Jaume Casaldáliga

Hypertrophic cardiomyopathy in mucopolysaccharidoses: Regression after bone marrow transplantation

SummaryMucopolysaccharide storage disease (MPS) presents clinically with a broad spectrum of abnormalities, among which cardiovascular involvement has been described.The echocardiographic findings have recently been reported for the various types of MPS. Among these, asymmetric septal hypertrophy (ASH) has been documented.We present a case of a 9-year-old girl suffering from type I MPS, atypical variant, with echocardiographic signs of ASH. She was given a bone marrow transplant after which the hypertrophic cardiomyopathy regressed.

Eplerenone in systemic right ventricle: Double blind randomized clinical trial. The evedes study☆ , ☆☆

BACKGROUND There is no proven pharmacological strategy for the treatment of the failing systemic right ventricle (SRV) but myocardial fibrosis may play a role in its pathophysiology. METHODS We designed a double-blind, placebo-controlled clinical trial to assess the effects of eplerenone 50mg during 12 months on cardiac magnetic resonance parameters (SRV mass and ejection fraction) and neurohormonal and collagen turnover biomarker (CTB) levels. RESULTS Twenty six patients with atrial switch repair for transposition of the great arteries were randomized to eplerenone (n=14) or placebo (n=12) and 14 healthy volunteers served as controls for comparison of baseline neurohormones and CTB levels. The study population showed a good baseline profile in terms of SRV mass (57.4 ± 17 g/m(2)) and ejection fraction (54.9 ± 7.5%). However, levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), C terminal propeptide of type I procollagen (CICP) and C-terminal Telopeptide of type I Collagen (ICTP) were significantly elevated when compared to healthy controls. After one year of treatment, a trend toward reduction of CICP, N-terminal pro-Matrix Metalloproteinase 1 (NT-proMMP1), Tissue Inhibitor of Metalloproteinases 1 (TIMP1) and galectin 3 levels and a lower increase in ICTP in patients under eplerenone was observed. The reduction of SRV mass and the improvement of SRV function with eplerenone were not conclusive. CONCLUSIONS Patients with SRV treated with eplerenone showed an improvement of an altered baseline CTB profile suggesting that reduction of myocardial fibrosis might be a therapeutic target in these patients.

Embarazo y cardiopatías congénitas

Introduccion y objetivos Desde la creacion de las Unidades de Cardiopatias Congenitas (CC) del Adulto y las Unidades Obstetricas de Alto Riesgo Cardiologico, ha habido creciente interes por la evolucion hemodinamica y obstetrica de embarazadas con CC. Metodos Estudio descriptivo retrospectivo de 56 mujeres con CC y media de edad de 25 (18-40) anos, que iniciaron 84 gestaciones entre enero de 1992 y agosto de 2006. Se las distribuyo en 3 grupos de riesgo gestacional: A, bajo; B, moderado y C, alto. Resultados Las incidencias de complicaciones durante la gestacion fueron del 1,6, el 15 y el 20%, y durante el puerperio, el 2, el 23 y el 50%; la mortalidad materna fue 0, del 7,6 y del 25% de los grupos A, B y C respectivamente. Nacieron 69 ninos y las tasas de prematuridad fueron del 11, el 15 y el 100% respectivamente. Los factores de riesgo principales fueron: la hipertension pulmonar (HTP), la cianosis, la arritmia, la obstruccion del tracto de salida del ventriculo izquierdo, el ventriculo derecho (VD) dilatado, el VD sistemico necesidad de y la anticoagulacion. La HTP fue el factor mas importante asociado a morbimortalidad maternofetal. Conclusiones La estratificacion por riesgo en las gestantes con CC ofrece informacion pronostica que permite adecuar la atencion de equipos multidisciplinarios para conseguir resultados exitosos.

Embolización percutánea de fístulas vasculares con el tapón vascular de Amplatzer o coils

Introduccion y objetivos Hay gran variedad de fistulas vasculares que son remitidas a los laboratorios de cateterismo cardiaco para su diagnostico y oclusion percutanea. Existe tambien una amplia gama de dispositivos utilizables en su tratamiento percutaneo. El objetivo es evaluar la utilidad, las dificultades, las complicaciones y los resultados en el tratamiento percutaneo de las fistulas vasculares mediante el uso de coils de liberacion controlada o tapones vasculares de Amplatzer. Metodos Revision retrospectiva de las embolizaciones percutaneas realizadas desde enero de 2004 a junio de 2008. Resultados Se embolizaron 51 fistulas vasculares en 30 pacientes con edades entre 6 dias y 28 anos (media, 8,4 anos), con buen resultado. Diagnosticos: 27 colaterales venosas en 16 pacientes con Glenn; 4 fistulas quirurgicas (Blalock-Taussig); 11 fistulas arteriovenosas pulmonares en 3 pacientes; 2 arterias colaterales aortopulmonares en 2 pacientes; 1 colateral venosa en 1 paciente con Fontan previo; 1 arteria aortopulmonar en un sindrome de cimitarra; 1 fistula arteriovenosa coronaria; 3 fistulas arteriovenosas sistemicas en 1 recien nacido, y una fistula entre auricula izquierda y vena cava superior tras la cirugia de un retorno venoso pulmonar anomalo. En el tratamiento percutaneo de estas lesiones se emplearon 34 tapones vasculares y 19 coils. Conclusiones Las fistulas vasculares pueden ser ocluidas percutaneamente con buen resultado. Los coils permiten el cierre de fistulas de menor diametro y los tapones vasculares estan indicados en las mas amplias. Ambos dispositivos alcanzan una alta eficacia oclusora y no se han observado especiales dificultades ni complicaciones significativas.

Percutaneous embolization of vascular fistulas using coils or Amplatzer vascular plugs.

INTRODUCTION AND OBJECTIVES A great variety of different types of vascular fistula are referred to cardiac catheterization laboratories for diagnosis and percutaneous occlusion. In addition, a wide range of devices is available for treating them percutaneously. The objectives of this study were to assess the usefulness and difficulty of treating vascular fistulas percutaneously using controlled-release coils or Amplatzer vascular plugs and to report on the complications and overall outcomes observed with these two devices. METHODS Retrospective review of percutaneous embolizations performed from January 2004 through June 2008. RESULTS In total, 51 vascular fistulas in 30 patients aged from 6 days to 28 years (mean, 8.4 years) underwent successful embolization. The underlying diagnoses were: 27 venous collaterals in 16 patients after the Glenn procedure, four surgical (i.e. Blalock-Taussig) fistulas, 11 pulmonary arteriovenous fistulas in three patients, two aortopulmonary collateral arteries in two patients, one venous collateral in a patient who underwent the Fontan procedure, one aortopulmonary artery fistula in a patient with Scimitar syndrome, one coronary arteriovenous fistula, three systemic arteriovenous fistulas in a newborn, and one fistula from the left atrium to the superior vena cava after the repair of anomalous pulmonary venous return. The lesions were treated percutaneously using 34 vascular plugs and 19 coils. CONCLUSIONS Vascular fistulas can be occluded percutaneously with good RESULTS Small fistulas can be closed using coils, while vascular plugs are preferable for large lesions. Both devices are highly effective as occluders and no particular difficulty or significant complication was observed.

Prueba de esfuerzo con función cardiopulmonar en niños operados de cardiopatía congénita. Recomendaciones de ejercicio físico en el ámbito escolar

INTRODUCTION AND OBJECTIVES To analyze and discover if stress testing with exhaled gases in children who have had congenital heart surgery is useful so we could make physical exercise recommendations according to heart disease, type of surgery performed, present hemodynamic state and level of exercise practiced. METHODS Prospective study of 108 children, who performed stress testing with exhaled gases, electrocardiogram monitoring and blood pressure. A questionnaire was used to obtain variables concerning heart disease, surgery, present functional condition and level of exercise practiced. Exercise recommendations were given after stress testing, and after a year 35 patients answered a questionnaire. RESULTS There were significant differences between lesion severity and heart rate at rest and during effort, systolic pressure at rest and during effort, oxygen uptake, oxygen pulse, carbon dioxide production and test duration. A relationship was observed between level of weekly exercise and greater oxygen uptake and test duration, but this was not observed with the underlying heart disease. We observed that best performance occurred with fast repairing for 59 children with cyanotic heart disease. Increased exercise level was recommended for 48 children. CONCLUSIONS The cardiopulmonary function study allows us to examine the physical performance of children who have had congenital heart surgery and provides us with important data so that we can recommend better physical exercise planning.