[Criss-cross heart with atrioventricular discordance, straddling A-V valve, double outlet right ventricle and preexcitation].

We report the case of a newborn with cyanosis, dyspnea, and supraventricular tachycardia due to reentry with good response to medical therapy. The diagnosis was made by echocardiography. The patient died suddenly at 6 days of follow-up.

[The treatment of postoperative junctional ectopic tachycardia].

Objective: To evaluate treatment of junctional ectopic tachycardia after cardiac surgery.

Material And Methods: Twenty-seven patients (5.5 % of 488 patients who underwent surgery) were treated for junctional ectopic tachycardia between 1994 and 1998.

[Surgical correction of symptomatic ventricular septal defects in patients less than 6 months of age].

Objective: Our objective was to evaluate the efficiency of a single surgical intervention in patients with symptomatic interventricular septal defects during the first six months of life.

Patients And Methods: Between 1989 and 1997, 42 patients, 20 males and 22 females with an average age of 3.9 +/- 0.

[An anomalous origin of the left coronary artery in the right pulmonary artery with interventricular communication].

A six-month-old female baby with a left coronary artery of anomalous origin in the right pulmonary artery, associated with a interventricular septal defect and a slight mitral pathology, was operated on direct implantation of the left coronary artery with a small segment of the pulmonary artery in the posterior wall of the ascending aorta and closure of the perimembranous defect was performed. The patient has been asymptomatic for one year since surgery.

[Diagnostic value of reactive C protein in suspected acute appendicitis in children].

Objective: The aim of this study was to evaluate the accuracy of C-reactive protein (CRP) measurements in the diagnosis of suspected acute appendicitis (AA) during childhood.

Patients And Methods: A prospective study of 195 consecutive children, aged between 2 and 14 years and suspected of having AA, that attended a pediatric emergency room was carried out. We obtained a careful patient history, physical signs, blood test results, final diagnosis and the histological findings in the cases who underwent appendectomy (classified as normal appendix, simple appendicitis (SA) or gangrenous appendicitis (GA).

[Bacterial endocarditis in the aortic valve of a three month old infant. Correction with an aortic homograft].

We report the case of a three-month-old infant with an infective endocarditis of the aortic valve, showing perforation of two valve leaflets and a perivalvular abscess. The patient was operated on using a cryopreservated homograft with anastomosis of both coronary arteries. The outcome is good two years after surgery.

[Magnetic resonance of congenital cardiopathies].

Objective: The objective of this study was to determine the usefulness of magnetic resonance in the diagnosis and follow-up in congenital heart disease.

Patients And Methods: Between 1992 and 1994, we have evaluated 250 patients (between 1 day-22 years of age) with congenital heart disease. We used a Philips-Gyroscan 0.

[Aortic valve stenosis. Surgical treatment in children].

Objectives: The results of aortic commissurotomy, as the first step in the treatment of aortic valve stenosis in children, have been estimated after a medium-term follow-up.

Material And Methods: Twenty-two patients were operated at a mean age of 7.3 +/- 3.

[Tracheobronchial aspiration of foreign bodies in children].

Objective: Our objective was to study the characteristics and follow-up of children admitted to our Pediatric Emergency Room (PER) due to tracheobronchial inhalation of foreign bodies (FB).

Patients And Methods: We have reviewed retrospectively the 55 cases admitted over the last five years to our PER in whom the diagnosis of FB inhalation was confirmed.

Results: Mean age was 4 years with the peak incidence (25) occurring between 1 and 2 years of age.

Thirteen cystic fibrosis patients, 12 compound heterozygous and one homozygous for the missense mutation G85E: a pancreatic sufficiency/insufficiency mutation with variable clinical presentation.

To study the severity of mutation G85E, located in the first membrane spanning domain of the CFTR gene, we studied the clinical features of 13 Spanish patients with cystic fibrosis (CF) carrying this mutation. G85E accounts for about 1% of Spanish CF alleles. One patient was homozygous G85E/G85E and the rest were compound heterozygotes for G85E and other mutations (delta F508 nine patients, delta I507 two patients, and 712-1G > T one patient).

[Mononucleosis syndromes with serology doubly positive to Epstein-Barr virus and cytomegalovirus].

Objective: The objective of this study is to present cases of infectious mononucleosis syndromes (i.m.) with dual rises in antibodies towards Epstein-Barr virus (EBV) and cytomegalovirus (CMV).

[Acquired aortic coarctation after surgical operation of ductus arteriosus in a preterm infant].

The coarctation of the aorta can be acquired or secondary to the surgical correction of congenital anomalies of the great vessels. We report the cases of two newborns operated for patency of the ductus arteriosus (normal aortic arch and a great left-to-right-shunt). During the postoperative period the patients were asymptomatic and the echocardiogram showed no abnormalities.

[Surgical treatment of partial anomalous pulmonary venous drainage].

Objectives: We studied all patients operated for partial anomalous pulmonary venous drainage and half-time follow-up.

Materials And Methods: Twenty-eight patients with a mean age of 5 +/- 5 years (5 patients were less than 1 year old). There were sixteen male and twelve female patients.

[Pulmonary artery sling. A nuclear magnetic resonance study].

We present a symptomatic newborn infant with anomalous pulmonary artery (pulmonary artery sling) studied by magnetic resonance imaging. Of the ones diagnosed by this method, is the first case published in our country and the eight of all that have been reported until this moment. The different methods used for the diagnostic, are discussed.

Pathophysiology of the renal acidification defect present in the syndrome of familial hypomagnesaemia-hypercalciuria.

A distal acidification defect is frequently observed in the syndrome of familial hypomagnesaemia-hypercalciuria and hence this condition can be confused with primary distal renal tubular acidosis (RTA). This study demonstrates that in four unrelated patients with familial hypomagnesaemia-hypercalciuria the acidification defect is functionally different from that present in primary distal RTA. All patients exhibited hypomagnesaemia, hypermagnesuria, hypercalciuria, hyposthenuria, nephrocalcinosis and slight reduction of glomerular filtration rate (GFR).

[An imaging and color echo-Doppler study of interventricular defects].

Objective: Evaluation of ventricular septal defect with two-dimensional echocardiography and color flow Doppler.

Patients And Methods: We had studied by this method 180 patients; 97 males and 83 (mean age 1.5 +/- 1 years) diagnosed of a VSD.

[The usefulness of continuous Doppler in the diagnosis of aortic coarctation].

Were studied 32 patients with coarctation of the aorta by continuous wave Doppler ultrasound in order to assess the usefulness of this diagnostic method. Nineteen (59%) had native coarctation and 13 (41%) recoarctation. Seventeen (53%) were male and 15 (47%) female.

[Biventricular rhabdomyoma or a case of spontaneous regression. A two-dimensional echocardiographic study].

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.

[Aortic atresia with situs inversus].

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.