bluuebunny/crossref_metadata_2025_split huggingface dataset:
P678 Late gadolinium enhancement cardiac magnetic resonance and layer-specific longitudinal strain in treatment naive Anderson Fabry disease
Authors: Esposito R , Sorrentino R , Ponsiglione A , Santoro C , Lembo M , Riccio E , Imbriaco M , Pisani A , Galderisi M 
Published: January 2020

Abstract

Background
In Anderson-Fabry disease (AFD), the accumulation of glycosphingolipids in cardiomyocytes causes inflammation, hypertrophy and interstitial fibrosis which is first limited to the mid-myocardial layers, then spreads to transmural fibrosis. Speckle tracking echocardiography allows the estimation of layer-specific strain, differentiating longitudinal strain (LS) at subendocardium and subepicardium (LSsubendo and LSsubepi, respectively).

Purpose
To investigate the matching of functional and structural abnormalities in newly diagnosed, never treated AFD patients by comparing multi-layer LS and late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR).

Methods
Twenty newly diagnosed, never treated AFD patients (age = 37 ± 13 years; F/M =10/10) and 20 healthy controls, matched for age and sex, underwent comprehensive evaluation of target organs and a standard echo-Doppler exam, including assessment of relative diastolic wall thickness (RWT) and left ventricular mass index (LVMI). Left ventricular hypertrophy (LVH) was defined as LVMi &gt; 47 g/m^2.7 in women and &gt;50 g/m^2.7 in men. Speckle tracking echocardiography derived left ventricular transmural global longitudinal strain (GLS), LSsubendo, LSsubepi and LS gradient (LSsubendo - LSsubpepi) were also determined. CMR sequences including assessment of LGE were also performed.

Results
AFD patients had normal renal function and comparable body mass index, blood pressure, heart rate, ejection fraction and diastolic indices with healthy controls. LVMi (p = 0.006) and RWT (p &lt; 0.02) were greater in AFD patients than in controls. GLS (p = 0.006), LSsubendo (p = 0.005) and LSsubepi (p &lt; 0.001) were lower in AFD patients. By CMR, only four patients (F/M = 3/1), 3 with LVH (75%), exhibited focal LGE, always localized in the midwall. LGE was detected at basal lateral wall in three patients and at the septal insertional points with the right ventricle in the remaining one. AFD patients with LGE had higher LVMi than those without LGE (47.2 ± 11.0 vs. 34.5 ± 11.6 g/m^2.7, p = 0.04). There was no correspondence of LGE with both regional LS and layer specific LS impairment (Figure). However, AFD patients with LGE had higher LS gradient compared to those without LGE (5.8 ± 0.65 vs. 4.8 ± 0.66, p &lt; 0.02).

Conclusions
Treatment naïve AFD patients show a reduction of longitudinal deformation, which involves both subendocardial and subepicardial layers. Patients with LGE present higher LV mass and increased LS gradient, without a correspondence of LGE with both regional and layer specific LS impairment. Accordingly, this impairment might due to myocardial inflammation occurring in the early disease stages more than to myocardial fibrosis itself.
Abstract P678 Figure. Multilayer bull"s eye and LGE-CMR in AFD
P681 Surprise imaging findings in an asymptomatic patient with an ECG suggestive of apical hypertrophic cardiomyopathy
Authors: Cadil D , Calin A , Calin C , Popescu B A 
Published: January 2020

Abstract
We present the case of a 55-year old woman with an unremarkable past medical history, allergic to eggs and cinnamon and without any clinical complaints, who presented for a routine ECG prior to a dental implant procedure.
The ECG revealed signs of left ventricular (LV) hypertrophy with deep negative T waves in V2-V6 (Figure) suggesting apical hypertrophic cardiomyopathy. Her physical exam was unremarkable and a transthoracic echocardiography (TTE) was ordered. The TTE revealed normal LV dimensions, a normal global systolic function of both ventricles, but most remarkably, an isoechoic mass that occupied the whole apex and one third of the LV cavity (Figure), with a maximum thickness of 22 mm and a 2 mm hyperechogenic lining towards the LV, all these findings being suggestive of Loeffler endocarditis.
The routine laboratory tests showed (apart from a slightly increased LDL-cholesterol and normal BNP values) hypereosinophilia: more than 10% of the white blood count.
Chest X-Ray and the abdominal scan showed no signs of pulmonary or hepatic infiltration. Cardiac magnetic resonance imaging was scheduled, but the patient has claustrophobia and was unable to perform the exam.
The aetiological workup included the investigation of allergic, hematologic, as well as parasitic causes of the hypereosinophilic syndrome. Consequently, serological tests for Coxiella, Chlamydia, Echinococcus and Toxocara were all performed. The IgG antibodies for Toxocara canii were found in a very high level, indicating a chronic parasitic infection as a possible cause of the hypereosinophilic syndrome. The hematologic exams were all within normal limits. The endomyocardial biopsy, the gold standard for diagnosis could not be performed for technical reasons.
The first step in the management of hypereosinophilia (HE) is stopping exposure to the trigger, as well as specific treatment- albendazole. The patient received oral anticoagulant treatment in order to prevent thromboembolic complications, which are frequently reported in this clinical setting.
Loeffler’s endocarditis is usually a late cardiac manifestation of the HE syndrome. This is a rare and surprising case of an incidental imaging diagnosis in a totally asymptomatic patient after a routine ECG that was suggestive of hypertrophic cardiomyopathy. The challenge consisted in finding the cause of HE in order to attempt an aetiological treatment. Although the allergic and parasitic causes were most likely, considering the patient’s history, a hematologic malignancy had to be excluded. HE associated with toxocariasis rarely causes cardiac manifestations, but it should not be excluded when the patient has a history of canine exposure. The most fearsome complication in this case is the embolization of the eosinophilic mass, but the prompt management and routine follow-up allow the prevention of a thromboembolic event.
Abstract P681 Figure. ECG and TTE apical views
P690 Pulmonary valve endocarditis complicated with fistula between right ventricle outflow tract and aorta in a patient with patent ductus arteriosus
Authors: Toader D , Craciun Mirescu A , Cocora M , Munteanu O , Mustafa E R , Florescu C , Berceanu M , Popescu M , Cojocaru A , Oprian A 
Published: January 2020

Abstract

Background
The majority of cases of right sided infective endocarditis involve the tricuspid valve. Isolated pulmonary valve (PV) endocarditis is rare. Congenital heart disease are risk factors. Material and methods: We present the case of 36 years old male, without any known cardiovascular disease, who was admitted with signs and symptoms of heart failure, pulmonary embolism and fever. He was evaluated clinically, 12 lead ECG, pulmonary radiography, thoracic computer tomography (CT) scan, transthoracic and transesophageal echocardiography, laboratory investigations. Results: Transthoracic and transesophageal echocardiogram revealed large vegetations located on pulmonary valve, pulmonary regurgitation and dilation of the pulmonary valve, patent ductus arteriosus (PDA) with bidirectional shunt and local complications: fistula between right ventricle outflow tract (RVOT) and aorta, pleural and pericardial effusion. Blood cultures were negative. ECG aspect was right bundle branch block. Radiography and thoracic CT scan revealed pulmonary embolism aspect. The patient was treated with antibiotics and surgical option included debridement of the infected area, vegetation excision with valve replacement, relief of RVOT, fistula closure with pericardial patch and ligature of PDA. Evolution after surgical intervention was good.Conclusions: This presentation reveals a favorable evolution of a patient with infective endocarditis located to pulmonary valve. In most of cases, right heart endocarditis presents with signs and symptoms of respiratory disease and fever; these are due to septic pulmonary embolization. Isolated PV endocarditis still remains a challenging and needs carefully echocardiographic evaluation for a correct diagnosis and risk factors identification.
P703 A breathtaking heart. The loeffler"s endocarditis
Authors: Carcaterra A , Moch S , Sologashvili T , Muller H , Testuz A 
Published: January 2020

Abstract
This is a 72 year-old man known for idiopathic HES with endomyocardial fibrosis and moderate aortic regurgitation, treated with corticosteroids and antithrombotics. Two months prior to admission, he develops progressive exertional dyspnea reaching NYHA 3 class, with concomitant peripheral edema and rise in NT-proBNP. Cardiac transthoracic work-up shows a preserved left ventricular ejection fraction, but more marked dilatation with decrease in left ventricular volume due to a large echodense mass in the apex. The transmitral flow is restrictive, and the aortic regurgitation moderate. In view of this rapid evolution, resection of the left ventricular mass with concomitant aortic valve replacement is performed. Pathology confirmed eosinophilic infiltration.
First described in 1975, hypereosinophilic syndrome (HES) is a rare pathology that is defined as an absolute eosinophil count (&gt;1,5 G/L) in the peripheral blood with eosinophil-mediated organ damage and /or dysfunction. We know three mains categories; primary (or neoplastic), secondary (or reactive) and idiopathic. Cardiac involvement is uncertain and cardiac injury does not clearly correlate with degree of peripheral eosinophilia. Heart tissue damage evolves through three stages (necrotic, intermediate and fibrotic). Endomyocardial fibrosis and eosinophilic myocarditis, also known as "Loeffler’s endocarditis", are major causes of morbidity and mortality among patients with HES. Echocardiography plays a crucial role in initial diagnosis of endomyocardial fibrosis, displaying myocardial wall thickening, signs of restriction (mitral inflow with large E wave and small A wave; E/A ratio greater than 2, short deceleration time less than 150 ms) and intracardiac thrombus (fixed mass embedding left ventricular apex). Valve regurgitation is another possible complication due to entrapment of the chordae tendinae and/or leaflets during the fibrotic stage of this disease. Cardiac MRI is another important diagnostic tool for precising cardiac involvement. Despite of steadily improving noninvasive diagnostic imaging methods, endomyocardial biosy still remains the gold standard exam and ultrasounds work-up is crucial for follow-up.
In conclusion, endomyocardial fibrosis is a rare cause of restrictive cardiomyopathy characterized by echocardiographic sign of elevated filling pressure, progressive endomyocardial thickening, valve regurgitation and possible intracardiac thrombus formation. This case underlines the importance of echocardiography in initial diagnosis and regular follow-up of this type of patients, in order to adapt medical treatment and monitor hemodynamic evolution of the restrictive physiology and of valvular damage.
Abstract P703 Figure. HES-3
P704 Mechanical complications of acute myocardial infarction in the era of early reperfusion therapy: a case report
Authors: Proenca T , Alves Pinto R , Martins Carvalho M , Nunes A , Araujo P M , Torres S , Resende C X , Grilo P D , Dias P , Paiva M , Casanova J , Maciel M J , Macedo F 
Published: January 2020

Abstract

Introduction
Left ventricular pseudoaneurysm is a rare mechanical complication of myocardial infarction, and its incidence has decreased with the widespread use of reperfusion therapies. Pseudoaneurysm is the result of a free wall rupture contained by pericardial adherences and mural thrombi, which contain the bleeding and prevent cardiac tamponade.

Clinical Presentation
A 68-year-old woman who had hypertension, diabetes mellitus and chronic kidney disease (caused by diabetic nephropathy) was first admitted with acute myocardial infarction of the inferior wall. Emergent coronary angiography revealed proximal occlusion of the right coronary artery. Primary angioplasty was performed with three stents implantation. However due to transitory no reflow, verapamil, nitrate and intracoronary abciximab were administered with recovery of coronary flow. Patient remained stable, without recurrence of symptoms. Echocardiography, at discharge, showed normal biventricular function and no mechanical complications.
Two months later, the patient was readmitted in the emergency room with constant chest pain, fatigue, prostration and loss of appetite beginning ten days earlier and an episode of syncope. Physical examination revealed fever, cardiac auscultation was rhythmic and without murmurs or pericardial friction rub, and pulmonary auscultation revealed crackles in inferior hemithorax. 12-lead electrocardiogram showed sinus rhythm, Q waves and negative T waves in inferior leads. Blood tests revealed leucocytosis, high sensibility troponin I was 28,8 ng/L and brain natriuretic peptide was 264,9 pg/mL. Chest-X-ray demonstrated enlargement of the cardiac silhouette and echocardiography showed moderate to large pericardial effusion with large amounts of fibrin close to right cardiac chambers and a basal inferior pseudoaneurysm with 23 mm x 24 mm; intracavitary contrast was administered without opacification of pericardial space; biventricular function remained normal.
Patient was promptly admitted on Cardiac Intensive Care Unit with diagnosis of pseudoaneurysm due to myocardial infarction. Therapeutic with ticagrelor was suspended and surgical correction was proposed, after discussion in Heart Team. False aneurysm correction was performed with a bovine pericardial patch without complications, and the patient was discharged asymptomatic eight days later.

Conclusion
Even with lower incidence, pseudoaneurysms remains as a potential life-threatening due to its high risk of rupture. Prompt diagnosis, usually with echocardiography and surgical referral are crucial.
Abstract P704 Figure. Inferior Pseudoaneurysm
P715 When "wait and see" is the best option: a case report of a spontaneous coronary artery dissection
Authors: Castelo A , Silva T , Ramos R , Fiarresga A , Moreira R , Mano T , Ferreira V , Bras P , Ferreira R 
Published: January 2020

Abstract

Introduction
Spontaneous coronary artery dissection is a rare cause of acute coronary syndrome and is now being identified more frequently, in part because of increased awareness.

Case report
We present the case of a 63-year-old female, without cardiovascular risk factors or relevant past medical history. She complained of atypical chest pain in the last year. The patient was admitted due to acute chest pain at rest, hemodynamically stable, with unremarkable physical examination, including absence of heart failure signs. The EKG revealed a dynamic ST depression in leads V4-V6. The peak of high sensitivity troponin I was 13744pg/mL (ULN&lt; 15.6) and CK 874U/l (ULN &lt;168). The echocardiogram showed preserved left ventricular ejection fraction and hypokinesia of mid-apical segments of anterior wall. Considering the diagnosis of NSTEMI the patient underwent coronary angiography that revealed luminal narrowing of 70% in left main artery, 70% in proximal anterior descending artery (LAD) and 99% in first obtuse marginal. Given the absence of cardiovascular risk factors, the smooth angiographic appearance of coronary lesions and absence of calcium, we suspected of spontaneous dissection or vasculitis. Considering the absence of angina revascularization was delayed. A first coronary angio-CT confirmed the luminal narrowing and suggested a spontaneous dissection. Two weeks later the coronariography and the angio-CT were repeated with a significant improvement, showing only intermediate stenosis of proximal LAD. The additional imaging study revealed a 45mm ascending aortic dilation and a left primitive carotid stenosis without other vascular territory alterations, excluding fibromuscular dysplasia. The auto-immune study was unremarkable. With all these results it was assumed the diagnosis of a spontaneous coronary artery dissection and the patient was discharged asymptomatic under single antiplatelet therapy and Rivaroxaban. Three months later a new coronary angio-CT showed no significant coronary artery stenosis and the patient was asymptomatic.

Discussion and conclusion
The recognition of spontaneous coronary artery dissection is essential to the correct management of these cases because, unlike acute coronary syndrome due to atherosclerotic disease, the results of revascularization in these patients are suboptimal and conservative management is probably the best option.
Abstract P715 Figure. angio-CT
P719 Complex cyanotic congenital heart disease and its complications- it is never too late to treat
Authors: Radu D N , Stoica S , Dorobantu D M , Olaru-Lego G , Buta A S , Platon P , Iosifescu A , Coman I M , Ginghina C , Popescu B A , Enache R 
Published: January 2020

Abstract
Noncardiac complications significantly contribute to the morbidity and mortality of adults with congenital heart disease (CHD), mainly because life expectancy and quality of life for those born with CHD have greatly improved in the last decades. Double-outlet right ventricle (DORV) with a subaortic ventricular septal defect (VSD) associated with subpulmonary stenosis is a complex cyanotic congenital heart disease from the spectrum of tetralogy of Fallot.
We present the case of a young male who was diagnosed at the age of 18 with DORV with subaortic VSD (Figure A), subpulmonary stenosis (Figure C) and mitral valve malformation (anterior leaﬂet cleft (Figure B) and both leaﬂets prolapse) with secondary moderate mitral regurgitation (MR), associated with major aortopulmonary collaterals, without pulmonary hypertension. There was no surgical correction at that moment. At the age of 26 he presented with recurrent hemoptysis and embolisation of bronchial arteries was performed. In the same year, a cerebral MRI showed signs of multiple infratentorial and supratentorial ischemic strokes. One year later, he was diagnosed with brain abscess and otomastoiditis that were surgically managed; multiple microorganisms were isolated from the two sources (M. morganii, K. pneumoniae, P . aeruginosa) and the patient received prolonged antibiotic therapy. Two months later he presented with clinical, biological and echocardiographic signs of infective endocarditis (IE) and blood cultures confirmed the diagnosis of Candida albicans associated mitral valve IE (Figure D, E, F, G). Antifungal therapy was administered for one month, with the complete disappearance of the vegetation, but worsening of the mitral regurgitation and moderate left ventricular systolic dysfunction. Thus, the patient had a clear indication for mitral valve replacement together with complete repair of the cardiac malformation that seemed still feasible as the pulmonary arteries were well developed, there was no pulmonary vascular disease and the systolic function of the RV was normal. Correction of CHD was performed in March 2019 consisting of repair of the DORV (Figure H), mitral valve replacement (33 mm bileaflet mechanical valve) (Figure I), tricuspid valve repair with RVOT remodelling. Postoperative, the patient clinical status improved significantly and transthoracic echocardiography revealed a mild residual subpulmonary stenosis (Figure J) and normally functioning mitral prosthesis.
There are frequent, various complications in the natural history of congenital heart diseases, especially unrepaired. This case illustrates how vicious the circle of complications can get in a case of unrepaired cyanotic cardiac malformation and the difﬁculty of breaking this circle. The correction of CHD is the best prevention method and should be performed whenever it is feasible, even at an adult age.
Abstract P719 Figure.
P728 Left main coronary artery thrombus found in a teenager with Kawasaki disease following cardiac arrest
Authors: Roque H , Monteiro De Sousa D , Bowes M 
Published: January 2020

Abstract

Background
A male teenager presented with an out of hospital cardiac arrest while exercising requiring CPR and DC Cardioversion. Incidentally, he was stung by a wasp minutes before the event.
After being admitted, the patient was intubated, ventilated and treated for anaphylaxis and seizures. The patient had no significant past medical or family history of note. Investigations were performed after the patient was stabilized including 15 lead ECG, 24 hours ECG monitor, Echocardiogram and blood sample for troponins and cardiomyopathy screening. The ECG and 24 Hours ECG were reported as normal. The patient also had an exercise treadmill test which showed ischaemic changes at peak exercise. Cardiomyopathy screening was negative.
Echocardiogram showed globally mildly impaired left ventricular systolic function with no other significant abnormalities.
The patient underwent a cardiac MRI a couple of weeks later which showed an aneurysmal and tortuous proximal left coronary artery with a thrombus and also an aneurysmal mid left circumflex artery. Anticoagulation therapy was initiated, and a Cardioverter Defibrillator was implanted.
Another echo was performed focused on the LV function and visualisation of the coronary arteries. This confirmed the cardiac MRI findings, demonstrating two coronary artery aneurysms and a filling defect suggestive of thrombus in the more distal of the two aneurysms.
Due to the lack of evidence of any other potential condition, a diagnosis of Kawasaki disease was made and anticoagulation therapy was continued.

Conclusion
Although Kawasaki disease (KD) has been investigated for over four decades, its cause is still unknown. Current understanding of the immune system response indicates response to a classic antigen, that in most patients is protective against future exposure (1).
KD is an acute, self-limited vasculitis that affects young children. In a significant proportion of patients, it can originate coronary artery abnormalities, predominantly if the diagnosis is not achieved or treatment gets delayed. In the course of acute illness, the vascular architecture is destroyed by a necrotizing arteritis, causing hydrostatic pressure and leading to aneurysms in the affected areas (2).
The imaging modality preferred for assessment of myocardial function and detection of coronary artery abnormalities is Echocardiography (2).
In this case, given the unusual presentation of cardiac arrest and the diagnosis of KD we recommend a thorough assessment of the coronary arteries in echocardiography routinely.
Abstract P728 Figure.