Biomedical subjects
Publications and source records attributed to E Pastor.
We present the case of an infant diagnosed by prenatal echocardiography as having a biventricular rhabdomyoma which was confirmed after birth. In the series of two-dimensional echocardiographies this tumor was seen to decrease in size until its complete disappearance.
Object of the study was to assess educative needs in the field of drug therapy among 99 chronic patients with advanced chronic lung disease admitted to the Respiratory Service of the Hospital Sta. Creu i Sant Pau (Barcelona, Spain). The knowledge of the patients about their previous drug treatment, sources of information as well as patients' conception of side-effects, was gathered from medical records and by means of a questionnaire. The majority of patients studied were male (63%), aged over 60 years (average 63 +/- 12), illiterate or with basic education (88%), had a chronic obstructive, pulmonary disease (76%) and with an important degree of chronicity. They had been treated for their condition during a mean of 8.4 years and took an average of 4.6 drugs. 31% Of patients did not answer any question about their medication properly and only 17% gave a correct answer to all the questions. The lower the age and the higher the educational level, the higher the proportion of correct answers to knowledge questions. 35% Of the patients stated not to have been informed about their medication and 55% stated that they had read the patient package inserts.
Between 1985-1990 we studied by means of two-dimensional echocardiography 286 patients with isolated ventricular septal defect (VSD). A hundred and sixty three being male and 123 female, as a mean age of 5 +/- 3 months. Sixty two cases (22.3%) reveales associated anomalies and in other 63 (22.4%) there was in the evolution an aneurysm of the septum membranous. The projections used were apical 4-chamber, with or without aortic root; parasternal long-short axis of great arterias or at ventricular level; subcostal 4-chamber; right oblique outlet right outflow tract and left oblique outlet left and right outflow tracts. Patients with VSD smaller than 3 mm were excluded. Perimembranous defects, 189 cases (66%), were more frequent than muscular, 91 31.8%), and subarterial defects, 6 (2.1%). Only 19 (9 apical muscular, 6 perimembranous outlet and 4 perimembranous trabecular) were initially misclassified. The diameter of the VSD was large in subarterial (0.85 +/- 0.1 cm) and perimembranous outlet VSD (0.75 +/- 0.5 cm) compared with the rest. Each group of defects was more easily shown by one particular projection except trabecular muscular defect. In conclusion, we are able to state that two-dimensional echocardiography enables us to discover the size and situation of isolated ventricular defects.
We present 15 patients with atrioventricular and ventriculoarterial discordance diagnosed with two-dimensional echocardiography from January 1973 to June 1991. The 15 had situs solitus and six dextrocardia. A different level of insertion of the atrioventricular valves worth from 0.77 +/- 0.3 cm, was observed in all the cases which didn't perimembranous inlet ventricular septal defect. Ten patients, presented septal insertion of one of the chords of the left atrioventricular valve, while, this insertion wasn't observed in the right atrioventricular valve. The ventricular morphology and trabeculation, permitted the identification of both ventricles in all the cases. The subxifoidea view resulted in being fundamental, for the examination of the ventriculoarterial connections. Ten cases (66.6%) had an obstruction in the pulmonary outflow tract (6 in the valve and 4 in subvalvular area) and nine (60%) ventricular septal defect (7 perimembranous and 2 muscular). We believe that the two-dimensional echocardiography gives enough information to arrive at the correct diagnosis of this cardiac malformation and of the associated anomalies. This information can be completed with the use of the color-flow Doppler.
We report a case of mitro-aortic atresia with situs inversus. The patient was studied with two-dimensional echocardiography and the diagnosis was confirmed in the necropsy. Our newborn infant is the second case reported with this abnormality and the first in our country.
A Blalock-Taussig shunt was performed in a child of 14 months diagnosed with tetralogy of Fallot associated with a complete atrioventricular defect. Seven months later, a balloon dilatation was performed because the shunt was stenosed in the site of the distal anastomosis. Repeat angiography we proved the patency of the shunt without restenosis 4 year later.
The ventricular septal defect (VSD) may close spontaneously in the first few years of life. The closure occurs by muscle's growth of the borders of the defect or by the appearance of an aneurysm of ventricular septum composed mostly by tricuspid tissue. We believe that the two-dimensional echocardiography is the best method to determine the mechanisms that take part in the aneurysm formation. The study was carried out on 58 patients with aneurysms of ventricular septum observed in 230 patients with ventricular septal defects. 29 were male and 29 were female patients. The mean age at the time of diagnosis of aneurysm was 30 months (range 1 month-13 years). Forty seven patients had a perimembranous ventricular septal defect (perimembranous inlet 29, perimembranous trabecular nine, perimembranous outlet nine and of mixed's type seven), muscular defect nine (muscular inlet seven, and muscular trabecular two) and VSD closed in two. The size of the defect was 0.47 +/- 0.2 cm (range 0.2-1.1). In 16 the defect was larger than 0.6 cm. Thirty two patients had associated anomalies. On study the aneurysm in relation with tricuspid valve leaflets: in 27 cases (46.3%), the aneurysm was entirely formed by tricuspid septal leaflets tissue (17 had perimembranous ventricular septal defect and seven muscular), in eight (13.6) the aneurysm had tricuspid valve leaflets and interventricular component of the membranous septum tissue (five perimembranous defect and two muscular) and in another 16 cases membranous septum tissue only. In 7 patients the origin of the aneurysm wasn't confirmed.(ABSTRACT TRUNCATED AT 250 WORDS)
Between May 1974 and June 1990, 28 double chambered right ventricle underwent surgical repair. Twenty-one (75%) had high obstructive anomalous muscle bundles and in seven (25%) the anomalous muscle bundles were in low position. A ventricular septal defect (VSD) was present in all cases and a subaortic stenosis in six. Longitudinal right ventriculotomy was performed in 15 (53.57%) patients and a combined pulmonary arteriotomy and right atriotomy was used in 13 (46.43%). In the group treated by ventriculotomy 2 patients died shortly after operation (one as a consequence of disseminated infection and one after a lung lobectomy); another patient developed patch dehiscence and reoperation was performed via right atrium a year after. In other 3 patients the Doppler revealed a residual gradient of 20 to 30 mmHg. There were no complications in the group of patients that underwent right atriotomy. In addition to resection of the anomalous muscle bundles, the associated cardiac anomalies were corrected in all patients. Follow-up of 5.5 +/- 3.8 years after operation showed that 26 (92.86%) patients were alive and asymptomatic. We conclude that double chambered right ventricle can be early repaired with good results using a combined procedure with right atriotomy and pulmonary arteriotomy.
Acute leukemia, hepatocellular carcinoma, and squamous cell carcinoma have been reported in patients with Fanconi's anemia. We report on a 31-year-old woman who developed squamous cell carcinoma of the esophagus and hepatocellular carcinoma. Jaundice and hepatic tumor developed in 1981, after she had received oxymetholone for 10 years. Liver biopsy revealed peliosis hepatis. Androgenic therapy was stopped and the jaundice resolved. However, the hepatic tumor was observed to be unchanged. The patient died of disseminated squamous cell carcinoma, but no metastatic lesions from hepatocellular carcinoma were detected in the autopsy. The association of Fanconi's anemia and squamous cell carcinoma is reviewed, and the malignant potential of androgen-related hepatic tumors is discussed.
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Between January 1976 and December 1989, 28 patients with fixed subaortic stenosis underwent surgical correction. Twenty-one were male and seven female. The mean age when surgery was performed was 7.4 years (range 14 months to 14 years). Angiography was not done in 8 patients (28.5%). Fourteen had a subaortic stenosis due to a fibrous shelf and the other 14 to a fibromuscular one. Fifteen had associated anomalies, seven aortic stenosis, three aortic regurgitation and four mitral disturbances. Three surgical procedures were carried out: resection in 16 patients (57.1%), resection and myotomy in ten (35.7%), and resection with myectomy in two (7.1%). In 5 patients a residual stenosis was detected, one of whom required repeated surgery, and 2 other patients developed recidive of the stenosis (both required repeated surgery). Prosthetic valves (five aortic and one mitral) were inserted in 6 patients (21.4%). One of these required an aortic valve conduct. Pacemaker was placed in other 4 patients (two with prosthesis). No patient died and 20 (71.4%) are normal, nowadays, with a mean follow-up of 5.5 years. We conclude that fixed subaortic stenosis is a serious disease which requires correction at the time of diagnosis, independently of gradient. The type of surgical procedure influences in recidive. The older patients with the fibromuscular type and associated anomalies are candidates for a prosthetic valve.
Congenital heart diseases were studied in children diagnosed of trisomy 13 and trisomy 18 in our hospital between January 1973 and July 1990. Twenty patients with trisomy 18 were diagnosed (18 females and two males). All had cardiac malformations. The findings were: ventricular septal defect in 16 cases (80%), valvular anomalies in 12 (63%), patent ductus arteriosus in nine (47%) and atrial septal defect or patent foramen ovale in 7 cases (36%). We found some complex congenital cardiac diseases: one atrioventricular canal, one tetralogy of Fallot, one hypoplastic left ventricle with mitral atresia and double outlet right ventricle, one case of univentricular heart with aortic outlet from a rudimentary cavity, a right ventricular atresia with pulmonary and tricuspid valves atresia. Nine cases of trisomy 13 were diagnosed (seven females and two males). We found: ventricular septal defect in 7 cases (77%), valvular disease in five (100% of the necropsy studies), secundum atrial septal defect in 4 patients (80%) and patent ductus arteriosus in two. Two cases presented hypoplastic left ventricle with aortic arch hypoplasia, one of them had subaortic stenosis and left superior vena cava being connected to the right atrium via coronary sinus; one case showed fibroelastosis. Our results have been similar to the previously reported and confirm the invariably presence of cardiac malformations in these syndromes. These malformations are an important sign of suspicion in fetal ultrasonography.
Lipases from different origins have been immobilized in supports chosen by its different aquaphilicity and used as biocatalysts for the hydrolysis of tributyrin. The changes of the concentration of tri-, di-, monobutyrin, glycerol, and butyric acid during the reactions catalyzed by soluble, as well as immobilized, lipases were evaluated by gas chromatography. The experimental data were fitted to a simple kinetic model for the sequential reaction of tributyrin hydrolysis. The calculated apparent rate constants were different for the biocatalysts used and were apparently related to diffusional effects and aquaphilicity of the supports. Maximal yields of dibutyrin were found with the soluble Candida lipase, whereas the highest yield of monobutyrin (90%) was obtained with the least aquaphylic derivative (Candida-Celite).
Between 1983-1988 cross-sectional echocardiography was performed in 63 patients having an atrioventricular septal defect with common atrioventricular orifice. We excluded from this study all those patients with separate right and left orifices ("ostium primum" defects), those with isomerism of the right and left atrial appendages, those with univentricular atrioventricular connexions and those with discordant atrioventricular and ventriculo-arterial connexions. Parasternal long- and short-axis views, apical 4-chamber views and subcostal long-axis views were employed in all patients. In the last 26 cases, we also obtained the subcostal short-axis view. Nineteen patients showed ventricular dominance, with the right ventricle being dominant in 15. Ten patients had an associated defect in the oval fossa, while the atrial septum was partially or completely absent in the other 53. A ventricular septal defect was observed in all, but it was small in 10 and multiple in 2. Attachments of the superior and inferior bridging leaflets to the crest or the right side of the ventricular septum were seen in 32 cases. The inferior leaflet was hypoplastic in 19 patients. There was narrowing of the left ventricular outflow tract in 8 patients, and obstruction of the right ventricular outflow tract in 3. Abnormal attachment of the right portion of the common valvar orifice was present in 2 cases. A solitary papillary muscle supporting the left ventricular component of the common valve was seen in 6 cases producing a parachute-like arrangement. Our study shows that cross-sectional echocardiography is an excellent technique for the analysis of this anomaly.
We present a case of univentricular connexion to a dominant left ventricle with double orifice tricuspid valve, left atrioventricular valvar atresia, discordant ventriculo-arterial connexion and aortic arch hypoplasia. Diagnosis was made by cross-sectional echocardiography and angiocardiography. This case is exceedingly rare.
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A case of a previously unreported anomaly is presented in which absence of the aortic valve cusps, mitral atresia, a normal left ventricle, and an intact ventricular septum were diagnosed by cross sectional echocardiography. The development of a normal left ventricle, rather than the hypoplastic ventricle usually associated with mitral atresia, is explained by filling of the ventricular cavity via the regurgitant aortic valve.