Impact of Surgery in Ebstein's Anomaly Using Current Surgical Criteria.

BACKGROUND: In Ebstein's anomaly (EA) current surgical criteria may not translate into better long-term survival. The aim of this study was therefore to determine if surgical treatment for EA increases survival, and to analyze factors associated with mortality.Methods and Results:A retrospective study was carried out involving 103 patients with surgical indication using current criteria, comparing operated (n=49; 47.5%) and non-operated patients (n=54; 52.4%); the severity of disease was similar in all cases. Overall follow-up was 12 years (range, 1-49 years). There were no differences in mortality: in the surgical and non-surgical groups, survival at 10 years was 92.8% vs. 90.7%; 20 years, 85.7% vs. 81.0%; and 30 years, 78.5% vs. 72.2%, respectively. On multivariate analysis right ventricular fractional shortening (RVFS) was associated with mortality in both groups. Decreasing RVFS was associated with worse survival according to severity: when RVFS was <20%, survival at 20, 40 and 60 years was 58%, 39%, and 12.5%, respectively (P<0.0013). Left ventricular ejection fraction also correlated with survival (P<0.0013). CONCLUSIONS: Surgery did not translate into benefit in terms of survival, and this was clearly associated with RV function; therefore this should be a key factor in the surgical decision making.

[Pediatric cardiovascular surgical data base registry in México. First report]. / El Registro Mexicano de Cirurgía Cardiaca Pediátrica. Primer informe.

INTRODUCTION: Current world tendency is the detection of health problems in order to offer solution alternatives by means of the development of computarized data bases. OBJECTIVE: To present the results of a computerized data base developed for the registry of pediatric cardiac surgery with the support of Asociación Mexicana de Especialistas en Cardiopatías Congénitas (AMECC, A.C.). MATERIAL AND METHODS: A one-year analysis (from August 1, 2011 to July 31, 2012) of a computerized data base was performed with the support of AMECC and the participation of the most important Mexican institutions for pediatric surgical heart disease health care, particularly for the uninsured population. RESULTS: There were 7 health institutions voluntarily incorporated to the national data base registry, and in the first year of observation, 943 surgical procedures in 880 patients and 7% re-operations (n = 63), were reported. Patients up to one-year old accounted for 38%. The most frequent types of operated congenital heart diseases were: patent ductus arteriosus (n = 96), ventricular septal defect (n = 86), tetralogy of Fallot (n = 72), atrial septal defect (n = 68), and aortic coarctation (n = 54). Elective procedures were 90%, and 62% of them were performed with the use of cardiopulmonary bypass. Overall mortality was 7.5% with the following RACHS-1 score risk distribution: 1 (n = 4.2%), 2 (n = 19.6%), 3 (n = 22.8%), 4 (n = 12.19%), 5 (n = 1.25%), 6 (n = 6.44%) and not classifiable (n = 2.9%). CONCLUSIONS: Although this analysis gives a representative vision of the cardiovascular surgical health care for the uninsured national pediatric population, the incorporation of other health institutions to this data base may lead us to have a most realistic overview in relation to the surgical cardiovascular health care for the up to 18 year-old population.

[Changing the paradigm of congenital heart disease: from the anatomy to the molecular etiology]. / Cambiando el paradigma en las cardiopatías congénitas: de la anatomía a la etiología molecular.

Heart development consists in a group of complex and specific morfogenetic interactions, that requires the proper activity of each factor implicated in this process. Congenital heart defects (CHD) are a group of multifactorial complex diseases with environmental and genetic factors playing important roles. There is not an exact relation between molecular mechanisms and morphological defects in CHD, because in most of the cases the proper development of an anatomical structure implies the adequate function of several pathways that may depend of the action of different genes. This review summarizes the genetic factors implied in the normal heart development and the most common gene mutations associated with CHD.

[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.

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.

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.

Mexican registry of pediatric cardiac surgery. First report.

BACKGROUND: Currently, there is a spreading worldwide tendency to characterize health issues and to propose alternative solutions via the creation of computerized databases. The aim of this study was to present the results in a computerized database of pediatric cardiac surgeries developed under the auspices of the Mexican Association of Specialists in Congenital Heart Diseases (Asociación Mexicana de Especialistas en Cardiopatías Congénitas A.C) and coordinated by the collegiate group of Pediatric Cardiology and Surgery as petitioned by the National Institutes of Health and High Specialty Hospitals Coordinating Commission. METHODS: We analyzed all cases registered in the database during a 1-year observation period (August 1, 2011 to July 31, 2012) by all major Health Ministry-dependent institutes and hospitals offering surgical services related to pediatric cardiopathies to the non-insured population. RESULTS: Seven institutions participated voluntarily in completing the database. During the analyzed period, 943 surgeries in 880 patients with 7% reoperations (n=63) were registered. Thirty-eight percent of the surgeries were performed in children <1 year of age. The five most common cardiopathies were patent ductus arteriosus (n=96), ventricular septal defect (n=86), tetralogy of Fallot (n=72), atrial septal defect (n=68), and aortic coarctation (n=54). Ninety percent of surgeries were elective and extracorporeal circulation was used in 62% of surgeries. Global mortality rate was 7.5% with the following distribution in the RACHS-1 score categories: 1 (n=4, 2%), 2 (n=19, 6%), 3 (n=22, 8%), 4 (n=12, 19%), 5 (n=1, 25%), 6 (n=6, 44%), and non-classifiable (n=2, 9%). CONCLUSIONS: This analysis provides a representative view of the surgical practices in cardiovascular diseases in the pediatric population at the national non-insured population level. However, incorporating other health institutions to the national registry database will render a more accurate panorama of the national reality in surgical practices in the population <18 years of age.

Congenital heart disease in Mexico: advances of the regionalization project.

Consistent with the mission of the World Society for Pediatric and Congenital Heart Surgery to promote health care for children with congenital heart disease all around the world, a Mexican Association of Specialists in Congenital Heart Disease (abbreviated in Spanish as AMECC) was created in Mexico in 2008. Our efforts were coordinated with those of the National Health Secretary with the objective being implementation of a national plan for regionalization of care for patients with congenital heart disease. To improve our knowledge related to technologic and human resources for management of congenital heart disease, we developed a national survey. Finally, a national database was created for collecting all Mexican centers' information related to congenital heart disease care in order to quantify the advances related to the proposed plans. The database utilized international consensus nomenclature. The aim of this article is to show the sequence of our actions in relation to direct accomplishments and the current status of congenital heart disease care in Mexico. This article emphasizes the main aspects of these actions: regionalization project implementation, national survey results, and cardiovascular pediatric surgical database creation. Knowledge of outcomes related to successful actions would be useful for those countries that face similar challenges and may lead them to consider adoption of similar measures with the respective adjustments to their own reality.

Fontan Procedure at 2,240 m Above Sea Level.

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.

Outcomes of coronary reimplantation for correction of anomalous origin of left coronary artery from pulmonary artery.

INTRODUCTION AND OBJECTIVES: We report our experience in the surgical correction of anomalous origin of left coronary artery from pulmonary artery (ALCAPA), with an emphasis on the coronary reimplantation technique and its outcome. METHODS: We designed a retrospective, longitudinal, descriptive study that included patients with ALCAPA who underwent surgery involving coronary reimplantation over a 19-year period. We describe perioperative details such as variations in the surgical technique and the postoperative outcome in terms of morbidity and mortality. RESULTS: We studied 15 patients (86% females) with a mean age of 6.2 years (range, 2 months to 24 years). Heart failure was the principal cause for hospitalization in 80% of our patients. Left ventricular dysfunction was present in 67%, and 27% had significant or severe mitral valve regurgitation. We describe 3 surgical techniques for coronary reimplantation, the choice of which depends on the site of origin of the anomalous left coronary artery. Four patients underwent an additional mitral valve procedure. The most common immediate postoperative complications were low cardiac output (38%), pleural effusion (17%), and transient ischemia (13%). There was no operative or medium-term mortality. CONCLUSIONS: Coronary reimplantation is the technique of choice for surgical correction of ALCAPA due to the excellent postoperative survival and low operative morbidity.

Surgical repair of congenital mitral valve malformations.

Surgical development of mitral valve repair techniques in pediatric patients has been slow because of the great variety in the presentation of congenital mitral valve malformations and the still unknown growing effect over the complex mitral valve apparatus. The aim of this study is to review our early an mid-term institutional outcomes in surgical repair of congenital mitral valve malformations. We studied retrospectively 14 patients with surgical repair of congenital mitral valve malformations in a 5 year period. Clinical and echocardiographic follow-up at a mean of 25 months was performed in all cases. Operative morbidity was 77% and operative mortality 7%. There were no late deaths. Clinical functional class stratification at the mid-term improved in 73% of survivors and did not change in the remaining 27%. Freedom from reoperation for mitral valve prosthetic replacement due to mitral valve repair failure was 84% at 30 days and 77% at 3.5 years. Surgical repair is probably the best technique option in the treatment of congenital malformations of the mitral valve, and transesophageal intraoperatory echocardiography must be highly recommended for evaluation of results.

Anatomic diagnosis of congenital heart disease. A practical approach based on the sequentiality principle.

Based on the sequentiality principle, this review proposes a practical method that allows the systematization of the anatomic diagnosis of congenital heart disease. We emphasize the need to use sequential connection between the different cardiac segments: atria, ventricles and great arteries. Five ordered steps are defined, which include determination of atrial situs and of the connection features between the ventricles and the great arteries. Related lesions and some additional special features are a second stage in the sequential analysis of congenital heart disease, which is also important for the integral diagnosis.

[Congenital heart disease in Mexico. Regionalization proposal]. / Problemática de las cardiopatías congénitas en México. Propuesta de regionalización.

Congenital cardiopathies are the most frequent congenital malformations. Reports of its prevalence around the world range from 2.1 to 12.3 for every 1000 newborns. Prevalence in our country remains unknown, but it probably occupies sixth place for mortality in infants less than a year old, and third place for mortality in those aged between 1 and 4 years. Based on birthrate, it is calculated that 10 to 12 000 infants in our country have some cardiac malformation. To understand the magnitude of the problem, it is important to identify the global number of newborns with some congenital cardiopathy each year and the type of malformation that they have, in order to determine the necessary resources and to plan their distribution. The main objective of regionalization is the justification of the resources with an emphasis in the specialized medical services to provide the best results for the patients. Hence, reason, based on the resources of each state, as well as their natality and infant mortality rates related to congenital cardiovascular pathology, we should proceed to regionalize the attention, and to simultaneously create a trustworthy database of the congenital cardiopathies. This should have many benefits, such as increase the number of total attended cases, improve the quality of attention, use appropriately the existent resources, and -surely- decrease the infant mortality.

[Anomalous origin of the left pulmonary artery from the ascending aorta with associated sub-aortic stenosis in an infant]. / Origen anómalo de la rama pulmonar izquierda a partir de la aorta ascendente en un niño con estenosis subaórtica asociada.

OBJECTIVE: The aim of this report is to describe a clinical case of an anomalous origin of the left pulmonary artery branch from the ascending aorta, and to present a current literature review of this rare disease. CLINICAL CASE: A 2 year-old infant was referred to our institution for surgical correction of sub aortic obstruction. The non invasive investigation disclosed a right aortic arch anatomy with left ventricle outflow tract obstruction due to sub aortic membrane associated but failed to show the anomalous origin of the left pulmonary artery branch from the ascending aorta. The diagnosis was made intraoperatively and the patient underwent a total surgical correction of the defects, including simultaneous anastomosis of the left pulmonary artery to the pulmonary trunk. Results of the surgical repair of this case were successful. CONCLUSIONS. Although anomalous origin of the left pulmonary artery from the ascending aorta is a rare disease which was not diagnosed appropriately, successful results can be obtained when opportune and fortunate total surgical corrections are performed.

Early and midterm results of an alternative procedure to homografts in primary repair of truncus arteriosus communis.

BACKGROUND: Repair of truncus arteriosus communis (TAC) in the neonatal and early infant period has become a standard practice. We report our experience on primary repair of TAC with a bovine pericardial-valved woven Dacron conduit as an alternative procedure to homografts, with a focus on early and midterm results. METHODS: From January 2001 to December 2007, 15 patients with mean age 1.5 years (range 3 months to 8 years), underwent primary repair of simple TAC. Cases with cardiogenic shock, complex-associated cardiac lesions, or adverse anatomy of the truncal valve were excluded. The Collett and Edwards anatomical type classification of TAC was as follows: type I, 13 (87%); and type II, 2 (13%). Right ventricular outflow tract was reconstructed in all the cases with a bovine pericardial-valved woven Dacron conduit. RESULTS: Overall mortality was 6.6% (1 death due to severe pulmonary hypertension). At a mean follow-up of 31 months (range 6-51), there were no deaths (5-year actuarial survival 93.4%). Out of the 14 midterm survivors, three developed stenosis of the pericardial-valved woven Dacron conduit, but only one underwent interventional procedure including percutaneous balloon dilation with stenting for associated left pulmonary artery hypoplasia. The rate of patients with no surgical or percutaneous reinterventions performed because of obstruction of the right ventricular outflow tract reconstruction in the midterm (5 years) was 86%. CONCLUSIONS: Truncus arteriosus communis repair with a bovine pericardial-valved woven Dacron conduit can be performed with a very low perioperative mortality and satisfactory midterm morbidity, favorably compared with that reported for the use of homografts. Interventional cardiac catheterization may delay the time of reoperation for inevitable conduit replacement due to stenosis.

[Importance of the heart-brain binomial in the management of congenital heart disease]. / Importancia del binomio corazón-cerebro en el manejo integral de las cardiopatías congénitas.

A case of a two month infant with complex congenital heart disease (aortic coarctation with ventricular septal defect) associated to a cellular brain migration failure is presented. The management strategy consisted on the correction of congenital heart disease by means of a two-stage surgery without a further preoperative evaluation of the neurological status. The patient developed several perioperative complications such as two episodes of cardiac arrest, reconnection to cardiopulmonary bypass, cardiac tamponade, chilothorax and septic shock. A neurological protocol consisting in electroencephalography, brain magnetic resonance and Single Photon Emission Computed Tomography (SPECT) was practiced during the postoperative period, which detected microgyria with paquigyria and a cellular brain migration failure was suspected. The final outcome was death due to multisystemic failure and the autopsy confirmed the neurological disease, as well as poor life function prognosis. Should the heart-brain binomial had been considered in an integral preoperative evaluation, the therapeutical approach could have been modified.

Mexican registry of pediatric cardiac surgery: First report

Background: Currently, there is a spreading worldwide tendency to characterize health issues and to propose alternative solutions via the creation of computerized databases. The aim of this study was to present the results in a computerized database of pediatric cardiac surgeries developed under the auspices of the Mexican Association of Specialists in Congenital Heart Diseases (Asociación Mexicana de Especialistas en Cardiopatías Congénitas A.C) and coordinated by the collegiate group of Pediatric Cardiology and Surgery as petitioned by the National Institutes of Health and High Specialty Hospitals Coordinating Commission. Methods: We analyzed all cases registered in the database during a 1-year observation period (August 1, 2011 to July 31, 2012) by all major Health Ministry-dependent institutes and hospitals offering surgical services related to pediatric cardiopathies to the non-insured population. Results: Seven institutions participated voluntarily in completing the database. During the analyzed period, 943 surgeries in 880 patients with 7% reoperations (n = 63) were registered. Thirty-eight percent of the surgeries were performed in children <1 year of age. The five most common cardiopathies were patent ductus arteriosus (n = 96), ventricular septal defect (n = 86), tetralogy of Fallot (n = 72), atrial septal defect (n = 68), and aortic coarctation (n = 54). Ninety percent of surgeries were elective and extracorporeal circulation was used in 62% of surgeries. Global mortality rate was 7.5% with the following distribution in the RACHS-1 score categories: 1 (n = 4, 2%), 2 (n = 19, 6%), 3 (n = 22, 8%), 4 (n = 12, 19%), 5 (n = 1, 25%), 6 (n = 6, 44%), and non-classifiable (n = 2, 9%). Conclusions: This analysis provides a representative view of the surgical practices in cardiovascular diseases in the pediatric population at the national non-insured population level. However, incorporating other health institutions to the national registry database will render a more accurate panorama of the national reality in surgical practices in the population <18 years of age.

Anatomic diagnosis of congenital heart disease. A practical approach based on the sequentiality principle / Diagnóstico anatómico de las cardiopatías congénitas

Based on the sequentiality principle, this review proposes a practical method that allows the systematization of the anatomic diagnosis of congenital heart disease. We emphasize the need to use sequential connection between the different cardiac segments: atria, ventricles and great arteries. Five ordered steps are defined, which include determination of atrial situs and of the connection features between the ventricles and the great arteries. Related lesions and some additional special features are a second stage in the sequential analysis of congenital heart disease, which is also important for the integral diagnosis.

En esta revisión se propone, en base al principio de secuencialidad, un método práctico que permite sistematizar el diagnóstico anatómico de las cardiopatías congénitas. Se enfatiza la necesidad de utilizar la conexión secuencial entre los distintos segmentos cardiacos que son los atrios, los ventrículos y las grandes arterias. Se definen cinco pasos ordenados que incluyen, en primera instancia, la determinación del situs atrial y de las características de la conexión entre los ventrículos y las grandes arterias. Las lesiones asociadas y algunas particularidades adicionales ocupan una segunda etapa en el análisis secuencial de las cardiopatías congénitas, que también tienen importancia en el diagnóstico integral.

Problemática de las cardiopatías congénitas en México: Propuesta de regionalización / Congenital heart disease in Mexico: Regionalization proposal

Las malformaciones congénitas más frecuentes son las cardiopatías congénitas. La prevalencia reportada a nivel mundial va de 2.1 a 12.3 por 1000 recién nacidos. En nuestro país, se desconoce su prevalencia real; como causa de muerte infantil, se ubica en el sexto lugar en menores de un año y como la tercera causa en los niños entre uno y cuatro años; con base en la tasa de natalidad, se calcula que alrededor de 10 mil a 12 mil niños nacen con algún tipo de malformación cardiaca. Es de suma importancia conocer la magnitud del problema, identificar el número de niños que nacen cada año con una cardiopatía congénita y de manera desglosada por el tipo de la malformación; lo que permitiría determinar con mayor exactitud los recursos necesarios y planear su distribución. La regionalización tiene como objetivo la racionalización de los recursos con énfasis en servicios médicos muy especializados con la finalidad de lograr un mejor resultado para los pacientes. Por lo que, de manera simultánea a la creación de una base de datos fidedigna de las cardiopatías congénitas y, con base a la tasas de natalidad y mortalidad infantil secundaria a patología cardiovascular congénita y recursos en cada Estado, se debería proceder a regionalizar la atención. Lo anterior tendría diversos beneficios, ya que permitiría aumentar el número de casos atendidos, mejorar la calidad de la atención, aprovechar adecuadamente los recursos existentes y, seguramente, obtener una disminución de la mortalidad infantil.

Congenital cardiopathies are the most frequent congenital malformations. Reports of its prevalence around the world range from 2.1 to 12.3 for every 1000 newborns. Prevalence in our country remains unknown, but it probably occupies sixth place for mortality in infants less than a year old, and third place for mortality in those aged between 1 and 4 years. Based on birthrate, it is calculated that 10 to 12 000 infants in our country have some cardiac malformation. To understand the magnitude of the problem, it is important to identify the global number of newborns with some congenital cardiopathy each year and the type of malformation that they have, in order to determine the necessary resources and to plan their distribution. The main objective of regionalization is the justification of the resources with an emphasis in the specialized medical services to provide the best results for the patients. Hence, reason, based on the resources of each state, as well as their natality and infant mortality rates related to congenital cardiovascular pathology, we should proceed to regionalize the attention, and to simultaneously create a trustworthy database of the congenital cardiopathies. This should have many benefits, such as increase the number of total attended cases, improve the quality of attention, use appropriately the existent resources, and -surely- decrease the infant mortality.

Surgical repair of congenital mitral valve malformations / Reparación quirúrgica de las malformaciones congénitas de la válvula mitral

Surgical development of mitral valve repair techniques in pediatric patients has been slow because of the great variety in the presentation of congenital mitral valve malformations and the still unknown growing effect over the complex mitral valve apparatus. The aim of this study is to review our early an mid-term institutional outcomes in surgical repair of congenital mitral valve malformations. We studied retrospectively 14 patients with surgical repair of congenital mitral valve malformations in a 5 year period. Clinical and echocardiographic follow-up at a mean of 25 months was performed in all cases. Operative morbidity was 77% and operative mortality 7%. There were no late deaths. Clinical functional class stratification at the mid-term improved in 73% of survivors and did not change in the remaining 27%. Freedom from reoperation for mitral valve prosthetic replacement due to mitral valve repair failure was 84% at 30 days and 77% at 3.5 years. Surgical repair is probably the best technique option in the treatment of congenital malformations of the mitral valve, and transesophageal intraoperatory echocardiography must be highly recommended for evaluation of results.

El desarrollo de las técnicas de plastía mitral en el paciente pediátrico ha sido lento debido a la gran variabilidad de presentación de las lesiones mitrales congénitas, y al poco conocido efecto del crecimiento sobre el complejo aparato valvular mitral. El objetivo de este estudio fue revisar nuestra experiencia institucional en el corto y mediano plazo en la reparación quirúrgica de las valvulopatías mitrales congénitas. En forma retrospectiva se estudió una serie de 14 pacientes operados de reparación quirúrgica por valvulopatía mitral congénita en un lapso de cinco años. Se realizó seguimiento clínico y ecográfico en todos los casos a los 25 meses en promedio. La morbilidad operatoria de la serie fue de 77% y la mortalidad operatoria de 7%. No hubo mortalidad tardía. En el mediano plazo, la capacidad funcional mejoró en 73% de los sobrevivientes y se mantuvo en 27%. El índice de sobrevida libre de re-operación para cambio valvular por falla de la plastía mitral fue de 84% a los 30 días y de 77% a los 3.5 años. La reparación valvular mitral en el paciente pediátrico es probablemente la técnica de elección en el tratamiento quirúrgico de la patología valvular mitral congénita, y la ecocardiografía transesofágica intraoperatoria debe ser considerada como una herramienta altamente recomendable para evaluar los resultados.