Fibrosis endomiocárdica tropical en nuestro medio / Tropical endomyocardial fibrosis in Spain

La fibrosis endomiocárdica es una miocardiopatía restrictiva de etiología desconocida, prevalente en regiones tropicales. Se caracteriza por la obliteración fibrótica del ápex de uno o ambos ventrículos, que puede extenderse a la cámara de entrada ventricular. Afecta predominantemente a niños y adultos jóvenes. No existe un tratamiento específico y suele tener un pobre pronóstico. No es una entidad frecuente en nuestro medio pero debido a la inmigración estamos presenciando un auge de la misma. Presentamos 4 casos clínicos de fibrosis endomiocárdica en niños diagnosticados y tratados en nuestro medio, y realizamos una revisión de la misma (AU)

Endomyocardial fibrosis is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions. It is characterized by fibrotic obliteration of the apex of one or both ventricles, which can be extended to the ventricular inlet chamber. It predominantly affects children and young adults. There is no specific treatment and usually has a poor prognosis. It is uncommon in Spain, but due to immigration, it appears to be increasing. Four cases of endomyocardial fibrosis in children diagnosed and treated in Spain are presented, as a well as a review of this disease (AU)

[Tropical endomyocardial fibrosis in Spain]. / Fibrosis endomiocárdica tropical en nuestro medio.

Endomyocardial fibrosis is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions. It is characterized by fibrotic obliteration of the apex of one or both ventricles, which can be extended to the ventricular inlet chamber. It predominantly affects children and young adults. There is no specific treatment and usually has a poor prognosis. It is uncommon in Spain, but due to immigration, it appears to be increasing. Four cases of endomyocardial fibrosis in children diagnosed and treated in Spain are presented, as a well as a review of this disease.

Defecto congénito de pericardio: a propósito de tres casos / Congenital pericardial defects: a report of three cases

Introducción: La atrofia prematura de la vena cardinal común producirá defectos en el pericardio y, a veces, también pleurales. Esos defectos pericárdicos pueden ser asintomáticos o, por el contrario, producir dolor inespecífico, dolor anginoso, isquemia miocárdica, embolias, arritmias y muerte súbita. Las pautas de actuación varían, desde la corrección quirúrgica de todo defecto a una actitud expectante.Casos clínicos: Presentamos tres casos de defecto congénito de pericardio, dos de ellos correspondientes a dos niñas lactantes de 3 y 11 meses respectivamente, en cuya radiografía de tórax se observó una alteración compatible con un defecto parcial de pericardio, confirmado con posteriores exámenes (ecocardiograma, angiocardiografía, resonancia magnética nuclear). El tercer caso se diagnosticó durante el transcurso de una intervención quirúrgica de ductus permeable, en la que pudo visualizarse directamente la ausencia de pericardio.Discusión: El interés de nuestros casos radica en la posibilidad de establecer un diagnóstico de presunción mediante la radiografía simple (en dos de ellos). Se concluye destacando la importancia de tener un alto índice de sospecha de anomalía congénita por el riesgo vital que comportan ciertos defectos parciales de pericardio ante la posibilidad de herniación ventricular

Introduction: Premature atrophy of the left common cardinal vein results in pericardial and pleuropericardial defects. Patients with these congenital defects can be entirely asymptomatic, but they may experience vague chest pain, angina, myocardial ischemia, emboli, dysrhythmia and sudden death. Practical guidelines suggest different approaches, from expectant management to surgical correction of the defects.Patients and method: We report three cases of this congenital entity. Two involved three-month-old and eleven-month-old girls with asymptomatic pericardial defects, discovered incidentally on chest X-ray and later confirmed by other techniques (echocardiography, angiocardiography, magnetic resonance imaging). The third case was diagnosed during surgical repair of a patent ductus in which the absence of pericardium was observed.Discussion: We highlight the fact that, in two of our cases, it was possible to establish a presumptive diagnosis on the basis of plain chest radiography. We conclude by stressing the importance of maintaining a high degree of suspicion for this congenital anomaly because of the life-threatening risk associated with certain partial pericardial defects should they result in ventricular herniation

[Pericardial defect with atrial herniation]. / Defecto pericárdico con herniación auricular.

Pericardial defects are rare in childhood and outcome is usually benign. Patients can be asymptomatic, but they may experience vague chest pain, angina, myocardial ischemia, emboli, and arrhythmia and some cases of sudden death have even been described in the literature. We report the case of a 3-month-old girl who, in the context of an episode of fever, underwent chest X-ray examination, which showed a left heart border anomaly. This finding strongly suggested a congenital partial pericardial defect, which was subsequently confirmed by other techniques. The interest of this case lies in the ability of the pediatrician to establish a presumptive diagnosis by examining the chest X-ray. We conclude by stressing the need for a high degree suspicion for congenital heart anomalies because of the life-threatening nature of some partial pericardial defects, which can lead to left-ventricular herniation.

Defecto pericárdico con herniación auricular / Pericardial defect with atrial herniation

Los defectos del pericardio constituyen una entidad poco común en pediatría con curso generalmente benigno. Los pacientes pueden estar asintomáticos, o presentar dolor inespecífico, dolor anginoso, isquemia miocárdica, embolias, arritmias e incluso se han descrito en la literatura médica casos de muerte súbita. Se presenta el caso de una niña lactante de 3 meses de edad, a quien, en el curso de un síndrome febril, se le realizó una radiografía de tórax, en la que se observó una alteración en el borde izquierdo de la silueta cardíaca. Dicho hallazgo era muy indicativo de un defecto parcial congénito de pericardio que fue confirmado con posteriores exámenes. El interés de nuestro caso radica en la posibilidad de establecer un diagnóstico de presunción por parte del pediatra al visualizar la radiografía de tórax. Se concluye destacando la importancia de tener un alto índice de sospecha de anomalía congénita por el riesgo vital que comportan ciertos defectos parciales de pericardio ante la posibilidad de herniación ventricular (AU)

[Clinical presentation, diagnosis and treatment of four cases of cor triatriatum]. / Presentación clínica, diagnóstico y tratamiento de cuatro casos de cor triatriatum.

We report four cases of cor triatriatum. In two of them the fibromuscular membrane was obstructive and associated with an atrial septal defect between the left atrial accessory chamber and the right atrium; in those cases the clinical findings were due to the pulmonary hypervascularity. The third case was associated to a small patent foramen ovale, and the clinical findings were due to the obstruction to flow through the membrane in the left atrium, producing venocapilar pulmonary hypertension. In these three cases surgical treatment was carried out through a right atriotomy. In all of them a good corelationship was found between the echocardiographic and the surgical findings. The follow-up has been good, all previous symptoms disappearing, remaining symptom free. The fourth case is an asymptomatic 5-year-old girl, with a non-obstructive membrane and without any associated anomalies.

Diagnóstico prenatal de rabdomioma cardiaco / Prenatal diagnosis of cardiac rhabdomyoma

Presentamos un caso clínico de diagnóstico precoz prenatal de rabdomioma cardiaco. El seguimiento ecográfico prenatal revela un aumento progresivo de tamaño de la masa tumoral. Ante la presencia de derrame pericárdico, se programa cesárea a las 38 semanas de gestación. Durante el periodo neonatal ha permanecido estable (AU)

Meandering right pulmonary vein: a case of scimitar variant.

We report a healthy, asymptomatic 15-year-old girl with a meandering right pulmonary vein draining to the left atrium. A meandering pulmonary vein may or may not be associated with the scimitar syndrome. The differential diagnosis with the scimitar syndrome and other forms of scimitar variants is discussed.

[Diverticulum of the right ventricle with biventricular myocardiopathy]. / Divertículo de ventrículo derecho con miocardiopatía biventricular.

We report a 18-days-old newborn boy with right ventricular diverticulum associated with biventricular cardiomyopathy, diagnosed by two-dimensional and Doppler echocardiography and angiography. Heart transplant was carried out at the age of four months, dying the next day. The histologic study showed endocardial fibroelastosis.

[Severe obstructions to outflow (and inflow) of the left ventricle in the newborn. Diagnostic and therapeutic heart catheterization]. / Cateterismo cardíaco diagnóstico y terapéutico.

The findings of cardiac catheterization and angiocardiography in newborns with severe obstruction to the left ventricular inflow and outflow tracts are commented. It is analyzed too the actual situation of mitral and aortic valvuloplasty in this patients.

[Neonatal cardiac insufficiency caused by a cerebral arteriovenous fistula. Review of the authors' cases]. / Insuficiencia cardiaca neonatal por fístula arteriovenosa cerebral. Revisión de nuestros casos.

UNLABELLED: Four cases of newborn infants presenting congestive heart failure in the first two weeks of life with the clinical suspicion of cerebral arteriovenous fistula which was subsequently confirmed by cerebral echography or TAC and, after death, by necropsy, are described. Only one patient survived, the one treated surgically. CONCLUSION: It is necessary to carry out urgent diagnosis with an early surgical operation, the only possibility of survival for these children.

[Myxoma of the right atrium]. / Myxome de l'oreillette droite.

A case of right atrial myxoma is reported in a 6-year-old boy. This type of cardiac tumour is infrequent at this age and its localisation on the right atrium is extremely rare. The clinical diagnosis may be evoked in the presence of a changing cardiac murmur; two-dimensional cardiac ultrasonography is currently the best method for non-invasive assessment. Good surgical results are linked to an early resection, with careful manipulation of the tumour and large removal of the implantation base.

[Aneurysm of the interauricular septum: an exceptional cause of late systolic click. Echocardiographic diagnosis]. / Aneurisma del tabique interauricular: causa excepcional de chasquido sistólico tardío. Diagnóstico ecocardiográfico.

A 54 years old patient was referred for echocardiographic study because of a non-ejective click, diagnosed as mitral valve prolapse syndrome. 2d and M mode ultrasonic study showed a normal mitral valve and atrial septum aneurysm. The pathogenic and echocardiographic features of the inter atrial septum aneurysms are discussed.

[Spontaneous pneumopericardium in a 9-year-old child]. / Pneumopéricarde spontané chez un enfant de 9 ans.

The authors report the first case of spontaneous pneumopericardium of unknown cause, in their hospital, affecting an adolescent who had not had any previous asthmatic process. The differential diagnosis, evolution and treatment are commented.

[Acute rheumatic carditis. Follow-up (author's transl)]. / La carditis reumática en nuestro medio. Clínica y evolución a largo plazo.

Thirteen cases with a first attack of acute theumatic carditis are presented, analyzing the clinical picture and long-term follow-up. Although the clinical acute attack was quite benign, incidence and importance of residual heart disease has not changed.