title: Prevalence of Pulmonary Hypertension in Patients with Thalassemia Intermedia in 2009: a single center's experience.
abstract: BACKGROUND
  There are various clinical symptoms of thalassemia intermedia, and they lie roughly between those of major and minor forms of the disease. Patients with thalassemia intermedia occasionally require blood transfusions. This renders them susceptible to pulmonary arterial hypertension (PAH) syndrome, which is one of the most significant complications in patients with thalassemia intermedia. PAH is more common in in thalassemia intermedia than in thalassemia major, and it may cause cardiac complications in patients who are older than 30. The objective of this study was to estimate the prevalence of PAH in thalassemia intermedia patients so that they can be referred expeditiously for treatment, thereby preventing the complications that occur later.
  
  
  METHODS
  This cross sectional study was conducted under the supervision of hematology department of Mashhad Medical University. Forty-one patients with thalassemia intermedia were examined at the Sarvar Thalassemia and Hemophilia Clinic of Mashhad. Electrocardiography, chest radiography, and echocardiography tests were performed for all of the patients by the same pediatric cardiologist. The data were processed by SPSS software, version 11.5, and the results were analyzed using chi-squared, Student's t, and Mann-Whitney tests.
  
  
  RESULTS
  The mean age of the patients was 21.93±8.34. They had been under pediatric heart specialists' constant examination and treatment since their childhood when they were diagnosed with TI, and continue to receive regular follow-up care. The prevalence of pulmonary hypertension was 24% in our study population. In patients with thalassemia intermedia, the left ventricular (LV) mass indices were about 3-5 times higher than would be expected in a normal population. Patients with higher LV mass indices have a greater risk of developing pulmonary hypertension, and those with serum ferritin levels below 1000 ng/ml are less susceptible to diastolic dysfunction.
  
  
  CONCLUSIONS
  Pulmonary hypertension is common in patients with thalassemia intermedia. Irregular chelation therapy or absence of this treatment might lead to diastolic dysfunction, and serum ferritin levels below 1000 ng/ml could be an important factor in preventing the development of diastolic dysfunction or slowing down its progression.
authors.affiliation: Associate Professor of Pediatric Cardiology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
authors.forename: Hassan Mottaghi
authors.initials: HM
authors.lastname: Moghaddam
authors.affiliation: Associate Professor of Pediatric Hematology & Oncology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
authors.forename: Zahra
authors.initials: Z
authors.lastname: Badiei
authors.affiliation: Assistant Professor of Pediatric Gastroenterology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Kambiz
authors.initials: K
authors.lastname: Eftekhari
authors.affiliation: Fellow of Pediatric Cardiology, Modarres Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Reza
authors.initials: R
authors.lastname: Shakeri
authors.affiliation: Assistant Professor of Pediatric Hematology & Oncology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
authors.forename: Hamid
authors.initials: H
authors.lastname: Farhangi
keywords: echocardiography; ferritin; pulmonary hypertension; thalassemia intermedia