Περίληψη:
Background Although acute pericarditis is a common complication of
beta-thalassemia major, the prevalence and consequences of myocarditis
in this disease have not been investigated.
Methods and Results A prospective 5-year follow-up study was carried out
in all patients with beta-thalassemia major in whom the diagnosis of
acute infectious myocarditis could be established between 1977 and 1986.
A similar number of age- and sex-matched control subjects with
beta-thalassemia and normal left ventricular function and no evidence of
myocarditis were also followed for 5 years. Of 1048 patients with
beta-thalassemia major, 47 patients (age, 15 +/- 2.5 years) with
precordial chest pain were diagnosed as having acute infectious
myocarditis. Myocardial biopsy was diagnostic in 26 patients, borderline
in 14 patients, and nondiagnostic in 7 patients. Acute heart failure
with left ventricular dysfunction (left ventricular ejection fraction,
25+/-11%) developed in 11 patients (23.4%) with myocarditis, and 8 of
them died within 1 month to 1 year after diagnosis. Thirteen patients
with myocarditis (27.6%) developed chronic heart failure (left
ventricular ejection fraction, 26+/-13%) within 3+/-1.3 years, and 10
of them died within 8+/-3 months. Left ventricular systolic and
diastolic functions of the control subjects did not change significantly
during the 5-year period (left ventricular ejection fraction, 63+/-11%
versus 65+/-7%; P=NS). However, left ventricular restrictive
abnormalities (early diastole/late diastole, >2.2; deceleration time,
<110 milliseconds) combined with right ventricular dilatation (>30 mm
internal diameter) and right-sided heart failure developed in 3 patients
with extremely high mean serum ferritin levels. No significant
difference was found in mean levels of serum ferritin and pretransfusion
hemoglobin between patients with and those without myocarditis.
Conclusions In patients with beta-thalassemia, myocarditis appears to be
involved in the pathogenesis of left ventricular systolic dysfunction,
being the main cause of death. Iron overload appears to provoke left
ventricular restrictive abnormalities combined with right ventricular
enlargement and dysfunction.
Συγγραφείς:
KREMASTINOS, DT
TINIAKOS, G
THEODORAKIS, GN
KATRITSIS, DG
and TOUTOUZAS, PK
