Impacto de la miectomía septal ampliada sobre los cambios ecocardiográficos en la miocardiopatía hipertrófica obstructiva / Impact of Extended Septal Miectomy on Echocardiographic Changes in Hypertophic Obstructivec Cardiomyopathy

RESUMEN Introducción: La miectomía septal ampliada (MSA) ha demostrado ser una estrategia útil para mejorar los síntomas de los pacientes con miocardiopatía hipertrófica obstructiva (MCHO). Objetivos: El objetivo de este trabajo fue analizar el impacto de la MSA en parámetros estructurales y funcionales ecocardiográficos a corto y mediano plazo, en pacientes con MCH y obstrucción dinámica del tracto de salida del ventrículo izquierdo (VI). Material y métodos: Se analizaron los ecocardiogramas en los períodos preoperatorio, postoperatorio inmediato (1 mes post cirugía) y posoperatorio alejado (2 a 3 años) de pacientes sometidos a MSA. Resultados: Se intervinieron 94 pacientes con una edad media de 57,6 ± 13,8 años. Se observó una reducción significativa del máximo espesor septal en el postoperatorio inmediato, que se mantuvo en el posoperatorio alejado, y en el tamaño auricular izquierdo en el postoperatorio inmediato que se profundizó en el alejado (p < 0,001). El gradiente intraventricular en reposo pasó de 49,2 mmHg basal a 6,4 mmHg (p < 0,001) y de allí a 4,6 mmHg (p=0,224) en los períodos inmediato y alejado, y con la maniobra de Valsalva de 93,9 mmHg a 8,7 mmHg (p < 0,001) y de allí a 7,2 mmHg (p=0,226) respectivamente. La función diastólica fue valorada como grado II en el 58,5 % de los pacientes en el preoperatorio, 51,7 % en el postoperatorio inmediato y 29 % en el postoperatorio alejado. En la misma línea se evidenció un cambio en la relación E/e´ y la presión sistólica pulmonar, grado de insuficiencia mitral y dimensiones de la aurícula izquierda. Conclusión: En esta cohorte de pacientes con MCHO, la realización de una MSA se asoció a una mejoría significativa de la función diastólica del VI, reducción de las presiones de llenado y pulmonares y grado de insuficiencia mitral, y remodelado reverso de la aurícula izquierda. Es posible que esta combinación de efectos explique los beneficios clínicos de la intervención.

ABSTRACT Background: Extended septal myectomy (ESM) has proven to be a useful strategy to improve symptoms in patients with hypertrophic obstructive cardiomyopathy (HOCM). Objectives: The aim of this study was to analyze the impact of ESM on short and mid-term structural and functional echocardiographic parameters in patients with HCM and left ventricular (LV) outflow tract dynamic obstruction. Methods: Preoperative, immediate postoperative (1 month after surgery) and late postoperative (2 to 3 years) echocardiograms of patients undergoing ESM were analyzed. Results: A total of 94 patients with mean age of 57.6 ± 13.8 years underwent surgery. A significant reduction was observed in maximum septal thickness in the immediate postoperative period, which was sustained in the late postoperative period, and in atrial size in the immediate postoperative period, which deepened in the late postoperative period (p < 0.001). Intraventricular gradient at rest dropped from 49.2 to 6.4 mmHg (p < 0.001) and then to 4.6 mmHg (p=0.224) in the immediate and late periods and with Valsalva maneuver from 93.9 to 8.7 mmHg (p < 0.001), and then to 7.2 mmHg (p=0.226), respectively. Preoperative diastolic function was assessed as grade II in 58.5% of patients, decreasing to 51.7% in the immediate postoperative period and to 29% in the late postoperative period. In agreement with these results, a change was evidenced in the E/e´ ratio and pulmonary artery systolic pressure, degree of mitral regurgitation and left atrial dimensions. Conclusion: In this cohort of patients with HOCM, ESM was associated with a significant improvement in LV diastolic function, reduction in filling and pulmonary pressures and degree of mitral regurgitation, and left atrial reverse remodeling. It is possible that this combination of effects explains the clinical benefits of the intervention.

Application of Computed Tomography Angiography in Preoperative Diagnosis of Coarctation of Aorta and Evaluation of Aortic Dilatation in Infants

ABSTRACT Objective: To evaluate the occurrence of aortic dilatation and its associated predictors with coarctation of the aorta (CoA) in infants using multi-slice computed tomography (MSCT). Methods: The clinical data of 47 infantile patients with CoA diagnosed by MSCT and 28 infantile patients with simple ventricular septal defect were analyzed retrospectively. Aortic diameters were measured at six different levels, and aortic sizes were compared by z score. The coarctation site-diaphragm ratio was used to describe the degree of narrowing. Relevant clinical data were collated and analyzed. Results: The dilation rate and z score of the ascending aorta in the severe CoA group were significantly higher than those in the mild CoA group (11 [52.38%] vs. 21 [80.77%], P=0.038 and 2.00 ± 0.48 vs. 2.36 ± 0.43, P=0.010). Pearson's correlation analysis found that the z score of the ascending aorta was negatively correlated with the coarctation site-diaphragm ratio value (r=-0.410, P=0.004). A logistic retrospective analysis found that an increased degree of coarctation was an independent predictor of aortic dilatation (adjusted odds ratio 0.002; 95% confidence interval 0.00-0.819; P=0.043). The z score of the ascending aorta in the severe CoA group was significantly higher than that in the ventricular septal defect group (P<0.05). Conclusion: Most infants with CoA can also have significant dilatation of the ascending aorta, and the degree of this dilatation is related to the degree of coarctation. Assessment of aortic diameter and related malformations by MSCT can predict the risk of aortic dilatation in infants with CoA.

The Impact of Transcatheter or Surgical Defect Closure on Self-Reported Sleep Quality in Adults with Atrial Septal Defect

ABSTRACT Objective: Sleep quality in those with cardiovascular disease is significantly lower than in the general population. This study aimed to explore the effect of transcatheter or surgical closure of atrial septal defect (ASD) on sleep quality. Methods: One hundred nineteen adult patients with ASD who underwent transcatheter or surgical closure were included in the study. Sleep quality was investigated prospectively just before defect closure and six months after defect closure. Pittsburgh Sleep Quality Index (PSQI) was used to evaluate sleep quality of these patients. Results: PSQI scores were similar in both groups before the procedure in patients who underwent both transcatheter and surgical closure. The PSQI scores six months after transcatheter closure was significantly improved compared to the PSQI score before transcatheter ASD closure (3.5 ± 2.0 vs. 6.9 ± 3.4, respectively; P<0.001). The PSQI scores six months after surgical ASD closure was significantly improved compared to the PSQI score before surgical closure (4.8 ± 2.1 vs. 7.1 ± 2.0, respectively; P<0.001). Total PSQI scores were also statistically different at six months after transcatheter and surgical closure (3.5 ± 2.0 vs. 4.8 ± 2.1, P=0.014). However, six months after both transcatheter and surgical closure, PSQI scores were significantly decreased in both groups which was more pronounced in patients who underwent transcatheter closure. Conclusion: Transcatheter or surgical closure of the defect may be beneficial in improving the sleep quality of adult patients with ASD. Delayed improvement of sleep quality after surgical closure may be an important advantage for transcatheter closure.

Infant Barlow's Disease in Association with Atrial Septal Defect

ABSTRACT Clinical data: Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect. Technical description: Echocardiographic findings compatible with Barlow's disease and atrial septal defect, ostium secundum type. Operation: She was submitted to mitral valvuloplasty with chordal shortening and prosthetic posterior ring (Gregori-Braile®) along with patch atrioseptoplasty. Comments: Mitral valve regurgitation is a rare congenital heart disease and Barlow's disease is probably rarer. Mitral valve repair is the treatment of choice.

Migraña poscierre percutáneo de defectos septales auriculares

La migraña es una enfermedad que se ha visto asociada a defectos septales auriculares y a su cierre percutáneo, estipulándose en la literatura que sería una rara complicación, pero la evidencia al respecto es escasa. Se realizó una revisión narrativa sobre definiciones, epidemiología, fisiopatología y tratamiento de la migraña y de la entidad migraña poscierre percutáneo de defectos del septum auricular, incluyendo trabajos observacionales (retrospectivos, prospectivos), estudios randomizados, reportes de casos, artículos de revisión y metaanálisis existentes en PubMed y Cochrane, para aportar al conocimiento de esta entidad.

Migraine is a disease that has been associated with atrial septal defects and its percutaneous closure, stipulating in the literature that it would be a rare complication, but evidence is scarce. A narrative review was conducted on definitions, epidemiology, pathophysiology and treatment of migraine and the migraine entity after percutaneous closure of atrial septum defects, including observational studies (retrospective, prospective), randomized studies, case reports, review articles and meta-analyses existing in PubMed and Cochrane, to contribute to the knowledge of this entity.

A enxaqueca é uma doença que tem sido associada a defeitos do septo atrial e seu fechamento percutâneo, estipulando na literatura que seria uma complicação rara, mas as evidências são escassas. Foi realizada uma revisão narrativa sobre definições, epidemiologia, fisiopatologia e tratamento da enxaqueca e da entidade migranosa após fechamento percutâneo de defeitos do septo atrial, incluindo estudos observacionais (retrospectivos, prospectivos), estudos randomizados, relatos de caso, artigos de revisão e metanálises existentes no PubMed e Cochrane, para contribuir com o conhecimento dessa entidade.

Defecto septal ventricular postinfarto: complicación devastadora / Post-infarction ventricular septal defect: a devastating complicatio

Introducción: la comunicación interventricular es la complicación mecánica más frecuente después de un infarto agudo del miocardio en especial si cursa con elevación del ST, cuya frecuencia es alrededor de 0.21%, aumentando cuando es extenso y no reperfundido en pacientes con mayor edad, si hay compromiso multivaso y sin colateralidad; es una situación devastadora de mal pronóstico. Presentación de los casos: se describen 2 casos en un hospital universitario de Bogotá posteriores a infarto agudo del miocardio con elevación del ST y sin reperfusión temprana, ambos en choque cardiogénico, el primero con evolución tórpida y mortalidad temprana y la segunda fue llevada a los 10 días a cierre transcatéter, documentándose periprocedimiento un aumento significativo del tamaño del defecto septal, con mayor inestabilidad hemodinámica y muerte posterior al cierre.

Introduction: ventricular septal communication is the most frequent mechanical complication of acute myocardial infarction (MI), especially if associated with ST-segment elevation, featuring a rate of around 0.21%, which increases in older patients with extensive defects and no reperfusion therapy. In patients with multivessel involvement and no collateral circulation; it is a catastrophic situation which carries a poor prognosis. Case reports: two patients admitted to a university hospital in Bogotá, with ST-elevation post-MI VSD, undergoing no early reperfusion, both in cardiogenic shock. The first patient had a torpid evolution and early mortality. The second patient underwent a VSD transcatheter repair 10 days after MI, determining significant periprocedural increase in the size of the septal defect, with greater hemodynamic instability and death.

Retiro de dispositivo de oclusión septal transcateterismo en una paciente con comunicación interventricular / Removal of Transcatheter Septal Occlusion Device in a Patient with Ventricular Septal Defect

Introducción: Las complicaciones asociadas al procedimiento de colocación de un dispositivo de oclusión septal se presentan en menos del 10 por ciento de los casos. La embolización requiere de cirugía cardíaca, por lo que se incrementa el riesgo de la mortalidad. Objetivo: Presentar el caso en una paciente con comunicación interventricular e hipertensión pulmonar severa que se le retiró el dispositivo de oclusión septal transcateterismo. Presentación de caso: Se presentó una mujer de 24 años de edad con comunicación interventricular, insuficiencia cardíaca (New York Heart Association) clase IV e hipertensión pulmonar tipo 2, que se programó para la colocación de dispositivo de oclusión septal, sin embargo, presentó fallo en la colocación del dispositivo y defecto residual de 7 mm, por lo que se realizó el retiro de este 48 h después sin presentar complicaciones. Conclusiones: Aunque el cierre transcateterismo de la comunicación interventricular es una alternativa segura y efectiva a la cirugía, no está exento de complicaciones. En caso de translocación del dispositivo de oclusión septal este debe ser retirado durante el mismo procedimiento, ya que el riesgo de embolización es elevado y en caso de presentarse, el riesgo de muerte se incrementa(AU)

Introduction: Complications associated with the placement procedure of a septal occlusion device occur in less than 10percent of cases. Embolization requires cardiac surgery, which increases the risk of mortality. Objective: To report the case of a patient with ventricular septal defect and severe pulmonary hypertension who had the transcatheter septal occlusion device removed. Case report: We report the case report of a 24-year-old woman with ventricular septal defect, heart failure (New York Heart Association) class IV and type 2 pulmonary hypertension. She was scheduled for placement of a septal occlusion device, however, the placement of the device failed and had a residual defect of 7 mm, so the placement was removed 48 hours later without complications. Conclusions: Although transcatheter closure of the ventricular septal defect is a safe and effective alternative to surgery, it is not free of complications. In case of translocation of the septal occlusion device, it must be removed during the same procedure, since the risk of embolization is high and if it occurs, the risk of death increases(AU)

Epidemiología del diagnóstico prenatal de defectos congénitos en la provincia de Holguín / Epidemiology of prenatal diagnosis of congenital defects in Holguín province

Introducción: En Cuba, los defectos congénitos constituyen la segunda causa de muerte en niños menores de un año, por lo cual ocupan un lugar prioritario en los programas médicos sociales del país. Objetivo: Evaluar el comportamiento epidemiológico del diagnóstico prenatal de los defectos congénitos en Holguín, Cuba. Métodos: Se realizó un estudio descriptivo y transversal de la epidemiología de los defectos congénitos en la provincia de Holguín, Cuba, en el periodo de enero 2011- junio de 2020. Resultados: Los años con mayor número de defectos congénitos diagnosticados fueron: 2011, 2012, 2017 y 2018 con 308, 253, 290 y 236 pacientes, respectivamente. Los defectos congénitos más frecuentes fueron: cardiovasculares (comunicación interventricular, canal auriculoventricular, transposición de grandes vasos e hipoplasia de cavidades), renales (pielocaliectasia, hidronefrosis, riñones poliquísticos), y del sistema nervioso central (ventriculomegalia, hidrocefalia). El grupo de edad materna donde se realizó mayor número de diagnósticos fue entre 20-24 años, la mayoría en el segundo trimestre de la gestación; en el primer trimestre, el mayor número de defectos congénitos correspondió a los defectos de pared anterior. La tasa de mortalidad infantil por defectos congénitos se mantuvo estable en la mayoría de los años estudiados. Conclusiones: La estabilidad y perfeccionamiento del programa de diagnóstico prenatal de los defectos congénitos, y el asesoramiento genético adecuado, han tenido un resultado epidemiológico favorable en la provincia.

Introduction: congenital defects in Cuba are the second cause of death in children under one year of age that is why they occupy a priority place in the social medical programs of the country. Objective: to evaluate the epidemiological manifestation of the prenatal diagnosis of congenital defects in Holguín, Cuba. Methods: a descriptive and cross-sectional study of the epidemiology of birth defects was carried out in Holguín province, Cuba from January 2011 to June 2020. Results: the years with the highest number of diagnosed birth defects were 2011, 2012, 2017 and 2018 with 308, 253, 290 and 236 patients, respectively. The most frequent birth defects were cardiovascular (ventricular septal defect, atrioventricular canal, transposition of the great vessels and hypoplasia of cavities), renal (pyelokaliectasia, hydronephrosis and polycystic kidneys), and central nervous system (ventriculomegaly and hydrocephalus). The maternal age group in which the highest number of diagnoses was made was between 20-24 years, mostly in the second trimester of pregnancy; the largest number of congenital defects in the first trimester corresponded to anterior wall defects. The infant mortality rate due to congenital defects remained stable in most of the years studied. Conclusions: the stability and improvement of the prenatal diagnosis program for congenital defects, as well as an adequate genetic counseling, have had a favourable epidemiological result in the province.

Sufficient versus deficient rims during percutaneous closure of ostium secundum type atrial septal defect: A systematic review and meta-analysis

Background: The aim of this meta-analysis was to compare the efficacy and adverse events of percutaneous occlusion among patients with sufficient and deficient rims. Methods: A systematic review of all articles published in the Pubmed, MEDLINE and Google Scholar databases was performed. Odds ratio (OR) and 95% CI were used as a measure of effect of the combination of studies. I2 with 95% CI was estimated to assess study heterogeneity. For the meta-analysis, a random effects model was used. Results: The systematic search identified ten studies which included 4355 patients; 2661 of those had sufficient rim and the remaining 1694 patients showed some rim deficiency. Implant failure rate was 4.13% CI 95% 3.53e4.72%. Compared to frequency of failures in the group with a deficient rim (5.43% CI 95% 4.35e6.50%), implant failure in patients with a sufficient rim was significantly lower (3.30% CI 95% 2.62e3.97%), OR 2.27 CI 1.34e3.83 (p 0.002). The combined adverse events were 5.19% CI 95% 4.22e6.35% vs 2.7% CI 95% 2.08e3.31% in the deficient vs sufficient rim groups respectively (OR 2.21 CI 0.93e5.29; p 0.07). Implant failures and adverse events were more frequent in patients with posterior inferior rim deficiency. Conclusion: Patients presenting a posteroinferior rim deficiency are associated to both, an increased incidence of closure failure and a combined adverse events occurrence. More studies on posterior rim deficiency are necessary to ensure the feasibility and safety of the percutaneous approach.

A PROSPECTIVE STUDY OF ASSOCIATION OF ANTERIOR ETHMOIDAL NERVE SYNDROME (SLUDER'S NEURALGIA) WITH NASAL SEPTAL SPUR AND ITS MANAGEMENT

Background: Anterior ethmoidal nerve syndrome is headache resulting from irritation of the terminal branches of the anterior ethmoidal nerve. The headache arises when the septal spur compresses against the middle turbinate or the lateral nasal wall. Here we studied the association of anterior ethmoidal nerve syndrome (Sluder's neuralgia) with nasal septal spur and its management. A prospective observational study carried out on 31 patients Methods: who ful?lled the clinical diagnostic criteria. The patients were treated surgically. The outcomes of surgical treatment Results: were quite promising with 29 patients reporting in improvement in pain after surgery out of the 31 surgeries performed. Conclusion: The diagnosis requires a strong clinical suspicion and appropriate evaluation including nasal endoscopy, scan and anaesthesia of the suspected point of contact

A-prospective-study-comparing-the-outcomes-of-nasal-septal-clip-versus-merocel-application-after-nasal-septal-corrective-surgery

Nasal splint application is an important step of septal corrective surgeries to keep the septum in midline after the surgery. Septal clip is most commonly used. In our study we have compared the use of nasal septal clip and Merocel application post surgery and the morbidities associated with it.

Venoarterial Extracorporeal Membrane Oxygenation as A Bridge to Surgery in Post-Myocardial Infarction Ventricular Septal Defect with Cardiogenic Shock: Case Report

ABSTRACT We describe a 60-year-old woman with post-myocardial infarction (MI) ventricular septal defect (VSD) and cardiogenic shock who was successfully stabilized with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge therapy for the surgical closure of her VSD. This case highlights the role of VA-ECMO in the management of post-MI VSD to improve the results of surgical repair and patient survival.

Clinical study of endoscopic surgery for recurrent nasopharyngeal carcinoma / 临床耳鼻咽喉头颈外科杂志

Objective:To compare the clinical effects and complications of surgery + chemotherapy and radiotherapy + chemotherapy in patients with nasopharyngeal carcinoma recurrence, so as to compare the safety and efficacy of two different therapeutic methods. Methods:A retrospective analysis was performed on 40 patients with recurrent nasopharyngeal carcinoma after radiotherapy and chemotherapy admitted to our hospital from January 2016 to June 2020. Among them, 26 patients were treated with surgery. The recurrent tumor was removed under nasal endoscope, and the frozen resection margin was negative during the operation. Chemotherapy was continued for stage Ⅲ and Ⅳ patients from 3 to 5 weeks after surgery. Fourteen patients received secondary radiotherapy and chemotherapy. Postoperative complications and survival rate were observed. Results:There were 14 patients in the secondary chemoradiotherapy group（control group） and 26 patients in the nasal endoscopic surgery group（observation group）. Among the 26 patients, 19 patients underwent nasal septal mucosal repair, 5 patients underwent temporal muscle flap repair, 2 patients underwent submental flap repair, 2 patients had nasal septal mucosal flap necrosis and cerebrospinal fluid leakage, and the temporal muscle flap was used for secondary repair in the second stage operation, and 8 patients needed cervical lymph node dissection. The patients recovered well after surgery, and the patients in stage Ⅲ and Ⅳ were treated with chemotherapy after 3 weeks to 5 weeks according to the patient's wound condition. There were significant differences in the incidence of complications and 1-, 2-, and 3-year survival rates between the two groups（P<0.05）. Conclusion:Patients with recurrent nasopharyngeal carcinoma can be treated by nasal endoscopic surgery to remove the tumor, and the use of pedicled nasal septal mucosal flap or temporal muscle flap for skull base reconstruction, The operation can effectively prevent major complications such as internal carotid artery rupture and hemorrhage, and improve the survival rate and quality of life of patients. It provides a safe and effective treatment for patients with recurrent nasopharyngeal carcinoma.

Effect of preoperative anemia on perioperative red blood cell transfusion and prognosis in children with septal defect / 中国输血杂志

【Objective】 To analyze the impact of preoperative anemia on perioperative red blood cell transfusion and prognosis of children undergoing septal defect repair. 【Methods】 The medical records of 208 patients under 18 years old with septal defect, i. e. ventricular septal, atrial septal, ventricular septal with atrial septal defect, in a hospital from December 2018 to March 2022 were collected. They were divided into anemic group (n=52) and non-anemic group (n=156) according to whether they were anemic before operation. The basic information, as well as preoperative, intraoperative and postoperative blood transfusion, postoperative ICU stay, postoperative infection rate and average length of stay were compared between the two groups. 【Results】 The incidence of preoperative anemia in the children with septal defect was 25.0% (52/208). The age, preoperative body weight(kg) and hemoglobin (g/L) of anemic group and non-anemic group was 0.67(0.33, 2) vs 2(1, 3), 6.5(5, 10) vs10.5(8, 14) and 102(91.5, 107) vs 127(121, 134) respectively, all P<0.05. Preoperative, intraoperative and postoperative blood transfusion rates in the anemic and non-anemic groups were 11.54% (6/52) vs 0% (0/156), 92.31% (48/52) vs 72.44% (113/156), 51.92% (27/52) vs 25.0% (39/156), all P<0.05. Postoperative ICU stay (d) and mean length of stay(d) of anemia group and non-anemia group was 3 (2, 6) vs 2 (2, 3) and 19(13, 25) vs14(11, 18) respectively, P<0.05. 【Conclusion】 Preoperative anemia is an important factor affecting perioperative red blood cell transfusion in children with septal defect repair, and also an important reason for prolonging postoperative ICU stay and hospital stay.

Clinical results of surgical repair combining an occluder and a patch for ventricular septal rupture after myocardial infarction / 中国胸心血管外科临床杂志

@#Objective     To investigate the effect and prognosis of patients with ventricular septal rupture after myocardial infarction treated by surgical repair combining an occluder and a patch. Methods     Clinical data of 42 patients with myocardial infarction complicated with ventricular septal rupture admitted to the First Affiliated Hospital of Zhengzhou University from January 2010 to September 2021 were retrospectively analyzed. According to the surgical methods, 27 patients were divided into a traditional group, including 17 males and 10 females, with an average age of 62.81±6.81 years, who were repaired by patch only, and 15 patients were divided into a modified group, including 11 males and 4 females, with an average age of 64.27±9.24 years, who were repaired by surgery combining an occluder and a patch. Perioperative and follow-up data of the two groups were compared and analyzed. Results     There were statistical differences between the two groups in preoperative Killip grading, rate of intra-aortic balloon pump use, interval from myocardial infarction to operation, and the number of culprit artery (P<0.05). There was no statistical difference in other preoperative data, the cardiopulmonary bypass time, aortic cross-clamping time, postoperative hospital stay or in-hospital death rate between the two groups (P>0.05). No residual shunt occurred in the modified group, and the difference was statistically significant compared with the traditional group (P=0.038). There was no statistical difference in other complications between the two groups (P>0.05). The median follow-up time was 4 years. Two patients in the traditional group and one in the modified group died during follow-up. The follow-up cardiac function grading of patients in the modified group was statistically different from that in the traditional group (P=0.023). Conclusion     The perioperative  mortality of ventricular septal rupture after myocardial infarction is high, but the long-term effect is satisfactory. Surgical repair combining an occluder and a patch is a safe and effective treatment for ventricular septal rupture, which can effectively reduce postoperative residual shunt.

A comparative study of three palliative surgical approaches for pulmonary atresia with ventricular septal defect / 中国胸心血管外科临床杂志

@#Objective    To compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD). Methods    A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed. Results    There were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001). Conclusion    All these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.

Application Value of Plasma vWF and ADAMTS-13 in Evaluating Postoprative Vascular Endothelial Injury and Prognosis in Children with VSD / 中国实验血液学杂志

OBJECTIVE@#To dynamically observe the levels and activities of von Willebrand factor (vWF) and ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) in plasma of children with congenital ventricular septal defect (VSD) during perioperative period, and explore the value of plasma vWF antigen (vWF:Ag) and ADAMTS-13 activity (ADAMTS-13: AC) in evaluating vascular endothelial injury and prognosis in children with VSD.@*METHODS@#In this cross-sectional study, a total of 74 children with VSD who underwent surgical treatment in TEDA International Cardiovascular Hospital from September 2018 to March 2019 were enrolled in the observation group. Among them, there were 28 cases of pure VSD, 32 cases of VSD combined with pulmonary hypertension, and 14 cases of VSD combined with valvular heart disease. 31 healthy children who underwent physical examination in Tianjin Children's Hospital during the same period were collected as the control group. The biochemical indexes of the children at admission were recorded. Peripheral plasma was collected at admission, postsurgery day 0 and day 1, respectively, and the levels of vWF activity (vWF:AC), vWF:Ag, ADAMTS-13 antigen (ADAMTS-13:Ag) and ADAMTS-13:AC were detected.@*RESULTS@#The level of plasma vWF:Ag and vWF:AC in the observation group before surgery were significantly lower than those in the control group (P<0.001), and increased continuously, on postsurgery day 0 and day 1 (P<0.001). The level of ADAMTS-13:Ag in the observation group before surgery was significantly higher than that in the control group (P<0.001), which decreased significantly on postsurgery day 0 (P<0.001), and increased significantly on postsurgery day 1 compared with postsurgery day 0 (P=0.033). The level of ADAMTS-13:AC in the observation group before surgery was significantly lower than that in the control group (P=0.015), which decreased significantly on postsurgery day 0 (P=0.037), and increased on postsurgery day 1, but the difference was not statistically significant (P=0.051). The changes of vWF and ADAMTS-13 in the three subgroups were basically similar to the observation group. vWF: Ag/ADAMTS-13: AC ratio on postsurgery day 0 and day 1 had high diagnostic value in vascular endothelial injury (AUC=0.80, P<0.001; AUC=0.93, P<0.001). Preoperative vWF and ADAMTS-13 levels, and related baseline indicators were not correlated with postoperative infection, bleeding, thrombosis,etc.@*CONCLUSION@#Preoperative vWF: Ag, vWF: AC and ADAMTS-13: AC levels in children with VSD are low, while the level of ADAMTS-13: Ag is high. After surgery, the levels of vWF: Ag and vWF: AC are increased and the level of ADAMTS-13: Ag is decreased. The postoperative vWF: Ag/ADAMTS-13: AC ratio shows high diagnostic value in evaluating vascular endothelial injury. There is no correlation between preoperative vWF and ADAMTS-13 levels with perioperative clinical events.

Association of ventricular septal defect with rare variations of the HAND2 gene / 中国当代儿科杂志

OBJECTIVES@#To study the association of ventricular septal defect (VSD) with rare variations in the promoter region of HAND2 gene, as well as related molecular mechanisms.@*METHODS@#Blood samples were collected from 349 children with VSD and 345 healthy controls. The target fragments were amplified by polymerase chain reaction and sequenced to identify the rare variation sites in the promoter region of the HAND2 gene. Dual-luciferase reporter assay was used to perform a functional analysis of the variation sites. Electrophoretic mobility shift assay (EMSA) was used to investigate related molecular mechanisms. TRANSFAC and JASPAR databases were used to predict transcription factors.@*RESULTS@#Sequencing revealed that three variation sites (g.173530852A>G, g.173531173A>G, and g.173531213C>G) were only observed in the promoter region of the HAND2 gene in 10 children with VSD, among whom 4 children had only one variation site. The dual-luciferase reporter assay revealed that g.173531213C>G reduced the transcriptional activity of the HAND2 gene promoter. EMSA and transcription factor prediction revealed that g.173531213C>G created a binding site for transcription factor.@*CONCLUSIONS@#The rare variation, g.173531213C>G, in the promoter region of the HAND2 gene participates in the development and progression of VSD possibly by affecting the binding of transcription factors.

Adult Congenital Heart Disease: Report from a Public Reference Hospital in Northeastern Brazil

ABSTRACT Introduction: The increasing worldwide number of adults with congenital heart disease (CHD) demands greater attention from health professionals. The purpose of this report is to describe the clinical demographic profile, frequency, and invasive treatment status of adults with CHD in a public reference hospital in northeastern Brazil. Methods: This is a retrospective cross-sectional study including 704 patients attended between August 2016 and August 2020. Data were collected from virtual database. Results: Patients' age varied from 17 to 81 years (mean 32±14; median 27 years); 294 (41.8%) patients were male, and 410 (58,2%) were female; 230 (32,7%) had diagnosis from age 18 and up. Cardiac complexity categories were "simple defects" (134 [19%] patients), "moderate complexity" (503 [71.5%]), and "great complexity" (67 [9.5%]). Atrial septal defect (ASD) was diagnosed in 216 (30.7%) patients, ventricular septal defect (VSD) in 101 (14.3%), tetralogy of Fallot in 93 (13.2%), and other CHD in 294 (41.8%). New York Heart Association (NYHA) functional classes were I (401 [57%]), II (203 [28.8%]), III (76 [10.8%]), and IV (24 [3.4%]). Complications were arrhythmias (173 [24%]) and severe pulmonary hypertension (69 [9.8%]). Invasive treatments were corrective surgery (364 (51.6%]), reoperation (28 [4.0%]), palliation (11 [1.6%]), interventional catheterization (12 [1.7%]), surgery plus interventional catheterization (5 [0.7%]), and preoperation (91 [12.9%]). Treatment was not required in 102 (14,5%) patients, and 91 (12.9%) were inoperable. Conclusion: The leading diagnosis was ASD. Frequency of unrepaired patients was high, mainly ASD, due to late diagnosis, which favored complications and denotes a matter of great concern.

Long-term Recovery Patterns of Olfactory Function after Trans-sphenoidal Approach with Nasoseptal Flap Elevation

Abstract Introduction Nasoseptal flap is widely used in reconstruction of the skull base to prevent cerebrospinal fluid leakage after surgery for skull base lesions. There has been a debate on whether more severe olfactory dysfunction occurs after nasoseptal flap elevation than the conventional trans-sphenoidal approach. Objective To compare the long-term recovery patterns associated with nasoseptal flap and the conventional trans-sphenoidal approach. Methods The subjects were divided into the conventional trans-sphenoidal approach group and the nasoseptal flap elevation group. We followed up self-reported olfactory score using the visual analogue scale and threshold discrimination identification (TDI) score of the Korean Version of the Sniffin Stick test II for 12 months, with olfactory training. Results The study included 31 patients who underwent the trans-sphenoidal approach. Compared with preoperative status, the mean visual analogue scale and TDI scores in the conventional trans-sphenoidal approach group recovered 2 months postoperatively, while in the nasoseptal flap elevation group the visual analogue scale and TDI scores recovered 6 months and 3 months after surgery, respectively. Twelve months after surgery, the visual analogue scale and TDI scores in the conventional trans-sphenoidal approach group were 9.3 ± 0.5 and 28.5 ± 4.3, while those from the nasoseptal flap elevation group were 8.9 ± 1.5 and 27.2 ± 4.7 (p = 0.326; 0.473). Only one of the patients in the nasoseptal flap elevation group had permanent olfactory dysfunction. Conclusion The olfactory function recovered more gradually in the nasoseptal flap elevation group than in the conventional trans-sphenoidal approach group, but there was no difference between the two groups after 6 months.