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.

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.

Secondary subaortic stenosis following ventricular septal defect closure: A retrospective study in a single center / 中国胸心血管外科临床杂志

@#Objective     To summarize the characteristics of children diagnosed with secondary subaortic stenosis after the surgical closure for ventricular septal defect and explore its potential mechanism. Methods     We retrospectively collected patients aged from 0 to 18 years, who underwent ventricular septal defect closure and developed secondary subaortic stenosis, and subsequently received surgical repair from 2008 to 2019 in Fuwai Hospital. Their surgical details, morphological features of the subaortic stenosis, and the follow-up information were analyzed. Results     Six patients, including 2 females and 4 males, underwent the primary ventricular septal defect closure at the median age of 9 months (ranging from 1 month to 3 years). After the first surgery, patients were diagnosed with secondary subaortic stenosis after 2.9 years (ranging from 1 to 137 months). Among them, 2 patients underwent the second surgery immediately after diagnosis, and the other 4 patients waited 1.2 years (ranging from 6 to 45 months) for the second surgery. The most common type of the secondary subaortic stenosis after ventricular septal defect closure was discrete membrane, which located underneath the aortic valve and circles as a ring. In some patients, subaortic membrane grew along with the ventricular septal defect closure patch. During the median follow-up of 8.1 years (ranging from 7.3 to 8.9 years) after the sencond surgery, all patients recovered well without any recurrence of left ventricular outflow tract obstruction. Conclusion     Regular and persistent follow-up after ventricular septal defect closure combining with or without other cardiac malformation is the best way to diagnose left ventricular outflow tract obstruction in an early stage and stop the progression of aortic valve regurgitation.

Mid-term outcomes of tricuspid valve detachment versus non-detachment for ventricular septal defect closure / 中国胸心血管外科临床杂志

@#Objective 聽 聽To evaluate the mid-term outcomes between tricuspid valve detachment (TVD) and non-detachment (NTVD) for ventricular septal defect (VSD). Methods 聽 聽 The patients who underwent perimembranous VSD repair in the Department of Cardiovascular Surgery, West China Hospital from 2015 to 2020 were included. According to the surgical method, the patients were divided into a TVD group and a NTVD group. The clinical data of the two groups were compared. Results 聽 聽 Totally 538 patients were included in the study. There were 240 patients in the TVD group, including 121 males and 119 females, with an average age of 3.85卤8.42 years and an average weight of 14.12卤12.97 kg. There were 298 patients in the NTVD group, including 149 males and 149 females, with an average age of 4.42卤9.36 years and an average weight of 14.87卤12.51 kg. There was no statistical difference in the age, weight, sex, preoperative New York Heart Association (NYHA) classification or tricuspid regurgitation (TR) degree between the two groups (P>0.05). Median follow-up was 30 (23, 40) months in the TVD group, and 29 (23, 41) months in the NTVD group (P=0.600). After operation, one patient in each group developed third-degree atrioventricular block and recovered to sinus rhythm before discharge (P=0.848). No pacemaker was needed. There was no statistical difference in the length of stay (P=0.054), mortality (P=1.000), in-hospital reoperation (P=0.199), or follow-up reoperation (P=0.505). More than 98% of patients in both groups had postoperative TR less than moderate (P=0.926). At the last follow-up, only 7 (2.9%) patients in the TVD group were detected trivial residual shunting, and 14 (4.7%) in the NTVD group (P=0.289). No one needed to have reoperation because of residual VSD. The TVD group showed less TR during the follow-up (P=0.019). Conclusion     TVD is an alternative technique which can be safely used in the closure of VSD, especially in technologically mature medical center. Appropriate tricuspid valve detachment for those hard-to-expose VSDs does not result in poorer tricuspid valve function or higher risk of atrioventricular block, and might reduce the incidence of residual shunting.

Effect of preoperative duration of mechanical ventilation on prognosis in patients with ventricular septal defect: A retrospective cohort study / 中国胸心血管外科临床杂志

@#Objective    To summarize the relationship between preoperative duration of mechanical ventilation and prognosis in patients with ventricular septal defect. Methods    The clinical data of patients with ventricular septal defect requiring ventilator support preoperatively and undergoing surgical treatment in our hospital from May 2009 to May 2020 were retrospectively reviewed. Based on the duration of preoperative ventilation, the patients were divided into three groups: a group A (0-47 hours), a group B (48-96 hours), and a group C (>96 hours). Each group's postoperative recovery, complications, and medical costs were analyzed. Results     Finally 272 patients were enrolled, including 154 males and 118 females, with a median surgical age of 2 (1, 4) months. There were 43 patients in the group A, 75 patients in the group B, and 154 patients in the group C. Early death occured in 3 (1.3%) patients, all in the group C. No statistical difference in mortality was found among the three groups (P=0.734). The mean postoperative duration of mechanical ventilation in the three groups was 158.6±133.5 hours, 101.2±56.1 hours, and 133.1±97.9 hours, respectively. The group B had significantly shorter duration than the other two groups (P<0.05). The mean postoperative hospital stay in the three groups was 17.5±9.9 days, 13.5±5.8 days, and 16.5±10.8 days, respectively. Postoperative hospital stay in the group B was significantly shorter than that in the other two groups (P<0.05). The mean total in-hospital cost in the three groups were 89 000±34 000 yuan, 87 000±21 000 yuan, and 109 000±41 000 yuan, respectively. The costs in the group C were significantly higher than those in the other two groups (P≤0.001). Conclusion    Prompt surgical repair is necessary for patients with ventricular septal defects requiring ventilator support preoperatively. However, attention should be paid to surgical timing. Preoperative duration of mechanical ventilation is associated with better surgical outcomes within 48-96 hours than 0-47 hours or >96 hours.

Gut microbiota profiles in the perioperative period and long-term postoperative period in children with ventricular septal defect treated with cardiopulmonary bypass / 中华实用儿科临床杂志

Objective:To explore the characteristics of gut microbiota in the preoperative, short-term postoperative and long-term postoperative period at (15.61±4.51) months in children with ventricular septal defect (VSD) of congenital heart disease (CHD) treated with cardiopulmonary bypass (CPB).Methods:A prospective study was conducted.In Guangzhou Women and Children′s Medical Center, 13 patients with VSD who were scheduled for CPB and additional 10 age- and gender-matched healthy infants as pre-CPB control group from January 2021 to January 2022 were enrolled.Fecal samples were collected at pre- and early post-CPB.Meanwhile, 18 gender- and CHD diagnosis and operation-matched patients at (15.61±4.51) months after CPB and 8 healthy age- and gender-matched children as long-term control group after CPB were also enrolled, and fecal samples were collected.16S rRNA sequencing of fecal samples from all subjects were performed and comparing the differences in gut microbiota between two groups via comparing alpha and beta diversity, parameter test or nonparametric test, and LEfSe analysis.Results:Compared with those of pre-CPB control group, there was a significant difference in the composition of gut microbiota in the preoperative period of VSD children, with significantly increased abundances of Enterobacteriaceae and Shigella, and decreased abundance of Bifidobacterium (all P<0.05). The diversity of gut microbiota was comparable in VSD children before CPB and in the short period time after CPB (all P>0.05), except for the abundances of Clostridium and Streptococcus (all P<0.05), and there was no significant difference in the relative abundances of other highly abundant gut bacteria between the two periods (all P>0.05). Compared with that in VSD children in the short period time after CPB, the abundances of short-chain fatty acids-producing microbes were significantly higher at (15.61±4.51) months postoperatively (all P<0.05), and the gut bacteria profile was similar to that of the long-term control group after CPB (all P>0.05). Conclusions:Gut microbiota imbalance exists in VSD children before CPB.The gut microbiota profile is not influenced by CPB, which returns normal at (15.61±4.51) months postoperatively.

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.

Frequency of chromosome 22q11.2 deletion among newborns with non-syndromic congenital heart defects from western Mexico / Frecuencia de la deleción del cromosoma 22q11.2 en recién nacidos con cardiopatías congénitas no sindrómicas del occidente de México

Abstract Background: Congenital heart defects (CHD) are among the most frequent manifestations of 22q11.2 deletion syndrome. Although we found relatively few studies aimed at specifically detecting 22q11.2 deletion in newborns (NB) with CHD, none of them has been performed in Mexico. Methods: We conducted a prospective hospital-based study from January 2017 to March 2021 in the Genetics and Pediatric Cardiology Services of the Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, Mexico). All consecutive NBs identified with any non-syndromic major CHD confirmed by echocardiography were eligible to participate. A total of 98 NBs were included, 51 males and 47 females. Fluorescence in situ hybridization (FISH) analysis was conducted to search for deletion of chromosome 22q11.2 in interphase nuclei of standard lymphocyte cultures. Results: We found eight patients (8.2%) with CHD and the 22q11.2 deletion, all of them with conotruncal defects, particularly of the truncus arteriosus (p = 0.013), tetralogy of Fallot (p = 0.024), and pulmonary atresia with ventricular septal defect (p = 0.031) subtypes. With de exception of one infant with hypocalcemia and another with hypocalcemia and thymic aplasia, the diagnosis of 22q11.2 deletion was not clinically suspected in the other patients. Conclusions: Our results confirm the importance of excluding the presence of the 22q11.2 deletion in every NB with CHDs, particularly of the conotruncal subtype, even in the absence of other manifestations.

Resumen Introducción: Las cardiopatías congénitas (CC) son una de las manifestaciones más frecuentes del síndrome de deleción 22q11.2. A pesar de que existen relativamente pocos estudios dirigidos a detectar específicamente la deleción 22q11.2 en recién nacidos (RN) con CC, ninguno de ellos ha sido realizado en México. Métodos: Se realizó un estudio prospectivo de base hospitalaria desde enero de 2017 hasta marzo de 2021 en los Servicios de Genética y Cardiología Pediátrica del Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, México). Todos los RN consecutivos identificados con cualquier tipo de CC mayor no sindrómica confirmada por ecocardiografía fueron elegibles para participar. Se incluyeron 98 recién nacidos, 51 de sexo masculino y 47 de sexo femenino. Mediante el análisis de hibridación fluorescente in situ (FISH, por sus siglas en inglés) se realizó la búsqueda de la deleción del cromosoma 22q11.2 en núcleos en interfase de cultivos de linfocitos estándar. Resultados: Se encontraron ocho pacientes (8.2%) con CC y la deleción 22q11.2, todos ellos con defectos conotruncales, particularmente de los subtipos tronco arterioso (p = 0.013), tetralogía de Fallot (p = 0.024) y atresia pulmonar con comunicación interventricular (p = 0.031). Con excepción de un lactante con hipocalcemia y otro con hipocalcemia y aplasia tímica, el diagnóstico de deleción 22q11.2 no se sospechó clínicamente en los demás pacientes. Conclusiones: Los resultados de este trabajo confirman la importancia de excluir la presencia de la deleción 22q11.2 en todos los RN con CC, particularmente del subtipo conotruncal, incluso en ausencia de otras manifestaciones.

Disfunción ventricular derecha en paciente adulto con comunicación interventricular posinfarto / Right ventricular dysfunction in adult patient with post-infarction ventricular septal defect

RESUMEN Introducción: La disfunción ventricular derecha es la alteración aguda o crónica de la morfología y función de dicha estructura, de origen multifactorial. En los últimos años existe consenso acerca de su función como determinante del estado funcional y factor pronóstico en diversas enfermedades. Objetivo: Destacar la importancia del manejo multidisciplinario y algunas de las opciones terapéuticas que se les pueden brindar a estos pacientes. Presentación del caso: Paciente de 58 años de edad, con antecedentes de salud aparente, que acudió al Servicio de emergencias, donde se le diagnosticó un infarto agudo de miocardio de topografía inferior. Se trasladó a la unidad de cuidados intensivos quirúrgicos del Servicio de Cirugía Cardiovascular debido a una complicación mecánica por comunicación interventricular. Se recibió con elementos clínicos de insuficiencia cardíaca aguda clase funcional IV. Progresivamente presentó deterioro del estado hemodinámico por lo que requirió el uso de apoyo vasoactivo con norepinefrina; hipoxia, hipercapnia, presiones venosas centrales entre 15 y 18 cmH2O y elementos clínicos de disfunción de ventrículo derecho; se decidió instrumentar vía aérea, administrar sedación y relajación por asincronías a paciente ventilado. Sin otra opción que el tratamiento quirúrgico se llevó a discusión donde se decidió esperar a la mejoría del estado hemodinámico con tratamiento médico. Conclusiones: Las enfermedades cardiovasculares no dejan de aparecer en escenarios cada vez más complejos con elevada morbimortalidad. Se evidencia la importancia del consenso clínico-quirúrgico en el momento de la toma de decisiones.

ABSTRACT Introduction: Right ventricular dysfunction is the acute or chronic alteration of the morphology and function of this structure, of multifactorial origin. In recent years there is consensus about its role as a determinant of functional status and prognostic factor in various diseases. Objective: Highlight the importance of multidisciplinary management and some of the therapeutic options that can be provided to these patients. Case Presentation: A 58-year-old patient with an apparent health history went to the Emergency Service, where he was diagnosed with an acute myocardial infarction of lower topography. He was transferred to the surgical intensive care unit of the Cardiovascular Surgery Service due to a mechanical complication from ventricular septal defect. He was received presenting clinical elements of acute heart failure functional class IV. He progressively presented deterioration of the hemodynamic state, so it required the use of vasoactive support with norepinephrine; hypoxia, hypercapnia, central venous pressures between 15 and 18 cmH2O and clinical elements of right ventricular dysfunction; it was decided to implement airway, administer sedation and relaxation by asynchrony to ventilated patient. With no other option than surgical treatment, it was brought to discussion and it was decided to wait for the improvement of the hemodynamic state with medical treatment. Conclusions: Cardiovascular diseases do not cease to appear in increasingly complex scenarios with high morbidity and mortality. The importance of clinical-surgical consensus at the time of decision-making is evident.

Study on the correlation between preoperative echocardiography indicators and postoperative prognosis in children with ventricular septal defect / 中华超声影像学杂志

Objective:To explore the correlation between preoperative echocardiography indicators and surgical prognosis of children with ventricular septal defect (VSD) and conduct verification based on significant indicators and indicator ratios.Methods:A total of 1 357 children with VSD who were admitted to the Children′s Hospital, Zhejiang University School of Medicine from June 2016 to June 2021 were selected. Various measurements including the size of the VSD, left ventricular ejection fraction (LVEF), left atrial (LA) diameter, the aortic (AO) flow rate, the tricuspid regurgitation velocity and pressure gradient were extracted from preoperative echocardiography reports. This paper explored the correlation between echocardiography reports indicators, indicator ratios and postoperative auxiliary ventilation time, respectively. The patients were divided into two groups according to whether there were complications, and the differences of echocardiography reports indicators between the two groups were compared. A linear regression model was established to predict the postoperative auxiliary ventilation time using these indicators, and the least absolute shrinkage and selection operator (LASSO) regression model was used for variable selection.Results:The VSD size and AO flow velocity were weakly correlated with the postoperative auxiliary ventilation time ( r=0.32, 0.25; all P<0.01). There was no significant correlation between VSD flow velocity and postoperative auxiliary ventilation time. The AO flow velocity/VSD flow velocity and LVEF/VSD flow velocity were strongly correlated with the postoperative auxiliary ventilation time ( r=0.67, 0.51; all P<0.01). In the significance test, there were no significant differences in tricuspid regurgitation flow velocity, tricuspid regurgitation pressure gradient, LA diameter, and LVEF between the complication group and the non-complication group(all P>0.01). However, the ratio of LVEF/tricuspid regurgitation velocity in the complication group was significantly lower than that in the non-complication group, and the ratio of tricuspid regurgitation pressure gradient/LA diameter was significantly higher than that in the non-complication group (all P<0.01). The postoperative auxiliary ventilation time of VSD patients was predicted on an independent test set, with an R2 of 0.51. Conclusions:Echocardiography report indicator ratios of AO flow velocity/VSD flow velocity and LVEF/VSD flow velocity have strong correlations with postoperative auxiliary ventilation time in children with VSD, and the ratios of LVEF/tricuspid regurgitation velocity and tricuspid regurgitation pressure gradient/LA diameter are significantly different between groups with and without postoperative complications. The ratios of indicators can significantly improve this correlation and difference, which can be used to predict the prognosis of VSD operation.

Advances in ventricular septal defect treatment with different types of occluders / 国际儿科学杂志

Ventricular septal defect(VSD) is the most common congenital heart disease, accounting for approximately 25% to 40% of all congenital heart diseases.The traditional treatment for VSD is transthoracic repair under extracorporeal circulation, which is more effective but more traumatic for the patient, and this method cannot completely avoid potential complications related to extracorporeal circulation.In the past 35 years, various types of occluders have been gradually used for the treatment of VSD by means of percutaneous intervention under X-ray or ultrasound guidance, and the main occluders widely used in clinical practice are the domestic membrane VSD occluders(symmetric occluder, eccentric occluder, thin-waist occluder), the domestic muscular VSD occluders and the Amplatzer duct occluders(first and second generation). This article reviews the advantages and disadvantages, indications and use of different types of occluders for transcatheter intervention of VSD.

Echocardiographic diagnosis of special type of atrioventricular septal defect / 中华超声影像学杂志

Objective:To improve the understanding of special type of atrioventricular septal defect (AVSD) and reduce clinical missed diagnosis and misdiagnosis by investigating its echocardiographic features.Methods:Echocardiographic images of patients with special type of AVSD diagnosed by echocardiography and confirmed by surgery in Pediatric Cardiovascular Center, Beijing Anzhen Hospital, Capital Medical University from November 2011 to November 2021 were retrospectively analyzed. Depending on the presence of ventricular septal defect(VSD), special type of AVSD were divided into two types, namely AVSD with intact ostium primum and AVSD with intact ostium primum and ventricular septum. The echocardiographic features of special type of AVSD were summarized.Results:Twenty-five cases of special type of AVSD were diagnosed by echocardiography and 13 cases were confirmed by surgery, including 12 cases with intact ostium primum and 1 case with intact ostium primum and ventricular septum. There were 5 cases without surgical indications, and 1 case was kept under observation due to small VSD. One patient with complex malformation only underwent pulmonary artery banding operation without treating intracardiac malformation. The other 4 cases of perimembrane VSD and 1 case of partial AVSD were misdiagnosed as special type of AVSD by echocardiography. All patients had mild to severe left atrioventricular regurgitation before surgery, and most patients remained left atrioventricular regurgitation of varying degrees after left atrioventricular valve formation. Characteristic sonographical findings included: ①The ostium primum was continuous and there was no ostium primum defect. ②The attachment points of the left and right atrioventricular valves were at the same level. ③The left atvioventricular valve was a trileaflet left atrioventricular valve and attached to the crest of the ventricular septum, forming the so-called "anterior mitral valve leaflet cleft". ④The two groups of papillary muscles of the left ventricle changed the normal anterolateral and posterolateral alignment, but showed a anteriorly and posteriorly relationship (counterclockwise transposition). ⑤In the presence of VSD, an inlet ventricular septal defect would be present. ⑥Color Doppler showed varying degrees of regurgitation signals from the left atrioventricular valve during systolic period. ⑦Other complex deformities may be combined with special type of AVSD.Conclusions:Echocardiography can make a definite diagnosis for special types of AVSD. The prognosis of this malformation is closely related to the left atrioventricular valve, and the shaping effect depends on the development of left atrioventricular valve and regurgitation.

Comunicación interventricular post-infarto agudo de miocardio: una complicación inusual pero mortal / Ventricular septal defect after acute myocardial infarction: an unusual but life-threatening complication

Resumen Introducción: la revascularización temprana ha reducido la presencia de complicaciones mecánicas del infarto agudo de miocardio; la comunicación interventricular suele ocurrir entre el tercer y quinto día después del infarto. Se presenta el caso de un paciente poco usual y en quien los síntomas predominantes fueron principalmente gástricos. Descripción: paciente masculino de 65 años, con historia de hipertensión arterial, tabaquismo activo y consumo frecuente de alcohol, quien consultó por síntomas gastrointestinales y dolor torácico de características atípicas. En el examen físico se encontró un soplo holosistólico de predominio en los focos de la base. El electrocardiograma documentó QS en pared inferior, sin alteraciones del segmento ST-T, biomarcadores positivos y el ecocardiograma encontró disfunción sistólica, aneurisma del segmento basal y medio de la pared inferior con comunicación interventricular con flujo de izquierda a derecha. Fue llevado a coronariografía diagnóstica encontrándose enfermedad severa de dos vasos y luego fue intervenido quirúrgicamente para corrección de aneurisma ventricular, comunicación interventricular y bypass coronario. Conclusión: reconocer las manifestaciones clínicas atípicas del infarto agudo de miocardio y un examen físico bien realizado permiten identificar problemas muy serios, como las complicaciones mecánicas del infarto.

Abstract Introduction: early coronary revascularization has reduced the occurrence of mechanical complications of acute myocardial infarction; ventricular septal defect (interventricular communication) usually occurs between the third and fifth days after the event. We present an unusual case where the predominant symptoms were mainly gastrointestinal. Description: A 65-year-old male patient with a history of high blood pressure, active smoking and frequent alcohol consumption, consulted for gastrointestinal symptoms and chest pain with atypical characteristics. In the physical examination a holosystolic murmur with predominance in the foci of the base was found, the electrocardiogram documented QS in the inferior wall without alterations of the ST-T segment, positive cardiac biomarkers and the echocardiogram reported systolic dysfunction, basal and middle segment aneurysm of the lower wall, with ventricular septal defect with left to right flow. A diagnostic coronary angiography was performed founding two vessel severe disease, then the patient was surgically intervened for ventricular aneurysm correction, ventricular septal defect and coronary bypass. Conclusion: Recognizing the atypical clinical manifestations of acute myocardial infarction and a well-performed physical examination make it possible to identify serious problems such as the mechanical complications of infarction.

Major cardiac defects in cattle in southern Brazil: retrospective study of necropsies over 20 years (2000-2019) / Principais defeitos cardíacos em bovinos no sul do Brasil: estudo retrospectivo de necropsias em 20 anos (2000-2019)

ABSTRACT: Cardiac malformations are reported to be relatively rare in bovine species, with ventricular septal defects being the most common abnormality observed predominantly in young calves. This report aims to describe the cardiac malformations observed in bovids and review the literature related to those malformations in this species. The necropsy reports of 1,783 bovids were reviewed, and 0.4% were diagnosed with a cardiac congenital defect. The most common cardiac congenital malformation detected in this study was ventricular septal defect, observed alone or associated with other anomalies (in one case, as part of tetralogy of Fallot); followed by atrial septal defect, also alone or with other cardiac malformations. Secondary findings in the lungs and liver were observed in 75% of all cases. Histologic cardiac lesions were identified in 25% of all cases, characterized mainly by fibrosis. Other congenital malformations in different organs were observed in 25% of the cases. Considering the importance of cardiac congenital malformations observed in this study, these conditions should be considered as a differential diagnosis for calves who died with no specific clinical signs. Furthermore, macroscopic evaluation is crucial for the diagnosis of cardiac congenital malformations.

RESUMO: Malformações cardíacas são descritas como relativamente raras para bovinos, e defeitos do septo interventricular são as anomalias mais observadas em bezerros. O objetivo deste artigo é descrever os casos de malformações cardíacas observadas em bovinos e uma revisão de literatura sobre estas malformações nesta espécie. Os relatórios de necropsia de 1.783 bovinos foram revisados, e 0,4% destes foram diagnosticados com defeitos cardíacos congênitos. A malformação cardíaca congênita mais diagnosticada neste estudo foi defeito do septo interventricular, observado sozinho ou associado a outras anomalias (em um caso de tetralogia de Fallot); seguida de defeito no septo interatrial, também observado sozinho ou juntamente com outras malformações cardíacas. Lesões secundárias nos pulmões e fígado foram observadas em 75% dos casos. As alterações histológicas no coração, visualizadas em 25% dos casos, foram caracterizadas principalmente por fibrose. Outras malformações congênitas em diferentes órgãos foram identificadas em 25% dos casos. Considerando a importância de malformações cardíacas congênitas observadas neste estudo, estas condições devem ser consideradas como diagnósticos diferenciais para bezerros que morrem sem sinais clínicos específicos. Adicionalmente, a avaliação macroscópica é fundamental para o diagnóstico de malformações cardíacas congênitas.

Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect: 15-year experience of a single center / 中国胸心血管外科临床杂志

@#Objective    To explore the appropriate method of biventricular repair and analyze the risk factors for reoperation, by summarizing the 15-year treatment experience of biventricular repair for double outlet right ventricle with non-committed ventricular septal defect (DORVncVSD). Methods    Clinical data of 162 consecutive patients with DORVncVSD who had biventricular repair from 2005 to 2019 in our center were retrospectively analyzed. The children were divided into two groups according to the path of intracardiac tunnel: 110 patients with ventricular septal defect rerouted to the aorta were recruited into a group A (75 males and 35 females aged 3.6±3.2 years); 52 patients with ventricular septal defect rerouted to the pulmonary artery were into a group B (30 males and 22 females aged 2.8±2.7 years). In order to establish a smooth intracardiac tunnel, enlargement of VSD, the resection of conus muscle and the transfer of tricuspid tendon or papillary muscle, etc were performed at the same time. Results    In the patients with biventricular repair, there were 9 (5.6%) early deaths and 6 (3.7%) early intracardiac baffle obstructions. During the follow-up of 7.5±7.0 years, and 8 (4.9%) late deaths occurred. The 1-year, 5-year, 10-year and 15-year survival rates of the group A were 92.7%, 91.1%, 91.1%, 85.4%, respectively and those of the group B were 92.2%, 85.2%, 85.2%, 85.2%, respectively. The difference between the two groups was not statistically significant (P=0.560). The follow-up results showed that 10 (6.2%) patients had late-onset intracardiac tunnel obstruction, and 8 patients underwent reoperation. There were more late-onset intracardiac tunnel obstruction patients and overall intracardiac tunnel obstruction patients in the group A than those in the group B (9 patients vs. 1 patient, P=0.017; 15 patients vs. 1 patient, P=0.001). No significant difference of early mortality and late mortality was noted for the group A (P=0.386) and the group B (P=0.223). Also it was noted that performing tricuspid valve operation at the same time in the group A had a significant impact to reduce the occurrence rate of intracardiac obstruction (1/46 vs. 15/64, P=0.004), without any tricuspid regurgitation or stenosis. The reoperation rate of patients with Rastelli after right ventricular outflow tract lesions was significantly higher than that of REV surgery and double root replacement surgery (5/14 vs. 0/38, P<0.001). Conclusion    The effect of biventricular repair for DORVncVSD is satisfactory. And concomitant tricuspid procedures can help reduce the occurrence of intracardiac obstructions. Reconstruction of right ventricular outflow tract with biological valved conduit is a risk factor for reoperation.

The mechanism of volume-related mitral regurgitation from anatomy of mitral valve / 中国胸心血管外科临床杂志

@#Objective    To explore the mechanism of volume-related mitral regurgitation (MR) from the anatomy of mitral valve. Methods    A total of 32 patients with ventricular septal defect (VSD) combined MR meeting inclusion criteria in West China Hospital from September 2018 to November 2019 were enrolled in this study. The direction relative to the cardiac axis: the deviation of the MR bundle along the left atrial wall was eccentric, otherwises it was central. There were 23 patients of VSD and eccentric MR (EMR, a VSD-EMR group), including 10 males and 13 females aged 21 (10, 56) months, and 9 patients of VSD and central MR (CMR, a VSD-CMR group), including 4 males and 5 females aged 26 (12, 87) months. Besides, 9 healthy children were enrolled in a control group, including 4 males and 5 females aged 49 (15, 72) months. All patients underwent transthoracic echocardiography (TTE) examination at 2 weeks before surgery and 6 months after surgery, respectively, The MR degree, end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF), antero-posterior diameter (AP), annulus circumference (AC), commissural diameter (CD) were assessed. Results    Before operation, EDV, ESV, SV, AP, AC and CD in the VSD-EMR and VSD-CMR groups were significantly larger or longer than those in the control group (P<0.05); after operation, EDV, ESV, SV, AP and CD decreased compared with those before operation (P<0.05), but there was no significant difference compared with the control group (P>0.05). Compared with the control group, AC was slightly decreased (P<0.05). There was no significant difference in EF between and within groups before and after operation (P>0.05). The improvement rate of MR was 78.9%(15/19) in the VSD-EMR group and 100.0% (9/9) in the VSD-CMR group. Conclusion    After unloading of volume, the valve structure is back to normal except AC. The improvement rate of MR in the VSD-EMR group is lower than that in the VSD-CMR group, which may indicate that the mechanism of VSD-EMR is more complicated.

Risk factors for deleyed recovery after surgical closure of congenital ventricular septal defect in infants with low weight / 中国胸心血管外科临床杂志

@#Objective    To reveal the risk factors for delayed recovery and complications in infants with weight≤ 5.0 kg after surgical ventricular septal defect (VSD) closure. Methods    We retrospectively reviewed a consecutive series of 86 patients with weight≤5.0 kg who were admitted to our institution for surgical VSD closure between January 2016 and July 2019, including 31 males and 55 females with an age of 17-266 (80.3±40.4) d and a weight of 2.5-5.0 (4.4±0.6) kg. The VSDs were divided into perimembranous (n=65, 75.6%), subaortic (n=17, 19.8%) and subaortic combined muscular types (n=4, 4.7%). Mechanical ventilation (MV) time≥24 h or ICU stay≥72 h were defined as delayed recovery. Death, sudden circulatory arrest, complete heart block requiring a permanent or temporary pacemaker implantation, neurological complications, reoperation (for residue shunt or valvular regurgitation), reintubation and diaphragmatic paralysis were considered as significant major adverse events. Results    There was no death, reoperation due to residual VSD or neurological complication. Totally 51 (59.3%) patients had MV timec≥24 h and 51 (59.3%) patients stayed in the ICU≥ 72 h. Two (2.3%) patients required temporary pacemaker and six (7.0%) patients required reintubation. During the follow-up of 3-36 (15.8±8.8) months, 1 patient died of pneumonia after discharge, 5 patients suffered mild tricuspid valve regurgitation and 1 patient suffered decreased left ventricular systolic function in the follow-up. No aortic valve injuries occurred. Conclusion    For patients whose weight≤5.0 kg, short-term results of surgical VSD closure are excellent. Low weight and age may prolong MV time; low birth weight and pulmonary hypertension may prolong ICU stay, but are not independent risk factors.

Yasui procedure for surgical repair of interruption of aortic arch and ventricular septal defect associated with aortic stenosis or aortic atresia / 中华胸心血管外科杂志

Objective:To study the surgical results of Yasui procedure for interruption of aortic arch and ventricular septal defect(IAA/VSD) associated with aortic stenosis(AS) or aortic atresia(AA).Methods:A retrospective study was performed on all 4 children with IAA/VSD/AS(AA) that underwent a Yasui procedure from Dec 2014 to Dec 2019. Three patients had AS, and 1 patient had AA. The age was from 10 days to 25 months and the weight was from 2.7 kg to 10 kg. The type of IAA was type A in 1 and type B in 3. The diameter and Z value of the aortic annulus were 1.9-4.3 mm and -6.7--3.6. The diameter and Z value of the ascending aorta were 2.6-5.8 mm and -5.4--2.6. The mean Z value of LVEDD and LVEDS was 3.6±2.6 and 3.6±2.9 resparately.Results:All 4 patients survived after the Yasui procedure. Three patients underwent primary repair and 1 patient underwent staged repair. CPB time was 128-283 min and aortic cross-clamping time was 98-171 min. Ventilation time was 93-296 h, and ICU and hospital length of stay was 7-25 days and 18-39 days. Follow-up was complete in all patients from 8 to 92 months, and there was no late death. All the patients were in good biventricular function with NYHA grade Ⅰ in 3 and NYHA grade Ⅱ in 2 patients. No residual left ventricular outflow tract obstruction was detected.Conclusion:The Yasui procedure can be an option for patients with IAA/VSD/AS(AA) and good developed left ventricle and can achieve good early surgical results.

Surgical outcomes of interrupted aortic arch with ventricular septal defect in neonates and infants / 中华胸心血管外科杂志

Objective:To summarize the surgical outcomes of interrupted aortic arch with ventricular septal defect (IAA/VSD) in neonates and infants.Methods:This was a retrospective review of clinical data of 123 neonates and infants who received surgery for IAA/VSD from Jan 2009 to Jan 2019. Median age at repair was 48 days. Forty-four patients (36%) were neonates. One-hundred-and-twenty-two patients (99%) underwent standard aortic arch reconstruction with VSD closure, and one neonate (1%) underwent staged Yasui operation. Risk factors for early mortality was analyzed by decision tree model.Results:Early mortality after surgery was 13%. Duration of cardiopulmonary bypass longer than 135 min, surgery received during neonatal period and before 2016 was identified as higher risk group for mortality. Median follow-up time was 3.5 years (range, 1-10 years). Freedom from aortic arch obstruction at 6 months, 1 year, 5 years after surgery was 75%, 72% and 72% respectively. Freedom from left ventricular outflow tract (LVOT) obstruction at 6 months, 1 year, 5 years after surgery was 91%, 83% and 73% respectively. A total of 17 patients received 21 reoperations. The patient who received Yasui operation experienced no residual obstruction during the follow-up.Conclusion:Early outcomes after surgery for IAA/VSD in neonates and infants are satisfactory. However, patients with standard aortic arch reconstruction have a higher risk for aortic and LVOT obstruction, and require multiple reoperations.

Effect of different sufentanil doses on hemodynamics and interleukin-6 in children with ventricular septal defect repair / 中国医师进修杂志

Objective:To study the effect of different sufentanil doses on hemodynamics and interleukin (IL)-6 in children with ventricular septal defect repair (VSDR).Methods:A total of 96 children who underwent VSDR surgery in Three Gorges Hospital Affiliated to Chongqing University from January 2016 to June 2019 were selected. Children with VSDR were enrolled and divided into A group (0.8 μg/kg), B group (1.0 μg/kg) and C group (1.1 μg/kg) according their sufentanil doses. The heart rate (HR), contraction pressure (SBP), the brain electric double frequency index (BIS) and IL-6 in different time were studied and compared. The adverse cardiovascular events and other indicators were studied and compared.Results:At t 2 to t 6, there were significant difference among the three groups in HR, A group: (104.62 ± 10.58), (128.73 ± 13.29), (127.59 ± 13.53), (125.62 ± 12.60) and (118.49 ± 11.62) times/min, B group: (100.27 ± 10.11), (119.33 ± 12.62), (116.57 ± 11.40), (113.57 ± 11.94) and (113.37 ± 11.46) times/min, C group: (87.92 ± 8.87), (98.62 ± 9.69), (94.42 ± 9.38), (88.72 ± 8.62) and (89.36 ± 9.17) times/min; SBP, A group: (79.54 ± 7.59), (102.75 ± 10.62), (90.62 ± 9.52), (85.19 ± 8.62) and (83.98 ± 8.62) mmHg (1 mmHg = 0.133 kPa), B group: (76.74 ± 7.28), (90.45 ± 9.57), (87.38 ± 8.51), (84.72 ± 8.50) and (83.77 ± 8.58) mmHg, C group: (70.62 ± 7.27), (75.62 ± 7.83), (72.69 ± 7.80), (71.28 ± 7.43) and (71.39 ±7.16) mmHg, P<0.05. At t 2 to t 4, there were significant differences among the three groups in BIS, A group: 48.64 ± 5.03, 53.58 ± 5.71 and 59.61 ± 5.87, B group: 48.79 ± 5.12, 50.23 ± 5.24 and 57.75 ± 5.66, C group: 43.59 ± 4.62, 50.31 ± 5.34, 55.26 ± 5.53, P<0.05. At T 2 to T 6, there was a significant difference among the three groups in IL-6, A group: (0.41 ± 0.06), (0.49 ± 0.11), (0.53 ± 0.13), (0.82 ± 0.17) and (0.61 ± 0.15) μg/L, B group: (0.38 ± 0.05), (0.42 ± 0.08), (0.46 ± 0.09), (0.75 ± 0.14) and (0.53 ± 0.12) μg/L, C group: (0.35 ± 0.05), (0.40 ± 0.06), (0.43 ± 0.07), (0.72 ± 0.12) and (0.51 ± 0.11) μg/L, P<0.05. Compared with that in A group and C group, HR, SBP and BIS in B group had relatively low volatility. At T 2 to T 6, IL-6 level in A group was significantly higher than that in B group and C group, and there was a statistically significant difference ( P<0.05). The incidence of cardiovascular adverse events in A group and C group was significantly higher than that in B group, and there was statistical difference ( P<0.05). Conclusions:1.0 μg/kg sufentanil can have less effect on hemodynamics and IL-6 in children with VSDR with less adverse cardiovascular events and other indicators.