PULMONARY HYPERTENSION IN -THALASSEMIA MAJOR AND THE ROLE OF L-CARNITINE THERAPY

被引:25
作者
El-Beshlawy, Amal [1 ]
Youssry, Ilham [1 ]
El-Saidi, Sonia [1 ]
El Accaoui, Ramzi [2 ]
Mansi, Yassmin [1 ]
Makhlouf, Ali [1 ]
Taher, Ali [2 ]
机构
[1] Cairo Univ, Dept Pediat, Cairo, Egypt
[2] Amer Univ Beirut, Med Ctr, Dept Internal Med, Beirut, Lebanon
关键词
L-carnitine; echocardiography; pulmonary hypertension; -thalassemia;
D O I
10.1080/08880010802244035
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Cardiac complications, such as pulmonary hypertension (PHT), are the leading cause of death in -thalassemia patients. L-Carnitine, due to its role in fatty acid oxidation, might help control the elevation in pulmonary artery systolic pressure (PASP). The objectives of this study were to assess the prevalence of PHT in -thalassemia major patients, identify clinical predictors for its development, and determine the potential effects of L-carnitine. In total, 32 patients with -thalassemia major were recruited; 16 age- and sex-matched children constituted the control group. Cardiac evaluation was performed by using echocardiography. The patients with PHT received 50 mg/kg/day L-carnitine orally for 3 months and were then reevaluated. Based on PASP, the patients were divided into group A without PHT and group B with PHT. The prevalence of PHT was 37.5%. The other echocardiographic measurements were not significantly different between groups A and B. PASP did not have any significant correlation with the following variables: age, total number of blood units received, splenic status, serum ferritin level, and ejection fraction. Following the administration of L-carnitine, there was a significant decrease in the mean PASP from 33.96 7.85 to 24.11 7.61. All cardiac dimensions decreased following L-carnitine, but the changes were not statistically significant. Even though -thalassemia major resulted in an elevation in the PASP in only a fraction of the patients, it seems to have an impact on the heart dimensions and function of all patients. No clinical predictors were identified. Oral administration of L-carnitine appears to significantly improve PASP.
引用
收藏
页码:734 / 743
页数:10
相关论文
共 26 条
  • [1] Cardiac status in well-treated patients with thalassemia major
    Aessopos, A
    Farmakis, D
    Hatziliami, A
    Fragodimitri, C
    Karabatsos, F
    Joussef, J
    Mitilineou, E
    Diamanti-Kandaraki, E
    Meletis, J
    Karagiorga, M
    [J]. EUROPEAN JOURNAL OF HAEMATOLOGY, 2004, 73 (05) : 359 - 366
  • [2] Cardiac involvement in thalassemia intermedia: a multicenter study
    Aessopos, A
    Farmakis, D
    Karagiorga, M
    Voskaridou, E
    Loutradi, A
    Hatziliami, A
    Joussef, J
    Rombos, J
    Loukopoulos, D
    [J]. BLOOD, 2001, 97 (11) : 3411 - 3416
  • [3] Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease
    Ambrusko, Steven J.
    Gunawardena, Sriya
    Sakara, Allison
    Windsor, Beth
    Lanford, Lizabeth
    Michelson, Peter
    Krishnamurti, Lakshmanan
    [J]. PEDIATRIC BLOOD & CANCER, 2006, 47 (07) : 907 - 913
  • [4] Acute and chronic effects of propinoyl-L-carnitine on the hemodynamics, exercise capacity, and hormones in patients with congestive heart failure
    Anand, I
    Chandrashekhan, Y
    De Giuli, F
    Pasini, E
    Mazzoletti, A
    Confortini, R
    Ferrari, R
    [J]. CARDIOVASCULAR DRUGS AND THERAPY, 1998, 12 (03) : 291 - 299
  • [5] Andren B, 1996, EUR HEART J, V17, P1800
  • [6] COMPARISON OF 3 DOPPLER ULTRASOUND METHODS IN THE PREDICTION OF PULMONARY-ARTERY PRESSURE
    CHAN, KL
    CURRIE, PJ
    SEWARD, JB
    HAGLER, DJ
    MAIR, DD
    TAJIK, AJ
    [J]. JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 1987, 9 (03) : 549 - 554
  • [7] A practical guide to assessment of ventricular diastolic function using Doppler echocardiography
    Cohen, GI
    Pietrolungo, JF
    Thomas, JD
    Klein, AL
    [J]. JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 1996, 27 (07) : 1753 - 1760
  • [8] Myocardial infarction and left ventricular remodeling: Results of the CEDIM trial
    Colonna, P
    Iliceto, S
    [J]. AMERICAN HEART JOURNAL, 2000, 139 (02) : S124 - S130
  • [9] PROPHYLAXIS OF EARLY VENTRICULAR-FIBRILLATION BY INHIBITION OF ACYLCARNITINE ACCUMULATION
    CORR, PB
    CREER, MH
    YAMADA, KA
    SAFFITZ, JE
    SOBEL, BE
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1989, 83 (03) : 927 - 936
  • [10] EVALUATION OF PULMONARY-ARTERY PRESSURE AND RESISTANCE BY PULSED DOPPLER ECHOCARDIOGRAPHY
    DABESTANI, A
    MAHAN, G
    GARDIN, JM
    TAKENAKA, K
    BURN, C
    ALLFIE, A
    HENRY, WL
    [J]. AMERICAN JOURNAL OF CARDIOLOGY, 1987, 59 (06) : 662 - 668