-thalassaemia major. Int J Cardiovasc Imaging 25(4):379–386CrossRefPubMed Uçar T, Ileri T, Atalay S, Uysal Z, Tutar E, Ertem M (2009) Early detection of myocardial dysfunction in children with beta-thalassaemia major. Int J Cardiovasc Imaging 25(4):379–386CrossRefPubMed
33.
go back to reference Vaccari M, Crepaz R, Fortini M, Gamberini MR, Scarcia S, Pitscheider W (2002) Left ventricular remodeling, systolic function, and diastolic function in young adults with beta thalassemia intermedia. Chest 121:506e12CrossRef Vaccari M, Crepaz R, Fortini M, Gamberini MR, Scarcia S, Pitscheider W (2002) Left ventricular remodeling, systolic function, and diastolic function in young adults with beta thalassemia intermedia. Chest 121:506e12CrossRef
34.
go back to reference Vogel M, Anderson LJ, Holden S, Deanfield JE, Pennel DJ, Walker JM (2003) Tissue doppler echocardiography in patients with beta thalassemia detects early myocardial dysfunction related to myocardial iron overload. Eur Heart J 24:113–119CrossRefPubMed Vogel M, Anderson LJ, Holden S, Deanfield JE, Pennel DJ, Walker JM (2003) Tissue doppler echocardiography in patients with beta thalassemia detects early myocardial dysfunction related to myocardial iron overload. Eur Heart J 24:113–119CrossRefPubMed
35.
go back to reference Westwood M, Anderson LJ, Firmin DN, Gatehouse PD, Charrier CC, Wonke B, Pennell DJ (2003) A single breath-hold multiecho T2* cardiovascular magnetic resonance technique for diagnosis of myocardial iron overload. J Magn Reson Imaging 18:33–39CrossRefPubMed Westwood M, Anderson LJ, Firmin DN, Gatehouse PD, Charrier CC, Wonke B, Pennell DJ (2003) A single breath-hold multiecho T2* cardiovascular magnetic resonance technique for diagnosis of myocardial iron overload. J Magn Reson Imaging 18:33–39CrossRefPubMed
36.
go back to reference Wood JC, Tyszka JM, Carson S, Nelson MD, Coates TD (2004) Myocardial iron loading in transfusion-dependent thalassemia and sickle cell disease. Blood 103:1934–1936CrossRefPubMed Wood JC, Tyszka JM, Carson S, Nelson MD, Coates TD (2004) Myocardial iron loading in transfusion-dependent thalassemia and sickle cell disease. Blood 103:1934–1936CrossRefPubMed
37.
go back to reference Wood JC, Origa R, Agus A, Matta G, Coates TD, Galanello R (2008) Onset of cardiac iron loading in pediatric patients with thalassemia major. Haematologica 93:917–920CrossRefPubMedCentralPubMed Wood JC, Origa R, Agus A, Matta G, Coates TD, Galanello R (2008) Onset of cardiac iron loading in pediatric patients with thalassemia major. Haematologica 93:917–920CrossRefPubMedCentralPubMed
38.
go back to reference Yip G, Abraham T, Belohlavek M, Chandheria BK (2003) Clinical application of strain rate imaging. J Am Soc Echocardiogr 16:1334–1342CrossRefPubMed Yip G, Abraham T, Belohlavek M, Chandheria BK (2003) Clinical application of strain rate imaging. J Am Soc Echocardiogr 16:1334–1342CrossRefPubMed
Metadata
Title
Early Detection of Right Ventricular Diastolic Dysfunction by Pulsed Tissue Doppler Echocardiography in Iron Loaded Beta Thalassemia Patients
Authors
Hala Mounir Agha
Amal Beshlawy
Mona Hamdy
Alae Sobeih
Fatma El Zahrae
Inas Abd El Satar
Antoine AbdelMassih
Fadwa Said
Ossama Abd El Aziz
Mona El Tagui
Dudley J. Pennell
Publication date
01-03-2015
Publisher
Springer US
Published in
Pediatric Cardiology / Issue 3/2015
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-014-1035-y

Other articles of this Issue 3/2015

Pediatric Cardiology 3/2015 Go to the issue