[Cardiac involvement in mucopolysaccharidosis. Apropos of an echocardiographic study in 8 cases].
Of all the storage diseases, mucopolysaccharidosis is the one whose cardiac manifestations are probably the least well known. Clinical and above all echocardiographic findings of heart involvement were studied in 8 patients with mucopolysaccharidosis, including four with Hunter disease. The paucity of clinical manifestations was in sharp contrast with the highly informative echocardiographic results. Valvular dystrophy, usually of the left side of the heart, was the most common anomaly, with five patients affected. Whereas some valvular lesions had no consequences, others led to stenosis or incompetence. Asymmetrical hypertrophy of the septum was found in one patient. No patient had evidence suggestive of vascular involvement, in particular of the coronary arteries.
Ata
J
J
Service de Cardiologie, CHU de Sousse.
Brigui
M
M
Jerad
T
T
Belkhiria
N
N
Yacoub
M
M
Harbi
A
A
Essoussi
A S
AS
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D015150:Echocardiography, Doppler; D005260:Female; D006331:Heart Diseases / Q000000981:diagnostic imaging*; D006801:Humans; D008297:Male; D009083:Mucopolysaccharidoses / Q000000981:diagnostic imaging*; D008059:Mucopolysaccharidosis I / Q000000981:diagnostic imaging; D016532:Mucopolysaccharidosis II / Q000000981:diagnostic imaging; D009084:Mucopolysaccharidosis III / Q000000981:diagnostic imaging
L'atteinte cardiaque dans la mucopolysaccharidose. A propos d'une étude échocardiographique de huit cas.