Enfermedad de Kawasaki neonatal grave / Severe neonatal Kawasaki disease

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Implantación de stents en cardiología pediátrica. Resultados iniciales / Use of stents in pediatric cardiology. Initial results

Antecedentes y objetivo: Un stent es un dispositivo útil en el tratamiento de lesiones estenóticas vasculares asociadas a cardiopatías congénitas. En el año 1997 iniciamos nuestra experiencia, cuyos resultados se exponen en este trabajo. Pacientes y método: Se han implantado 17 stents, en 12 niños con edad media de 82 meses (2-168 meses) mediante 13 procedimientos. Ocho pacientes tenían estenosis de ramas pulmonares posquirúrgicas; 3 pacientes recoartaciones de aorta y uno, cardiopatía compleja dependiente del ductus. En los primeros 8 pacientes se efectuó cateterismo derecho y se siguió la técnica habitual de implantación con guía de alto soporte y vaina de Mullins. En los restantes el abordaje fue por vía arterial femoral. Se utilizaron 13 stents de Palmaz; 3 stents Express; y uno, Intrastent. Resultados: El diámetro de las lesiones se incrementó tras el implante desde 3,1 (2,8-5,6) hasta 12 (10-15) mm (p<=0,002), y el gradiente a través de las estenosis descendió desde 32 (21-45) a 8,5 (0-11,5) mmHg (p<=0,002). En el paciente dependiente del ductus permitió la suspensión de prostaglandinas y el alta hospitalaria. Tras un seguimiento medio de 27 meses (1-60) ha habido una oclusión completa tardía de rama pulmonar y una parcial de arteria lobular superior derecha. Un enfermo falleció por causas ajenas al stent (shock cardiogénico previo), el resto están asintomáticos y no precisan medicación. Conclusión: El stent es un dispositivo seguro y eficaz. Puede ser utilizado en una amplia variedad de lesiones estenóticas (AU)

[Use of stents in pediatric cardiology. Initial results]. / Implantación de stents en cardiología pediátrica. Resultados iniciales.

BACKGROUND AND AIM: Balloon-expandable stents are useful in treating vascular stenotic lesions associated with congenital heart defects. We report our experience of this device since 1997. PATIENTS AND METHOD: Seventeen stents were implanted in 12 children in 13 procedures. The mean age of the patients was 82 months (range: 2-168 months). Eight patients had postsurgical stenoses in branch pulmonary arteries, three patients had aortic re-coarctation and one patient had a ductus-dependent complex heart defect. In the first eight patients we performed right catheterization, following the usual technique of percutaneous implantation with super-stiff wire and Mullins sheath. In the remaining patients, vascular access was through the femoral artery. The Palmaz stent was used in 13 patients, Express stents in three and Intrastent in one patient. RESULTS: After stent implantation, the diameter of the narrowings increased from 3.1 mm (2.8-5.6) to 12 mm (10-15) (p < 0.002) and the gradient was reduced from 32 mmHg (21-45) to 8.5 mmHg (0-11.5) (p < 0.002). In the patient with ductus-dependent heart defect, the procedure allowed prostaglandin E1 withdrawal and hospital discharge. After a mean follow-up of 27 months (range: 1-60 months) one occurrence of late complete occlusion of branch pulmonary artery and one case of side-branch partial occlusion (right superior lobe branch) have been detected. One patient died from causes unrelated to the stent (prior cardiogenic shock). The remaining patients are asymptomatic and medication free. CONCLUSION: The stent is a safe and effective device that could be used in a wide variety of stenotic vascular lesions.

[The psychoneurological status of newborn infants with congenital heart disease before surgical intervention]. / Estado psiconeurológico de los recién nacidos afectados de cardiopatía congénita antes de su intervención.

AIMS: This study was conducted to evaluate, from a psychological and neurological point of view, the situation of newborn infants (NI) suffering from congenital heart disease before they are treated surgically. At the same time, we aimed to compare this group of patients with another group of children who were considered to be healthy. PATIENTS AND METHODS: Patients psychological development was evaluated using developmental landmarks from the Observational Scale of Development (OSD), which provides a percentage of elements acquired in different areas. Behaviour was evaluated by the observational recording of particular patterns of behaviour. The neurological study was based on a detailed neonatal examination, performed by people from the Child Neurology Section at our hospital, and which took into account the different behaviours displayed by the NI: reactive, grasping, communicative and affective. RESULTS: Findings show significant differences both in the psychological and neurological variables studied. They show how, generally speaking, healthy infants score higher in the areas of development that were evaluated than children suffering from heart disease. Likewise, lower average scores were observed in heart disease patients who require an early intervention, as compared with those who will be submitted to surgery later on. From the neurological point of view, a number of significant differences were found in their muscular activity. CONCLUSIONS: This analysis, a groundbreaker in the study of infants suffering from heart disease before their surgical intervention, shows how newborn infants who have been submitted to surgery in the neonatal period present a significant degree of hypotonia, a certain frailness and retardation in their motor development.

Estado psiconeurológico de los recién nacidos afectados de cardiopatía congénita antes de su intervención / The psychoneurological status of newborn infants with congenital heart disease before surgical intervention

Objetivo. Este estudio se llevó a cabo para evaluar, desde un punto de vista psicológico y neurológico, la situación de los recién nacidos que padecen una cardiopatía congénita, antes de tratarse quirúrgicamente, y, a su vez, comparar este grupo de pacientes con otro grupo de niños considerados sanos. Pacientes y métodos. El desarrollo psicológico se evaluó mediante hitos evolutivos procedentes de la escala observacional del desarrollo, la cual proporciona un porcentaje de elementos adquiridos en diversas áreas. El comportamiento se evalúa mediante el registro observacional de determinados patrones de conducta. El estudio neurológico se basó en un detallado examen neurológico neonatal, realizado por personas adscritas a la Sección de Neurología Infantil de nuestro hospital, en el que se tuvieron en cuenta las distintas conductas del recién nacido: reactiva, prensora, comunicativa y afectiva. Resultados. Los resultados muestran diferencias significativas, tanto en las variables psicológicas estudiadas como en las neurológicas. Muestran que, en general, los niños sanos obtienen puntuaciones más altas en las áreas de desarrollo evaluadas que los niños afectados de cardiopatías. Asimismo, se observan menores puntuaciones medias en los cardiópatas que deben intervenirse precozmente, que en los que se intervendrán con posterioridad. Desde el punto de vista neurológico, se encontraron unas diferencias significativas en la actividad muscular. Conclusiones. El presente análisis, pionero en el estudio de los niños cardiópatas antes de su intervención, demuestra que los neonatos que se han intervenido en el período neonatal ya presentaban, de forma significativa, hipotonía, cierta fragilidad y retraso en el desarrollo motor (AU)

Aims. This study was conducted to evaluate, from a psychological and neurological point of view, the situation of newborn infants (NI) suffering from congenital heart disease before they are treated surgically. At the same time, we aimed to compare this group of patients with another group of children who were considered to be healthy. Patients and methods. Patients’ psychological development was evaluated using developmental landmarks from the Observational Scale of Development (OSD), which provides a percentage of elements acquired in different areas. Behaviour was evaluated by the observational recording of particular patterns of behaviour. The neurological study was based on a detailed neonatal examination, performed by people from the Child Neurology Section at our hospital, and which took into account the different behaviours displayed by the NI: reactive, grasping, communicative and affective. Results. Findings show significant differences both in the psychological and neurological variables studied. They show how, generally speaking, healthy infants score higher in the areas of development that were evaluated than children suffering from heart disease. Likewise, lower average scores were observed in heart disease patients who require an early intervention, as compared with those who will be submitted to surgery later on. From the neurological point of view, a number of significant differences were found in their muscular activity. Conclusions. This analysis, a groundbreaker in the study of infants suffering from heart disease before their surgical intervention, shows how newborn infants who have been submitted to surgery in the neonatal period present a significant degree of hypotonia, a certain frailness and retardation in their motor development (AU)

[Fibrinolytic treatment with tissue plasminogen activator in a thrombosed modified Blalock-Taussig shunt]. / Tratamiento fibrinolítico con activador tisular de plasminógeno de una fístula modificada de Blalock-Taussig trombosada.

Thrombolytic therapy of a systemic pulmonary fistula is a rare method of treatment in newborns with thrombosed systemic-pulmonary shunt. We report the case of a newborn girl with a complex congenital heart defect. On the ninth day of life a modified right Blalock-Taussig shunt was performed. The ductus arteriosus was not ligated. Six days later the baby developed severe hypoxemia. The results of echocardiography and cardiac catheterization showed a total thrombosis of the fistula and complete absence of flow throughout the shunt. After insertion of an arterial catheter into the proximal end of the shunt we started fibrinolytic treatment with recombinant tissue plasminogen activator (r-TPA) in continuous infusion. After 14 hours of treatment we confirmed by angiography complete clot dissolution. The baby left the hospital in good condition when she was 23 days old. In the follow-up (4th month of life) the shunt is still permeable. Thrombolytic therapy with r-TPA locally applied in case of acute thrombosis of a systemic-pulmonary shunt appears to be a good therapeutic option avoiding the risks of a new surgical procedure.

[Progressive subaortic stenosis caused by accessory mitral valve tissue]. / Estenosis subaórtica progresiva causada por tejido mitral accesorio.

Accessory mitral valve tissue is a rare cause of subaortic obstruction. The reported case correspond to a two days old patient diagnosed by 2D-Echocardiography. Serial doppler flow analysis showed progressive left ventricular outflow obstruction. A cardiac catheterization at the age of 9 months confirmed the obstruction without defining the cause. Five months later, transesophageal echocardiography clearly defined the intraventricular connections of the accessory mitral tissue. At the age of 18 months the patient suffered from cardiac failure and underwent surgery for removing the accessory tissue, assisted by intraoperative transesophageal echocardiography pre and post by-pass. Follow-up echocardiography at 25 months showed no left ventricle outflow obstruction and the patient is now asymptomatic.

[Anatomic correction of transposition of great vessels with entire interventricular septum. Initial results]. / Corrección anatómica de la transposición de las grandes arterias con septo interventricular íntegro. Resultados iniciales.

INTRODUCTION: Optimal management for patients with simple transposition of great arteries is currently the arterial switch operation. We review our initial experience to evaluate the results. METHOD: From 1988 to 1993, 21 children with simple transposition of the great arteries underwent arterial switch operation. Mean age at surgery was 10.5 +/- 5.6 days, excluding two cases with two-stage arterial switch and one with late diagnosis. Patent ductus arteriosus was present in 12 cases, and a small ventricular septal defect in two. The coronary artery pattern was unusual in 9 cases. Balloon atrial septostomy was performed in 19 cases, seven of them using two-dimensional echocardiography. It was considered no necessary in the remaining two, with a large ductus arteriosus. RESULTS: Total circulatory arrest was used in 13 patients (mean time 29.7 +/- 22.6 min). Three patients died in the early postoperative period (14.3%) in a refractory cardiac failure, one of them secondary to myocardial necrosis. The coronary artery pattern was unusual in two of died patients. All patients but three were in sinus rythm. Mean age at follow-up was 21 months (follow-up range 2 months to 5 years). Seventeen of surviving patients are in a functional state grade I and one in grade II of NYHA. There haven't been late deaths. CONCLUSION: These results compared with the ones of atrial switch operation, have encouraged to us to use the arterial switch operation in all children with simple transposition of the great arteries. Increasing experience will likely lead to improve our results.

[Doppler echocardiographic evaluation of pulmonary hypertension in children]. / Valoración con ecocardiografía Doppler de la hipertensión pulmonar del niño.

INTRODUCTION: We analysed the usefulness of Doppler echocardiography to determine the presence and severity of pulmonary hypertension (PH) in children. METHOD: The whole group consisted of 63 patients, 42 with congenital heart disease that underwent cardiac catheterization (32 of whom had PH = study group) and 21 healthy children. These 21 patients and the remaining 10 without PH at cardiac catheterization made up the control group. All children were studied with Doppler Echocardiography to evaluate the pulmonary flow pattern with the sample volume placed in the pulmonary artery trunk, 1 cm distal to the pulmonic valve. The preejection period (PEP), ejection period (EP), acceleration time (AcT), the indexes PEP/EP, PEP/AcT, AcT/EP and the morphologic pattern of the pulmonary flow (type I: with peak flow velocity at midsystole; type II: with peak flow velocity in early systole; type III: with midsystolic notching) were analysed and quantitative parameters corrected according to hear rate by dividing theirs value by the square root of R-R interval. In the hemodynamic study we analysed the systolic (SPAP), diastolic and mean pulmonary artery pressure, and the mean pulmonary pressure/mean systemic pressure ratio (Pp/Sp). We compared the echocardiographic variables in both, study and control groups, and analysed the hemodynamic and echocardiographic correlation between the variables in question. RESULTS: Pattern I of pulmonary flow was associated with absence of PH and pattern II and III with PH (p < 0.001). The best results of quantitative variables were either corrected AcT (AccT) rather less in the study group than in control group (2.89 +/- 0.56 vs 4.05 +/- 0.56 ms, p < 0.001) and PPE/AcT index, 1.28 +/- 0.3 in the hypertensive group and 0.78 +/- 0.16 in the control group (p < 0.001). The best correlation were AcT with SPAP (r = -0.82) and Act with Pp/Sp ratio (r = -0.84). CONCLUSIONS: We consider that pulmonary flow analysed with Doppler echocardiography is a reliable, suitable and non-invasive method to evaluate PH in children.

[The hypoplastic left heart syndrome. Initiation of a therapeutic program]. / Síndrome del corazón izquierdo hipoplásico. Iniciación de un programa terapéutico.

The hypoplastic left heart syndrome is a very severe congenital heart disease dependent on patency of ductus arteriosus in the newborn. The survival after neonatal period, without surgical treatment, is exceptional. Nowadays, there are basically two types of therapeutic procedures: Palliation with the Norwood operation and/or cardiac transplantation. Both methods have showed advantages and disadvantages; at present, there is not consensus of them. In our hospital, we have recently begun a medical-surgical therapeutic program for the management of neonates with hypoplastic left heart syndrome. Because of this, we report our little experience. We have treated three children in the last year: The first of them dead in the operating room; the second was exitus due to a sepsis two months after surgery, and the third, who is three-month-old now, remained well and was discharged to home.

[Percutaneous transluminal pulmonary valvuloplasty. Results in 34 cases]. / Valvuloplastia transluminal percutánea pulmonar. Resultados en 34 pacientes.

Percutaneous transluminal valvuloplasty with catheter-balloon were performed in thirty-four patients with pulmonary valve stenosis, aged 2 to 11 years (mean = 4.8 +/- 2.4 years). It was a slight stenosis in 24 cases (70.5%) and severe in 10 cases (29.5%). In three of them it was a dysplastic valve. We achieved good results, since right ventricle systolic pressure, decreased from mean = 67 +/- 18 mmHg to mean = 23 +/- 12 mmHg (p less than 0.0001). In the three cases with dysplastic valve, results were rather poor. Procedure was well stood-up and there were no serious complication. We conclude that at the present time valvuloplasty its the elective treatment in pulmonary stenosis in childhood except in cases with dysplastic valve.

[Vertebral arteriovenous fistula and neonatal cardiac insufficiency: diagnosis using duplex Doppler echography]. / Fístula arteriovenosa vertebral e insuficiencia cardiaca neonatal: diagnóstico por ecografía Doppler dúplex.

An extracranial arteriovenous fistula case, placed in vertebral artery territory that dealt with congestive heart failure and which developed advantageously without a surgical treatment is shown. Authors emphasize the fact of being a rarity the so premature beginning of the signs in a arteriovenous communication at that level, results in Doppler duplex echography and the need to have in count this entity previous to congestive heart failure with a deep origin in neonatal period.