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Biomedical subjects

S Fuse

Publications and source records attributed to S Fuse.

At least 37 records · Page 2Linked to original sources

Is early surgery always necessary in patients with aortic valve prolapse complicating an Eisenmenger-type ventricular septal defect?

BACKGROUND: The report presents three patients who showed a typically prolapsed aortic cusp with or without aortic regurgitation associated with a malalignment-type perimembranous defect, the so called Eisenmenger-type ventricular septal defect. RESULTS: Each patient developed spontaneous complete or near closure of the ventricular septal defect without worsening of the aortic regurgitation. CONCLUSIONS: These cases suggest that prolapsed aortic cusp complicating a malalignment perimembranous ventricular septal defect is not always an absolute indication for early surgery to prevent progressive aortic regurgitation.

Aortic Valve Insufficiency↗

Stretched minimal diameter of the ductus and coil occlusion.

BACKGROUND: Stretching of the ductus was supposed to determine the size mismatch of the coil with the ductus in coil occlusion that results in residual leak or migration. METHODS: We measured the minimal diameter (D) and the stretched minimal diameter (S) of the ductus in 12 patients with patent ductus arteriosus (PDA). The stretch index (SI) was calculated as S divided by D. We calculated ratios of the loop diameter of the first implanted coil to D (C1/D) and S (C1/S) and those of the sum of all the loop diameters of all implanted coils for complete closure to D (total C/D) and to S (total C/S). RESULTS: Stretched minimal diameter divided by D was 1.8 +/- 0.3. Ratios of the loop diameter of the first implanted coil to D (C1/D), and S (C1/S) were 3.7 +/- 1.8 and 2.1 +/- 0.9, respectively. Ratios of the sum of all the loop diameters of all coils to D (total C/D) and S (total C/S) were 5.2 +/- 1.6 and 2.9 +/- 0.9, respectively. Standard deviations of C1/S and total C/S are significantly smaller than those of C1/D and total C/D, respectively. CONCLUSIONS: The narrowest segment of the ductus could be stretched to twice the size of the minimal diameter. The stretched minimal diameter may be a more reliable parameter to select the loop diameter of coils than the angiographic minimal diameter.

Catheterization↗

An infant with Costello syndrome complicated with fatal hypertrophic obstructive cardiomyopathy.

We report a 3-month-old girl with Costello syndrome complicating fatal hypertrophic obstructive cardiomyopathy. She had typical findings of this syndrome, slight dyspnea and persistent wheezing. Doppler echocardiography revealed asymmetric septal hypertrophy and systolic anterior movement of the anterior mitral leaflet. There was grade 1 mitral regurgitation. Although once her heart failure had been controlled medically, she died suddenly following deterioration of her heart condition. Costello syndrome can complicate fatal hypertrophic obstructive cardiomyopathy.

Abnormalities, Multiple↗

Plasma thromboxane B2 concentration in patients with ventricular septal defect and pulmonary hypertension.

Plasma thromboxane B2 (TXB2)-a stable metabolite of thromboxane A2 (TXA2)-conventional hemodynamic parameters, mean pulmonary input energy (input energy), and mean pulmonary output energy (output energy) were measured to assess platelet activation in 21 patients with pulmonary hypertension and a ventricular septal defect (VSD). The patients were divided into 2 groups: group A (normal range) and group B (high level). TXB2 levels were measured by radioimmunoassay. There was no relationship between plasma TXB2 concentrations and conventional hemodynamic parameters except the pulmonary-to-systemic pressure ratio. Output-to-input energy ratio was correlated logarithmically with the plasma TXB2 concentration. We concluded that patients with output/input energy ratio >0.15 had a high TXB2 concentration and activated platelets in pulmonary capillaries.

Blood Pressure↗

Disopyramide: a promising new approach to the medical treatment of the hypercyanotic spell complicating tetralogy of Fallot.

Under continuous ECG and oxygen saturation (SpO2) monitoring, the following measurements were taken by Doppler echocardiography in 6 consecutive patients with tetralogy of Fallot (TF) before and after intravenous administration of disopyramide (2mg/kg): left ventricular shortening fraction (LVSF); peak velocities in the right ventricular outflow tract (RVOT); diastolic and systolic internal diameters of the right ventricular outflow tract (dRVOT, sRVOT); and systolic blood pressure. SpO2 increased (p<0.01) from 78 to 98 (89 +/- 7, mean +/- standard deviation)% to 86-99 (94 +/- 5)%. LVSF decreased (p<0.05) from 0.34-0.56 (0.42 +/- 0.08) to 0.22-0.54 (0.33 +/- 0.13). The systolic blood pressure fell slightly (p<0.05) from 68-92 (79 +/- 8) to 64-92 (71 +/- 11)mmHg. The sRVOT increased (p<0.05) from 2.1-4.8 (2.7 +/- 1.5)mm to 3.0-8.1 (4.9 +/- 2.4)mm, while RVOT peak velocity decreased (p<0.05) from 2.20-4.88 (3.70 +/- 0.97)m/sec to 2.05-4.07 (2.92 +/- 0.72)m/sec. Disopyramide alleviates hypoxia in patients of TF through its negative inotropic action on right ventricular outflow obstruction.

Blood Flow Velocity↗

Epinephrine-induced constriction of the persistent ductus arteriosus and its relation to distensibility.

The aim of this study was to investigate whether distensibility of the persistent ductus arteriosus (PDA) might predict its potential for vasoconstriction. Epinephrine-induced constriction and stretching were investigated in 10 patients with PDA. Age, sex, ductus type, its minimal diameter, its stretched minimal diameter, and its stretch index were compared between epinephrine responders and non-responders. Age, sex, weight or the configuration or size of the PDA did not predict stretchability. The stretch index was significantly greater in responders (1.7-2.0, 1.8+/-0.1) than in non-responders (1.3-1.7, 1.4+/-0.2, p<0.01). Stretching of the PDA may depend on the elasticity of the vascular wall, which in turn is determined by preservation of vascular smooth muscle.

Child↗

[Somatic mosaicism of p(CTG)n expansion in a case of myotonic dystrophy with parotid tumor].

Myotonic dystrophy (MD) is an autosomal dominant systemic disorder with an unstable expansion of the CTG triplet repeat in the 3'-untranslated region of the gene encoding myotonine protein kinase (DMPK) which maps to chromosome 19q13.3. Somatic mosaicism of CTG repeats in MD has been reported; and it has been observed that CTG repeats in tumor tissues associated with MD are more expanded than the other tissues. It is not rare that parotid tumors are found in patients with MD. We performed Southern blot analysis for tissues from the parotid tumor, the normal parotid gland, the skeletal muscles, and the leukocyte from a 60-year-old patient with MD. CTG repeat was most expanded in the parotid tumor, and the normal parotid gland had longer expansion of CTG repeat than the skeletal muscles. The leukocyte had the shortest expansion of CTG repeat. The expansion of CTG repeat in the parotid tumor may be related to active cell division and may underlie the occurrence of tumors in MD.

Adenoma, Pleomorphic↗

Combined use of detachable coil against persistent mechanical hemolysis after transcatheter occlusion using Rashkind umbrella device in adult patient with patent ductus arteriosus.

Mechanical hemolysis after transcatheter occlusion of the patent ductus arteriosus using the Rashkind umbrella device has been postulated as a rare, but serious complication, even necessitating surgical repair. A rare, case of an adult patient with massive hemolytic anemia, successfully controlled by the combined use of several detachable coil devices is reported. The use of coil devices might be considered as a therapeutic option in such cases.

Anemia, Hemolytic↗

Coil occlusion for patent ductus arteriosus in Japan.

We surveyed Japanese experience of coil occlusion of patent ductus arteriosus up to 30 September 1996 by sending questionnaires to 175 hospitals. Thirty-four hospitals reported outcome data for 231 procedures in 218 patients. Successful implantation was achieved in 94% and acute complete closure of the ductus occurred in 71% of those in whom implantation of the coil was successful. Of the latter, 83% reported late complete closure. When those patients who underwent reocclusion for residual shunt are included, 89% attained complete closure. No life-threatening complications have occurred so far. Late reopening was reported in 3 cases. Although the angiographic type of ductus was significantly related to successful implantation (p < 0.01), there was no significant correlation with complete occlusion. Ductuses with a minimum diameter greater than 3 mm had a decreased chance of successful implantation, whereas those less than 2 mm had a greater incidence of complete closure.

Child↗

Antegrade or retrograde catheterization across a ventricular septal defect.

The success rate and the most suitable catheter tip for crossing over various types of ventricular septal defects (VSDs) were examined as a preliminary study for transcatheter closure of VSD. The 18 consecutive patients with various types of VSD were aged from 1 to 95 (mean [+/- SD] 13 +/- 22) months. Body weight was 3.7 to 25.0 (8.0 +/- 5.1) kg. Two-dimensional echocardiography showed that the maximal diameter of the defects ranged from 1.0 to 12.0 (7.5 +/- 3.1) mm. There were 10 patients with perimembranous defects, 4 with outlet defects, 2 with muscular defects, and 2 with tetralogy of Fallot with perimembranous defects. An angiographic balloon catheter, or an original or modified Judkins right coronary catheter could be passed through the VSD antegradely or retrogradely in 16 of 18 patients. In only two patients, with a small VSD of 2.0 and 1.0 mm, the catheter could not cross over the defect. The catheter entered the ascending aorta antegradely in 12 cases. The Judkins right coronary catheter is most suitable for crossing a VSD either antegradely or retrogradely.

Aortography↗

High dose of intravenous antithrombin III without heparin in the treatment of disseminated intravascular coagulation and organ failure in four children.

In several animal experiments, high doses of antithrombin III concentrates have shown beneficial effects on mortality and reversal of coagulation abnormalities which had resulted from disseminated intravascular coagulation. Other experiments have suggested that antithrombin III infusion without heparin is effective in the treatment of organ failure. We clinically treated children suffering disseminated intravascular coagulation only with antithrombin concentrate. Four patients suffering disseminated intravascular coagulation with organ failure were selected. We started antithrombin III concentrate infusion as soon as the diagnosis was established. The dosage of antithrombin III was 120-250 units/kg/day for 2 or 3 days. Heparin was not used. All 4 patients recovered completely and quickly without any complications within 14 days. We suggest that the high-dose antithrombin III infusion without heparin is an effective and safe therapy for disseminated intravascular coagulation with organ failure.

Antithrombin III↗

Diagnosis of renovascular hypertension by echo-Doppler velocimetry.

A 13 year old boy suffering hypertension was examined for peripheral plasma renin, angiotensin-I, angiotensin-II and aldosterone. All data were within the normal range. The Captopril test and renal scintigraphy (both with and without Captopril) also showed normal patterns. Echo-Doppler velocimetry of the renal artery revealed that left renal arterial peak flow velocity was fast (3.4 m/s). The patient was therefore diagnosed with left renal arterial stenosis. Angiography demonstrated the duplex of the left renal artery and stenosis of the left lower renal artery. Percutaneous transluminal angioplasty was successfully performed.

Adolescent↗

Plasma concentration and acute clinical effects of docarpamine, orally active dopamine prodrug, in infants.

Currently, there are no data available on the optimal doses and efficacy of docarpamine in infants. In the present study, three doses of docarpamine, 15.0-20.4 (19.0 +/- 1.9; mean +/- SD) mg/kg per dose every 8 h to 10 infants suffering heart failure. Age and bodyweight were from 1 to 4 (1.4 +/- 1) months and 2960-5160 (3350 +/- 872) g, respectively. In all infants, plasma concentrations of free dopamine were measured 1, 2 and 3 h after the first administration. Heart rate and systolic blood pressure were examined before and at the same time as the first administration. In seven infants, the 24 h urinary output and urinary excretion of electrolytes and creatinine before and during docarpamine were measured. Peak plasma concentration of free dopamine (ng/mL) was achieved after 1 or 2 h of administration, 0-163.1 (37.9 +/- 47.2) and 0-105.0 (37.8 +/- 39.3), respectively. The concentration had decreased rapidly by 3 h to 0-34.2 (12.4 +/- 11.0). Both heart rate (b.p.m.) and blood pressure (mmHg) tended to increase from 120-154 (140 +/- 15) and 56-90 (76 +/- 11) to a peak of 124-162 (148 +/- 14) and 70-92 (79 +/- 8), respectively (P = 0.197, P = 0.289). There were no significant changes in urinary output or excreta. Oral docarpamine of 15-20 mg/kg per dose can achieve plasma free concentrations of dopamine that increase heart rate and systolic blood pressure.

Administration, Oral↗

[Pure word deafness after cerebral hemorrhage in the left temporal lobe: a case report].

We report a patient with pure word deafness after subcortical hemorrhage in the left temporal lobe. Repetition and auditory comprehension were severely impaired, while reading and visual comprehension of the same material were almost normal. He did not show hearing loss, but speech discrimination and melody recognition was poor. On the speech discrimination test, his score was low especially in the right ear. The threshold on the directional hearing test was mildly elevated. There was no temporal summation by click sounds. CT and MRI disclosed a subcortical hematoma in the left superior temporal gyrus. PET demonstrated hypoperfusion in the surrounding area, which was not activated by hearing a story. It was considered that pure word deafness in this case was due to the interruption of auditory inputs to Wernicke's area from both hemisphere by the hematoma. After 5 months, auditory comprehension recovered so that he did not have difficulty in conversation. Speech discrimination improved in both ears, probably due to the recovery of two auditory pathways; the ipsilateral pathway through the left auditory radiation and the contralateral pathway through the right auditory radiation and the corpus callosum. This case suggests that in pure word deafness due to a unilateral lesion, the improvement in speech discrimination during follow-up period may provide a clue as to the site of the responsible lesion and its recovery.

Aged↗

Plasma thromboxane B2 concentration in pulmonary hypertension associated with congenital heart disease.

BACKGROUND: We investigated the plasma concentration of thromboxane B2 (TXB2), a stable metabolite of thromboxane A2 (TXA2), to assess platelet activation in 78 patients who had pulmonary hypertension associated with congenital heart disease (PH group) and 16 patients with almost normal hemodynamics (control group). METHODS AND RESULTS: The PH group was divided into two subgroups: pulmonary vascular resistance (Rp) < or = 10 U/m2 (Rp < or = 10 group) and > 10 U/m2 (Rp > 10 group). In addition, the Rp < or = 10 group was divided on the basis of clinical symptoms into groups with dyspnea (dyspnea[+] group) and without dyspnea (dyspnea[-] group). Plasma TXB2 levels were measured by radioimmunoassay. Plasma TXB2 levels in the three groups (control, Rp < or = 10, and Rp > 10) were significantly different (P < .005); the TXB2 levels in the Rp < or = 10 group were significantly higher than the others. Among the Rp < or = 10 patients, the plasma TXB2 levels were significantly higher in the dyspnea(+) group than in the dyspnea(-) group (P < .0001). In addition, the pulmonary-to-systemic flow ratio and pulmonary blood flow divided by body surface area were significantly higher in the dyspnea(+) group than in the dyspnea(-) group (P < .02 and P < .002, respectively). CONCLUSIONS: These findings suggest that platelet activation led to increased TXA2 release in patients with pulmonary hypertension, especially those with dyspnea and Rp < or = 10. TXA2 release from platelets probably caused constriction of the pulmonary arterioles and the bronchi, thus worsening pulmonary hypertension and dyspnea in these patients. In the patients with high Rp values, it was considered that the number of pulmonary arterioles where platelets could be activated had been reduced.

Adolescent↗