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Biomedical subjects

E Pastor

Publications and source records attributed to E Pastor.

At least 91 records · Page 5Linked to original sources
PubMed1990

[Right aortic arch with retro-esophageal aorta. Diagnosis by computerized axial tomography].

The case of a patient with right aortic arch and retro-esophageal aorta associated with a perimembranous ventricular septal defect with a mild fibrous subaortic stenosis is presented. The existence of the retro-esophageal segment of the aorta was confirmed by left ventriculography and computerized axial tomography. This patient is the first to be diagnosed by axial tomography in our country.

Adolescent↗
PubMed1990

[Multiple aneurysms of the left auricula, ascending aorta and sinuses of Valsalva with interventricular communication, fibromuscular subaortic stenosis and a single coronary artery].

We report the case of a male newborn infant with aneurysm of atrial appendage, ascending aorta and sinus of Valsalva associated to ventricular septal defect, fibromuscular subaortic stenosis and single coronary artery. The diagnosis was carried out by means of two-dimensional echocardiography and angiocardiography. This complex cardiac malformation has not been reported before.

Abnormalities, Multiple↗
PubMed1990

[Dilated myocardiopathy in children].

Between 1971-1988 we have studied 46 children with dilated cardiomyopathy. Twenty were males and 18 females, with a median age of 17 months (range from 1 day to 11 years). The incidence of 3.4 cases a year during the first nine years dropped to 1.3 cases a year in the following years. Two patients were siblings. Seventeen patients were studied with Eco-Doppler, cardiac catheterization and angiocardiography. The most frequent presentation was heart failure, present in 45 cases (98%). By bidimensional echocardiography it was observed that all patients had a dilated left ventricle, with a left atrial/aorta ratio of 1.5 +/- 0.3; the ejection fraction was diminished in 12 patients (11-36%) and the length of the left ventricle in the long parasternal axis view 4.2 +/- 0.7. All the patients studied had an elevated telediastolic pressure in the left ventricle, four with pulmonary artery pressure of 96 +/- 11 and seven with right atrial pressure of 24 +/- 5. There was mitral insufficiency in 4 patients and tricuspid insufficiency in three. Clinical course was favorable in 18 patients with median age at present of 112 months (range: 11 months to 15 years). The last 6 patients, treated with vasodilators, are living. In conclusion, dilated cardiomyopathy is a moderately frequent disease, with trend to diminish in incidence prognosis improved after treatment with vasodilator was introduced, and easily assessed with non-invasive methods.

Cardiomyopathy, Dilated↗
PubMed1990

[Aortic atresia with hypoplastic left ventricle. Bidimensional echocardiographic correlation. Anatomy of 14 cases].

Fourteen cases (10 males, 4 females) of aortic atresia with hypoplastic left ventricle are reviewed. All were studied by two-dimensional echocardiography, treated by prostaglandin E1 and proved by necropsy. Surgery was also performed in 3 cases by Norwood technique. A good echo-anatomical correlation was found in the size of pulmonary artery (1.14 +/- 0.17 and 1.28 +/- 0.33 respectively), mitral valve (0.4 +/- 0.3 and 0.33 +/- 0.24) and aortic diameter ring with atresic valve (0.38 +/- 0.12 and 0.31 +/- 0.1). However, echocardiography overestimated the diameter of ascending aorta (p less than 0.001) and tricuspid valve (p less than 0.001) and the length of the left ventricle. These differences may be related to the greater distension of the aorta in vivo and to the difficulty of determining the exact direction of the ultrasounds. We conclude that two dimensional echocardiography is of great value not only for the diagnosis but also for the selection of patients for surgery.

Aortic Valve↗
PubMed1989

Fixed subaortic stenosis: the value of cross-sectional echocardiography in evaluating different anatomical patterns.

We present a study using cross-sectional echocardiography in 39 patients (29 male and 10 female) with discrete subaortic stenosis. Five parameters were evaluated in the study: the morphology of the obstruction, the distance of the stenosis from the aortic valve, the type of insertion, the base of implantation, and any associated anomalies. The lesion could be divided into groups with either fibrous or fibromuscular shelves. In the group of 14 patients with fibrous shelves, the distances from the stenotic lesion to the aortic valve was less than 15% of the length of the left ventricle. The obstructive fibrous tissue was inserted on the septum and extended onto the aortic leaflet of the mitral valve (circumferential lesion) in 12 cases. The base of implantation was narrow in all 14 of them. In the 25 patients having fibromuscular lesions, the distance between obstruction and valve was greater than 18.4% of the length of the left ventricle. The insertion of the obstructive lesion was circumferential in 18 cases and its base of implantation was wide in 20 of the patients. Nineteen patients, 16 of whom had fibromuscular lesions, showed associated anomalies. Statistical analysis showed a good correlation between the type of stenosis and the base of implantation of the stenotic lesion (P less than 0.001) or associated anomalies (less than 0.01), but there was no distinction between the groups with regard to the type of insertion (less than 0.38). Twenty-five patients underwent surgical correction which was carried out between the ages of 4-14 years. Prosthetic valves (3 aortic and 1 mitral) were inserted in 4 patients.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1989

[Recurrent thrombotic thrombocytopenic purpura and pregnancy].

Two women are presented who were diagnosed of thrombotic thrombocytopenic purpura (TTP) during their first pregnancies, leading in both cases to spontaneous abortion. Both patients improved after plasma exchange or fresh plasma infusion. Two new pregnancies on each patient, closely watched on gynaecological and haematological grounds, led to TTP reactivation. Obstetrical and haematological management was similar for the two women (fresh-frozen plasma administration) but it yielded different results, since, although the risks for the mothers were controlled, one of the patients gave birth successfully on two occasions while the other developed stillborns on two occasions as well. These observations suggest the inter-relationship between TTP and pregnancy, and point to some pathophysiological aspects of the foetal deth repeteadly presented by one of the patients. A review of the literature is presented on the basis of these cases of PTT associated to pregnancy.

Adult↗
PubMed1989

[Cardiovascular pathology in Marfan syndrome. Study of 11 children using two-dimensional echocardiography].

UNLABELLED: From 1983 to 1987 we have studied by two-dimensional echocardiography 11 pediatric patients with Marfan's syndrome. All of them presented cardiovascular lesions localized mainly in the aortic and mitral valves and in the ascending aorta. Nine patients had a dilated aortic ring in both transversal and longitudinal diameters 2.03 and 2.29 cm/m2 of body surface (bs), respectively. The average value of the aortic valvular area was 4.71 cm2/m2 bs. The 9 patients presented also dilatation of the ascending aorta (2.01 cm/cm2 bs). There was thickening of the mitral valve in 5 cases and a prolapse was present in 8 (73%). In 4 cases the prolapse was localized in anterior leaflet valve in 2 in the posterior and in 2 in both. The maximal mitral valve diastolic area was 3.25 cm2/m2 bs. None of them has needed during follow up valvular replacement. Considering the importance of this cardiovascular disease and knowing its unpredictable evolution we recommend, in the absence of valvular insufficiency, and annual echocardiographic reevaluation, and more often if valvular insufficiency is already present. IN CONCLUSION: two-dimensional echocardiographic is useful and necessary for the diagnosis and follow up of patients with Marfan's syndrome.

Adolescent↗
PubMed1989

[Scimitar syndrome in the newborn child and infant].

We present 10 symptomatic patients with scimitar syndrome. Their age was below 1 year and four were newborn babies. In 5 patients there was secundum atrial septal defect and in one there was a patent ductus arteriosus. Sixty per cent of the patients had pulmonary sequestration. The right lung was moderately or severely hypoplasic in 8 cases and seven had frequent respiratory infections. The anomalous venous drainage produced a left-to right shunt at atrium level which in our series had a value of 2.87 +/- 0.5. Five patients had pulmonary hypertension either moderate or severe. The severity of this disease in early infancy is related to the pulmonary hypertension (40%), requiring a prompt surgical correction. Eight patients were operated upon, the drainage being connected to the left atrium. The six surviving patients are asymptomatic at 12 years of follow-up. Our series is the biggest published in our country and the second in the world literature in infancy.

Abnormalities, Multiple↗
PubMed1989

[Double orifice mitral valve. Study with two-dimensional echocardiography].

Double orifice mitral valve is a rare congenital anomaly which is frequently associated with atrioventricular defects. According to its structure it is classified as central, commissural or valvar. Diagnosis by cross-sectional echocardiography was verified in 4 patients with a double orifice mitral valve of the central type. Age of the patients at the time of diagnosis ranged from 3 months to 17 years. Three of them had an associated ostium primum atrial septal defect. The defect was observed in parasternal long-short axis views and subcostal 4 chamber axis views. The parasternal short axis gave information on the central type but subvalvular structures of the double orifice and insertion of the chords in papillary muscle were best observed in parasternal long and subcostal four chamber axis views.

Adolescent↗
PubMed1988

Acute promyelocytic leukemia. Therapy results and prognostic factors.

From December 1976 to July 1986, 34 patients with acute promyelocytic leukemia (APL) were treated with daunorubicin (DNR) alone and simultaneous supportive therapy with low-dose heparin, platelet transfusions, and fresh frozen plasma. Two consecutive maintenance therapy regimens were employed in patients who achieved complete remission (CR): (1) a classical maintenance with methotrexate and 6-mercaptopurine, with DNR plus methyl-GAG re-inductions; (2) from 1982 an intensive sequential combination therapy regimen was administered. CR was achieved in 23 patients (68%). Only one patient had leukemic resistance. Other failures were a consequence of post-chemotherapy complications. A multivariate logistic regression analysis has been performed to evaluate the prognostic importance on response to remission induction of 25 patient and disease characteristics at diagnosis. The significant variables in decreasing order of significance were: serum albumin level, fever at diagnosis, serum creatinine level, and age. The median duration of remission and survival by Kaplan-Meier analysis were projected to be 24 and 25 months, respectively. Relapses occurred in 11 of 23 CR patients. Nine patients remained in the first remission from 5+ to 37+ months. Short-term (CR) and long-term results (duration of remission and survival) in APL treated for induction with DNR alone were similar to those obtained in other subtypes of acute myeloblastic leukemia by intensive combination chemotherapy.

Adolescent↗