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.

[Dilated cardiomyopathy secondary to atrial ectopic tachycardia]. / Miocardiopatía secundaria a taquicardia auricular ectópica.

Ectopic atrial tachycardia is an uncommon cause of supraventricular tachycardia in children. It can resolve spontaneously or induce dilated cardiomyopathy. It is important to recognize this entity as a reversible cause of dilated cardiomyopathy, characterized by the presence of an abnormal focus located in the atrial myocardium and distinct to sinus node. Treatment strategies include the use of antiarrhythmic drugs, radiofrequency ablation, and, in patients refractory to medical treatment, surgical resection of the ectopic focus. We describe a patient with dilated cardiomyopathy due to an atrial ectopic focus and its resolution after medical treatment.

[Twin-twin transfusion syndrome and cardiovascular complications]. / Transfusión fetofetal y complicaciones cardiovasculares.

INTRODUCTION: Twin-twin transfusion syndrome carries high morbidity and mortality, related in part to the cardiovascular alterations that it produces. CASE REPORT: Patient 1 underwent a cesarian section at week 28 due to myocardial dysfunction and severe tricuspid regurgitation in the recipient twin. Twelve hours after birth the recipient twin developed refractory shock. Severe biventricular hypertrophy was observed and the twin died 60 hours after birth from multisystemic failure. Patient 2 underwent a cesarian section at week 29 due to cardiomegaly and cardiac insufficiency in the recipient twin. At birth, he showed hypertension and poor peripheral perfusion. Biventricular hypertrophy was observed, which increased until it produced a mild right midventricular stenosis at 72 hours, decreasing after the 8th day. Mild hypertrophy persisted at 6 months of life. DISCUSSION: Cardiovascular alterations appear only in recipient fetuses affected by twin-twin transfusion syndrome. The most frequent findings are cardiac dilation, hypertrophy, myocardial dysfunction, tricuspid regurgitation and pulmonary stenosis. These alterations usually disappear during the first few months of life, but may also worsen.

[Percutaneous closure of a mid-muscular residual ventricular septal defect using the Amplatzer(TM) device]. / Cierre percutáneo de una comunicación interventricular muscular residual mediante el dispositivo tipo Amplatzer.

We present the case of a 6-year old boy with a mid-muscular residual ventricular septal defect (VSD) of difficult surgical access, who underwent transcatheter closure using the Amplatzer(TM) VSD occluder. Transcatheter closure was guided by transthoracic echocardiography and successful closure was achieved with a 12 mm diameter VSD occluder, with no complications. Chest radiography showed a considerable decrease in cardiomegaly and normalization of pulmonary vascular markings before 24 hours, and echocardiography showed correct positioning of the Amplatzer(TM) VSD device without residual shunt through the device and minimal residual shunt in an apical VSD. After a 3-month follow-up, the patient was asymptomatic and echocardiography showed that the device was correctly positioned with minimal residual apical shunt.

Cierre percutáneo de una comunicación interventricular muscular residual mediante el dispositivo tipo Amplatzer / Percutaneous closure of a mid-muscular residual ventricular septal defect using the amplatzer device

Se presenta el caso de un niño de 6 años de edad, afectado de una comunicación interventricular (CIV) muscular residual medioventricular de difícil acceso quirúrgico, en la que se procedió al cierre mediante cateterismo con el dispositivo Amplatzer Muscular VSD Occluder. El procedimiento se realizó guiado por ecocardiografía transtorácica consiguiendo la implantación del dispositivo de 12 mm de tamaño sin incidencias ni complicaciones. A las 24 h el paciente fue dado de alta previa realización de una radiografía de tórax que muestra una disminución importante tanto de la cardiomegalia como de la circulación pulmonar y una ecocardiografía que evidencia el dispositivo correctamente situado en el tabique interventricular sin flujo a su través, y con una CIV residual mínima. Tras un seguimiento de 3 meses el paciente esta asintomático y la ecocardiografía muestra el dispositivo correctamente situado con mínima CIV residual apical (AU)

[Transposition of the great arteries and pulmonary hypertension: inhaled nitric oxide as a therapy and early surgical correction]. / Transposición de grandes arterias e hipertensión pulmonar: tratamiento con óxido nítrico inhalado y corrección anatómica precoz.

OBJECTIVE: The objective of this study is to present our preliminary experience with pulmonary hypertension inhaled nitric oxide therapy with two newborns presenting with transposition of the great arteries, as well as the usefulness of the Doppler in pulmonary hypertension diagnosis. PATIENTS AND METHODS: Revision of the variations of the hypoxic parameters according to the therapy and revision of the diagnostic procedures in both cases was carried out. RESULTS: Nitric oxide therapy was followed by an evident improvement of the hypoxia and the patient's hemodynamic stabilization, which allowed surgical correction in optimal conditions. The ductal flow pattern allowed recognition of the presence of pulmonary hypertension. CONCLUSIONS: Inhaled nitric oxide must be taken into account as a very useful option in the treatment of this kind of patient and the Doppler examination allows the evaluation of the presence of pulmonary hypertensions.

[Ebstein's anomaly of the tricuspid valve. Apropos 35 cases]. / Anomalía de Ebstein de la válvula tricúspide. A propósito de 35 casos.

Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect which consists of an apical displacement of the valvar annulus with a deformity of the septal and posterior leaflets. We report 35 cases studied from 1969 to 1993 (45% males and 55% females). One patient presented a polymalformation syndrome and another had a case of Ebstein's anomaly amongst his siblings. We performed an echocardiography in 31 patients and a catheterization in 15. The mean follow-up was 7.6 years (range: 1 day to 16.1 years). The diagnosis was established during the neonatal period in 66.7%. We found other associated cardiac anomalies in 57.1%, with radiologic cardiomegaly in 80.7%. Upon analysis of the EKGs, the P wave was higher than 0.3 mV in 48.4% and 16.1% showed Wolff-Parkinson-White syndrome. Paroxysmal supraventricular tachycardia developed in 12.9%. Long term cardiotonic treatment was needed in 27.3%. In 24.2% cardiac surgery was performed, including one heart transplantation. The mortality rate was 27.3%. The severity of Ebstein's anomaly is extremely variable, not only in its anatomy, but also in the clinical presentation. The severe neonatal form, the associated cardiac defects and heart failure worsen the prognosis.

[Usefulness of nuclear magnetic resonance in the diagnosis of aortic coarctation and recoarctation]. / Utilidad de la resonancia nuclear magnética para el diagnóstico de la coartación y recoartación aórtica.

OBJECTIVE: To assess nuclear magnetic resonance (NMR) usefulness in aortic coarctation and recoarctation diagnosis. MATERIAL: 46 NMR performed on 43 patients: aortic coarctation was suspected in 30 patients and recoarctation in 13 by means of clinical, echocardiographic and Doppler examination. METHOD: Comparison of the NMR observations with the previous diagnosis and the surgical and therapeutic catheterism findings. RESULTS: 41 patients were diagnosed of aortic coarctation or recoarctation by NMR, although a second examination was needed in 3 cases. This diagnosis was confirmed in the 26 treated children (surgical treatment or by angioplasty). CONCLUSIONS: NMR offers excellent aortic images; positive diagnoses have a high safety level, but patients with negative diagnoses must be clinically followed; children suffering from native coarctation can be operated without previous angiographic examination.