bluuebunny/crossref_metadata_2025_split huggingface dataset:
P1448 Thoracic aorta and circulating transforming growth factor-b in marfan syndrome and marfanoid habitus
Authors: Malev E , Reeva S , Timofeev E , Luneva E 
Published: January 2020

Abstract

Purpose
Current understanding of the pathogenesis of thoracic aortic aneurysm (TAA) in Marfan syndrome (MS) focuses upon abnormal activity of the transforming growth factor beta (TGF-β) signalling pathway. Circulating TGF-β predicts cardiovascular events in patients with MS and is elevated in the entire spectrum of aortic syndromes. Marfanoid habitus (MH) patients not meeting the MS criteria (TAA, ectopia lentis, family history), but share the same skeletal features and are the part of the Marfan continuum. Our aim was to evaluate the possible role of elevated TGF-β level in the aortic dilatation at mid-term follow-up in Marfanoid habitus patients.

Methods
33 consecutive patients with a presumptive clinical diagnosis of Marfan syndrome were referred to Almazov centre and enrolled in our observational, prospective, single-center study. Nine of them (mean age 27.9 ± 9.3) fulfilled diagnosis of MS according to revised Ghent criteria. 24 subjects (mean age 21.8 ± 3.4) with skeletal features of Marfanoid habitus have had no major findings of MS. Proximal aortic segments were visualized in the parasternal long-axis and suprasternal views. Concentration of TGF-β1 and TGF-β2 in serum was determined using a test system Human Platinum ELISA. End points analyzed during 5 years of follow-up were mortality, aortic-related events, and aortic dimension changes.

Results
During 122 person-years of the follow-up (median 5.1 years) no deaths or aortic-related events occurred in Marfanoid habitus patients.
TGF-β1 and TGF-β2 serum levels were elevated in patients with Marfanoid habitus (14.2 ± 27.6 and 2.1 ± 1.7 ng/ml, respectively) but were lower than in MS group (44.6 ± 47.3 ng/ml, p = 0.03 and 2.7 ± 1.7 ng/ml, p = 0.39, respectively). A high TGF-β1 serum level (cutoff &gt;14.75 ng/ml, provided by the manufacturer of our TGF-β assay) was detected in 44% and TGF-β2 (&gt;2.0 ng/ml) in majority patients (67%) of the MS group. In Marfanoid habitus group we found a high TGF-β1 serum level only in 4 (17%) patients and TGF-β2 in 9 (38%) patients. Aortic diameter at the sinuses of Valsalva and Z-score were significantly lower in Marfanoid habitus group (29.2 ± 2.8 mm and 1.56 ± 0.93) than in MS patients (43.1 ± 15.1 mm, p = 0.0007 and 6.86 ± 5.83, p = 0.004) at the beginning of study and significantly increased during the follow-up (31.3 ± 2.9 mm and 1,69 ± 0,15, p &lt; 0.001 for both).
There was no correlation between TGF-β level and aortic dimensions in patients with MS and marfanoid habitus.

Conclusion
In young adults with Marfanoid habitus and the current absence of ascending aortic aneurysm we found the increased TGF-β level and aortic root enlargement during the follow-up. High TGF-β serum level may contribute to the excessive progression of aortic dilatation later over mid-to-late aging and requires further investigation to establish its role in the aortic aneurysm pathogenesis.
P1449 CMR-driven computational modeling of right ventricular flow dynamics
Authors: Capuano F , Romano L , Loke Y H , Dellegrottaglie S , Notorio M , Cocchia R , Ranieri B , Ferrara F , Contaldi C , Mirto G , Coppola G , Mauro C , Balaras E , Bossone E 
Published: January 2020

Abstract

Introduction
The analysis of intracardiac blood flow patterns can significantly contribute to improve the understanding and treatment of cardiovascular disease. In contrast to the substantial literature on the left side of the heart, there is currently a significant lack of knowledge about the fluid mechanics of the right heart – pulmonary circulation unit (RH-PCU), both in healthy and diseased conditions.

Purpose
It is conjectured that computational modeling can be a key element to enhance current imaging techniques and provide quantitative insights into the unique RH-PCU biomechanics. Here we present a novel methodology that allows personalized numerical simulations of right heart flows, through a proper combination of cardiac magnetic resonance (CMR) with computational fluid dynamics (CFD).

Methods and results
We developed a patient-specific pipeline from medical images to computational models, as depicted in the figure. First, the RV geometry is reconstructed from time-resolved CMR cine images, comprising short-axis and longitudinal slices of the heart, where feature-tracking techniques are used to extract the motion of the RV endocardium contours. A time-continuous description of the moving geometry is obtained through an image-registration algorithm based on diffeomorphic mappings. The moving model of the RV, including the outflow tract and proximal pulmonary arteries, is finally fed to a dedicated CFD solver. The tool is able to provide a detailed description of the velocity and pressure fields inside the right ventricle and proximal pulmonary arteries during all phases of the cardiac cycle. From these fields, global hemodynamic quantities such as vortex properties, kinetic energy, pressure gradients and hemodynamic forces can be computed.

Conclusions
CMR-driven computational modeling of intra-ventricular flow enables a promising approach for understanding and evaluating the biomechanical environment of the right heart. This high-fidelity framework can be applied to investigate the RV response and adaptation to abnormal pressure and/or volume load conditions. It can also be used to reproduce the virtual flow that would realize in hypothetical conditions, and therefore adds predictive capabilities to modern flow imaging. The analysis may allow to determine an association between blood flow patterns and disease progression, and ultimately lead to derive and validate imaging biomarkers of clinical significance.
Abstract P1449 Figure. Pipeline for patient-specific modeling
P1451 Massive fatal venous thrombosis
Authors: De La Torre Carpente M M , Redondo Bermejo B , Perez Sanz T M , Acuna Lorenzo M A , Revilla Martinez M I , Tapia Ballesteros C , Guillem Ares E S , Munoz Fuster A C , Rollan Gomez M J 
Published: January 2020

Abstract
We present the case of a 63-year old woman with previous history of high-grade liposarcoma. of the lower extremity. She had been treated with radiotherapy and chemotherapy and then she underwent surgical treatment with wide local excision several months ago. She was awaiting a new surgical procedure to remove a right suprarrenal metastasis.
The patient presented with lower extremity edema and abdomen and increasing weight in the previuos week.
A thoracic and abdominal CT showed the suprarrenal mass had become of greater size. Enlargement of superior vena cava, partial filling defects in several segments of the right lung suggestive of acute pulmonary embolism. Extensive thrombosis from right iliac vein, common iliac vein, intrahepatic cava vein, inferior vena cava, right atrium and right ventricle. Bilateral pleural effusion and ascites.
A transthoracic echocardiogram revealed a big mass (6.1 cm) in the right atrium prolapsing into the right ventricle. There was a mean diastolic gradient of 3 mmHg and maximal gradient of 6 mmHg in the tricuspid valve. Left ventricle systolic function was moderately depressed due to abnormal movement of the interventricular septum suggestive of pulmonary hypertension.
The clinical course was characterized by rapid deterioration and the patient died from cardiogenic shock.
The source of thrombi in the right side of the heart most of the times is venous thrombi that have embolized. Cancer patients have an increased risk of venous thromboembolism compared with the general population. The risk varies depending on the type and the stage of the cancer. Metastatic disease has the highest risk. Most clinically significant pulmonary embolisms originate as venous thromboembolism in the lower extremities or pelvic veins. However in most of the cases it is difficult to image the thrombus "in-transit". In this case the most striking feature is not imaging the thrombus "in-transit" but its massive size.
Abstract P1451 Figure. liposarcoma Euro Echo 2019
P1456 Pacemaker and percutaneous aortic valve endocarditis - a complex decision
Authors: Cardoso A , Dias G , Von Hafe P , Faria B , Fernandes M , Azevedo O , Leite S , Machado I , Lourenco A 
Published: January 2020

Abstract

CASE REPORT
We describe the case of a 64-year-old man who was referred because of a fever. He had history of hypertension, dyslipidemia, atrial fibrillation, chronic obstructive pulmonary disease and a right lung pneumectomy due to epidermoid lung cancer diagnosed twenty-five years ago. He implanted dual-chamber definite pacemaker ten years ago and had a transcatheter aortic valve implantation (TAVI) in the previous year due to severe aortic stenosis and a high surgical risk.
He was hospitalized 2 months ago because of a respiratory infection. Blood cultures were positive for an Enterococcus faecalis. A transthoracic echocardiogram was performed and had no evidence of vegetations.
After one month, he had recurrence of fever and was again admitted. On physical examination he was hemodynamically stable, with signs of peripheral congestion and no heart murmur on cardiac auscultation. Blood cultures were again positive for Enterococcus faecalis. The transesophageal echocardiogram (TEE) showed a biologic aortic valve with thickened leaflets and small nodular structures suggestive of vegetations. There was a small periprosthetic leak and no obstruction or suspected perivalvular abscesses. There was also a small vegetation with 0,45*0,3 cm in the auricular pacemaker lead. A thoraco-abdomino-pelvic computed tomography scan showed no embolic complications. A diagnosis of pacemaker and TAVI endocarditis was made. The patient started directed antibiotic therapy (ceftriaxone and ampicillin).
The case was discussed in Heart Team and because of high surgical risk, medical treatment was decided. The patient completed antibiotic treatment with negative blood cultures and apyrexia. A repeated TEE showed persistence of vegetations, without development of local complications.
It was decided to discharge the patient under palliative suppressive antibiotic treatment with levofloxacin after discussion with the infectious disease doctor. In a follow-up evaluation, he remained clinically stable, without recurrence of fever.

DISCUSSION
As TAVI procedures are performed more frequently, a higher number of late complications are expected. Prosthetic valve endocarditis after TAVI is a complex situation, whose treatment strategy is not well-defined, particularly because these patients are usually of high surgical risk. This case describes a complex clinical picture and highlights the difficulty in decision-making in these situations. Also, it pretends to reinforce the need to discuss in a Heart Team the best treatment options.
P1460 Atrial myxoma with atypical location in an asymptomatic patient
Authors: Shirka E , Doko A , Paparisto V , Osmenaj R , Gjergo H , Demiraj A , Kacani A , Prendi B , Beqiraj A , Rrapo A , Goda A 
Published: January 2020

Abstract

Introduction
Primary cardiac tumours are rare. Most of them are benign, among which myxomas are the most common. Usually they occur in the left atrium (75%) but there are cases of right atrial myxomas. The majority of patients with atrial myxoma present with one or more symptoms of clinical triad of embolic events, intracardiac obstruction, or nonspecific manifestations. We report a rare case of cardiac myxoma arising from the right atrium as an accidental finding during routine medical checkup.

Case report
A 52 years-old woman was admitted to ambulatory care for a general checkup. At presentation, her heart rate was 82 bpm, regular and blood pressure was 150/90 mmHg. Other investigative results were normal. Her ECG showed normal sinus rhythm. She was sent for a routine echocardiography to judge for further treatment of the arterial hypertension. Transthoracic echocardiogram showed normal left ventricular ejection fraction. There was a mobile echogenic mass of nearly 6 cm2 in the right atrium, prolapsing through the tricuspid valve with mild tricuspid regurgitation without causing obstruction and protruding into the inferior vena cava (IVC). The transesophageal echocardiographic examination confirmed the presence of a mobile multilobular mass in the right atrial free wall close to the IVC origin.
A total body angio-CT scan showed an intraatrial mass measuring approximately 5 × 4 cm, without infiltration of the adjacent structures, suggesting the diagnosis of myxoma. Coronary angiography revealed normal coronary arteries. The patient underwent median sternotomy under general anesthesia. The tumor was completely excised through a right atriotomy. The resected mass was sent for histological assessment which confirmed the diagnosis of myxoma.

Discussion
RA myxomas usually originate in the fossa ovalis or base of the interatrial septum, but in this case, the myxoma was implanted in the atrial inferior vena cava junction. Myxomas are usually polypoid and pedunculated tumors (approximately 83% of cases). In this report, our patient had a solitary, pedunculated mass with polypoid areas and a lobulated surface. Echocardiography remains the best diagnostic method for locating and assessing the extent of myxomas and for detecting their recurrence, with a sensitivity of up to 100%. However, transthoracic echocardiogram may not identify tumors smaller than 5 mm in diameter, and a transesophageal echocardiogram is required when there is suspicion of a very small tumor. In this case, an echocardiogram suggested the hypothesis of RA myxoma, which was confirmed by a histopathological exam. Myxomas are friable with high chance of systemic or pulmonary embolization depending on tumour location. Early diagnosis and timely surgical resection is the treatment of choice to prevent possible fatal consequences such as sudden death.
Abstract P1460 Figure. Right Atrial Myxoma
P1464 A case of cortriatriatum dexter accidentally discovered in an adult
Authors: Abdelgawad H S A , Abdelnabi M , Almaghrabi A , Shehata M , Abdelhay M A 
Published: January 2020

Abstract

Introduction
Cor triatriatum dexter, or partitioning of the right atrium (RA) to form a triatrial heart, is an extremely rare congenital anomaly that is caused by the persistence of the right valve of the sinus venosus. The incidence of cor triatriatum is approximately 0.1% of congenital heart malformation. Typically, the right atrial partition is due to exaggerated fetal eustachian and the besian valves, which together form an incomplete septum across the lower part of the atrium. This septum may range from a reticulum to a substantial sheet of tissue
Case report:
45-years old female patient with history of surgical closure of an atrial septal defect at the age of 14 years .She presented to our medical facility complaining of exertional dyspnea and bilateral lower limb edema for 4 years. On clinical examination, she had bilateral congested neck veins, a pansystolic murmur over the tricuspid area and a tender hepatomegaly. 2D Transthoracic Echocardiography revealed an unusual membranous structure that stretched across the right atrium with attachments superiorly at the free wall and inferiorly at the inter-atrial septum with a severe tricuspid regurgitation (Panel A)Intravenous agitated saline injection revealed an incomplete membrane. (Panel B).2D Transesophageal echocardiography showed an unusually prominent eustachian valve arose normally from the ostium of the inferior vena cava (IVC) and was pointing towards the interatrial septum just below the level of the fossa ovalis and no residual ASD could be seen. (Panel C) . 3D transesophageal echocardiography with zoomed mode from right atrial perspective confirmed the presence of an incomplete membrane extending transversely from the ostium of IVC and interatrial septum immediately below the fossa ovalis but not reaching RA free wall (arrow), no obstruction to the flow of the IVC, superior vena cava (SVC) , coronary sinus (CS) and the tricuspid valve (TV) was seen .(Panels D,E,F).

Conclusion
Since many patients are asymptomatic, the diagnosis of cor triatriatum dexter often is determined at postmortem examination. Antemortem diagnosis can be determined by echocardiography. 3D transesophageal echocardiography was able to detect cor triatriatum dexter that can be easily missed by 2D echocardiography.
Abstract P1464 Figure.
P1465 Ventricular septal defect and pulmonary artery aneurysm; How does it look by three dimensional echocardiography
Authors: Abdelgawad H S A , Abdelnabi M , Almaghrabi A , Shehata M , Abdelhay M A 
Published: January 2020

Abstract

Introduction
Pulmonary artery (PA) aneurysms (PAAs) are rare and infrequently diagnosed . The upper limit of the main PA diameter (29 mm) was defined as a PAA.
Congenital causes have been recognized as the major reason for PAA formation. More than 50% of all cases were associated with congenital heart disease. The 3 most frequent congenital heart defects associated with a PAA are, in decreasing order, persistent ductus arteriosus (PDA), ventricular septal defects (VSD) , and atrial septal defects (ASD).En face visualization of the whole circumference of the VSD and its relation to the surrounding structures can help in better understanding of the shear stress that promote PAA formation.

Case report
A 35 year old female patient with no previous cardiac history .She presented to our medical facility complaining of exertional dyspnea since 2 years . On clinical examination, she had harsh pansystolic murmur heard over left parasternal area . 2D Transthoracic Echocardiography revealed an aneurysmally dilated pulmonary artery (40mm in maximum diameter) and a wide VSD. 3D transthoracic and transesophageal echocardiography showed a wide subarterial VSD which is immediately located below the pulmonary valve. Therefore , that increased flow caused by left-to-right shunt caused by the intimate location of the VSD to the pulmonary valve results in increased hemodynamic shear stress on the vessel walls and therefore promotes aneurysm formation .Surgical closure of the VSD was done successfully with no residual defects.

Conclusion
PAAs seldom occur, are rarely diagnosed, and do not present with distinct symptoms. With the help of 3D echocardiography , direct visualization of the surrounding septal defects helps better understanding of mechanism of the aneurysm formation.
Abstract P1465 Figure.
P1471 Aortic, mitral and tricuspid valve transcatheter therapy in patient with severe chronic heart failure
Authors: Szlosarczyk B M , Golinska-Grzybala K , Rzucidlo-Resil J , Trebacz J , Stapor M , Konieczynska M , Gackowski A 
Published: January 2020

Abstract

Introduction
The objective of this case is to present a novel approach in percutaneous treatment of complex valvular heart disease in patient disqualified from cardiac surgery.

Case Description
A 59 year-old-man with a history coronary heart disease, myocardial infarctions (in 1993, 2011), percutaneous right coronary angioplasty (2011,2012), chronic kidney disease, persistent atrial flutter, Hodgkin"s lymphoma treated with radiotherapy and chemotherapy, was admitted to hospital due to congestive heart failure in NYHA class IV, despite optimal, maximal tolerated pharmacological treatment (furosemide 40 mg tid, torasemide 20 mg qd, bisoprolol 5 mg qd, perindopril 5 mg qd, spironolactone 25 mg qd, acetylsalicylic acid 75 mg qd, atorvastatin 40 mg qd)

Physical examination showed
BMI tachycardia 110/sec, blood pressure 95/68 mmHG, systolic murmur grade 5/6 best heard at the apex, moderate leg oedema. Chest auscultation revealed crepitations.
Echocardiography revealed severe, functional mitral (MR) - 4+(VC 8/20 mm) and tricuspid (TR) regurgitation (4+); combined aortic valve disease (moderate stenosis (SA), mild regurgitation (AR) - SA max. grad. 39/23mmHg, valve area -1.3-1.4 cm2, LV end diastolic diameter (LVEDD)/LV end-systolic diameter (LVESD) 57/44 mm, LV ejection fraction 48%, both atrium enlargement (left atrium 38 cm2, right atrium 35 cm2).
Angiography didn`t show significant changes in coronary arteries.
Because of high surgical risk (Euroscore II 9,14%, STS 7,29%) and porcelain aorta confirmed in CT scan Heart Team disqualified patient from cardiac surgery (mitral and aortic valve replacement and tricuspid valve annuloplasty). Afterward he was qualified to complex, percutaneous treatment – TAVI (trans-aortic valve implantation) in first stage, and transcatheter Mitraclip and Triclip implantation in second stage.
The Portico transcatheter aortic valve (29mm) was implanted – max. grad. was 11 mmHg, residual small paravalvular leak was noted.
Two weeks later transcatheter Mitraclip and Triclip implantation was performed and significant reduction of both MR (2+/3+) and TR (2+) was observed.
Gradually after percutaneous treatment dyspnoea improved to class NYHA I/II and one month later patient was discharged to home.

Discussion
Percutaneous treatment of valvular heart diseases becomes a promising alternative for patients disqualified from cardiac surgery.
P1473 Detection of cardiac sources of cerebrovascular events in patient with systemic lupus erythematosus with neuropsychiatric manifestations
Authors: Gegenava M , Gegenava T , Huizinga T 
Published: January 2020

Abstract

Introduction
Systemic lupus erythematosus (SLE) is an idiopathic connective tissue disease, characterized with multiorgan involvement. Central nervous system (CNS) involvement is one of the most frequent manifestation of SLE and is termed as neuropsychiatric SLE (NPSLE) Prevalence of NPSLE varies between 12% and 95% among SLE patients. cerebrovascular accidents (CVA) and/or transient ischemic attack (TIA) represents one of the most frequent neuropsychiatric manifestation in SLE patients. Cerebral embolism may have cardioembolic source. Purpose: we described one case of SLE patient with NPSLE diagnosis and concomitant nonsignificant size of ASD II and tried to find its possible association with cerebrovascular accident. Method: We performed retrospective analysis of SLE (NPSLE) patient who had undergone echocardiographic and brain MRI evaluation. Case-Results: A 63 years old women was diagnosed with SLE in 1996 based on positive ANF, positive anti-cardiolipin IGg , arthritis, discoid lupus, positive coombs test and neurological manifestations (hemiplegia and aphasia). Echocardiographic evaluation showed pericarditis and nonsignificant ASD II (&lt;3mm). Ischemic changes were observed on Brain MRI study. Two years later in 1998 brain MRI showed a white matter defect (suspected vascular nature). Patient received 6 standard course of treatment with Cyclophosphamide ,Prednisone (from 60 mg gradually decreased to 10 mg) and oral anticoagulation drugs. After 9 years from the first diagnosis of SLE patient achieved complete remission, but soon in 2006 patient developed TIA and in 2013 developed lacunar infarct. Cardiac source of embolism was excluded according to performed analysis. In addition myocardial infarction was excluded based on Single-photon emission computed tomography (SPECT) perfusion scan. Since then patient remained under the observation of multidisciplinary team. Conclusion: We demonstrated one case of SLE patient with life threating neurological manifestations developed several times. Transthoracic echocardiographic examination showed small ASD II which was not considered as source of embolism, but we believe that in SLE patients with PFO/ASD, even though the size of defect is not large, cerebrovascular accidents may develop due to underlying inflammatory mechanism predisposing possible thromboembolism and early diagnosis, follow-up and management can be paramount to avoid future complications.