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Atrioesophageal fistula (AEF) formation is a rare but formidable complication after catheter radiofrequency ablation (RFA) for atrial fibrillation. We present four patients who underwent urgent primary intracardiac repair of the left atrium via sternotomy with central cardiopulmonary bypass and early aortic cross-clamp, followed by repair of the esophagus. We believe that this approach represents the safest strategy for addressing this morbid and often fatal complication.

Background Postpneumopnectomy syndrome (PPS) is an extreme rotation and malposition of mediastinum causing dynamic and symptomatic central airway compression, arising after pneumonectomy or more uncommonly, in congenital single-lung physiology. Affected patients present with severe respiratory compromise. Intrathoracic prosthesis placement is an evolving technique in children that mitigate the effects of thoracic dead space. Research Question Assessment of clinical recovery and functional benefit in children undergoing placement of intrathoracic prosthesis following pneumonectomy or in congenital single lung situations. Study Design and Methods Retrospective chart review of patients at Great Ormond Street Hospital from 2010 and 2020 was performed of all patients who underwent intrathoracic tissue expander placement. We summarize the outcomes of twenty-four children, including those with both congenital and postpneumonectomy PPS etiology. Results 24 children who underwent placement of intrathoracic prosthesis for PPS in the study period with median age of 3.5 months and weight of 5 kg. Single lung etiology was congenital in 15 children (6 agenesis, 9 hypoplasia), and postpneumonectomy in 9 children. In seven patients, there was associated long segment tracheal stenosis. Pre-operative ECMO was required in 2 patients, and pre-operative ventilation was required in 12 patients – all of whom had congenital single lung. Intrathoracic prosthesis placement was concurrent with intracardiac repair in 5 patients. There were no operative deaths, but one early postoperative death related to septicaemia. Median follow up was 75 months with 10 patients on continued respiratory support and 3 on nocturnal support with good quality of life. Two children needed reoperations for replacement of prosthesis. Conclusion The use of tissue expanders is within the armamentarium of most plastic surgeons’ practice. We also therefore advocate for a collaborative team approach involving Plastic and Cardiothoracic Surgery for surgical treatment of these patients. This multidisciplinary strategy has improved management of this rare and debilitating condition of PPS, thereby offering significant improvements in general progress of these sick children having single lung physiology. Evidence is still lacking on functional outcomes in these children and further work is necessary to prove that this is indeed achievable.

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis. The primary management strategy for most patients with PAH-CHD is medical therapy, although defect closure is considered in select cases.

Delayed atrioventricular (AV) conduction most commonly occurs in the AV node, resulting from AH prolongation on an intracardiac electrocardiogram and PR prolongation on a surface electrocardiogram. AV conduction may be blocked in a 2:1 manner, with a normal PR interval and wide QRS suggesting infranodal disease, whereas a prolonged PR interval and narrow QRS are more suggestive of AV nodal disease. Block within the His is suspected when there is 2:1 AV block with normal PR and QRS intervals. Complete heart block occurs when the atrial rhythm is totally independent of a junctional or lower escape rhythm.

BACKGROUND The interatrial communication, one of the most frequent congenital heart defects, represents an important intracardiac shunt between systemic and pulmonary circulations. Direction and magnitude of the interatrial shunting depends upon several features, including defect size, shape and location, pressure difference between right and left atrium, and difference in right and left ventricular compliance. METHODS In this review article, the presence or absence of interatrial communication, and its role, have been analyzed, as they can have a critical impact on the cardiovascular physiopathology, and the interatrial communication can prove to be either clinically harmful, useful or indispensable. Accordingly, the utility and role of the interatrial communication in modern congenital, pediatric and adult, disease has evolved, with modification of the indications to close, maintain patency, or create an interatrial communication. RESULTS The interatrial communication and shunting can be manipulated to maximize the oxygen delivery to the tissues, accordingly with the underlying congenital heart defect. While not always relevant to patients with bi-ventricular circulations, this becomes extremely important in children and adults with complex congenital heart defects. CONCLUSIONS With improving long-term survival for the vast majority of congenital heart patients, an advanced understanding of the role and utility of the interatrial communication, and of all the possibilities of its manipulation, is essential to improve the patient outcomes.

INTRODUCTION The Fontan procedure, used to palliate univentricular physiology, eliminates direct venous access to the ventricle and complicates implantable cardioverter-defibrillator (ICD) placement. METHODS AND RESULTS We describe two patients with Fontan palliation who underwent a novel transvenous approach to ICD placement. The approach uses a transvenous bipolar lead placed in a coronary sinus branch for ventricular sensing, and a defibrillation lead placed in the right atrium for atrial sensing and ventricular defibrillation. CONCLUSION Transvenous ICD implantation is possible in some patients with an atriopulmonary Fontan. This approach avoids a redo sternotomy for epicardial leads and excludes the need for lead placement in the systemic circulation.

INTRODUCTION Common arterial trunk with aortic dominance has well-developed bilateral pulmonary arterial arborization without any essential major aortopulmonary collateral arteries (MAPCAs), whereas "solitary" arterial trunk is characterized by collateral arterial supply to all bronchopulmonary segments and absent pulmonary arteries. CASE REPORT We report a term female neonate with common arterial trunk with aortic dominance with confluent pulmonary arteries with a large MAPCA as the sole blood supply to the lower lobe of the left lung. RESULTS Initial diagnostic workup missed this MAPCA from the descending thoracic aorta. It was suspected during surgery due to massive left atrial return and confirmed by cardiac catheterization study in the early postoperative period and treated successfully by unifocalization. DISCUSSION A large essential MAPCA supplying an entire lobe in the common arterial trunk with aortic dominance has not been described. It can present as heart failure and inability to wean off the ventilator in the early postoperative period following intracardiac repair. CONCLUSION This is a case hitherto undescribed and possibly falling outside the well-entrenched classifications of the common arterial trunk and a large MAPCA could be an addition to the list of lesion modifiers. Our experience with this case underlines the importance of a thorough and open-minded approach to the initial imaging assessment of even well-described conditions.

Background Left pulmonary artery (LPA) sling is a rare anomaly characterized by the origin of the LPA from the right pulmonary artery with a course between the trachea and esophagus. It is often associated with airway and cardiac anomalies. Methods This is a retrospective case series of consecutive patients who underwent LPA sling repair (LPASR) at a tertiary care center over a 35-year period with a focus on tracheal and/or LPA reinterventions and survival. Results Between June 1983 and July 2018, 42 patients were identified: isolated LPASR was performed in 16 (38%), LPASR/intracardiac repair in 10 (24%), and LPASR/tracheal repair in 16 (38%). There were 5 (12%) in-hospital and 4 (10%) late deaths. Survival rates (15 years) were as follows: 100% (isolated LPASR), 65% (concomitant intracardiac repair), and 52% (concomitant tracheal surgery). Preoperative intensive care unit (ICU) hospitalization was associated with future intervention on the LPA/trachea (61%, 11/18). The median distribution of blood flow to the left lung post-index surgery was 38%. Freedom from isolated LPA intervention was 100% after isolated LPASR, 93% after LPASR/tracheal surgery, and 69% after LPASR/intracardiac repair. Freedom from isolated tracheal intervention was 92% after isolated LPASR, 73% after LPASR/tracheal surgery, and 78% after LPASR/intracardiac repair. Conclusions ICU hospitalization prior to index surgery may indicate the severity of associated cardiac/tracheal abnormalities as this is associated with increased morbidity and mortality. Patients who underwent LPASR/intracardiac surgery were more likely to undergo isolated LPA intervention and those who underwent LPASR/tracheal surgery were more likely to undergo isolated tracheal intervention.

Background Transvenous permanent pacemaker (PPM) implantation is an available option for Fontan patients with sinus node dysfunction. However, the thrombogenic potential of leads within the Fontan baffle is unknown. Objective The purpose of this study was to compare the clot burden in Fontan patients with a transvenous atrial PPM to those without a PPM and those with an epicardial PPM. Methods This was a retrospective cohort study of all transvenous PPM implantations in Fontan patients followed at our institution (2000–2018). We performed frequency matching on Fontan type and age group. Primary outcome was identification of intracardiac clot, pulmonary embolus, or embolic stroke. Results Of 1920 Fontan patients, 58 patients (median age 23 years; interquartile range [25th–75th percentiles] 14–33) at the time of transvenous PPM implantation and 174 matched subjects formed our cohort. The type of Fontan performed in case subjects was right atrium–pulmonary artery or right atrium–right ventricle conduit (54%), lateral tunnel (43%), and extracardiac (3%). The cumulative incidence of clot was highest in patients with transvenous PPM, followed by patients with epicardial PPM and no PPM (1.2 vs 0.87 vs 0.67 per 100 person-years of follow-up, respectively). In multivariable analysis, anticoagulation and/or antiplatelet therapy were protective against clot and resulted in reduction of clot risk by 3-fold (incidence rate ratio 0.33; 95% confidence interval 0.21–0.53; P Conclusion In a large cohort of Fontan patients matched for age and Fontan type, patients with transvenous PPM had a higher but not statistically significant incidence of clot compared to those with no PPM and epicardial PPM. Patients treated with warfarin/aspirin had lower clot risk.

BACKGROUND Intracardiac blood cysts are an exceedingly rare occurrence in adulthood. Detailed imaging modalities aid in the diagnosis of such incidental lesions and procedure planning. METHODS We report the case of a 51-year-old male accusing dyspnea on exertion as a sole symptom which led to the discovery of multiple cardiac anomalies, namely, severe aortic valve insufficiency on a bicuspid aortic valve, ascending aortic aneurysm, a cystic mass on the tricuspid valve, patent foramen ovale, and an occluded right coronary artery. RESULTS The disorders were managed in a single surgical intervention, the resected mass being confirmed as a blood cyst. CONCLUSIONS Our case presents a unique association of cardiac disorders, including a highly uncommon intracardiac blood-filled cyst, and underlines the importance of multimodality imaging and interdisciplinary approach in the successful management of such complex cases.

Primary cardiac tumors are usually found incidentally on imaging and are much less common than tumors that metastasize to the heart. Cardiac lipomas are benign cardiac tumors that are usually found in the right atrium or left ventricle. Primary intravascular venous lipomas of the great cardiac vessels are extremely rare and there are few reported cases of a lipoma originating from the superior vena cava causing direct compressive intracardiac effects. Here we describe a case of a symptomatic right atrial lipoma originating from the superior vena cava.

Background Quadripolar left ventricular (LV) leads are capable of sensing and pacing the left ventricle from 4 different electrodes, which may potentially improve patient response to cardiac resynchronization therapy (CRT). Objective We measured 3 different time intervals: right ventricular (RV)-sensed to LV-sensed during intrinsic rhythm (RVs-LVs), RV-paced to LV-sensed (RVp-LVs), and LV-paced to LV-sensed (LVp-LVs, between distal [LV1] and proximal pole on a quadripolar LV lead), and assessed their association with CRT response in terms of LV end-systolic volume (LVESV) and a composite benefit index (CBI) comprising LVESV, LV ejection fraction (LVEF), brain natriuretic peptide level, and NYHA class. Methods A CRT-defibrillator system with quadripolar LV lead was implanted in 196 patients (mean age 69 years, mean LVEF 30%, left bundle-branch block [LBBB] 58%). Conduction intervals were measured before hospital discharge. At baseline and 7-month follow-up, echocardiographic and other components of CBI were determined. Results The mean RVs-LV1s, RVp-LV1s, and LVp-LVs delays were 68 ± 38 ms, 132 ± 34 ms, and 99 ± 31 ms, respectively. From baseline to 7 months, LVESV decreased by 17.3% ± 28.6%. The RVs-LV1s interval correlated stronger with CBI (R2 = 0.12, P < .00001) than with LVESV change (R2 = 0.05, P = .006). In contrast, RVp-LV1s did not correlate and LVp-LVs correlated only weakly with CRT response. The subgroup of patients (44%) with LBBB and RVs-LV1s above the lower quartile (≥34 ms) showed the greatest response to CRT. Conclusion The RVs-LVs interval during intrinsic rhythm is relevant for CRT success, whereas RVp-LVs and LVp-LVs intervals did not predict CRT response.

This study was to evaluate the effects of ischemic mitral regurgitation (IMR) on vortex formation and leaflet dynamics using an established porcine infarct model of IMR. Using direct coronary ligation, five animals were subjected to a posterolateral myocardial infarction (MI) followed by an MRI at 12-weeks post MI. MR imaging consisted of 4D time-resolved left ventricular (LV) flow, full coverage 2D LV cine, and high resolution 2D cine of mitral valve dynamics. Five additional naive animals underwent identical imaging protocols to serve as controls. Image analysis was performed to obtain mitral transvalvular flows as well as LV volumes throughout the cardiac cycle. In addition, anterior to posterior mid-leaflet tip distances were measured throughout the cardiac cycle for determination of temporal leaflet dynamics. It was found IMR caused asymmetric vortex ring formation with the anterior vortex having a lower vorticity relative to its posterior counterpart. In contrast, normal ventricles create symmetric and tightly curled vortices in the basal chamber just underneath the mitral leaflets which conserve kinetic energy and aid in effective ejection. IMR animals were also evaluated for leaflet separation and were found to have a greater leaflet opening and achieved peak vorticity and peak leaflet opening later than control animals. In conclusion, this study shows the effects that altered vortex formation, due to IMR, can have on ventricular filling and leaflet dynamics. These findings have important implications for understanding blood flow through the dilated heart and how ring annuloplasty and volume reduction interventions may influence mitral valve dynamics.

INTRODUCTION: Filamin C (FLNC) gene variants associated with atrial cardiomyopathies have not been reported so far. The aim of this study was to assess the genetics of two siblings presenting with recurrent right atrial arrhythmias, severe right atrial dilatation, and skeletal myopathy. METHODS: A family with subjects affected by recurrent atrial arrhythmias and skeletal myopathy was extensively evaluated by the means of electrocardiographic recordings, magnetic resonance, intracardiac high-density mapping, and genetic testing. RESULTS: Two siblings with right atrial arrhythmias and severe right atrial disease were found to be heterozygous carriers of the variant FLNC-c.925G>A p.(Glu309Lys), previously reported as a variant of uncertain significance. Despite the presence of a severe dilatation of the right atrium in both patients, one presented with skeletal muscle myopathy and an atrial arrhythmia refractory to pharmacological and invasive treatment, while the other one did not have any myopathy, and rhythm control was easily achieved by drugs. CONCLUSION: Filamin C missense variant c.925G>A p.(Glu309Lys) is associated with the severe right atrial disease. Considering cosegregation with the disease (PP1 supporting), this variant should be classified as likely pathogenic.

Background Post-vaccination myopericarditis is reported after immunization with coronavirus disease 2019 (COVID-19) messenger RNA (mRNA) vaccines. The effect of inadvertent intravenous injection of this vaccine on the heart is unknown. Methods We compared the clinical manifestations, histopathological changes, tissue mRNA expression, and serum levels of cytokine/chemokine and troponin in Balb/c mice at different time points after intravenous (IV) or intramuscular (IM) vaccine injection with normal saline (NS) control. Results Although significant weight loss and higher serum cytokine/chemokine levels were found in IM group at 1–2 days post-injection (dpi), only IV group developed histopathological changes of myopericarditis as evidenced by cardiomyocyte degeneration, apoptosis, and necrosis with adjacent inflammatory cell infiltration and calcific deposits on visceral pericardium, although evidence of coronary artery or other cardiac pathologies was absent. Serum troponin level was significantly higher in IV group. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike antigen expression by immunostaining was occasionally found in infiltrating immune cells of the heart or injection site, in cardiomyocytes and intracardiac vascular endothelial cells, but not skeletal myocytes. The histological changes of myopericarditis after the first IV-priming dose persisted for 2 weeks and were markedly aggravated by a second IM- or IV-booster dose. Cardiac tissue mRNA expression of interleukin (IL)-1β, interferon (IFN)-β, IL-6, and tumor necrosis factor (TNF)-α increased significantly from 1 dpi to 2 dpi in the IV group but not the IM group, compatible with presence of myopericarditis in the IV group. Ballooning degeneration of hepatocytes was consistently found in the IV group. All other organs appeared normal. Conclusions This study provided in vivo evidence that inadvertent intravenous injection of COVID-19 mRNA vaccines may induce myopericarditis. Brief withdrawal of syringe plunger to exclude blood aspiration may be one possible way to reduce such risk.

OBJECTIVES Intravenous leiomyomatosis (IVL) is a histologically benign but biologically aggressive tumor. This study aimed to summarize the echocardiography and contrast-enhanced ultrasound (CEU) characteristics of IVL to provide a basis for clinical diagnosis and therapy. METHODS Fourteen IVL patients with uterus leiomyoma history (female, 46.4 ± 5.6 years) were enrolled in this study from March 2008 to December 2020 in our hospital. Preoperative imaging examination data were collected, including echocardiography computed tomography data; six patients also underwent CEU. All patients underwent successful resection, confirmed by histopathology. RESULTS Echocardiographic characteristics: The mean sizes of intracardiac parts of IVL tumors were 54.0 ± 17.9 mm (length) and 24.6 ± 9.8 mm (width). IVL tumors exhibited two echocardiography types: isoechoic solid mass (71.4%, 10/14) and anechoic cystic conduits (28.6%, 4/14), with enlargements of the right atrium (57.1%,8/14), right ventricle (1 patient, 7.1%), and inferior vena cava (57.1%, 8/14). About 21.4% of the patients (3/14) had right ventricular dysfunction. Right heart obstruction was observed in 42.8% (6/14) of the patients. CEU characteristics: the solid mass type exhibited an earlier perfusion and lower perfusion intensity than the conduits type. CEU was helpful in determining origins and pathways: from the internal iliac vein (pathway I, 71.4%), from the ovarian vein (pathway II, 14.3%), or both (14.3%). The echocardiographic appearances of the 14 cases were consistent with the features of the resection specimens. CONCLUSION Combined echocardiography and CEU can provide a more valuable information for the diagnosis of IVL and essential basis for treatment.

OBJECTIVES: This first-in-human feasibility study was undertaken to translate the novel low-voltage MultiPulse Therapy (MPT) (Cardialen, Inc., Minneapolis, Minnesota), which was previously been shown to be effective in preclinical studies in terminating atrial fibrillation (AF), into clinical use. BACKGROUND: Current treatment options for AF, the most common arrhythmia in clinical practice, have limited success. Previous attempts at treating AF by using implantable devices have been limited by the painful nature of high-voltage shocks. METHODS: Forty-two patients undergoing AF ablation were recruited at 6 investigational centers worldwide. Before ablation, electrode catheters were placed in the coronary sinus, right and/or left atrium, for recording and stimulation. After the induction of AF, MPT, which consists of up to a 3-stage sequence of far- and near-field stimulation pulses of varied amplitude, duration, and interpulse timing, was delivered via temporary intracardiac leads. MPT parameters and delivery methods were iteratively optimized. RESULTS: In the 14 patients from the efficacy phase, MPT terminated 37 of 52 (71%) of AF episodes, with the lowest median energy of 0.36 J (interquartile range [IQR]: 0.14 to 1.21 J) and voltage of 42.5 V (IQR: 25 to 75 V). Overall, 38% of AF terminations occurred within 2 seconds of MPT delivery (p

Objective A substantial proportion of infective endocarditis (IE) cases are complicated by local invasion. The purpose of this study was to identify patient and disease characteristics associated with local invasion in surgically treated IE patients. Methods This was a nested case–control study. All episodes of IE for patients admitted to Cleveland Clinic from 1 January 2013 to 30 June 2016 were identified from the Cleveland Clinic IE Registry. Patients ≥18 years of age who underwent surgery for IE were included. Among these, cases were those with local invasion, controls were those without. Local invasion, defined as periannular extension, paravalvular abscess, intracardiac fistula or pseudoaneurysm, was ascertained from the surgical operative note. Associations of selected factors with local invasion were examined in a multivariable logistic regression model. Results Among 511 patients who met inclusion criteria, 215 had local invasion. Mean age was 56 years; 369 were male. Overall 345 (68%) had aortic valve, 228 (45%) mitral valve, and 66 (13%) tricuspid or pulmonic valve involvement. Aortic valve involvement (OR 6.23, 95% CI 3.55–11.44), bioprosthetic valve (OR 3.88, 95% CI 2.36–6.44), significant paravalvular leak (OR 3.80, 95% CI 1.60–9.89), new atrioventricular nodal block (OR 3.77, 95% CI 1.87–7.90), infection with streptococci other than viridans group streptococci (OR 7.54, 95% CI 2.42–24.87) and presence of central nervous system emboli (OR 1.85, 95% CI 1.13–3.04) were associated with local invasion. Discussion Intracardiac and microorganism factors, but not comorbid conditions, are associated with local invasion in IE.

Pidlubna A. Assessment of the state of intracardiac hemodynamics and myocardial remodeling in women with gonarthritis, hypertension and overweight. Journal of Education, Health and Sport. 2021;11(07): 185-194. eISSN 2391-8306. DOI http://dx.doi.org/10.12775/JEHS.2021.11.07.017 https://apcz.umk.pl/czasopisma/index.php/JEHS/article/view/JEHS.2021.11.07.017 https://zenodo.org/record/5154010 The journal has had 5 points in Ministry of Science and Higher Education parametric evaluation. § 8. 2) and § 12. 1. 2) 22.02.2019. © The Authors 2021; This article is published with open access at Licensee Open Journal Systems of Nicolaus Copernicus University in Torun, Poland Open Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author (s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non commercial license Share alike. (http://creativecommons.org/licenses/by-nc-sa/4.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. The authors declare that there is no conflict of interests regarding the publication of this paper. Received: 25.06.2021. Revised: 12.07.2021. Accepted: 29.07.2021. ASSESSMENT OF THE STATE OF INTRACARDIAC HEMODYNAMICS AND MYOCARDIAL REMODELING IN WOMEN WITH GONARTHRITIS, HYPERTENSION AND OVERWEIGHT A. M. Pidlubna State Institution «Zaporizhzhia Medical Academy of Post-Graduate Education», Ministry of Health of Ukraine ORCID iD: 0000-0003-2136-8288 Abstract. The problem of comorbidity is one of the most pressing problems of modern medicine. The presence of arterial hypertension and overweight in patients with gonarthritis is associated with an earlier development of target organ damage and subsequent cardiovascular accidents. In order to assess intracardiac hemodynamics and types of myocardial remodeling in women with gonarthritis, hypertension and overweight, depending on their combinations, from 2018 to 2021, a prospective study of 198 patients of the rheumatology department of the «City Hospital № 10» (Zaporizhzhia) was carried out. According to the results of the study, it was found that the presence of comorbid pathology in the form of arterial hypertension and overweight in female patients with gonarthritis negatively affects the contractile function of the heart, which is confirmed by a decrease in the main indicators of systolic function of the left ventricle; the specified combination of diseases leads to the formation of prognostically unfavorable types of left ventricular remodeling. Key words: arthritis; arterial hypertension; overweight; comorbidity; intracardiac hemodynamics.

Iatrogenic superior vena cava syndrome (SVCs) represents an emergent diagnostic entity and its correlation with deep vein thrombosis is extremely rare. Recently, the increased use of indwelling lines, pacemakers and intracardiac devices has led to more cases of SVC syndrome also associated with a higher frequency of DVT. We report an unusual complication in a 74-year-old female, who has been undergoing hemodialysis via CVC for 14 years, who referred at our Emergency Department complaining of shortness of breath, headache, face and neck swelling. She underwent chest Computed Tomography Angiography (CTA), that showed a thrombus extending from the superior vena cava to the azygos and hemiazygos veins. Acute SVCs should be suspected in emergency settings in symptomatic patients with indwelling central lines, catheters and pacemakers. CTA represents an accurate and quick imaging modality for the diagnosis and the assessment of the extension of the thrombus.

Purpose Patients with repaired Tetralogy of Fallot (rTOF) will develop dilation of the right ventricle (RV) from chronic pulmonary insufficiency and require pulmonary valve replacement (PVR). Cardiac MRI (cMRI) is used to guide therapy but has limitations in studying novel intracardiac flow parameters. This pilot study aimed to demonstrate feasibility of reconstructing RV motion and simulating intracardiac flow in rTOF patients, exclusively using conventional cMRI and an immersed-boundary method computational fluid dynamic (CFD) solver. Methods Four rTOF patients and three normal controls underwent cMRI including 4D flow. 3D RV models were segmented from cMRI images. Feature-tracking software captured RV endocardial contours from cMRI long-axis and short-axis cine stacks. RV motion was reconstructed via diffeomorphic mapping (Deformetrica, deformetrica.org), serving as the domain boundary for CFD. Fully-resolved direct numerical simulations were performed over several cardiac cycles. Intracardiac vorticity, kinetic energy (KE) and turbulent kinetic energy (TKE) was measured. For validation, RV motion was compared to manual tracings, results of KE were compared between CFD and 4D flow. Results Diastolic vorticity and TKE in rTOF patients were 4.12¿±¿2.42 mJ/L and 115¿±¿27/s, compared to 2.96¿±¿2.16 mJ/L and 78¿±¿45/s in controls. There was good agreement between RV motion and manual tracings. The difference in diastolic KE between CFD and 4D flow by Bland-Altman analysis was - 0.89910 to 2 mJ/mL (95% limits of agreement: - 1.351¿×¿10-2 mJ/mL to 1.171¿×¿10-2 mJ/mL). Conclusion This CFD framework can produce intracardiac flow in rTOF patients. CFD has the potential for predicting the effects of PVR in rTOF patients and improve the clinical indications guided by cMRI.

Migration of foreign bodies (FB) with the blood flow to the heart is a rare, but very alarming condition as it may lead to life-threatening complications and death. Objects that are larger than 5 mm in diameter and/or irregular in shape are recommended for removal from extra- and intracardiac areas to prevent incurable embolization. Surgical extraction of intracardiac objects is a serious surgical challenge associated with difficulties to operate, during the continuous movement of the heart, and identify the exact FB location. Early diagnosis and timely removal of FBs are crucial treatment factors for this rare case resolution. We report a case of accidental migration of a metal FB object (nail) about 1.0*0.3 cm from the right neck area jugular vein to the right ventricle apex in the heart. The FB localization was accurately detected using Bi-plane transesophageal echocardiography (TEE) with a special comet-tail artifact. TEE provided valuable information before surgery, and the nail was successfully removed through open-heart surgical procedures and cardiopulmonary bypass (CPB). Postoperative tests indicated no complications.

High-frequency QRS (HFQRS) analysis of surface ECG is a reliable marker of cardiac ischemia (CI). This study aimed to assess the response of HFQRS signals from standard intracardiac electrodes (iHFQRS) to CI in swine and compare them with conventional ST-segment deviations. Devices with three intracardiac leads were implanted in three swine in a controlled environment. CI was induced by inflating a balloon in epicardial coronary arteries. A designated signal-processing algorithm was applied to quantify the iHFQRS content before, during, and after each occlusion. iHFQRS time responses were compared to conventional ST-segment deviations. Thirty-three over thirty-nine (85%) of the occlusions presented significant reduction in the iHFQRS signal, preceding ST-segment change, being the only indicator of CI in brief occlusions. iHFQRS was found to be an early indicator for the onset of CI and demonstrated superior sensitivity to conventional ST-segment deviations during brief ischemic episodes.

We reported an exceedingly rare adult case of intracardiac teratoma with a bicuspid aortic valve. A small mass was discovered in the right ventricle of a 37-year-old man by accident after he was referred to our hospital due to chronic chest distress and aggravated palpitation. We performed a surgical exploration with excision of the lesion after a repeat transthoracic echocardiography suggested a space-occupying lesion in the interventricular septum. A mature cystic teratoma was pathologically confirmed. During the half-year follow-up, no symptoms were reported. This case constitutes the first report of the smallest primary intracardiac teratoma in an adult with a bicuspid aortic valve.

A 6-month-old, female, domestic shorthair cat weighing 1.8 kg presented with cardiomegaly seen on radiographs taken at a primary care veterinary center. Echocardiography revealed a single enlarged vessel overriding a ventricular septal defect and severe hypertrophy of the right ventricular free wall. There was no evidence of a pulmonary arterial trunk originating from the heart. The blood flow through the ventricular septal defect exhibited right-to-left shunting. The cat suddenly experienced dyspnea and died at home, and a postmortem examination was performed. A single large vessel was noted leaving the heart, from which the right and left pulmonary arteries arose separately; a main pulmonary artery was absent. There was only one single anomalous coronary ostium that arose from the brachiocephalic artery and divided into two branches. The walls of the extracardiac coronary artery were thick, but neither infarcts nor narrowing was observed within the coronary arteries. There were no abnormalities in the intracardiac coronary artery. These findings revealed a persistent truncus arteriosus with an anomalous coronary artery. A combination of these anomalies might have contributed to the early death of the cat.

Background Papillary fibroelastomas are rare but benign cardiac tumour that are often found on cardiac valvular surfaces. Their clinical manifestations ranging from clinically asymptomatic to substantial complications that are usually secondary to systemic embolism. Multiple theories have been proposed to explain the pathophysiology of its formation. Case presentation We reported a rare case of large papillary fibroelastoma in the right atrium of a young gentleman which was complicated with pulmonary embolism. Transthoracic echocardiography identified a large pedunculated mass measuring 3.4cmX3.4cmX2cm in right atrium with stalk attached to interatrial septum. The intracardiac mass was resected surgically, which revealed papillary fibroelastoma in histology examination. Conclusion Differential diagnosis of intracardiac masses requires clinical information, laboratory tests and imaging modalities including echocardiography. Incidentally discovered papillary fibroelastomas are treated on the basis of their sizes, site, mobility and potential embolic complications. Due to the embolic risk inherent to intraacardiac masses, surgical resection represents an effective curative protocol in treating both symptomatic and asymptomatic right sided and left sided papillary fibroelastomas, with excellent long term postoperative prognosis.

Background Behçet’s disease (BD) is also referred to as vascular BD when it frequently involves the heart and vessels. This study aimed to describe the cardiovascular manifestations in patients with BD and its correlation to disease activity. We conducted a cross-sectional study on 40 patients diagnosed with BD according to the International Criteria for Behçet’s Disease 2014. All the patients were subjected to detailed history taking, full clinical examination, lab investigations, resting electrocardiogram, trans-thoracic echocardiography, and carotid artery duplex for measuring intimal thickness, peripheral arterial and venous duplex, computed tomography pulmonary angiography, and full ophthalmological examination. Regarding the activity of the disease, it was assessed according to the score of Behçet’s Disease Current Activity Form (BDCAF). Results The most common cardiac manifestation was valvular lesion (67.5%) where the most frequently affected valve was the tricuspid valve (27.5%). Although 25% of patients had left ventricular diastolic dysfunction, only 5% had intracardiac masses. Approximately 52.5% of patients had vascular lesion (deep venous thrombosis 45%, arterial involvement 7.5% [as pulmonary artery thrombosis 5% and aneurysm 2.5%]). Increase in intima media thickness (IMT) was observed in 7.5% of patients, while 60% had abnormal lipid profiles. Hypercholesterolemia was the most common lipid abnormality (50%). BDCAF score range was 4–12, which was significantly correlated to multiple cardiovascular parameters as a mitral, tricuspid valve, and vascular venous involvement (p < 0.05), while not significantly correlated to lipid profile (p > 0.05). Conclusion Cardiovascular complications are frequent among patients with BD, even those who are asymptomatic; therefore, these complications must be screened for early detection and proper management.

Objective. To develop a method for predicting the persistence of hemodynamically significant ductus arteriosus in profoundly premature newborns based on the evaluation of clinical data and the results of additional studies. Material and methods. Sixty-nine profoundly preterm newborns were examined including 37 with very low and 32 with extremely low body weight. Clinical and anamnestic data, the results of laboratory radiographic and instrumental examinations were evaluated. The state of the arterial duct, as well as the parameters of central and intracardiac hemodynamics were determined by echocardiography. The criteria for hemodynamically significant open arterial duct (OAD) were the following: duct size greater than 1.5 mm, left-right blood bypass, the presence of retrograde blood flow in the aorta greater than 50 % of the antegrade value. There were 2 groups: comparison (n = 41) children with hemodynamically insignificant ductus arteriosus, main (n = 28) children with hemodynamically significant ductus arteriosus after 72 hours of postnatal life. Results. After 72 hours of the postnatal life, a hemodynamically significant ductus arteriosus is detected in 40.6 % of profoundly premature newborns. The factors of a long-term persistence of ductus arteriosus are as follows: birth at term of gestation less than 27 weeks with a low Apgar score within the first minute of life ( 4 points), a patient has a sharp weakening or absence of reflexes of the newborn, severe muscular hypotonia, signs of peripheral circulation, moist fine wheezing and crepitation in the lungs, strengthening of their images on the radiograph due to the interstitial and vascular components, dilation of the left and right ventricles and the left atrium. The method for predicting the dynamics of the open ductus arteriosus in profoundly premature newborns has been developed. Conclusion. The use of the developed prognostic table makes it possible to identify with an accuracy of 85.7 % profoundly premature newborns with a high risk of persistence of a hemodynamically significant ductus arteriosus after 72 hours of postnatal life.

Ventricular tachycardia and premature ventricular complexes (PVCs) arising from right ventricular outflow tract (RVOT) are the most common type of ventricular arrhythmias (VAs) in patients without structural heart disease. Radiofrequency ablation is now the gold standard of treatment in this setting due to high efficacy rates and optimal safety profile. During the last few years, the pulmonary valve (PV) and the pulmonary artery (PA) have attracted much attention as reliable sites of origin of RVOT-type arrhythmias. In the mean while intracardiac echocardiogram (ICE) has undoubtedly improved our understanding of the cardiac anatomy. Aim of this paper is to provide an illustrated step-by-step guide on how to use ICE with the CARTOSOUND module to visualize and reconstruct 3D shell of the RV, the PV, as well of other contiguous anatomical structures (i.e., the aortic valve and coronary arteries) to perform aware and safe ablation in this region. This article is protected by copyright. All rights reserved.

Background Autologous pericardium is widely used as a plastic material in intracardiac structures, in the pulmonary artery, and in aortic valve leaflets. For aortic valve reconstruction (AVRec) using the Ozaki procedure, it has produced excellent clinical results over a 10-year period. In-body tissue architecture (iBTA), which is based on the phenomenon of tissue encapsulation of foreign materials, can be used to prepare autologous prosthetic tissues. In this preliminary study, we examined whether biosheets can be used as valve leaflet material for glutaraldehyde-free AVRec by subchronic implantation experiments in goats and evaluated its performance compared with glutaraldehyde-treated autologous pericardium for AVRec. Methods Biosheets were prepared by embedding molds for two months into the dorsal subcutaneous spaces of goats. Autogenic biosheets (n = 4) cut into the shape of the valve were then implanted to the aortic valve annulus of four goats for three months without glutaraldehyde treatment. Autologous pericardium (n = 4) was used in four goats as a control. Valve function was observed using echocardiography. Results All goats survived the three-month study period. With biosheets, the leaflet surfaces were very smooth and, on histology, partially covered with a thin neointima (including endothelial cells). Biosheets were more thoroughly assimilated into the aortic root compared with autologous pericardium. Conclusions For the first time, biosheets were used for large animal AVRec. Biosheets could function as leaflets in the aortic position and may have the ability to assimilate into native valves.

OBJECTIVES We compared the clinical outcomes between tricuspid valve detachment (TVD) and non-TVD for ventricular septal defect (VSD) closure in infants METHODS From January 2004 to April 2020, 462 infants RESULTS The median age and body weight at operation were 1.9 months [interquartile range(IQR), 1.4–2.5] and 4.2 kg (IQR, 3.7–4.6). The median follow-up duration was 83.4 months (IQR, 43.5–130.4). After matching, 44 pairs were extracted from each group. There were no significant differences in all-cause mortality (P = 0.176), reoperation (P = 0.172), postoperative morbidities, including residual VSD, aortic regurgitation, atrioventricular block and significant tricuspid regurgitation (TR) (P = 0.346) between group D and group N. However, group D showed significantly less TR progression during follow-up (P = 0.019). CONCLUSIONS In infants

The aimof this study was to summarize, systematize, and extend analysis of data of two-year clinical use of intracardiac ECG (ECG-CS) in patients with coronary artery disease.Material and methods.131 patients with coronary artery disease who underwent a planned coronary stenting were included in a prospective study from 2018 till 2020 . Ischemia was monitored with the use of electrophysiological recording system with the following settings: combining electrodes into fi ve cathode-anode pairs with a frequency range of 30–500 Hz, signal cut-off of 1 cm, and an amplitude of 0.3 mV/cm.Results.According to the ROC-analysis, the sensitivity of the ECG-СS method in case of ST segment depression was 79.5%, the specifi city was 100%; with ST segment elevation — 83% with a specifi city of 100%, according to data obtained in 88 patients with single-vessel disease of the coronary arteries (CA). Out of 43 patients with coronary artery disease, compromise of the diagonal branch and signifi cant ischemic dynamics (p < 0.05) in the intracardiac lead CS 3–4 after stent placement in the ADA according to ECG-CS was observed in 11 (25.6%) patients, in 9 (20.9%) of which successful provisional stenting was performed, and 2 (4 .6%) patients required implantation of the second stent.Conclusion.ECG-CS is a promising method for detecting myocardial ischemia in patients with coronary artery disease and allows to optimize intervention in the area of coronary artery bifurcation.

Bone is a frequent site of metastases in advanced cancers including lung, breast, prostate, kidney, or myeloma. Lesions are commonly located on the spine. Neoplastic invasion of the vertebral body can result in painful vertebral fractures, leading to disability and substantial morbidity. Percutaneous vertebroplasty is a minimally invasive surgical procedure used to treat spinal fractures due to osteolytic tumors. It could result in pain reduction or resolution in 80–90% of patients with fractures, and it improves stability. Although considered safe, vertebroplasty has been associated over the years with life-threatening complications. We have reported the case of a 55-year-old patient with lung adenocarcinoma, who underwent vertebroplasty for a pathological neoplastic fracture of L2. The procedure was complicated by a leak of cement into the systemic venous circulation, characterized by an 11-cm filament in the right heart chambers and multiple pulmonary emboli. To our knowledge, only one similar case was previously reported, involving an intracardiac cement filament longer than 10 cm. The data are scant, hence the importance of collecting and reporting possible complications about what is perceived as a rather safe procedure. The case highlights the need for a robust postprocedure imaging plan to detect complications, which can impact patients’ morbidity and survival.

Coronary artery fistula (CAF) are a major coronary anomaly which involve one or more coronary arteries abnormally communicating with one of the cardiac chamber or the great vessels adjacent to the heart. The incidence of CAF is 0.05–0.9% in general population and the majority are congenital. Acquired CAF are less common and may occur after cardiac surgery or catheterism, chest trauma or disease related. We present a case of a 6-month-old male infant who underwent intracardiac repair for Tetralogy of Fallot. During the immediate postsurgical period, a CAF from the left coronary artery to the right ventricle (RV) was detected. Spontaneous closure occurred one month after the initial diagnosis. Resumen: Las fístulas arteriales coronarias (FAC) son una malformación coronaria mayor que consiste en una o más arterias coronarias anormalmente comunicadas con una de las cámaras cardíacas o de los grandes vasos adyacentes al corazón. La incidencia de las FAC es de 0.05-0.9% en la población general, y la mayoría son congénitas. Aquellas adquiridas son menos comunes y pueden aparecer tras cirugía cardiaca o cateterismo, traumas torácicos o en algunas patologías. Presentamos el caso de un lactante de 6 meses al que se realizó cirugía de Tetralogia de Fallot mediante técnica de reparación valvular. Durante el postoperatorio inmediato se detectó una fístula coronaria desde la arteria coronaria izquierda al ventrículo derecho. Un mes después del diagnóstico tuvo lugar su cierre espontáneo.

Although a patent foramen ovale (PFO) is relatively common, confirmed reports of thrombus entrapped within a PFO are uncommon. Management of impending paradoxical embolism (IPE), also called a thrombus in transit, lacks consensus but includes systemic anticoagulation (e.g., heparin), systemic thrombolysis, or surgical thrombectomy. We present a case of IPE diagnosed with intraoperative transesophageal echocardiography (TEE) as well as a novel en bloc approach to atrial septal aneurysmectomy to minimize embolism and facilitate repair of the interatrial septum. Timely use of intraoperative TEE may aid in diagnosis and help guide the surgical approach to minimize embolic risk with an IPE.

Background Ultralow temperature cyroablation (ULTC) is designed to create focal, linear, and circumferential lesions. The aim of this study was to assess the safety, efficacy, and durability of atrial and ventricular ULTC lesions in preclinical large animal models. Methods and results The ULTC system uses nitrogen near its liquid-vapor critical point to cool 11-cm ablation catheters. The catheter can be shaped to specific anatomies using pre-shaped stylets. ULTC was used in 11 swine and four sheep to create atrial (pulmonary vein isolation and linear ablation) and ventricular lesions. Acute and 90-day success were evaluated by intracardiac mapping and histologic examination. Cryoadherence was observed during all ULTC applications, ensuring catheter stability at target locations. Local electrograms were completely eliminated immediately after the first single-shot ULTC application in 49 of 53 (92.5%) atrial and in 31 of 32 (96.9%) ventricular applications. Lesion depth as measured on histology preparations was 1.96 ± 0.8 mm in atrial and 5.61 ± 2.2 mm in ventricular lesions. In all animals, voltage maps and histology demonstrated transmural and durable lesions without gaps, surrounded by intact collagen fibers without injury to surrounding tissues. Transient coronary spasm could be provoked with endocardial ULTC in the left ventricle in close proximity to a coronary artery. Conclusions ULTC created effective and efficient atrial and ventricular lesions in vivo without procedural complications in two large animal models. ULTC lesions were transmural, contiguous, and durable over 3 months.

Primary cardiac tumors are extremely rare. Obtaining a tissue diagnosis is difficult and commonly requires open-heart surgery with associated morbidity. Esophageal endoscopic ultrasound (EUS) and EUS with the EBUS scope (EUS-B) provide real-time sampling of centrally located lung tumors and mediastinal lymph nodes. They also provide an excellent view of the left atrium, since it is located adjacent to the esophagus. To date, left atrium tumor diagnostics by endosonography is poorly explored. We describe 2 exceptional diagnostic cases of left atrium tumors in which cardiac surgery was hazardous due to the clinical condition or previous surgical interventions. During EUS-B-guided fine-needle aspiration (FNA), the left atrial masses were successfully and safely sampled, revealing a Burkitt lymphoma and a synovial sarcoma. FNA including cell block analysis enabled specific tumor diagnosis and molecular subtyping. Our findings suggest that in selected cases, linear endosonography qualifies as a minimally invasive technique for intracardiac tumor diagnostics.

Introduction: Cardiac myxomas represent the most frequent forms of primary tumors of the heart. The most frequent location is the interatrial septum. We report the clinical case of a myxoma of the left atrium and discuss its epidemiological and therapeutic aspects through a review of the literature. Observation: This was a 41-year-old female patient who presented with sudden rotational dizziness associated with vomiting. MRI revealed multiple punctiform bilateral supra and subtentorial strokes of different ages, recent and semi-recent, suggesting an embologenic etiology. Transesophageal echocardiography found a large pedunculated, homogeneous, avascular tumor hanging from the interatrial septum. The patient is operated on urgently under cardiopulmonary bypass for resection of a large tumor located in the left atrium. The pathological examination concluded with the diagnosis of myxoma of the left atrium. The postoperative follow-up was straightforward and the patient was discharged from the hospital via home hospitalization. Conclusion: The diagnosis of cardiac myxomas is suspected in the presence of symptoms associated with echocardiographic images of intracardiac masses and confirmed by histological study. Embolic accidents are one of the formidable complications of myxomas. Surgical management is urgent, especially in the presence of predictive morphological features of embolism on echocardiography.

Objectives : Adult patients undergoing tetralogy of Fallot (TOF) repair have a higher risk of mortality compared to pediatric patients. Pulmonary regurgitation (PR) further predisposes these patients to heart failure, arrhythmias, and sudden death. Pulmonary valve replacement (PVR) may improve the symptoms in these patients but, fails to reverse the other deleterious effects. Aim of our study was to evaluate the effect of concomitant PVR with TOF repair on right ventricular (RV) parameters, cardiopulmonary exercise capacity, and bioprosthetic valve durability at mid-term. Materials and Methods: Between January 2013 and August 2018, 37 adolescents and adults with TOF who had hypoplastic pulmonary annulus underwent concomitant TOF repair with PVR at our institute. We retrospectively collected the data from the hospital records including follow-up. Results : Mean age of the patients was 18.48 ± 7.53 years. Bioprosthetic valve size ranged from 19 mm to 25 mm. There was no early or late mortality. No patient had developed significant perioperative complications. At a mean follow-up of 53.3 ± 16.4 months, there was no significant change in mean QRS duration, RV function, RV end-systolic and end-diastolic dimensions, RV myocardial performance index, and functional status (including NYHA class and 6-min walk test) compared to at-discharge values. Four patients developed prosthetic valve degeneration with mild PR and without significant increase in gradient. Conclusion : Concomitant PVR with TOF repair in adult provides excellent mid-term outcome, with a minimal rate of pulmonary valve degeneration. It not only eases the early postoperative course but also preserves the RV function as well as functional status at mid-term.

Angiomyolipoma is the most common benign mesenchymal neoplasm of the kidney. Despite its benign nature, it may, in rare instances, invade the inferior vena cava and right cardiac chambers. A tumor thrombus in the pulmonary artery is an extremely rare but potentially fatal complication. We report a rare case of 53-year-old woman showing a right renal angiomyolipoma with intracardiac extension and pulmonary embolism. The patient underwent open radical right nephrectomy and thrombectomy of the inferior vena cava and pulmonary artery. Our case highlights that close attention to these complications is important to determine the surgical plan.

Lead fracture is a serious complication of a pacemaker; its prevalence is 0.14.2%. The common site of lead fracture is at the space between the clavicle and the first rib. The causes are intense physical activity, which approximates the clavicle to the rib and compression of the lead, chest trauma, anatomical features, twiddlers syndrome. Diagnosis of lead fracture is can be made by electrocardiography there is a transient or permanent stimulation/sensitivity disturbance. When the programmer interrogates the pacemaker, a significant sign is an abrupt rise in the lead impedance, although cases of fracture with normal impedance values have been reported. The article presents an extremely rare clinical case of an intracardiac lead fracture in a 28-year-old patient. At the initial implantation, leads were passed through the accessory left superior vena cava, resulting in a loop in the right ventricle. The patient himself was subjected to increased physical activity. The question of the need to remove such leads remains open. Some authors note that the distal end is firmly fixed to the heart wall, and therefore does not expose the patient to a vital risk. Others consider that the lead can become a source of thrombus formation, or fragmentation with embolism in the pulmonary circulation can occur. In our case, the causes of the fracture were probably an intense physical activity and bending of the lead inside the right ventricle. The clinical situation was discussed with cardiac surgeons of the federal centers of cardiovascular surgery. Given the high risks of open-heart surgery, it was decided to refrain from removing the broken lead, and the patient was provided with atrial pacing.

Pulmonary atresia (PA) with intact ventricular septum is a rare, heterogeneous congenital heart defect with varying degrees of right ventricular and tricuspid valve hypoplasia and wide spectrum clinical features. Initial treatment at presentation is to establish systemic to pulmonary shunt or ductus stenting. The treatment options i.e. biventricular, one and half ventricular or one ventricular repair are often dictated by the degree of development of the tricuspid valve and right ventricle. X, 11 years old teenage girl got admitted to Combined Military Hospital (CMH) Dhaka at 5 months of age with severe cyanosis, respiratory distress, acidosis and shock. After immediate stabilization and work up, she was diagnosed as pulmonary atresia with intact ventricular septum, flapped atrial septal defect (ASD) and a small patent ductus arteriosus (PDA). On urgent basis, stenting of PDA done with 3.5 X 11 mm coronary stent at cardiac catheterization laboratory. Her SPO2 stepped up to 90 % from base line SPO2 from 50% following procedure. At 15 months of age she underwent intracardiac repair with short trans annular patch and ASD left open as a vent for RV. On follow up her ASD was getting larger and became desaturated and symptomatic. She was followed up for the right ventricular (RV) and tricuspid valve growth and ASD shunt. As her RV was developing nicely during follow up and achieved TV z score –1, ASD device closure was done with 18 mm Amplatzer ASD device at 5 years of age. She is now asymptomatic and thriving well. Cardiovasc. j. 2020; 13(1): 81-85

Background Increasing number of symptomatic patients with severe aortic stenosis is treated with transcatheter aortic valve implantation (TAVI). Stroke is one of the most serious complications of TAVI, and the majority of cerebral events in patients undergoing TAVI have an embolic origin. Case summary A 90-year-old female underwent trans-femoral TAVI for symptomatic severe aortic stenosis. Just before the implantation of the transcatheter heart valve (THV), transoesophageal echocardiography (TOE) showed a mobile, high-echoic mass attached to the THV, which gradually enlarged to 26 mm, then spontaneously detached from the THV and flowed up the ascending aorta, disappearing from the TOE field of. After the procedure, the patient presented with ischaemic stroke. The patient’s stroke was thought to have resulted from the embolism migrating to the distal cerebral arteries. Discussion The detailed images acquired with TOE during TAVI enabled the prompt identification of the unusual intracardiac mass.

A late preterm infant had pulmonary hypertension caused by a variety of mechanisms leading to complex management. This child had complete atrioventricular septal defect associated with mild left ventricular hypoplasia and Down syndrome diagnosed prenatally. The mother had been treated by antiretroviral HIV treatment during pregnancy. Aortic coarctation was diagnosed and rapidly repaired. After surgery, he required noninvasive ventilation for persisting elevated PCO2. Pulmonary CT scan showed normal bronchial tree, lung parenchymal abnormalities with mosaic aspect and hyperlucent zones, and indirect signs of lung hypoplasia with peripheral microbubbles. During follow-up, severe pulmonary hypertension was diagnosed on echocardiography without recoarctation, significant intracardiac shunting or diastolic dysfunction. The patient died after four months unable to be weaned from noninvasive ventilation. Post mortem lung biopsy showed abnormally muscularized arterioles with intimal fibrosis and pulmonary immaturity. Gentetic screening identified a BMPR-2 mutation. This patient illustrates the multifactorial origin of pulmonary hypertension in the neonatal period. The respective contribution of left-to-right shunt, post-capillary obstruction, and abnormally elevated pulmonary vascular resistances led to perform right heart catheterization to exclude excessive shunting and restrictive physiology of the left heart. Subjects with Down syndrome are also highly susceptible to decreased lung vascular and alveolar growth, which may increase the risk for pulmonary hypertension and lung hypoplasia. This case highlights two issues. The first one is that right heart catheterization should be discussed in neonates with unexplained pulmonary hypertension and the second is to extend indications of genetic testing for pulmonary hypertension genes in neonates who have unusual course of neonatal pulmonary hypertension, particularly in the setting of associated congenital heart disease (CHD).

The diagnosis of paradoxical emboli remains elusive in many cases. The causal association between the thrombotic source, the intracardiac shunt, and the final emboli location is seldom demonstrated. We present the case of a 42-year-old woman admitted to the hospital with a third stroke. The presence of a thrombus in transit through a patent foramen ovale (PFO), a deep vein thrombosis (DVT), bilateral pulmonary emboli, and an acute cerebral infarct were concurrently documented.

Background Endometrial stromal sarcoma (ESS) is a rare malignant mesenchymal tumor. Early in the disease, the findings on magnetic resonance imaging are similar to those of leiomyoma. When the lesion involves both vascular and cardiac tissue, it might be misdiagnosed as intravenous leiomyomatosis, which is not common in the clinic. Case summary We present the case of a 34-year-old female patient with tumor embolus, which extended from the right iliac vein and ovarian vein to the inferior vena cava (IVC), and then to the right atrium and right ventricle, and finally protruded into the pulmonary artery. The patient had undergone a hystero-myomectomy 7 years previously. Based on the findings of the imaging examinations, the diagnosis of intravenous leiomyomatosis was considered preoperatively. The patient then underwent complete resection of the endovascular and intracardiac tumor embolus. The postoperative pathology results confirmed metastatic ESS with endovascular and intracardiac involvement. The patient was discharged from hospital in good condition, and there was no sign of recurrence 5 mo after the operation. Conclusion Extending from the iliac vein and ovarian vein to the IVC, this metastatic ESS invaded both vascular and cardiac tissues. For patients with ESS involving vascular and cardiac tissues, pathological examinations are essential for the differential diagnosis, such as intravenous leiomyomatosis. In addition, due to the high recurrence rate of ESS, long-term and close follow-up evaluation is necessary.

Objective — to perform the comparative analysis of fibroblast growth factor (FGF)- 2 levels and basic echocardiographic parameters (ECP) in patients with mitral valve prolapse MVP, type 1 diabetes mellitus (DM1) and their combination, depending on the presence of signs of structural and functional changes in the heart valve apparatus and remodeling of the left ventricular (LV) myocardium.Materials and methods. The examinations involved 86 people aged 18 to 33 years (54 (56.8 %) women and 41 (43.2 %) men), including 24 people with MVP without concomitant pathology; 29 patients with MVP and DM1; 33 patients with DM1 without MVP. The control group included 22 practically healthy people matched by age and gender. The establishment of the diagnosis of MVP implemented by echocardiographic criteria L. Freed et al. (2002). Evaluation of the concentration of FGF2 implemented in blood plasma by the enzyme immunoassay (Quantikine reagent kit (Human FGF basic Immunoassay).Results. In patients with MVP and DM1, the higher values of the index of the relative LV wall thickness have been reveled in comparison with the groups of patients with monomorbid MVP and DM1. The presence of MVP, both monomorbid and in combination with type 1 diabetes, was accompanied by an increase in the FGF 2 levels compared to the control: with isolated MVP, it was higher, respectively, by 25 %, with comorbid pathology it was more than 1.5 times higher than the control values.Conclusions. The most significant differences in the indices of intracardiac hemodynamics and FGF 2 compared with control were observed in patients with combination of MVP and type 1 diabetes, which may indicate the influence of diabetic metabolic processes on further structural changes in the mitral valve CT, LV myocardium, the marker of which is FGF 2. Less difference in the FGF-2 levels was revealed between the patients with isolated mitral valve prolapse and control group.

We present an unusual case of atrial septal defect and ventricular septal defect with a vascular ring formed by a right-sided aortic arch with an aberrant left subclavian artery that gave rise to a patent ductus arteriosus connecting to the main pulmonary artery. We performed a single-stage repair of the intracardiac defects and division of vascular ring with a sternotomy instead of the traditionally practiced dual approach. This included division and reimplantation of the aberrant left subclavian artery to the left carotid artery after transection. This approach has not been described so far.

The prevalence of anomalous origin of coronary artery in tetralogy of Fallot has been reported to be around 4% to 6%. The association of high take-off of the right coronary artery from the distal part of the ascending aorta in tetralogy of Fallot in the presence of a partial anomalous pulmonary venous connection (PAPVC) to the left superior caval vein draining into the left atrium is not known to the best of our knowledge. We herein describe such a case when the anomalous right coronary artery and the PAPVC were detected incidentally during intracardiac repair; signifying the importance of a thorough assessment of the anatomy before surgery.

We present an unusual intracardiac mass posing a diagnostic dilemma. A middle-aged male patient was referred for workup of a symptomatic cardiac mass involving the mitral valve. Multimodality imaging consisting of cardiac magnetic resonance (CMR) imaging and 18F-fluorodeoxyglucose positron emission computerized tomography (18FDG-PET) scan was utilized to further characterize the mass after initial echocardiographic identification. CMR imaging identified extent of valvular mass into the interatrial septum and basal portion of the interventricular septum. On 18FDG-PET scan, the intracardiac mass was found to be metabolically active. It also revealed the presence of FDG avid lymph nodes in the abdomen. Histology of the lymph node revealed active granulomatous inflammation suggestive of tuberculosis. Treatment with antitubercular therapy resulted in resolution of the mass and mitral regurgitation, avoiding surgery.