[Progression to the dilated phase of hypertrophic cardiomyopathy in children].
Twenty-three children with hypertrophic cardiomyopathy (HCM) (aged 2 months to 15 years) were followed up for more than four years using thallium myocardial imaging (TMI) and echocardiography. With echocardiography, the left ventricular end-diastolic dimension (LVDd) and fractional shortening (FS) were measured. Perfusion defect (PD) was assessed using TMI. Cardiac catheterization and right ventricular endomyocardial biopsy were performed in 18 patients within one week before or after their TMI. During the follow-up period, two patients showed a marked increase in LVDd and a marked decrease in FS at ages 13 and 16 years, respectively. These two patients were judged to have progressed to the dilated phase. In these two patients, extensive PD was detected in the left ventricular wall on TMI, 15 and 31 months prior to the appearance of the echocardiographic changes, respectively. Right ventricular endomyocardial biopsy at the time of extensive PD revealed marked interstitial fibrosis along with hypertrophy and disarray of myocardial cells. In conclusion, progression to the dilated phase is not a rare event in children with HCM and TMI appears to be a useful tool for early detection of the progression.
Yoshibayashi
M
M
Department of Pediatrics, Kyoto University School of Medicine.
Ono
Y
Y
Kohata
T
T
Iwatani
H
H
Nogi
S
S
Kamiya
T
T
Nishimura
T
T
Takamiya
M
M
Yutani
C
C
D000293:Adolescent; D001706:Biopsy; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis / Q000473:pathology*; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000473:pathology*; D002648:Child; D004452:Echocardiography; D004719:Endomyocardial Fibrosis / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006321:Heart / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D008297:Male; D009206:Myocardium / Q000473:pathology; D011877:Radionuclide Imaging; D013794:Thallium Radioisotopes
D013794:Thallium Radioisotopes