Search PubMed⌕ Search

Biomedical subjects

T Sakamoto

Publications and source records attributed to T Sakamoto.

At least 1,135 records · Page 63Linked to original sources

[Echocardiographic manifestations of the heart in the Hunter syndrome: report of a case].

Cardiac involvement of mucopolysaccharidosis has not been well characterized by echocardiography. In this paper, we reported a case of Hunter syndrome with special reference to the noninvasive diagnosis of cardiac anomalies. A 21-year-old male of Hunter syndrome was referred to our noninvasive laboratory for the evaluation of his heart murmurs. He was strikingly dwarfed, 115 cm in height and 28 kg in weight, and had apparently Gargoyle-like facial appearance. Physical examination disclosed a mild funnel chest and a loud systolic murmur. Blood pressure was 98/56 mmHg and regular pulse rate was 100/min. The chest X-ray film revealed a shift of the heart to the left with a prominent pulmonary artery segment. There were no signs of pulmonary congestion. The electrocardiogram demonstrated right axis deviation, clockwise rotation and left atrial overload. In phonocardiograms, a basal ejection and an apical scratchy systolic murmurs were recorded. The latter was associated with a presystolic murmur and a loud first heart sound. A loud pulmonary second heart sound was also present. Outstanding findings were observed in the two-dimensional and M-mode echocardiograms, which showed remarkable thickening of both mitral valve leaflets with reduced opening. There was also generalized thickening of tricuspid and aortic valves, and endocardium of the free ventricular wall. Two leaflets of the tricuspid valve were visualized to prolapse, but the aortic valve motion appeared intact. In addition, echocardiograms revealed the dilatation of right-sided cardiac chambers and pulmonary artery, but the size of the left ventricle was rather small. Pulsed Doppler echocardiography demonstrated systolic turbulence in the right atrium in the vicinity of the tricuspid valve orifice. Stenotic turbulence was also recorded in the inflow tract of the left ventricle during diastole. Thus, the final diagnosis of predominant mitral stenosis associated with tricuspid valve prolapse and pulmonary hypertension was obtained. In conclusion, cardiac involvements in Hunter syndrome were precisely evaluated by noninvasive methods. Hunter syndrome may be considered as one of the etiologies producing mitral stenosis.

Adult↗

[Noninvasive evaluation of cardiac involvements and left ventricular function in schoolchildren with the history of mucocutaneous lymph node syndrome].

Mucocutaneous lymph node syndrome (MCLS) in the young has been known to have coronary aneurysms, and then it has been recently suspected as a cause of premature atherosclerosis and cardiomyopathy. Thirty-three schoolchildren who suffered from MCLS were studied to evaluate cardiac involvements and left ventricular function using two-dimensional (2-D) echocardiograms and submaximal stress test. Fifteen normal schoolchildren were studied as normal control. All these MCLS children were asymptomatic and had no significant findings in routine chest X-ray and electrocardiographic examinations. According to submaximal stress test, 8 cases showed a J type ST depression of only 0.5--1.0 mm, and there were no positive cases. Using 2-D echocardiograms, the left coronary artery was detected in 85% and the right coronary in 27%. One case showed an aneurysm of the left coronary artery. However, none of them showed abnormal left ventricular wall motion or the wall motion abnormality compatible with cardiomyopathy. There were no significant differences between MCLS and normal control in ejection fraction, mean VCF, diastolic descent rate of the anterior mitral valve, D/S ratio of the left ventricular wall, and Weissler's index (PEP/ET). These findings suggested that 1) most of MCLS schoolchildren do not have obvious cardiac involvement and their left ventricular function is within normal limits, 2) because of its low sensitivity, submaximal stress test is not so useful in screening coronary lesions, and 3) the 2-D echocardiogram works not only in detecting coronary aneurysms but also in evaluating left ventricular function.

Adolescent↗