Aortic iatrogenic perforation during transseptal puncture and successful occlusion with Amplatzer ductal occluder in a case of mitral paravalvular leak closure.

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.

Epigenetic Evaluation of the TBX20 Gene and Environmental Risk Factors in Mexican Paediatric Patients with Congenital Septal Defects.

The TBX20 gene has a key role during cardiogenesis, and it has been related to epigenetic mechanisms in congenital heart disease (CHD). The purpose of this study was to assess the association between DNA methylation status and congenital septal defects. The DNA methylation of seven CpG sites in the TBX20 gene promoter was analyzed through pyrosequencing as a quantitative method in 48 patients with congenital septal defects and 104 individuals with patent ductus arteriosus (PDA). The average methylation was higher in patients than in PDA (p < 0.001). High methylation levels were associated with a higher risk of congenital septal defects (OR = 4.59, 95% CI = 1.57-13.44, p = 0.005). The ROC curve analysis indicated that methylation of the TBX20 gene could be considered a risk marker for congenital septal defects (AUC = 0.682; 95% CI = 0.58-0.77; p < 0.001). The analysis of environmental risk factors in patients with septal defects and PDA showed an association between the consumption of vitamins (OR = 0.10; 95% CI = 0.01-0.98; p = 0.048) and maternal infections (OR = 3.10; 95% CI = 1.26-7.60; p = 0.013). These results suggest that differences in DNA methylation of the TBX20 gene can be associated with septal defects.

Clinical Characteristics and Outcomes in Adults With Moderate-to-Severe Complexity Congenital Heart Disease Undergoing Palliation or Surgical Repair.

Background: Congenital heart disease (CHD) survival rate has improved dramatically due to advances in diagnostic and therapeutic techniques. However, concerning the unrepaired CHD population of moderate and severe complexity, the data regarding risk predictors and surgical outcomes are scarce. Our aim was to describe the surgical results and predictors of in-hospital outcomes in adult patients with moderate-to-severe complexity CHD that were not repaired in childhood. Methods: We conducted a retrospective cohort study that included 49 adult patients with moderate-to-complex CHD who were treated in a single medical centre. Clinical and echocardiographic variables were obtained on admission, after surgical procedures and during follow-up. Results: Most of the patients were female (66%). Left ventricular ejection fraction and right ventricular outflow tract fractional shortening were within the normal range. The median pulmonary artery systolic pressure was 37 (27-55) mm Hg. The median time was 118 (80-181) minutes for extracorporeal circulation and 76 (49-121) minutes for aortic cross-clamping. The most frequent complication was postoperative complete atrioventricular block (12.2%). In-hospital survival rate was 87.7%. The development of low cardiac output syndrome with predominant right ventricle failure in the postoperative period was the most important predictor of in-hospital death (P = 0.03). Conclusions: Deciding to treat adults with CHD is challenging in moderate and severe unrepaired cases. Adequate clinical, functional, and imaging evaluation is essential to determine each patient's suitability for surgical management and to achieve the best clinical outcome for this population.

Contexte: Grâce aux avancées réalisées en matière de techniques diagnostiques et thérapeutiques, la survie des patients atteints d'une cardiopathie congénitale s'est considérablement améliorée. Cependant, en ce qui concerne les personnes atteintes d'une cardiopathie congénitale non corrigée présentant une complexité modérée ou extrême, les données portant sur les facteurs de risque prédictifs ainsi que sur les résultats chirurgicaux sont rares. Notre objectif était de décrire les résultats chirurgicaux ainsi que les facteurs prédictifs des résultats obtenus en milieu hospitalier chez les patients adultes atteints d'une cardiopathie congénitale présentant une complexité modérée ou extrême qui n'a pas été corrigée pendant l'enfance. Méthodologie: Nous avons mené une étude de cohorte rétrospective comprenant 49 patients adultes atteints d'une cardiopathie congénitale modérée ou complexe qui ont reçu leurs traitements dans un seul centre médical. Les variables cliniques et échocardiographiques ont été obtenues au moment de l'admission, après les interventions chirurgicales et pendant la période de suivi. Résultats: Les patients étaient en majorité des femmes (66 %). La fraction d'éjection du ventricule gauche ainsi que la fraction de raccourcissement de la voie d'éjection ventriculaire droite sont demeurées dans les limites de la normale. La pression systolique médiane de l'artère pulmonaire a été de 37 mmHg (27-55 mmHg). Le temps médian écoulé pour la circulation extracorporelle a été de 118 minutes (80-181 minutes) et pour le clampage de la crosse aortique, de 76 minutes (49-121 minutes). Le bloc auriculo-ventriculaire postopératoire complet a été la complication la plus fréquente (12,2 %). Le taux de survie en milieu hospitalier a été de 87,7 %. Le développement du syndrome du faible débit cardiaque accompagné d'une insuffisance prédominante du ventricule droit durant la période postopératoire a constitué le principal facteur prédictif de décès à l'hôpital (p = 0,03). Conclusion: Il est difficile de traiter les adultes qui présentent une cardiopathie congénitale modérée ou sévère non corrigée. Il est essentiel que les évaluations cliniques, fonctionnelles et par imagerie soient réalisées de façon adéquate pour déterminer si une prise en charge chirurgicale convient aux patients et pour garantir les meilleurs résultats cliniques chez ces derniers.

[Operative results in cardiovascular surgery of the neonate]. / Resultados inmediatos en cirugía cardiovascular neonatal.

OBJECTIVE: 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. MATERIAL AND METHODS: 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. RESULTS: 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: age < 15 days, body surface area < 0.20 m2, weight < 4 kg, and univentricular heart physiology. CONCLUSIONS: 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.

[Scimitar syndrome in infancy]. / Síndrome de la cimitarra en edad pediátrica.

OBJECTIVE: 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. MATERIAL AND METHODS: 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. RESULTS: We studied 22 patients with scimitar syndrome, 20 of them with associated congenital heart disease. Congestive heart failure (p > or = 0.0001) and severe pulmonary hypertension (p > or = 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 (p > or = 0.007). CONCLUSIONS: 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.

DNA Methylation Levels of the TBX5 Gene Promoter Are Associated with Congenital Septal Defects in Mexican Paediatric Patients.

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.

Application of radiofrequency perforation to recanalization late stent thrombosis of aortic coarctation.

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.

[Tetralogy of fallot and total anomalous pulmonary venous drainage to coronary sinus. Case report]. / Tetralogía de Fallot con conexión anómala total de venas pulmonares a seno coronario. Reporte de un caso con esta rara asociación.

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.

Outcomes of Delayed Sternal Closure in Pediatric Heart Surgery: Single-Center Experience.

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.

Treatment of seven patients with coarctation of the aorta treated using a Mexican-made platinum/iridium stent.

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.

Common Arterial Trunk Repair by Means of a Handmade Bovine Pericardial-Valved Woven Dacron Conduit.

BACKGROUND: Surgical repair of common arterial trunk (CAT) by means of a homograft conduit has become a standard practice. We report our experience in the correction of this heart disease with a handmade bovine pericardial-valved woven Dacron conduit as an alternative procedure to homografts, with a focus on early, mid-term, and long-term results. METHODS: We designed a retrospective study that included 15 patients with a mean age of 1.5 years (range: three months to eight years), who underwent primary repair of simple CAT. Right ventricular outflow tract was reconstructed in all the cases with this handmade graft that was explanted at the time of its biological stenotic degeneration. A peeling procedure was performed at this time, in order to reconstruct the right ventricle-to-pulmonary artery continuity. RESULTS: Overall mortality was 13.3% (one death at the early postoperative primary repair and the other at the mid-term postoperative peeling reoperation). Actuarial survival rate was 93.3%, 86.7%, and 86.7% at 5, 10, and 15 years, respectively. All of the 14 survivors developed stenosis of the handmade conduit at the mid-term period (8 ± 3 years), but after the peeling procedure, 13 survivors remain asymptomatic to date. CONCLUSIONS: Primary repair of common arterial trunk using a handmade conduit can be performed with very low perioperative mortality and satisfactory mid-term and long-term results, which can be favorably compared with those reported with the use of homografts. When graft obstruction develops, peeling procedure is a good option because it does not affect the overall survival, although long-term outcomes warrant further follow-up.

[Diagnostic catheterization in grown up congenital heart disease]. / Cateterismo diagnóstico en cardiopatías congénitas del adulto.

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.

[Clinic aspect of criss-cross: a case report]. / Aspectos clínicos de criss-cross: A propósito de un caso.

A 1-month-old girl was referred at our Institution with a history of heart failure and cyanosis. We established diagnosis of multiple congenital heart disease with criss-crossed atrioventricular connections but concordant. This type of anomaly has a variety of presentations of high diagnostic complexity. The multiple lesions included double outlet of the right ventricle, right subaortic stenosis, ventricular septal defect, coarctation of the aorta with hypoplasia of the arch, and persistent patency of the arterial duct, established through echocardiography, angiocardiography and magnetic resonance. These findings prompted palliative surgery and correction of the aortic arch.

[Patent ductus arteriosus in the adult: transcatheher treatment immediate and medium term results]. / Conducto arterioso en el adulto: tratamiento transcateterismo. Resultados inmediatos y a mediano plazo.

INTRODUCTION: The anatomic changes of the patent ductus arteriosus (PDA) in adult patients, such as aortic aneurysm, calcification, or being short and sometimes friable, could complicate the surgical treatment. The transcatheter occlusion of PDA with different devices is currently accepted as safe and effective. We presented our experience with percutaneous occlusion of PDA in adult patients by means of three different devices. METHODS AND RESULTS: Between January 2000 and March 2005, 53 adult patients (47 women and 6 men) with PDA were treated for occlusion by means of percutaneous procedures. Average age was 25.3 years (Range, 16 to 54.7 years). Three different devices were used, 39 patients with Amplatzer for ductus arteriosus, 1 patient with Amplatzer for muscular interventricular communication, 11 with Gianturco coils, and 1 patient with Nit-Occlud. All devices were implanted successfully. Immediate complete closure was achieved in 31 patients (58.4%), in 20 patients (37.7%) with minimal leakage, and in 2 patients (3.89%) with moderate leak. Previous systolic pulmonary pressure had an average of 37.08 +/- 22.8. mm Hg (Range: 12-138 mm Hg) and went down to 28.75 +/- 10.25 mm Hg (Range: 16-57 mm Hg) with p d x 0.001. The average time follow-up was of 16.8 months (Range: 0.9 to 51.8 months) the occlusion was completed in 100%, in neither patients were observed complications related to implant of the devices. CONCLUSION: Transcatheter closure at PDA in adult patients with different devices is safe and feasible.

[Anomalous origin of the right pulmonary artery and left pulmonary vein stenosis]. / Origen anómalo de la arteria pulmonar derecha y estenosis de vena pulmonar superior izquierda.

We present a case of a 9 month old female with a history of cyanosis. The cardiovascular evaluation included angiocardiogram and magnetic resonance. The diagnosis was: origin of the right pulmonary artery from the ascending aorta, stenosis of the left superior pulmonary vein, patent ductus arteriosus and severe pulmonary hypertension. Surgical treatment consisted in correcting all congenital heart defects. The patient is doing well at home.

[Extreme aortic coarctaction, severe aortic regurgitation and heart failure relieved immediately after stent aortoplasty, and complete resolution through Bental/de Bono surgical repair]. / Alivio inmediato de la insuficiencia cardíaca en la coartación de la aorta posterior a la aortoplastía con stent, y mejoría absoluta, posterior a la operación de Bentall y de Bono. (Presentación de un caso).

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.

[Comparison between patients undergoing Fontan operation with or without cardiopulmonary bypass]. / Comparación de pacientes sometidos a cirugía de Fontan con y sin derivación cardiopulmonar.

Fontan operation is the final palliative stage of patients with univentricular hearts. Cardiopulmonary bypass (CPB) decreases ventricular performance and increases pulmonary artery pressures in the post operative recovery period. It seems that Fontan operation performed without CPB decreases short term morbidity and intra hospitalary length of stay. OBJETIVE: Compare outcome in Fontan patients who have undergone surgery with or without CPB. METHOD: This is a retrospective review of patients undergoing Fontan operation from january 2009 to december 2012. Patients were grouped according to CPB use and comparative analyses were done. RESULTS: Ten patients were operated without CPB use. There was a discrepancy between age in both groups, being younger in the no CPB group. Around 80% of patients in both groups had a staged procedure. A 18mm graft was used in half of the cases; a fenestration was created in all cases. Length of stay was equal in both groups, there was less need of pharmacologic support and nitric oxide use in patients without CPB use. No deaths were reported also in this group. At folllow up, most patients had a class i functional status. CONCLUSIONS: In our experience, Fontan operation without CPB has similar outcomes compared with CPB use.

[Percutaneous closure of ductus arteriosus and muscular ventricular defect with amplatzer occluder in a patient with severe pulmonary hypertension]. / Cierre percutáneo de conducto arterioso y de comunicación interventricular muscular con dispositivos de Amplatzer en paciente con hipertensión pulmonar acentuada.

Surgical treatment of multiple muscular ventricular septal defects with associated lesions and severe pulmonary hypertension has a high morbility and mortality. Closure of these defects by the Amplatzer muscular VSD occluder is an alternative to surgery, avoiding the need of cardiopulmonary bypass. We present the case of a 38 year-old woman with signs of heart failure in NYHA functional class IV, with two muscular ventricular septal defects, patent ductus arteriosus and severe pulmonary hypertension, that were treated with three Amplatzer muscular VSD occluders, with significant reduction of pulmonary pressure and functional class improvement.

[Percutaneous closure of ventricular septal defect by Amplatzer occluder. Immediate and mid-term follow up results]. / Cierre percutáneo de la comunicación interventricular perimembranosa con el dispositivo de Amplatzer: resultados inmediatos y a mediano plazo.

OBJECT: To report the immediate and mid-term follow-up results of the Amplatzer membranous VSD occluder for the percutaneous occlusion of the perimembranous VSD. BACKGROUND: Percutaneous perimembranous VSD occlusion is still considered an experimental method where a variety of devices have been tested. Nowadays, more than 500 membranous Amplatzer devices have been implanted worldwide with encouraging results. METHOD: We included 6 patients (1 man and 5 women) with a mean age of 9.9 years (range, 3 to 17.5) in whom percutaneous perimembranous VSD closure was attempted. RESULTS: In one of the patients, positioning of the device was not possible (intention to treat success rate, 83.3%). In the remaining five patients, there was a single defect. The VSD mean diameter with echo was 7 +/- 1.7 mm (range, 5.1 to 9) and with angio was 6.9 +/- 1 (range, 6 to 8). Mean pulmonary pressure was 20.2 +/- 7.7 mm Hg (range, 12-30) and Qp/Qs was 1.69 +/- 0.65 (range, 1.2-2.8). A single device was use in all cases. Immediate angiographic control showed complete occlusion in two patients, trivial shunt in one, and mild shunt in two. Follow-up was at least 4 months. Only one patient has residual trivial shunt, the rest of the defects are completely closed. CONCLUSION: The special design of the Amplatzer membranous VSD occluder allows percutaneous closure of this defect in a safe and effective way, with good mid-term results. In selected cases, this is a good alternative to surgery in the treatmen of this cardiac defect.

[Fontan operation: short- and long-term risk factors]. / Cirugía de Fontan. Factores de riesgo a corto y mediano plazo.

The purpose of this retrospective study was to determine the outcome of patients who underwent a Fontan procedure at National Heart Institute "Ignacio Chávez", Mexico, from January 1989 to December 2003. We had 81 patients with a mean age of 7 years old: 53 with tricuspid atresia, 14 with pulmonary atresia and intact septum, 11 with univentricular atrioventricular connection and 3 with Ebstein's anomaly. An intra-atrial tunnel was performed on 61 patients and an extracardiac conduit on the rest. Of all, 53 underwent a fenestration. The surgical mortality was 13% for tricuspid atresia and 25% for the other diseases. We had an overall mortality of 28.4%, being the main causes cardiogenic shock and arrhythmia. The global survival was 71% in 55 months of following. Takedown was indicated on 5 patients. The patients without fenestration showed to have 2.8 times more risk of death. The mean pulmonary artery pressure > or = 20 mmHg plus the left atrium pressure > or = 10 mmHg increased the death risk 3.6 times. Of the 52% who required readmission, the main causes were hemodynamical failure and infections. The more relevant complications were: arrhythmia (38%), protein-losing enteropathy (8%) and thrombotic cerebral events (1.4%).