Extended Szabo (anchor-wire) technique concept for stent implantation in congenital heart lesions.

We report the usefulness of the Szabo (anchor-wire) technique and two modifications of such based on the same concept for stent implantation in congenital heart lesions. The modifications of the original technique are related to the localization of the cell of the stent through which the anchor wire, which stops the stent advancement, is introduced: proximal in the original technique and central or distal in the reported modifications. These techniques were performed in six patients: in two to maintain permeability of the ductus arteriosus, in three to achieve a fenestration of the interatrial septum, and in one to implant a stent in a right ventricle-to-pulmonary artery conduit close to the bifurcation. We describe the technique as well as the most important difficulties and complications encountered. The Szabo or anchor-wire technique concept is a new tool for stent implantation that can provide more accurate stent positioning compared with conventional angiographically guided implantation in different congenital heart defects. As with any new tool, this technique demands a learning curve and knowledge of potential complications.

Prueba de esfuerzo con función cardiopulmonar en niños operados de cardiopatía congénita. Recomendaciones de ejercicio físico en el ámbito escolar / Cardiopulmonary stress testing in children who have had congenital heart disease surgery. Physical exercise recommendations during school hours

Introducción y objetivos. El objetivo es analizar la utilidad de la prueba de esfuerzo con determinación de gases espirados en niños operados de cardiopatía congénita en edad escolar, para valorar la recomendación de ejercicio físico en relación con la cardiopatía de base, el tipo de cirugía realizada, la situación hemodinámica actual y el nivel de ejercicio habitual. Métodos. Estudio prospectivo de 108 niños que realizaron una prueba de esfuerzo con análisis de gases, monitorizando electrocardiograma y presión arterial. Se recogieron por cuestionario variables sobre cardiopatía de base, cirugía practicada, estado funcional actual y nivel de ejercicio habitual. Se emitió una recomendación de ejercicio después de la ergometría, y al año se controló por cuestionario a 35 de los pacientes. Resultados. Se observaron diferencias significativas entre la gravedad actual de la lesión y la frecuencia cardiaca de reposo y esfuerzo, la presión arterial sistólica en reposo y en esfuerzo, el consumo de oxígeno, el pulso de oxígeno, la producción de dióxido de carbono y la duración de la prueba. Se detectó relación entre el nivel de ejercicio semanal y mayores consumo de oxígeno y duración de la ergometría, pero no con la cardiopatía subyacente. En los 59 niños con lesiones cianóticas, se observó que la mejor capacidad funcional se correspondía con reparación de la lesión más precoz y mejor. Se pudo recomendar un incremento del nivel de ejercicio a 48 niños. Conclusiones. La prueba ergoespirométrica permite explorar la capacidad funcional de los niños operados de cardiopatía congénita y aporta datos importantes para una mejor planificación del ejercicio físico aconsejable (AU)

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 (AU)

[Cardiopulmonary stress testing in children who have had congenital heart disease surgery. Physical exercise recommendations during school hours]. / 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.

Implantación percutánea de válvula pulmonar / Percutaneous implantation of a pulmonary valve

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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.

Embolización percutánea de fístulas vasculares con el tapón vascular de Amplatzer o coils / Percutaneous embolization of vascular fistulas using coils or amplatzer vascular plugs

Introducción y objetivos. Hay gran variedad de fístulas vasculares que son remitidas a los laboratorios de cateterismo cardiaco para su diagnóstico y oclusión percutánea. Existe también una amplia gama de dispositivos utilizables en su tratamiento percutáneo. El objetivo es evaluar la utilidad, las dificultades, las complicaciones y los resultados en el tratamiento percutáneo de las fístulas vasculares mediante el uso de coils de liberación controlada o tapones vasculares de Amplatzer. Métodos. Revisión retrospectiva de las embolizaciones percutáneas realizadas desde enero de 2004 a junio de 2008. Resultados. Se embolizaron 51 fístulas vasculares en 30 pacientes con edades entre 6 días y 28 años (media, 8,4 años), con buen resultado. Diagnósticos: 27 colaterales venosas en 16 pacientes con Glenn; 4 fístulas quirúrgicas (Blalock-Taussig); 11 fístulas 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 síndrome de cimitarra; 1 fístula arteriovenosa coronaria; 3 fístulas arteriovenosas sistémicas en 1 recién nacido, y una fístula entre aurícula izquierda y vena cava superior tras la cirugía de un retorno venoso pulmonar anómalo. En el tratamiento percutáneo de estas lesiones se emplearon 34 tapones vasculares y 19 coils. Conclusiones. Las fístulas vasculares pueden ser ocluidas percutáneamente con buen resultado. Los coils permiten el cierre de fístulas de menor diámetro y los tapones vasculares están indicados en las más amplias. Ambos dispositivos alcanzan una alta eficacia oclusora y no se han observado especiales dificultades ni complicaciones significativas (AU)

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 controlledrelease 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 forlarge lesions. Both devices are highly effective as occluders and no particular difficulty or significant complication was observed (AU)

[Pregnancy and congenital heart disease]. / Embarazo y cardiopatías congénitas.

INTRODUCTION AND OBJECTIVES: Since the creation of the Adult Congenital Heart Disease Units and of the High Obstetric Risk Units, there has been increasing interest in hemodynamic and obstetric outcomes in pregnant woman with congenital heart disease. METHODS: Retrospective descriptive study of 56 women with congenital heart disease aged (mean [range]) 25 (18-40) years, who experienced a total of 84 pregnancies between January 1992 and August 2006. The women were divided into three pregnancy risk groups: A, low-risk; B, moderate-risk, and C, high-risk. RESULTS: The incidence of complications during pregnancy was 1.6%, 15%, and 20% in groups A, B, and C, respectively; the incidence during the puerperium was 2%, 23%, and 50%, respectively; and maternal mortality was 0%, 7.6%, and 25%, respectively. Overall, 69 children were born, and the prematurity rates in the three groups were 11%, 15%, and 100%, respectively. The following risk factors were studied: pulmonary hypertension, cyanosis, arrhythmia, left ventricular outflow tract obstruction, right ventricular dilatation, systemic right ventricle, and anticoagulation therapy. The risk factor most significantly associated with maternal or fetal morbidity or mortality was found to be pulmonary hypertension. CONCLUSIONS: Risk stratification in pregnant women with congenital heart disease provides prognostic information that can help multidisciplinary teams to target care to achieve the best results.

Embarazo y cardiopatías congénitas / Pregnancy and congenital heart disease

Introducción y objetivos. Desde la creación de las Unidades de Cardiopatías Congénitas (CC) del Adulto y las Unidades Obstétricas de Alto Riesgo Cardiológico, ha habido creciente interés por la evolución hemodinámica y obstétrica de embarazadas con CC. Métodos. Estudio descriptivo retrospectivo de 56 mujeres con CC y media de edad de 25 (18-40) años, que iniciaron 84 gestaciones entre enero de 1992 y agosto de 2006. Se las distribuyó en 3 grupos de riesgo gestacional: A, bajo; B, moderado y C, alto. Resultados. Las incidencias de complicaciones durante la gestación 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 niños y las tasas de prematuridad fueron del 11, el 15 y el 100% respectivamente. Los factores de riesgo principales fueron: la hipertensión pulmonar (HTP), la cianosis, la arritmia, la obstrucción del tracto de salida del ventrículo izquierdo, el ventrículo derecho (VD) dilatado, el VD sistémico necesidad de y la anticoagulación. La HTP fue el factor más importante asociado a morbimortalidad maternofetal. Conclusiones. La estratificación por riesgo en las gestantes con CC ofrece información pronóstica que permite adecuar la atención de equipos multidisciplinarios para conseguir resultados exitosos

Introduction and objectives. Since the creation of the Adult Congenital Heart Disease Units and of the High Obstetric Risk Units, there has been increasing interest in hemodynamic and obstetric outcomes in pregnant woman with congenital heart disease. Methods. Retrospective descriptive study of 56 women with congenital heart disease aged (mean [range]) 25 (18­40) years, who experienced a total of 84 pregnancies between January 1992 and August 2006. The women were divided into three pregnancy risk groups: A, low-risk; B, moderate-risk, and C, high-risk. Results. The incidence of complications during pregnancy was 1.6%, 15%, and 20% in groups A, B, and C, respectively; the incidence during the puerperium was 2%, 23%, and 50%, respectively; and maternal mortality was 0%, 7.6%, and 25%, respectively. Overall, 69 children were born, and the prematurity rates in the three groups were 11%, 15%, and 100%, respectively. The following risk factors were studied: pulmonary hypertension, cyanosis, arrhythmia, left ventricular outflow tract obstruction, right ventricular dilatation, systemic right ventricle, and anticoagulation therapy. The risk factor most significantly associated with maternal or fetal morbidity or mortality was found to be pulmonary hypertension. Conclusions. Risk stratification in pregnant women with congenital heart disease provides prognostic information that can help multidisciplinary teams to target care to achieve the best results

Gated-SPECT equilibrium radionuclide angiography in right ventricular assessment of patients with repaired tetralogy of Fallot.

BACKGROUND: The long-term prognosis of patients with tetralogy of Fallot (TF) who have undergone repair is determined by right ventricular dilatation resulting from residual pulmonary insufficiency. We have studied the values of right and left ventricle systolic function obtained by gated single photon emission computed tomography (SPECT) equilibrium radionuclide angiography (ERNA) in these patients. METHODS: A study population of 62 patients with surgically repaired TF underwent gated-SPECT ERNA to determine ejection fraction of the right and left ventricle and dimensions of the right ventricle and pulmonary infundibulum. Results were compared with those of a group of 11 patients without heart disease. RESULTS: RVEF (34% vs. 40%, P=0.02) and LVEF (49% vs. 54%, P=0.03) were significantly lower in patients with TF than in the control group. The RVEF and LVEF variation coefficients were 9% and 6.2%, respectively. Volumes of the right ventricle (P=0.003) were significantly greater than those of the control group, although variation coefficients were 15%. CONCLUSIONS: Gated-SPECT ERNA is a non-invasive method of assessing ejection fraction in patients with repaired TF. In these patients, the ejection fraction is decreased in both ventricles, whereas size of the right ventricle is significantly increased. Reproducibility of the RVEF calculation is good, but in the case of volumes it is suboptimal.

Percutaneus double stent atrial septostomy.

Graded balloon septostomy, bladed atrial septostomy, fenestrated Amplatzer devices and stent placement have been reported to obtain stable interatrial communications. We expose our favorable experience creating an interatrial septal defect, by the use of two stents concentrically placed. We think it can be a procedure to be taken into account whenever a wide interauricular septal orifice must be achieved.

Recurrent congenital heart block in neonatal lupus.

Congenital heart block (CHB) is the main complication of neonatal lupus (NL) and is strongly associated with the presence of anti-SSA/Ro and anti-SSB/La antibodies. The recurrence of CHB in subsequent pregnancies in mothers with these antibodies is uncommon, occurring in approximately 15% of cases. We describe here a case of recurrent CHB in a previously asymptomatic mother with Sjögren syndrome and discuss the current strategies for the prevention and treatment of CHB in NL.

Assessment of the markers of platelet and coagulation activation following transcatheter closure of atrial septal defects.

BACKGROUND: Aspirin has been routinely prescribed following transcatheter closure of secundum atrial septal defects (ASDs) but its rationale has not been clinically or biologically evaluated; and despite aspirin, thrombotic complications occur following transcatheter ASD closure. We therefore evaluated the presence, degree and timing of the activation of the coagulation and platelet systems following transcatheter closure of ASDs. METHODS AND RESULTS: Fourteen consecutive patients (9 females, mean age 41+/-22 years) who underwent successful transcatheter closure of an ASD defect with the Amplatzer septal occluder were prospectively studied. Measurements of the prothrombin fragment 1+2 (F1+2) levels and the percentage of activated platelets (determined by P-selectin expression detected by flow cytometry) were taken at baseline just before the procedure, and at 1, 7, 30 and 90 days following device implantation. F1+2 levels increased from 0.85+/-0.29 nmol/l at baseline to a maximal value of 1.20+/-0.52 nmol/l at 7 days, gradually returning to the baseline levels at 90 days (0.79+/-0.54 nmol/l) (p<0.001). F1+2 levels at 7 days were also significantly higher than those obtained in a control group of 20 healthy subjects (p=0.016). A greater increase in coagulation activation was observed in cases of residual shunt following ASD closure (r=0.53, p=0.050). No significant variations in the percentage of platelets expressing P-selectin were detected at any time. CONCLUSIONS: Transcatheter closure of ASDs with the Amplatzer septal occluder was associated with a significant increase in F1+2 levels during the first week after device implantation, but there was no detectable effect on platelet system activation. These findings raise the question whether the optimal prophylactic approach following transcatheter ASD closure should be anticoagulant instead of antiplatelet therapy.