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Biomedical subjects

G Cabezuelo-Huerta

Publications and source records attributed to G Cabezuelo-Huerta.

16 recordsLinked to original sources
PubMed1992

Relative incidence and mortality of congenital heart defects diagnosed by angiohemodynamic methods: a 17-year study.

Over a 17-year period (January 1971 to January 1988), 2322 children, aged 0-14 years, were diagnosed as having congenital heart disease (CHD) by cardiac catheterization and angiography. Excluding those with highly complex or undiagnosed defects, there were 2156 children with CHD, 72.4% of whom were treated surgically, with a total surgical mortality rate of 24.1%. After a mean follow-up of 9 years the overall mortality of the cohort was 29.9%, 29.1% occurring in the first month of life, 39.6% between 1 month and 1 year, and 31.2% between 1 and 14 years. The incidence, mortality, and age at death of each cardiac defect are presented and compared with the results of other studies. The overall mortality for congenital heart defects in eastern Spain remains elevated, whereas there has been a significant decrease in neonatal mortality and a trend towards a lower mortality in the last years of the study.

Adolescent↗
PubMed1992

Natural and modified history of isolated ventricular septal defect: a 17-year study.

We studied 882 cases of isolated ventricular septal defect (VSD) diagnosed from 1971 to 1988 with a mean follow-up period of 9.5 years. They represent 22.5% of all congenital heart defects diagnosed in this period. Six hundred eighty-three children (77.4%) did not develop symptoms, had small defects, and the rate of spontaneous closure was high (40.2%). The remaining 199 children presented symptoms; at the initial catheterization 25, 65, 107, and 2 cases were grouped in hemodynamic groups 2, 3, 4, and 5-6, respectively, on the basis of pulmonary flow and resistance. Only seven patients (0.7%) developed aortic regurgitation, and only five patients (0.5%) developed infective endocarditis. Complete surgical correction was performed in 137 children (15.5% of the total cases), with surgical mortality decreasing from 21.4% before 1983 to 3.5% afterward. Overall mortality was 3% for the entire cohort, 0% for hemodynamic groups 1 and 2, 3% for group 3, and 25% for hemodynamic groups 4-6. About two thirds of the deaths took place between 1 month and 1 year of life, and one thirds of the deaths occurred before surgical treatment. Surgical mortality rates for hemodynamic groups 2, 3, and 4 were 0, 4.7, and 15.3%, respectively. Actuarial survival curves show an important improvement in the prognosis after 1983. Our results stress the importance of early surgical complete correction on patients with large defects and severe hemodynamic changes.

Actuarial Analysis↗
PubMed1990

Natural and modified history of complete atrioventricular septal defect--a 17 year study.

We reviewed 103 cases of isolated complete atrioventricular septal defect. These cases represented 4.4% of the cases of congenital heart disease diagnosed in our hospital by catheterisation and angiography during 1971-88. Most children (n = 76) had Down's syndrome. Banding of the pulmonary artery was performed in seven cases and complete repair in 67 cases. In the period 1971-82 the complete correction was performed at a mean age of 23 months with a surgical mortality of 88.8%. In the period 1983-8 the mean age at complete correction was 13 months, the mortality 43.2%, and the five year actuarial survival was 46.8%. The 22 patients that survived after complete correction were in functional classes I and II of the New York Heart Association classification. After a mean follow up of 10 years only eight (36%) of the 22 who were followed up and treated medically survived; all had developed pulmonary vascular obstructive disease and were in functional classes III or IV. Our findings stress the importance of early complete surgical repair.

Endocardial Cushion Defects↗
PubMed1986

[Diagnostic problems of the aortopulmonary window].

Seven patients with aortopulmonary window type I (proximal) were studied over a period of 14 years. This report includes clinical, electrocardiographic, radiographic, cardiac catheterization and angiocardiographic data on the patients. Associated lesions were present in 6 patients: ventricular septal defect (1 case), atrial septal defect ostium secundum (1 case), persistent ductus arteriosus (1 case), membranous subaortic stenosis (1 case), aortic origin of the righ pulmonary artery (1 case) and single ventricle (1 case). Retrograde aortography is the most useful investigation in determining the precise location of the defect. Five patients underwent surgery but only two survive. Transaortic approach is the greater safety for surgical closure of the defect. The high mortality rate is the result of pulmonary complications caused by an excessive left-to-right shunt.

Abnormalities, Multiple↗
PubMed1983

[Isolated hypoplasia of the right ventricle with interatrial communication. Study of a case and review of the literature].

A 1 1/2 year old boy with cyanosis and congestive heart failure was found to have isolated right ventricular hypoplasia with atrial septal defect by cardiac catheterization and angiocardiography. On physical examination no murmur was heard. The ECG revealed a frontal plane AQRS of -45 degrees, right atrial enlargement and left ventricular hypertrophy. Chest X-ray examination showed slight cardiomegaly. Preoperative assessment included temporary occlusion of the atrial septal defect by means of a balloon catheter. Surgical closure of the atrial septal defect was performed at 4 years old. The clinical findings and surgical treatment of this rare malformation are discussed.

Cardiac Catheterization↗
PubMed1983

[Sjögren-Larsson syndrome. Study of 2 cases].

Two siblings, a 3 1/2 years-old male and a 2 years-old female, with Sjögren-Larsson syndrome (SLS) are described. The SLS is a genetically determined syndrome with autosomal recessive inheritance, characterized by the three main symptoms: congenital laminar ichthyosis, spastic displegia and mental deficiency. Parental consanguinity not was found. Pregnancy and perinatal period of the two children was normal. Ichthyosis was found at birth. Spastic displegia was noted before the age of one year and mental retardation was suspected at the same time. The EEG is abnormal. Glistening dots in the optic fundus were not observed. Diagnostic features of this rare syndrome are reported.

Child, Preschool↗
PubMed1983

[Body growth in the early diagnosis of Prader-Labhart-Willi syndrome].

Growth charts of five children with Prader-Labhart-Willi syndrome were examined. Clinical diagnosis was based on usual features of this condition. These included hypotonia in infancy, obesity, mental retardation, short stature, undescended testes in boys and typical physical features. Extensive investigations have failed to reveal pathognomonic abnormalities in this syndrome. Obesity and failure to thrive, beginning in early infancy and increasing with age is a precocious and typical feature. This pattern helps to early diagnosis. Only congenital hypothyroidism could show a similar pattern.

Age Factors↗
PubMed1981

[The fetal hydantoin syndrome (author's transl)].

The fetal hydantoin syndrome is a variable pattern of altered growth (pre and postnatal), mental deficiency, unusual facies, distal phalangeal hypoplasia, and other defects occurring in some infants exposed in utero to hydantoins. This altered pattern of morphogenesis is distinct from other recognized disorders. It has been reported only in the offspring of women using hydantoins. One case is presented and several aspects of the diagnosis are discussed.

Abnormalities, Drug-Induced↗
PubMed1979

[Atrial septal defect with pulmonary hypertension in infancy (author's transl)].

Four cases of atrial septal defect, "ostium secundum", associated with pulmonary hypertension and congestive heart failure in children under the age of two years are reported. A correct diagnosis could be made before surgery in only two of the case. In one case partial anomalous pulmonary venous connection was incorrectly diagnosed, while ventricular septal defect could not be excluded in the fourth case. The systolic pressure in the pulmonary artery was higher than 50 mm. Hg in all cases. One patient died after catheterization, and total surgical correction was performed in the other three cases, with good results. In all of them, a large atrial septal defect could be seen. There was no evidence of any other associated anomaly. After an average follow up of 4.5 years the three patients that where operated remain asymptomatic. The rarity of this entity, the difficulties in making a correct diagnosis and several pathogenetic aspects of this abnormality are discussed on the light of these and other cases previously reported in the literature.

Angiography↗
PubMed1979

[Toxic epidermal necrolysis (Lyell's syndrome) (author's transl)].

One case of toxic epidermal necrolysis (Lyell's syndrome) in a 6 1/2 year old girl is presented. The patient had received penicillin and streptomycin for four days two weeks before the onset, and was admitted to the hospital with epidermal lesions characteristic of the bullous stage of Lyell's disease. A biopsy of the skin confirmed the diagnosis. Cultures of blood, skin and exudate from the bullae were negative, and immunological tests were normal. No immunohistologic studies on the skin biopsy were done. The patient was treated with cephalosporins and corticosteroids, and the evolution was favorable. The main clinical traits found in this patient are discussed on the light of information available from previous reports. Several aspects of the diagnosis, management, and natural history of the disease are also discussed.

Cephalothin↗