bluuebunny/crossref_metadata_2025_split huggingface dataset:
P873 A rare and potentially treatable cause of left ventricular hypertrophy
Authors: Wong C Y 
Published: January 2020

Abstract

Introduction
A 67-year-old man was referred for care of "asymptomatic hypertrophic cardiomyopathy". He did not have hypertension. No significant positive family history could be elicited. Electrocardiogram showed sinus rhythm with voltage criteria of left ventricular hypertrophy (LVH). Outside Transthoracic Echocardiogram (TTE) reported normal ejection fraction with asymmetric septal hypertrophy without outflow obstruction. He was put on observation for few years and was not any treatment. On first encounter in our clinic, physical examination including skin and eye assessment, and laboratory tests including renal function were unremarkable.

Procedure
TTE was repeated in our clinic showing normal left ventricular size with ejection fraction 55%, and impaired diastolic relaxation. There was asymmetric septal hypertrophy with septal thickness 2.1 cm (Figure A). There was mild systolic anterior motion of mitral apparatus and mild mitral regurgitation, without resting or Valsalva provoked outflow obstruction. Global longitudinal strain was -7.7% with most prominent abnormalities seen at apex, mid to basal anteroseptal and anterior wall (Figure B). Further assessment by Cardiac MRI showed similar asymmetric septal wall thickening. Late gandolinium enhancement study demonstrated patchy fluffy hyperenhancement of the mid wall of the basal to mid anteroseptal segment, and mid to apical anterior segment, suggestive of myocardial fibrosis (Figure C1 and C2).
Dried spot blood was sent to Taiwan for enzyme study which revealed partial acid alpha-galactosidase A deficiency. Further genetic study detected a mutation of Hemizygous NM_000169.2(GLA):c.640-801[G &gt; A] at intron 4. Finally endomyocardial biopsy was done which confirmed the cardiac involvement of Fabry disease (Figure D, myelin body shown under electron microscopy). This gentleman was referred for consideration of Enzyme Replacement Therapy (ERT).

Discussion
Fabry disease is an X-linked glycolipid storage disease with accumulation of globotriaosylceramide in lysosomes in multiple cell types throughout the body leading to various organ involvement. Cardiac manifestations include unexplained LVH, valvular regurgitation, conduction abnormalities etc. It occurs in up to 0.3-5% of patients with hypertrophic cardiomyopathy. Fabry disease should be considered as a differential diagnosis in all men with sporadic or non-autosomal dominant transmission of unexplained LVH, since treatment with ERT is available which may reduce LVH and improve myocardial function, although any impact on long term outcome has not yet been established.

Conclusion
This case illustrated a rare but potentially treatable cause of hypertrophic cardiomyopathy. Myocardial strain imaging should be integrated in routine TTE study for assessment of unexplained left ventricular hypertrophy. Multi-modality imaging and multi-specialty approach help in identifying patients of cardiac variant of Fabry disease who may benefit from ERT.
Abstract P873 Figure.
P877 Multiple diverticula of left ventricule - rare atypical picture finded in asymptomatic patient
Authors: Rezek M , Groch L , Moravcova H 
Published: January 2020

Abstract
We report the case of 74 years old asymptomatic patient, who was initially sent for a transthoracic echocardiography examination, because of a new changes on his ECG - RBBB with LAH. We found atypical multiple aneurysmatic formations. Ischemic heart disease as a probable cause was suspected. The patient underwent a coronary angiography, where we found a critical stenosis of LAD and RCA. Both stenosis were treated by PCI. Atypical aneurysmatic ventricular changes were confirmed by left ventriculography and this picture didn’t correspond with ischemic cardiomyopathy. MRI confirmed multiple aneurysmatic formations of left ventricle, but without scars or fibrotic changes in aneurysmal parts of LV. The patient remained asymptomatic and our final diagnosis was multiple left ventricle diverticula - probably a congenital heart disease. The picture on echocardiography and MRI and patients clinical status remains unchanged in follow-up. LV diverticula are very rare finding and its multiple form in aged asymptomatic adult is rather very unique. MRI is crucial in differential diagnosis with ischemic aneurysmal changes and this is nicely demonstrated in our case.
Abstract P877 Figure. LV ventriculography
P888 How many diagnoses can one heart gather?
Authors: Sturzu A , Balahura A M , Dumitrascu S , Calmac L , Alexandrescu A , Vatasescu R , Badila E , Bartos D 
Published: January 2020

Abstract

Background
Apical hyperthrophic cardiomyopathy (AHCM) is an uncommon form of hyperthrophic cardiomyopathy (HCM) with less prevalent detection of gene mutations and sudden cardiac death compared with other types of HCM.

Purpose
We present the case of a 76 years old patient with multiple cardiovascular risk factors (hypertension, dyslipidemia, obesity, former smoker) with history of unprovoked pulmonary embolism - PE (2018), without evidence of deep venous thrombosis, in treatment with rivaroxaban, who presented with worsening severe dyspnea at effort and peripheral edemas, symptoms started a few days before admission. He denied angina or palpitations.

Methods
On clinical examination we identified obesity grade II and bilateral leg edema and routine laboratory tests revealed controlled dyslipidemia. The electrocardiogram (ECG) showed atrial flutter (AF) with block 5:1, heart rate 50/min, with negative T waves in DI, aVL and V2-V6. Transthoracic echocardiography with contrast was performed showing no wall motion abnormalities otherwise with a mild concentric left ventricle hypertrophy (LVH) except for the apex where there was severe LVH suggestive for AHCM; there was an increased aortic velocity with an aortic valve with degenerative changes. We thought that the changes on the ECG were most likely due to AHCM. A thoracic tomography scan with contrast was also performed because of associated dyspnea which excluded an acute recurrence of PE. The next day the T waves on ECG normalized, putting forward for consideration an acute coronary syndrome. We performed a coronarography which confirmed a 80% stenosis of proximal left anterior descending (LAD) artery with subsequent placement of a drug eluting stent. We also did a transesophageal echocardiography (noncompliant patient) for exclusion of intracardiac thrombi (which also showed bicuspid aortic valve) and then we performed radiofrequency ablation of the cavotricuspid isthmus followed by atrial overdrive pacing for typical AF, but unsuccessful, with degeneration into atrial fibrillation.

Results
Sometimes heart failure might occur due to multiple etiological factors and mechanisms of decompensation. We had a patient with AHCM with an ECG that could be interpreted as typical for this pathology but with concomitent severely affected LAD coronary artery, with AF and high grade atrioventricular block plus mild aortic stenosis with a bicuspid aortic valve with indication for strict follow up.

Conclusions
AHCM was first described in Japan where has the highest prevalence, but is also documented in other countries (rare in Caucasian population). Note that AHCM often mimic acute coronary syndromes through clinical manifestations and electrocardiographic aspects. Our case report showed a Caucasian patient with AHCM with concomitent severe atherosclerotic desease and aortic stenosis on a bicuspid valve.
P907 Ventriculo-vascular interaction in patients with severe aortic stenosis: a comparison of three different clinical settings
Authors: Vijiiac A E , Neagu C , Cherry A , Onciul S , Zamfir D , Onut R , Stoian M , Iancovici S , Dorobantu M 
Published: January 2020

Abstract

Funding Acknowledgements
This work was supported by CREDO Project - ID: 49182, financed through the SOP IEC -A2-0.2.2.1-2013-1 cofinanced by the ERDF
Ventriculo-arterial coupling (VAC) reflects the interaction between the ventricle and the arterial system and its prognostic role was studied in different clinical settings. VAC can be assessed with echocardiography as the ratio between the arterial elastance (Ea) and the end-systolic left ventricular elastance (EES). Data concerning the role of VAC in severe aortic stenosis (AS) are scarce.
We aimed to determine VAC in patients (pts) with severe AS and assess its relationship with symptoms.
We included 61 consecutive pts with severe AS (vave area &lt; 1 cm2) and we divided them in 3 groups according to their symptoms: group A consisted of 28 pts (81 ± 10 yrs, 14 men) admitted for acute pulmonary edema, group B consisted of 25 pts (76 ± 12 yrs, 13 men) with either angina, syncope or dyspnea class I-III NYHA and group C consisted of 8 asymptomatic patients (71 ± 19 yrs, 3 men). We determined the VAC non-invasively and we compared the results between the 3 groups using one-way analysis of variance and a post-hoc Tukey test.
There were no significant differences in age (p = 0.08) and aortic valve area (p = 0.18) between groups. Variations of Ea and EES between groups were not significant (p = 0.08 and p = 0.94, respectively). However, VAC differed significantly between the 3 groups, being most impaired in group A (1.11 ± 0.69), followed by 0.77 ± 0.23 in group B and 0.73 ± 0.16 in group C (p = 0.03). The left ventricular ejection fraction (EF) also differed significantly between groups: 41 ± 13% in group A, 51 ± 11% in group B and 57 ± 3% in group C (p &lt; 0.001). VAC and the EF had a moderate negative correlation in group A (r=-0.52, p = 0.004) and group B (r=-0.51, p = 0.009), but no correlation in group C (p = 0.37).
VAC is impaired in patients with severe AS and acute heart failure and it differs significantly from VAC in severe AS with chronic, stable symptoms and from VAC in asymptomatic severe AS. This suggests that the progression of symptoms in severe AS might be related to the interactions between the left ventricle and the vascular load, making thus VAC a potential therapeutic target and a parameter to be considered in the thorough evaluation of patients with severe AS.
Group A Group B Group C Ea 2.69 ± 1.31 2.09 ± 0.94 1.95 ± 0.60 p = 0.08 EES 2.93 ± 1.90 2.87 ± 1.33 2.71 ± 0.73 p = 0.94 VAC 1.11 ± 0.69 0.77 ± 0.23* 0.73 ± 0.16* p = 0.03 EF 41 ± 13% 51 ± 11%* 57 ± 3%* p &lt; 0.001 *significant difference with group A
P940 Usefulness of the subendocardial and subepychardial longitudinal strain in the differential diagnosis between cardiac amyloidosis and hypertrophic myocardiopathy
Authors: Ramos Polo R , Moral Torres S , Tiron De Llano C , Morales Fornos M , Frigola Marcet J M , Albert Beltran X , Robles Fernandez R , Graterol Torres F , Vilardell Rigau P , Brugada Terradellas R 
Published: January 2020

Abstract
INTRODUCTION
Differential diagnosis by echocardiography between cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM) is based on the evaluation of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) of the entire myocardial wall. Nevertheless, histopathological studies describe a higher involvement of subendocardial tissue in CA. The aim of our study was to evaluate whether the subanalysis of the GLS by layers (subendocardial and subepicardial) and segments (apical and basal) can provide further information.
METHODS
Retrospective study including 33 consecutive patients diagnosed with CA (with histological confirmation and imaging tests) or HCM by established criteria. Advanced myocardial deformation analysis software was used for both subendocardial and subepicardial evaluation of the left ventricle wall by transthoracic echocardiography.
RESULTS
Seventeen patients (52%) had CA and sixteen (48%) had HCM. Differences were observed in LVEF (52.9 ± 10.9% vs 62.4 ±5.0%; p = 0.004), but not in the analysis of the entire wall GLS (-12.3 ± 4.9 vs -13.4 ± 2.8; p = 0.457) nor in the LVEF/GLS ratio (4.7 ± 1.4 vs 4.8 ± 1.1; p = 0.718). In the layered analysis there was no difference in subendocardial GLS (-16.2 ± 5.0 vs -16.4 ± 3.2%; p = 0.916) or subepicardial GLS (-11.7 ± 4.1 vs -11.6 ±2.7%; p = 0.945); however, the increase in GLS from base to apex was greater for CA than for HCM both at subepicardial level (increase: 101% vs 16%; p = 0.006) and subendocardial level (increase: 242% vs 114%; p = 0.006), with inversion of the greatest values for each group (Fig. 1).The ratio (apical GLS/basal GLS) was diagnostic predictor of CA (area under the curve = 86%; p = 0.002): a value &gt;2 presented a sensitivity of 84% and a specificity of 85% for the diagnosis of CA.
CONCLUSIONS
CA presents an impairment of both subendocardial and subepicardial deformation in transthoracic echocardiography. These patterns provide additional information on differential diagnosis with HCM.
Abstract P940 Figure. Subendo vs subepicardial mean values
P942 Outcome prediction by exercise stress echocardiography and cardiopulmonary exercise testing assessment in patients with heart failure
Authors: Sakata K , Mitsuda H , Ito J , Isaka A , Gouda A , Soejima K 
Published: January 2020

Abstract

Background
Cardiopulmonary exercise testing (CPX) is essential to the assessment of functional impairment and prognosis in patients with heart failure (HF). Peak oxygen consumption (peak VO2) continues to be considered the gold standard for assessing prognosis in HF. The minute ventilation-carbon dioxide production (VE-VCO2) slope has recently demonstrated prognostic significance in patients with HF. Ergometer stress echocardiography (Erg-Echo) is useful to evaluate the exercise-induced pulmonary hypertension and the potential cardiac dysfunction that are difficult to evaluate in the resting state.

Objective
The aim of this study is to evaluate the relationship between CPX and Erg-echo indices, and the usefulness of Erg-echo to determine the severity of cardiac dysfunction and the prognosis in patients with HF.

Methods
We studied 58 patients with HF (age 65.2 ± 11.9 years) and performed CPX and Erg-Echo. The peak VO2 and the VE-VCO2 slope were measured by CPX. Cardiac output (CO) and estimated mean pulmonary artery pressure (mPAP) were measured by Erg-Echo at rest and peak exercise load, and the change ratio (ΔmPAP / ΔCO) were calculated. We evaluated the clinical outcome during a1 year period.

Results
The ΔmPAP / ΔCO was significantly correlated with the peak VO2 (R = -0.6767, P &lt;0.0001) and the VE-VCO2 slope (R = 0.6809, P &lt;0.0001). Cardiovascular events (1 patient of cardiovascular death, 8 patients of re-hospitalization due to HF, 4 patients of myocardial ischemia, 2 patients of Cardiac Resynchronization Therapy (CRT) devices implantation, 1 patient of ventricular tachycardia) developed in 16 of the 58 patients (27.5%: Group CE) during the 1 year. The peak VO2 was significantly lower (12.1 ± 2.5 vs. 16.1 ± 3.1ml/min/kg, P &lt;0.0001) and the VE-VCO2 slope was significantly higher (41.1 ±. 12.3 vs. 31.8 ± 6.1ml/ml, P &lt;0.0001) in Group CE compared to the other 42 patients (Group N). The ΔmPAP was significantly higher (19.1 ± 4.4 vs. 14.9 ± 6.4, P = 0.0408) and the ΔCO was significantly lower (2.4 ± 1.2 vs. 4.1 ± 2.0, P = 0.0078), and the ΔmPAP / ΔCO was significantly higher (9.7 ± 4.6 vs. 4.4 ± 2.4, P &lt;0.0001) in Group CE compared to Group N.

Conclusions
The ΔmPAP/ ΔCO by Erg-Echo is useful to evaluate the severity of cardiac dysfunction and the prognosis of HF patients.
P966 Impact of systolic dysfunction and elevated left ventricular end diastolic pressure on three years clinical outcome in patients with atrial fibrillation
Authors: Ahn J , Yu H Y 
Published: January 2020

Abstract

Background
Systolic and diastolic dysfunction is related with adverse clinical outcomes in the patients with sinus rhythm. Purpose: The aim of this study is to clarify the prognostic significance of both systolic and diastolic dysfunction in the patients with chronic persistent atrial fibrillation (AF). Methods: A total of 114 consecutive patients who have chronic persistent AF. Whole patients were divided into 2 groups according to left ventricular ejection fraction (LVEF): those with an LVEF &lt; 50 (n = 24) (REF) and those with an LVEF ≥ 50 (n = 90) (PEF). And PEF group was also divided into two groups according to left ventricular end diastolic filling pressure (LVEDP): patients with LVEDP ≥ 15 mmHg (n = 38) and those with &lt; 15 mmHg (n = 52). Results: 3-year clinical outcomes were compared between each groups (PEF groups vs. REF groups and LVEDP ≥ 15 mmHg vs LVEDP &lt; 15 mmHg). The incidence of death, hospitalization, stroke, bleeding, AF with rapid ventricular rhythm (RVR) and heart failure (HF) hospitalization were similar PEF and REF group. However, during 3-year follow up period, the incidence of HF hospitalization (29.2% vs 8.9%, p &lt; 0.02) and AF with RVR (20.8% vs 3.3%, p &lt; 0.01) were frequent in REF group compared with PEF group. In multivariate analysis, REF is an only predictor of HF hospitalization (Table 1). Conclusion: During 3-year follow up period, systolic dysfunction is an important predictor of HF hospitalization in AF patients. However, elevated LVEDP is not related with 3-year adverse clinical outcomes in AF patients without systolic dysfunction.
Table 1 variable Odd Ratio (HR) 95% Confidence Interval (CI) P Age .973 .925-1.023 .286 Diabetes mellitus .487 .138-1.721 .264 BNP 1.000 1.000-1.000 .908 Hypertension 1.061 .330-3.413 .921 LVEDP &gt; 15 mmHg 1.302 .396-4.285 .664 EF &lt; 50 4.712 1.478-15.016 .009 Predictors of 3-year follow-up clinical outcomes of all participants
1045 T1-mapping and CMR feature tracking in mitral valve prolapse
Authors: Guglielmo M , Fusini L , Muscogiuri G , Baggiano A , Loffreno A , Baessato F , Bonalumi G , Zanobini M , Alamanni F , Pepi M , Pontone G 
Published: January 2020

Abstract

BACKGROUND
Several studies suggest that mitral valve prolapse (MVP) can be related to sudden cardiac death, owing to sustained ventricular arrhythmias (VAs). In patients with sudden cardiac death and complex VAs, a high percentage of either left ventricle (LV) papillary muscle fibrosis or inferobasal fibrosis has been described using cardiac magnetic resonance (CMR) with late gadolinium enhancement technique (LGE). However, LGE presents several technical limitations and requires contrast agent administration. Thanks to T1 mapping (T1-map) and feature tracking (FT) techniques, CMR may identify myocardial fibrosis and deformation abnormalities respectively. We sought to demonstrate that, in patients with MVP, T1 map can accurately identify the presence of myocardial fibrosis which, being related to myocardial stiffness, is associated to abnormal deformation indexes at CMR FT strain evaluation.

METHODS
Consecutive patientswith indication to mitral valve surgery for severe mitral regurgitation due to mitral valve prolapse were prospectively enrolled. CMR including Modified Look-Locker (MOLLI) sequences for T1 mapping was performed in each patient. In addition, CMR FT analysis of steady state free precession (SSFP) cine images was performed to obtain 2D global and segmental circumferential and radial strains.

RESULTS
70 consecutive patients (age: 59 ± 12) were successfully evaluated with CMR. T1 native values were significantly higher in the basal and mid LV inferolateral wall compared to the remote myocardium (1074 ± 67 vs 1046 ± 40 msec, p&lt; 0.001). Moreover, the average radial and circumferential strains of the basal and mid LV inferolateral were significantly reduced compared to those of the remote myocardium (21.1 ± 10.4 and -12.8 ± 5.6 vs 31.6 ± 9.1 and -17.3 ± 3.6 respectively, p &lt; 0.001).

CONCLUSIONS
In patients with MVP and severe mitral regurgitation native T1 values of the LV inferolateral are higher as compared to remote myocardium and associated with reduced circumferential and radial strains. T1 mapping and CMR FT strain may be used as tools for the early identification of tissue changes in the LV inferolateral myocardial segment. Further studies are needed to evaluate if these changes are able to predict LGE development and are associated with higher risk for VAs
1232 Acute myocarditis: prognostic role of speckle tracking echocardiography and cardiac magnetic resonance
Authors: Trovato R L , La Franca E , Nugara C , Di Lisi D , Zarcone A , Bellavia D , Carmina G , Clemenza F , Novo S , Novo G 
Published: January 2020

Abstract

Background
Myocarditis is an inflammatory disease of the myocardium predominantly caused by infection with subsequent immunological response. Cardiac magnetic resonance (CMR) is the currently best imaging modality to confirm a suspected diagnosis of myocarditis. Conventional echocardiography can detect regional or global wall motion abnormalities but it could also be negative. Speckle tracking echocardiography can help to identify subtle systolic dysfunction, in patients with myocardits and apparently negative echocardiogram.

Purpose
the aim of this study was to identify cardiac imaging parameters predictive of cardiovascular events in patients with acute myocarditis.

Methods
a prospective study was carried out using 76 patients with acute myocarditis. Cardiological evaluation including echocardiogram and CMR with quantitative analysis of edema and delayed enhancement (DE) was performed in all patients at the hospital admission.
In 43 patients we assessed multilayer 2D speckle tracking analysis measuring GLS at three levels (mid-wall layer, endocardial and epicardial) and mechanical dispersion (MD). We assessed cardiovascular events during hospitalization in all patients (arrhythmias, heart failure, cardiogenic shock, syncope).

Results
we found significant higher value of DE mass in patients with cardiovascular events compared to patients without cardiovascular events (20,23 ± 11,47gr vs 11,58 ± 9,54gr; p = 0,021). GLS and multilayer strain at each level was significantly lower in patients with events than in patients without events (GLS epicardial -16,29 ± 1,76% vs -14,54 ± 2,19%; p = 0,013; GLS mid-wall -18,22 ± 1,94% vs -16,27 ± 2,48%; p = 0,013; GLS endocardial -19,83 ± 1,91% vs -17,81 ± 2,65%; p = 0,011).
MD was significantly higher in patients with cardiovascular events (48.50 ± 15.79 ms vs 34.12 ± 6.22ms; p &lt; 0,001). Logistic regression analysis showed that DE is a good predictor of events (ROCarea 0,81-OddsRatio 1,09). Also GLS seems to be predictive of events (GLS epicardial: ROC area 0,72-OddsRatio 1,57; GLS mid wall: ROC area 0,73-OddsRatio 1,52; GLS endocardial: ROC area 0,72-OddsRatio 1,48).
With segmental analysis, inferior and lateral walls seemed to be more predictive of events. Segment 9 had a good predictability in all layers (epicardial: ROCarea 0,8-OddsRatio 1,69; mid wall: ROCarea 0,83- OddsRatio 1,77; endocardial: ROC area 0,82-OddsRatio 1,69).
MD was a slight predictor of events with ROC area &lt; 0,7.

Conclusions
Speckle tracking echocardiography could be used in addiction to cardiac magnetic resonance to get more information about prognosis and events risk stratification in patients with acute myocarditis.