Results of percutaneous closure with device in congenital heart disease of the atrial septal defect type / Resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular

Congenital Interatrial Septal Defects (TEA) are among the most common Congenital Heart Diseases in the population, where Atrial Septal Defect stands out, which is produced by a left-right short circuit that provides greater survival and less morbidity and mortality. The objective was to determine the results of Percutaneous Closure with a Device in Congenital Heart Disease of the Atrial Communication type at the Carlos Andrade Marín Specialty Hospital (Quito) in the Hemodynamics service from January 2009 to July 2019. It is a retrospective descriptive cross-sectional study . With a population of 122 cases from the AS400 registry. Thus, it was determined: there is a female predominance in a ratio of 2:1, the ranges for defect intervention were 5.5 mm to 35 mm, post-procedure pulmonary artery pressures (systolic 18-25 mmHg and diastolic 6-10 mmHg) in 95% of patients, ventricular overload in 76% in pre-procedure patients. Concluding that 96% of our population has a significant post-procedure improvement.

Los defectos del tabique interauricular congénitos (TEA) se encuentran entre las cardiopatías congénitas más comunes en la población donde resalta la comunicación interauricular que es producido por un cortocircuito izquierda derecha que brinda mayor sobrevida y menor morbimortalidad. El objetivo fue determinar los resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular en el hospital de especialidades Carlos Andrade Marín (Quito) en el servicio de hemodinamia en el período enero 2009 a julio 2019. Es un estudio transversal descriptivo retrospectivo. Con una población 122 casos del registro de AS400. Encontramos un predominio en sexo femenino en una proporción de 2:1, los rangos para intervención del defecto fueron de 5,5 mm a 35 mm, las presiones de la arteria pulmonar pos-procedimiento (sistólica de 18-25 mmHg y diastólica de 6-10 mmHg) en un 95% de los pacientes, sobrecarga ventricular en un 76% en los pacientes pre-procedimiento. Concluyendo que el 96% de nuestra población tiene una mejoría significativa post-procedimiento.

Efficacy of fenestrated atrial septal defect occulders on pulmonary hypertension dogs / 中华心血管病杂志

Objective: To explore the short-term efficacy of fenestrated atrial septal defect (ASD) occulders in the treatment of pulmonary arterial hypertension (PAH). Methods: Thirty-six healthy dogs were divided into the balloon atrial septostomy (BAS)+fenestrated ASD occulders group (n=12), BAS group (n=12) and non-septostomy group (n=12). PAH was induced by intra-atrial injection of dehydrogenized monocrotaline (1.5 mg/kg) in all dogs. Animals in the BAS+fenestrated ASD occulders group underwent atrial septal puncture and fenestrated ASD occulders implantation. Animals in the BAS group underwent balloon atrial septostomy. The non-septostomy group received no surgical intervention. The hemodynamic indexes and blood N-terminal pro-B-type natriuretic peptide (NT-proBNP) of dogs were measured before modeling, 2 months after modeling, 1, 3, and 6 months after surgery, respectively. Echocardiography was performed to observe the patency of the shunt and atrial septostomy of the dogs in the BAS+fenestrated ASD occulders group and BAS group at 1, 3, and 6 months after surgery. Three dogs were sacrificed in each group at 1, 3, and 6 months after surgery, respectively. Atrial septal tissue and fenestrated ASD occulders were removed to observe the patency and endothelialization of the device. Lung tissues were obtained for hematoxylin-eosin (HE) staining to observe the inflammatory cells infiltration and the thickening and narrowing of the pulmonary arterials. Results: Among 36 dogs, 2 dogs died within 24 hours after modeling, and 34 dogs were assigned to BAS+fenestrated ASD occulders group (n=12), BAS group (n=11), and non-septostomy group (n=11). Compared with BAS group, the average right atrial pressure (mRAP) and NT-proBNP of dogs in the BAS+fenestrated ASD occulders group were significantly reduced at 3 months after surgery (P<0.05), and the cardiac output (CO) was significantly increased at 6 months after surgery, arterial oxygen saturation (SaO2) was also significantly reduced (P<0.05). Compared with non-septostomy group, dogs in the BAS+fenestrated ASD occulders group had significantly lower mRAP and NT-proBNP at 1, 3, and 6 months after surgery (P<0.05), and higher CO and lower SaO2 at 6 months after surgery (P<0.05). Compared with the non-septostomy group, the dogs in the BAS group had significantly lower mRAP and NT-proBNP at 1 month after surgery (P<0.05), and there was no significant difference on mRAP and NT-proBNP at 3 and 6 months after surgery (P>0.05). Echocardiography showed that there was a minimal right-to-left shunt in the atrial septum in the BAS group at 1 month after the surgery, and the ostomy was closed in all the dogs in the BAS group at 3 months after the surgery. There was still a clear right-to-left shunt in the dogs of BAS+fenestrated ASD occulders group. The shunt was well formed and satisfactory endothelialization was observed at 1, 3 and 6 months after surgery. The results of HE staining showed that the pulmonary arterials were significantly thickened, stenosis and collapse occurred in the non-septostomy group. Pulmonary microvascular stenosis and inflammatory cell infiltration in the pulmonary arterials were observed in the non-septostomy group. Pulmonary arterial histological results were comparable between BAS+fenestrated ASD occulders group and non-septostomy group at 6 months after surgery . Conclusions: The fenestrated ASD occulder has the advantage of maintaining the open fistula hole for a longer time compared with simple balloon dilation. The fenestrated ASD occulder can improve cardiac function, and it is safe and feasible to treat PAH in this animal model.

Atrial Septal Aneurysm and Atrial Septal Defect Association - an Uncommon But Well-Recognized Association

Abstract Atrial septal aneurysm (ASA) is an uncommon but well-recognized cardiac abnormality. This educational text reviews the case of a 54-year-old female presenting an ASA related to a small ostium secundum atrial septal defect. The considerable signs and symptoms, interestingly, have not been justified by the clinical and hemodynamic investigations. So, we opted for a better imaging investigation with cardiac catheterization and transesophageal echocardiography. The surgical process was earlier indicated and performed with aid of cardiopulmonary bypass.

Efficacy and safety of a parylene-coated occluder for atrial septal defect: a prospective, multi-center, randomized controlled clinical trial / 中华医学杂志(英文版)

BACKGROUND@#Nitinol-containing devices are widely used in clinical practice. However, there are concerns about nickel release after nitinol-containing device implantation. This study aimed to compare the efficacy and safety of a parylene-coated occluder vs. a traditional nitinol-containing device for atrial septal defect (ASD).@*METHODS@#One-hundred-and-eight patients with ASD were prospectively enrolled and randomly assigned to either the trial group to receive a parylene-coated occluder (n = 54) or the control group to receive a traditional occluder (n = 54). The plugging success rate at 6 months after device implantation and the pre- and post-implantation serum nickel levels were compared between the two groups. A non-inferiority design was used to prove that the therapeutic effect of the parylene-coated device was non-inferior to that of the traditional device. The Cochran-Mantel-Haenszel chi-squared test with adjustment for central effects was used for the comparison between groups.@*RESULTS@#At 6 months after implantation, successful ASD closure was achieved in 52 of 53 patients (98.11%) in both the trial and control groups (95% confidence interval (CI): [-4.90, 5.16]) based on per-protocol set analysis. The absolute value of the lower limit of the 95% CI was 4.90%, which was less than the specified non-inferiority margin of 8%. No deaths or severe complications occurred during 6 months of follow-up. The serum nickel levels were significantly increased at 2 weeks and reached the maximum value at 1 month after implantation in the control group (P  0.05).@*CONCLUSIONS@#The efficacy of a parylene-coated ASD occluder is non-inferior to that of a traditional uncoated ASD occluder. The parylene-coated occluder prevents nickel release after device implantation and may be an alternative for ASD, especially in patients with a nickel allergy.

Comparison of postoperative changes in inflammatory marker levels between transthoracic and transcatheter device closures of atrial septal defects in children

Abstract Objective: To explore the postoperative changes in inflammatory markers in children who underwent device closure of an atrial septal defect (ASD) via a transthoracic or transcatheter approach. Methods: The experimental and clinical data were retrospectively collected and analyzed for a total of 53 pediatric patients between September 2018 and December 2018. According to the different treatments, 19 patients who underwent transthoracic device closure were assigned to group A, and the remaining 34 patients who underwent a transcatheter approach were assigned to group B. Results: All patients were successfully occluded without any device-related severe complication. Compared with the preoperative levels, the postoperative levels of most inflammatory cytokines in both groups were significantly increased and reached a peak on the first day after the procedure. The level of postoperative inflammatory cytokines was significantly lower in group B than in group A. In addition, there was no significant difference in procalcitonin before and after the transcatheter approach. Conclusion: Systemic inflammatory reactions occurred after transthoracic or transcatheter device closure of ASDs in pediatric patients. However, these inflammatory reactions were more significant in patients who underwent a transthoracic approach than in patients who underwent a transcatheter approach.

Comparative study between surgical repair of atrial septal defect via median sternotomy, right submammary thoracotomy, and right vertical infra-axillary thoracotomy

Abstract Objective: To compare the results of surgical repair via median sternotomy, right submammary thoracotomy, and right vertical infra-axillary thoracotomy for atrial septal defect (ASD). Methods: This is a retrospective analysis of the relative perioperative and postoperative data of 136 patients who underwent surgical repair for ASD with the abovementioned three different treatments in our hospital from June 2014 to December 2017. Results: The results of the surgeries were all satisfactory in the three groups. No statistically significant difference was found in operative time, duration of cardiopulmonary bypass, blood transfusion amount, postoperative mechanical ventilation time, duration of intensive care unit, length of hospital stay, and hospital costs. However, the median sternotomy group had the longest incision. Meanwhile, there was no significant difference in postoperative complications. Conclusion: All three types of surgical incisions can be safely and effectively used to repair ASD. The treatments via right submammary thoracotomy and right vertical infra-axillary thoracotomy have advantages over the treatment via median sternotomy in cosmetic results and should be the recommended options.

Rare association between atrioventricular septal defect and partial anomalous pulmonary venous connection

Abstract Clinical data: Infant, 7 months, female, referred to our department at one month of age, suspecting of congenital heart disease for further investigation. Chest radiography: Demonstrates cardiomegaly and prominent pulmonary vascular markings. Electrocardiography: Shows right ventricular hypertrophy and left anterior fascicular block. Echocardiography: Evidenced common atrioventricular valve with two orifices and the left superior pulmonary vein draining on the brachiocephalic vein. Computed tomography angiography: This complementary imaging exam was performed to confirm the diagnosis. Diagnosis: The patient presented an association between AVSD and PAPVC, a rare combination. The clinical picture of heart failure was preponderant, characterized by need for diuretics and complementary exams findings, and early surgical treatment was indicated. Operation: The operation was performed through a median sternotomy with 123 minutes of cardiopulmonary bypass and 89 minutes of cross-clamping time. The patient had no postoperative complications, remaining 10 days hospitalized.

Reparación de válvula mitral y cierre de comunicación interauricular, asistida por robot: experiencia inicial / Robotic mitral valve repair and closure of atrial septal defect: report of 13 procedures

Background Robot-assisted minimally invasive heart surgery is an effective alternative when compared with classical approaches. It has a low mortality and postoperative complications and its long-term durability is comparable with conventional techniques. Aim: To report short- and long-term results with the use of a robot-assisted transthoracic approach. Patients and Methods: Review of patients undergoing heart surgery between 2015 and 2019 using a robot assisted minimally invasive technique in a single center. We analyzed demographic characteristics, surgical and early ultrasound results. Results: Thirteen procedures were reviewed, nine mitral valve repairs (MVR) in patients aged 61 ± 21 years (seven males) and four atrial septal defect (ASD) closures in patients aged from 24 to 52 years (three men). For MVR, the average extracorporeal circulation and myocardial ischemia times were 120 ± 20.9 and 89 ± 21 minutes, respectively. The median hospitalization was four days. Two cases of MVR had postoperative complications. There was no mortality. All cases showed improvement in their symptoms. Ultrasound findings showed no postoperative mitral insufficiency except in one case. Conclusions: We report very good results in both complex mitral repair and CIA closure, comparable to centers with high standards in minimally invasive robot-assisted heart surgery.

Anesthetic management for patient with severe cyanosis following bioprosthetic valve stenosis / Manejo anestésico de paciente com cianose grave após estenose de valva bioprotética

Abstract We presented a 39-year-old female patient with life-threatening hypoxemia after tricuspid valve replacement because of Ebstein's anomaly. And the severe cyanosis is due to bioprosthetic valve stenosis and atrial septal defect. Anesthetic management of a patient with severe obstructive prosthetic valve dysfunction can be challenging. Similar considerations should be given to patients with Ebstein's anomaly to maintain the pressure equalized between the right and left atrial. Transesophageal echocardiography and cerebral oxygen saturation provided real time information in perioperative care.

Resumo Apresentamos o caso de uma paciente de 39 anos, com hipoxemia em risco de vida após a substituição da valva tricúspide devido à anomalia de Ebstein e cianose grave devido à estenose de valva bioprotética e comunicação interatrial. O manejo anestésico de um paciente com disfunção obstrutiva grave de prótese valvar pode ser um desafio. Os pacientes com anomalia de Ebstein também precisam de atenção especial para manter a pressão equalizada entre o átrio direito e o esquerdo. A ecocardiografia transesofágica e a saturação cerebral de oxigênio forneceram informações em tempo real nos cuidados perioperatórios.

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Anterior minithoracotomy: a safe approach for surgical asd closure & asd device retrieval

Abstract Objective: Midline sternotomy is the preferred approach for device migration following transcatheter device closure of ostium secundum atrial septal defect. Results of patients operated for device migration were retrospectively reviewed after transcatheter closure of atrial septal defect. Methods: Among the 643 patients who underwent atrial septal defect with closure device, 15 (2.3%) patients were referred for device retrieval and surgical closure of atrial septal defect. Twelve patients underwent device retrieval and surgical closure of atrial septal defect through right antero-lateral minithoracotomy with femoral cannulation. Three patients were operated through midline sternotomy. Results: Twelve patients operated through minithoracotomy did not require conversion to sternotomy. Due to device migration to site of difficult access through thoracotomy, cardiac tamponade and hemodynamic instability, respectively, three patients were operated through midline sternotomy. Mean aortic cross-clamp time and cardiopulmonary bypass time were 28.1±17.7 and 58.3±20.4 minutes, respectively. No patient had surgical complication or mortality. Mean intensive care unit and hospital stay were 1.6±0.5 days and 7.1±2.2 days, respectively. Postoperative echocardiography confirmed absence of any residual defect and ventricular dysfunction. In a mean follow-up period of six months, no mortality was observed. All patients were in New York Heart Association class I without wound or vascular complication. Conclusion: Minithoracotomy with femoral cannulation for cardiopulmonary bypass is a safe-approach for selected group of patients with device migration following transcatheter device closure of atrial septal defect without increasing the risk of cardiac, vascular or neurological complications and with good cosmetic and surgical results.

Peratrial device closure of atrial septal defect under transesophageal echocardiographic guidance without fluoroscopy compared to conventional on-pump surgical closure

Abstract Objective: This study is designed to evaluate the advantages between peratrial device closure under transesophageal echocardiographic guidance and open heart surgery in atrial septal defect. Methods: From November 2011 to September 2014, 28 patients with atrial septal defect were treated. Fourteen patients received peratrial device closure under transesophageal echocardiographic guidance (TEE group) and 14 patients received cardiopulmonary bypass (CPB group). Clinical parameters during intraoperative and postoperative periods were examined. Results: All patients recovered after surgery without serious complications. Compared with that in CPB group, clinical observations in TEE group showed significant decreases in the operation time (193.6±35.5 vs. 77.4±22.7 min, P<0.05), periods in intensive care unit (31.6±23.3 vs. 17.5±8.1 hours, P<0.05), fluid volume after operation (502.5±439.3 vs. 32.5±7.3 ml, P<0.05), postoperative length of hospital stay (8.9±2.8 vs. 6.8±2.4 days, P<0.05) and total hospitalization cost (7205.9±1617.6 vs. 5882.3±441.2 $, P<0.05). Conclusion: The peratrial device closure of atrial septal defect under transesophageal echocardiographic guidance is a mini-invasive, simple, safe and effective intervention. Its use in the clinical practice should be encouraged.

Erosión muy tardía de la aurícula derecha con dispositivo Amplatzer®; reporte de un caso / Very late erosion of the right atrium with Amplatzer® device

Resumen La erosión tardía de la pared auricular y de la aorta es una causa infrecuente, pero potencialmente mortal en los pacientes que han sido sometidos al cierre percutáneo de un defecto del septum auricular, en especial cuando existe una alta relación entre el tamaño del dispositivo escogido con el tamaño del defecto y la presencia de bordes insuficientes. Presentamos el caso de un hombre de 70 años con un episodio de colapso hemodinámico secundario a taponamiento cardiaco debido a erosión muy tardía de la aurícula derecha por un Amplatzer® implantado 4 años atrás para el cierre de un defecto septal auricular. Conclusión La erosión tardía de los dispositivos de cierre percutáneo para los defectos atriales, aunque infrecuente, deberá ser tenida en cuenta en los pacientes que presentan colapso hemodinámico.

Abstract Late atrial wall and aorta erosion is a rare cause of potential fatallity in patients who have undergone percutaneous closure of an atrial septal defect, especially when a high ratio device to defect is chosen, and an insufficient aortic rim is present. We report the case of a 70 year old male with an episode of hemodinamic collapse secondary to cardiac tamponade due to a late erosion of a 4 years ago implanted Amplatzer® used to close an atrial septal defect. Conclusion Very late wall erosion of an atrial septal closure device, although uncommon, should be suspected in patients presenting with hemodynamic collapse.

Spectrum of cardiac lesions associated with Isolated Cleft Mitral Valve and their impact on therapeutic choices / Espectro de Lesões Cardíacas Associadas à Fissura Isolada da Valva Mitral e Impacto das Lesões Sobre as Opções Terapêuticas

Abstract Background: Isolated cleft mitral valve (ICMV) may occur alone or in association with other congenital heart lesions. The aim of this study was to describe the profile of cardiac lesions associated with ICMV and their potential impact on therapeutic management. Methods: We conducted a descriptive study with data retrieved from the Congenital Heart Disease (CHD) single-center registry of our institution, including patients with ICMV registered between December 2008 and November 2014. Results: Among 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had Down syndrome. Seventeen patients (77.3%) had associated lesions, including 11 (64.7%) with accessory chordae in the left ventricular outflow tract (LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD), three had secundum atrial septal defect, and four had patent ductus arteriosus. Thirteen patients (59.1%) required surgical repair. The decision to proceed with surgery was mainly based on the severity of the associated lesion in eight patients (61.5%) and on the severity of the mitral regurgitation in four patients (30.8%). In one patient, surgery was decided based on the severity of both the associated lesion and mitral regurgitation. Conclusion: Our study shows that ICMV is rare and strongly associated with Down syndrome. The most common associated cardiac abnormalities were VSD and accessory chordae in the LVOT. We conclude that cardiac lesions associated with ICMV are of major interest, since in this study patients with cardiac lesions were diagnosed earlier. The decision to operate on these patients must take into account the severity of both mitral regurgitation and associated cardiac lesions.

Resumo Fundamento: A fissura isolada da valva mitral (FIVM) pode ocorrer isoladamente ou em associação com outras lesões cardíacas congênitas. O objetivo deste estudo foi descrever o perfil das lesões cardíacas associadas à FIVM e o potencial impacto dessas lesões na terapêutica. Métodos: Realizamos um estudo descritivo com dados obtidos do registro unicêntrico Congenital Heart Disease (CHD) de nossa instituição incluindo pacientes com FIVM registrados entre dezembro de 2008 e novembro de 2014. Resultados: Entre 2177 pacientes identificados no registro CHD, 22 (1%) apresentavam FIVM. A mediana de idade ao diagnóstico foi de 5 anos (6 dias a 36 anos). Nove pacientes (40,9%) apresentavam síndrome de Down. Dezessete pacientes (77,3%) apresentavam lesões associadas, incluindo 11 (64,7%) com cordoalha acessória na via de saída do ventrículo esquerdo (VSVE) sem obstrução, 15 (88,2%) com comunicação interventricular (CIV), três com comunicação interatrial do tipo ostium secundum e quatro com persistência do canal arterial. Treze pacientes (59,1%) necessitaram reparo cirúrgico. A decisão de prosseguir com a cirurgia foi baseada principalmente na gravidade da lesão associada em oito pacientes (61,5%) e na gravidade da regurgitação mitral em quatro pacientes (30,8%). Em um paciente, a decisão por cirurgia foi baseada na gravidade tanto da lesão associada quanto da regurgitação mitral. Conclusão: Nosso estudo mostra que a FIVM é rara e está fortemente associada à síndrome de Down. As anormalidades cardíacas associadas mais comuns foram a CIV e cordoalha acessória na VSVE. Concluímos que as lesões cardíacas associadas à FIVM são de grande interesse, já que neste estudo, pacientes com lesões associadas foram diagnosticados mais precocemente. A decisão cirúrgica deve levar em conta a gravidade tanto da regurgitação mitral quanto das lesões cardíacas associadas.

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

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

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