Pulmonary hypertension in well-transfused thalassemia major patients.
Blood Cells Mol Dis
; 54(2): 189-94, 2015 Feb.
Article
em En
| MEDLINE
| ID: mdl-25488617
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.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Insuficiência da Valva Tricúspide
/
Talassemia beta
/
Transfusão de Eritrócitos
/
Sobrecarga de Ferro
/
Hipertensão Pulmonar
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Adult
/
Female
/
Humans
/
Male
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article