ABSTRACT
Background: Chronic administration of large quantities of blood and inappropriate increase in iron absorption from the gastrointestinal tract inevitably lead to severe hemosiderosis in the whole body organs with impaired functions. Cardiac disorders related to biventricular failure are the most frequent cause of death in patients with beta-thalassemia major. Pulmonary complications in the form of restrictive and obstructive lung disease due to pulmonary hemosiderosis, recurrent heart failure.
Objective: to assess cardiac and pulmonary functions in patients with beta thalassemia major.
Patients & methods: This prospective study was conducted on 25 known - thalassemia major children, 14 males and 11females, their age ranged from 5.4 years to 15 years. They were selected from the pediatric clinic of Benha University Hospital. The study also included 25 apparently healthy children as a control group with matched age and sex during the period from March 2003 to January 2004.
All patients were subjected to thorough history taking , full clinical examination , ECG , echocardiography , and pulmonary function testes.
Results: The mean LVEDD ( left ventricular end diastolic dimension ) among cases was (4.728 ¬+ 0.592) which was significantly higher than among controls (4.0 +0.504) ( p<0.05) .The mean LVESD ( left ventricular end systolic dimension ) among cases was (3.076 +0.58) which was significantly higher than among controls (2.464 + 0.429) (P < 0.01) .The mean LVM(left ventricular mass) among cases (123.68 +13.034) was significantly higher than among controls (97.44 + 10.52) and this difference was highly significant (P < 0.001).The tricuspid E/A ratio was reversed in 5 (20%) of patients indicating right ventricular diastolic dysfunction. The ECG results revealed voltage criteria consistent with the diagnosis of left ventricular hypertrophy in 11 patients (44%), two patients (8%) showed right ventricular hypertrophy ,one patient (4%) showed left atria dilatation, and none of patients showed right atria dilatation. The mean FVC ( forced vital capacity ) among cases was ( 81.68 + 11.025 ) and among controls ( 87.52 + 3.584 ) and this difference was statistically significant (P<0.05). The mean FEV1/FVC (forced expiratory volume in first second / forced vital capacity ) among cases (93.68 + 6.447) was significantly higher than the control group (90.36 + 1.868) (P < 0.05) .
Conclusions: we conclude that patients with B-thalassemia major have abnormal left and right dimensions as well as relaxation abnormalities which may be a reflection of early myocardial damage. This early damage is detectable by a widely applicable echocardiography. Also the present study has shown that restrictive disease and reduced lung diffusing capacity are the predominant abnormalities of pulmonary function due to lung fibrosis .
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