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Biomedical subjects

F Moreno Granado

Publications and source records attributed to F Moreno Granado.

At least 19 recordsLinked to original sources

[Williams-Beuren syndrome: presentation of 82 cases].

OBJECTIVE: We performed a retrospective review of a series of 82 cases of Williams-Beuren syndrome (WBS) and associated diseases. MATERIAL AND METHODS: A series of 82 patients (47 males and 35 females) who consulted at the hospital because of mental retardation and/or congenital cardiopathy were included. The patients were studied mainly from a neurological and cardiological point of view, and secondarily because of endocrinological and nephrological problems. Since description of the chromosomal abnormalities provoking the syndrome, we perform karyotyping in all patients with suspected WBS. RESULTS: Alterations mainly consisted of distinctive facial appearance (100 %), mental retardation with friendly behavior (90 %), congenital cardiopathy (85.4 %), mostly consisting of supravalvular aortic stenosis (72 %), with (12 %) or without (60 %) pulmonary stenosis, and behavior typical of attention deficit-hyperactivity disorder, which usually manifested at the age of 4 to 5 years in both boys and girls. Approximately 90 % started to walk and speak later than average. Birthweight was below 3000 g in 65 % of the patients in whom this datum was included in the medical record. Eleven of the 13 patients (84.5 %) studied showed the typical deletion of WBS. CONCLUSION: Study of patients with WBS should be multidisciplinary. Most patients require help during schooling and subsequent vocational guidance.

Adolescent↗

[Guidelines of clinical practice of the Spanish Society of Cardiology. Requirements and equipment of invasive techniques in pediatric cardiology: clinical application].

Invasive techniques in pediatric cardiology have experienced a big change since the 80's. The growth of non-invasive methods for diagnosing congenital heart defects has made the number of diagnostic catheterizations decrease remarkably. On the other hand, the notable development of pediatric interventional catheterization techniques will allow that, in the near future, the number of therapeutic catheterizations overcomes the diagnostic ones in our country. The former are more difficult and dangerous, so they require experienced and skilled hands and more economic resources. This chapter is divided in three main sections: I) Requirements and equipment needed for pediatric invasive techniques; II) Current indications, contraindications and complications of the diagnostic catheterization, and III) Techniques, indications and results of pediatric therapeutic catheterization: current state. Likewise, we state the suitability or not for these therapeutic procedures in different cardiac anomalies.

Age Factors↗

[Surgical treatment of Fallot's tetralogy with hyperplasia or agenesis of the conal septum].

INTRODUCTION: The clinical evolution, anatomosurgical aspects, and postoperative evolution of a specific group of Fallot's tetralogy with hipoplasic or agenesic of the conal septum (representing a 7% of our Fallot's tetralogy cases) is presented. METHODS: A total of 41 patients surgically corrected in our Service between February 1973 and December 1995 has been retrospectively studied. Ages ranged between 13 months and 13 year. Clinically all cases developed moderate or severe hypoxemia. In 43.9% of cases it was necessary to perform a palliative surgery at an age between 15 days and 4 years. RESULTS: There was no hypoxemics spells. In the ECG we have found a QS in the aVR in 40% of cases. There was 7 deaths (17% of mortality rate). Four cases required reoperation. The clinical evolution, ECG, EKO, and hemodynamics findings are commented. CONCLUSIONS: In this type of Fallot's tetralogy, specifically in those cases with a pulmonary artery ring of normal size, it is necessary to have a good preoperative diagnoses in order to perform a longitudinal ventriculotomy with right ventricular outflow tract enlargement. Also, it is important to be meticulous with the stitches in the closure of the ventricular septal defect with a patch in order to avoid the potential lesion of the aortic valve.

Abnormalities, Multiple↗

[Radiofrequency catheter ablation in permanent atrioventricular junctional reciprocating tachycardia in children].

BACKGROUND: The permanent form of junctional reciprocating tachycardia occurs predominantly in childhood and is frequently refractory to pharmacologic treatment. These patients are at high risk for developing tachycardia-induced cardiomyopathy. Radiofrequency transcatheter ablation appears promising as safe and effective therapy in children. PATIENTS, METHODS AND RESULTS: Six patients ranged in age from 3 months to 20 years who had the permanent form of junctional reciprocating tachycardia underwent radiofrequency catheter ablation. Five patients had been treated with 3.4 +/- 1.1 antiarrhythmic drugs and one patient did not receive treatment before ablation. Three accessory pathways were located in the right posteroseptal area, two in the left posteroseptal area and one in the left posterior free wall. The six patients had seven successful procedures performed including one after tachycardia recurrence in one patient six weeks after the first ablation session. Five ablation procedures were performed with temperature-guided catheter ablation. The temperature reached during successful radiofrequency applications was 55-60 degrees C at a power output from 24 to 47 W. In the other two procedures 25 W were given. No complications were observed. After a mean follow up of 11.6 months all patients are asymptomatic including a 3 months old patient with tachycardia-induced cardiomyopathy. CONCLUSIONS: Radiofrequency transcatheter ablation is an important option of therapy in children with the permanent form of junctional reciprocating tachycardia, including patients with tachycardia-induced cardiomyopathy.

Adolescent↗

[Dilated cardiomyopathy in infants and children].

Three cases of dilated cardiomyopathy manifested at 2, 5, and 13 months of age are reported. The clinical, radiographic, electrocardiographic, echocardiographic and angiocardiographic findings are analyzed. The follow up during 11, 14 and 16 years is described. After several years of the onset, 2 patients recovered a normal cardiac function. The third patient required a cardiac transplant when he was 9 years old, and now, after 2 and a half years, he is healthy, without congestive heart failure. Current references about indications of cardiac transplant in this disease are reviewed. We got the following conclusion: In patients with dilated cardiomyopathy, at least in those who begin before 2 years, the indication and the time of the cardiac transplant have to be established by individual valuation of each case, considering the follow up of the risk's criteria, mainly the congestive heart failure.

Cardiomyopathy, Dilated↗

[Balloon (Rashkind) atrio-septostomy in transposition of great vessels in the neonatal period].

Analysis of the balloon atrial septostomy (BA) for treatment of the transposition of the great arteries (TVG) during the neonatal period will allow the evaluation of some of the changes that have occurred in the diagnosis and therapy of this congenital cardiopathy. We performed a retrospective survey of the management, evolution complications of 24 BA as a palliative technique in TGV cases, admitted to the Neonatal Intensive Care Unit during a 5 1/2 year period. The aim of the study was to evaluate the factors that indicated a bad prognosis by comparing patients that are still alive (21) with those that are dead (3). The mean age at BA performance was 4.5 +/- 5.5 days. The biological constants prior to catheterization were normal, maintaining good oxygenation, administering prostaglandins in 80% of the patients and mechanical ventilation in 25% of the patients. BA outcome was considered as good in 68%, moderate in 12% and bad in 20% of the cases. Intra-catheterization complications, mainly hemorrhages and bradycardia, were suffered by 40% of the patients and one patient died during catheterization. We could see a progressive arterial desaturation due to the lack of efficacy of the BA. As bad post-catheterization outcome factors were found: hypotension, arrhythmia, hemorrhage, greater need of dobutamine, volume expansion and mechanical ventilation. Our principal conclusion is that, although BA has improved the prognosis of newborns with TGV, because of the progressive worsening of the patients during the months following the BA, it is necessary to perform early corrective surgery.

Catheterization↗

[Long-term results of surgical management of congenital aortic stenosis].

This retrospective study and review of the literature was undertaken to assess the long term results of valvotomy in congenital aortic stenosis (AS). One hundred and seven patients, aged between 14 days and 15 years (mean: 6.2 years), were operated on between 1966 and 1989. Thirty-three cases had valvular AS, 48 discrete subvalvular AS, 10 supravalvular AS and 16 combined AS. Thirty-three patients had associated cardiac anomalies. The surgical mortality was 6.5% (3.5% in children over 2 years of age). Three patients were lost. Ninety-seven patients had a follow-up period between 6 months and 16 years (mean: 5.4 years) with an actuarial survival of 95% at the age of 15 years. There were adverse events in 39 patients: AS, aortic regurgitation, bacterial endocarditis, third-degree heart block. Thirteen patients required re-operation and 4 balloon valvuloplasty; the late mortality was 5%. We conclude that this surgery has a low surgical mortality, but must be considered as palliative in most cases.

Adolescent↗

[Wolff-Parkinson-White syndrome: long-term follow up study in a pediatric population (86 cases)].

Eighty six patients (48 males and 38 females) with W-P-W diagnosed before the age of 15 years had a maximum follow-up of 20 years. The mean age at diagnosis was 3 y and 5 m., most cases being concentrated in the first 6 months of life (37%). Twenty seven (31.4%) had associated heart disease, the more common being: ventricular septal defect (6 cases), Ebstein malformation (5 cases) and transposition of the great arteries (4 cases). Supraventricular tachycardia (SVT) was the commonest form of clinical presentation, followed by examination because heart murmur or heart disease. Fifty patients (58%) had SVT along the follow-up. SVT was more frequent in W-P-W type A than in type B (p less than 0.05). There was a statistically significant correlation (p less than 0.001) between the association of SVT and the absence of heart disease, but the finding must be questioned because the true incidence of W-P-W in normal population is unknown. There also were a significant correlation (p less than 0.05) between the association of W-P-W type B and the presence of heart disease. An electrophysiologic study was performed in 11 patients, 6 of them being concealed W-P-W. Four patients were operated upon because of SVT. We have studied a total of 154 SVT episodes in the 50 patients with SVT. The treatment more frequently used to finish the SVT included: verapamil, DC-Countershock and Digoxin.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

[Anatomy and surgical implications of incomplete forms of atrioventricular septal defects].

We present an anatomic intraoperative study of 93 consecutive operated cases with an ostium primum defect. The type of the ostium primum, size of interauricular defect, position of the coronary sinus, tricuspid valve, mitral valve and subvalvular apparatus, are analyzed. Inside the mitral valve apparatus, the size, form, type and number of clefts are described as well as septal valve anomalies. We point out the importance of performing a detailed and fast anatomic study within a surgical protocol to obtain the optimal anatomic correction of the cardiopathy.

Child↗

[Cardiac surgery in the neonatal period. 17 years' experience].

Although the mortality rate with cardiac surgery in the neonatal period has decreased in the last years, due to the development of the neonatal intensive care units and the routinary use of prostaglandins, it remains higher than in whatever other ages. Since october 1967 to may 1984, 321 consecutive cases were operated on. Ages ranged between 12 hours and 31 days. In 301 cases closed cardiac surgery was performed, and cardiopulmonary bypass was employed in the remaining 20 cases. Taking off the mortality rate by periods of time, first (1968-1974), second (1975-1979), third (1980-1984), and by surgical groups (closed and open heart surgery) we found a statistically significant decrease in the mortality rate comparing the results over the time with the current period (1980-1984) having 114 cases of closed surgery and a 27% of mortality rate and 11 cases of open heart surgery with 2 deaths, 18% of mortality rate. Follow up was possible in 184 out of the 203 survivors, representing a 91% with a range between 3 month a 15 years. Medical and surgical management and long term follow up is described.

Aorta, Thoracic↗