Assessing pulmonary hypertensive vascular disease in childhood. Data from the Spanish registry.

RATIONALE: There is a lack of knowledge regarding the epidemiology, clinical characterization, and survival in pediatric pulmonary hypertension. OBJECTIVES: To describe the epidemiology, outcomes, and risk factors for mortality in pediatric pulmonary hypertension in Spain. METHODS: We analyzed data from the Spanish Registry for Pediatric Pulmonary Hypertension. From January 2009 to June 2012, a total of 225 patients diagnosed with pulmonary hypertension in 1998 or after were collected from 21 referral and nonreferral centers. We included all Nice etiologies, estimated incidence and prevalence of pulmonary hypertension in the Spanish pediatric population, and analyzed risk factors for mortality (Nice etiologic group, clinical and hemodynamic variables). Patients were classified as follows: group I, pulmonary arterial hypertension (n = 142; 61%); group II, left heart disease (n = 31; 14%); group III, respiratory disease (n = 41; 18%); group IV, thromboembolic pulmonary hypertension (n = 2; 1%); or group V, mostly inherited metabolic diseases (n = 10; 4.5%). Of the patients studied, 31% had multifactorial pulmonary hypertension. MEASUREMENTS AND MAIN RESULTS: Mean age at diagnosis was 4.3 ± 4.9 years (50% < 2 yr). Survival rates at 1 and 3 years were 80 and 74% for the whole cohort, and 89 and 85% for patients with pulmonary arterial hypertension. Independent risk factors for mortality included an etiologic group other than pulmonary arterial hypertension (P < 0.001), age at diagnosis younger than 2 years old (P < 0.001), advanced functional class at diagnosis (P < 0.001), and high right atrial pressure at diagnosis (P = 0.002). CONCLUSIONS: In moderate to severe pediatric pulmonary hypertension, the prognosis is better in pulmonary arterial hypertension than in other Nice categories. In pediatric pulmonary hypertension age at diagnosis younger than 2 years is a risk factor for mortality, in addition to the previously established risk factors.

[Three-dimensional echocardiography: preliminary experience in congenital cardiac disease]. / Ecocardiografía tridimensional: experiencia preliminar en las cardiopatías congénitas.

INTRODUCTION: Anatomical comprehension of congenital cardiac diseases by 2D echocardiography is occasionally very difficult. 3D echocardiography provides a more spatial anatomical information avoiding the need of two-dimensional reconstruction. METHODS: Of the 271 cases studied 80 were foetal and 191 patients. In all cases, 2D and 3D echocardiography was performed (Sonos 7500 with matrix probe). Four modes of 3D imaging were used. RESULTS: 3D echocardiography gave an accurate description of the size, form and wedges of septals defects. In atrioventricular septal defects and mitral anomalies, 3D echocardiography was useful for the assessment of dynamic valve morphology and mechanisms of regurgitation. In foetal screening the segmentary heart study was carried out from a single acoustic window. CONCLUSIONS: 3D real time echocardiography is a feasible, easy and rapid technique. It provides anatomical and functional details needed for an accurate comprehension of congenital cardiac diseases. In foetal screening, it provides an easier segmentary analysis of the entire foetal heart.

[Transcatheter closure of patent ductus arteriosus using the Amplatzer duct occluder: initial results and mid-term follow-up]. / Cierre percutáneo del ductus arterioso persistente con dispositivo de Amplatz: resultado inmediato y seguimiento a medio plazo.

INTRODUCTION AND OBJECTIVES: Transcatheter closure of patent ductus arteriosus is a well-established procedure. The aim of this study was to assess the initial and mid-term results of the treatment of PDA with the Amplatzer duct occluder. PATIENTS AND METHODS: From October 1999 to December 2001, 30 children underwent transcatheter closure of persistent ductus arteriosus at a mean SD age of 5 4.02 years (range: 3 months to 14 years) and weight of 20.3 10.3 kg (range: 4.5-45 kg). Infants under 10 kg weight made up 46% of total patients. A lateral view aortogram was made to determine the morphology of the ductus and select the size of the device. Occlusion was achieved using the anterograde venous approach. Follow-up evaluations were made with chest X-ray and echocardiogram at 24 hours and 1, 4 and 12 months after implantation. RESULTS: Twenty-eight patients (93.3%) immediately achieved complete occlusion, and on color Doppler examination the closure rate was 100% within 24 hours of implantation. There was no device embolization. In the follow-up, a 19-month-old patient developed a 20 mmHg gradient across the aortic arch. CONCLUSIONS: Patent ductus arteriosus can be easily occluded with the Amplatz Duct Occluder, which is effective and particularly useful in infants and children with relatively large PDA. Further experience and long-term follow-up are still needed to assess the safety of this device in smaller children.

[Thyroid function alterations in children with congenital cardiac disease after catheterization with iodinated contrast agents]. / Alteraciones de la función tiroidea en niños con cardiopatía congénita tras la realización de cateterismo con contrastes yodados.

INTRODUCTION AND OBJECTIVES: Iodinated contrast agents can block thyroid hormone synthesis. The aims of this study were: 1st) to study the incidence of thyroid function disturbances in children with congenital heart disease after cardiac catheterization, 2nd) to analyze the predisposing factors that may lead to the development of hypothyroidism after angiography, and 3rd) to determine the duration of these hypothyroidism states. PATIENTS AND METHODS: From february 1993 to April 1997 thyrotropine (TSH) and free thyroxine (FT4) serum values were analyzed before cardiac catheterization and in the two following weeks, in 99 children under three years of age, with congenital cardiac disease. Those patients who showed any postangiography increase in TSH were further evaluated by weekly measures of serum thyroid hormones and TSH until normal values were obtained or until the initiation of hormonal replacement therapy. The patients' data (age, previous exposure to contrast agents, cardiac disease, associated extracardiac malformations, renal failure, severity of illness, treatment) and the catheterism data (the dose and type of contrast and the fluoroscopy time) were included in the univariant analysis. RESULTS: 10 mUI/ml), that persisted beyond three weeks in six cases. The occurrence of multiple malformation syndromes was the most clearly associated risk factor (p < 0,01) not only in the development of postangiography hypothyroidism but also in longer hormonal dysfunction. CONCLUSIONS: Thyroid function should be tested in every patient with multiple malformation syndrome that undergo angiocardiography with iodinated contrast agents.

[Percutaneous closure of atrial septal defect with the Amplatz device: initial result and mid-term follow-up]. / Cierre percutáneo de la comunicación interauricular mediante dispositivo de Amplatz: resultado inicial y seguimiento a medio plazo.

OBJECTIVES: Percutaneous device occlusion of atrial septal defects (ASD) is, although with some limitations, an alternative to surgical closure. The aim of this study was to evaluate the efficacy and safety of percutaneous ASD closure using the Amplatz device. PATIENTS AND METHODS: From October 1999 to March 2000, 25 children underwent transcatheter closure of ASD at a mean +/- SD age of 8.7 +/- 3.1 years (range 3-15 years) and a mean weight of 31.8 +/- 16.7 kg (range 11-84 kg). Device selection was based on the stretched diameter of the ASD using the PTA OS balloon. The device was implanted under ultrasonographic and radiological guidance. All patients showed signs of volume-overload of the right ventricle. The ASD was single (n = 21), with two separate holes (n = 2), or cribiform (n = 2). RESULTS: The median +/- SD size of the device used was 21.7 +/- 5.4 mm (range 15-36 mm). In twenty-two patients (88%) the device was successfully implanted. A repeat echocardiogram was performed the next day before discharge. Two patients underwent surgery after deployment of the device due to mitral valve dysfunction (n = 1) or residual leak (n = 1). In a patient with a two-hole ASD, another device was percutaneously withdrawn, while still attached to the delivery cable due to incomplete occlusion. CONCLUSION: a) Transcatheter occlusion with the Amplatzer device is an effective treatment for ostium secundum atrial septal defects; b) the low complication rate and the short hospitalization period makes this procedure the treatment of choice in these patients, and c) ASD which are too large, cribiform or with deficient rims may require a different approach.

[Pulmonary tuberculosis in infants: apropos of 149 cases]. / Tuberculosis pulmonar en la infancia: a propósito de 149 casos.

One hundred and forty-nine cases of childhood pulmonary tuberculosis were analysed being a 77.6% of the 192 cases of tuberculosis diagnosed at the Pediatric Infectious Diseases Department of "La Paz" Hospital, in a ten year period. The children were aged 3 months to 14 years; 65% of them were less than 6 years of age. The source case was found in 67.1% of the children. The primary means of diagnosis was contact screening (38.1%), followed by a 27.5% that presented with non-specific symptoms, while 18.1% of the cases presented with respiratory symptoms. Gastric aspirates yield the organism in 25.8% of cases. Radiologically the following was found: 38.9% mixed patterns, 32.2% nodal patterns, 23.4% parenchymal patterns, 1.34 miliary tuberculosis, 1.34% caverns and 2.68% pleural effusions. Right lung location was more frequent in parenchymatous forms (68%), as well as in nodal forms (61.5%). The duration of the course of therapy changed along this ten years period: from twelve months initially to ten months posteriorly, subsequently becoming a six month course of treatment. All cases experienced full recovery.

Hipertensión pulmonar en Pediatría / Pulmonary hypertension in pediatrics

En la hipertensión pulmonar (HTP) en el niño podemos encontrar prácticamente cualquiera de las etiologías de la clasificación de la OMS y patologías específicas de la edad pediátrica, como la hipertensión pulmonar persistente del recién nacido, o la asociada a enfermedades metabólicas, aunque sin duda el grupo más numerosos lo constituye el de la hipertensión pulmonar asociada a cardiopatía congénita. Carecemos de clasificaciones, documentos de consenso y protocolos diagnósticos y terapéuticos específicos para la HTP pediátrica, a la que por defecto se aplican los protocolos creados para la enfermedad del adulto. Sin embargo, en los últimos años, se ha puesto de manifiesto que el espectro de la HTP en el niño es mucho más complejo y heterogéneo que en el adulto, siendo muy frecuente asociación de síndromes polimalformativos o cromosomopatías en los enfermos pediátricos con HTP. La publicación de registros de HTP pediátrica, como el de Suiza, Reino Unido, Holanda o Francia ha aportado información sobre la epidemiología de esta enfermedad en los niños, con prevalencia para la HTP idiopática en torno a 3,7 casos /millón de habitantes (similares a las reportadas en los adultos). La forma más frecuente de HTP en el niño es la asociada a cardiopatía congénita (tanto reparada como no reparada), que supone más de 50% de todas las series. También se han publicado interesantes revisiones sobre el diagnóstico, sobre los valores normales para el test de los 6 minutos en niños y marcadores biológicos como el ác. Úrico o el BNP, que han facilitado la estratificación del riesgo de los pacientes pediátricos con HTP. Se revisan los distintos protocolos de tratamiento de la hipertensión arterial en el niño (AU)

In pulmonary hypertension (PHT) in the child, we may find almost any one of the etiologies of the WHO classification and specific conditions of the pediatric age, such as persistent pulmonary hypertension of the newborn, or that associated to metabolid diseases, although undoubtedly the most numerous group is made up of those with pulmonary hypertension associated to congenital heart disease. We lack classifications, consensus documents, and diagnostic and therapeutic protocols specific for pediatric PHT, for which, the protocols created for the disease of the adults are applied. However, in recent years, it has become manifest that the spectrum of the PHT in the child is much more complex and heterogeneous than in the adult, cases with multifactorial etiologies are common in the pediatric pulmonary hypertension, and also the association of multiple congenital malformation syndromes or chromosomopathies in the pediatric patients with PHT. The publication of registries of pediatric PHT, as that of Switzerland, the United Kingdom, Holland, or France, have provided information on the epidemiology of this disease in children, with prevalence’s for idiopathic PHT at approximately 3.7 cases/million inhabitants (similar to those reported in the adult). The most frequent form of PHT in the child is that associated with congenital heart disease (bot repaired and not repaired), that accounts for more than 50% of all the series. Interesting reviews have also been published on the diagnosis, on the normal values for the 6-minute test in children, and biological markers such as that of uric acid or BNP, that have facilitated the stratification of risk of the pediatric patients with PHT. The different arterial hypertension treatment protocols in the child are reviewed (AU)