[Restrictive myocardiopathy in children. A study of 4 patients]. / Miocardiopatía restrictiva en el niño. Estudio de cuatro pacientes.

Restrictive cardiomyopathy is an unusual form of cardiomyopathy during childhood. Only occasional cases or reduced populations have been reported in international journals. The purpose of this report is to document the clinical, echocardiographic and hemodynamic profile and outcome of restrictive cardiomyopathy in a group of four children (two boys and two girls). The mean age at admission was 3.5 +/- 2.7 years (range 1(3/12) to 7(10/12) years). The diagnosis was made by clinical, echocardiographic and hemodynamic criteria. The main symptoms were bronchial disease (two patients), hepatomegaly and a cardiac murmur. All of the patients had impaired growth and physical development, normal or near-normal Rx cardiothoracic ratio, evidence of congestive heart failure and only one of them showed a cardiac murmur. The most common ECG finding was biatrial enlargement and incomplete right-bundle branch block. The echocardiographic feature was severe biatrial dilatation in the presence of normal ventricular cavity size. Left ventricular shortening was normal and there was diastolic dysfunction. Doppler mitral flow was restrictive, with an E/A ration > 1.5. Marked elevation of left ventricular end-diastolic pressure was noted in cardiac catheterization (x = 32 +/- 7 mmHg). Right ventricular end-diastolic pressure was elevated, but less than the left side (x = 19 +/- 12 mmHg). The outcome was very poor. Two patients died, one remains with heart failure NYHA II and the other with heart failure NYHA IV. We conclude that restrictive cardiomyopathy during childhood may simulate a bronchial or liver disease, so we must bear this in mind in order to make the differential diagnosis. Because of the poor prognosis and lack of specific treatment, the best therapeutic option may be heart transplantation.

[Severe aortic stenosis in the neonate and infant under 6 months old]. / Estenosis aórtica severa en el recién nacido y lactante menor de seis meses.

24 patients with diagnosed aortic stenosis are reported. Clinical symptoms started before 6 months of age (between 24 hours and 6 months, average 87 days). In all cases aortic stenosis was the only existing malformation, except in one patient who had a small muscular ventricular septal defect and in another case who had a patent ductus arteriosus. Clinical symptoms were early and notable. In nineteen there was congestive heart failure whose appearance in the first week of like was a bad prognosis. Relationship between severity of aortic stenosis and electrocardiographic findings was closer than in older patients. Death rate was very high in children who had not been operated (5 out of 12). Of children who received surgery only one died out of 12. Treatment, therefore, in this pathology must be aggressive.

[Aortic coarctation in children. Analysis of 100 cases]. / Coartación de aorta en el niño. Análisis de 100 casos.

One hundred clinical records of coarctation of aorta are presented with 78% catheterization and 31% postmortem examination. Surgical treatment was performed in 46%, 8, in the first month of life, 13 from 1 to 6 month, 5, from 6 to 12 month, 9 from 12 to 24 month and 11 older than 2 years. The surgical mortality was 13%. There was recoarctation in 7.5%. The mortality in the patients not operated on was 31%. This mortality was influenced by the severity of associated malformations. The most frequent associated cardiac malformations were left to right shunts followed by left heart pathology. The association with complex cardiopathies was frequent.

[Oto-cranio-facial syndromes with asymmetrical involvement of the face]. / Síndromes otocraneofaciales con afectación asimétrica de la cara.

Four cases otocraniumfacial syndromes with asymmetrical affectation of the face are presented. Clinic, radiologic and genetic studies are made. Authors comment the difference between Goldenhar's syndrome and hemifacial microsomia. Finally the etiopathogenic thesis, differential diagnostics and treatment are commented.

[Kawasaki's disease. a case presentation]. / Síndrome ganglionar mucocutáneo (enfermedad de Kawasaki) A propósito de un caso.

A case of Kawasaki's disease in a girl of 2 10/12 years of age is presented. She had a typical clinical picture, without cardiovascular afectation, and with good final results. Apparently this is the third case reported in the Spanish medical literature. Attention is called to the low incidence of MLNS in the western countries--only 50 cases reported--as compared with the great number reported from Japan. The problems of diagnosis and pathogeny are discussed, particularly their intimate relation to infantile polyarteritis nodosa, because of the similarities in the cardiovascular alterations in both processes, above all in mortal cases.

[Coarctation of the aorta. In infancy (author's transl)]. / Coartación aórtica en el lactante.

We review our experience in 38 patients with coarctation of the aorta during infancy. Cardiac failure was present in 30 patients, being the maximal incidence during the first and second weeks of the life. Sixteen infants died, 43% of them during the first week. Cardiac catherization and angiocardiography were performed in 22 infants. The coarctation of the aorta was isolated in 38 infants (36%). The most frequently associated malformations were: patent ductus arteriosus (6 cases), ventricular septal defect (5 cases) and the pathology of the left heart. The post mortem examination was performed in 11 infants; in all of them the CoAo was preductal with patent ductus arteriosus; the most frequently associated malformation was ventricular septal defect (6 cases). 5 infants were operated upon with succes during the first year of the life.

[Di Giorge's syndrome with congenital heart disease (author's transl)]. / Síndrome de Di Giorge Estudio cardiológico en tres casos.

Authors describe three cases of Di Giorge's syndrome (thymic and parathyroid aplasia) proved through anatomical study postmorten, associated with cardiac malformations (Tetralogy of Fallot with pulmonary atresia, persistent truncus arteriosus and large patent ductus arteriosus). Clinical characteristics of the stated syndrome are analized with special reference to congenital heart diseases that are included in it.

[Diagnostic of the heart tumors in newborn and children (author's transl)]. / Diagnóstico de los tumores cardíacos en el recién nacido y lactante

Heart tumors in children are rare. Though of benign pathology they are clinically malignant and should be diagnosed early and promptly treated by surgery in spite of the poor results usually obtained. Any heart enlargement in the absence of congenital heart disease accompanied by rhythm and conduction troubles must be considered as suspect. Five cases of heart tumor are reported and clinically described. Four occurred in newborns and the remaining one before the age of one. Heart enlargement was a constant finding. The ECG findings were: W.P.W. syndrome, intraventricular conduction troubles and ventricular tachycardia, wandering pacemaker (one case each) and ventricular hypertrophy with surprisingly low voltages in the corresponding chest leads (two cases).

[Congenital heart block and pacemaker implantation (author's transl)]. / Bloqueo cardíaco congénito con implantación de marcapaso

A six years old boy was sent to our service due to a loss of conscience spell. He had no preceding history E.K.G. showed a complete A-V block. Laboratory findings were within normal limits. Cardiac catheterism was performed and it ruled out any antomic abnormality, making evident a good contraction ability. To decide pacemaker implantation we evaluted: 1. That cardiac frequency was not increased by exercise neither by action of pharmacologyc agents (atropine, insoproterenol).-2. The existence of wide and bizarre QRS ocmplex.-3. And dizziness spels. The patient has been controlled during the past two years. Parasistolia has appeared, not showing modification till today.