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, 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, Ellis Neufeld, Jennifer Braunstein, Amber Smith, Latoya Lashley, Charles Quinn, Deborah Boger, Leah Adix, Sandra Richardson, Jeanne Boudreaux, Leann Hassen, Brigitta Mueller, Patricia Giardina, Elizabeth Evans, Mark Weinblatt, Linda Skelly, Janet L Kwiatkowski, Alan Cohen, Marie Martin, Owen Beams, Sage Green, Alexis Thompson, Janice Beatty, Tiffany Drinkwater, Elliott Vichinsky, Dru Foote, Nancy Sweeters, Olivia Vega, Thomas Coates, Susan Carson, Eun Ha Pang, Rachna Khanna, Michael Jeng, Kokil Bakshi, John Wu, Heather McCartney, Colleen Fitzgerald, Stephanie Badour, Nancy F Olivieri, Vivek Thayalasuthan, Isaac Odame, Manuela Merelles-Pulcini, John Porter, Cindy Bhagwandin, Farrukh Shah, Kathryn Hassell, Sonja McKinlay, Lisa Virzi, Felicia Trachtenberg, Claudia R Morris, 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, Ellis Neufeld, Jennifer Braunstein, Amber Smith, Latoya Lashley, Charles Quinn, Deborah Boger, Leah Adix, Sandra Richardson, Jeanne Boudreaux, Leann Hassen, Brigitta Mueller, Patricia Giardina, Elizabeth Evans, Mark Weinblatt, Linda Skelly, Janet L Kwiatkowski, Alan Cohen, Marie Martin, Owen Beams, Sage Green, Alexis Thompson, Janice Beatty, Tiffany Drinkwater, Elliott Vichinsky, Dru Foote, Nancy Sweeters, Olivia Vega, Thomas Coates, Susan Carson, Eun Ha Pang, Rachna Khanna, Michael Jeng, Kokil Bakshi, John Wu, Heather McCartney, Colleen Fitzgerald, Stephanie Badour, Nancy F Olivieri, Vivek Thayalasuthan, Isaac Odame, Manuela Merelles-Pulcini, John Porter, Cindy Bhagwandin, Farrukh Shah, Kathryn Hassell, Sonja McKinlay, Lisa Virzi, Felicia Trachtenberg
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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Source: PubMed