bluuebunny/crossref_metadata_2025_split huggingface dataset:
P233 A new onset pulmonary artery stenosis in a young man
Authors: Scheggi V , Mazzoni C 
Published: January 2020

Abstract

Background
Poorly differentiated and undifferentiated sarcomas are the most common primary tumors of the pulmonary arteries. They usually affect large caliber vessels and present with predominantly intraluminal growth. Dyspnea, cough, chest pain, and hemoptysis are the most common presenting symptoms; patients may have signs of chronic pulmonary hypertension. Clinical and imaging manifestations can mimic pulmonary embolism.

The overall prognosis is poor
combined therapy with surgical resection and chemo-radiotherapy offers the best survival rates.

Case presentation
A 31-year-old male was referred to our department because of recent onset dyspnea, a pre-syncopal episode and a new heart murmur.
He had normal spirometry results but a significant desaturation during the 6MWT. A thoracic CT scan showed an incremented diameter of the pulmonary artery.The echocardiogram showed the presence of a mass with irregular borders attached to the pulmonary trunk almost obliterating its lumen. The mass determined a flow acceleration with maximal velocity of 3.8 m/sec, and a peak gradient of 60mmHg; Doppler findings on the pulmonary valve and right heart function were within normal values. Compression ultrasonography ruled out the presence of deep vein thrombosis.
The lesion showed a dishomogeneous impregnation in the contrastographic phase at cardiac MRI and had an intense glucidic metabolism at a PET-CT scan. These findings were highly suggestive of an angiosarcoma of the pulmonary artery. Biopsy specimens were taken through bronchoscopy.
The patient then decided to continue treatment in another hospital, where the histologic samples were sent; the cytologic results showed atypical cellular elements. The patient died a few months later.

Discussion
The presence of a unique mass in the main pulmonary artery or proximal branches and rapidly progressive dyspnea in a patient at low risk of pulmonary embolism should raise the suspicion of primary sarcoma of the pulmonary artery, which is a rare but aggressive tumor with a very poor prognosis.
Abstract P233 Figure.
P1581 The global myocardial work index is a powerful predictor of major arrhythmic events in patients with organic heart disease and reduced left ventricular ejection fraction
Authors: Previtero M , Sammarco G , Genovese D , Azzolina D , Tenaglia R M , Ruozi N , Palermo C , Iliceto S , Muraru D , Badano L P 
Published: January 2020

Abstract

Background
Current guidelines recommend implantable cardioverter defibrillator (ICD) for primary prevention of sudden cardiac death in patients with left ventricular ejection fraction (2DE LVEF) by two-dimensional echocardiography≤ 35%. However, new echocardiography parameters of LV function such as the mechanical dispersion (MD), the LVEF by three-dimensional echocardiography (3DE) and the global myocardial work index (GWI) have been reported to provide a more accurate stratification of the arrhythmic risk, and potentially improve ICD patient selection.

Purpose
We wanted to compare the arrhythmic risk predictive power of the new parameters of LV function with the conventional 2DLVEF.

Material and Methods
we prospectively enrolled 216 patients (63 ± 12 years, 88% men) with organic heart diseases and 2DE LVEF &lt;50%, in whom we re-measured LVEF using 3DE, and obtained MD and GWI using 2DE speckle tracking. Major arrhythmic events were defined as sudden cardiac death, sustained ventricular tachycardia, ventricular fibrillation and appropriate ICD shocks. We assessed the predictive power of 4 different parameters: 2DE LVEF&lt; 35%; 3DE LVEF&lt; 35%; MD &gt; 80 ms; and GWI&lt; 672 mmHg% to identify patients at risk of major arrhythmic events.

Results
During a mean follow-up of 27 ± 24 months, 24 patients (10%) experienced sudden cardiac death, whereas 28 patients (13%) presented major arrhythmic events. The predictive power in terms of major arrhythmic events prediction (Harrel C statistics) improved from 0.67 (95%CI 0.57-0.76) for 2DE LVEF&lt; 35%, to 0.73 (95%CI 0.64-0.82) for 3DE LVEF&lt; 35%, and 0.77 (95%CI 0.68-0.86) for GWI &lt; 672 mm Hg%. Whereas, MD &gt; 80 ms showed a limited predictive power (HCS= 0.53, 95%CI 0.41-0.76)).

Conclusions
GWI&lt; 672 mm Hg% was the most accurate predictor of major arrhythmic events among echocardiography parameters in patients with organic heart disease and LVEF &lt; 50%.
P1586 Lung ultrasound and left atrial deformation analysis: a promising methods for the diagnosis of heart failure with preserved ejection fraction
Authors: Agoston G , Gargani L , Szabo I , Illes B , Varga A 
Published: January 2020

Abstract

Background
Heart Failure with Preserved Ejection Fraction (HFpEF) is a growing healthcare burden and its prevalence is increasing. Diagnosing HFpEF is challenging. Lung ultrasound (LUS) and left atrial strain are promising screening tools to assess pulmonary congestion and left atrial dysfunction in patients with suspected HFpEF.

Aim
To evaluate the relationship between LUS, left atrial strain and NT-proBNP level in patients with HFpEF. Also to assess the diagnostic power of B-lines in HFpEF population.

Methods
Forty-seven consecutive patients (24 women, mean age 69 ± 11 years) with clinical signs of heart failure were prospectively enrolled. Exclusion criteria were: ejection fraction &lt;55%, more than mild mitral and/or aortic valve disease, pulmonary disease, pulmonary arterial hypertension. Within one hour all patients underwent comprehensive echocardiographic evaluation including left atrial strain analysis (peak atrial longitudinal strain-PALS), lung ultrasound assessment of B-lines on the antero-lateral and posterior chest wall, and NT-proBNP levels.

Results
In 34 patients (72%) a significant number of B-lines (≥15) were observed. We found a positive correlation between the number of B-lines and NT-proBNP levels (p &lt; 0,0001, r = 0,74, Figure 1.), left atrial volume (p &lt; 0,05, r = 0,45), and PALS (p &lt; 0,02, r = 0,4 ). We didn’t found any correlation between the number of B-lines and E/e’ ratio (p = 0,1, r = 0,28), or between E/e’ ratio and NT-proBNP level (p = 0,1, r = 0,2). We also assessed the diagnostic ability of ≥15 B-lines to predict markedly elevated pro-BNP level (≥ 220pg/ml), AUC was 0.89. If the total number of B-lines was greater or equal to 28, the sensitivity was 68% with the specificity of 100%, but if we changed the cut-off value to 12, the sensitivity grew to 89% with the specificity of 71%. (Figure 2.).

Conclusion
LUS is a simple, feasible tool to detect pulmonary congestion in HFpEF and it has a strong diagnostic power to predict elevated NTpro-BNP level. B-lines correlate with parameters of left atrial dysfunction. PALS is promising too, which better reflects pulmonary congestion and elevated NT-proBNP values than the conventional echocardiographic parameter E/e’.
Abstract P1586 Figure. NT-pBNP vs B-lines, AUC of Blines
P1594 Relationship between left ventricular morphology and systolic performance and coronary microcirculatory dysfunction in hypertrophic cardiomyopathy
Authors: Aguiar Rosa S , Branco L , Galrinho A , Fiarresga A , Lopes L , Celas M , Silva F , Carvalho A F , Mota Carmo M , Cruz Ferreira R 
Published: January 2020

Abstract

Background
Myocardial ischemia constitutes one of the most important pathophysiological features in hypertrophic cardiomyopathy (HCM). Chronic and recurrent myocardial ischemia leads to fibrosis, which may culminate in myocardial dysfunction.

Objective
To analyse the relationship between left ventricular (LV) morphology and systolic performance and coronary microcirculatory dysfunction in HCM.

Methods
The present study prospectively included HCM patients (P) who underwent transthoracic echocardiography. Left ventricular (LV) function was evaluated by ejection fraction (LVEF), global longitudinal strain (GLS) and tissue Doppler septal and lateral s’. The evaluation of coronary flow velocity reserve (CFVR) was performed in apical three chambers view for the left anterior descending (LAD) artery and in an apical three chambers view for the posterior descending (PD) artery. Diastolic coronary flow velocity was measured in basal conditions and in hyperemia, induced by adenosine perfusion (0.14 mg/kg/min intravenously, during 2 minutes). Absolute CFVR was calculated as the ratio of hyperemic to basal peak diastolic flow velocities; relative CFVR was calculated as the ratio between CFVR LAD and CFVR PD.

Results
23 P were enrolled (57% male, mean age 57.9 ± 13.7 years). Asymmetric septal hypertrophy was verified in 70% of P, with maximal wall thickness of 21.6 ± 4.3mm. Obstructive HCM was documented in 35% of patients.
CFV was successfully measured in the LAD in all patients, but only in 70% of patients in the PD due to technical issues related to poor acoustic window and anatomical constraints. 78% of P (n = 18) presented CFVR &lt;2, denoting microcirculatory dysfunction. Relative CFVR (LAD CFVR/ PD CFVR) was ≥1 in 43% of P.
P with maximal wall thickness (MWT)&gt;20mm presented higher CFV PD at baseline (46.5 ± 17.4 vs 32.5 ± 12.6 cm/s; p = 0.072), lower CFVR PD (1.3 ± 0.3 vs 2.5 ± 0.8; p = 0.003) and greater regional difference of microcirculation (relative CFVR 1.4 ± 0.6 vs 0.8 ± 0.3; p = 0.048).
At baseline conditions, CFV LAD was higher in obstructive HCM (44.0 ± 4.8 vs 35.3 ± 10.6 cm/s; p = 0.040).
P with impairment in global longitudinal strain (GLS&gt;-18%) had higher basal CFV LAD (40.1 ± 8.6 vs 30.0 ± 12.2 cm/s; p = 0.059) and PD (44.5 ± 15.2 vs 20.0 ± 5.0 cm/s; p = 0.015) but lower CFVR PD (1.5 ± 0.5 vs 2.8 ± 1.1; p = 0.039). The reduction in CFVR PD was also noted in P with time to peak longitudinal strain dispersion &gt;90mseg (CFVR PD 1.2 ± 0.2vs1.9 ± 0.9;p = 0.012).

Conclusion
Higher CFV at baseline was noted in P with greater MWT, obstructive HCM and worse GLS. Coronary microcirculatory dysfunction was associated with the degree of LV hypertrophy and impairment in LV systolic performance.
P1595 Prognostic role of late gadolinium enhancement for sudden cardiac death risk in non-ischemic dilated cardiomyopathy
Authors: Rodriguez Sanchez I , Onaindia J J , Gomez V , Aguirre Larrakoetxea U , Velasco S , Urkullu A , Ullate A , Capelastegui A , Astigarraga E , Florido J , Natividad R , Garcia Ibarrondo N , Goena C , Rilo I 
Published: January 2020

Abstract

OBJECTIVES
to evaluate the prognosis role of late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (cMRI) in patients with non-ischemic dilated cardiomyopathy (NIDM).

BACKGROUND
Risk stratification in NIDM needs to be improved.

METHODS
We included 210 patients with NIDM and cMRI from 2005 to 2018 in our study population. Outcomes were retrospectively assessed by medical records. The pattern of LGE was classified as mid-wall, sub-epicardial, or both patterns. Primary endpoint was sudden cardiac death (SCD) or aborted SCD. Secondary endpoints were global mortality and a composite endpoint of cardiovascular mortality and heart failure hospitalization.
Demographic and clinical parameters were also evaluated. Patients with LGE (LGE+) were more likely to be male (80,6% vs 66,7%, p= 0,03). No significant differences were observed between LGE+ and LGE- patients in comorbidities, NYHA class, left ventricular ejection fraction (LVEF), or neurohormonal treatment.

RESULTS
Of 210 patients (71,4% men, median age 59,8 years) with a median follow up of 5,6 years (3,24-8,15), 72 patients (34,3%) had non ischemic LGE (LGE+) on cMRI. Mean left ventricular ejection fraction (LVEF) was 34%.
SCD or aborted SCD occurred in 11 patients (5,2%): 6 patients (9,5%) with LGE+ vs 5 patients (4,07 %) of LGE- (p = 0,19).
Patients with LGE+ had a higher risk for the composite endpoint (cardiovascular mortality and heart failure hospitalization): OR 2,45, confidence interval (CI): 1,16-5,17, (p = 0,02).
LGE presence was not associated with global mortality.
The subepicardial pattern of LGE was associated with SCD or aborted SCD. 3 out of 11 patients (27%), with subepicardial pattern of LGE suffered from SCD or aborted SCD (p= 0,01).

CONCLUSIONS
In our cohort of 210 patients with NIDM, LGE was not significatively associated with SCD or aborted SCD, probably because of a low event rate (5,2%) in a relatively small and well treated population, despite a long follow-up (5,6 years). On the other hand, LGE presence was associated with a higher risk for the composite endpoint of cardiovascular mortality and heart failure hospitalization. Finally, the subepicardial pattern of LGE identified a group of patients at high risk of SCD and aborted SCD.
P1601 Relationship between aortic distensibility and aortic regurgitation assessed by CMR in bicuspid valve patients
Authors: La Mura L , Teixido-Tura G , Guala A , Ruiz-Munoz A , Lopez Sainz A , Valente F , Pisaniello M , Strada S , Granato C , Galian Gay L , Gonzalez-Alujas T , Servato M L , Ferreira I , Rodriguez-Palomares J F , Evangelista A 
Published: January 2020

Abstract

Funding Acknowledgements
Spanish Ministry of Economy and Competitiveness RTC-2016-5152-1, ISCIII PI17/00381, La Marató de TV3 (20151330), Eur FP7/People 267128 and CIBERCV

BACKGROUND
The severity of aortic regurgitation can be evaluated with cardiac magnetic resonance (CMR) through calculation of regurgitant fraction (RF) in phase contrast sequences acquired at the aortic root (as close as possible to the aortic valve). However, the impact of aortic distensibility in this evaluation remains unkown.

PURPOSE
The aim of the study was to evaluate the relation between aortic distensibility and RF valve in bicuspid aortic valve patients.

METHODS
We enrolled bicuspid aortic valve patients without significant aortic stenosis (maximum velocity &lt;2.5 m/s) and connective tissue disease. All patients underwent a CMR study with phase contrast sequences for evaluation of regurgitant fraction at the level of the aortic valve. Aortic regurgitation was considered as mild, moderate or severe depending on RF value (mild &lt;15%; moderate 15-30%; severe &gt;30%). Furthermore we used cine-sequences of aortic root, ascending and proximal descending aorta to estimate aortic diameters and distensibilities, using Art Fun software. Distensibility was calculated as (change in aortic area between systole and diastole/diastolic area)/brachial pulse pressure.

RESULTS
A total of 98 bicuspid aortic valve patients were included (30% female, 49.7 ± 14.5 years). 75 (76,5%) AR was mild, 17 (17,4%) moderate and 6 (6,1%) severe. RF valvewas significantly correlated with aortic root diameter (r= 0.430 y p &lt; 0.001 )and aortic distensibility at the level of the ascending (r = 0.273 p =0.016) and descending aorta (r = 0.502 and p&lt; 0.001). Aortic distensibility was positively correlated with RFvalve even after adjustment for aortic diameter ( p = 0.002 and p &lt;0.001 respectively) . (Table) (IMG)

CONCLUSIONS
In our study, aortic regurgitation in bicuspid valve patients, evaluated by CMR using RF valve, is related to aortic distensibility. Thus, aortic distensibility should be included in the evaluation of aortic regurgitation by CMR as additional parameter. However, longitudinal studies are needed to evaluate the impact of including aortic distensibility in the evaluation of AR severity by CMR.
AR SEVERITY MILD MODERATE SEVERE Descending aorta distensibility(mean ± std. deviation) 2693,68 ± 997,5 3285,8 ±1952,7 5042,99 ±2873,44 Correlation between AR severity (by RFvalve) and descending aorta distensibility
Abstract P1601 Figure.
P1602 An adult with mitochondrial cardiomyopathy
Authors: Kandil A , Daniel J , Nata R , Felix P 
Published: January 2020

Abstract

Introduction
Mitochondrial diseases are a group of rare inherited disorders with diverse phenotypes that are caused by mutation in nuclear or mitochondrial DNA. The prevalence of mitochondrial disease is estimated to be one in 5000 livebirths. The heart depends mainly on the energy produced through aerobic respiration and hence cardiac involvement is common, progressive and its presence is an independent predictor of mortality in patients with mitochondrial disease and may occur as the principal clinical manifestation or part of a multisystem disease.

Case report
We present a 31 years old lady who was referred to our hospital with a newly diagnosed hypertension and non-specific ECG changes. The patient had no shortness of breath, no palpitation and no chest pain. She was overweight and had short stature. Her blood pressure was elevated 155/90. There was no signs of heart failure and no murmurs on auscultation of the heart and lung. Her ECG showed sinus bradycardia 55-60 b/min, ST segment elevation in the anterior chest leads with non-specific widespread t wave inversion.
An Echocardiogram was done and showed concentric left ventricular hypertrophy (LVH) at 1.5 cm with speckling and granite-like appearance of the myocardium with no LV out flow tract (LVOT) obstruction and with normal systolic function and no significant valvular disease. A cardiac MRI was done and showed mildly dilated LV with normal geometry, normal systolic function, concentric LV hypertrophy with papillary muscles hypertrophy, relative sparing of the apical segments and with no LVOT flow acceleration and no late gadolinium enhancement.
Our patient had mild hearing loss which is maternally inherited with her mother and her maternal uncle had cochlear implants. She also had borderline diabetes mellitus and she was also found to have the m.3243 &gt; G mutation suggesting a mitochondrial disorder . A diagnosis of mitochondrial cardiomyopathy was made and the patient was started on an antihypertensive and planned to have regular cardiology clinic follow up.

Conclusion
Hypertrophic remodelling is the dominant pattern of cardiomyopathy in all forms of mitochondrial disease; occurring in up to 40% of patients and its presence is associated with higher mortality. The severity can vary from asymptomatic as in our patient to severe heart failure with acute decompensation that can occur with metabolic disorders or general illnesses. Treatment of mitochondrial disorders is mainly symptomatic with no curative therapy available. We aimed at increasing awareness of this rare disease.
Abstract P1602 Figure.
1674 Is Arterial hypertension an intermediate step to heart failure with preserved ejection fraction?
Authors: Pugliese N R , Mazzola M , Fabiani I , De Biase N , Natali A , Gargani L , Pedrinelli R , Dini F L 
Published: January 2020

Abstract

Background
Arterial hypertension (HT) is one of the main risk factors for the development of heart failure with preserved ejection fraction (HFpEF). The evaluation of cardiorespiratory fitness during the exercise may provide a clearer insight into this association.

Purpose
We assessed the hemodynamic, respiratory and metabolic characteristics of HT subjects and patients with HFpEF and HT (HFpEF-HT), combining cardiopulmonary exercise test (CPET) and exercise stress echocardiography (ESE).

Methods
We studied 170 consecutive subjects, undergoing a symptom-limited graded ramp bicycle CPET-ESE: 52 stable (NYHA I-III) outpatients with HFpEF-HT (69 ± 13 years; 44 males, 85%) on optimal medical therapy, 86 well-controlled HT subjects (66 ± 10 years; 72 males, 84%) and 32 age and sex-matched healthy controls (59 ± 15 years; 24 males, 75%). Oxygen consumption (VO2), left ventricular (LV) ejection fraction (EF), global longitudinal strain (GLS), E/e’ and LV compliance (expressed as end-diastolic volume [EDV] / E/e’ ratio) were measured during exercise, including rest and peak lung ultrasound for B-lines evaluation.

Results
HT patients showed peak VO2 values (18.7 ± 2 ml/min/kg) higher than HFpEF-HT (15.2 ± 2 ml/min/kg, p &lt; 0.0001) but lower than controls (24.4 ± 7.3 ml/min/kg, p &lt; 0.0001). Regardless of a similar EF, HT subjects had lower low-load (at 4 min of effort) GLS (18.2 ± 3%) than controls (20.9 ± 3%, p &lt; 0.0001), but higher than HFpEF-HT (16.8 ± 5%, p = 0.04). Likewise, peak diastolic function (E/e’ and LV compliance) was altered in HT patients (9.1 ± 2 and 13.4 ± 2) when compared to controls (6.2 ± 1 and 17.8 ± 2, all p &lt; 0.0001), but less than in HFpEF (12.7 ± 3, p &lt; 0.0001 and 12.1 ± 3, p = 0.003), as confirmed by peak B-lines (HFpEF-HT: 16 [interquartile range (IQR): 10 – 22], HT: 8 [IQR: 4 – 10] and controls: 0 [IQR: 0 – 2]; p &lt; 0.0001). LV hypertrophy was reported in 37 patients (27%); it was more common in HFpEF (24/37, 65%) and associated with a significantly worse peak VO2 and systo-diastolic profile (all p &lt; 0.05).

Conclusions
HT subjects represent an intermediate step from healthy subjects to HFpEF, showing altered functional capacity and systo-diastolic profile (Figure). LV hypertrophy characterises the clinical stage and portends a more severe cardiorespiratory fitness impairment.
Abstract 1674 Figure
P1684 Cardiac amyloidosis hits the valve first
Authors: Iakovlev S , Kalinskaya A I 
Published: January 2020

Abstract

Case report.
60-year-old man without previous history of coronary artery disease was admitted to our hospital. He complained of weakness, low extremities edema. Physical examination revealed also enlargement of the liver, positive hepatojugular reflux, multiple ecchymoses on patient’s face, especially in periorbital area. The arterial blood pressure was 100/60 mm Hg. ECG showed sinus rhythm 83/min, low R waves in V2, V3 with biphasic T waves in V4-V6. The last coronary angiography revealed normal coronary arteries, it was performed 6 months ago because of atypical chest pain and inconclusive stress test.
Echocardiography revealed severe tricuspid regurgitation (TR) due to leaflets restriction and malcoaptation. TR gradient was about 10 mm Hg. Interventricular septum 12 mm, left ventricle posterior wall 9 mm, mass index 87 g/m2, relative wall thickness 0.38; no LV regional wall motion abnormalities was noted, left ventricular ejection fraction (LV EF) was about 50%, mitral valve E/A ratio was 1,4, but average E/E" = 17. Left atrium volume 33 ml/m2. Insignificant amount of pericardial effusion also was found.
3D evaluation of tricuspid valve (TV) showed no leaflets defects and chordal ruptures. LV global longitudinal strain (LV GLS) was – 11,1 % with the apex/(mid + base) ratio 1,3 - apical sparing pattern. Cardiac MRI with gadolinium also showed severe TR and diffuse late subendocardial gadolinium enhancement in both ventricles. We suspected cardiac amyloidosis with significant tricuspid valve involvement, torrential TR, and right ventricle volume overload. The rectum biopsy was negative. The cardiac muscle biopsy with congo red straining was positive for amyloidosis. The patient was transferred to hematology clinic where the diagnosis of AL-amyloidosis was confirmed. The treatment with lenalidomide and prednisone was started. Unfortunately, one month later the patient died. The autopsy was not performed due to religious reasons.

Discussion
AL-amyloidosis is a systemic disease characterized by multiple organ and tissue changes and associated with poor prognosis. Cardiac involvement is a major prognostic factor as it accounts for approximately 75% of death due to heart failure or arrhythmias. Infiltration of myocardium with amyloid leads to diastolic than systolic dysfunction of the heart and to developing of the heart failure. The most common presentation of heart involvement in AL-amyloidosis is fatigue and dyspnea. In this case the main complaint of the patient was peripheral edema, echocardiography showed damaged TV and preserved LV systolic function. In literature, we found only one case report describing cardiac amyloidosis presented as severe TR.

Conclusion
In patients presenting with significant isolated valvular dysfunction and heart failure the cardiac amyloidosis can be suspected. The comprehensive echocardiography is the most useful tool to detect this problem.
Abstract P1684 Figure. 3D picture of tricuspid valve