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. 2011 Oct 6;118(14):3794-802.
doi: 10.1182/blood-2010-11-319152. Epub 2011 Jul 19.

Risk factors and mortality associated with an elevated tricuspid regurgitant jet velocity measured by Doppler-echocardiography in thalassemia: a Thalassemia Clinical Research Network report

Claudia R Morris  1 , Hae-Young Kim, Felicia Trachtenberg, John Wood, Charles T Quinn, Nancy Sweeters, Janet L Kwiatkowski, Alexis A Thompson, Patricia J Giardina, Jeanne Boudreaux, Nancy F Olivieri, John B Porter, Ellis J Neufeld, Elliott P Vichinsky; Thalassemia Clinical Research Network
Collaborators, Affiliations

Risk factors and mortality associated with an elevated tricuspid regurgitant jet velocity measured by Doppler-echocardiography in thalassemia: a Thalassemia Clinical Research Network report

Claudia R Morris et al. Blood. .

Abstract

An elevated tricuspid regurgitant jet velocity (TRV) is associated with hemolysis and early mortality in sickle cell disease, yet risk factors, clinical parameters, and mortality associated with this biomarker in thalassemia are poorly defined. This report summarizes the prevalence of an elevated TRV in 325 patients screened by Doppler echocardiography in the Thalassemia Clinical Research Network. A documented TRV was reported in 148 of 325 (46%) of patients. Average age was 25.9 years (range, 5-56 years) and 97% were transfusion-dependent. Mean TRV was 2.3 ± 0.4 m/s (range, 0.2-3.5 m/s). An abnormal TRV ≥ 2.5 m/s was identified in 49 of 148 (33%) of patients with a documented TRV, 5% (8/148), with a TRV ≥ 3.0 m/s, suggesting significant PH risk. Older age was strongly associated with a high TRV; however, 16% of children had a TRV ≥ 2.5 m/s. A history of splenectomy, hepatitis C, smoking, or high white blood cell count was associated with TRV elevation. In summary, an elevated TRV is noted in one-third of transfusion-dependent thalassemia patients with a documented value and develops in both children and adults. Age, splenectomy, hepatitis C, and smoking are significant univariate risk factors, with splenectomy surfacing as the dominant risk factor over time. Mortality was low in this cohort. Prospective longitudinal studies are needed. This study is registered at http://www.clinicaltrials.gov as NCT00661804.

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Figures

Figure 1
Figure 1
TRV in m/s. Left, adults > 18 years of age with thalassemia (n = 103); right, children with thalassemia < 18 years of age (n = 45). Filled circles represent patients with a TRV ≥ 2.5 m/s; filled black circles reflect patients with a TRV ≥ 3.0 m/s, and filled gray circles represent patients with a TRV between 2.5 and 2.9 m/s. Unfilled circles represent patients with a TRV < 2.5 m/s.
Figure 2
Figure 2
Prevalence of high TRV on Doppler echocardiography in the thalassemia cohort by age. Specific prevalence of a TRV ≥ 2.5 m/s for each age group is demonstrated, with the actual number of patients with a TRV ≥ 2.5 m/s listed above each age category. The greatest prevalence of TRV elevation occurs in the 41- to 50-year age group of the TCRN cohort.
Figure 3
Figure 3
Risk of high TRV in patients with splenectomy compared with those without splenectomy by age of patients (years). Age is a risk factor for TRV ≥ 2.5 m/s only in patients with splenectomy.
Figure 4
Figure 4
Risk of high TRV developing over years from splenectomy. The risk of developing pulmonary hypertension increases over time after splenectomy.
See this image and copyright information in PMC

Comment in

  • Guilt by association.
    Nathan DG. Nathan DG. Blood. 2011 Oct 6;118(14):3758-9. doi: 10.1182/blood-2011-08-370338. Blood. 2011. PMID: 21980046 No abstract available.

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