Pulmonary hypertension in congenital heart disease of children

Background: Pulmonary arterial hypertension is the dreaded complication of congenital heart disease with left-right shunt. The aim of this study was to describe the epidemio-clinical aspects of PAH associated with congenital heart disease.Methods: This was a retrospective descriptive multicentre study over a 2-year period (1 January 2020 to 31 December 2021) conducted in three University Hospitals in Antananarivo. Hospitalized children with congenital left-right shunt heart disease were included.Results: A total of 74 cases were recruited. The sex ratio was 0.7. The mean age of the children was 10.9 (SD=18.2) months. The mean age of onset of heart disease was 8 (SD=13.6) months. Heart disease was dominated by ventricular septal defect (51.3%), patent ductus arteriosus (39.2%), and atrioventricular canal (8.1%). PAH was encountered in 49 cases (66.2%). The majority of children with PAH were under 12 months of age (71.4%) and malnourished (70.2%). According to the type of heart disease, PAH was encountered in all complete CAVs, in 76.3% of CIVs and in 55.2% of PCAs. Eseinmenger's syndrome was present in 4% of cases, and the death rate was 20.3%.Conclusions: Improved management of children with congenital heart disease is needed, both in terms of diagnosis and surgical treatment, to prevent the onset of PAH.

¿Será realmente una verdadera comunicación interventricular? / Could it really be a true ventricular septal defect?

Ventricular septal defect (VSD) is one of the most common congenital defects. It has a wide variety of clinical spectrum of presentation depending on the location and size of the defect. Generally, small restrictive VSDs present spontaneous closure during follow-up. A clinical case is presented with suspected persistent perimembranous VSD, with described intraoperative anatomical findings of aneurysmal cribriform membrane, ruling out VSD.

La comunicación interventricular (CIV) es uno de los defectos congénitos más comunes. Tiene una amplia variedad de espectro clínico de presentación dependiendo de la localización y tamaño del defecto. Por lo general, en los CIV restrictivos pequeños presentan un cierre espontáneo durante el seguimiento. Se presenta un caso clínico con sospecha de CIV perimembranosa persistente, con hallazgos anatómicos intraoperatorios descritos de membrana cribiforme aneurismática descartandose CIV.

Interventional Treatment of Muscular Ventricular Septal Defect in Children / 昆明医科大学学报

Objects To explore the effectiveness and safety of using the Cardio-O-Fix Plug occluder in the treatment of muscular ventricular septal defect(mVSD)in children.Methods 14 patients with mVSD were taken to the cardiology department of First Affiliated Hospital of Kunming Medical University from July 2015 to June 2021 as research subjects.They were divided into two groups:14 children who received Cardi-O-Fix Plug occluder as the experimental group,and 10 children who received Cardi-O-O-Fix mVSD occluder as the control group.Electrocardiogram and transthoracic echocardiography were used to evaluate the occlusive efficacy and incidence of complications 1 day after surgery and 1 month,3 months,and 6 months of follow-up.Results Among the 24 pediatric patients,22 cases were successfully occluded,and 2 cases were unsuccessful(1 in the experi-mental group and 1 in the control group).The success rate of the experimental group was 92.8%(13/14),while the success rate of the control group was 90.0%(9/10).The average surgical duration of the experimental group was(71.93±14.85)minutes,while the average surgical duration of the control group was(90.70±19.78)minutes.There was a significant statistical difference between the two groups(P<0.05).Both the experimental group and the control group did not experience serious complications during surgery and follow-up.There was no significant difference in cardiac ultrasound indicators(including left ventricular ejection fraction,left ventricular end-diastolic diameter,and pulmonary artery pressure)between the two groups at different time points(P>0.05).Conclusion Trans-catheter closure of mVSD using Cardi-O-Fix Plug occluder in children is both safe and effective.The incidence of arrhythmia is low in the short,medium and long term.

A Case of Ross Procedure for Aortic Regurgitation due to Prolapse of a Right Coronary Cusp Caused by Natural Closure of Ventricular Septal Defect / 日本心臓血管外科学会雑誌

A 44-year-old man had been pointed out to have a heart murmur during his elementary school days. He came to us complaining of chest pain and palpitation. He was diagnosed to have severe aortic regurgitation caused by prolapse of the right coronary cusp, and the left ventricular function was depressed. At surgery, the aortic valve was repaired with the RCC resuspension technique, but regurgitation was not controlled because the RCC annulus was retracted by natural supracristal closure of the ventricular septal defect. We proceeded to the Ross procedure. A pulmonary autograft was harvested and sewed in the aortic annulus. The right ventricular outflow tract was reconstructed using a stentless bioprosthetic valve with glutaraldehyde-treated bovine pericardium. He was discharged after a straightforward postoperative course.

A Case of Infective Endocarditis Treated with Ventricular Septal Defect Closure with the Sandwich Method, Aortic Valve Replacement, and Tricuspid Valve Replacement / 日本心臓血管外科学会雑誌

A 74-year-old man was diagnosed with infective endocarditis (IE) involving the aortic and tricuspid valves, ventricular septal defect (VSD), and complete atrioventricular block. He was admitted to a previous hospital with complaints of fever and neck pain, and he developed complete atrioventricular block during the course of his illness. An echocardiogram revealed severe aortic regurgitation, aortic valve vegetations, and a ventricular septal defect. He was then transferred to our hospital, and he underwent emergent surgery. The aortic valve cusps were calcified and thick, with significant cusp destruction. The vegetations partly extended to the subvalvular area of the right and non-coronary cusp. The vegetations also extended from the atrial septum to the tricuspid valve septal leaflet and perimembranous VSD. Ventricular septal reconstruction using the sandwich technique with two bovine pericardial patches, aortic valve replacement, and tricuspid valve replacement were performed. Postoperatively, he received antibiotic therapy for six weeks and was discharged from our hospital after the implantation of a cardiac resynchronization therapy pacemaker. Echocardiography showed no residual shunts. Our case suggests that the sandwich technique can be a useful method of septal reconstruction for IE with extensive destruction of the ventricular septum.

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.

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.

Study on ultrasound diagnosis of different types of fetus with simple ventricular septal defect and neonatal closure period / 中国现代医生

Objective To investigate the diagnosis of different types of fetuses with simple ventricular septal defect by ultrasound and to analyze the results of neonatal follow-up during the closed period.Methods A total of 100 fetuses diagnosed with simple ventricular septal defect from January 2019 to December 2021 were selected from the 903rd Hospital of the Joint Logistics Support Force of the People's Liberation Army of China,and echocardiography was performed to observe the type and defect diameter.The healing rate of the children was observed after 1 year follow-up.Results In 100 cases,64 cases were medial defect,21 cases were infundibular defect,11 cases were muscular defect and 4 cases were superior ventricular ridge defect.In the membrane defect type,the proportion of defect diameters>5mm was highest at 46.88%,followed by 3-5mm at 34.38%,and<3mm at 18.75%.In the infundibular defect type,the defect diameter<3mm was higher(66.67%),followed by 3-5mm(33.33%).The probability of<3mm defect mouth diameter and 3-5mm defect mouth diameter were 45.45%and 54.55%,respectively.The diameter of the defect<3mm and 3-5mm were the same,both of which were 50.00%.The healing rate of intrauterine and 1 year after birth was the highest in muscular defect type(72.73%),followed by membrane defect type(26.56%),infundibular defect type and superior ventricular crest defect type did not heal in intrauterine and 1 year after birth.Conclusion Simple ventricular septal defect is common in the membranous part,while the membranous part and muscle part defect can heal naturally.Echocardiography is a reliable examination method in the prenatal diagnosis and postnatal follow-up of the fetus with simple ventricular septal defect,which can provide a reliable basis for future treatment.

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.

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.

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.

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.

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.

Mid-term follow-up of perventricular device closure of subarterial ventricular septal defect / 中国胸心血管外科临床杂志

@#Objective    To evaluate the mid-term safety, efficacy and feasibility of perventricular device closure for subarterial ventricular septal defect. Methods    Clinical data of 97 patients diagnosed with subarterial ventricular septal defect through echocardiography in West China Hospital from September 2013 to December 2015 were retrospectively analyzed. There were 66 males and 31 females aged 3 (1.8, 5.9) years. All the patients underwent perventricular device closure under the guidance of transesophageal echocardiography using eccentric occlusion device. Possible complications such as residual shunt, valvular regurgitation, atrioventricular conduction block and arrhythmias were recorded during the 1-year, 3-year and 5-year follow-up. Results    All the patients received perventricular device closure successfully except that 1 patient underwent open surgical repair under cardiopulmonary bypass 3 months later because of the migration of device. So the rate of complete closure was 99.0%. No residual shunt, moderate or severe valvular regurgitation, atrioventricular conduction block or arrhythmias were observed 5 years later. Conclusion    Treating subarterial ventricular septal defect through perventricular device closure exhibits remarkable mid-term efficacy, safety and feasibility with high success rate.