A Hybrid Strategy for Geometrical Reshaping of the Main Pulmonary Artery and Transcatheter Pulmonary Valve Replacement.

Transcatheter pulmonary valve replacement has become an attractive alternative to surgical approach in patients with dysfunctional right ventricular outflow tract. However, in certain cases, an unfavorable anatomy might complicate optimal valve deployment and stability. Several techniques have been described to reshape the landing zone and allow proper implantation of the transcatheter valve. Among them, the hybrid approach has gained attention as an interesting method for off-pump pulmonary valve replacement in patients with dilated right ventricular outflow tract. But to date, there is no standardized method to resize and reshape the landing zone for the stented valve. Here, we describe a reproducible method based on simple geometric rules to allow adequate remodeling of the main pulmonary artery to the desired dimensions in a single attempt, followed by perventricular implantation of a Venus P-valve.

Initial Experience in Chile with Stent Implantation in the Right Ventricle Outflow Tract in High-Risk Patients with Tetralogy of Fallot.

Tetralogy of Fallot (ToF) treatment is difficult in patients with surgical risk factors or unfavorable anatomy. Stent implantation in the right ventricular outflow tract (RVOT) is an option for these patients. We report our initial experience in Chile with RVOT stenting in patients with ToF. Retrospective and descriptive study conducted in three pediatric cardiovascular centers in Chile between 2012 and 2015, including all ToF patients with stent in the RVOT as first procedure. Clinical records, echocardiographic, interventional, and surgical reports were reviewed for demographics and information of RVOT and pulmonary arteries. 12 newborns with ToF were included (75% female). Median age was 20 days (1-70) and mean weight was 2178 g (1400-3414). Saturations increased after the procedure from 74.3% (55-88) to 88.5% (80-98%), (p < 0.01). No complications or mortality were related to interventions. Follow-up was 11 months (7-36). Median right and left pulmonary arteries Z-score increased from - 4.0 (- 5.2 to - 0.3) and - 1.5 (- 4.8 to - 0.26) to + 0.53 (0.0 to 2.2) and + 1.1 (0.5 to 2.9), (p < 0.05), respectively. Nakata index increased from 63 mm2/mm2 (35 to 143) to 162 mm2/mm2 (107 to 197), (p < 0.05). Surgical repair was performed at a median of 4 months (2-7). Transannular patch repair was necessary in all patients and there was no surgical mortality. RVOT stenting is a safe and useful option for patients with ToF and surgical risk factors or unfavorable anatomy. It increases the pulmonary blood flow, improving saturation and pulmonary artery growth as a bridge for surgical repair.

Co-occurrence of Noonan and Cardiofaciocutaneous Syndrome Features in a Patient with KRAS Variant.

We report the case of a 3-year-old girl, who is the third child of nonconsanguineous parents, with short stature, hypertrophic cardiomyopathy, and mild dysmorphic features; all suggestive of Noonan syndrome. In addition, the patient presents with feeding difficulties, deep palmar and plantar creases, sparse hair, and delayed psychomotor and language development, all characteristics frequently observed in cardiofaciocutaneous syndrome. Molecular analysis of the Ras/ MAPK pathway genes using high-resolution melting curve analysis and gene sequencing revealed a de novo KRAS amino acid substitution of leucine to tryptophan at codon 53 (p.L53W). This substitution was recently described in an Iranian patient with Noonan syndrome. The findings described in this report expand the phenotypic heterogeneity observed in RASopathy patients harboring a KRAS substitution, and advocate for the inclusion of genes with low mutational frequency in genetic screening protocols for Noonan syndrome and other RASopathies.

Chemoenzymatic lignin valorization: Production of epoxidized pre-polymers using Candida antarctica lipase B.

An innovative chemoenzymatic catalytic system for functionalizing lignin from Organosolv and Kraft pulping processes to obtain oxirane rings was investigated. Novozym435 (immobilized C. antarctica lipase B: CalB) was used to catalyze the peroxidation of caprylic acid to peroxycaprylic acid, which in turn reacted with unsaturated CC bonds to form the oxirane ring. The conversion of OH groups to oxirane rings (epoxides) reached 90% and 55% after 12 h for the two processes, respectively. The residual enzyme activity over the time course of the reactions indicated transient denaturing due to association with the lignin substrate (10-50%) as well as irreversible denaturation due to exposure to hydrogen peroxide. Functionalized lignin has potential applications in the production of epoxy adhesive resins, and chemoenzymatic synthesis represents a "greener" pathway to this synthesis.

Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica / Hypoplastic left heart syndrome: 10 year experience with staged surgical management

Resumen El síndrome de hipoplasia de corazón izquierdo (SHCI) es una cardiopatía congénita con letalidad superior al 95%. La etapificación quirúrgica es la principal vía de tratamiento, y se inicia con la operación de Norwood; la sobrevida a largo plazo de los pacientes tratados es desconocida en nuestro medio. Objetivos 1) Revisar nuestra experiencia en el manejo de todos los pacientes con SHCI evaluados entre enero 2000 y junio 2010. 2) Identificar factores de riesgo de mortalidad quirúrgica. Pacientes y método Estudio retrospectivo de una única institución con una cohorte de pacientes con SHCI. Se revisan antecedentes clínicos, quirúrgicos, y registros de seguimiento. Resultados Se evaluaron 76 pacientes con SHCI; 9/76 tenían comunicación interauricular (CIA) restrictiva, y 8/76, aorta ascendente de < 2 mm; 65/76 fueron tratados: 77% tuvieron operación de Norwood con conducto entre ventrículo derecho y ramas pulmonares como fuente de flujo pulmonar, 17% Norwood con shunt de Blalock-Taussig, y 6% otra cirugía. La mortalidad en la primera etapa quirúrgica fue del 23%, y en operación de Norwood, del 21,3%. En el período 2000-2005 la mortalidad en la primera etapa quirúrgica fue del 36%, y entre 2005-2010, del 15% (p = 0,05). La sobrevida global fue del 64% a un año y del 57% a 5 años. Por análisis multivariado fueron factores de riesgo para mortalidad la presencia de aorta ascendente diminuta y CIA restrictiva. Conclusiones Nuestros resultados inmediatos y a largo plazo en la etapificación quirúrgica de SHCI son similares a la experiencia de grandes centros. Hay una mejoría en mortalidad operatoria en la segunda mitad de la serie. Se identifican factores de riesgo de mortalidad.

Abstract Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. Objectives 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. Patients and method Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. Results Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤ 2 mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P = .05). Actuarial survival was 64% at one year, and 57% at 5 years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. Conclusions Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

[Hypoplastic left heart syndrome: 10 year experience with staged surgical management]. / Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica.

UNLABELLED: Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. OBJECTIVES: 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. PATIENTS AND METHOD: Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. RESULTS: Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤2mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P=.05). Actuarial survival was 64% at one year, and 57% at 5years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. CONCLUSIONS: Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

Tratamiento endovascular exitoso de aneurisma de aorta torácica secundario a infección de catéter de arteria umbilical en un recién nacido pretérmino / Successful endovascular treatment of thoracic aortic aneurysm secondary to infection of the umbilical artery catheter in preterm infants

Los aneurismas aórticos en la población pediátrica son poco frecuentes. El uso de catéteres de arteria umbilical en neonatos se ha asociado a infección y en algunas oportunidades a formación de aneurismas aórticos. La reparación quirúrgica de estos aneurismas es una forma de terapia; sin embargo, la intervención percutánea con stents pudiese proveer una vía alternativa de tratamiento con menores complicaciones. El objetivo de este reporte es dar a conocer el alcance terapéutico de un procedimiento híbrido, en el que el desarrollo de la técnica quirúrgica y percutánea en conjunto ofrece otra alternativa terapéutica menos invasiva que la cirugía vascular abierta, para la reparación de aneurismas aórticos o de sus ramas principales. Caso clínico: Recién nacido de pretérmino, 30 semanas, peso 1.335 g. Se instaló catéter en arteria umbilicar que se retiró a los 14 días por infección. Evolucionó con sepsis a Staphylococcus aureus. Ecocardiograma y angiotac confirman AAT, se manejó mediante procedimiento híbrido, cirugía e instalación endovascular de 2 stents recubiertos (Atrium V12 XR Medical Corp, Hudson, NH). Los controles clínicos post procedimiento como la ecotomografía abdominal confirmaron el éxito del tratamiento. Conclusión: El procedimiento endovascular de reparación de aneurisma de la aorta en recién nacidos prematuros puede ser considerado en el momento de decir la terapéutica de esta enfermedad y podría evitar los riesgos asociados a cirugía abierta. Sin embargo, obliga a un seguimiento y control durante el crecimiento del paciente por la eventual necesidad de redilatar los stents implantados. Se desconoce cómo será la evolución de los procedimientos endovasculares neonatales en el futuro.

Aortic aneurysms (AA) in the paediatric population are uncommon. The use of umbilical catheters in neonates has been associated with infections and, on some occasions, the formation of aortic aneurysms. The surgical repair of these aneurysms is one type of treatment; however, percutaneous intervention with stents could provide an alternative treatment route, with fewer complications. The aim of this report is to present the therapeutic scope of a hybrid procedure, in which the combined surgical and percutaneous technique offers a less invasive alternative to open surgery for the repair of aortic aneurysms or their main branches. Clinical case: The case concerns a pre-term newborn of 30 weeks weighing 1,335 g. An umbilical catheter was introduced, which was withdrawn at 14 days due to an infection. It developed as Staphylococcus aureus with sepsis. The echocardiogram and Angio-CT confirmed AA, which were managed using a hybrid procedure of surgery and the endovascular implantation of 2 coated stents (Atrium V12 XR Medical Corp, Hudson, NH). The post-procedure clinical follow-ups, including abdominal echo-tomography, confirmed the success of the treatment. Conclusion: The endovascular aortic aneurysm repair procedure in premature newborns may be considered when deciding treatment of this disease, and could avoid the risks associated with open surgery. However, follow-up and monitoring is required while the patient grows up, due to the possibility that the implanted stents require re-dilating. The outcomes of neonatal endovascular procedures in the future are unknown.

[Successful endovascular treatment of thoracic aortic aneurysm secondary to infection of the umbilical artery catheter in preterm infants]. / Tratamiento endovascular exitoso de aneurisma de aorta torácica secundario a infección de catéter de arteria umbilical en un recién nacido pretérmino.

UNLABELLED: Aortic aneurysms (AA) in the paediatric population are uncommon. The use of umbilical catheters in neonates has been associated with infections and, on some occasions, the formation of aortic aneurysms. The surgical repair of these aneurysms is one type of treatment; however, percutaneous intervention with stents could provide an alternative treatment route, with fewer complications. The aim of this report is to present the therapeutic scope of a hybrid procedure, in which the combined surgical and percutaneous technique offers a less invasive alternative to open surgery for the repair of aortic aneurysms or their main branches. CLINICAL CASE: The case concerns a pre-term newborn of 30 weeks weighing 1,335 g. An umbilical catheter was introduced, which was withdrawn at 14 days due to an infection. It developed as Staphylococcus aureus with sepsis. The echocardiogram and Angio-CT confirmed AA, which were managed using a hybrid procedure of surgery and the endovascular implantation of 2 coated stents (Atrium V12 XR Medical Corp, Hudson, NH). The post-procedure clinical follow-ups, including abdominal echo-tomography, confirmed the success of the treatment. CONCLUSION: The endovascular aortic aneurysm repair procedure in premature newborns may be considered when deciding treatment of this disease, and could avoid the risks associated with open surgery. However, follow-up and monitoring is required while the patient grows up, due to the possibility that the implanted stents require re-dilating. The outcomes of neonatal endovascular procedures in the future are unknown.

Use of the amplatzer vascular plug II device to occlude different types of patent ductus arteriosus in pediatric patients.

INTRODUCTION: Transcatheter patent ductus arteriosus (PDA) closure is difficult in small patients in part due to the diverse PDA morphology. We describe a multicenter pediatric experience using the Amplatzer Vascular Plug II device (AVP II) to occlude PDA. METHODS: All patients undergoing transcatheter closure of PDA with AVP II from April 2008 until May 2012 were included. Clinical, angiographic, and echocardiographic data were collected. RESULTS: One hundred and seventy-seven procedures were performed. Median age was 14 months (2-180) with median weight 9.7 kg (4.7-68). The morphological PDA classification was Type A in 66 (37.1%), Type C in 27 (15.3%), Type D in 17 (9.6%), and Type E in 67 (37.9%). The median minimum PDA diameter was 2.6 mm (1.2-7.7 mm). The implanted device sizes were: 4 mm in 17 patients (9.6%), 6 mm in 81 (45.8%), 8 mm in 56 (31.6%), 10 mm in 18 (10.2%), and 12 mm in 5 (2.8%). The implanted device was mean of 2.6 + 0.7 times the ductus narrowest diameter and mean of 1 + 0.5 times the ductus largest diameter. Complications included: one severe left pulmonary artery stenosis and one device embolization. No aortic obstruction occurred. Closure was complete in the 175 remaining patients. CONCLUSIONS: The AVP II is an effective and safe device for PDA closure. It is particularly useful when dealing with nontypical PDA shapes and in small infants where it eliminated the risk of device-related aortic obstruction. The AVPII is an addition to the PDA device closure armamentarium in pediatric patients.

Resultados inmediatos y alejados del switch arterial en pacientes condextrotransposición de grandes arterias: experiencia de 20 años / Early and late results of the switch operation for d-transposition of the great vessels. clinical experience in 108 patients operated on between 1992 and 2012

Objetivos: Comunicar los resultados de la operación de switch arterial en pacientes portadores de dextro Transposición de Grandes Arterias (D-TGA) y evaluar su evolución en el tiempo. Pacientes y Método: Estudio retrospectivo de pacientes sometidos a switch arterial entre mayo de 1992 y noviembre de 2012. Se comparó período 1 (1992 a 2002) con período 2 (2003 a 2012). Se definió D-TGA simple aquella sin lesiones asociadas y D-TGA compleja aquella con asociación de comunicación interventricular o coartación aórtica. Resultados: Un total de 108 pacientes componen la serie, 44 en el período 1 y 64 en el período 2, sin diferencias demográficas y anatómicas entre ambos períodos. Setenta tenían D-TGA simple y 38 D-TGA compleja. La mortalidad operatoria en el período 1 fue 33% versus 8,4 por ciento en el período 2 (p< 0.025); hubo tendencia a mayor mortalidad en pacientes con anatomía coronaria compleja. Se observó una disminución del riesgo relativo de mortalidad de 68,7 por ciento en el período 2. El 28,7 por ciento presentaron complicaciones postoperatorias, sin diferencias entre ambos períodos. Un paciente falleció en forma alejada. La mediana de seguimiento fue de 60 meses y la supervivencia de 84,25 por ciento a 10 y 20 años. Se realizaron 16 re intervenciones, principalmente plastías percutáneas de ramas pulmonares. La mayoría de los pacientes se mantenían asintomáticos. La mortalidad operatoria de los últimos 5 años fue 2,6 por ciento. Conclusiones: La mortalidad operatoria ha disminuido significativamente a lo largo de 20 años; persiste un mayor riesgo en ciertos patrones de anatomía coronaria. La supervivencia alejada y libre de reintervenciones es muy favorable.

Aim: to report the results of the arterial Switch operation in patients with D-transposition of the great vessels (D-TGA) and to evaluate their late course. Patients and Methods: A retrospective review of the clinical data on patients consecutively operated on for D-TGA using the switch procedure. Results obtained in patients operated on between 1992 and 2002 (Period 1) were compared to those obtained in patients undergoing their operation between 2013 and 2012. Patients with D-TGA and no complications were compared to those who had interventricular septal defect or aortic coarctation associated their TGA. Results: 44 patients belonged in Period 1 and 64 in Period 2 (total 108). Demographic and anatomical characteristics were similar in both periods. Simple D-TGA was present in 70 patients and complex D-TGA in 38. Operative mortality was higher in Period 1 compared to Period 2 (33 per cent vs. 8.4 percent, p<0.025). A higher, albeit not statiscally significant mortality was observed in patients with complex as opposed to simple D-TGA. A 68.7 per cent relative reduction mortality risk was observed in Period 2. Complications developed in 28.7 per cent of patients, with no difference between periods. Only 1 patient died during late follow up. Median follow up was 60 months and survival rate was 84.3 percent at 10 y 20 years. Sixteen patients required re-intervention, mainly to perform percutaneous plastic procedures on pulmonary artery branches. Most patients had an asymptomatic course during follow up. Surgical mortality for the last 5 years was 2.6 percent. Conclusion: surgical mortality for the arterial switch operation in patients with D-TGA has significantly decreased along a 20 year period. An increased surgical risk persists for patients with certain forms of coronary artery anomalies. Late survival free of re-intervention was the rule in these patients.

Tratamiento endovascular de la coartación aórtica nativa y recurrente en pacientes mayores de 4 años: resultados y complicaciones / Endovascular repair of native and recurrent aortic coarctation in patients older than 4 years of age

Objetivos: Reportar nuestra experiencia en el tratamiento endovascular de la Coartación Aórtica (CoAo). Métodos: Se revisaron los registros clínicos de los pacientes mayores de 4 años intervenidos por una CoAo nativa o recoartación en el Hospital Clínico de la Universidad Católica entre los años 2007 y 2012. Se realizaron estadísticas descriptivas y se utilizó el test de Wilcoxon signed rank, con una p <0,05. Resultados: En ese período se realizaron 27 intervenciones en pacientes con CoAo. El promedio de edad fue 20.6 años (5 - 64); 8 (30 por ciento) eran mujeres y 8 (30 por ciento) menores de 14 años. Quince pacientes (55 por ciento) tenían una cardiopatía congénita asociada, de los cuales 9 (33 por ciento) tenían válvula aórtica bicúspide, 3 (11 por ciento) una comunicación interventricular (CIV) y 3 coexistencia de ambas entidades (11 por ciento). Nueve (33 por ciento) pacientes eran hipertensos. En 20 (74 por ciento) pacientes la intervención fue sobre una coartación aórtica nativa y en 7 (26 por ciento) sobre una recoartación (2 con cirugía previa y 5 con angioplas-tia anterior). Se implantó un stent en 23 (85 por ciento) pacientes y se utilizó sólo balón en 4 (15 por ciento). En 26 pacientes (96 por ciento) se logró una reducción del gradiente a menos de 20 mmHg (gradiente pre 32 vs gradiente post 6 mmHg, p<0,01). Hubo complicaciones del sitio de punción en 3 pacientes (2 hematomas y 1 disección focal en el origen de la arteria ilíaca externa) y complicaciones mayores en 2 pacientes (disección aórtica tipo B) que requirieron tratamiento intervencional (uno percutáneo y uno quirúrgico). No hubo mortalidad en nuestra serie. Conclusiones: La intervención endovascular es una opción de tratamiento efectiva para la CoAo.

Aim: There is an increasing interest in the endovascular therapy of both native and recurrent aortic coarcta-tion (Aco). In this article we report the results of endo-vascular treatment of ACo in children and adults at our institution, from 2007 to 2012. Method and Results: The clinical, angiographic and hemodynamic data of all patients submitted to endovascular repair of ACo at the Endovascular Therapy Center of the Catholic University Hospital were reviewed. Children under 4 years of age were excluded. A total of 27 subjects were included. Mean age was 20.6 years (range 5-64); 30 per cent were females and 30 per cent were under 14 years of age. 15 patients had associated congenital defects: bicuspid aortic valve ( 9), VSD (3), or both VSD and bicuspid aortic valve (3). Hypertension was present in 33 per cent of patients. 74 per cent had endovas-cular repair of a native Aco and 26 per cent of a re-coarctation (2 of them post surgical treatment and 5 post endovas-cular repair). 85 per cent of cases had stent implantation. Repair resulted in a significant (p<0.01) decrease in aortic pressure gradient (mean 32 vs 6 mmHg). There was no mortality associated to the procedure. Two patients developed aortic dissection post procedure, successfully treated by surgery or endovascular repair. There were no instances of stent migration. Conclusion: Endovascular repair is an effective means of correcting Aco. Potential complications need careful technique and monitoring during the procedure.

[Validation of quality of life questionnaire ConQol for Chilean children with congenital heart diseases]. / Validación del cuestionario de calidad de vida ConQol, en niños chilenos portadores de cardiopatías congénitas.

BACKGROUND: ConQoL questionnaire assesses health related quality of life among children with congenital heart diseases. It has a version for children aged 8 to 11 years and another for children aged 12 to 16 years. AIM: To validate ConQol questionnaire for Chilean children with a congenital heart disease. MATERIAL AND METHODS: Using a multicentric cross sectional design, 334 children from four hospitals (54% males), were surveyed. Among them 45% were aged 8 to 11 years and 55%, 12 to 16 years. The study involved three stages: cross cultural adjustment of the original questionnaire, pre-test study, and estimation of its psychometric properties. Content, construct and criterion validity and internal consistency with Cronbach's alpha, were assessed. RESULTS: The version for children aged 8 to 11 years and comprised by three domains (symptoms, activity and relationships), obtained and α ≥ 0.60. In the questionnaire for children aged 12 to 16 years, there is one more domain called coping, which obtained an α of 0.53, that was different to the other three domains that obtained an α > 0.70. The correlation between Health Quality of Life and Perception of Health Quality of Life was statistically significant for both groups. The association between Health Quality of Life and health capability was only significant among children aged 12 to 16 years (p < 0.01). CONCLUSIONS: The adapted ConQol questionnaire matched properly with the original one. The adapted questionnaire is valid and reliable to assess Health Quality of Life among Chilean children with congenital heart diseases.

Utilidad del cierre del foramen oval permeable en pacientes con AVE criptogénico / Closure of patent foramen ovale in patients with cryptogenic stroke

Antecedentes: El foramen oval permeable (FOP) es un hallazgo frecuente en pacientes con accidente vascular encefálico criptogénico (AVEC), y se discute la utilidad de su cierre percutáneo (CP). Objetivo: Evaluar el riesgo de recurrencia de eventos neurológicos en pacientes con AVEC y FOP, y compararlos entre aquellos tratados médicamente y los sometidos a CP. Métodos: Entre los 106 pacientes admitidos por AVEC y FOP, en el período 2003 a 2006, determinamos la aparición de nuevos eventos neurológicos isquémicos (NEN), y estudiamos sus factores determinantes y comparamos los que se sometieron a CP versus lo que continuaron con tratamiento médico, según criterio del tratante. Se consignaron las características clínicas y de la antomía del FOP en el ecocardiograma Los NEN se confirmaron por examen neurológico y/o neuro-imágenes. Para el análisis de los datos se utilizó chi-cuadrado y regresión logística.Resultados: Entre los 106 pacientes evaluados, 87 siguieron tratamiento médico y 19 CP. Los pacientes sometidos a CP presentaban mayor asociación de FOP con aneurisma del septum interauricular (ASI) (57,9% versus 35,6%, p=0,05). El seguimiento fue de 27 +/-13 meses. En este período se demostró un 12,6% de nuevo evento neurológico entre los tratados médicamente, mientras que ninguno lo presentó entre los sometidos al CP (NS). El único predictor independiente para NEN fue el ASI asociado con FOP; OR: 8,45 (1,56-60,46). Conclusiones: De acuerdo a nuestros resultados, los pacientes con AVEC y FOP tienen alto riesgo de recurrencia cuando el FOP se asocia a ASI y aparentemente se benefician con CP.

Background: Patent Foramen Ovale (PFO) is a frequent finding in patients with cryptogenic stroke (CS). Theeffect of closing the PFO in this setting is debated. Aim: to evaluate de risk of stroke recurrence in patients with CS and PFO; to compare this risk in patients followed under medical treatment with those undergoing percutaneous closure of PFO. Methods: From 2003 to 2006, 106 patients were admitted with a CS and the presence of PFO was documented by echocardiography. New ischemic strokes and risk factors were compared between those who weresubmitted to percutaneous closure of PFO and those treated in a conventional way. The decision to close thePFO was taken by the physician in charge. Clinical findings and echocardiographic characteristics of thePFO were recorded. New ischemic events were diagnosed by neurologic assessment and/or imaging techniques. Data was analyzed by chi square testing and logistic regression. Results: 87 patients were followed under medical treatment and 19 had closure of the PFO. The latter group had a greater incidence of atrial septal aneurysm (57.9% vs. 35.6%, p=0.05). The mean follow up was 27 +/- 13 months.New ischemic stroke occurred in 12.6% in the medically treated group while none was observed in the PFO closure group (NS). The sole independent predictor of new stroke was the presence of atrial septal aneurysm (OR: 8.45, 95% C.I. 1.56 - 60.46) Conclusion: Patients with CS and PFO are at considerable risk of developing new strokes, especially those with concomitant atrial septal aneurysm. Closure of PFO was apparently useful to prevent this risk.

Multiple Amplatzer septal occluder devices for multiple atrial communications: immediate and long-term follow-up results.

OBJECTIVES: We report on the feasibility, effectiveness, and long-term outcome of transcatheter closure of multiple ASD's using multiple ASO devices. BACKGROUND: Little is known about the use and long-term outcome of multiple Amplatzer Septal Occluder (ASO) devices to close multiple atrial septal defects (ASD's). METHODS: From May 1997 to June 2006, 33 patients (mean age 38.9 years and mean weight 68 kg) underwent transcatheter closure of multiple ASD's under transesophageal (TEE) or intracardiac echocardiographic (ICE) guidance. RESULTS: Unless not available, the device size chosen to be deployed was +/-2 mm larger than the stretched diameter or no more than 30-40% larger than the ICE/TEE 2D diameter. Sixty-seven devices were deployed in 33 patients. The mean diameters of the larger and smaller defects were 12.9 and 7.7 mm, and the corresponding mean balloon stretched diameters were 19.2 and 12.0 mm, respectively. The mean larger and smaller device diameters were 19.0 and 13.4 mm, respectively. The mean QP: Qs ratio was 1.8:1. The mean fluoroscopy time, and procedure time were 16.4 and 81.6 min respectively. Immediately after the procedure 15 patients had complete closure, 8 had trivial shunt, 9 had small shunt and one had large shunt. Complications included a device embolization within 24 hr and left atrial-aortic wall erosion and pericardial effusion at 2 years. The mean follow-up interval was 34.8+25.7 months. CONCLUSIONS: Device closure of multiple ASD's using multiple ASO's is safe and effective. Continued follow-up is important to assess the long-term outcome.

Percutaneous replacement of pulmonary valve using the Edwards-Cribier percutaneous heart valve: first report in a human patient.

We report on the use of the Edwards-Cribier percutaneous heart valve (PHV) in a stenotic right ventricle to pulmonary artery homograft in a 16-year old patient who underwent the Ross operation. Initially, the homograft was stented and at the same procedure, the PHV was deployed inside it. This is the first human case of the application of this valve in the pulmonary valve position.

[New surgical approach to device closure of multiple apical ventricular septal defects]. / Cierre perventricular de la comunicación interventricular múltiple con dispositivo Amplatzer.

We present an alternative technique for closing multiple ventricular septal defects difficult to access during surgery. A guidewire is advanced through the right ventricular free wall and through the main apical defect to the left ventricle, and this approach is used to place an Amplatzer device to occlude the ventricular septal defects. The procedure is performed in the beating heart, under intraoperative transesophageal echocardiographic guidance, and without extracorporeal circulation. It appears to be a simple and reproducible procedure with excellent short-term results.

Cierre perventricular de la comunicación interventricular múltiple con dispositivo Amplatzer / New Surgical Approach to Device Closure of Multiple Apical Ventricular Septal Defects

Se presenta una técnica alternativa para el cierre de la comunicación interventricular de difícil acceso quirúrgico. Con el corazón latiendo y sin necesidad de circulación extracorpórea, se punciona el ventrículo derecho y se avanza una guía de alambre hasta el ventrículo izquierdo a través del defecto apical principal; esto permite colocar un dispositivo Amplatzer que ocluye completamente los defectos. El procedimiento se realiza con visión ecocardiográfica transesofágica, es simple y con excelentes resultados a corto plazo (AU)

We present an alternative technique for closing multiple ventricular septal defects difficult to access during surgery. A guidewire is advanced through the right ventricular free wall and through the main apical defect to the left ventricle, and this approach is used to place an Amplatzer device to occlude the ventricular septal defects. The procedure is performed in the beating heart, under intraoperative transesophageal echocardiographic guidance, and without extracorporeal circulation. It appears to be a simple and reproducible procedure with excellent short-term results (AU)

[Clinical findings and immunologic variability in 9 patients with DiGeorge syndrome]. / Manifestaciones clínicas y variabilidad inmunológica en nueve pacientes con síndrome de DiGeorge.

BACKGROUND: DiGeorge syndrome is characterized by developmental defects of the heart, parathyroid glands and thymus. AIM: To describe the clinical variability of DiGeorge syndrome and its relation with immunodeficiency. PATIENTS AND METHODS: A three years retrospective chart review from three hospitals of Santiago, Chile was conducted. We included patients with neonatal diagnosis of DiGeorge syndrome. Clinical and immunologic data were collected from their initial evaluation. RESULTS: We found 9 patients with DiGeorge syndrome. All had dysmorphic facies, hypocalcemia and congenital heart disease. Three patients had hypoparathyroidism, 4 had interrupted aortic arch type B, 4 had tetralogy of Fallot and 1 had coarctation of aorta. Six patients had other malformations and associated diseases. FISH studies, performed in 8 patients, found the 22q11.2 microdeletion in all. Most patients had low CD3, CD4 and CD8 T cell counts, that ranged for CD3 T cells, between 256/mm3 and 3,664/mm3, for CD4 T cells, between 224/mm3 and 2,649/mm3, for CD8 T cells, between 26/mm3 and 942/mm3. Three patients had CD4 T cells counts <400/mm3 and one had a phytohemagglutinin stimulation index <10. Airway malformations and primary hypoparathyroidism were present in 3 out of 4 patients that died before 18 months compared with the surviving patents (p=0.048). CONCLUSIONS: We found variable clinical manifestations as well as CD3, CD4 and CD8 T cell counts in patients with DiGeorge syndrome. Airway malformations were associated with a higher mortality.

[Immediate results of palliative surgery for different forms of univentricular heart using the Norwood procedure]. / Cirugía paliativa mediante operación de Norwood en distintas formas de corazón univentricular: resultados quirúrgicos inmediatos.

BACKGROUND: Norwood procedure is used as the first stage in the palliative treatment of the hypoplastic heart syndrome and can be used, with some technical modifications, in other forms of univentricular heart with aortic stenosis or hypoplasia. These patients have a high mortality (50%), derived from the procedure itself and from their abnormal physiological status. AIM: To report our experience with the Norwood procedure. PATIENTS AND METHODS: Retrospective analysis of all patients subjected to the Norwood procedure between February, 2000 and June 2003. RESULTS: Thirteen patients (9 females, age range 5-60 days and median weight of 3.3 kg) were operated. Eight had hypoplastic heart syndrome and five had a single ventricle with aortic arch hypoplasia. The diagnosis was done in utero in eight patients. All technical variations, according to the disposition and anatomy of the great vessels, are described. Cardiac arrest with profound hypothermia was used in all and regional cerebral perfusion was used in nine. Three patients died in the perioperative period and three died in the follow up (two, four and 10 months after the procedure). Gleen and Fontan procedures were completed in five and one patients, respectively. CONCLUSIONS: Our results with the Norwood procedure are similar to other series. There is an important mortality in the immediate operative period and prior to the Glenn procedure.