Cardiac Laterality: Surgical Results of Right Atrial Isomerism.

Right atrial isomerism (RAI) is a complex entity with varying diagnostic and treatment outcomes due to its rarity. Treatment options range from palliative to corrective surgeries, resulting in heterogeneous outcomes. The aim of this study was to analyze the results obtained after cardiac surgery in patients with RAI. A retrospective study was conducted, including patients diagnosed with RAI who underwent cardiac surgery. Their follow-up was from 1 January 2010 to 31 March 2020. Demographic characteristics and perioperative conditions were described. Thirty-eight patients were included, the median age was 4 years (IQR 2-9.2) and 57.9% were men. The main diagnoses were atrioventricular canal (63.2%) and pulmonary stenosis (55.3%). The most common surgical procedures were modified Blalock-Taussig shunt (65.8%) and total cavopulmonary connection with an extracardiac conduit fenestrated without cardiopulmonary bypass (15.9%). We did not find any factors associated with negative outcomes in these patients. The overall survival was 86.8%, with a better outcome in those who did not require reintubation (log rank, p < 0.01). The survival of RAI was similar to other centers. Individuals with RAI should be evaluated rigorously to determine an adequate repair strategy, considering high morbidity and mortality.

Abordaje terapéutico de cardiopatías congénitas en el síndrome de Down / Congenital heart disease in Down's syndrome

Resumen Antecedentes: El síndrome de Down es la anomalía cromosómica más frecuente y se asocia con defectos cardiacos congénitos, elementos clínicos de una alta morbilidad y mortalidad infantil. Objetivo: Describir los desenlaces clínicos de los pacientes con síndrome de Down sometidos a cirugía e intervencionismo como tratamiento de las cardiopatías congénitas en esta institución. Material y métodos: Estudio retrospectivo que incluyó pacientes menores de 18 años con síndrome de Down y patología cardiaca asociada durante los últimos 10 años. Las variables estudiadas fueron: peso, talla, sexo, edad, tipo de cardiopatía, procedimiento correctivo, tiempo de estancia intrahospitalaria y en unidad de terapia intensiva, morbilidad y mortalidad. Resultados: 368 pacientes pediátricos fueron llevados a corrección quirúrgica o intervencionista, de los cuales 197 (54%) pertenecían al sexo femenino, la mediana de edad fue de 24 meses (rango intercuartílico [RIQ]: 14-48) en el grupo quirúrgico y de 36 meses (RIQ: 17-85) en el intervencionista. Las cardiopatías congénitas más frecuentes fueron: persistencia del conducto arterioso (31%), comunicación interventricular (28%), canal atrioventricular (CAV) (20%), comunicación interauricular (16%) y tetralogía de Fallot con el 4% respectivamente. La estancia hospitalaria fue de 9 días (RIQ: 7-15) en el grupo quirúrgico y de 3 días (RIQ: 2-5) en el hemodinámico. Las morbilidades fueron infección postoperatoria en 30 pacientes (14%) y en 19 pacientes (9%) bloqueo atrioventricular completo. La mortalidad global incluyendo tanto el quirúrgico como el intervencionista fue del 2%. Conclusiones: Los resultados terapéuticos, quirúrgicos e intervencionistas, en los niños con síndrome de Down y cardiopatías congénitas han mejorado en forma muy satisfactoria. Es de destacar la menor prevalencia del CAV en la población mexicana. Es indispensable realizar evaluación cardiológica a los niños con síndrome de Down y aquellos con cardiopatías congénitas llevarlos a corrección de manera oportuna para favorecer la sobrevida y calidad de vida.

Abstract Background: Down syndrome is the most common chromosomal abnormality, it is associated with a wide variety of congenital heart defects, being considered as clinical elements of high infant morbidity and mortality. Objective: To describe the clinical outcomes of patients with Down syndrome undergoing surgery and interventionism as treatment for congenital heart disease at this Institution. Material and methods: 368 patients with Down syndrome and associated congenital heart disease were diagnosed. The variables studied were weight, stature, sex, age, type of heart disease, corrective procedure, length of stay in the hospital and intensive care unit, morbidity and mortality. Results: 368 pediatric patients underwent surgical or interventional correction. Of which 197 (54%) were female, the median age was 24 months (interquartile range [IQR]: 14-48) in the surgical group and 36 months (IQR: 17-85) in the interventional group. The most frequent congenital heart diseases were: PCA (31%), IVC (28%), CAV (20%), ASD (16%) and tetralogy of Fallot with 4% respectively. Hospital stay was 9 days (IQR: 7-15) in the surgical group and 3 days (IQR: 2-5) in the hemodynamic group. Morbidities were postoperative infection in 30 patients (14%) and complete atrioventricular block in 19 patients (9%). Overall mortality including both surgical and interventional was 2%. Conclusions: The therapeutic, surgical and interventional results in children with Down syndrome and congenital heart disease have improved very satisfactorily. The lower prevalence of the atrioventricular canal in the Mexican population is noteworthy. It is essential to carry out a cardiological evaluation of children with Down syndrome and those with congenital heart disease to correct them in a timely manner to promote survival and quality of life.

[Congenital heart disease in Down's syndrome]. / Abordaje terapéutico de cardiopatías congénitas en el síndrome de Down.

Background: Down syndrome is the most common chromosomal abnormality, it is associated with a wide variety of congenital heart defects, being considered as clinical elements of high infant morbidity and mortality. Objective. Objective: To describe the clinical outcomes of patients with Down syndrome undergoing surgery and interventionism as treatment for congenital heart disease at this Institution. Material and methods: 368 patients with Down syndrome and associated congenital heart disease were diagnosed. The variables studied were weight, stature, sex, age, type of heart disease, corrective procedure, length of stay in the hospital and intensive care unit, morbidity and mortality. Results: 368 pediatric patients underwent surgical or interventional correction. Of which 197 (54%) were female, the median age was 24 months (interquartile range [IQR]: 14-48) in the surgical group and 36 months (IQR: 17-85) in the interventional group. The most frequent congenital heart diseases were: PCA (31%), IVC (28%), CAV (20%), ASD (16%) and tetralogy of Fallot with 4% respectively. Hospital stay was 9 days (IQR: 7-15) in the surgical group and 3 days (IQR: 2-5) in the hemodynamic group. Morbidities were postoperative infection in 30 patients (14%) and complete atrioventricular block in 19 patients (9%). Overall mortality including both surgical and interventional was 2%. Conclusions: The therapeutic, surgical and interventional results in children with Down syndrome and congenital heart disease have improved very satisfactorily. The lower prevalence of the atrioventricular canal in the Mexican population is noteworthy. It is essential to carry out a cardiological evaluation of children with Down syndrome and those with congenital heart disease to correct them in a timely manner to promote survival and quality of life.

Antecedentes: El síndrome de Down es la anomalía cromosómica más frecuente y se asocia con defectos cardiacos congénitos, elementos clínicos de una alta morbilidad y mortalidad infantil. Objetivo: Describir los desenlaces clínicos de los pacientes con síndrome de Down sometidos a cirugía e intervencionismo como tratamiento de las cardiopatías congénitas en esta institución. Material y métodos: Estudio retrospectivo que incluyó pacientes menores de 18 años con síndrome de Down y patología cardiaca asociada durante los últimos 10 años. Las variables estudiadas fueron: peso, talla, sexo, edad, tipo de cardiopatía, procedimiento correctivo, tiempo de estancia intrahospitalaria y en unidad de terapia intensiva, morbilidad y mortalidad. Resultados: 368 pacientes pediátricos fueron llevados a corrección quirúrgica o intervencionista, de los cuales 197 (54%) pertenecían al sexo femenino, la mediana de edad fue de 24 meses (rango intercuartílico [RIQ]: 14-48) en el grupo quirúrgico y de 36 meses (RIQ: 17-85) en el intervencionista. Las cardiopatías congénitas más frecuentes fueron: persistencia del conducto arterioso (31%), comunicación interventricular (28%), canal atrioventricular (CAV) (20%), comunicación interauricular (16%) y tetralogía de Fallot con el 4% respectivamente. La estancia hospitalaria fue de 9 días (RIQ: 7-15) en el grupo quirúrgico y de 3 días (RIQ: 2-5) en el hemodinámico. Las morbilidades fueron infección postoperatoria en30 pacientes (14%) y en 19 pacientes (9%) bloqueo atrioventricular completo. La mortalidad global incluyendo tanto el quirúrgico como el intervencionista fue del 2%. Conclusiones: Los resultados terapéuticos, quirúrgicos e intervencionistas, enlos niños con síndrome de Down y cardiopatías congénitas han mejorado en forma muy satisfactoria. Es de destacar lamenor prevalencia del CAV en la población mexicana. Es indispensable realizar evaluación cardiológica a los niños consíndrome de Down y aquellos con cardiopatías congénitas llevarlos a corrección de manera oportuna para favorecer la sobrevida y calidad de vida.

Stent en una fístula sistémico-pulmonar de Blalock-Taussig modificada en un adulto paliado con atresia tricúspide con estenosis infundibular pulmonar crítica / Stenting of a modified Blalock-Taussig shunt in an adult with palliated tricuspid atresia and critical pulmonary stenosis

Resumen: Los adultos con cardiopatía congénita compleja, con fisiología univentricular y flujo pulmonar disminuido, constituyen un reto terapéutico. Muchos de ellos reciben tratamiento paliativo con fístula sistémico-pulmonar desde la etapa pediátrica. Dicha fístula puede presentar oclusión o estenosis y ocasionar deterioro de la capacidad funcional y clínica. Colocar una nueva fístula sistémico-pulmonar a través de una cirugía se considera de alto riesgo, por lo que el uso de stents a través de cateterismo cardíaco surge como una opción que ha tenido buenos resultados. Se describe el caso de un adulto con atresia tricúspide con fístula sistémico-pulmonar en la etapa pediátrica, quien acudió al servicio de urgencias por deterioro de su clase funcional y desaturación en aire ambiente de hasta un 64%; en la tomografía cardíaca se evidenció estenosis del tercio distal de la fístula sistémico-pulmonar, por lo que se decidió colocar, mediante cateterismo cardíaco intervencionista, dos stents (Express LD vascular 6 x 37 mm y 6 x 27 mm), luego de lo cual la saturación sistémica fue del 75%, por lo que se consideró exitosa la colocación de los dispositivos. La oclusión de estas fístulas es una complicación habitual, que genera disminución de la perfusión pulmonar con los subsecuentes síntomas respiratorios, disminución de la saturación y la oxigenación, cianosis y acidosis metabólica, y puede generar un evento que amenaza la vida si se presenta de manera aguda. La recanalización transcatéter de una fístula sistémico-pulmonar con stent es una alternativa adecuada para evitar un riesgo quirúrgico y arroja resultados óptimos.

Abstract: Adults with complex congenital heart disease with univentricular physiology and decreased in the pulmonary flow constitute a therapeutic challenge, many of these patients are palliated with modified Blalock-Taussig shunt (mBTS). The mBTS can develop occlusion or stenosis over time with deterioration of functional class and poor exercise tolerance. Dysfunction of a mBTS is a life-threatening situation requiring urgent therapy. A new surgical palliation is a high-risk procedure, so stenting a mBTS can be an alternative. We report a 29-year-old female with tricuspid atresia and pulmonary infundibular stenosis palliated with mBTS with progressive cyanosis (oxygen saturation of 54%) and dyspnea; computed tomography revealed a stenosed mBTS, and an interventional percutaneous approach was made. The stenting of the mBTS was made with two stents (Express LD vascular 6 x 37 mm and 6 x 27 mm). Oxygen saturation post-procedure increase to 70-75%. Occlusion of these shunts are a common major complication, leading to a decrease in pulmonary perfusion with subsequent respiratory symptoms, low saturation and oxygenation, cyanosis, metabolic acidosis and can generate a life-threatening event if it occurs acutely. Stent implantation into a previous mBTS through cardiac catheterization can be an alternative to shunt operation in patients with cyanotic congenital heart disease having a good long-term results.

Coronary Revascularization in Patients With Cardiac Sequelae of Kawasaki Disease at a Single Center.

Background: Kawasaki disease (KD) is a self-limited vasculitis that mainly affects infants and preschool-age children, characterized by coronary aneurysms and/or stenoses that may lead to the occurrence of ischemia, heart attack, and sudden death. This study aimed to evaluate the clinical and surgical results of pediatric patients with KD and cardiac sequelae who underwent coronary artery bypass grafting. Methods: A retrospective study was carried out. Patients with a diagnosis of KD and cardiac sequelae who underwent coronary artery bypass grafting from January 1, 2004, through March 31, 2021, were included: preoperative characteristics and conditions. Results: Ten patients were included, with a mean age of 6.4 ± 3.7 years; 80% males. Seven (70%) had the compromise of 2 coronary arteries, the most affected being the left anterior descendent artery (36.9%) with aneurysm and the right coronary artery (36.8%) with stenosis. 40% had preoperative moderate-to-severe ventricular dysfunction. The graft most frequently used was the right internal thoracic artery (47.6%), and the most frequent coronary bypass target vessel was the left anterior descending artery (38.1%). There was no early mortality or deaths during follow-up; 90% remained in functional class I. Among the cohort as a group, there was an overall improvement in postoperative ventricular function (P = .03). This persisted, being unchanged at subsequent follow-up (P = .95). Conclusions: Coronary artery bypass grafting is an appropriate treatment option for children with cardiac sequelae of KD, with excellent surgical results. Furthermore, this surgical procedure avoids the deterioration of left ventricular function in pediatric age.

Total anomalous pulmonary venous connection: 16 years of surgical results in a single center.

PURPOSE: The aim of the study was to analyze the surgical outcome of patients with total anomalous pulmonary venous connection (TAPVC) who underwent cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of TAPVC undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2003 and June 30, 2019 were included. Descriptive statistics were calculated, as well as a bivariate analysis of the variables associated with mortality. A logistic regression model was included to determine risk factors associated with the main outcome and survival was analyzed using the Kaplan-Meier method. RESULTS: A total of 5314 patients diagnosed with congenital heart disease (CHD) underwent surgery, 414 (7.8%) were patients with TAPVC, with an average age of 17.1 ± 34.6 months, 58.2% were male. It was frequent in infants (61.6%) and preschool (19.6%). Predominant type was supracardiac TAPVC (47.4%). Pulmonary venous obstruction (PVO) occurred in 32.1%. Risk factors associated with mortality were infracardiac TAPVC (odds ratio [OR]: 3.26; 95% confidence interval [CI]: 1.17-9.03; p = .02), PVO (OR: 2.56; 95% CI: 1.05-6.22; p = .03) and postoperative mechanical ventilation (OR: 1.005; 95% CI: 1.002-1.008; p = .01). Overall survival was 87.2%, with better outcomes in adolescents (100%), children (94.1%), mixed TAPVC (96%), and cardiac TAPVC (91.9%; p < .001). CONCLUSIONS: The survival of our institution after surgical correction of TAPVC is similar to that of other referral centers, where patients with infracardiac TAPVC and newborns worse outcomes. All patients must undergo a rigorous evaluation to determine an adequate repair strategy.

Cardiac surgery in patients with atrial isomerism: Long-term results and outcomes.

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 Chávez, 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.

Impact of the COVID-19 Pandemic on Congenital Cardiac Surgeries at a National Referral Center in Mexico.

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.

Rehabilitation of pulmonary arteries: Hybrid treatment in the unilateral absence of the pulmonary artery.

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.

Transcatheter closure of paravalvular leaks: short- and medium-term outcomes.

Background: Paravalvular leak (PVL) is a frequent and important complication after surgical valvular replacement that can cause heart failure and 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 PVLs. Methods: Single-center registry of consecutive patients with post-surgical PVLs 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. Results: Twenty-one PVLs (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 three-dimensional 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, three cases required valvular surgery (15%). Conclusions: Transcatheter closure of PVLs 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.

Introducción: 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. Métodos: 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. Resultados: 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%). Conclusiones: 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.

Transcatheter closure of paravalvular leaks: short- and medium-term outcomes / Cierre transcatéter de fugas paravalvulares: resultados a corto y mediano plazo

Abstract Background: Paravalvular leak (PVL) is a frequent and important complication after surgical valvular replacement that can cause heart failure and 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 PVLs. Methods: Single-center registry of consecutive patients with post-surgical PVLs 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. Results: Twenty-one PVLs (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 three-dimensional 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, three cases required valvular surgery (15%). Conclusions: Transcatheter closure of PVLs 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.

Resumen Introducción: 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. Métodos: 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. Resultados: 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%). Conclusiones: 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.

Transcatheter closure of paravalvular leaks: short and medium-term outcomes.

Background: 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. Methods: 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. Results: 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%). Conclusions: 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.

Introducción: 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. Métodos: 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. Resultados: 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%). Conclusiones: 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.

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.

[Kawasaki's disease, current concepts of coronary revascularization surgery in the pediatric poblation]. / Enfermedad de Kawasaki, conceptos sobre la cirugía de revascularización coronaria en edad pediátrica.

Kawasaki's disease is by now the first cause of pediatric acquired cardiopathies in many countries, even more than rheumatic fever. Probably the most common complication of this disease is coronary affection, which often causes stenosis. Treatment of the acute and chronic coronary events in children is based on the knowledge acquired from the disease in adults. The increasing experience in pediatric patients with this pathology has led to better ways of handling and treating this disease.

Enfermedad de Kawasaki, conceptos sobre la cirugía de revascularización coronaria en edad pediátrica / Kawasaki's disease, current concepts of coronary revascularization surgery in the pediatric poblation

Kawasaki's disease is by now the first cause of pediatric acquired cardiopathies in many countries, even more than rheumatic fever. Probably the most common complication of this disease is coronary affection, which often causes stenosis. Treatment of the acute and chronic coronary events in children is based on the knowledge acquired from the disease in adults. The increasing experience in pediatric patients with this pathology has led to better ways of handling and treating this disease.

Diverticulum of Kommerell / Divertículo de Kommerell

A case of an 11-months-old girl with vomiting and laryngeal stridor is presented, and in whom a Kommerell diverticulum was demonstrated, which is a rare variant of the incomplete vascular ring. It is well known that the magnetic resonance is the best study to define this malformation. In this patient, it was the cardiac catheterization which allowed to define the structures that constituted the vascular ring. The patient was surgically treated in a successful way. The embryological knowledge of the transformations of the aortic arches offers great assistance in the understanding of all the types of vascular rings.

Se presenta el caso de lactante de 11 meses con historia de vómitos y estridor laríngeo secundaria a una rara variante de anillo vascular, divertículo de Kommerell. La resonancia magnética es considerada como el estudio ideal para definir la malformación, sin embargo, en este paciente, fue la angiografía la que permitió definir las estructuras del anillo vascular. El paciente fue tratado quirúrgicamente de manera exitosa. El conocimiento embriológico permite un adecuado entendimiento de los diferentes tipos de anillo vascular.

Risk factors for prolonged mechanical ventilation after surgical repair of congenital heart disease / Factores de riesgo por ventilación mecánica prolongada tras cirugía cardíaca por cardiopatía congénita

Objective: The purpose of this study was to determine factors contributing to prolonged mechanical ventilation in children following surgery for congenital heart defects. Design: Prospective cohort trial. Setting: Critical Care Unit. "Ignacio Chavez" National Heart Institute, México; from January to December 2000. Patients: A total of 318 consecutive patients <18 years old who underwent cardiovascular surgical procedures for congenital heart defects were enrolled in this study. Of these, 297 patients were admitted to the intensive care unit with respiratory support and 2.8% required prolonged mechanical ventilation (MV) > 120 hours. Measurements and main results: Patients with cardiac failure had MVt ime of 214 ± 200 hours, whereas those without it had MV time of 33 ± 73 hours (p > 0.001). Patients with severe pulmonary hypertension had MV time of 160 ± 176 hours, while those who did not had MV time of 47 ± 105 hours (p < 0.001). Twenty-four patients (8.5%) had extubation failure, in 79% them due to hemodynamic alteration during the respiratory support time of 277 ± 188 hours versus the rest of the group with MV time of 41 ± 92 hours (p < 0.001). Factors associated with prolonged MV(> 120 hours) were: patients ages of < 1 year, pulmonary hypertension, and cardiac failure, and represented the greatest risk (90%, Cl 58 to 99) of prolonging MV. Mortality rate was 34% for patients with prolonged MV. Times of aortic clamping and cardiopulmonary bypass were not significant risk factors for prolonged respiratory support. Conclusion: Patients ages of < 1 year old, pulmonary hypertension, and cardiac failure were significant risk factors for prolonged respiratory support.

Objetivo: El propósito del estudio fue establecer los factores de riesgo para ventilación mecánica prolongada en pacientes menores de 18 años sometidos a cirugía cardíaca. Tipo de estudio: Prospectivo. Unidad de Cuidados Intensivos Postoperatorios. Instituto Nacional de Cardiología "Ignacio Chávez". Del 1º de enero al 31 de diciembre de 2000. Material y métodos: Fueron analizados 318 pacientes consecutivos menores de 18 años con cardiopatía congénita sometidos a cirugía cardíaca paliativa o correctiva. Resultados: Los pacientes con insuficiencia cardíaca tuvieron un tiempo de ventilación mecánica de 214 ± 200 horas, mientras los pacientes sin insuficiencia cardíaca sólo requirieron 33 ± 73 horas (p > 0.001). En presencia de hipertensión pulmonar severa el tiempo de asistencia ventilatoria fue de 160 ± 176 horas, en comparación con los que no la tenían, que fue de 47 ± 105 (p < 0.001). En 24 pacientes (8.5%) fue la extubación fallida siendo por factor hemodinámico en 79% y requirieron de ventilación mecánica de 277 ±188 horas versus el resto del grupo con 41 ± 92 horas (p < 0.001). Los factores asociados con ventilación mecánica prolongada (> 120 horas) fueron: edad < 1 año, hipertensión pulmonar severa e insuficiencia cardíaca (90%, LC 58 a 99). La mortalidad, en los pacientes que requirieron ventilación mecánica prolongada, fue del 34%. El tiempo de circulación extracorpórea o de pinzamiento aórtico no fueron factores de riesgo para la necesidad de ventilación mecánica prolongada. Conclusiones: En nuestra institución los factores de riesgo para ventilación mecánica prolongada, en pacientes menores de 18 años operados de cirugía cardíaca paliativa o correctiva fueron: pacientes menores de 1 año, hipertensión pulmonar severa e insuficiencia cardíaca.

Risk factors for prolonged mechanical ventilation after surgical repair of congenital heart disease.

OBJECTIVE: The purpose of this study was to determine factors contributing to prolonged mechanical ventilation in children following surgery for congenital heart defects. DESIGN: Prospective cohort trial. SETTING: Critical Care Unit. "Ignacio Chávez" National Heart Institute, México; from January to December 2000. PATIENTS: A total of 318 consecutive patients < 18 years old who underwent cardiovascular surgical procedures for congenital heart defects were enrolled in this study. Of these, 297 patients were admitted to the intensive care unit with respiratory support and 2.8% required prolonged mechanical ventilation (MV) > 120 hours. MEASUREMENTS AND MAIN RESULTS: Patients with cardiac failure had MV time of 214 +/- 200 hours, whereas those without it had MV time of 33 +/- 73 hours (p > 0.001). Patients with severe pulmonary hypertension had MV time of 160 +/- 176 hours, while those who did not had MV time of 47 +/- 105 hours (p < 0.001). Twenty-four patients (8.5%) had extubation failure, in 79% them due to hemodynamic alteration during the respiratory support time of 277 +/- 188 hours versus the rest of the group with MV time of 41 +/- 92 hours (p < 0.001). Factors associated with prolonged MV (> 120 hours) were: patients ages of < 1 year, pulmonary hypertension, and cardiac failure, and represented the greatest risk (90%, CI 58 to 99) of prolonging MV. Mortality rate was 34% for patients with prolonged MV. Times of aortic clamping and cardiopulmonary bypass were not significant risk factors for prolonged respiratory support. CONCLUSION: Patients ages of < 1 year old, pulmonary hypertension, and cardiac failure were significant risk factors for prolonged respiratory support.