Elsevier

American Heart Journal

Volume 134, Issue 3, September 1997, Pages 532-537
American Heart Journal

Pulmonary hypertension in patients with thalassemia major,☆☆

https://doi.org/10.1016/S0002-8703(97)70091-7Get rights and content

Abstract

To evaluate the pulmonary artery pressure in patients with thalassemia major, Doppler echocardiography was performed in 33 patients with thalassemia major (aged 2 to 24 years) and 33 normal controls. Pulmonary artery pressure was estimated by (1) measuring the systolic transtricuspid gradient from tricuspid regurgitation and adding it to the right atrial pressure, estimated by the response of the inferior vena cava to deep inspiration, and (2) measuring the time to peak velocity of pulmonary flow. The results showed that of 33 patients, 28 had tricuspid regurgitation with a pulmonary systolic pressure ranging from 18 to 94 mm Hg (47 ± 15 mm Hg). Pulmonary systolic pressure was >30 mm Hg in all 22 patients >10 years old and in four of six patients <10 years old. Pressure correlated with left ventricular ejection fraction ( r = –0.74), the ratio of mitral peak early diastolic flow velocity and peak velocity during the atrial contraction ( r = 0.52), age ( r = 0.56), and total blood transfusion units ( r = 0.59). The pulmonary time to peak velocity was shortened compared with controls ( p < 0.05). We concluded that pulmonary hypertension, as another cardiovascular complication of multiple factors of cause, seems to occur more frequently and at an early stage of the cardiac involvement in patients with thalassemia major. (Am Heart J 1997;134:532-7.)

Section snippets

Subjects

Thirty-three consecutive patients (18 male, 15 female) with β-thalassemia major were studied from December 1994 to December 1995. All patients were regularly monitored in our pediatric hematology clinic. Age varied from 2 to 24 years (mean ± SD 12.1 ± 6.6 years). All but two patients had no cardiac symptoms or clinically apparent heart failure, and none of them were taking cardioactive medications at the time of examination. Each of the patients received blood transfusions approximately every 3

Clinical features

Of 33 patients, 25 had a serum ferritin level >1000 ng/ml, eight had a level <1000 ng/ml; 21 patients had undergone splenectomy; 27 of the 30 patients (three cases did not have liver function test results) had abnormal liver function test results (aspartate aminotransferase >40 U/L, total bilirubin >1 mg/dl, and direct bilirubin >0.2 mg/dl). Only two patients had cardiac symptoms at the time of cardiac evaluation. Cardiac failure developed in three patients 4 months after the examination. Two

Discussion

Impaired left ventricular systolic and diastolic function has been well investigated in patients with thalassemia major. 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12 A few studies have also noticed right ventricular dysfunction in these patients, which has been found to be more prevalent and present earlier than left ventricular dysfunction. 8, 25 Therefore it has been speculated that pulmonary hypertension may be the cause, although without direct evidence. Recently Aessopos et al. 14 measured

Acknowledgements

We thank Drs. Elizabeth Shternberg, Larisa Lukina, and Manhal A. Hanna for their help in collecting clinical data.

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    Reprint requests: Zhong-Dong Du, MD, Division of Cardiology, Capital Institute of Pediatrics & The Children’s Hospital, No. 2, Ya Bao Rd., 100020 Beijing, China. E-mail: [email protected]

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