Interventricular Communication Research Articles

Cardiac development demystified by use of the HDBR atlas.

Much has been learned over the last half century regarding the molecular and genetic changes that take place during cardiac development. As yet, however, these advances have not been translated into knowledge regarding the marked changes that take place in the anatomical arrangements of the different cardiac components. As such, therefore, many aspects of cardiac development are still described on the basis of speculation rather than evidence. In this review, we show how controversial aspects of development can readily be arbitrated by the interested spectator by taking advantage of the material now gathered together in the Human Developmental Biology Resource; HDBR. We use the material to demonstrate the changes taking place during the formation of the ventricular loop, the expansion of the atrioventricular canal, the incorporation of the systemic venous sinus, the formation of the pulmonary vein, the process of atrial septation, the remodelling of the pharyngeal arches, the major changes occurring during formation of the outflow tract, the closure of the embryonic interventricular communication, and the formation of the ventricular walls. We suggest that access to the resource makes it possible for the interested observer to arbitrate, for themselves, the ongoing controversies that continue to plague the understanding of cardiac development.

Major evidence of the relationship of cardiovascular disease and tooth extraction: a systematic review

Introduction: Cardiovascular diseases can present a congenital origin, that is present from birth, such as interatrial and interventricular communication, defects of the artery, coronary arteries, or acquired coronary artery anomalies that involve several risk factors such as smoking, alcohol, old age, and sedentary lifestyle. Thus, the acquired ones are the most frequent in the routine of a dental practice, so the dentist should be able to attend to those patients who need special care, regarding the correct use of medications due to the risk of drug interactions, the type of local anesthetic to be used and the management of patients who use anticoagulant therapy. Objective: It was to develop a concise systematic review to highlight the main clinical outcomes of the relationship between cardiovascular diseases and tooth extraction. Methods: The research and development of the work were carried out from December 2023 to February 2024 in the databases Scopus, PubMed, Science Direct, and Scielo, following the PRISMA rules. The quality of the studies was based on the GRADE instrument and the risk of bias was based on the Cochrane instrument (Funnel Plot). Results and Conclusion: A total of 112 studies were found that were subjected to eligibility analysis. The final sample had 36 eligible studies and 21 studies were described in the systematic review. Most studies showed homogeneity in their results, with X2 =63.9% >50%, with p<0.05. It was concluded that the virtual reality technique was effective in controlling blood pressure and heart rate within an acceptable range and can help in the management of blood pressure and heart rate during tooth extraction in patients with hypertension. It is necessary for the dental treatment of these patients with heart disease a good knowledge of the subject and requires medical and dental coordination. Dental surgeons need to be prepared to identify possible medical and dental emergencies and take steps to prevent or treat them effectively and quickly.

A Rare Case of Myocarditis Causing Interventricular Communication and Acute Heart Failure

Myocarditis is a rare but potentially life-threatening condition that involves inflammation of the heart muscle. It can present with a wide range of clinical manifestations, including acute heart failure, arrhythmias, and ventricular dysfunction. In rare cases, myocarditis can lead to the formation of interventricular communication, which is an abnormal communication between the left and right ventricles. We present a case report of a 31-year-old male who presented to the emergency department with acute heart failure and was subsequently diagnosed with myocarditis causing interventricular communication.

Which Phenotypes Should We Include in the Hypoplastic Left Heart Syndrome?

The recent special issue of the World Journal for Pediatric and Congenital Heart Surgery devoted to hypoplastic left heart syndrome, and its related anomalies, contained significant information of great clinical relevance. Very little attention, however, was devoted to the integrity of ventricular septum as providing a criterion to distinguish between the phenotypes to be included within the syndrome, as opposed to the related anomalies. In this commentary, we summarize the evidence in support of the notion that the phenotypes to be included within the syndrome can be interpreted on the basis of an acquired disease of fetal life. We suggest that it is the integrity of the ventricular septum that provided the major criterion for the distinction between the lesions making up the syndrome and the related anomalies. The subsets of lesions to be included within the syndrome can then be recognized in terms of the time, subsequent to the closure of the embryonic interventricular communication, at which the left ventricle ceased its growth relative to the remainder of the cardiac components. On this basis, it is possible to recognize the combinations of aortic and mitral atresia, mitral stenosis with aortic atresia, combined mitral and aortic stenosis, and hypoplasia of the left ventricle with commensurate hypoplasia of the aortic and mitral valves; the latter combination now recognized as the hypoplastic left heart complex.

Right-Sided Infective Endocarditis

Right-sided infective endocarditis (RSIE) is relatively rare, accounting for only 5 to 14% of all cases of infective endocarditis (IE). Its incidence is increasing due to the growing use of intravascular and intracardiac devices, as well as the rising prevalence of intravenous drug use. What distinguishes RSIE is that it often occurs on previously healthy valves. It can be associated with left-sided involvement (infective endocarditis with interventricular communication or complicated by septal perforation) or be limited to the valvular orifices of the right heart, more commonly affecting the tricuspid valve than the pulmonary valve. The diagnosis is established based on the Duke criteria. The prognosis can be poor, and the treatment primarily relies on antibiotic administration, with surgery reserved for specific indications related to vegetation size, hemodynamic issues, or infectious complications.

Can Isolated Discordant Atrioventricular Connections Survive Without Interatrial or Interventricular Shunt?

Clinical presentation of isolated discordant atrioventricular connections is akin to transposition of the great arteries. In the absence of a significant intracardiac shunt, profound cyanosis is expected at birth. We report one such 5-month-old infant who had only mild cyanosis. The left-sided tricuspid valve straddled the interventricular septum with a closed interventricular communication, a type of "Double Outlet Left Atrium with three atrioventricular valves," which provided the necessary "left to right" shunt while severe regurgitation through the straddling part and a patent ductus arteriosus provided the effective pulmonary blood flow.

Infective endocarditis of a forgotten valve

A 62-years-old man with interventricular communication was admitted to the hospital with a 6-month history of constitutional symptoms. The patient was feverish and a grade III/VI continuous murmur was noted in all precordium. Patient’s blood analysis showed elevation of inflammatory markers and bacteriaemia caused by Granulicatella adiacens. Transoesophageal echocardiogram diagnosed infective endocarditis of the aortic valve. The patient completed 3 weeks of antibiotic therapy and was submitted to aortic valve replacement and correction of the interventricular communication. Before hospital discharge, routine transthoracic echocardiogram was done and a pulmonary valve vegetation measuring 6x7 mm, not previously described, was noted, as well as de novo severe pulmonary valve insufficiency. The surgical risk was very high in this patient. Antibiotic therapy was extended and the patient was maintained under close follow-up in appointments. The patient remained asymptomatic and clinically stable.

Impact of coronavirus disease 2019 on adult patients with congenital heart disease

Abstract Background COVID-19 is responsible for a worldwide pandemic, causing more than 18,000 deaths to date in Portugal. Data already exists regarding the increased risk of adverse events in patients with cardiovascular diseases, however the impact of SARS-CoV-2 infection in patients (P) with congenital heart disease (CHD) is still under investigation. Purpose To study the impact of COVID-19 in a adult patients with CHD Methods Adult patients seen at the CHD outpatient's clinic at a tertiary centre, who became infected with SARS-CoV-2 infection up to December 2021 were included. Assessment of patients' symptoms, need for hospitalization and admission in an intensive care unit was assessed based on medical records. Results We identified seventy-nine patients (pts) with COVID-19 infection. Symptoms were present in 67 (84%). The median age was 44 (15) years, 52% were females. Eight P (10%) had complex cyanotic disease; seven Tetralogy of Fallot; five (6%) transposition of great arteries; eight (10%) right ventricle obstacle; two (3%) atrioventricular canal defect; sixteen (20%) atrial septal defect; nine (11%) ventricular septal defect; eight (10%) aortic coarctation; two (3%) had Eisenmenger syndrome. 49% of P had previous surgery or percutaneous procedure. 63% of P were at New York Heart Association (NYHA) class of I and 30% at NYHA II. Mild symptoms were reported by 56 P (71%). Ten adults (7,9%) experienced moderate symptoms (dyspnea and hypoxia) that led to hospitalization for oxygen therapy, none required mechanical ventilation. One death was reported in an 83-year-old patient with non-corrected interventricular communication and compromised biventricular function. There was a significant association between the gravity of CHD and hospitalizations (p=0.02). Conclusion Our pts had mainly mild to moderate symptoms and did not appear to have a disproportionately negative outcome; the need for hospitalization was more frequent in patients with higher CHD gravity. These findings are in line with the emerging data regarding COVID-19 in CHD P, and may be in part explained by the patient's young age and functional status. Funding Acknowledgement Type of funding sources: None.

EP26.18: Megabladder: five cases reported in the last five years in our tertiary centre

The aim of this study was to present a series of cases of megabladders diagnosed prenatally in the last 5 years in our centre. This was a descriptive retrospective study of fetus with megabladder diagnosed in our hospital in last 5 years and their follow-up. Mean age women patients was 27 years old (SD +− 10). Mostly of the patients were not any medical conditions (only an interventricular communication without follow-up). 4 was primiparous and one gestation was with reproduction techniques. Ultrasound diagnoses were at first trimester ultrasound in three cases, remaining two were in early morphological ultrasound (17wk and 18wk). Mean bladder volume of fetuses with diagnosed in first trimester was 14.5 mm3 and in fetuses with diagnosed in second trimester was 27.83 mm3. Two fetus had not any associated malformations and one of them has a spontaneous resolution (already pregnancy is in course). The other one had a Berdon Syndrome, diagnosed with amniocentesis (parents decided termination). In the remaining fetus, one of them was diagnosed of hydrops and late miscarriage was happened. Other one also had other urinary anomalies and the last one severe oligohydramnios (both of them were interrupted legally). In 4 of the 5 cases, pregnancies ended in late miscarriage or in legal termination, similar statistic has been described in bibliography. Prevalence of megabladder diagnosed ranges between 0.06% and 0.3%. Differential diagnosis should be made with other abdominal masses such as ovarian cysts or peritoneal cysts. Once confirmed associated abnormalities or syndrome should be taken into account such as posterior urethral valves, urethral stricture, Prune-Belly syndrome or other rarer syndromes such as Beuren syndrome. Termination or miscarriage is the major described end.

A reassessment of the anatomical features of multiple ventricular septal defects.

Over the course of time, new developments associated with the embryogenesis of the murine heart have served to clarify the developmental processes observed in the human heart. This evidence allows for the creation of a developmental framework for many congenital cardiac defects. We aim to solidify the framework related to the categorization of both solitary and multiple ventricular septal defects. Mice having genetic perturbation of the Furin enzyme have demonstrated perimembranous and juxta-arterial ventricular septal defects, permitting the inference to be made that these defects can co-exist with defects occurring within the apical muscular septum. Basis of developmental evidence, furthermore, all interventricular communications can be placed into one of three groups, namelythose which are perimembranous, juxta-arterial, and muscular. All of the defects are described based on their borders as seen from the morphologically right ventricle. Our focus here will be on those defects within the muscular ventricular septum, recognizing that such defects can co-exist with those that are perimembranous. We discuss the differentiation of multiple discrete defects from those referred to as the "Swiss cheese" variant. As we show, appropriate surgical management requires an understanding of the specific terminology, as the surgical approach may differ depending on the combination of the individual defects. Data from the Society for Thoracic Surgeons revealed that both mortality and morbidity were increased in the setting of multiple as opposed to solitary ventricular septal defects.

Clinical treatment of infectious endocarditis with vegetation in pregnant women and gestational outcomes

Infectious endocarditis is a rare condition in pregnant women. Study clinical treatment of infectious endocarditis with vegetation in pregnant women. Nine cases of pregnant women diagnosed as infectious endocarditis with vegetation from January 2014 to December 2020 were included in a retrospective analysis. Clinical treatments including general, anti-infective treatments, cardiac surgery, termination of pregnancy, were carried out through collaboration between the relevant services. Clinical traits, treatment regimens, maternal and neonatal outcomes of the 9 cases were analyzed. The age was 25 to 36 years. The onset gestational age was 19 to 36 weeks. Clinical symptoms: fever, cough and progressive anemia were the main symptoms. The heart murmur was audible and splenomegaly was affected during the physical examination. Hemocultures were positive. Types of heart disease: 7 cases of congenital heart disease included 2 cases of aortic insufficiency, 1 of mitral insufficiency, 1 of arterial canal persistence, 1 of right ventricular stenosis and 2 of interventricular communication. Rheumatic heart disease included 2 cases of mitral stenosis. General treatment included oxygen intake, cardiac strengthening and diuretic therapy. Nine cases of pregnant women all underwent surgical treatment of heart disease and removal of endocardic vegetation. Caesarean sections were performed for 2 cases in the second trimester and 7 in the last trimester. Cardiac surgery and cesarean section were operated on in 6 cases at the same time over 22 to 34 weeks of pregnancy. Cardiac surgeries were operated on in 2 cases after cesarean section. While cardiac surgery was operated on before caesarean section in another case. Maternal and neonatal outcomes: 7 cases of pregnant women were successfully saved. The risk is extremely high from pregnancy with infectious endocarditis.But there is still a way to save maternal and neonatal life.

The Tietz syndrome associated with cardiac malformation: a case report with literature review

BackgroundTietz syndrome is a very rare clinical entity characterized by the association of profound bilateral congenital sensorineural deafness and generalized hypopigmentation of skin, eyes, and integuments (snow white appearance). It is an autosomal dominant syndrome due to a mutation in the melanocyte inducing transcription factor (MITF) gene. The association of a heart malformation has never been reported in this syndrome.Case presentationWe report two cases of two cousins aged 5 years and 20 months respectively with a history of first degree consanguineous parents. Both girls presented with diffuse hypopigmentation of the skin, blond hair, blue eyes, and bilateral diffuse retinal hypopigmentation at ocular fundus exam. Bilateral profound sensorineural hearing loss was confirmed by auditory brainstem response in both cases. Echocardiography revealed a cardiac malformation such as interventricular communication in the older cousin and interatrial communication in the younger cousin. The family investigation did not reveal a similar case among ancestors. The diagnosis of Tietz syndrome was based on clinical criteria and pedigree. The older cousin underwent a total optical correction and a right unilateral cochlear implantation followed by speech therapy with a satisfactory result after a follow-up of two years. Unfortunately, the little cousin died following a head trauma.ConclusionsTietz syndrome is a rare autosomal dominant genetic disorder, characterized by generalized albinism with bilateral profound hearing loss. It results from a nontruncating mutation in the basic domain of in the MITF gene. Its management must include, in addition to hearing and ophthalmic rehabilitation, the research and treatment of cardiac malformations which may be life-threatening.

Enhanced 3D visualization for planning biventricular repair of double outlet right ventricle: a pilot study on the advantages of virtual reality.

We aim to determine any additional benefit of virtual reality (VR) experience if compared to conventional cross-sectional imaging and standard three-dimensional (3D) modelling when deciding on surgical strategy in patients with complex double outlet right ventricle (DORV). We retrospectively selected 10 consecutive patients with DORV and complex interventricular communications, who underwent biventricular repair. An arterial switch operation (ASO) was part of the repair in three of those. Computed tomography (CT) or cardiac magnetic resonance imaging images were used to reconstruct patient-specific 3D anatomies, which were then presented using different visualization modalities: 3D pdf, 3D printed models, and VR models. Two experienced paediatric cardiac surgeons, blinded to repair performed, reviewed each case evaluating the suitability of repair following assessment of each visualization modalities. In addition, they had to identify those who had ASO as part of the procedure. Answers of the two surgeons were compared to the actual operations performed. There was no mortality during the follow-up (mean = 2.5 years). Two patients required reoperations. After review of CT/cardiac magnetic resonance images, the evaluators identified the surgical strategy in accordance with the actual surgical plan in 75% of the cases. When using 3D pdf this reached only 70%. Accordance improved to 85% after revision of 3D printed models and to 95% after VR. Use of 3D printed models and VR facilitated the identification of patients who required ASO. Virtual reality can enhance understanding of suitability for biventricular repair in patients with complex DORV if compared to cross-sectional images and other 3D modelling techniques.

Congenital heart diseases, surgical treatment and complications in pediatric sample of Hospital Vicente Corral Moscoso, 2017-August 2019

BACKGROUND: The heart is the most commonly affected organ by congenital diseases, with and incidence of 0.8 per 100 newborns. Nearly two thirds of all the surgical procedures are now a days performed before the first year of life, improving survival rate and life quality. This study aims to determine the frequency of the surgical interventions performed to treat congenital heart diseases and its complications. METHODS: An observational, descriptive cross sectional study was carried out; with 70 pediatric patients diagnosed and surgically treated for congenital heart diseases. The data was collected from the patient’s medical records using a form. Statistical analysis was performed using SPSS version 15 software. RESULTS: The median age was 1.1 years, 60% of the sample were women. 90% of the heart diseases were non-cyanogenic. The most frequent diagnosis was: persistence of the arterial duct (58.57%), followed by interventricular communication (12.86%). The type of procedures corresponds to the heart disease, thus 58.57% were performed for closure of arterial duct persistence and 12.86% were surgical repairs for closure of interventricular communication. The median stay in the intensive care unit was 4 days and the median stay in general hospitalization room was 5 days. The main complications in this pediatric population undergoing a surgical procedure were: pneumonia (11.4%) and sepsis of unspecified origin (8.6%). CONCLUSION: Treatment for heart diseases were performed at early ages (average age= 2.5±3.2). More than half of the surgical procedures for congenital heart disease were performed to correct the persistence of the ductus arteriosus, the main complication was pneumonia.

Acute lower extremity ischaemia after surgical interventricular communication closure in a newborn.

We report a 5-month-old infant who developed an unexpected acute ischaemia of the right lower limb following a surgical perimembranous interventricular communication closure. This rare case of ischaemia was due to an occlusive right common iliac artery dissection. It was early managed by angioplasty with two ABSORB® bioresorbable stents, resulted in complete revascularisation of the right leg.

Concordance of Congenital Heart Defect in Monozygotic Twins in Brazil

Tetralogy of Fallot (TOF) consists of four cardiac changes: interventricular communication, pulmonary stenosis, aortic dextroposition and right ventricular hypertrophy. The prevalence is 0.1 per 1,000 live births. A primiparous mother with gestational age of 38 weeks came to the emergency. At the time, it was verified in her twin pregnancy that one fetus had TOF, thus a pregnancy interruption was scheduled for the following day. In the neonatal physical examination of both, localized acrocyanosis, regular heart rhythm with the [...]

Tetralogy of Fallot with pulmonary atresia and aortopulmonary window may mimic common arterial trunk.

Tetralogy of Fallot with pulmonary atresia is a group of congenital cardiac malformations, which is defined by the absence of luminal continuity between both ventricles and the pulmonary artery, and an interventricular communication. Pulmonary arterial supply in patients with tetralogy of Fallot with pulmonary atresia can be via the arterial duct or from collateral arteries arising directly or indirectly from the aorta (systemic-to-pulmonary artery collaterals), or rarely both. The rarest sources of pulmonary blood flow are aortopulmonary window and fistulous communication with the coronary artery.Herein, we describe an outflow tract malformation, tetralogy of Fallot with pulmonary atresia and aortopulmonary window, which was misdiagnosed as common arterial trunk. We emphasise the morphological differences.

Dental treatment in child with congenital heart disease under general anesthesia: Clinical case report

Congenital heart diseases consist of structural defects of the heart and represent serious malformations, which manifest in newborns, contributing significantly to perinatal mortality. The dental management of patients with this condition may require caution by professionals, owing to the possibility of bleeding and the risk of causing an infectious endocarditis due to the variety of microorganisms residing in the oral cavity. The present case report aimed to detail dental care under general anesthesia in a hospital for a child with congenital heart disease. Female patient, 4 years old, was diagnosed with atrial and interventricular communication and hemodynamic repercussion 21 days after birth. The patient received dental treatment according to the proposed plan, which included dental restorations, conservative pulp therapy and extraction of lower anterior teeth. Posteriorly, a removable partial prosthesis was made for the patient for the purpose of functional aesthetic rehabilitation. The use of general anesthesia may reduce the risk of infective endocarditis and allows the performance of several dental procedures with a single antibiotic prophylaxis, since the cardiac pediatric patient needs a correct planning of the procedures to be performed.

Diagnosis of 22q11.2 Deletion Syndrome in a Child With Congenital Heart Disease and Facial Dimorphism: A Case Report

22q11.2 deletion syndrome is caused by a deletion in chromosome 22q11.2 and has more than 180 distinct phenotypes; however, no finding is pathognomonic or even mandatory. This syndrome can be diagnosed by fluorescence in situ hybridization. Thus, we report herein a patient from Manaus, Brazil, who has congenital heart disease and facial dimorphism with the presence of 22q11.2 deletion in the N25 region. Male patient, a 1-year-old son of non-consanguineous parents and without a family history of genetic disease. The patient was hospitalized in the cardiac intensive care unit of the Francisca Mendes University Hospital for surgery. The patient was diagnosed with interventricular communication, low atrial implantation, hypertelorism, and macroglossia. The FISH result revealed the presence of a proximal deletion in the N25 region (22q11.2) in only one of the pairs in chromosome 22. This finding revealed a diagnosis of 22q11.2 deletion syndrome, in other words, a hemizygotes deletion with haploinsufficiency of the CLTCL1 gene in this region. However, it is valid to say that the CLTCL1 gene is related to the clinical picture of the patient reported in this study. Cytogenetic analysis was essential for the etiological diagnosis and revealed 22q11.2 deletion in the N25 region, which resulted in 22q11.2 deletion syndrome. The importance of diagnosing this syndrome lies in the best therapeutic conduct, thus allowing a better quality of life for the patient and adequate genetic counseling. Other cytogenetic studies are essential in order to elucidate the size of the deletion and low copy repeats involved in this deletion.

Benefícios do exercício físico na hipertensão arterial pulmonar associada à cardiopatia congênita não corrigida

Introdução: as cardiopatias congênitas são classificadas como um dos principais grupos para desenvolver hipertensão arterial pulmonar. O programa de exercício físico pode auxiliar no aumento da capacidade de vida diária, melhora clínica e funcional, minimizando as consequências deletérias da patologia.Relato do caso: reportamos um caso de um paciente do gênero masculino, 31 anos, com diagnóstico clínico de hipertensão arterial pulmonar grave em decorrência da dupla via de saída do ventrículo direito com comunicação interventricular, sem intervenção cirúrgica. Após ser encaminhado ao programa de Reabilitação Cardiopulmonar e Metabólica em um centro de referência em cardiologia do Rio Grande do Sul, foram realizadas avaliações clínicas e, também, da capacidade funcional pelo teste de caminhada de 6 minutos, da força muscular e resistência periférica, pelo teste Sentar-Levantar, e da força muscular respiratória, pelo exame de manovacuometria. Os valores apresentavam-se abaixo do valor previsto. O programa de exercício proposto foi realizado durante 30 sessões constando de exercícios aeróbicos com duração de 33 minutos, combinados ao treino de fortalecimento dos grandes grupos musculares.Conclusão: após o término do programa observou-se um acréscimo de 83 metros no teste de caminhada de 6 minutos e um aumento da força muscular respiratória, assim como, redução redução nos parâmetros da escala de Borg.