Pulmonary Hypertension and Right Heart Failure in Patients With β-Thalassemia Intermedia

doi:10.1378/chest.107.1.50

Chest

Volume 107, Issue 1, January 1995, Pages 50-53

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We analyzed seven patients with β -thalassemia intermedia presenting with congestive heart failure secondary to pulmonary hypertension. This condition has been recognized only recently as part of the clinical spectrum of/2-thalassemia. Our group of patients included two men and five women with the clinical picture and laboratory data typical of β -thalassemia intermedia. The mean age was 37.7±11.4 years, mean hematocrit value was 28.5±1.8%, mean number of transfused blood units was 171±153, and mean serum ferritin levels were 4,428±2,006 ng/mL. All but one of these patients had undergone splenectomy. Common findings of the investigative procedures include the following: dilatation of the main pulmonary artery and cardiac enlargement in the chest radiograph; signs of right ventricular hypertrophy in the ECG; and dilated right ventricle with good left ventricular function in the echo study. Right heart catheterization showed the pulmonary systolic pressure to range from 55 to 90 mm Hg (74.1±10.3), pulmonary diastolic pressure from 25 to 50 mm Hg (37.7±8.7), mean pressure from 35 to 60 mm Hg (49.7±7.9), and pulmonary vascular resistance from 267 to 667 dynes • s • cm⁻⁵. Pulmonary capillary wedge pressure was within the normal range of values. The pathophysiologic condition of pulmonary hypertension in these patients is most probably associated with β -thalassemia. There are mechanisms that increase cardiac output and at the same time restrict the pulmonary vascular bed. The results of this study imply that treatment decisions should be reconsidered for such patients.

Section snippets

Materials and Methods

We investigated the clinical and laboratory data of seven patients with β -thal intermedia who developed right heart failure and pulmonary hypertension. This group is a part of the group of
patients with β -thal who are receiving regular follow-up at the cardiology outpatient clinic, a service provided by the First Department of Medicine, University Hospital of Athens (Greece).
The patients, two men and five women, ranged in age from 26 to 62 years (Table 1). All of them had commenced blood

Results

Signs and symptoms of congestive heart failure were present in all: fatigue, dyspnea, peripheral edema mainly in the feet and ankles, prominent jugular veins, and profound liver enlargement (about 12 cm below the right costal margin). The nonsplenectomized individual had significant splenomegaly (15 cm below the left costal margin). All patients had warm extremities. Heart examination revealed vivid parasternal impulse, gallop rhythm, loud second heart sound over the pulmonary valve, and an

Discussion

Cardiovascular complications, and most notably congestive heart failure resulting from dilatation and decreased left ventricular function, are the most common cause of death in patients with β -thal.³ Pulmonary hypertension is a clinical picture less commonly reported, although our preliminary findings and some evidence from the literature⁴ suggest that it requires further investigation.

The wide spectrum of clinical manifestations of β -thal mostly depend on the following two general factors:

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Chest

Clinical Investigations: Cardiology: ArticlesPulmonary Hypertension and Right Heart Failure in Patients With β-Thalassemia Intermedia

Section snippets

Materials and Methods

Results

Discussion

Blood

Lancet

Chest

J Am Coll Cardiol

The thalassemia syndromes: molecular basis and prenatal diagnosis in 1990

Semin Hematol

Restrictive diastolic abnormalities identified by Doppler echocardiography in patients with thalassemia major

Circulation

Clinical Investigations: Cardiology: Articles
Pulmonary Hypertension and Right Heart Failure in Patients With β-Thalassemia Intermedia