title: Pulmonary hypertension in well-transfused thalassemia major patients.
abstract: The risk for pulmonary hypertension (PH) in thalassemia major (TM) patients remains controversial. We report echocardiography results from 60 TM patients: we evaluated the association between tricuspid regurgitation velocities (TRV), iron stores, and serologic markers of hemolysis and arginine dysregulation. Patients were enrolled from August 2004 until May 2009. All parameters were inversely weighted by the number of exams. TRV was comparable between sexes and it was uncorrelated with age. At the first exam, TR velocities at the upper limits of normal (2.5-2.7m/s) were observed in 8 patients. An abnormal TRV (2.9m/s) was found in 1 patient. Borderline increases in TRV were associated with a reduced global arginine bioavailability (R=-0.399 P=0.005), increased anemia (hemoglobin: R=-0.219 P=0.0461), cardiac index (R=0.223 P=0.0481), and diastolic dysfunction (E/A: R=0.289 P=0.0088; E/E': R=0.223 P=0.0453), but not hemolysis, iron overload and systolic function evaluated by Magnetic Resonance Imaging, and splenectomy. Well-transfused TM patients have a lower risk for PH than thalassemia intermedia patients. However, they do have vascular stressors that raise their lifetime PH risk to levels higher than for the general population. Consequently, we support recommendations for annual echocardiographic screening and cardiac catheterization for persistent TRV above 3m/s.
authors.affiliation: CMR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy; Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Antonella
authors.initials: A
authors.lastname: Meloni
authors.affiliation: Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Jon
authors.initials: J
authors.lastname: Detterich
authors.affiliation: CMR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
authors.forename: Alessia
authors.initials: A
authors.lastname: Pepe
authors.affiliation: Division of Gastroenterology, Children's Hospital Oakland, Oakland, CA, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Harmatz
authors.affiliation: Section of Hematology, Children's Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Tom D
authors.initials: TD
authors.lastname: Coates
authors.affiliation: Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA; Department of Radiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. Electronic address: jwood@chla.usc.edu.
authors.forename: John C
authors.initials: JC
authors.lastname: Wood
mesh_terms: D000293:Adolescent; D000328:Adult; D001120:Arginine / Q000097:blood; D017707:Erythrocyte Transfusion; D005260:Female; D006461:Hemolysis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000517:prevention & control*; D007501:Iron / Q000097:blood; D019190:Iron Overload / Q000097:blood* / Q000000981:diagnostic imaging / Q000628:therapy; D008297:Male; D012307:Risk Factors; D014262:Tricuspid Valve Insufficiency / Q000097:blood* / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy; D014463:Ultrasonography; D017086:beta-Thalassemia / Q000097:blood* / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy
chemical_list: D001120:Arginine; D007501:Iron
keywords: Echocardiography; Risk for pulmonary hypertension; Thalassemia major; Tricuspid regurgitant jet velocity