title: Risk factors and mortality associated with an elevated tricuspid regurgitant jet velocity measured by Doppler-echocardiography in thalassemia: a Thalassemia Clinical Research Network report.
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.
authors.affiliation: Department of Emergency Medicine, Children's Hospital & Research Center Oakland, Oakland, CA, USA. claudiamorris@comcast.net
authors.forename: Claudia R
authors.initials: CR
authors.lastname: Morris
authors.forename: Hae-Young
authors.initials: HY
authors.lastname: Kim
authors.forename: Felicia
authors.initials: F
authors.lastname: Trachtenberg
authors.forename: John
authors.initials: J
authors.lastname: Wood
authors.forename: Charles T
authors.initials: CT
authors.lastname: Quinn
authors.forename: Nancy
authors.initials: N
authors.lastname: Sweeters
authors.forename: Janet L
authors.initials: JL
authors.lastname: Kwiatkowski
authors.forename: Alexis A
authors.initials: AA
authors.lastname: Thompson
authors.forename: Patricia J
authors.initials: PJ
authors.lastname: Giardina
authors.forename: Jeanne
authors.initials: J
authors.lastname: Boudreaux
authors.forename: Nancy F
authors.initials: NF
authors.lastname: Olivieri
authors.forename: John B
authors.initials: JB
authors.lastname: Porter
authors.forename: Ellis J
authors.initials: EJ
authors.lastname: Neufeld
authors.forename: Elliott P
authors.initials: EP
authors.lastname: Vichinsky
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012307:Risk Factors; D013789:Thalassemia / Q000150:complications* / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000401:mortality; D014262:Tricuspid Valve Insufficiency / Q000150:complications* / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000401:mortality; D055815:Young Adult