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G56(P) The frequency of pulmonary hypertension in nontransfusion dependent thalassemias
  1. AB Batool
  1. Paediatric Department, Sir Ganga Ram Hospital, Lahore, Pakistan


Objective The aim of study was to determine the frequency of pulmonary hypertension in non transfusion dependent thalassemias (NTDT).

Material and Methods This crossectional study was conducted from 01–02-2016 to 31–07-2016. One hundred and seventy patients of NTDT of age 5–12 years and of both gender. The NTDT patients with congestive cardiac failure having ejection fraction <60% on echocardiography, spleenectomy and who are shifted to regular transfusion regimen were excluded. After taking informed consent, pulmonary hypertension in form of tricuspid-valve regurgitant jet velocity (TRV) was measured via echocardiography in the same hospital, while their identity was kept confidential. The demographic information of patients like name, age, gender were collected. Pulmonary hypertension was labelled when TRV was equal or greater than three metre per second on echocardiography in absence of congestive cardiac failure.

Results Out of 170 patients 122 were male and 48 female. Age group was 5 to 12 years with mean of 8.5 years. Diagnosis in order of frequency was thalasemia intermedia 128 (75%), HbE 23 (13.5%), HbS 10 (5.9%), HbH 8 (4.7%) and Hb alpha 1 (0.6%). 11 patients (6.5%) had evidence of pulmonary hypertension. Out of them 3 had age of 12 years and 8 were male. Among patients with pulmonary hypertension 7 had thalassemia intermedia, 2 hbs, 1 hbe and 1 hbh.

Conclusion Evidence of pulmonary hypertension was found in 6.5 percent of patients and majority (7 of them) had thalassemia intermedia. It was more pravelent In males. Study confirmed that pulmonary hypertension in b-thalassemia patients including NTDT is a serious morbidity associated with subsequent right ventricular dysfunction, warranting prompt intervention. No local study was available about the magnitude of pulmonary hypertension in NTDT. This complication can be prevented or treated timely by incorporating the TRV in routine protocols and follow ups.

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