bluuebunny/crossref_metadata_2025 huggingface dataset:
114 A case of progressive wall thinning of apical myocardium in 5 year- old boy with hypertrophic cardiomyopathy
Authors: Jung J W
Published: January 2020

Hypertrophic cardiomyopathy (HCM) is defined by the presence of increased left ventricular (LV) wall thickness that is not explained by abnormal loading conditions without a priori assumptions about etiology or myocardial pathology even in neonate, children and adults. The diagnosis of HCM is mainly the detection of increased LV wall thickness by any imaging modality. Due to its diverse etiology, the disease phenotype includes chest pain, palpitation, syncope and heart failure. In most patients, there is a life-long process of progressive and adverse cardiac remodeling, characterized by myocardial fibrosis and wall thinning. However, there was no report about cardiac remodeling of progressive wall thinning in children with HCMP.

A 5 year-old boy was evaluated due to familiar HCMP, and finally confirmed with HCMP by echocardiography. His father was diagnosed as asymmetric HCMP and treated with septal alcohol ablation 3 years ago. His family referred for genetic counselling. His symptom was vague because he was very young. Initial electrocardiographic finding was normal sinus rhythm with left ventricular hypertrophy. Ambulatory electrocardiography (ECG) showed isolated premature ventricular contraction. Initial echocardiographic findings revealed asymmetric septal hypertrophy with diffuse hypertrophied LV myocardium, systolic anterior motion of anterior leaflet of mitral valve without LV outflow tract obstruction (LVOTO) and only mild dynamic obstruction on mid-septum of LV. His cardiovascular magnetic resonance imaging (CMR) showed similar to echocardiographic findings and no late gadolinium enhancement (LGE). He started to take medication with beta blocker, and later addon verapamil. After 3 years later, his ambulatory ECG showed non-sustained ventricular tachycardia of 4 episodes. Follow-up echocardiographic findings revealed progressive septal wall thickness from 13mm to 19 mm, marked LVOTO with peak pressure gradient of 128 mmHg and profound dynamic obstruction on mid-septum of LV. Follow-up CMR imaging showed additional progressive apical wall thinning without suspicious or definite LGE on any other myocardium. Urgently he undertook the implantation of implantable cardioverter defibrillatorsfor prevention of sudden cardiac death. Now he is listing and waiting for heart transplantation. Cardiac remodeling is progressively life-long process in most HCMP, but in this case with pediatric HCMP, even though he took medical treatment its progression was rapid cardiac remodeling.
153 Multiparametric assessment of diastolic function in heart failure
Authors: Uejima T , Cho J , Hayama H , Takahashi L , Yajima J , Yamashita T
Published: January 2020

Background

The assessment of diastolic function is still challenging in the setting of heart failure (HF). We tested the hypothesis that applying a machine learning algorithm would detect heterogeneity in diastolic function and improve risk stratification in HF population.

Methods

This study included consecutive 279 patients with clinically stable HF referred for echocardiographic assessment, for whom diastolic function variables were measured according to the current guidelines. Cluster analysis, an unsupervised machine learning algorithm, was undertaken on these variables to form homogeneous groups of patients with similar profiles of the variables. Sequential Cox models paralleling the clinical sequence of HF assessment were used to elucidate the benefit of cluster-based classification over guidelines-based classification. The primary endpoint was a hospitalization for worsening HF.

Results

Cluster analysis identified 3 clusters with distinct properties of diastolic function that shared similarities with guidelines-based classification. The clusters were associated with brain natriuretic peptide level (p < 0.001, figure A). During follow-up period of 2.6 ± 2.0 years, 62 patients (22%) experienced the primary endpoint. Cluster-based classification exhibited a significant prognostic value (c2 = 20.3, p < 0.001, figure B), independent from and incremental to an established clinical risk score for HF (MAGGIC score) and left ventricular end-diastolic volume (hazard ratio = 1.677, p = 0.017, model c2: from 47.5 to 54.1, p = 0.015, figure D). Although guideline-based classification showed a significant prognostic value (c2 = 13.1, p = 0.001, figure C), it did not significantly improve overall prognostication from the baseline (model c2: from 47.5 to 49.9, p = 0.199, figure D).

Conclusion

Machine learning techniques help grading diastolic function and stratifying the risk for decompensation in HF.

Abstract 153 Figure.
159 LV volumes, EF, MAPSE, myocardial mass and systolic compression and relation to ageing. Results from the HUNT study
Authors: Stoylen A , Molmen H E , Dalen H E
Published: January 2020

Background

Myocardial mass increase with age, but the impact of recently discovered length reduction by age has not been taken into account. Long axis shortening is considered the main contributor to LV stroke volume. There is still disagreement about the relative contributions of cross sectional vs long axis shortening. Long axis shortening decrease with age, but EF do not. It has been postulated that this is due to increased transverse function, but this has not been demonstrated. Also, the degree of systolic myocardial compression is unresolved.

Methods

The HUNT study examined 1266 subjects without evidence of heart disease from a mixed urban/rural population of North Trøndelag county, Norway. Linear dimensions of diastolic left ventricular annulus to apex distance, systolic and diastolic wall thicknesses (WT), chamber diameter (LVID), and mitral annular plane displacement (MAPSE) were measured. Measurements were applied to a half-ellipsoid model of the left ventricle with assumption of 50% thinner apex than midway thickness, for calculations of left ventricular length (LVL), LV cavity volume (LVEDV), stroke volume (SV) and EF as shown in fig. 1.

Results

Dimensions and volumes are given in table 1. LV internal diameter was unchanged by age, and so was fractional shortening (FS). Wall thickness increased by age (p < 0.001), corresponding to an increase in external diameter, but LVL decreased (p < 0.001), so increase in myocardial volume (MyoV) although significant (p < 0.001), was less. Wall thickening decreased from 59 to 52% by age group (p < 0.001). MAPSE decreased by age (p < 0.001). Similarly systolic external diameter reduction (from 13.6 to 11.9%), and SV also decreased by age, while EF was unchanged. MAPSE contributed 74% to stroke volume, external diameter reduction to 26% These relative contributions did not change by age. Systolic myocardial compression was 2.5%, at all ages.

Conclusion

LVEDV decreases with age due to LV shortening, not wall thickening. EF is maintained by a simultaneous reduction in SV and EDV. MAPSE decreases by age, and so does transverse function. Thus, both are decreasing in proportion, and the reduction in SV is due to both, not to any increase in transverse function.

Table 1 Age (years) WT (mm) LVL (mm) LVID (mm) LVEDV (ml) MyoV (ml) MAPSE (mm) SV (ml) EF (%) <40 8.1 (1.3) 94.2 (7.5) 50.9 (4.9) 124.0 (30.0) 101.4 (27.9) 17.3 (2.0) 83.7 (21.4) 67 (6) 40 - 60 8.9 (1.4) 92.9 (8.1) 50.9 (5.4) 122.5 (31.0) 111.2 (29.2) 15.8 (2.3) 82.4 (22.8) 67 (6) >60 9.5 (1.4) 89.0 (7.9) 50.3 (6.1) 113.8 (31.7) 113.9 (31.7) 14.0 (2.2) 76.0 (24.1) 66 (9) 8.8 (1.4) 92.4 (8.1) 50.8 (5.4) 121.1 (31.1) 109.2 (29.1) 15.8 (2.5) 81.4 (22.9) 67 (8) Diastolic dimensions volumes and functional measures by age.

Abstract 159 Figure 1
P169 Uterine leiomyoma extension into right side cardiac
Authors: Diniz Ferrer K , Silveira CARLOS , Reis ADRIAN , Abreu E Lima PAULA , Diniz ROBERT , Navarro CAROL , Silva JUCIAN , Melo MARCIA
Published: January 2020

Funding Acknowledgements

governmental grants

Uterine leiomyoma is a commom disease in women, however, intravenous leiomyomatosis of uterine origin extending via inferior vena cava into the right side of the heart,known as intracardiac leiomyomatosis is a rare condition (3%). In 1907, Durk reported the first case of intracardiac extension.

The patient was a Woman, 35 years old, admitted to our emergency department for an intracardiac mass. She had shortness of breath,fatigue and chest pain. The transthoracic two dimensional echocardiography showed an echogenic oval mass mobile in right atrium and projected through right ventricle in diastole. This mass was observed to extend from inferior vena cava to the right atrium. The echotransesophageal three dimensional showed a large mobile mass that extended from inferior vena cava to the right atrium. A Computer tomographic (CT) scan showed a hypodense multilobulated mass in the pelvis, which had invaded the inferior vena cava and right atrium. The patient underwent a two stages surgery. In first stage (transatrial tumor resection). The operation was performed normal temperature with establisment of cardiopulmonary bypass (CPB). Subsequently, the pathological report was confirmed uterin smooth muscle origin. The second stage surgery ( total histerectomy) was done four weeks later for removing lobulated mass uterin with dimensions 20x15x7.5cm with confirmed histopathological of leiomyoma.

Because of it is nonspecific clinical presentation and rarity, an intracardiac Leiomyomatosis continues to be a misdiagnosed as either thrombus or myxoma. The cardiac imaging techniques like a transthoracic echocardiography 2d and transesophageal echocardiography 3d have been used to define the presence, extension of tumor as appearance of the mass and involvement of adjacent structures.

Abstract P169 Figure. Echotransesophageal 3D (bicaval view)
P181 An uncommon mechanism of severe mitral regurgitation due to infective endocarditis mimicking acute myocardial infarction
Authors: Collevecchio A , Simeti G , Previtero M , Iliceto S , Muraru D , Badano L P
Published: January 2020

A 53-year-old man, smoker, with diabetes mellitus, presented to the Emergency Department because of intense chest and abdominal pain, accompanied by dyspnea and high fever (39.5 °C) in the previous 4 days. Physical examination revealed an apical holosystolic murmur, with no signs of peripheral or pulmonary edema. An ECG showed sinus rhythm (90 bpm), complete right bundle branch block and minimal ST elevation in the inferior leads. A transthoracic echocardiography showed a mild reduction in left ventricle ejection fraction (EF 44%) due to akinesia of the infero-lateral wall, and mild mitral regurgitation (MR) due to mitral valve prolapse. An abdominal ultrasound ruled out signs of acute cholecystitis. Blood cultures were collected, and an empirical antibiotic therapy was started. Urgent blood exam showed high Troponin I (72000 ng/L) and high C-reactive protein (290 mg/L).

An acute coronary syndrome was suspected based on clinical, ECG and echocardiography exam, and the patient underwent coronary angiography (Figure 1, Panel A) that showed no significant coronary stenosis, except for two small filling defects in the very distal part of both the left anterior descendent and the circumflex coronary arteries suspected for coronary emboli. The patient was then admitted in the coronary care unit, but after just a few hours his clinical and hemodynamic condition deteriorated. A transesophageal echocardiography was performed to rule out mechanical complications related to the acute myocardial infarction and revealed severe MR (Panel D), elongated, hyperechogenic and dysfunctioning antero-lateral papillary muscle (ALPM) with an abnormal mobility suggestive for myocardial abscess, and a mobile mass attached on the aortic valve suggestive for vegetation (Panel B and C). Due to the worsening hemodynamic status, the patient underwent urgent cardiac surgery. Histological analysis confirmed the presence of an abscess of the ALPM due to Staphylococcus Aureus. The patient died after a week because of cerebral hemorrhage. Autopsy reported multiple lungs, renal and cerebral embolic septic infarctions.

Learning points

coronary artery embolization and papillary muscle abscess are very rare and often fatal consequences of infective endocarditis (IE). High (otherwise unexplained) fever and signs of embolism are minor Duke modified criteria for IE that should lead the physician to look for major criteria, such as positive blood cultures or echocardiography suggestive for IE. Emboli seen in the very distal part of the coronary arteries might have caused the ALPM abscess.

Abstract P181 Figure
P183 Cor medusae
Authors: Vladova M , Tasheva I , Simova Y , Petrov I , Polomski P , Mihailov M
Published: January 2020

A 75-years-old man presented in our cardiology service with easy fatigue, shortness of breath and palpitations for last two months. His ECG showed atrial fibrillation and LBBB. Until now he was not anticoagulated. We performed first transthoracic echocardiography and found reduced EF - 33%, with global hypokinesia of the LV and LV hypertrophy. Many atrial and ventricular septal perforating arteries. Dilated right coronary sinus. Mild mitral, aortic and tricuspid regurgitation with no pulmonary hypertension. We identified a strongly curved vascular structure in right atrioventricular groove. We performed coronary CT angiography and found abnormal coronary vessels "Cor Medusae" (coronary arteriovenous fistula). Giant RCA - 31mm in diameter with many branches, one of them has comuniction with left atrium and one of the other comunicate with truncus pulmonalis. LAD, LCx and D1 came from truncus pulmonalis. A very rare congenital condition. On a cardiology discussion we decided to apply conservative treatment.

Abstract P183 Figure. CT reconstruction cor medusae
P191 Pellets in rigth ventricle. Without entry point?
Authors: Pinillos Francia G , Gutierrez Fernandez A , Alvarez Roy L , Ruiz Zamora I , Gomez Llorente M , Sufrate Sorzano E , Alonso Perez L , Aguiar Souto P , Azcarate Aguero P M
Published: January 2020

Case Report

A 39-year-old male patient was transferred to emergency room by mobile intensive care unit after receiving a firearm wound in the neck while hunting: accidentally hit by a shotgun. Physical examination highlighted the existence of multiple entry holes at right side face and neck and some above the shoulder girdle line, the rest of the thorax and abdomen did not present skin lesions. Axial tomography (CT) showed multiple pellets in the face and neck tissues and two intracardiac pellets in the right ventricle. He did not present pneumothorax, pneumopericardium or pericardial effusion. He underwent surgical intervention by otolaryngology and vascular teams due to right cervical hematoma and secondary alterations to the shot in the right internal jugular vein. Enucleation of the right eye was also necessary. Echocardiographic study showed the presence of a small hyperechogenic mass with posterior acoustic shadow located in interventricular septum. Another similar structure was also found, in the right ventricle, next to the tricuspid annulus: normal tricuspid valve function. Considering the trajectory (blue triangle) of the shot and the absence of thoracic complications, we thought about the plausibility of projectile embolization from the right internal jugular vein.

Discussion

The presence of projectiles in different cardiac structures can occur by direct impact (the most frequent mechanism), passing through thoracic and/or abdominal structures to be lodged in the pericardium and/or myocardium. They are often accompanied by manifestations such as pericardial effusion or pneumothorax as a result of their trajectory. Embolization to the heart is a much more uncommon mechanism by impacting in another anatomical location such as the neck or lower extremitie. In these cases there may be no extra-cardiac manifestations and can be an incidental finding of a previous event. In our case the intracardiac pellets were s was handled conservatively and the rest of the wounds produced by the shot showed a good evolution. The patient remained asymptomatic from a cardiological point of view during hospitalization and follow up. There is no evidence to support the therapeutic attitude that should be followed in these cases. When the patient is stable and the projectiles are non-mobile, conservative treatment with periodic follow-up is a suitable option.

Abstract P191 Figure. A. 3D echo, B. Xplane, C. CT VR.
P200 The impact of left venticule ejection fraction on long-term outcome in patients with high- and low-gradient severe aortic stenosis - data from ASRegistry
Authors: Strozyk A , Marlega J , Nowak M , Galaska R , Fijalkowski M
Published: January 2020

Background

Aortic stenosis (AS) is the most common primary valve disease leading to surgery or catheter intervention with a growing prevalence due to the ageing population. Severe aortic stenosis with reduced transaortic flow and gradient although it is a common finding still remains diagnostic challenge.

Purpose

The aim of study was to analyse the outcomes of patients with high- and low-gradient aortic stenosis depending on the ejection fraction.

Methods

621 patients hospitalized in the First Department of Cardiology, with severe aortic stenosis defined as aortic valve area <1cm2 were enrolled to the Aortic Stenosis Registry (ASRegistry). The high-gradient aortic stenosis (HG-AS) [mean transvalvular pressure gradient (PGmean) ≥40mmHg and peak transvalvular velocity (Vmax) ≥ 4 m/s] and low-gradient aortic stenosis (LG-AS) [mean transvalvular pressure gradient (PGmean) <40mmHg and peak transvalvular velocity (Vmax) <4 m/s] were observed in 54%(n = 340) and 45% (n = 281) patients, respectively. In the subgroup of HG-AS were 80% (n = 275) and in the subgroup of LG-AS were 61%(n = 174) of patients with preserved left ventricle ejection fraction (LVEF > 50%). The patients were observed for a period of 6 years (2012-2018). The primary end-point, all-cause mortality, was obtained from Nation Health Registry.

Results

Patients with LG-AS had a significantly higher risk of mortality compared to patients with HG-AS: 35% (n = 101) vs 26% (n = 87), p < 0,05. Mortality in both group with HG-AS and LG-AS was significantly higher in the subgroup with diminished left ventricle dysfunction than in the subgroup with preserved ejection fraction: HG-AS: 44%(n = 29) vs 21%(n = 58), p < 0,05 and LG-AS: 53%(n = 57) vs 25%(n = 44), p < 0,05. The highest mortality rates was observed in the LG-AS group with left ventricule dysfunction, p < 0,001.

Conclusions

The study shows the negative impact of left ventricle dysfunction in both groups of patients: high- and low-gradient severe aortic stenosis. The worst prognosis is in patients with LG-AS and low LVEF.
P202 Two-dimensional and three-dimensional transesophageal echocardiography in the diagnosis of infective endocarditis of native and prosthetic valves
Authors: Capotosto L , Tanzilli G , Mangieri E , Ciardi M R , Gaudio C , Miraldi F , Vullo V , Vitarelli A
Published: January 2020

Purpose

The purpose of this study was to examine the incremental value of three-dimensional transesophageal echocardiography (3D-TEE) compared to two-dimensional transesophageal echocardiography (2D-TEE) in the diagnosis of infective endocarditis (IE) of native and prosthetic valves.

Methods

Forty-three patients with clinically suspected IE were studied. The patients had clinical, microbiological and echocardiographic assessment to establish a diagnosis of IE in accordance to current guidelines recommendations. Presence, location and size of vegetations, new or progressive valve regurgitation, possible chordae tendineae rupture, paravalvular extension, and new dehiscence of a valve prosthesis were assessed by echocardiography.

Results

In 25 (58%) patients the diagnosis of IE was established. Thirteen patients had native valves and 12 patients had prosthetic valves (9 mechanical, 3 biological). 2D-TEE and 3D-TEE showed a sensitivity, specificity, positive and negative predictive value for diagnosis of IE of 91% and 89%, 88% and 91%, 84% and 86%, and 93% and 90%, respectively, in native valves, and of 92 and 90% 91% and 97% (p = 0.002), 84% and 82% and 89% and 95% (p = 0.01), respectively, in patients with prosthetic valves. Major vegetation diameter was 18mm for 3D-TEE and 16mm for 2D-TEE in native valves and 19mm for 3D-TEE and 14mm for 2D-TEE in prosthetic valves (p = 0.04). Peri-annular extension was detected by any of the echocardiographic modalities in three patients with native valve IE and two patients with prosthetic valves, and by 3D-TEE only and not by 2D-TEE in one patient with prosthetic valve.

Conclusions

Patients with prosthetic valve infective endocarditis seem to have more additive benefit from 3D-TEE compared to patients with native valve IE.
P208 Abnormal coronary flow reserve assessed with stress echocardiography induces a reduction in transmural strain gradient and an increase in heterogeneous myocardial electrical activation
Authors: Sierra Lara M J D , Rodriguez-Zanella H , Calvillo O , Posada E L , Ortiz X O , Arroyo C , Raymundo G , Ruiz Esparza M E , Fritche J F , Wierzbowska-Drabik K , Gaibazzi N , Picano E , Arias J A
Published: January 2020

OnBehalf

Echo Lab INC

Background

The integrated quadruple stress echo (IQ-SE) is a state-of-the-art protocol that expands the risk stratification potential of SE. The coronary flow reserve (CFVR) has pronostic implication mainly in the absence of wall motion abnormalities. Longitudinal multilayer strain analysis assesses trasmural strain gradient. The prognostic value of multilayer strain analysis alongside the IQ-SE has not been established. We aim to determine the additive value of multilayer strain analysis during IQ-SE.

Methods

Prospective observational study. We evaluated one hundred twenty intermediate-high risk patients without obstructive coronary artery disease (mean age 61 ± 12 years, female 43%) with IQ-SE dipyridamole at our institution. Abnormal CFVR was defined as CFVR <2. Multilayer strain analysis and mechanical dispersion, were performed during rest and stress, with an automatic frame-by-frame with speckle tracking.

Results

The IQ-SE was feasible in all patients. At rest, mean three-dimensional left ventricular ejection fraction (3D LVEF) was 59% (53-62) without wall motion abnormalities. Abnormal CFVR was seen in 38 patients (32%) and reduced left ventricular contractile reserve (LVCR) in 56 patients (46%). Patients with abnormal CFVR, showed a reduction in EndoLS and transmural strain gradient, and an increase in mechanical dispersion and in lung B-lines during stress. No differences were observed in 3D LVEF and LVCR.

Conclusions

Endocardial ischemia due to microvascular dysfunction leads to a reduction in transmural strain gradient. Adding multilayer strain analysis to IQ-SE might detect myocardial subclinical dysfunction, arrhythmic risk and pulmonary congestion due to microvascular dysfunction.

Left ventricular mechanics analysis CVFR <2 (38) CVFR >2 (81) P Rest EpiLS (%) -14.9 ±4.2 -18 ± 3.7 0.001 EndoLS (%) -20.2 ± 5.9 -23.5 ± 4.5 0.001 EndoLS- EpiLS 5.1 (3.8-6.3) 5.4 (4.2-6.3) 0.27 Mechanical Dispersion (ms) 57.7 (42.2-69) 44.2 (35-54) 0.006 Stress EpiLS (%) -17.2 ± 4.8 -20 ± 4.4 0.003 EndoLS (%) -23 ± 6 -26.6 ± 5.7 0.002 EndoLS- EpiLS 5.6 (4.5-6.7) 6.3 (5.4-7.9) 0.01 Mechanical Dispersion (ms) 47 (39.5 - 59) 39 (30-54.6) 0.007 CVFR: coronary flow reserve; EpiLS: Epicardium longitudinal strain; EndoLS: endocardial longitudinal strain; EndoLS- EpiLS: trasmural strain gradient.

Abstract P208 Figure. IQ-SE and Multilayer Strain
P218 Accuracy and reproducibility of aortic root assessment by eSie Valves in patients candidate to transcatheter aortic valve implantation: a comparative study with computed tomography
Authors: Granata G , Veltri A , Iuliano S , Romano V , Stella S , Capogrosso C , Ancona F , Ingallina G , Melillo F , Montorfano M , Agricola E
Published: January 2020

Background

Accurate imaging assessment of the aortic root (AR) is critical for prosthesis sizing in transcatheter aortic valve implantation. Multislice computed tomography (MSCT) is the gold standard for this purpose. 3D transesophageal (3D-TOE) reconstruction tools have recently been introduced, which automatically configures a geometric model of AR from 3D-TOE dataset and perform quantitative analyses of the AR.

Purpose

The aim of the study was to compare semi-automated measurements of AR obtained by eSie Valves (EV) (Siemens Medical Solution, California, USA) tool with MSCT.

Methods

We prospectively enrolled 26 consecutive patients (mean age 79.5 ± 7.5 years; 38% men) with severe symptomatic aortic stenosis (mean gradient 48.8± 13.6 mmHg) who underwent both 3D-TOE and MSCT as part of TAVI evaluation protocol. Volumetric datasets of the AR, acquired with 3D-TOE in mid-esophageal view, were analyzed with EV tool. EV tool automatically detected AR landmarks and, after user validation, created 3D model of AR providing values of area, perimeter, diameters of aortic annulus (AA) and coronary ostia heights (Fig 1).

Results

EV tool analysis on 3D-TOE volumetric data sets was feasible in all patients.

Strong correlation between EV tool and MSCT assessment for AA major diameter (r = 0.79), AA minor diameter (r = 0.81), AA perimeter (r = 0.89) and AA area (r = 0.89) (all p< 0.0001) was found. On average EV tool underestimated MSCT measurements of AA major diameter (1.2 mm, 4.5%), AA minor diameter (2.6 mm, 11.3%), AA perimeter (4 mm, 5.2%) and AA area (65.3 mmq, 13.6%).

Moderate correlation between the two methods, already in this initial sample, for right coronary artery ostium height (r = 0.53, p = 0.007) was discovered. Finally, weak correlation for left coronary artery ostium height (r = 0.33, p = 0.1) was revealed.

EV tool measurements from two different volumetric datasets of the same patient showed an excellent reproducibility

intraclass correlation coefficient (ICC) for AA area 0.94 and ICC for right coronary height 0.98.

Conclusion

With these initial results EV tool could be used in clinical practice for quick and reliable assessment of AA area, perimeter and diameters. A larger group of patients will be needed to assess the consistency of coronary ostia height evaluation by EV tool.

Abstract P218 Figure. eSie Valve landmarks and 3D model of AR
P219 The role of color-coded duplex sonography in evaluating coronary-subclavian steal syndrome
Authors: Antova P , Tsvetanov T S , Gelev V , Staneva M
Published: January 2020

Background

Coronary-subclavian steal syndrome is a rare phenomenon leading to dysfunction of left internal mammary artery (LIMA)-graft after coronary artery grafting with the occurrence of proximal stenosis of the left subclavian artery (LSA), which causes myocardial ischemia due to a blood-steal phenomenon through a fully patent LIMA-graft.

Purpose

To evaluate the role of color-coded duplex sonography (CCDS) in follow-up of all symptomatic patients with LIMA-graft after aorto-coronary bypass grafting (ACBG).

Methods

Two men and one woman with recurrence of angina pectoris symptoms and vertigo after ACBG with LIMA-LAD graft. Patients were hospitalized in cardiology for coronary graft assessment via selective coronary arteriography. Due to the discovery of fully patent grafts with a combination of varying degrees of proximal stenosis of the LSA, CCDS with a high-frequency linear probe was used to evaluate cervical arteries, LSA and the LIMA-graft flow rate applying functional probe for hyperemia (PH) of the left upper limb for hemodynamic assessment of significance of the steal syndrome.

Results

One woman at age 72 after ACBG – LIMA-LAD; RM1; RCA -s.v.g. Selective coronary arteriography showed patent bypass grafts with 70% proximal stenosis of LSA. The performed CCDS showed a LIMA-graft blood flow reduction from 36 ml/min to 12 ml/min in a sample for left upper limb hyperemia. There was a difference in the blood pressure of the upper limbs within 20 mmHg. Dobutamine stress echocardiography was performed, confirming ischemic zones in the LAD- peak segments.

A 63 year old man after ACBG x 4 with a difference in blood pressure of upper limbs within 40 mmHg. The performed CCDS showed thrombosis of the left internal carotid artery, high-grade stenosis of the right internal carotid artery, high-grade stenosis of LSA, alternating blood flow in the left vertebral artery – a sign of hemodynamically significant steal syndrome.

A 65 year old male after ACBG x 3 (LIMA-LAD, RCA, OM1 -s.v.g.). The selective coronary arteriography showed patent LIMA-graft with 50% proximal stenosis of LSA with a trans-stenotic pressure gradient of 20 mmHg. To identify the steal phenomenon we used CCDS, which showed a blood flow reduction into the LIMA-graft from 14.2 ml/min to 7.5 ml/ min - data of hemodynamically significant steal syndrome.

Patients were treated with percutaneous transluminal angioplasty and stenting of the LSA with postprocedure reduction of angina pectoris complaints, no difference in the blood pressure of upper limbs and no reduction of LIMA-graft blood flow during the probe for hyperemia of the upper limb.

Conclusion(s) Color-coded duplex sonography is an important and indispensable technique, part of the overall assessment of hemodynamics in case of coronary-subclavian steal syndrome and probably may prove to be a first method of choice in follow-up of all symptomatic patients with LIMA-LAD graft.
P229 Primary cardiac angiosarcoma of the right atrium: a rare entity presenting with an atrial arrhythmia
Authors: Garcia Bras P , Aguiar Rosa S , Ferreira L , Moura Branco L , Castelo A , Vaz Ferreira V , Branco Ferrao J , Martins F , Sousa L , Fiarresga A , Pinto E , Ferreira R C
Published: January 2020

Introduction

Primary cardiac tumors are rare entities and 75% are benign. Angiosarcoma is the most common malignant primary cardiac tumor.

We report the case of cardiac angiosarcoma presenting with an atrial arrhythmia.

Clinical case

A 39-year-old female patient with no past medical history presented to the emergency department with heart palpitations and atypical chest pain.

Electrocardiogram on admission showed atrial flutter with a heart rate of 153 beats per minute.

Laboratory analysis were performed showing elevated D-dimer levels (2210 ug/L).

A thoracic CT scan was performed, which ruled out pulmonary embolism, but showed multiple pulmonary nodules and a right atrial (RA) mass measuring 48 mm that could correspond to a thrombus or neoplasia.

The patient was admitted in the Cardiology ICU of our hospital and was started on beta-blocker and amiodarone with conversion to sinus rhythm. Additional exams were performed:

- Transthoracic echocardiogram (TTE) revealed an heterogenous 32,6 x 17,7 mm mass in the lateral wall of the RA with an adherent mobile mass near the tricuspid valve with 28 mm diameter (possible adherent thrombus).

- Cardiac magnetic resonance imaging confirmed a RA tumor with invasion of the atrial free wall and compression of the superior vena cava.

Due to the unclear etiology of the RA mass, ultrasound-guided intracardiac biopsy was performed. Pathological examination revealed spindle cell proliferation, consistent with the diagnosis of angiosarcoma. Immunohistochemical staining was positive for Vimentin, CD34 and CD31, with 70% Ki67 expression.

Later on, the patient developed melena with significant drop of hemoglobin levels, requiring daily red blood cell transfusions and anticoagulation had to be stopped.

The patient was transferred to the Internal Medicine ward and thoracic-abdomen-pelvis staging computed tomography (CT) scan showed a significant increase in the number of pulmonary nodules, bilateral ovarian masses, 4 hepatic nodules and ileum metastization.

During hospitalization, the patient developed right leg deep venous thrombosis and thoracic CT scan revealed bilateral pulmonary embolism.

After improvement of the clinical status, palliative chemotherapy was started and the patient was discharged, maintaining regular outpatient follow-up in the Oncology Department for 1 month.

Cardiac angiosarcoma generally presents in a late stage of the disease with metastatic involvement. When surgical treatment is not possible, despite agressive chemotherapy, the prognosis remains poor.

Abstract P229 Figure. Echocardiogram: right atrium mass
P232 A cardiac thrombus... or maybe not
Authors: Oliveira C C , Quina C , Campos I , Rodrigues C , Medeiros P , Braga C , Costeira A , Marques J , Pinho P
Published: January 2020

A 61 years old female with history of rheumatic arthritis and Sjogren Syndrom resorted to the emergency room in December of 2017 for pleuritic chest pain and dyspnea. An angioCT showed a luminal filling defect at the left inferior pulmonary artery with extension to the segmentar vessels. A pulmonary embolism (PE) was diagnosed and the patient was admitted. The study performed, including transthoracic echocardiogram (TTE) was unremarkable. Patient was discharged after 15 days treated with a direct oral anticoagulant. Six months later she returned to the emergency room with the same complaints. A new angioCT showed reduction of the previous luminal filling defects, but a focal defect in the filling of the right atrium (RA) was visualized. A TTE showed a mass (17 mm) at the RA with apparent origin at the superior vena cava. A presumptive diagnostic of atrial thrombus was done and patient started therapy with vitamin K antagonist. Three months later, TTE was repeated and the mass was still present (19 mm). Five months later, the mass was still visualized at TTE (17 mm). A transesophagic echocardiogram confirmed the presence of a RA mass with 30x22 mm of major dimensions, with close relation with the interatrial septum suggestive of a tumor. A cardiac magnetic resonance revealed a RA mass without vascularization. A cardiac gated CT showed at the RA a low density nodular image of 26x22 mm at the axial plan, with an extension of 28 mm, adjacent to the posterior wall of the RA. After contrast administration, some areas did not had significant captation while some did, aspects compatible with a "pseudoenhancement" aspect. A positron tomography showed mild to moderate FDG captation at the RA. Meanwhile, patient was under anticoagulation for 2 years with no regression of the mass. For this reason, patient was oriented to cardiac surgery. The mass was resected and the histology revealed a RA myxoma.

Cardiac masses can be due to tumour, thrombus or vegetation. In this case, the mass was highly considered to be a thrombus due to the presence of multiple risk factors: prothrombotic disease and pulmonary embolism. However, the fact that the mass did not reduce with therapy raised suspicion of other diagnosis. Although rare, myxomas can be found in the RA, and should be included in the differential diagnosis of right-sided intracardiac masses. Although myxomas are histologically benign, potential for embolization and sudden death make surgical resection a priority. Its diagnosis has now increased with the use of echocardiogradiography, and has made it the main modality for the evaluation of myxomas. PE is the most dreaded and devastating complication of right-sided myxoma. In cases of RA myxomas, clinically evident PE events are uncommon. Nevertheless, there have been reports of embolization of thrombi or tumor fragments into the pulmonary vessels in cases of right in approximately 3.2% of myxoma patients.
P234 Giant right atrial myxoma presenting as right heart failure
Authors: Carazo Vargas G , Fuentes Mendoza J A , Ruiz Esparza M E
Published: January 2020

Introduction

Myxomas are the most frequent primary cardiac neoplasms. It is currently believed that myxomas are derived from multipotent mesenchymal cells capable of both neural and epithelial differentiation Histologically, these tumors are composed of dispersed cells within a stroma of mucopolysaccharides Myxomas produce vascular endothelial growth factor, which probably contributes to the growth induction at the initial stages of tumor growth. Tumors vary widely in size, ranging from 1 to 15 cm in diameter and weighing between 15 and 180 g. About 35 percent of myxomas are friable, and they tend to present emboli. The clinical characteristics of these tumors are closely related to their location, size, and mobility; there are no specific signs and symptoms that suggest the presence of a myxoma. There are several mechanisms by which cardiac tumors can cause symptoms. The blockage of circulation through the heart or heart valves produces symptoms of heart failure. Atrial myxoma can interfere with the valves of the heart and cause regurgitation. They can also produce systemic embolisms and constitutional signs.

Clinical Case

We present a 29-year-old female patient who started with fatigue, weight loss, increased abdominal perimeter and dyspnea of one month"s effort that progressed to dyspnea at rest in the last week associated with syncope, so she decided to go to the emergency department of our institution. Upon arrival at the emergency room, the patient was found with vital signs within normal parameters, however with dyspnea at rest, jugular plethora and important lower limb edema. On auscultation, a systolic-diastolic murmur was found with an increase in the Rivero Carvallo maneuver, with reinforcement of the pulmonary component of the second noise and parasternal high-left rise. In clinical analysis, NT-proBNP 5698pg/mL, AST 34, INR 1.65 and serum lactate of 4.2 was found. A Transthoracic Echocardiogram (TTE) was performed, where a 10cm mass effusion was documented that occupied the entire right atrium and protruded into the right ventricular outflow tract. With these findings, medical treatment was started for right heart failure and it was to cardiac surgery for resection of the right atrium where it had been performed, however during the immediate postoperative state, presented biventricular failure and later asystole without achieving a return of spontaneous circulation despite resuscitation maneuvers.

Conclusion

In this case, it is an unusual presentation of a rare cardiac pathology that started with symptoms of right heart failure due to the obstruction of the right ventricular outflow tract. We consider that it is an interesting clinical case and with important educational aspects to take into consideration the differential clinical diagnoses of a patient presenting to the emergency department with right heart failure.

Abstract P234 Figure. Giant Myxoma
P236 Pinball game over
Authors: Carvalho R , Antunes C , Miragaia A , Guardado J , Morais J
Published: January 2020

Introduction

Transthoracic echocardiogram (TTE) is recommended for the diagnosis of acute pulmonary embolism (PTE) only in unstable patients, as it lacks specificity and sensitivity in stable ones. However, the rare documentation of intracavitary thrombus confirms the diagnosis and is associated with right ventricular dysfunction and high mortality, up to 42%.

We report a 69year-old female patient with history of hypertension and obesity, presenting at the emergency department with hypothermia (35º C), cyanosis, tiredness and breathless to minimal efforts. At examination she had no measurable blood pressure, low peripheral perfusion, tachycardia, tachypneia and no other abnormalities. Arterial blood gases on 10L/min facemask showed an uncompensated metabolic acidosis with pCO2 27mmHg, HCO3 12mmol/L, hyperlactacidemia and hypoxemia (pO2 57mmHg). Lab results had leucocytosis and neutrophilia, CRP 30mg/L, serum creatinine 2.53mg/dL, K+ 5.1mmol/L and high sensitivity troponin I 305pg/ml. Chest X-ray showed enlargement of the right chambers with a nodular image next to the right hila.

Although sepsis complicating acute pneumonia was firstly assumed and intravenous antibiotics and fluids were rapidly started; persistent hypoxemia with hypocapnia and tachycardia lead to the suspicion of acute PTE and workup proceeded in that direction. A TTE was primarily done because of the renal injury, showing a dilated right ventricle with flattening of the interventricular septum and a large, long, mobile mass in the right atria, protruding to the right ventricle, consistent with a thrombus. As the patient was no more hemodynamic unstable, prompt anticoagulation with low molecular weight heparin (LMWH) was initiated. Lately performed thoracic angio-CT with no contrast showed mild dilation of pulmonary artery and scintigraphy confirmed extensive perfusion defects of the left lung, preserving only posterobasal segment, with normal ventilation.

Additional study revealed a deep venous thrombosis of left popliteal and right gemelar veins and a heterozygosity for two polymorphisms of methyltethahdrofolate reductase enzyme

C677T and A1298C, clinically relevant in the context of patient’s hyperhomocysteinemia.

The patient recovered initially, with compression stockings and anticoagulation. A follow-up TTE showed no remaining intracavitary mass and no signs of right side overload. In the day after, the patient evolved with sudden refractory hypotension and signs of hypoperfusion with cardiorespiratory collapse and death.

Conclusion

The incidence of intracardiac thrombus in the right chambers in a patient with PTE is low (3-23%) and probably underestimated by the absence of early echocardiography in all patients. This case highlights the lability of the clinical evolution of these patients, even tough disappearance of the right atrium thrombus, which is in line with their high early mortality.

Abstract P236 Figure. Imaging study
P1593 Myocardial work in a cohort of systemic sclerosis patients
Authors: Magda S L , Popa E C , Rimbas R , Gheorghiu A M , Ciobanu A C , Vinereanu D
Published: January 2020

Funding Acknowledgements

Grants 83/2018 and TE49/2018

Context

Myocardial strain analysis provides important information on cardiac performance adding to standard parameters of left ventricular (LV) systolic function. Myocardial work (MW) is emerging as an alternative tool for studying LV systolic function, because it isn’t influenced by changes in afterload that could alterate strain analysis.

Systemic sclerosis (SSc) is an autoimmune disease frequently associated with myocardial fibrosis determining diastolic and subclinical systolic dysfunction.

Aim

to assess parameters of myocardial work in patients with SSc and in age and cardiovascular risk factors matched controls.

Methods

60 subjects (51 ± 9 years, 53 women) were studied: 40 patients with SSc (75% limited, 25% generalized form, mean time since onset 5 ± 4 yrs, mean time since treatment start 2.5 ± 0.5 yrs), and 20 age and cardiovascular risk factors matched controls. A comprehensive echocardiographic examination was performed using EACVI recommended protocols. Myocardial work parameters were calculated during mechanical systole and isovolumetric relaxation (IVR), by 2DSTE: global constructive work (GCW), performed during shortening in systole adding negative work during lengthening in IVR; global wasted work (GWW), performed during lengthening in systole adding work performed during shortening in IVR; global work efficiency (GWE), as the constructive work divided by the sum of constructive and wasted work and global work index (GWI), as the GCW added to GWW.

Results

2DEF, 2D longitudinal strain, GWW, GWE and GWI were similar in both groups, with values expected for sex and age. GCW was significantly lower in SSc patients (Table).

Conclusion

Patients with treated SSc have lower myocardial constructive work (GCW) compared to matched controls, probably due to subtle myocardial fibrosis, persisting under adequate treatment. Longer follow up and larger cohorts are needed in order to establish the role of MW analysis in the serial assessment of patients with SSc at risk of developing overt cardiovascular disease.

Parameter Systemic sclerosis Controls p GCW (mmHg%) 2124.2 ± 449.5 3102.8 ± 337.5 0.02 GWW (mmHg%) 81.0 ± 51.3 73.5 ± 38.9 NS GWE (mmHg%) 95.0 ± 2.8 95.9 ± 2.1 NS GWI (mmHg%) 1869.9 ± 410.9 2023.2 ± 321.7 NS EF (%) 61.3 ± 6.5 60.4 ± 8.7 NS 2DLS (%) 22.9 ± 3.4 22.4 ± 4.1 NS
1637 Percutaneous treatment of severe tricuspid regurgitation in a "bicuspid" tricuspid valve
Authors: Safi L , Pasala T , Shah A , Dudiy Y , Ruiz C
Published: January 2020

A 72 year old female with a past medical history of bicuspid aortic valve with severe aortic regurgitation status post placement of a 21 mm Magna Ease aortic valve replacement on 6/1/16, chronic atrial fibrillation, diabetes mellitus, and severe mitral regurgitation presented with symptoms of dyspnea upon exertion.

Transesophageal echocardiogram (TEE) showed normal left ventricular function with severe mitral regurgitation, severe tricuspid regurgitation and severe pulmonary hypertension. Upon close interrogation of the tricuspid valve, there was evidence of fusion with a raphe present between the septal and anterior leaflets ("a bicuspid" tricuspid valve).

The patient was referred for surgical evaluation where she was deemed to be at increased surgical risk and referred for percutaneous treatment for both severe mitral and severe tricuspid regurgitation. After an uncomplicated transseptal puncture, she underwent a placement of a single MitraClip XT clip to the A2/P2 portions of the mitral valve leaflets leaving mild residual mitral regurgitation by color Doppler. Attention was then taken towards the tricuspid valve where using TEE guidance, a MitraClip XT was positioned over the central aspect of the "bicuspid" tricuspid valve with the opened clip arms perpendicular leaflet coaptation. The MitraClip XT was advanced into the right ventricle and slowly retracted to grab both TV leaflets. There was mild, residual tricuspid regurgitation by color Doppler. The mean gradient across the tricuspid valve was 1 mmHg obtained at a heart rate of 52 bpm.

This case describes the first known percutaneous treatment of severe tricuspid regurgitation from a "bicuspid" tricuspid valve with a MitraClip device.

Abstract 1637 Figure.
1639 Echocardiographic photorealistic imaging of leiomyomatosis intravascularis
Authors: Budnik M , Czub P , Majstrak F , Kochanowski J , Scislo P , Opolski G
Published: January 2020

39-years old female patient was admitted to the Department of Cardiac Surgery because of the suspicion of additional mass in the right atrium (RA). She had no symptoms. Two weeks before she underwent surgery because of the tumours of the uterus and the bladder. Moreover, she was treated for papillary thyroid carcinoma 1 year before. D-dimer levels, NTproBNP and markers of cardiac necrosis weren’t elevated. In histopathological examination leiomyomatosis intravascularis was diagnosed.

In the transthoracic echocardiography (TTE) we confirmed the presence of additional inhomogeneous, mobile mass in the RA with morphology corresponding to thrombus. Moreover the inferior vena cava (IVC) was also involved. We decided to perform transesophageal echocardiography (TEE). Bicaval view showed that the structure enters through the IVC to the RA and protrude across the tricuspid valve throughout the cardiac cycle. In the photorealistic imaging we could exactly see huge mass that was elongated, mobile, inhomogeneous, had no adhesion with the wall of heart and vein and waved along with the heartbeat (figure 1 A,B). The surgery was performed through median sternotomy. A huge mass with about 30cm length was excised through RA oblique incision, on beating heart with cardiopulmonary bypass (figure 1C).

Abstract 1639 Figure. Leiomyomatosis intravascularis