Interventricular Septal Defect Research Articles

Course and treatment of diabetes: from cardiovascular risk to catastrophe. Clinical case

As of today, type 2 diabetes mellitus and its complications impose a significant burden on healthcare systems worldwide. Myocardial infarction stands as a leading cause of death among patients with type 2 diabetes mellitus due to macroangiopathy resulting from glucose metabolism disorders and endothelial damage [5]. Unfortunately, even with timely assistance, patients with myocardial infarction may develop mechanical complications such as cardiac aneurysm, posing serious risks of myocardial wall rupture with a high likelihood of fatality. In most cases, interventricular septal rupture occurs, manifesting in 0,21% of cases of ST-segment elevation myocardial infarction (STEMI) and 0,04% of cases of non-STEMI. Surgical correction of the defect remains the only effective treatment method, albeit associated with a high risk of mortality. Consequently, intra- and post-operative complications in such patients prompt the search for alternative treatment modalities, including transcatheter closure of the defect [6]. Interventricular septal rupture more commonly occurs in older patients, women, those with hypertension, and chronic kidney disease. Typically, the defect arises against the backdrop of the first myocardial infarction in the absence of timely or absent reperfusion therapy. Interventricular septal rupture may develop within 1–14 days after STEMI. However, the incidence usually demonstrates a bimodal peak within the first 24 hours or 3–5 days after the infarction [12]. A clinical case of a patient with combined cardiometabolic pathology, namely, severe type 2 diabetes mellitus and post-infarction interventricular septal defect, is presented. The case underscores the issue of comorbidity in severe patients. Successful treatment of the patient was achieved through collaborative efforts of cardiac surgeons, intensivists, cardiologists and endocrinologists.

Analysis of risk factors and outcomes of acute kidney injury in young children after cardiac surgery

The aim of this study was to analysis of risk factors and outcomes of acute kidney injury (AKI) in young children with congenital heart disease (CHD) after cardiac surgery. The study included 137 young children with CHD after various types of cardiac surgery. The stages of AKI and indications for peritoneal dialysis (PD) were determined based of Kidney Disease: Improving Global Outcomes (KDIGO) guidelines. The incidence of AKI in young children in the general group was 40.9% (n=56). Stage 1 AKI was diagnosed in 21.9% (n=29) of patients, stage 2 AKI in 12.4% (n=17), and stage 3 AKI in 7.3% (n=10) of patients. Peritoneal dialysis was performed in 11.7% (n=16) of children. The incidence of AKI development after radical correct transposition of the great vessels (TGV) was 55.5% (n=5), truncus arteriosus (TA) was 100%, pulmonary atresia (PA)=25%, tetralogy of Fallot (TF)=38.1%, total anomalous pulmonary vein drainage (TAPVD)=60%, partial anomalous pulmonary vein drainage (PAPVD)=37.5%, atrioventricular canal (AVC)=44.4%, double outlet of main vessels from the right ventricle (DOMV from the RV)=60% , interventricular septal defect with high pulmonary hypertension (VSD)=21.6%, and combined operations was 46.6%. The need for PD after TGV correction was 22.2% (n=2), after TA=100%, after TF=33.3%, after TAPVD=20%, after AVC=11.1%, after VSD=1.9%, and after combined operations was 13.3%. Risk factors for AKI in young children were: younger age, initial heart failure, type of operation, prolonged cardiopulmonary bypass (CPB) and aortic clamping (AC), low cardiac output syndrome, inotropic and vasopressor therapy, hyperlactatemia. The development of AKI led to increased length of stay in the intensive care unit, overall hospitalization and infant mortality.

CLINICAL CASE OF CORRECTION OF VENTRICULAR SEPTAL DEFECT COMBINED WITH AN ANOMALY OF THE SYSTEMIC VENOUS CONNECTION OF THE INFERIOR VENA CAVA

Interrupted inferior vena cava is a rare condition that can occur either in isolation or in combination with asplasia or polysplasia syndromes. Abnormal development of systemic veins is closely related to atrial situs. In levocardia, there are signs of abdominal organ inversion, which is called visceral situs. The present paper describes a clinical case of a infant with a large interventricular septal defect combined with interrupted inferior vena cava with azygous continuation of visceral situs ambiguous heterotaxy. The defect plasty was performed at the operation, and the complete venous cannulation required for artificial circulation was performed by the correctly chosen method of drainage of the superior venous system - through the auricle of the right atrium and the inferior venous system - through a separate hepatic vein cannulation, with a good clinical result.

Analysis of the distribution and orientation of oxygenated and non-oxygenated blood in a double outlet right ventricle

Double outlet right ventricle (DORV) is a malformation of the fetal heart in utero that affects the ventricular chambers. It usually presents with a displacement of the aorta and more than half the circumference of both arterial valves in the right ventricle. A peculiar characteristic is given by an interventricular septal defect (VSD), which allows communication between the left ventricle and the right with consequent mixing of oxygenated and non-oxygenated blood inside the cavities. A crucial question in assessing the degree of severity of functional dysfunction concerns the percentage of oxygenated blood that is ejected into the primary circulation via the aorta, a result that depends on the details of the vortex flow pattern within the two ventricular chambers. This study analyzes a complete DORV case through the use of numerical simulations that allow to identify the concentrations of oxygenated and non-oxygenated blood passing through this geometry. Results show that the VSD presents a significant impact on the fluid dynamic performance of the two ventricles. The analysis of blood concentration allowed to quantify the presence of oxygenated blood ejected into the pulmonary artery and of non-oxygenated blood into the aortic artery. The analysis of this specific case aims to demonstrate how the fluid dynamics analysis of this rare malformation, properly coupled with imaging technology, can provide information that could not be obtained otherwise and that are relevant for a careful clinical management including timely therapeutic intervention.

Mechanical complications of myocardial infarction: basic aspects of pathogenesis and approaches to surgical treatment

Ischemic heart disease is a leading cause of mortality of the adult population. The most mortality of all forms of ischemic heart disease is occurred with development of myocardial infarction. Meanwhile, the direct cause of death are complications of myocardial infarction. Since the advent of catheter-based and surgical reperfusion, there has been a significant decrease in the frequency of these complications. However, patients with large myocardial infarctions or those who do not receive necessary revascularization in time remain at risk of mechanical complications of acute myocardial infarction. The most common mechanical complications are rupture of the papillary muscles of the mitral valve and acute mitral regurgitation, interventricular septal defect, forming of aneurysms and pseudoaneurysms, and free-wall rupture. Although these complications are rare, their prognosis is unfavourable and is associated with high mortality at the prehospital and hospital stage. Early diagnosis, determination of optimal treatment tactics are crucial for increasing the survival rate of patients.

Congenital Heart Disease in Rural Senegal: A Retrospective Study of 79 Patients

Introduction: Congenital heart disease (CHD) is a malformation of the heart present at birth and resulting from a developmental defect during embryonic life. The aim was to assess CHD in rural areas. Methodology: This is a retrospective study conducted over a period of 2 years in rural Senegal. Results: During the study period, we collected 79 patients with CHD, representing a hospital prevalence of 2.6%. The mean age at diagnosis was 17.05 months and the sex ratio was 1.19. The mean birth weight was 2826 g. The main comorbidities were anaemia (54.43%) and respiratory infections 38 cases (48.10%). Respiratory distress was the most common sign with 98.73%. Radiological cardiomegaly was noted in 86.7%. The most frequent CHD were interventricular septal defect (IVD) 21.51% and atrial septal defect (ASD) 8.86%. No patient was able to receive surgical treatment. Seven children died (8.86%) and 21 children were lost to follow-up (26.58%). On Doppler echocardiography, 16.45% of the patients had pulmonary arterial hypertension (PAH). Conclusion: In light of this work, emphasis should be placed on the quality of antenatal consultations, the quality of management and the regular availability of echocardiography and a cardio-paediatrician in order to reduce morbidity and mortality.

262 AN UNUSUAL CAUSE OF PACEMAKER MALFUNCTION WITH NON-COMMON ELECTROCARDIOGRAPHIC PRESENTATION AND UNEXPECTED ECHOCARDIOGRAPHIC FINDING

Abstract This clinical case shows the diagnostic challenge and the management of a 79 y-o male with arterial hypertension, tabagism, obesity and a recent hospitalization for infero-lateral STEMI undergoing primary PCI of Right Coronary Artery with 2 DES and following DDDR MRI PM (pacemaker) implantation (through left cephalic vein) due to the development of persistent 2:1 AV block (atrio-ventricular block). One month later, admission at Emergency Department for syncope. The admission therapy included Aspirin plus Clopidogrel, Ramipril, Atorvastatin. Normal parameters were found at laboratory analyses. The electro-stimulator activity evaluation showed a PM malfunction with ventricular capture failure. Thus, the increase of PM output up to electric ventricular capture (8 Volt/120 msec) was performed, but the following Electrocardiogram (ECG) showed a PM-induced rhythm with RBBB-morphology. Then, the bedside Echocardiogram showed an apparent presence of a linear hyper-echogenic image (lead?) into left ventricle with no inter-ventricular septal defect neither inter-ventricular shunt, inter-atrial septal hypermobility with no evident inter-atrial shunt at rest; while a chest Radiography identified the atrial and ventricular leads with no accurate individuation of their position. The patient was admitted in Intensive Cardiac Care Unit with confirmation of intermittent failure to ventricular capture and RBBB-morphology during PM-induced rhythm at telemetry monitoring. Due to Electrocardiographic and (above all) Echocardiographic findings, a strong suspect of abnormal position of the ventricular lead into left ventricle through a possible PFO has been raised. A chest Computed Tomography was performed confirming the anomalous position of the ventricular lead: the atrial lead was in the right atrium whereas the ventricular lead reached the left atrium through an inter-atrial defect/patent foramen ovale (PFO), then crossing the mitral valve up to left ventricle. The PM-lead repositioning was scheduled two days after. With intra-operative fluoroscopy, a further verify of leads position was observed (the ventricular one into left ventricle through PFO and mitral valve, the atrial one mildly displaced upwards) and the leads repositioning was performed. After positioning of temporary stimulator lead in right ventricle, the ventricular lead has been withdrawn from left-side chambers and positioned into septal-apical right ventricle; furthermore, the position of right atrial lead has been optimized (because of frequent spontaneous passage to the left atrium through the PFO, during manual maneuvers). Finally, the optimal stability of the device was obtained, as well as the optimal atrial and ventricular capture during stimulation test. During the observation in Cardiology Unit in following days, the telemetry monitoring and ECG showed a PM-induced rhythm, without capture nor sensing failure. The chest Radiography confirmed the correct position of atrial and ventricular leads. Finally, the pre-discharge transthoracic Echocardiography showed a LVEF 57% with no valvular pathology nor pericardial effusion, and with atrial and ventricular leads into right-side chambers.

Percutaneous closure of interventricular septal defect in a pediatric patient with dextrocardia. Clinical case report

Percutaneous closure of interventricular septal defect in a pediatric patient with dextrocardia. Clinical case report

ОСОБЕННОСТИ КАЧЕСТВА ЖИЗНИ ДЕТЕЙ С ДЕФЕКТОМ МЕЖЖЕЛУДОЧКОВОЙ ПЕРЕГОРОДКИ

A ventricular septal defect is the most common congenital heart disease. With the improvement of cardiac surgery, the survival rates of children with heart disease have improved. In addition to clinical and hemodynamic improvement, an important task is to study the perception of the quality of life of children with an interventricular septal defect. The purpose of the study is to compare the quality of life of children with a ventricular septal defect. Materials and methods of the study: a survey was conducted of children with ventricular septal defect 3-17 years old (median age 9 years (Q1-Q3 - 5-12 years), as well as their parents using the Russian-language version of the international tool Pediatric Quality of Life Inventory (PEDsQL) 3.0 Cardiac Module used to assess the quality of life of children with diseases of the cardiovascular system. Results: the quality of life on the “heart problems” scale in children operated on for an interventricular septal defect was significantly lower than in children without surgical defect correction. In patients with FC | and || (n = 14) according to NYHA and Ross, the quality of life was statistically significantly lower on the scales “heart problems”, “anxiety associated with treatment”, “learning problems” than in patients with FC 0 (p <0.05). The quality of life of children with a body mass index below the norm on the “heart problems” scale was significantly lower than in children with a body mass index higher and within the norm (p <0.05). Conclusions: the quality of life of children with a ventricular septal defect may be impaired. Nevertheless, children without operational correction, with normal and above normal levels of physical development, in the absence of heart failure, better perceive the quality of life. In order to provide high-quality care, in addition to a thorough clinical assessment, quality of life should be an important aspect of the comprehensive treatment of heart disease

Clinical and molecular characterization of 1q43q44 deletion and corpus callosum malformations: 2 new cases and literature review

BackgroundCorpus callosum malformations (CCM) represent one of the most common congenital cerebral malformations with a prevalence of around one for 4000 births. There have been at least 230 reports in the literature concerning 1q43q44 deletions of varying sizes discovered using chromosomal microarrays. This disorder is distinguished by global developmental delay, seizures, hypotonia, corpus callosum defects, and significant craniofacial dysmorphism. In this study, we present a molecular cytogenetic analysis of 2 Tunisian patients with corpus callosum malformations. Patient 1 was a boy of 3 years old who presented psychomotor retardation, microcephaly, behavioral problems, interventricular septal defect, moderate pulmonary stenosis, hypospadias, and total CCA associated with delayed encephalic myelination. Patient 2 was a boy of 9 months. He presented a facial dysmorphia, a psychomotor retardation, an axial hypotonia, a quadri pyramidal syndrome, a micropenis, and HCC associated with decreased volume of the periventricular white matter. Both the array comparative genomic hybridization and fluorescence in situ hybridization techniques were used.ResultsArray CGH analysis reveals that patient 1 had the greater deletion size (11,7 Mb) at 1q43. The same region harbors a 2,7 Mb deletion in patient 2. Here, we notice that the larger the deletion, the more genes are likely to be involved, and the more severe the phenotype is likely to be. In both patients, the commonly deleted region includes six genes: PLD5, AKT3, ZNF238, HNRNPU, SDCCAG8 and CEP170. Based on the role of the ZNF238 gene in neuronal proliferation, migration, and cortex development, we hypothesized that the common deletion of ZNF238 in both patients seems to be the most responsible for corpus callosum malformations. Its absence may directly cause CCM. In addition, due to their high expression in the brain, PLD5 and FMN2 could modulate in the CCM phenotype.ConclusionOur findings support and improve the complex genotype–phenotype correlations previously reported in the 1qter microdeletion syndrome and define more precisely the neurodevelopmental phenotypes associated with genetic alterations of several genes related to this pathology.

Frequency of congenital heart diseases in newborn: ten years’ single clinic experience

Congenital heart disease occurs in the population with a frequency of 8 per 1000 live births. Information on the incidence of various congenital heart defects in newborns in connection with the widespread introduction of prenatal ultrasound diagnostics and an increase in its accuracy allows for planning pregnancy and management of newborns with critical heart defects. The aim of this study wasto retrospectively study the incidence of various congenital heart defects according to echocardiographic studies among newborns in the framework of the experience of one clinic over the period from 2009 to 2019. In the clinic of the Science Research Institute of Cardiology, echocardiographic studies were performed in 34298 children aged 1 day to 17 years, the average age was 3.68 years, the median 2.0 years. The newborns accounted for 4.6% of them (n = 1607). The results of echocardiographic studies were analyzed in 1607 newborns aged from 1 to 27 days. In 247 (15.3%) of the examined no congenital defects were detected, 1360 newborns had various congenital heart diseases. It was found that the most common anomaly in newborns were interventricular septal defects (37%), more than 60% of all interventricular septal defects were clinically insignificant small muscle defects. It has been shown that the incidence of congenital heart defects in newborns with complex prenatal diagnosis (coarctation of the aorta, double outlet right ventricle) has increased in recent years. A decrease in the incidence of atrioventricular septal defect was observed over the past five years. The possibilities of neonatal cardiac surgery in a particular clinic influenced the structure of the occurrence of individual congenital heart disease due to prenatal pregnancy planning and surgical tactics.

Innocent Murmur since Childhood: Elderly Patient with Undiagnosed Interventricular Septal Defect

Ventricular septal defects in adulthood only account for 10 per cent of congenital heart defects, In patients with evidence of LV volume overload and no Pulmonary Arterial Hypertension (no non-invasive signs of Pulmonary artery pressure elevation or invasive confirmation of Pulmonary Vascular Resistance <3 Woods Units in case of such signs), VSD closure is recommended regardless of symptoms. “Maladie de Rogers” is referred to as a VSD with a small left-to-right shunt, the pulmonary vascular resistance is not significantly elevated and the left-to-right shunt is small (Qp: Qs <1.5:1), in the absence of aortic valve prolapse and regurgitation or Infective endocarditis, may be managed by observation without need for operative intervention.

Innocent Murmur since Childhood: Elderly Patient with Undiagnosed Interventricular Septal Defect

Innocent Murmur since Childhood: Elderly Patient with Undiagnosed Interventricular Septal Defect

P427 LATE TREATMENT OF A CASE OF PULMONARY ARTERY HYPOPLASIA

Abstract Here we want to present the case of a woman who came to our observation for the onset of dyspnea due to exertion. It is a subject followed from a young age for a congenital malformation of the urinary tract. At that time the presence of stenosis of the branches of the pulmonary artery associated with an interventricular septal defect was accidentally diagnosed; however the blood pressure values in the pulmonary artery were within the limits, so no interventional therapy was undertaken. In the following years the patient developed systemic arterial hypertension associated with renal failure for which medical therapy was started and an annual follow–up was scheduled. Over the years various echocardiographic checks were repeated without ever finding high pressures in the pulmonary artery. At the last check, performed precisely for the onset of dyspnea from exertion, the estimated systolic pressure in the pulmonary artery was equal to 100 associated with a dilation of the right ventricle with hypertrophy, hepatomegaly was also present in the absence, however, of signs of peripheral congestion. The catheterization of the right heart confirmed the presence of severe precapillary pulmonary hypertension and the CT scan with three–dimensional reconstruction highlighted the almost total agenesis of the left lung branch thus revealing the presence of a pulmonary hypertension of the fourth group, also supported by the negativity of the other tests proposed in the diagnostic work of the ESC Guidelines. In agreement with a national reference center for the treatment of pulmonary hypertension, we started Bosentan therapy with increasing dosage up to the target dose and long–term oxygen therapy. After four months of follow–up, the patient shows an improvement in functional capacity and the haemodynamic data remain stable. To our knowledge there are only the experiences of single centers regarding the treatment of forms of pulmonary hypertension due to anatomical malformations of the pulmonary circulation and for this reason medical therapy is not standardized. This subset of patients represents a challenge for the clinician as hypertension can be secondary both to the lack of part of the vascular bed and to reactive hyperplasia of the endothelium of the remaining circulation due to parietal stress due to pressure overload.

Cardio-facio-cutaneous syndrome and gastrointestinal defects: report on a newborn with 19p13.3 deletion including the MAP 2 K2 gene

BackgroundCardio-facio-cutaneous syndrome (CFCS) belongs to RASopathies, a group of conditions caused by mutations in genes encoding proteins of the rat sarcoma/mitogen-activated protein kinase (RAS/MAPK) pathway. It is a rare syndrome, with about 300 patients reported. Main clinical manifestations include facial dysmorphisms, growth failure, heart defects, developmental delay, and ectodermal abnormalities. Mutations (mainly missense) of four genes (BRAF, MAP 2 K1, MAP 2 K2, and KRAS) have been associated to CFCS. However, whole gene deletions/duplications and chromosomal microdeletions have been also reported. Specifically, 19p13.3 deletion including MAP 2 K2 gene are responsible for cardio-facio-cutaneous microdeletion syndrome, whose affected subjects show more severe phenotype than CFCS general population.Case presentationHereby, we report on a female newborn with prenatal diagnosis of omphalocele, leading to further genetic investigations through amniocentesis. Among these, array comparative genomic hybridization (a-CGH) identified a 19p13.3 microdeletion, spanning 1.27 Mb and including MAP 2 K2 gene. Clinical features at birth (coarse face with dysmorphic features, sparse and friable hair, cutaneous vascular malformations and hyperkeratotic lesions, interventricular septal defect, and omphalocele) were compatible with CFCS diagnosis, and further postnatal genetic investigations were not considered necessary. Soon after discharge, at around 1 month of life, she was readmitted to our Neonatal Intensive Care Unit due to repeated episodes of vomiting, subtending a hypertrophic pyloric stenosis (HPS) which was promptly identified and treated.ConclusionsOur report supports the 19p13.3 microdeletion as a contiguous gene syndrome, in which the involvement of the genes contiguous to MAP 2 K2 may modify the patients’ phenotype. It highlights how CFCS affected subjects, including those with 19p13.3 deletions, may have associated gastrointestinal defects (e.g., omphalocele and HPS), providing further data on 19p13.3 microdeletion syndrome, and a better characterization of its genomic and phenotypic features. The complex clinical picture of such patients may be worsened by additional, and even precocious, life-threatening conditions like HPS. Clinicians must consider, anticipate and/or promptly treat possible medical and surgical complications, with the aim of reducing adverse outcomes. Extensive diagnostic work-up, and early, continuous, and multidisciplinary follow-up, as well as integrated care, are necessary for the longitudinal clinical evolution of any single patient.

Diagnóstico ecocardiográfico de defeito do septo interventricular com agravo de síndrome de Eisenmenger em cão adulto – relato de caso

Interventricular septal defect (IVSD) is a congenital heart disease characterized by communication or non-closure of the interventricular septum in the embryonic or postnatal period, causing a failure in the separation between the systemic and pulmonary circulation. The severity of the hemodynamic consequences depends on the extent of the defect and the magnitude of the flow through it. Blood flow commonly occurs from left to right, because systemic vascular resistance is greater than pulmonary resistance. This report describes the echocardiographic findings in a 4-year-old male Poodle treated at the Veterinary Hospital with a history of exercise intolerance, cyanosis and episodes of syncope. On echocardiography, perimembranous IVSD was diagnosed with atrial and right ventricular dilatation, discontinuity of the interventricular septum in the subaortic region measuring approximately 5 mm. In the Doppler evaluation, reverse turbulent flow was evidenced, characterizing shunt from right to left (Eisenmenger syndrome). Despite preserved systolic function, abnormal relaxation was observed. Doppler echocardiography was a fundamental complementary imaging test for the diagnosis of this congenital heart disease since its suspicion is not commonly raised in adult patients. Doppler echocardiography allowed us to identify the defect, define its size and extension, determine the direction of blood flow as well as its hemodynamic consequences.

Патологічні стани у новонароджених дітей на тлі недиференційованої дисплазії сполучної тканини в їхніх матерів

Undifferentiated connective tissue dysplasia (UCTD) in pregnant women reduces the adaptive capacity of their newborns and is an unfavorable background for the development of certain pathological conditions of the perinatal period. At the same time, information on the spectrum of diseases in children born to mothers with clinical manifestations of UCTD is rather contradictory. Purpose — to study the nature and direction of the correlation between certain pathological conditions in newborns and clinical signs of UCTD in their mothers. Materials and methods. We examined 75 women in labor aged 16 to 44 years old and their 75 newborn children (38 boys and 37 girls) during 2018–2020. The number of girls and boys is approximately the same, the gender difference between children did not affect on the results of the study. The newborns gestational age (GA) was 28–42 weeks, and their birth weight (BW) was 1500–4070 g. Among them there were 51 (68%) full-term, and 24 (32%) — preterm infants. Anthropometric indices (AI), which were used to confirm dolichostenomelia as a UCTD marker, were calculated for mothers and their children. The integral indicator of dolichostenomelia (IID) was determined by summing the standardized values of these coefficients for each mother and child. The presence of neonatal encephalopathy, congenital pneumonia, interventricular septal defect (IVSD), intrauterine growth retardation (IUGR), respiratory distress syndrome, asphyxia at birth, grade I–II of intraventricular hemorrhage (IVH), and necrotizing enterocolitis were taken into account in children. As for mothers, the presence of complications of their pregnancy and labor associated with UCTD was taken into consideration, and in addition, hypermobility of the joints was assessed according to the Beighton scale. Women were divided into two groups: group I (n=45) consisted of women with >1 anthropometric marker of UCTD, group II (n=30) involved mothers who did not have any of these markers. Newborn children were divided into two groups, respectively: group I involved newborns from mothers with signs of UCTD, group II consisted of borned from mothers without signs of UCTD. Results. It was found that mothers with UCTD markers reliably more often had children weighing less than 2500 g (ϕс=0.251; р=0.029). According to the results of the morbidity analysis, it was clarified that children borned from mothers with UCTD manifestations, were more often suffering from congenital pneumonia (ϕс=0.218; р=0.049), IVSD (ϕс=0.241; р=0.037) and IUGR (ϕс=0.31; р=0.029). According to the results of the rank correlation analysis, a direct moderate correlation was established between the presence of progression of varicose veins in women during pregnancy and IID both in them (ρ=0.463; p<0.001) and in their children (ρ=0.369; p=0.001); a similar in degree and direction correlation of >3 UCTD associated obstetric complications with IID of mothers (ρ=0.305; p=0.008) and their children (ρ=0.326; p=0.004) was also found. At the same time, a positive weak correlation was established between mothers' IID and registration of placental dysfunction (ρ=0.231; p=0.046), polyhydramnios (ρ=0.234; p=0.043) in them. As for newborns, their IID had a direct moderate correlation with their mothers' IPD (ρ=0.364; p=0.001), and a weaker correlation with joint hypermobility in their mothers (ρ=0.258; p=0.025) and obstetric ruptures (ρ=0.230; p=0.047). Simultaneously, there were no statistically reliable differences between the groups of children by gender, their GA, age of their mothers and the mode of delivery. Conclusions. Consequently, children borned from mothers with clinical markers of UCTD are more often have low BW (p=0.029); during the neonatal period they have IUGR (p=0.029), congenital pneumonia (p=0.049) and IVSD (p=0.037) more often registered. It should also be noted that integral anthropometric markers of UCTD in newborn children have the most significant correlation with similar markers in their mothers (p=0.001), on the one hand, as well as with the progression of varicose disease in women during pregnancy (p=0.001) and a combination >3 UCTD associated obstetric complications (p=0.004), on the other hand. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: newborns, postpartum women, undifferentiated connective tissue dysplasia.

Tratamento intervencionista e cirúrgico híbrido de lesões cardíacas traumáticas complexas por arma branca

Penetrating injuries to the heart are usually devastating and few patients survive the initial trauma. The most frequent penetrating injuries are caused by projectile injuries and less commonly by melee weapons. Most of these injuries involve chamber free walls and a small percentage can affect the interventricular septum. We report a case in which an emergency surgical procedure was successful in controlling cardiac tamponade, and repairing a right ventricular laceration caused by multiple stab wounds. Subsequently, a successful interventional occlusion of a large interventricular septal defect, associated with significantly augmented pulmonary flow was performed.

Valsalva Sinus Aneurism Associated with Interventricular Septal Defect, Right Medioventricular Stenosis and Bicuspid Aortic Valve

Backgroud: Valsalva sinus aneurysms are abnormalities of the heart that can be acquired or congenital. The most often associated congenital anomaly is interventricular communication which is a predisposing factor to the occurrence of aortic insufficiency. Transcatheter closure has recently emerged as an alternative but surgery still has indications. Aim: We report our experience on the management of this pathology in a subsaharian country. Patient and method: We report the observation of a 7.5-year-old child whose discovery was fortuitous upon exploration of a heart murmur. Results: Doppler echocardiography made the diagnosis possible showing a large under pulmonary interventricular septal defect with a right left shunt, a small non-stenotic sub-aortic membrane, a bicuspid aortic valve without leakage or stenosis, a significant dilation of the aortic root. The surgical management was an intervention according to Tyrone-David technique with closure of the interventricular septal defect. Postoperative outcomes were simple and the short- to medium-term evolution favorable. Conclusion: Depending on the definition used to diagnose valsalva sinus aneurysms, the frequency of this pathology may be underestimated. What seems most appropriate to us is the definition considering the diameter of the sinuses relative to the body surface. An another endpoint is the need to explore the aortic root in the presence of interventricular communication and bicuspid aortic valve.

Cardiovascular risks in infants born with reproductive technologies

Russia is on the second place among European countries in the number of completed cycles of assisted reproductive technologies (ART). The proportion of children conceived using ART becomes annually higher. The present review summarises data on the impact of new reproductive technologies on the risk of developing cardiovascular diseases are presented. Potential health risks in the introduction of ART in babies born are discussed in numerous studies, the impact on all stages of embryo- and organogenesis is noted. The greatest problem is a possible increase in the incidence of congenital malformations (CM) in children after ART compared to the latter in the natural population. The frequency of CM is due not only to the use of APT, but also to factors such as genetic characteristics, age and state of health of the mother, aggravated obstetric-gynecological history, multiplicity, low birth weight of the fetus. In children conceived with ART, often appear early symptoms of systemic endothelial dysfunction, metabolic disorders (lipid metabolism, glucose level), congenital heart defects (interventricular septum defect, small cardiac development abnormality (false chord), open arterial duct, open oval window). There is a need to improve prenatal diagnostics of CM in early pregnancy and regular medical examinations of children conceived with ART.