|
|
 Previous Article | Table of Contents | Next Article 
Blood, 1 June 2001, Vol. 97, No. 11, pp. 3411-3416
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Cardiac involvement in thalassemia intermedia: a
multicenter study
Athanasios Aessopos,
Dimitrios Farmakis,
Markisia Karagiorga,
Ersi Voskaridou,
Aphrodite Loutradi,
Antonia Hatziliami,
Jacqueline Joussef,
John Rombos, and
Dimitris Loukopoulos
From the First Department of Internal Medicine,
University of Athens School of Medicine, and the Thalassemia Unit,
Laiko General Hospital; and the Thalassemia Unit, Aghia Sophia
Children's Hospital, Athens, Greece.
Cardiac complications in 110 patients (mean age, 32.5 ± 11.4
years) with thalassemia intermedia (TI) were studied. Sixty-seven (60.9%) of them had not been transfused or were minimally transfused (group A). The rest had started transfusions after the age of 5 years
(mean, 15.1 ± 10.1 years), initially on demand and later more
frequently (group B). Overall mean hemoglobin and ferritin levels were
9.1 ± 1.1 g/dL and 1657 ± 1477 ng/mL, respectively. Seventy-six
healthy controls were also studied. The investigation included thorough
history taking, clinical examination, electrocardiography, chest
radiograph, and full resting echocardiography. Of 110 patients, 6 (5.4%) had congestive heart failure (CHF), and 9 (8.1%) had a history
of acute pericarditis. Echocardiography showed pericardial thickening,
with or without effusion, in 34.5% of the patients. Valvular
involvement included leaflet thickening (48.1%), endocardial calcification (20.9%), and left-sided valve regurgitation (aortic, 15.4%; mitral, 47.2%). All patients had normal left ventricular contractility (fractional shortening, 0.43 ± 0.05), and high cardiac output (CO; 9.34 ± 2.28 L/min). Pulmonary hypertension (PHT), defined as Doppler peak systolic tricuspid gradient greater than 30 mm
Hg, developed in 65 patients (59.1%). PHT correlated positively with
age and CO and did not differ significantly between groups. Cardiac
catheterization in the 6 patients with CHF revealed severe PHT,
increased pulmonary resistance (PVR), and normal capillary wedge
pressure. It was concluded that in patients with TI, the heart is
primarily affected by PHT, which is the leading cause of CHF. High CO
resulting from chronic tissue hypoxia and increased PVR are the main
contributing factors. Doppler tricuspid gradient measurement should be
considered, in addition to other factors, when determining the value of
transfusion therapy for patients with TI.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
V. V. McLaughlin, S. L. Archer, D. B. Badesch, R. J. Barst, H. W. Farber, J. R. Lindner, M. A. Mathier, M. D. McGoon, M. H. Park, R. S. Rosenson, et al.
ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association
J. Am. Coll. Cardiol.,
April 28, 2009;
53(17):
1573 - 1619.
[Full Text]
[PDF]
|
|
|
|
|
|
|
Writing Committee Members, V. V. McLaughlin, S. L. Archer, D. B. Badesch, R. J. Barst, H. W. Farber, J. R. Lindner, M. A. Mathier, M. D. McGoon, M. H. Park, et al.
ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: Developed in Collaboration With the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association
Circulation,
April 28, 2009;
119(16):
2250 - 2294.
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. P. Minniti, C. Sable, A. Campbell, S. Rana, G. Ensing, N. Dham, O. Onyekwere, M. Nouraie, G. J. Kato, M. T. Gladwin, et al.
Elevated tricuspid regurgitant jet velocity in children and adolescents with sickle cell disease: association with hemolysis and hemoglobin oxygen desaturation
Haematologica,
March 1, 2009;
94(3):
340 - 347.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. T. Gladwin and E. Vichinsky
Pulmonary Complications of Sickle Cell Disease
N. Engl. J. Med.,
November 20, 2008;
359(21):
2254 - 2265.
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. D. Pashankar, J. Carbonella, A. Bazzy-Asaad, and A. Friedman
Prevalence and Risk Factors of Elevated Pulmonary Artery Pressures in Children With Sickle Cell Disease
Pediatrics,
April 1, 2008;
121(4):
777 - 782.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Anthi, R. F. Machado, M. L. Jison, A. M. Taveira-DaSilva, L. J. Rubin, L. Hunter, C. J. Hunter, W. Coles, J. Nichols, N. A. Avila, et al.
Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension
Am. J. Respir. Crit. Care Med.,
June 15, 2007;
175(12):
1272 - 1279.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Voskaridou, G. Tsetsos, A. Tsoutsias, E. Spyropoulou, D. Christoulas, and E. Terpos
Pulmonary hypertension in patients with sickle cell/{beta} thalassemia: incidence and correlation with serum N-terminal pro-brain natriuretic peptide concentrations
Haematologica,
June 1, 2007;
92(6):
738 - 743.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Aessopos, M. Kati, and D. Farmakis
Heart disease in thalassemia intermedia: a review of the underlying pathophysiology
Haematologica,
May 1, 2007;
92(5):
658 - 665.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A Phrommintikul, A Sukonthasarn, R Kanjanavanit, and W Nawarawong
Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia
Heart,
October 1, 2006;
92(10):
1467 - 1472.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. F. Machado, A. Anthi, M. H. Steinberg, D. Bonds, V. Sachdev, G. J. Kato, A. M. Taveira-DaSilva, S. K. Ballas, W. Blackwelder, X. Xu, et al.
N-terminal pro-brain natriuretic peptide levels and risk of death in sickle cell disease.
JAMA,
July 19, 2006;
296(3):
310 - 318.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Farmakis, A. Polonifi, S. Deftereos, M. Tsironi, I. Papaioannou, and A. Aessopos
Aortic Valve Replacement in a Patient With Thalassemia Intermedia
Ann. Thorac. Surg.,
February 1, 2006;
81(2):
737 - 739.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A J Peacock
Pulmonary hypertension after splenectomy: a consequence of loss of the splenic filter or is there something more?
Thorax,
December 1, 2005;
60(12):
983 - 984.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Aessopos, D. Farmakis, S. Deftereos, M. Tsironi, S. Tassiopoulos, I. Moyssakis, and M. Karagiorga
Thalassemia Heart Disease: A Comparative Evaluation of Thalassemia Major and Thalassemia Intermedia
Chest,
May 1, 2005;
127(5):
1523 - 1530.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. P. Rother, L. Bell, P. Hillmen, and M. T. Gladwin
The Clinical Sequelae of Intravascular Hemolysis and Extracellular Plasma Hemoglobin: A Novel Mechanism of Human Disease
JAMA,
April 6, 2005;
293(13):
1653 - 1662.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. T. Gladwin and G. J. Kato
Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia
Hematology,
January 1, 2005;
2005(1):
51 - 57.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
V. Atichartakarn, S. Chuncharunee, P. Chandanamattha, K. Likittanasombat, and K. Aryurachai
Correction of hypercoagulability and amelioration of pulmonary arterial hypertension by chronic blood transfusion in an asplenic hemoglobin E/{beta}-thalassemia patient
Blood,
April 1, 2004;
103(7):
2844 - 2846.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. T. Gladwin, V. Sachdev, M. L. Jison, Y. Shizukuda, J. F. Plehn, K. Minter, B. Brown, W. A. Coles, J. S. Nichols, I. Ernst, et al.
Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease
N. Engl. J. Med.,
February 26, 2004;
350(9):
886 - 895.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. R. Cohen, R. Galanello, D. J. Pennell, M. J. Cunningham, and E. Vichinsky
Thalassemia
Hematology,
January 1, 2004;
2004(1):
14 - 34.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. L. Jison and M. T. Gladwin
Hemolytic Anemia-associated Pulmonary Hypertension of Sickle Cell Disease and the Nitric Oxide/Arginine Pathway
Am. J. Respir. Crit. Care Med.,
July 1, 2003;
168(1):
3 - 4.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Parsi and F. X. Kleber
Anaemia in heart failure: its diagnosis and management
Eur J Heart Fail,
January 1, 2003;
5(1):
3 - 4.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Littera, G. La Nasa, G. Derchi, M. D. Cappellini, C. Y. P. Chang, and L. Contu
Long-term treatment with oral sildenafil in a thalassemic patient with pulmonary hypertension
Blood,
July 30, 2002;
100(4):
1516 - 1517.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Aessopos, D. Farmakis, and D. Loukopoulos
Elastic tissue abnormalities resembling pseudoxanthoma elasticum in beta thalassemia and the sickling syndromes
Blood,
January 1, 2002;
99(1):
30 - 35.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. R. MINTER and M. T. GLADWIN
Pulmonary Complications of Sickle Cell Anemia . A Need for Increased Recognition, Treatment, and Research
Am. J. Respir. Crit. Care Med.,
December 1, 2001;
164(11):
2016 - 2019.
[Full Text]
[PDF]
|
|
|
|
|
