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Background Unilateral abscence of pulmonary artery is a rare congenital heart disease. If these anomaly is unrecognized and treated the risk of pulmonary hypertension in the contralateral lung can be lethal for the patients. Aims To report the successful sequential hybrid-surgical approach of two cases of rescue of pulmonary branches only fed by ductus arteriosus. Materials & methods Description of two rare cases of absence of a unilateral pulmonary branch successfully treated in our institution. Results, discusssiion and conclusion The timely identification of these cases and the strategic planning of interventional and surgical hybrid sequential treatment of these rare cases is effective.

Aim: To analyze the association of NKX2.5 gene with congenital heart disease (CHD), and to determine if the variants rs703752, rs3729753 and rs2277923 increase the risk for developing CHD. Materials & methods: PubMed, EBSCO and Web of Science databases were screened to identify eligible studies. Through a comprehensive meta-analysis software, the association between NKX2.5 gene variants and susceptibility of CHD was calculated by pooled odd ratio (ORs) and 95% CI. Results: We observed that the allelic model of rs703752 and rs2277923 increased the risk in the overall population: OR = 1.24; 95% CI: 1.00–1.55; Z p-value = 0.049; OR = 1.18; 95% CI: 0.01–1.37; Z p-value = 0.036; respectively. Conclusion: Our results suggested that the rs703752 and rs2277923 polymorphisms of the NKX2.5 gene are associated with CHD.

La enfermedad de Kawasaki (EK), ha sobrepasado en muchos países a la fiebre reumática como primera causa de cardiopatía adquirida en la edad pediátrica, siendo su secuela más grave los aneurismas coronarios. Presentamos el caso de revascularización quirúrgica exitosa en un paciente de 5 años de edad, con lesión trivascular coronaria secundaria a EK, a quien se le realizó una derivación coronaria con anastomosis de la arteria mamaria interna izquierda a la descendente anterior, la arteria mamaria derecha a la obtusa marginal y la arteria radial a la descendente posterior. Un seguimiento estrecho y la decisión oportuna de la cirugía evitarán sucesos desfavorables como incapacidad permanente del paciente, disfunción ventricular, arritmias, infarto al miocardio y muerte súbita.

Simple Summary One of the most important health problems in the paediatric population, due to its prevalence worldwide, is the occurrence of congenital heart defects. The integration of multidisciplinary approaches to clinical diagnosis will allow us to detect this type of problem in patients in a timely way. The TBX5 gene has an important participation in cardiogenesis. Therefore, we performed a case-control study, that involved the DNA methylation assessment of the TBX5 gene promoter region in patients that were non-syndromic with congenital septal defects, to identify an epigenetic marker. Moreover, we evaluated the exposure to environmental factors during pregnancy in mothers of these patients. Additionally, we used bioinformatic tools to identify transcription factors binding to the TBX5 gene region of interest and to learn their possible functional effect. These results could help to clarify the mechanisms that regulate these pathologies and establish risk markers, which can be used in future in clinical practice. Abstract The TBX5 gene regulates morphological changes during heart development, and it has been associated with epigenetic abnormalities observed in congenital heart defects (CHD). The aim of this research was to evaluate the association between DNA methylation levels of the TBX5 gene promoter and congenital septal defects. DNA methylation levels of six CpG sites in the TBX5 gene promoter were evaluated using pyrosequencing analysis in 35 patients with congenital septal defects and 48 controls. Average methylation levels were higher in individuals with congenital septal defects than in the controls (p < 0.004). In five CpG sites, we also found higher methylation levels in patients than in the controls (p < 0.05). High methylation levels were associated with congenital septal defects (OR = 3.91; 95% CI = 1.02–14.8; p = 0.045). The analysis of Receiver Operating Characteristic (ROC) showed that the methylation levels of the TBX5 gene could be used as a risk marker for congenital septal defects (AUC = 0.68, 95% CI = 0.56–0.80; p = 0.004). Finally, an analysis of environmental factors indicated that maternal infections increased the risk (OR = 2.90; 95% CI = 1.01–8.33; p = 0.048) of congenital septal defects. Our data suggest that a high DNA methylation of the TBX5 gene could be associated with congenital septal defects.

Severe acute respiratory syndrome coronavirus 2, the etiologic agent of coronavirus disease 2019, has caused more than 160 million infections globally. The experience of our department showed that the execution of a strict surgery protocol, universal severe acute respiratory syndrome coronavirus 2 screening, surgical prioritization, and an orderly reactivation of the cardiac surgery program is a feasible way to keep congenital heart diseases surgery program.

Purpose The aim of the study was to identify, determine, and analyze the clinical and surgical outcomes of patients with atrial isomerism (AI) undergoing cardiac surgery. Methods A retrospective study was carried out. Patients with diagnosis of AI undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chavez, from January 1, 2010 and March 31, 2020 were included; demographic characteristics and perioperative conditions of the patients were considered. Results Sixty-five patients were included, with an average age of 6.4 ± 4.9 years, 50.8% males. Thirty-eight (58.5%) had right atrial isomerism (RAI) and 27 (41.5%) had left atrial isomerism (LAI); univentricular physiology (78.5%) predominated. Atrioventricular septal defect (AVSD) in RAI and septal defects in LAI were identified as the main associated defects. The most common surgical procedures performed were modified Blalock-Taussig shunt (MBTS) (27.6%), MBTS with total anomalous pulmonary venous connection (TAPVC) repair (15.3%) and total cavopulmonary connection (TCPC) with an extracardiac conduit fenestrated (10.8%); 100% RAI required a univentricular approach, while in LAI it was 48.1%. Overall survival was 92.3%, with 100% survival in LAI with biventricular physiology and 86.8% in RAI with univentricular physiology. Conclusions The survival of our institution is similar to that of other referral centers, where patients with LAI had a better evolution than RAI; in addition, the univentricular approach was required in all with RAI. Patients with AI must undergo a rigorous evaluation to determine an adequate repair strategy, considering univentricular RAI with a high possibility of morbidity and mortality.

Paravalvular leak is a frequent and important complication after surgical valvular replacement that can cause heart failure, hemolytic anemia and is associated with poor clinical outcomes. Surgical reoperation has been the standard treatment, but it is associated with high morbidity and mortality. Transcatheter closure is a therapeutic alternative. The aim of the present study is to analyze the feasibility and the short and medium-term outcomes of the transcatheter closure of paravalvular leaks.Single-centre registry of consecutive patients with post-surgical paravalvular leaks, that underwent transcatheter closure, between January 2006 and December 2016. Efficacy and safety results were analyzed during the procedure and at 6-month follow-up.21 paravalvular leaks (15 mitral, 5 aortic and 1 tricuspid) were closure during 20 procedures. In the initial echocardiography 91% of the leaks were severe. The most used device was the Amplatzer Vascular Plug III® in 10 procedures (50%). The 3D transesophageal echocardiography was used in 70% of cases. The device was successfully implanted in 95% of cases, a regurgitation reduction ≥ 1 grade was achieved in 95% of the cases, and the clinical success was 79%. Six month survival was 100%; however, 3 cases required valvular surgery (15%).Transcatheter closure of paravalvular leaks is a feasible and safe procedure with high rates of technical, echocardiographic and clinical success in the short and medium term. It is an adequate therapeutic alternative, mainly in high surgical risk patients and multiples comorbidities.La fuga paravalvular es una complicación frecuente e importante posterior al reemplazo valvular quirúrgico que puede ocasionar insuficiencia cardiaca, anemia hemolítica y se relaciona con malos resultados clínicos. La reintervención quirúrgica ha sido el tratamiento habitual, pero se acompaña de alta morbimortalidad. El cierre transcatéter es una alternativa terapéutica. El objetivo del presente estudio es analizar la factibilidad y los resultados a corto y mediano plazo del cierre transcatéter de fugas paravalvulares con dispositivos oclusores.Registro unicéntrico de una serie consecutiva de pacientes con fugas paravalvulares posquirúrgicas que fueron cerradas vía transcatéter con dispositivos oclusores, entre enero del 2006 y diciembre del 2016. Se analizaron los resultados de eficacia y seguridad durante el procedimiento y a seis meses.Se trataron 21 fugas paravalvulares (15 mitrales, 5 aórticas y 1 tricuspídea) durante 20 procedimientos. El 91% de las fugas fue grave en la ecocardiografía inicial. El dispositivo utilizado con más frecuencia fue el Amplatzer Vascular Plug III® en 10 procedimientos (50%). Se utilizó ecocardiografía transesofágica tridimensional en 70% de los casos. Se logró implantar el dispositivo con éxito en el 95% de los casos; se consiguió una reducción ≥ 1 del grado de regurgitación en el 95% de las veces y se alcanzó el éxito clínico en el 79%. A seis meses la supervivencia fue del 100%; sin embargo, tres casos requirieron cirugía valvular (15%).El cierre transcatéter de fugas paravalvulares es un procedimiento factible, seguro y con tasas elevadas de éxito técnico, ecocardiográfico y clínico a corto y mediano plazo. Es una alternativa terapéutica adecuada, en particular en pacientes considerados de alto riesgo quirúrgico y múltiples comorbilidades.

Objectives. To demonstrate safety and efficacy of using different generations of softer Amplatzer™ devices for ventricular septal defect (VSD) closure to avoid serious complications at follow-up. Background. Transcatheter closure of perimembranous ventricular septal defects (PmVSD) is a well-established procedure; however, it is associated with unacceptable incidence of complete heart block. Great advantages have been achieved by using softer devices for VSD transcatheter closure. The first and second generation of Amplatzer™ occluders (AVP II, ADO, and ADO II) seem to offer a safe and attractive alternative for this procedure. These devices can be delivered using either an arterial (retrograde) or venous (prograde) approach. Methods and Results. Patients with congenital PmVSD who underwent transcatheter closure using ADO, ADO II, and AVP II devices were included. Primary end point was to determine efficacy and safety of these generations of devices and to determine the incidence of complications at follow-up (complete AV block and aortic/tricuspid/mitral regurgitation). One hundred and nineteen patients underwent VSD closure at a median age of 5 years (8 months–54 years). During the catheterization, there were only minor complications and at follow-up of 36 ± 25.7 months (up to 99 months), the closure rate was high of 98.3% and freedom from AV block was 100%. Conclusions. The use of softer Amplatzer™ devices is a good alternative to achieve PmVSD closure safely with no risk of AVB during the procedure or at midterm follow-up.

Background. Delayed sternal closure (DSC) after cardiac surgery is a therapeutic option in the treatment of the severely impaired heart in pediatric cardiac surgery. Methods. A single-center retrospective review of all bypass surgeries performed over a 10-year period (2003–2012). Results. Of a total of 2325 patients registered in our database, the DSC group included 259 cases (11%), and the remaining 2066 cases (89%) constituted the control group (PSC). RACHS-1 risk was higher for the DSC group (74% had a score of 3 or 4) than for the PSC group (82% had a score of 2 or 3). The most frequent diagnosis for the DSC group was transposition of the great arteries (28%). We found out that hemodynamic instability was the main indication observed in patients aged ≤ 8 years (63%), while bleeding was the principal indication for patients aged ≥ 8 years (94%) (p≤0.001). The average time between surgery and sternal closure was 2.3±1.4 days. Overall mortality rates were higher for patients of the DSC group (22%) than for the PSC group (8.7%) (OR: 0.4 (95% CI: 0.4 to 0.5), p<0.05). There were six patients with DSC who developed mediastinitis (2.3%). The risk of mediastinitis was significantly higher when DSC was performed 4 days after the primary surgery. Conclusions. DSC is an important management strategy for congenital cardiac surgery in infants and children. The prolonged sternal closure time is associated with an increased rate of postoperative mediastinitis.

El espectro del ventrículo derecho hipoplásico es amplio y desafiante, ya que dependiendo de su variabilidad anatómica y funcionalidad se planteará qué tratamiento es el adecuado. Presentamos el caso de un lactante de sexo femenino, de 15 meses de edad, quien cursó asintomática, valorada en consulta de seguimiento por cianosis con saturación de oxígeno del 70%, motivo por el cual es referida a nuestra institución.

El pronóstico de las cardiopatías congénitas complejas del adulto ha mejorado con el desarrollo de las nuevas técnicas de imagen para el diagnóstico oportuno y tratamiento temprano. Asimismo, la valoración de la función sistólica del ventrículo derecho ha tenido importantes avances. El siguiente caso ilustra la importancia de las nuevas técnicas en la valoración de la función sistólica derecha tras la cirugía de un paciente con una cardiopatía congénita compleja.

Complications of transseptal puncture are significant and potentially life threatening. Aortic perforation is one of these complications and it needs to be repaired immediately. We report the case of a 48-year-old female with a history of rheumatic mitral valve disease. She underwent three previous mitral valve replacements. Her last echocardiogram reported an anterolateral leak in the mitral prosthesis. Catheterization was performed. During the procedure, when attempting to perform transseptal puncture, catheterization was complicated by a forcefully puncture of the aortic root by the Brockenbrough needle followed by an immediately advancement of an 8-Fr Mullins sheath. We decided to leave the 8-Fr sheath in the aortic root recognizing the danger of removing the sheath and finally we advanced a 6/4 mm Amplatzer ductal occluder (ADO I) through the Mullins sheath and under fluoroscopy and TEE guidance we achieved a successful deployment of the device and closure of the perforation. Subsequently, the paravalvular leak was closed with an Amplatzer Vascular Plug (AVP III 10/5 mm). There are only three cases of a similar technique for the same injury in which the authors describe successful closing of aortic perforation with percutaneous closure devices. Our case is the first described during a mitral paravalvular leak closure. It is also the first to describe a different technique of leaving the Mullins sheath in the aortic root and advancing the Amplatzer device through it to achieve successful closure of the aortic perforation. We preferred the percutaneous closure over open-heart repair. © 2015 Wiley Periodicals, Inc.

Background. The relevance ofTBX20gene in heart development has been demonstrated in many animal models, but there are few works that try to elucidate the effect ofTBX20mutations in human congenital heart diseases. In these studies, all missense mutations associated with atrial septal defect (ASD) were found in the DNA-binding T-box domain, none in the transcriptional activator domain.Methods. We search forTBX20mutations in a group of patients with ASD or ventricular septal defect (VSD) using the High Resolution Melting (HRM) method and DNA sequencing.Results. We report three missense mutations (Y309D, T370O, and M395R) within the transcriptional activator domain of human TBX20 that were associated with ASD.Conclusions. This is the first association of TBX20 transcriptional activator domain missense mutations with ASD. These findings could have implications for diagnosis, genetic screening, and patient follow-up.

El cierre percutaneo de la comunicacion interauricular es una alternativa al tratamiento quirurgico. Objetivos: Comunicar los resultados del cierre percutaneo de la comunicacion interauricular con el dispositivo Amplatzer®. Metodo: Se incluyeron los pacientes sometidos a hemodinamia de septiembre de 1997 a diciembre de 2011. Resultados: Se procedio al cierre del defecto en 721 pacientes y en 85 no se cerro por considerarse no apto anatomicamente. El diametro expandido del defecto fue de 23 ± 6.7 mm (limites, 5-42). Todos los dispositivos se colocaron con exito. En 15 casos (2.1%) se cambio el dispositivo por considerarse inestable y en 6 (0.8%) se presento embolizacion en las primeras 24 h, 4 fueron rescatados y recolocados (exito final con intencion de tratamiento 719/806-89.2%). El control inmediato mostro oclusion completa en 247 pacientes (34.3%), fuga intradispositivo en 395 (54.9%), ligera en 75 (10.4%) y moderada en 2 (0.3%). Se hace seguimiento en 626 pacientes (87.1%) durante 33 ± 27.5 meses, presentandose cefalea en 54 (8.6%), arritmias supraventriculares nuevas en 10 (1.6%), complicaciones en el sitio de puncion en 4 (0.6%) y accidente vascular cerebral en uno (0.1%). Conclusion: El diseno del dispositivo Amplatzer® permite el cierre de la comunicacion interauricular con una tecnica sencilla y con seguridad para el paciente

Background: Percutaneous closure of patent ductus arteriosus has become the treatment of choice in many centres. In patients with large ducts and pulmonary hypertension, transcatheter closure has been achieved with success using the Amplatzer Duct Occluder or even the Amplatzer Muscular Ventricular Septal Defect Occluder. Materials and methods: We present a series of 17 patients with large and hypertensive ductus arteriosus who were treated with an Amplatzer Septal Occluder. The group had 11 female patients (64.7%) and a mean age of 18.6±12.1 years. Results: The haemodynamic and anatomical data are as follows: pulmonary artery systolic pressure 71.3±31.8 mmHg, pulmonary to systemic flow ratio 3.14±1.36, ductal diameter at the pulmonary end 12.5±3.8 mm, and at the aortic end 20.2±7.7 mm; 14 cases (82.3%) had type A ducts. In 11 patients, we began the procedure using a different device – six with duct occluder and five with ventricular septal occluder – and it was changed because of device embolisation in six (35.3%). All septal occluders were delivered successfully. Residual shunt was moderate in six patients (35.3%), mild in eight (47%), trivial in two (11.8%), and no shunt in one (5.9%). Pulmonary systolic pressure decreased to 48.9±10.8 mmHg after occlusion (p=0.0015). Follow-up in 15 patients (88.2%) for 28.4±14.4 months showed complete closure in all cases but one, and continuous decrease of the pulmonary systolic pressure to 31.4±10.5 mmHg. No complications at follow-up have been reported. Conclusions: The Amplatzer Septal Occluder is a good alternative to percutaneously treat large and hypertensive ductus arteriosus.

Paravalvular leak is a significant complication after surgical valve replacement. Traditionally, redo surgery has been considered the treatment of choice, but is associated with high morbidity and mortality as well as increase risk of developing new leaks. Percutaneous treatment of paravalvular leak appears as an attractive alternative. Such treatment has been made possible by the current ability to integrate multiple imaging modalities that have helped to define the anatomy of the defect and planning and guiding the percutaneous closure procedure, thus resulting in better outcomes. The Amplatzer® Vascular Plug III (St. Jude Medical) is the first device specifically designed for percutaneous closure of paravalvular leak with promising results. We present the three-dimensional transesophageal echocardiographic images of a successful outcome of percutaneous closure of a residual mitral paravalvular leak with an Amplatzer® Vascular Plug III device (St. Jude Medical), after an initial procedure of percutaneous closure with an Amplatzer® duct occluder (St. Jude Medical).

Takayasu’s arteritis (TA), also known as "pulseless disease", is the third most common vasculitis in childhood. It is a chronic, idiopathic, granulomatous vasculitis that involves large vessels. It occurs most commonly in females with a 4:1 ratio over males; the average age of appearance is 26 years. Its cause is unknown. Here we report the case of a 7-year-old girl, with type III TA according to the Numano classification, in the ischemic phase, treated with corticosteroids and immunosuppressive agents and early angioplasty due to the severity of the disease. The outcome was satisfactory. The diagnosis of TA in children under 10 years of age is made only in 2% of them. The delay in diagnosis reaches a mean of 19 months. The course of the disease is variable despite surgical and immunosuppressive treatment.

Resumen La arteritis de Takayasu (AT) o «enfermedad sin pulsos» es la tercera vasculitis mas frecuente en la infancia. Es cronica, idiopatica, granulomatosa y afecta a vasos grandes. Afecta a las mujeres, con una relacion 4:1; con una edad promedio de 26 anos. Su causa es desconocida. Presentamos el caso de una nina de 7 anos y 7 meses de edad, con AT tipo III de la clasificacion de Numano, en fase isquemica, a la que se le inicio tratamiento con glucocorticoides e inmunosupresores, asi como angioplastia temprana, por la severidad del cuadro clinico. Tuvo una evolucion satisfactoria. El diagnostico de AT antes de los 10 anos se realiza en el 2% de los pacientes; el retraso en el diagnostico es en promedio de 19 meses; el curso de la enfermedad es variable a pesar del tratamiento inmunosupresor y quirurgico.

The modified Fontan procedure represents the final stage of reconstructive surgery for most patients with functionally univentricular hearts. Although outcomes following Fontan procedures performed at sea level are widely reported, less has been documented and reported concerning outcomes in regions at high altitude. To clarify the main features involved, we present our institutional experience with Fontan operations performed in Mexico city (2,240 m above the sea level), with an emphasis on historical evolution of treatment. A retrospective and observational study was undertaken, which included 98 patients over a period of 18 years, and clinical outcomes in terms of morbidity and mortality were analyzed. A change in operative technique from intra-cardiac nonfenestrated Fontan procedure to extra-cardiac fenestrated technique occurred in 2001. Early mortality rates before and after this change in surgical approach were 26% and 4.7%, respectively. The most common morbidity was the occurrence of pleural effusions (98% of patients), which also appears to be a risk factor for operative mortality. Much remains unknown about the pathophysiology of the Fontan circulation at high altitude, and we need to develop morphological study protocols that include pulmonary biopsy to increase our knowledge and inform our therapeutic actions.

Background Nowadays, stenting is the treatment of choice in patients >15 years of age with coarctation of the aorta without hypoplastic aortic isthmus. The platinum/iridium stent manufactured in Mexico may be an affordable alternative with the same benefits as the imported stent. Methods This is a series of cases in which we present the immediate results of the first seven patients with coarctation of the aorta treated with the platinum/iridium stent manufactured in Mexico. The first four patients were selected and treated at the Cardiology Hospital of the National Medical Center during the year 2003, two more patients were selected and treated at the National Institute of Cardiology, and another patient was treated at the Military Hospital, Mexico City, in the year 2005. Results A total of seven patients were successfully treated with the platinum/iridium stent without any complications. Average initial gradient was 56.4 mmHg (range: 30–90 mmHg). In six patients, the final gradient was 0.0 mmHg and in one it was 2 mmHg. In the four patients treated in the Cardiology Hospital of the National Medical Center, mean follow-up time was 17.5 ± 2.5 months, with a gradient of 0.0 mmHg. All patients are in NYHA Class I. At 12 months follow-up, the gradient remains as 0 mmHg and the patients are still in NYHA Class I functional state. Conclusions This stent can be a safe and effective alternative to other stents available on the market for the treatment of coarctation of the aorta.

Objectives. To compare the short- and mid-term outcomes of surgical (SUR) vs. transcatheter closure of secundum atrial septal defect (ASD) using Amplatzer septal occluder (ASO) in adults with a very similar spectrum of the disease; and to identify predictors for the primary end point. Design. Single-center, concurrent comparative study. Surgically treated patients were randomly matched (2:1) by age, sex, date of procedure, ASD size, and hemodynamic profile. Setting. Tertiary referral center. Patients. One hundred sixty-two concurrent patients with ASD submitted to ASO (n = 54) or SUR closure (n = 108) according with their preferences. Main Outcome Measures. Primary end point was a composite index of major events including failure of the procedure, important bleeding, critical arrhythmias, serious infections, embolism, or any major cardiovascular intervention-related complication. Predictors of these major events were investigated. Results. Atrial septal defects were successfully closed in all patients, and there was no mortality. The primary event rate was 13.2% in ASO vs. 25.0% in SUR (P = .001). Multivariate analysis showed that higher rate of events was significantly associated with age >40 years; systemic/pulmonary output ratio 50 mm Hg; while in the ASO group the event rate was only associated with the ASD size (>15 cm2/m2; relative risk = 1.75, 95% confidence interval 1.01–8.8). There were no differences in the event-free survival curves in adults with ages 40 years. The length of hospital stay was shorter in the ASO group. Surgical closure is a safe and effective treatment, especially in young adults. There is certainly nothing wrong with continuing to do surgery in countries where the resources are limited.

Paravalvular leak is a significant complication after surgical valve replacement. Traditionally, redo surgery has been considered the treatment of choice, but is associated with high morbidity and mortality as well as increase risk of developing new leaks. Percutaneous treatment of paravalvular leak appears as an attractive alternative. Such treatment has been made possible by the current ability to integrate multiple imaging modalities that have helped to define the anatomy of the defect and planning and guiding the percutaneous closure procedure, thus resulting in better outcomes. The Amplatzer® Vascular Plug III (St. Jude Medical) is the first device specifically designed for percutaneous closure of paravalvular leak with promising results. We present the three-dimensional transesophageal echocardiographic images of a successful outcome of percutaneous closure of a residual mitral paravalvular leak with an Amplatzer® Vascular Plug III device (St. Jude Medical), after an initial procedure of percutaneous closure with an Amplatzer® duct occluder (St. Jude Medical).

Percutaneous closure of atrial septal defects is an alternative to surgical treatment.We report the results of percutaneous closure of atrial septal defects with the Amplatzer(®) device.We include patients taken to the catheterization laboratory from September 1997 to December 2011.We proceeded with defect closure in 721 patients and in 85 the defect was considered not suitable for closure. The stretched diameter of the defect was 23±6.7mm (limits, 5-42). All devices were positioned successfully. In 15 cases (2.1%) the device was changed due to instability and in 6 (0.8%) the device embolized within the first 24h, 4 were retrieved and repositioned (final success with intention to treat 719/806-89.2%). Immediate control showed complete closure in 247 patients (34.3%), leak through the device in 395 (54.9%), mild residual leak in 75 (10.4%) and moderate in 2 (0.3%). We have follow-up in 626 patients (87.1%) for 33±27.5 months, with headache in 54 (8.6%), new supraventricular arrhythmia in 10 (1.6%), puncture site complications in 4 (0.6%), and stroke in one (0.1%).The design of the Amplatzer(®) device allows effective closure of atrial septal defects with a simple technique and patient safety.

To present our institutional postoperative results in cardiovascular surgery of the neonate, and to give an idea of its contribution to the national problematic knowledge in this area.A retrospective, descriptive, and observational study was carried out, including all neonates with congenital heart disease who were operated at the Ignacio Chávez National Cardiology Institute in a 7 year period. We made special emphasis in postoperative morbidity and mortality, as well as in the risk factors for early mortality.We operated on 484 neonates with congenital heart disease due to total anomalous pulmonary venous conection, classic transposition of great arteries, pulmonary atresia, and aortic coarctation. Causes for early mortality were cardiac failure, pulmonary hypertension, pulmonary sepsis, and arrhythmias. Operative mortality was 12.2% and was due mainly to congenital heart disease with 5 and 6 RACHS-1 risk score. Risk factors for operative mortality were: age15 days, body surface area0.20 m2, weight4 kg, and univentricular heart physiology.There is still a high operative mortality for complex neonatal heart disease in this series. Our institutional outcomes can not be extrapolated to the rest of the country, but highlights several challenges that national pediatric health care institutions and associations must assume in order to improve the attention that neonatal patients demand.

To expose our 26 year experience in clinical management, interventional catheterization and surgical treatment of patients younger than 18 years with scimitar syndrome at the National Cardiology Institute.We reviewed retrospectively all patients with scimitar syndrome in infancy between 1984 and 2010. Patients were divided in two groups: younger an older than one year at the time of the diagnosis. Medical records were analized, as well as chest radiography, electrocardiogram, echocardiogram and helicoidal tomography. All therapeutic procedures performed and their outcomes were analized.We studied 22 patients with scimitar syndrome, 20 of them with associated congenital heart disease. Congestive heart failure (por = 0.0001) and severe pulmonary hypertension (por = 0.002) were more frequent in patients younger than one year. We documented dextroposition and right lung hypoplasia in 14 patients, and aorto-pulmonary collateral arteries in 15 of them. Pulmonary hypertension was an important mortality risk factor (por = 0.007).Congestive heart failure and pulmonary hypertension are more frequent in patients younger than one year, and the former is a mortality risk factor. It is important to suspect this congenital heart disease in infants with dextroposition and congestive heart failure in order to provide an opportune treatment.

Intracardiac echocardiography (ICE) is a relatively new method of ultrasound images useful during cardiac percutaneous interventional procedures. The first human experience with this method was published in 2000 and, since then, several original trials have enrolled this useful method, proving similar and more useful than transesophageal echocardiography in percutaneous treatment of several congenital cardiopathies, like interatrial communication, permeable oval foramen, mitral stenosis, and atrial fibrillation ablation. In this presentation, we publish a single case report of percutaneous mitral valvuloplasty under ICE guidance.

We present an interesting complication of late stent thrombosis after percutaneous treatment of aortic coartaction and a new modality of treatment with radiofrequency perforation and implantation of additional stent to resolve this problem.

Background The Amplatzer duct occluder (ADO) has been used with success to close large patent ductus arteriosus (PDA),butsomeproblemsexistespeciallywithhypertensive PDAs, such as incomplete closure, haemolysis, left pulmonary artery stenosis, obstruction of the descending aorta and progressive pulmonary vascular disease. Methods and results We analysed a group of 168 patients with isolated PDA and pulmonary artery systolic pressure (PSAP) $50 mm Hg. Mean age was 10.3 6 14.3 years (median 3.9), PDA diameter was 6.4 6 2.9 mm (median 5.9), PASP was 63.5 6 16.2 mm Hg (median 60), Qp/Qs was 2.7 6 1.2 (median 2.5), total pulmonary resistance index (PRI) was 3.69 6 2.15 (median 3.35) and vascular PRI was 2.73 6 1.72 (median 2.37). We used ADOs in 145 (86.3%) cases, Amplatzer muscular ventricular septal defect occluders (AMVSDO) in 18 (10.7%), Amplatzer septal occluders (ASO) in three (1.8%) and the Gianturco-Grifka device in two (1.2%) cases. Device diameter was 106.3% 6 51% higher than PDA diameter. PASP decreased after occlusion to 42.5 6 13.3 mm Hg (p

We report the case of an infant 3 months old with a rare association, tetralogy of Fallot with total anomalous pulmonary veins connection the diagnosis was made by echocardiography and a successful complete reparation was achieved.

When aneurysm of the root of aorta, bicuspid aorta valve and coarctation coexist, surgical repair involves technical problems. We present the case of a patient in whom initial correction of the coarctation was made by means of interventional treatment, with an impressive and practically immediate resolution of heart failure. In a second intervartional, the aortic root pathology was corrected through the Bentall and de Bono's surgical technique. We present the short- and mid-term results. In addition this case demostrates the little well-know fact, that the patients with bicuspid aorta also have cystic media degeneration of the wall of the aorta.

Non invasive techniques are the procedures of choice in the assessment of the adult patient with congenital heart disease. Currently cardiac catheterization is mainly pointed towards interventional purposes; however in some cases such as complex congenital heart conditions, it is a valuable auxiliary method. Preassure measurements, oximetry and angiography are fundamental in the assessment of patients with selected cardiac disease without treatment; palliative or corrective surgery.