Practice patterns of pulmonary hypertension secondary to left heart disease among pediatric pulmonary hypertension providers.

Development of pulmonary hypertension (PH) in patients with left side heart disease (LHD) is a predictor of poor prognosis. The use of pulmonary vasodilators in PH associated with LHD (PH-LHD) is controversial. In this study, we describe the practice patterns regarding the use of pulmonary vasodilators in PH-LHD among a group of international pediatric PH specialists. A survey was distributed to the members of three pediatric PH networks: PPHNet, PVRI, and REHIPED. The survey queried participants on the rationale, indications, and contraindications of the use of pulmonary vasodilators in children with PH-LHD. Forty-seven PH specialists from 39 PH centers completed the survey. Participants included PH specialists from North America (57%), South America (15%), and Europe (19%). The majority of participants (74%) recommended the use of pulmonary vasodilators only in patients with combined pre-capillary and post-capillary pulmonary hypertension. Participants required the presence of clinical symptoms or signs of heart failure (68%) or right ventricular dysfunction by echocardiography (51%) in order to recommend pulmonary vasodilator therapy. There was no agreement regarding hemodynamic criteria used to recommend pulmonary vasodilators or the etiologies of LHD considered contraindications for using pulmonary vasodilators to manage PH-LHD. Of the available PH-targeted drugs, most participants preferred the use of phosphodiesterase-5-inhibitors for this indication. In conclusion, the practice of recommending pulmonary vasodilators in PH-LHD is highly variable among international pediatric PH specialists. Most specialists of those surveyed (57% in North America) would consider the use of pulmonary vasodilators in PH-LHD only if pre-capillary pulmonary hypertension and right ventricular dysfunction are present.

Cateterismo intervencionista tras cirugía tipo Norwood / Interventional catheterization after the Norwood procedure

Introducción y objetivos. Análisis retrospectivo de las indicaciones, los resultados y las complicaciones del cateterismo terapéutico en pacientes tras cirugía tipo Norwood. Métodos. Entre febrero de 1993 y diciembre de 2006 se realizaron 25 procedimientos intervencionistas en 14 pacientes con cirugía tipo Norwood, previo al Glenn o Fontan. Resultados. En 7 de los 14 pacientes se realizaron 9 angioplastias de recoartación (2 presentaron nuevamente reestenosis tras la primera angioplastia). En 3 pacientes con Norwood clásico se encontró inicialmente o evolutivamente desconexión de arteria pulmonar izquierda; 7 pacientes precisaron 10 angioplastias de arterias pulmonares; 3 pacientes precisaron embolizaciones: de colaterales venosas (n = 1, con coils), de vena cava superior izquierda (n = 1, con oclusor Amplatzer para ductus) y de fístula Blalock-Taussig izquierdo (n = 1, con oclusor Amplatzer para ductus). En 2 casos se realizó angioplastia del Glenn y en otro, fibrinolisis de trombosis de vena cava superior y arteria pulmonar. Otros hallazgos, sobre los que no se realizaron técnicas intervencionistas, fueron: estenosis de troncos supraaórticos (n = 6), estenosis en las arterias femorales (n = 2), trombosis venosa femoral (n = 5) o de vena subclavia (n = 1). Como complicaciones se registraron: isquemia arterial (n = 2), parada cardiaca o bradicardia (n = 4) y bloqueo auriculoventricular transitorio (n = 1). Conclusiones. Tras la cirugía tipo Norwood debe realizarse una valoración angiohemodinámica precoz para detectar y tratar estenosis de arterias pulmonares y/o del arco aórtico. Aunque la angioplastia de la recoartación puede ser efectiva, la reestenosis es frecuente. La técnica de Sano evita la desconexión de la arteria pulmonar izquierda, pero no el desarrollo de estenosis de arterias pulmonares. Tras el estadio II, debe estudiarse si hay colaterales venovenosas, pues éstas con frecuencia precisan embolización. El cateterismo terapéutico en estos pacientes comporta una incidencia de complicaciones mayor que en otros grupos de pacientes (AU)

Introduction and objectives. To carry out a retrospective analysis of the indications for, and the results and complications of interventional catheterization after the Norwood procedure. Methods. Between February 1993 and December 2006, 25 interventional catheterizations were performed in 14 patients who had undergone the Norwood procedure, prior to the Glenn or Fontan procedure. Results. Nine angioplasties were carried out for recoarctation in seven of the 14 patients (2 patients developed restenosis after their first angioplasty). Detachment of the left pulmonary artery occurred either immediately or during follow-up in 3 patients who underwent the classical Norwood procedure. Overall, 10 pulmonary artery angioplasties were required in 7 patients. Three patients needed embolization: one of venous collaterals (using coils), one of the left superior vena cava (using an Amplatzer duct occluder), and one of a left Blalock-Taussig shunt (using an Amplatzer duct occluder). Two patients required a cavopulmonary (Glenn) anastomosis, and another underwent fibrinolysis for thrombosis of the superior vena cava and pulmonary artery. Other findings, which were not treated percutaneously, included: stenosis of the supra-aortic trunk (n=6), femoral artery stenosis (n=2), femoral vein thrombosis (n=5), and subclavian vein thrombosis (n=1). The following complications were recorded: arterial ischemia (n=2), cardiac arrest or bradycardia (n=4), and transient atrioventricular block (n=1). Conclusions. Following stage I of the Norwood procedure, the angiographic and hemodynamic assessments needed for the diagnosis and treatment of pulmonary artery or aortic arch stenosis must be carried out promptly. Although treating recoarctation by angioplasty can be effective, restenosis frequently occurs. With the Sano procedure, detachment of the left pulmonary artery, but not pulmonary artery stenosis, can be avoided. After stage II, the presence of venovenous collaterals must be ruled out, because they frequently require embolization. In these patients, interventional catheterization is associated with a higher incidence of complications than in other groups (AU)

Miocardiopatía hipertrófica: baja frecuencia de mutaciones en el gen de la cadena pesada de la betamiosina cardiaca / Hypertrophic Cardiomyopathy: Infrequent Mutation of the Cardiac Beta-Myosin Heavy- Chain Gene

El objetivo del presente estudio es la búsqueda de mutaciones en el gen de la cadena pesada de la betamiosina cardiaca (MYH7b) en pacientes españoles con miocardiopatía hipertrófica. Se incluyó en el estudio a 36 familias con al menos un individuo enfermo de miocardiopatía hipertrófica. Se secuenció el ADN de los exones 3 al 24 del gen de la cadena pesada de la betamiosina cardiaca (gen MYH7b). Se ha identificado 2 mutaciones (Arg858Cys y Met515Val) en 2 familias distintas, una de ellas de origen marroquí, lo que representa una frecuencia de mutaciones en el gen MYH7b inferior al 5%. Al contrario de lo que ocurre en otras poblaciones caucásicas, las mutaciones en el gen MYH7b en este grupo de familias españolas con miocardiopatía hipertrófica son poco frecuentes (AU)

The aim of this study was to identify mutations in the cardiac heavy-chain beta-myosin gene (MYH7b) in a group of Spanish patients with hypertrophic cardiomyopathy. The study included 36 families with at least one member who had hypertrophic cardiomyopathy. DNA from exons 3 to 24 of the MYH7b gene was sequenced. Two mutations were identified: Arg858Cys and Met515Val. They occurred in two families, one of which was of Moroccan origin. This corresponds to a MYH7b gene mutation frequency of less than 5%. In contrast to findings in other Caucasian populations, MYH7b gene mutation occurred infrequently in this group of Spanish families with hypertrophic cardiomyopathy (AU)

Angioplastia con balón de la coartación nativa en la edad pediátrica: resultado inicial y a medio plazo / Balloon angioplasty for native coarctation in children: immediate and medium-term results

Introducción y objetivos. La angioplastia percutánea de la coartación nativa en la edad pediátrica es una técnica controvertida. El objetivo de este estudio es aportar nuestra experiencia con esta técnica, y comparar retrospectivamente los resultados obtenidos en 2 períodos distintos. Sujetos y método. Los 53 pacientes tratados con angioplastia se dividieron en 2 grupos: a) tratados entre 1985 y 1988 (n = 26), y b) tratados entre 1993 y 2003 (n = 27). Se realizó seguimiento en todos.Resultados. La angioplastia fue inicialmente efectiva (gradiente < 20 mmHg) en 18/26 pacientes del grupo A (69,2%), y en 22/27 (81,5%) en el grupo B. Presentaron recoartación el 33% del grupo A y el 25,6% del grupo B. Los pacientes recoartados del grupo A fueron derivados a cirugía, mientras que en 5 pacientes del grupo B se realizó una segunda angioplastia. Al final del seguimiento, se había conseguido una angioplastia efectiva en el 62% (16/26) en el grupo A frente al 85% (23/27) en el grupo B. La incidencia de complicaciones graves fue menor en el grupo B (4%) que en el A (19,2%), así como la de aneurismas (grupo B: 4%; grupo A: 15%). Conclusiones. La angioplastia percutánea es una alternativa eficaz en el tratamiento de la coartación nativa, circunscrita. La mejor selección de los casos, la utilización de balones de menor perfil y la optimización de los cuidados generales puede disminuir la incidencia de complicaciones. La aplicación de una segunda angioplastia (simple o con stent) en los casos de recoartación ha mejorado los resultados de esta técnica (AU)

Introduction and objectives. The use of balloon angioplasty to treat native aortic coarctation in pediatric patients is controversial. Our aims were to report our experience with this technique and to compare retrospectively the immediate and medium-term results obtained during 2 distinct time periods. Subjects and method. 53 patients who underwent balloon angioplasty for native coarctation were divided into 2 groups: (A) those treated between 1985-1988 (n=26); and (B) those treated between 1993-2003 (n=27). Follow-up data were available for all patients. Results. The immediate result was good (i.e., pressure gradient, <20 mmHg) in 18/26 patients in group A (69.2%) and 22/27 (81.5%) in group B. On follow-up, the recoarctation rate was similar in both groups: 33% in group A and 25.6% in group B. Group A patients with recoarctation were referred for surgery, whereas group B patients underwent a second angioplasty (either balloon or stent). At the end of follow-up, angioplasty had been successful in 62% (16/26) of patients in group A vs 85% (23/27) in group B. The incidence of serious complications was lower in group B (4%) than group A (19.2%), as was the incidence of aneurysm: 4% in group B vs 15% in group A. Conclusions. Balloon angioplasty is an effective alternative to surgery for the treatment of native, localized aortic coarctation. Better selection of suitable patients, use of low-profile catheters, and improved patient care can reduce the incidence of complications. Repeat angioplasty (either balloon or stent) in cases of recoarctation has improved results with this technique (AU)