Intact Interventricular Septum Research Articles

Case Report of Absent Pulmonary Valve with Intact Interventricular Septum

Abstract Absent pulmonary valve syndrome is a rare cardiac malformation, usually associated with tetralogy of Fallot. Its presence with intact interventricular septum is even less common with case reports or very small series in the literature. We describe a case who presented with symptoms of right heart failure due to an absent pulmonary valve with severe pulmonary regurgitation and small atrial septal defect. Diagnostic evaluation revealed grossly dilated main pulmonary artery and rudimentary leaflets of the pulmonary valve with severe regurgitation. Ventricular septum was intact and there was no patent ductus arteriosus. Later, the patient underwent surgical prosthetic pulmonary valve implantation.

A Rare Combination of Transposition of Great Arteries and Total Anomalous Pulmonary Venous Connection

The association of total anomalous pulmonary venous connection (TAPVC) with transposition of great arteries (TGA) is a very rare combination of 2 congenital cyanotic heart diseases. TAPVC, in the case of TGA with the intact interventricular septum (IVS), is a cause of early regression of the left ventricle (LV). Very few cases of this rare combination (TAPVC and TGA with or without VSD) have been reported in the world of literature. We found 10 case reports in the literature. The minimum age was 6 days and the maximum age of 16 years. 5 of them, which were operated before 1990, underwent atrial switch with TAPVC repair. Other 4 of them, operated after 1995, underwent arterial switch with TAPVC repair. In this case report we are reporting 2 such rare cases. In both cases, large VSD prevented LV regression.

Prenatal Diagnosis of Fetal Heart Failure.

Fetal heart failure (FHF) is a condition of inability of the fetal heart to deliver adequate blood flow for tissue perfusion in various organs, especially the brain, heart, liver and kidneys. FHF is associated with inadequate cardiac output, which is commonly encountered as the final outcome of several disorders and may lead to intrauterine fetal death or severe morbidity. Fetal echocardiography plays an important role in diagnosis of FHF as well as of the underlying causes. The main findings supporting the diagnosis of FHF include various signs of cardiac dysfunction, such as cardiomegaly, poor contractility, low cardiac output, increased central venous pressures, hydropic signs, and the findings of specific underlying disorders. This review will present a summary of the pathophysiology of fetal cardiac failure and practical points in fetal echocardiography for diagnosis of FHF, focusing on essential diagnostic techniques used in daily practice for evaluation of fetal cardiac function, such as myocardial performance index, arterial and systemic venous Doppler waveforms, shortening fraction, and cardiovascular profile score (CVPs), a combination of five echocardiographic markers indicative of fetal cardiovascular health. The common causes of FHF are reviewed and updated in detail, including fetal dysrhythmia, fetal anemia (e.g., alpha-thalassemia, parvovirus B19 infection, and twin anemia-polycythemia sequence), non-anemic volume load (e.g., twin-to-twin transfusion, arteriovenous malformations, and sacrococcygeal teratoma, etc.), increased afterload (intrauterine growth restriction and outflow tract obstruction, such as critical aortic stenosis), intrinsic myocardial disease (cardiomyopathies), congenital heart defects (Ebstein anomaly, hypoplastic heart, pulmonary stenosis with intact interventricular septum, etc.) and external cardiac compression. Understanding the pathophysiology and clinical courses of various etiologies of FHF can help physicians make prenatal diagnoses and serve as a guide for counseling, surveillance and management.

Staged Percutaneous Management of Pulmonary Atresia and Intact Interventricular Septum: Stretching the Limits.

Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by catheter-based interventions and complemented by various surgical procedures. We aim to determine a long-term treatment strategy to enable patients to be surgery free, depending solely on percutaneous interventions. We selected five patients from among a cohort of patients with PA/IVS treated at birth with radiofrequency perforation and dilatation of the pulmonary valve. Patients had reached a pulmonary valve annulus of 20 mm or larger on their biannual echocardiographic follow-up, with right ventricular dilatation. The findings, together with the right ventricular outflow tract and pulmonary arterial tree, were confirmed by multislice computerised tomography. Based on the angiographic size of the pulmonary valve annulus, all patients were successfully implanted with either Melody® or Edwards® pulmonary valves percutaneously, regardless of their small weights and ages. No complications were encountered. We managed to stretch the age and weight limitations for performing percutaneous pulmonary valve implantation (PPVI): interventions were attempted whenever a pulmonary annulus size of >20 mm was reached, which was rationalised by the prevention of progressive right ventricular outflow tract dilatation and accommodating valves between 24 and 26 mm, which is enough to sustain a normal pulmonary flow in adulthood.

Alternative Approach for Pulmonary Valvuloplasty in Critical Congenital Pulmonary Stenosis

Critical congenital pulmonary stenosis with intact interventricular septum is a cyanotic and potentially lethal neonatal cardiac anomaly. Percutaneous transcatheter balloon pulmonary valvuloplasty for critical pulmonary stenosis often presents technical difficulties due to supra-systemic pressure in right ventricle. Using transjugular approach instead of the femoral vein helps in dealing with this issue. We describe the successful use of right internal jugular vein approach in a case of critical pulmonary stenosis.

Successful completion of Senning surgery in a patient with dextro-transposition of the great arteries (DTGA) with presentation in the fourth decade.

Transposition of great arteries (TGA) is a common congenital heart disease presenting in newborns. Nearly 90% of patients with TGA with intact interventricular septum die by one year of age if not treated. We present an interesting case of The Senning procedure , which was successfully performed on a 40-year-old lady.

Infective Endocarditis Caused by Granulicatella elegans in a Child With Patent Ductus Arteriosus.

To the editors: Infective endocarditis (IE) is an inflammatory process of infectious origin that affects cardiovascular structures. The term endarteritis is currently included in the definition of infective endocarditis. Granulicatella elegans is a rare cause of infective endocarditis, which has a subacute presentation of the disease, with nonspecific symptoms and mild elevation of inflammatory biomarkers. It is associated with a high-mortality rate in adults.1 In the pediatric age group, there are 3 cases of IE described by Granulicatella spp., associated with congenital heart disease: 1 with pulmonary atresia with intact interventricular septum and mild Ebstein’s malformation already operated,2 another one with Shone’s syndrome and the last one with infundibular pulmonary stenosis.1 Our case represents the fourth case worldwide with IE caused by Granulicatella spp., in congenital heart disease, and the first with patent ductus arteriosus (PDA). We report an 8-year-old female patient with a history of PDA was diagnosed at another hospital at 3 years of age. She was referred from another hospital with a 7-month history of fever, predominantly during the evening, between 38.4°C and 38.6°C. On admission hyperdynamic precordium with thrill, normal S2, left infraclavicular continuous murmur grade 4/6, slightly grainy sound, calling attention to a metallized acoustic component and wide pulses. Three blood cultures tested positive after 48 hours. Gram staining of the blood culture revealed Gram-positive cocci, that were, nonsporulating in pairs and short chains and, pleomorphic (Fig. 1A). Blood cultures were identified with Granulicatella elegans nutritionally variant streptococci using a commercial kit MALDI Sepsityper (Bruker Daltonics). Little growth on sheep-blood agar in a microaerophilic atmosphere after 72 hours.FIGURE 1.: A. Gram staining showing Gram-positive cocci, nonsporulating in pairs and, short chains, pleomorphic. B, (day 30) TTE. Parasternal 2D short axis view and color Doppler showing PDA with left to right shunt and hyperechogenic mass (green arrow) in the PA following the flow of the ductus. PA, pulmonary artery; PDA, patent ductus arteriosus; TTE, transthoracic echocardiogram.Vegetations were not found on the transthoracic echocardiogram (TTE) at admission, but after 30 days reveled; image suggestive of vegetation attached the pulmonary trunk (13 mm × 3 mm) (Fig. 1B). After completing 40 days of ceftriaxone, the vegetation was removed percutaneously, on the 58th day, the PDA was closed with Amplatzer ADO 8/6. Despite advances in diagnosis and treatment, infective endocarditis in the 21st century can be a life-threatening disease with high morbidity and mortality.1 Cyanogenic congenital heart disease, left heart lesions and atrioventricular defects are associated with an increased risk of IE in childhood.3 In children, the most frequent microorganism in subacute IE is Streptococcus viridans group4; however, in the second group nutritionally variant Streptococcus species (NVS) have been identified as etiological agents responsible for 5% of the cases with bacteriologic diagnosis.5 Physicians should suspect subacute IE in patients presenting with intermittent fever of prolonged evolution, constitutional symptoms, and changes in auscultatory phenomena in the context of children with congenital heart disease, despite having negative initial blood culture and absence of initial IE findings on echocardiogram. Granulicatella spp., IE has a subacute presentation with a mild increase in inflammatory biomarkers, and its morbidity and mortality can be significant. The modified Duke criteria are of great value; however, in some patients with subacute IE the diagnosis can be missed, therefore, the clinical features should be considered when a patient has a fever of unknown origin.

Comparison of the Methods of Surgical Treatment of Complex Congenital Heart Defects Combined with Right Ventricular Hypoplasia

Surgical treatment of complex congenital heart defects (CHD) in patients who cannot undergo radical correction due to pronounced hypoplasia or dysfunction of the right ventricle (RV) remains one of the most urgent problems in pediatric cardiac surgery. In 1989, Billingsley et al. first reported the addition of bidirectional cavopulmonary anastomosis to definitive biventricular repair in patients with RV hypoplasia, pulmonary artery atresia, and intact interventricular septum in four patients, and introduced the term one and half ventricle repair (1.5VR). The method of 1.5VR consists of bidirectional cavopulmonary anastomosis, which reduces the volume load on the RV, in addition to the complete repair of other CHDs.
 The aim. To assess perioperative characteristics of patients, immediate and long-term results after surgical correction of complex CHD through the method of 1.5VR.
 Materials and methods. In the period from 1996 to 2022, surgical correction was performed in 33 patients with complex CHD combined with hypoplasia and/or dysfunction of the RV at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 21 male patients (64%) and 12 female patients (36%). The mean age of the patients at the time of surgery was 57.9 months. Me = 34 [3.5; 312] months. The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities, which made it possible to assess the morphology, kinetics of the heart structures, and hemodynamic state. Depending on the selected tactics of surgical treatment, all the patients were divided into two groups: group І (n=21) with patients operated through the method of 1.5VR, and group ІІ (n = 12) including those operated through the single ventricle pathway.
 Results. In the early postoperative period, there were 3 (9%) deaths in both groups: one (4.7%) death in group I, and 2 (16%) deaths among patients of group II. In all the deceased patients, the cause of death was acute heart failure, which subsequently led to multiple organ failure. In one patient of the group II, in addition to heart failure, acute cerebrovascular accident occurred. In the group I, the average indicator of systemic saturation (92±6.2%) at discharge from the hospital was higher, than that in group II (87±4.7%). Average duration of mechanical ventilation (10±7.3 vs. 15±7.8 hours), total exudation (65±34 vs. 88.8±39.9 hours), total duration of sympathomimetic support (71±27.5 vs. 108±75.5 hours) and its doses (4.8±2 vs. 6.5±3.8 μkg/kg/hour) were significantly lower in the group I, therefore, the patients of the group II stayed almost twice as long in the intensive care department (146±56 vs. 96±49.8 hours), although the total length of hospital stay did not differ significantly (25±3.8 vs. 26±12.3 days). An uncomplicated course of the early postoperative period was observed in 15 (46%) patients. Other 18 (54%) patients had complications: 8 patients (24%) of group I had 9 complications, and 10 patients (30%) of group II had 14 complications. The mean observation period was 48±29.8 months, (6 to 190 months). No deaths were observed during the observation period in 26 patients (79%) of both groups, 4 (12%) patients were lost to follow-up. Total cavоpulmonary anastomosis (Fontan procedure) was performed in 7 patients (58%) of the group II. Hemodynamic indicators in the examined 17 patients (81%) of the group I showed good long-term results that did not require other interventions. In 15% (3 of 20) of the patients of the group I, a high mean pressure in the right atrium of 14±1.8 mmHg attracts attention with dilatation of the inferior vena cava and hepatic veins, and 5 (20%) patients had high mean pressure of 16±2.7 mmHg in the superior vena cava. Transplantation-free survival during the observation period in patients of both groups was 100%.
 Conclusions. 1.5VR is a reasonable alternative to the palliative Fontan strategy in children in whom the anatomic or functional status of the RV is between biventricular reconstruction and the single ventricle pathway and shows good immediate and long-term results.

Patent ductus arteriosus stenting versus modified Blalock-Taussig shunt for palliation of duct-dependent pulmonary blood flow lesions.

Patent ductus arteriosus (PDA) stenting is an alternative to modified Blalock-Taussig shunt (MBTS) as first-stage palliation of duct-dependent lesions. The superiority of one approach over the other is still controversial. Our objective was to compare PDAstent versusMBTS for palliation in regard to safety, efficacy, and efficiency. From 2010 to 2021, 134 patients had first-stage palliation with either PDAstent (n = 83) or MBTS (n = 51). Twenty-seven patients failed the primary treatment and were converted to the other group. The study endpoints were hospital outcomes, interstage reintervention, and concomitant procedures at the second-stage palliation. Patients with PDAstent were significantly younger. The prevalence of antegrade pulmonary blood flow (PBF) was higher in patients who had MBTS and graft thrombosis was higher in the PDAstent. Hospital stay was significantly longer in patients who had MBTS. Predictors of prolonged mechanical ventilation were low-weight, MBTS, and conversion. Intensive careunit stay significantly increased with conversion, low-weight, and antegrade PBF. The interstage intervention was required more frequently in PDA-stent group. Predictors of reintervention were conversion and pulmonary atresia with the intact interventricular septum. Pulmonary artery plasty was required more frequently during the second-stage palliation in PDA-stent group. PDAstent is an alternative to MBTS for first-stage palliation. It is associated with shorter hospital stays and avoidance of surgery at the expense of a high rate of stent thrombosis and interstage reintervention. Conversion increased the risk of the procedure. More studies are needed to determine factors that affect PDA-stent outcomes and patient selection criteria.

Outcome Predictors in Catheter Interventions for Severe Right Ventricular Outflow Tract Obstructions.

Introduction & aim of workTranscatheter treatment for critical pulmonary stenosis and membranous pulmonary atresia has become the gold standard of care in many centers. We aimed at evaluating the predictors of outcome in interventions for treatment of duct-dependent right ventricular outflow tract obstruction with intact interventricular septum.Subjects & methods68 cases with pulmonary atresia with intact interventricular septum (PA/IVS) and 50 cases with critical pulmonary stenosis (CPS), all younger than 3 months of age, were operated during the period of 10 years; excluding patients with tricuspid valve annulus Z-score smaller than −4, evidence of right ventricular-dependent coronary circulation or additional malformations.ResultsAge, weight, body surface area as well as tricuspid & pulmonary valve Z-scores were significantly less in PA/IVS; right ventricular pressure was similar in both groups however procedural success and survival to hospital discharge was higher in the CPS group. Lower age, weight and body surface area were associated with procedural failure. Weight was the only predictor of procedural success; while weight and lower post-procedural right ventricular pressure were independent predictors for survival to hospital discharge. Post-procedural right ventricular pressure and length of stay were less in the CPS group. tricuspid and pulmonary valve annulus Z-scores were the only independent predictors of the post-procedural milrinone duration in PA/IVS.ConclusionWe advocate for the use of larger balloon/pulmonary annulus ratio, to achieve a lower right ventricular pressure not fearing excessive pulmonary regurgitation that might be beneficial for right ventricular growth; and for the combination with ductal stenting in borderline or bipartite right ventricles.

Molecular markers of cardiac fibrosis after myocardial infarction

Aim. To perform a screening for molecular markers of cardiac fibrosis upon myocardial infarction.Materials and Methods. We carried out echocardiography-guided endomyocardial biopsy of affected and intact interventricular septum segments of 7 patients with anterior myocardial infarction. Fibrotic and adjacent intact cardiac tissue was dissected into 2 equal segments and: 1) homogenized with the further RNA extraction, reverse transcription, and quantitative polymerase chain reaction; 2) fixed in formalin and embedded into paraffin with the further van Gieson staining for the histological verification of cardiac fibrosis.Results. We found that the expression of ACTA2, VIM, CTGF, COL1A1, TGFB1, TGFBR1, AGTR1, CCL2 and TNF genes in fibrotic cardiac tissue was ≥ 3-fold higher as compared with the adjacent intact myocardium reflective of active extracellular matrix production by fibroblast-derived myofibroblasts.Conclusion. We have for the first time shown AGTR1, CCL2, and TNF genes as candidates for post-infarction cardiac fibrosis in addition to ACTA2, VIM, CTGF, COL1A1, TGFB1, and TGFBR1 genes.

Young adults after arterial switch operation for transposition of the great arteries in Switzerland: a growing population.

Adults with transposition of the great arteries (d-TGA) after the arterial switch operation (ASO) are an evolving cohort in adult cardiology. We aimed to analyze cardiac function and cardiac events after transition to the adult clinic in Switzerland. Adults with prior ASO enrolled in the Swiss Adult Congenital HEart disease Registry (SACHER) were included. We analyzed initial cardiac anatomy, surgical history, residual lesions and cardiac function at the time of inclusion, as well as cardiac events during follow-up. Patients were classified as complex (with ventricular septal defect) or simple (with intact interventricular septum) d-TGA. The cohort included 149 patients (99 simple d-TGA, 50 complex d-TGA; age 21±3 years; 71% male, follow-up 27 [15-46] months). Prior to inclusion, patients with complex d-TGA had undergone more interventions related to the left ventricular outflow tract (16% vs. 3%, p = 0.01). Functional and cardiovascular status were similar between the groups. Eleven patients (7%) had a total of 19 cardiac events (5 complications and 14 re-interventions) during follow-up. Patients with complex d-TGA had more cardiac-related complications compared to those with simple d-TGA (8% vs. 1%, p = 0.03). The frequency of re-interventions was not statistically different between the two groups (12% vs. 4%, p = 0.07). During follow-up, an increase in QRS duration was observed. Other parameters of cardiac function remained unchanged. The majority of adult ASO patients have normal functional class and cardiac function. Complex anatomy and residual lesions play a key role when regarding the occurrence of cardiac-related complications during follow-up. The role of QRS prolongation over time needs to be investigated further.

Hypoplastic Left Heart Syndrome: Diagnostic Difficulties in Prenatal Period

The article discusses the possible diagnostic errors in the hypoplastic left heart syndrome (HLHS) with mitral atresia and intact interventricular septum. “Atrioventricular canal defects” can be commonly and mistakenly diagnosed the prenatal period in such cases. The aspects and possibilities of differential diagnosis of these defects in the prenatal period are discussed. Ultrasound sign is presented in the article, we have named it “hockey stick with puck” that characterizes the retrograde blood flow in the aortic arch. This sign is diagnosed by color Doppler mapping of the sagittal section of the ductus arteriosus and can serve as a marker of mitral/aortic atresia.

Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum.

Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

Retrograde Transcatheter Perforation in Adult with Pulmonary Atresia and Single Ventricle: A Unique Case of Ventricular Outflow Tract Opening

Radiofrequency-assisted perforation and subsequent balloon dilatation technique is accepted for treating pulmonary stenosis and pulmonary atresia in patients with intact interventricular septum, but its high cost limits the widespread use. Perforation of the atretic pulmonary valve using the stiff end of a coronary wire seems to be an effective alternative to the radiofrequency perforation. We report on a 47-year-old man with a complex cardiopathy including a pulmonary valve atresia, who underwent a partial surgical repair. Due to high surgical risk, a completion could not be foreseen and a percutaneous retrograde perforation of the pulmonary valve was proposed. The perforation was managed by means of BMW wires with stiff tip from the right internal jugular vein because the femoral vein access was not accesible. Following three Armada balloon inflations, an Express stent was properly introduced to keep the ventricular outflow tract (VOT) open. We describe a successful unusual technique of atretic pulmonary valve percutaneous retrograde perforation that can be an efficient alternative when surgery or anterograde percutaneous access are not possible.

Urgent neonatal balloon atrial septostomy in simple transposition of the great arteries: predictive value of fetal cardiac parameters

To investigate the impact of abnormal perinatal loading conditions on cardiac geometry and function in term fetuses and neonates with transposition of the great arteries with intact interventricular septum (simple TGA), and to explore the predictive value of fetal cardiac parameters for an urgent balloon atrial septostomy (BAS) after birth. This was a prospective longitudinal follow-up study of women delivering at term, including both uncomplicated pregnancies with normal outcome and pregnancies affected by fetal simple TGA. Conventional, spectral-tissue Doppler and speckle-tracking echocardiographic parameters were obtained within 1 week before delivery and within the first few hours after delivery. Neonates with simple TGA that required urgent BAS were assessed after the procedure and before corrective arterial switch surgery. Cardiac parameters were normalized by cardiac cycle length, ventricular end-diastolic length or end-diastolic dimension, as appropriate. Fetal and neonatal cardiac parameters were compared between simple-TGA cases and controls, and perinatal changes in the simple-TGA group were assessed. Receiver-operating-characteristics (ROC)-curve analysis was used to assess the predictive value of fetal cardiac parameters for urgent BAS after birth in the simple-TGA group. A total of 67 pregnant women delivering at term were included in the study (54 normal pregnancies and 13 with a diagnosis of fetal simple TGA). Compared with normal term fetuses, term fetuses with simple TGA exhibited more globular hypertrophied ventricles, increased biventricular systolic function and diastolic dysfunction (right ventricular (RV) sphericity index (SI), 0.58 vs 0.54; left ventricular (LV)-SI, 0.55 vs 0.49; combined cardiac output (CCO), 483 vs 406 mL/min/kg; LV torsion, 4.3 vs 3.0 deg/cm; RV isovolumetric relaxation time (IVRT'), 127 vs 102 ms; P < 0.01 for all). Compared with normal neonates, neonates with simple TGA demonstrated biventricular hypertrophy, a more spherical right ventricle and altered systolic and diastolic functional parameters (RV-SI, 0.61 vs 0.43; RV myocardial performance index, 0.47 vs 0.34; CCO, 697 vs 486 mL/min/kg; LV-IVRT', 100 vs 79 ms; RV-IVRT', 106 vs 71 ms; P < 0.001 for all). Paired comparison of neonatal and fetal cardiac indices in the simple-TGA group showed persistence of the fetal phenotype, increased biventricular systolic myocardial contractility and CCO, and diastolic dysfunction (RV systolic myocardial velocity (S'), 0.31 vs 0.24 cm/s; LV-S', 0.23 vs 0.18 cm/s; CCO, 697 vs 483 mL/min/kg; LV torsion, 1.1 vs 4.3 deg/cm; P < 0.001 for all). Several fetal cardiac parameters in term fetuses with simple TGA demonstrated high predictive value for an urgent BAS procedure after birth. Our proposed novel fetal cardiac index, LV rotation-to-shortening ratio, as a potential marker of subendocardial dysfunction, for a cut-off value of ≥ 0.23, had an area under the ROC curve (AUC) of 0.94, sensitivity of 100% and specificity of 83%. For RV/LV end-diastolic area ratio ≥ 1.33, pulmonary-valve-to-aortic-valve-dimension ratio ≤ 0.89, RV/LV cardiac output ratio ≥ 1.38 and foramen-ovale-dimension-to-total-interatrial-septal-length ratio ≤ 0.27, AUC was 0.93-0.98, sensitivity was 86% and specificity was 83-100% for all. Simple-TGA fetuses exhibited cardiac remodeling at term with more profound alterations in these cardiac parameters after birth, suggestive of adaptation to abnormal loading conditions and possible adaptive responses to hypoxemia. Perinatal adaptation in simple TGA might reflect persistence of the abnormal parallel arrangement of cardiovascular circulation and the presence of widely patent fetal shunts imposing volume load on the neonatal heart. The fetal cardiac parameters that showed high predictive value for urgent BAS after birth might reflect the impact of late-gestation pathophysiology and progressive hypoxemia on fetal cardiac geometry and function in simple TGA. If these findings are validated in larger prospective studies, detailed cardiac assessment of fetuses with simple TGA near term could facilitate improvements in perinatal management and refinement of the timing of postnatal intervention strategies to prevent adverse pregnancy outcomes. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Критический порок сердца (атрезия легочной артерии с интактной межжелудочковой перегородкой) в педиатрической практике

Atresia of the pulmonary artery with an intact interventricular septum is a rare critical heart disease (CHD) in which there is no communication between the right ventricle and the pulmonary artery and the life of a newborn depends on the functioning of the open arterial duct. The article presents a clinical observation of a child diagnosed in the first hours of life with Atresia of the pulmonary artery valve. Open arterial duct. Open oval window. Ductus-dependent pulmonary circulation. In the Cardiac Surgery Department, an operation was performed: open valvuloplasty of the pulmonary artery valve and creation of a systemic pulmonary anastomosis in modification. After discharge on the 18th day of life, the patient was under the supervision of a district pediatrician with regular monitoring of clinical and laboratory data and instrumental research methods (ECG, echocardiography, measurement of oxygen saturation) and consultation with a cardiologist and cardiac surgeon. At the age of 2 years 5 months radical surgery was performed: Plastic valve pulmonary artery, tricuspid valve plasty, plasty of the right branch of the pulmonary artery, the elimination of systemic-pulmonary anastomosis, plastic ASD in conditions of artificial circulation. The patient was discharged with recovery. Careful monitoring of the child’s health by a pediatrician, cardiologist and cardiac surgeon allowed preventing the occurrence of complications in the postoperative period and determining the optimal timing of anatomical radical correction of the defect, which led to full recovery and ensured the harmonious development of the girl.

A better approach for left ventricular training in transposition of the great arteries and intact interventricular septum: Bidirectional cavopulmonary anastomosis and pulmonary artery banding.

Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients. Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage-1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re-anastomosis of the superior vena cava to right atrium after an interstage period of 21-30months (stage-2: anatomical repair). Any mortality was not encountered. The left ventricular mass indices increased from 18-32to 44-74g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow-up period was 20months (9-32months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function. Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic-to-pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.

Nursing care for one case of newborn with delayed closure of chest and acute renal injury after Switch operation and underwent Peritoneal dialysis

Objective To summarize the nursing experience of delayed closure of chest with acute renal injury after switch operation and underwent peritoneal dialysis in neonates and to improve the therapeutic effect. Methods To summarize the curative effects and perioperative nursing experience of one case of the complete transposition of great arteries with intact interventricular septum neonate who underwent delayed closure of chest with acute renal injury and peritoneal dialysis after Switch operation under general anesthesia and extracorporeal circulation in November 2017 in our department. Results The child was postponed to close the chest after surgery. Low cardiac output syndrome and acute renal function injury occurred 1 hour after operation. Through monitoring hemodynamic indexes during ICU, the child recovered after timely treatment of low cardiac output syndrome, maintaining stabilization of circulation, diuresis, peritoneal dialysis, keeping water, electrolyte and acid-base balance, nursing care for delayed closure of chest and other related treatment. Postoperative assisted mechanical ventilation time was 168 hours, postoperative ICU hospitalization time was 12 days, and postoperative total hospitalization time was 19 days. Conclusion The infants who have complete transposition of the great arteries and the intact interventricular septum after Switch operation have many complications and rapid changes in the state of illness. Rigorous and meticulous nursing plays a key role in reducing the postoperative complications and improving the achievement ratio of the operation. Key words: Switch operation; Low cardiac output syndrome; Delayed closure of chest; Peritoneal dialysis

Echocardiographic assessment of hemodynamics in fetus with transposition of the great arteries and intact interventricular septum: Impact on immediate post-natal desaturation

Little is known about physiology and distribution of ventricular flows in fetuses with transposition of the great arteries and intact ventricular septum (TGA-IVS), especially regarding the risk for immediate post natal desaturation. Ductal flow is the result of a complex interaction between left and right ventricular outputs as well as pulmonary and peripheral resistances. Longitudinal hemodynamical assessment of 60 fetuses with TGA-IVS was done 3 times during the pregnancy at 18–22, 28–32 and 35–38 weeks of gestation (WG). We measured the size of the inter-atrial defect and calculated left and right ventricular output as well as systolic and diastolic ductal flow. Fetuses were divided into 2 groups depending on their postnatal saturation. Fetuses with postnatal saturation < 65% at the upper extremities were included in group 1. Echocardiographic parameters were compared between the 2 groups. Twenty-two fetuses had a postnatal saturation < 65%. The velocity time integral in the pulmonary veins was higher in group 1 (6.25 vs. 5.09 cm, P = 0.021). There was no difference in left or right ventricular output between the 2 groups at first evaluation but there was one at the second exam with a lower left ventricular output in group 1. The inter-atrial defect was smaller in group 1 all along the pregnancy. Ductal flow was lower in group 1 after 28 weeks. Ductal flow was retrograde in diastole for 54% and 26% of the fetuses in group 1 and 2 respectively at 28–32 WG ( P = 0.006) and for 82% and 11% at 35–38 WG ( P = 0.01). There is a higher pulmonary venous return at 18–22 weeks in fetuses at risk for post-natal hypoxia. Redistribution of ventricular flow occurs at 28–32 weeks with lower left ventricular output and decreased or retrograde diastolic flow in the ductus arteriosus. At the end of the pregnancy, the majority of fetuses at risk for post-natal hypoxia have a retrograde diastolic flow in the ductus arteriosus.