bluuebunny/crossref_metadata_2025 huggingface dataset:
1673 Accuracy and prognostic value of physiologist-led stress echocardiography for coronary disease
Authors: Khan J N , Griffiths T , Sandhu K , Cabezon S , Kwok C S , Baig S , Naneishvili T , Lee V C K , Pasricha A , Robins E , Kanagala P , Butler R , Duckett S , Heatlie G
Published: January 2020

Funding Acknowledgements

No funding sources

Background

Cardiac physiologist-led stress echocardiography (PLSE) services provide potential for expansion of SE services and increased productivity for cardiologists. There is no published data on the accuracy or prognostic value of PLSE.

Purpose

To assess and compare the accuracy and prognostic value of PLSE and cardiologist-led stress echocardiography (CLSE) for CAD assessment

Methods

Retrospective study of 898 subjects undergoing PLSE (n = 393) or CLSE (n = 505) for CAD assessment using exercise or dobutamine. For accuracy assessment, the primary outcome was the ability of stress echocardiography to identify significant CAD on invasive angiography (ICA). Incidence of 24-month non-fatal myocardial infarction (MI), total and cardiac mortality, revascularisation and combined major adverse cardiac events (MACE) were assessed.

Results

Demographics, comorbidities, CAD predictors and cardiac medications were matched between the PLSE and CLSE groups. PLSE had high sensitivity, specificity, positive and negative predictive value and accuracy (85%, 74%, 69%, 88%, 78% respectively). PLSE accuracy measures were similar and non-inferior to CLSE. There was a similar incidence of individual and combined outcomes in PLSE and CLSE subjects. Negative stress echocardiography conferred a low incidence of non-fatal MI (PLSE 1.4% vs. CLSE 0.9%, p = 0.464), cardiac mortality (0.6% vs. 0.0%, p = 0.277) and MACE (6.8% vs. 3.1%, p = 0.404).

Conclusion

This is the largest study of PLSE accuracy and first study of the prognostic value of PLSE. PLSE demonstrates high and non-inferior accuracy compared with CLSE for CAD assessment. Negative PLSE and CLSE confer a similarly very low incidence of cardiac outcomes, confirming for the first time the important prognostic value of PLSE.

Accuracy of PLSE and CLSE for CAD Marker of diagnostic test Total (n = 72) PLSE (n = 32) CLSE (n = 40) p Significant CAD present (n, %) 20 (27.8%) 12 (37.5%) 8 (20.0%) 0.167 * Single-vessel CAD 12 (60.0%) 7 (58.3%) 5 (62.5%) 0.325 * Multi-vessel CAD 8 (40.0%) 5 (41.7%) 3 (37.5%) 0.325 Sensitivity 76% (66-76%) 85% (73-97%) 63% (48-78%) Non-significant Specificity 73% (63-73%) 74% (59-89%) 72% (58-86%) Non-significant Positive predictive value (PPV) 53% (42-64%) 69% (53-85%) 29% (15-43%) Significant Negative predictive value (NPV) 88% (80-96%) 88% (77-99%) 88% (78-98%) Non-significant Overall accuracy 74% (64-84%) 78% (64-92%) 70% (56-84%) Non-significant Accuracy data expressed as value (95% confidence interval). CAD= coronary artery disease. Differences in values between PLSE and CLSE considered statistically significant if no crossover in 95% confidence intervals

Abstract 1673 Figure. Predicted coronary artery lesion from SE
1675 ABCDE vasodilator stress echocardiography in non-ischemic heart failure
Authors: Kuznetsova N , Borguezan Daros C , Zanella H , Ciampi Q , Cortigiani L , Gaibazzi N , Zagatina A , De Castro E Silva Pretto J L , Djordjevic-Dikic A , Simova I , Amor M , Merlo P M , Lowenstein J , Torres M A R , Picano E
Published: January 2020

OnBehalf

Stress Echo 2020 study group of the Italian Society of Cardiovascular Imaging

Background

ABCDE-stress echocardiography (SE) may explore different aspects in the heterogeneous entity of known or suspected heart failure (HF).

Aim

To assess ABCDE-vasodilator SE in non-ischemic HF

Methods

In a prospective, observational, multicenter study, we recruited 428 patients with angiographically normal coronary arteries: 148 patients with HF and preserved (>50%) ejection fraction (HFpEF, Group 1); 100 with HF and mid-range (40-50%) ejection fraction (HFmrEF, Group 2); 180 with HF and reduced (<40%) ejection fraction (HFrEF, Group 3). A group of 75 healthy asymptomatic patients (30 male, 40%, age 57 ± 14 years) referred to testing for screening was also selected (Controls). All patients underwent vasodilator SE with dipyridamole (0.84 mg/kg) in 14 accredited laboratories of 5 countries (Argentina, Brasil, Russian Federation, Serbia and Italy). The ABCDE-SE protocol was adopted: A for regional wall motion abnormalities; B for B-lines (positivity criterion: stress ≥ rest for ≥ 2 points in a 4-site simplified scan available in 181 pts and 10 controls); C for left ventricular contractile reserve (LVCR) based on force (systolic blood pressure/end-systolic volume, positivity criterion: peak/ rest ≤1.1); D for pulsed wave Doppler-based assessment of coronary flow velocity reserve (CFVR) in left anterior descending coronary artery (positivity criterion: peak/rest ≤2.0); E for EKG-based assessment of heart rate reserve (positivity criterion: peak/rest ≤1.22).

Results

All positivity criteria, except A, were more prevalent (p<.01) in Group 3 compared to Group 2, in Group 2 compared to Group 1, and Group 1 compared to controls: see figure. In particular, a blunted heart rate reserve was found in 4/75 controls (5%), 27/148 pts of Group 1 (18%), 28/100 of Group 2 (28%) and 98/180 of Group 3 (54%).

Conclusions

ABCDE-vasodilator SE can help to identify the profound pathophysiological heterogeneity underlying a similar clinical presentation in patients with known or suspected HF with angiographically normal coronary arteries. These patients rarely show stress-induced regional wall motion abnormalities (A), but may exhibit pulmonary congestion (B), reduced myocardial functional reserve (C), altered coronary microcirculation (D) and cardiac autonomic dysfunction (E).

Abstract 1675 Figure. The positivity rate of ABCDE criteria
1679 E/e predicts exercise capacity and adverse cardiovascular outcomes in patients with chronic kidney disease
Authors: Gan G , Kadappu K , Bhat A , Fernandez F , Chen H , Eshoo S , Thomas L
Published: January 2020

Funding Acknowledgements

Nil

OnBehalf

NA

Background

Patients with chronic kidney disease(CKD) have reduced physical fitness that contributes to the disproportionately elevated risk of cardiovascular disease in this population. Our aim was to assess the association between E/e’ and exercise capacity in CKD patients and the prognostic role of E/e’.

Methods

Patients with Stage 3/4 CKD, without previous cardiac disease were prospectively recruited. Recruited patients underwent transthoracic echocardiogram and exercise stress echocardiogram with assessment of exercise E/e’. Patients were compared, one to one, to age, gender and risk factor matched controls and were followed annually for 5 years for cardiovascular death and major adverse cardiovascular events (MACE). Exercise capacity was assessed as metabolic equivalents (METs) with reduced exercise capacity defined as METS of ≤7. Raised exercise E/e’ was defined as exercise E/average e’ of >13.

Results

156 CKD patients (62.8 ± 10.6 yrs, male 62%) were compared to 156 matched controls. CKD patients had higher rates of anemia (p < 0.01), larger left ventricular indexed mass (p < 0.01), larger LAVI (p < 0.01) and higher resting (p < 0.01) and exercise E/e’ (p < 0.01). Overall, CKD patients achieved lower METs (p < 0.01) with exercise and a greater proportion of CKD patients had METs ≤7 (p < 0.01). Receiver operating curves (Figure1) showed exercise E/e’ (AUC 0.89, CI 0.84-0.95, p < 0.01) to be the strongest predictor of reduced exercise capacity in CKD patients. Exercise E/e’ of >13 was also associated with higher rates of cardiovascular death and MACE amongst CKD patients.

Conclusion

Exercise E/e’ is a strong predictor of exercise capacity amongst CKD patients, who commonly have reduced exercise capacity presumably consequent to diastolic dysfunction. Raised exercise E/e’ in CKD patients is predictor of cardiovascular death and MACE.

Abstract 1679 Figure.
P1685 Loeffler endocarditis mimicking myocardial infarction
Authors: Cerne Cercek A , Berden P , Zbacnik R
Published: January 2020

Loeffler endocarditis is a major cause of morbidity and mortality in patient with hypereosinophilia-associated syndromes. It is a rare disease that occurs due to eosinophilic inflammation of the endocardium with fibrosis and thrombus formation. We describe a case of Loeffler endocarditis with unusual clinical course.

61-year-old woman was admitted due to chest pain and elevated troponin I level. Her past medical history was remarkable for persistent asthma, rhinitis, and productive cough for almost 1 year. Laboratory test revealed hypereosinophilia. Further workup for parasitic diseases, allergy, malignancy, hematological causes were all negative Coronary angiography excluded coronary artery disease. Transthoracic echocardiography (TEE) showed endocardium thickening at the septal left ventricular wall, diastolic dysfunction (Grade II), and moderate pericardial effusion (Figure 1A). Cardiac magnetic resonance imaging (cMR) confirmed endocardial inflammation. The Loeffler endocarditis was diagnosed and corticosteroid treatment was started. After one year the patient was asymptomatic, TEE showed improvement of left ventricular diastolic function (Grade I) and complete regression of myocardial thickening (Figure 1B). Therefore, corticosteroids were slowly tapered. After 16 months she relapsed with severe chest pain, marked eosinophilia and elevated troponin I level. Left ventricular systolic function rapidly deteriorated to ejection fraction of 40%. Urgent cMR showed diffuse edomyocardial inflammation with apical thrombus formation. She was immediately treated with high doses of corticosteroid and heparin, which resulted in systolic function normalization and thrombus disappearance.

In conclusion, Loeffer endocarditis still represents diagnostic challenge, may have unpredictable clinical course and generally respond well to corticosteroid treatment in early stages.

Abstract P1685 Figure 1.
P1688 Rare cause of acute heart failure in patient with scleroderma
Authors: Ali A M F , Abo El Soud A M
Published: January 2020

A 32-year old female patient, known to have scleroderma, presented with symptoms of recurrent attacks of palpitations, dyspnea (NYHA class III) & dizziness for 3 days before presentation. No chest pain or syncope. Physical examination at presentation revealed hemodynamic instability (BP: 80/50 mmHg, HR: 200 bpm/ regular thready pulse with fine bilateral basal chest crackles. ECG revealed ventricular tachycardia (figure 1), so electrical cardioversion was immediately done upon which the reverted to multifocal atrial tachycardia with RBBB and restoration of hemodynamics and disappearance of chest crackles.

She also had thickening of the fingertips’ skin, amputated tip of the right middle finger with severe associated Raynaud’s phenomenon, skin tightness of both upper and lower limbs, Buccal buckling with limited mouth opening (figure 2 and 3). Renal function and serum electrolytes were normal with elevated troponin I, creatine kinase and CK-MB serum levels.

Echocardiogram showed mild concentric LV hypertrophy with normal internal dimensions and severely impaired systolic function (EF∼ 20%) with severe global hypokinesia . The RV was also dilated and impaired (TAPSE∼8 mm). Mild pericardial effusion with no chamber collapse (Figure 4, 5 and 6). Coronary angiography revealed normal epicardial coronaries.

Echocardiography one month prior to presentation revealed normal LV dimensions, preserved systolic function and mild diastolic dysfunction with no resting wall motion abnormalities, upper normal RV size with preserved RV systolic function and mild elevation of pulmonary artery systolic pressure.

The presence of new acute biventricular failure in the absence of CAD and elevated cardiac biomarkers are highly suggestive of acute myocarditis.

Medical therapy included loop diuretics, angiotensin-converting-enzyme inhibitor, beta-blocker and aldosterone receptor antagonist. She was discharged in NYHA I-II, with normal cardiac enzymes and left ventricle ejection fraction of 30% and resolution of the pericardial effusion.

Discussion

Scleroderma is rarely present with impaired LV contractility but more commonly presented with diastolic dysfunction (in up to 40% of cases) (1,2). This case was presented with acute myopericarditis with severe biventricular systolic dysfunction which is very rare with scleroderma and only mentioned in few case studies, so no consensus on specific treatment. Few patients with myocarditis and scleroderma were treated with immunosuppressants along with diuretics, beta-blockers, and ACE inhibitors with most of them showed improvement clinically with recovery of systolic function after 12 months of follow up (3). We noted improvement of patient symptoms within few days of diuretics, beta-blockers, Aldosterone antagonists and ACE inhibitors. This case had baseline conduction abnormalities (RBBB with multifocal atrial tachycardia) which could be present in up to 20% of cases (4).

Abstract P1688 Figure.
P1700 Recurrent inflammatory myofibroblastic tumor of the right ventricle and pulmonary artery
Authors: Akhunova S , Dzhordzhikiia R , Rafikov A , Paschenko E , Gizatullina N
Published: January 2020

Introduction

Inflammatory myofibroblastic tumor (IMT), also known as plasma granuloma, is a very rare tumor with varying biological behavior, from completely benign to malignant. IMT can occur in virtually every organ, but cardiac origin is extremely rare. IMT occurs mainly in children and young people. The mainstay of treatment is complete surgical resection of the tumor.

We present a case of 28 year old white woman with no contributory medical and family history, who was admitted to hospital because of dyspnea and cough. The transthoracic echocardiography (TTE) showed elevated pulmonary artery systolic pressure up to 61 mm Hg and mild dilation of right chambers. Further assessment by computed tomography showed thrombosis of the main pulmonary artery (PA), narrowing its lumen by 90%, with spread to the lobar and segmental arteries of the left lung - signs of massive pulmonary embolism. During thromboendarterectomy revealed a mass, about 4,1 x 3,1 x 1,8 cm in size, with thrombotic overlays, locating at the right ventricular outflow tract (RVOT) and the pulmonary trunk. Histological examination and Immunohistochemistry studies was consistent with IMT with an intermediate potential of malignancy.

A year after the operation, the patient became pregnant. Routine TTE showed low density mobile mass narrowing the pulmonary trunk. The transvalvular blood flow velocity was 2.4 m/s. Cardiac magnetic resonance (CMR) was consistent with echo findings. The pregnancy has been preserved, and after 36 weeks it was decided to perform the birth of a girl by means of a Cesarian section.

After 2 months, due to an increase in the size of the tumor and the dyspnea, a reoperation was performed to remove the tumor lining the RVOT and intimately connected with the PA valve, excision of the PA valve. A bioprosthesis was installed. The tumor had an identical morphological picture.

Conclusion

This case report describes a rare recurrent cardiac inflammatory myofibroblastic tumor involving RVOT and PA. The prognosis after surgical treatment of IMT is usually favorable, but it is known that some tumors recur, and rarely they can even metastasize. The long-term outcome is not well established due to the rarity of these tumors.

Abstract P1700 Figure.
P1703 Cardiac metastasis of germ cell tumor "like beads" in the right heart chambers and also left atrium
Authors: Akturk S , Tan T S , Mammadli A , Mammadov M , Dincer I , Erol C
Published: January 2020

Introduction

Testis tumors constitute 1-2% of all malignant tumors in men. But it is the most common solid tumor in men between 15-35 years of age. Germ cell tumors constitute for almost 90% of all testis tumors. Intracardiac metastasis of testicular carcinomas is rare. We now report a case of a testicular germ cell tumor with right atrial metastasis.

Case report

A 30-year-old male patient was diagnosed with B-cell ALL.Chemotherapy and radiotherapy were completed in 2016. In January 2017, the patient applied to the hospital with pain in the right testicle.A mass detected and orchiectomy was performed.Pathologic examination revealed mixed germ cell tumor and B-cell ALL infiltration.Chemotherapy was started. The patient was admitted to our hospital with fever, in March 2018.Antibiotics were started but fever contuniued.Transthoracic echocardiography showed a large,hypoechogen,mobile mass in the right atrium.Then transesophageal echocardiography revealed a 2x3,3 cm mobile mass within the right atrium that prolapsed through the tricuspid valve into the right ventricle in diastole.We could not distinguish if it is a vegetation or a metastatic mass. The patient underwent cardiac surgery.Pathologic examination revealed mixed germ cell tumor metastasis.

After the surgery, the patient was transferred to the intensive care unit because of sepsis. Antibiotics were expanded due to fever. Control transthoracic echocardiography and also transesophageal echocardiography showed a 1,8 x 0,6 cm mobile mass extending from the inferior vena cava into the right atrium and through the patent foramen ovale into the left atrium. One week after the surgery, a mass was detected in the transthoracic echocardiography. But no further examination was done. We thought that the mass may not have been completely removed in the the operation (residual tumor?). The patient was evaluated with the department of oncology and cardiovascular surgery. It was decided that reoperation would be very risky.

Conclusion

Metastatic tumors of the heart are seen more frequently than primary tumors. Although intracardiac metastasis of testicular germ cell tumors are rare (less than %1), it has been related to short survival. They may lead to the congestive heart failure, paradoxical systemic emboli and vena cava superior syndrome. Most cases in the literature are associated with right atrial mass. But in our case, the mass was extending from the inferior vena cava into the right atrium and through the patent foramen ovale into the left atrium. We wanted to share our experience and also wanted to discuss the treatment modality for similar patients.

Abstract P1703 Figure.
P1708 Analysis of ventricular mechanics in echo-guided endocardial radiofrequency septal ablation for hypertrophic obstuctive cardiomyopathy
Authors: Vasquez Orozco R J , Ortiz-Avalos M , Rodriguez-Diez G , Morales Portano J D
Published: January 2020

Introduction

Hypertrophic Obstructive Cardiomyopathy (HOCM) is an important cause of sudden death and heart failure, specially in young adults. Approximately 5% to 8% of patients are not suitable for transcoronary ablation, if myectomy is not feasible, Endocardial Radiofrequency Ablation of Septal Hypertrophy (ERASH) has shown acceptable efficacy and safety for left ventricular outflow tract (LVOT) gradient reduction. 20 ERASH cases have been reported in the literature.

Case report

39 year old woman with diagnosis of HOCM, symptoms of dyspnea (NYHA III), and aborted sudden death as first clinical manifestation; a cardiac defibrillator was implanted. A transthoracic echocardiogram (TTE) revealed normal left ventricle (LV) ejection fraction, moderate mitral regurgitation (systolic anterior valve motion), basal inter-ventricular septal wall thickness of 20 mm generating a LVOT gradient of 51 mmHg. A coronary angiography showed inadequate septal branch anatomy to perform alcohol ablation. The patient refused myectomy. Heart Team decided to perform ERASH. Using a 3D Cardiac Mapping System and 2D Echocardiography, the basal interventricular septum received 50 Watts/50˚C radiofrequency ablation without any acute complication. Three days later, a TTE showed LVOT gradient 26 mmHg. The description of the LV mechanics is described on Table 1. Pre and post ERASH ventricular mechanics was analyzed including circumferential endocardial and mesocardial strain and strain rate, as well as longitudinal strain involved in the LV segments that received ERASH (Figure 1.) .

Conclusion

We report the analysis of ventricular mechanics after a successful case of ERASH, an alternative technique for treating HOCM. In our analysis, pre- ERASH longitudinal strain was significantly more negative at the basal anterior and anteroseptal segments than the other LV segments. This may be a predictor of focal LV remodeling at these segments in the future.

Table 1. Pre ERASH Post ERASH Ventricular Segment Strain Strain Rate Strain Strain Rate Longitudinal Strain Basal Anteroseptal -14.4% -1.2 1/s -7% -0.6 1/s Endocardial Circumferential Strain Basal Anteroseptal -13.7% -1.9 1/s -32% -2.2 1/s Basal Anterior -44.6% -4.2 1/s -18.6% -1.6 1/s Mesocardial Circumferential Strain Basal Anteroseptal -7.7% -1.0 1/s -20.2% -1.2 1/s Basal Anterior -24.9% -1.7 1/s -13.8% -0.9 1/s Left ventricular mechanics pre and post ERASH.

Abstract P1708 Figure 1.
P1720 Hammock mitral valve, a challenging echocardiographic diagnosis
Authors: Rodrigo Carbonero D , Estandia U , Perez C , Voces R , Perez P , Panera E
Published: January 2020

We report a 43 year-old female with a past TTE echocardiography of rheumatic valve disease performed in her district hospital , ( No clear symptomatology of rheumatic fever in the past). She was transferred to our tertiary hospital for elective cardiac surgery.

Preoperative echocardiogram showed a non-dilated left ventricle with preserved contractility, mild-moderate left atrium enlargement with severe mitral regurgitation and basal displacement of papillary muscles and severe tricuspid regurgitation.All of it resembling a hammock mitral valve instead of former echocardiogram described as rheumatic valve disease.

Preoperative cardiac study showed severe pulmonary hypertension with increased pulmonary vascular resistances. Preserved biventricular cardiac output and increased proto and telesystolic pressures.

During surgery , ifindings were described as a mitral valve with a large papillary muscle inserted in the distal third of the left ventricle with none tendinous cords at the anterior leaflet and without cords in the posterior leaflet with an isolated papillary muscle with cords at A3 and P3 scallops, compatible with hammock mitral valve. A tendinous muscle/fibrous or fibromuscular band connecting the septum to the posterior wall of the left ventricle was described. Moreover over, there was an enlarged tricuspid ring with very short tendinous cords on the septal leaflet, although the leaflet was bigger than usual.

Surgery consisted of resection of the mitral valve preserving A3 and P3 scallops with a 29mm Bicarbon Sorin mechanical mitral prosthesis and a 32mm Carpentier tricuspid ring implantation and pulmonary veins ablation combined with occlusion of left atrial appendage. After 112 minutes of cross-clamping time, the patient was weaned from cardiopulmonary bypass. She had important left ventricle dysfunction which improved with dobutamine and AAI pacemaker at 90lpm. Postoperative TEE showed moderate dysfunction of right ventricle, mild left ventricular dysfunction, moderate tricuspid regurgitation and a good functioning of the prosthesis. TTE before discharge showed good function of mitral valve prosthesis, good left ventricle function, mild tricuspid regurgitation, mild-moderate right ventricular enlargement, although less than preoperatively.

Conclusion

Congenital mitral valulophaty is a rare condition in the adulthood. The estimated prevalence is 0,5%. The hammock mitral valve is a more uncommon pathology which affects the mitral valve and subvalvular apparatus. This anomaly, was first described in 1967 and it is characterised by anomalous papillary muscles directly connected to the anterior mitral valve by a fibrous bridge without chordae tendineae in between them. This fibrous bridge hampers the opening and closure of the mitral valve.

Diagnosis requires a high index of suspicion, both ultrasound studies and medical history, to avoid misdiagnosis.

Abstract P1720 Figure.
P1726 A case of double chamber right ventricle with membranous ventricular septal defect: multimodality approach before and after surgical repair
Authors: Mehtap Yeni M Y , Ibrahim Basarici İ B , Nermin Bayar N B , Sakir Arslan S A
Published: January 2020

21 year old male admitted with progressive dyspnea. Transthoracic echocardiogram(TTE) showed membranous type ventricular septal defect(VSD).Additionally there was an evident turbulence and nearly 120mm Hg (5.5m/s maximal velocity) gradient in the mid region of right ventricle(RV) a muscle bundle was restricting the flow. Double chamber right ventricle(DCRV) and VSD have been confirmed also with right heart catheterisation.Measured RV pressures were 90/0/5 mmHg and pulmonary artery pressures were 17/4(mean:8mmHg).Qp/Qs was 1.3. Cardiac magnetic resonance imaging (CMRİ) excluded other congenital defects like atrial septal defect,abnormal pulmonary venous return or aortic coarctation. Cardiac BT also clearly showed the hypertrophied muscular bundle which is close to the apex; was dividing the right ventricle cavity into two parts. Patient has been referred for surgery.On follow up, patient was doing well and TTE nicely showed a clear decrease of gradient as measured maximal velocity was 2m/s and no residual shunt of VSD. Control CMRİ also showed good RV function with a mild residual muscle structure.

Our case was a nice example of VSD without pulmonary hypertension because of restricted blood flow of pulmonary artery by a muscle bundle. If VSD is presented with a restricted RV outflow ; instead of a dilated RV chamber as been expected; a concomitant DCRV should be kept in mind.

Abstract P1726 Figure. video 1
P1728 Pulmonary hypertension in a pregnant Women - a rare anatomical aetiology
Authors: Grade Santos J , Ferreira F , Loureiro M , Almeida A , Pereira A , Repolho D , Sebaiti D , Alegria S , Sousa L , Almeida S , Pereira H
Published: January 2020

A 32 year old female patient, with a medical history of an ill-characterized Pulmonary Arterial Hypertension associated with congenital heart disease, lost in the follow up with no medical therapy, attended an emergency department for a gynecological hemorrhage at 16 weeks of pregnancy. Due to high maternity mortality risk, informed consent was obtained, and termination of pregnancy was performed. She was then referred to our pulmonary hypertension center.

At our center she had complains of fatigue with moderate intensity exertion, classified in a class II of the World Health Organization (WHO) classification, but was otherwise asymptomatic, with no history of dyspnea, angina or syncope. There was allusion to a self-limited episode of hemoptysis in the past. On physical examination she had an increased pulmonary component of the second heart sound, continuous heart murmur in left sternal border and no cyanosis (O2 peripheral saturation in the upper and lower limbs of 99% at room air).

The performed echocardiograms (both transthoracic and transesophageal) showed an estimated systolic pulmonary artery pressure of 120 mmHg with severe right ventricular hypertrophy and systolic dysfunction. There was dilatation of the trunk and right pulmonary artery. The left pulmonary artery was not seen.

Biochemical evaluation and viral serologies were unremarkable. The pulmonary function tests and the arterial blood gases were normal.

Cardiac MRI demonstrated the presence of a right aortic arch and a right patent arterial duct. An anomalous origin of the left pulmonary artery from the ascending thoracic aorta could be noted. Associated congenital cardiac defects were excluded.

A right heart catheterization confirmed the presence of severe pulmonary hypertension with mean pulmonary artery pressure of 86 mmHg and Pulmonary vascular resistance of 11 Wood Units. A large persistent arterial duct to the right pulmonary artery was confirmed with persistent left to right shunt. The left pulmonary artery was visualized when injection was performed in the aortic root. Coronary arteries were normally implanted.

The patient was started on Sildenafil and Bosentan (later replaced by Macitentan due to hepatic toxicity). After 3 years of follow up, there was an improvement in symptoms and in the 6 minutes walking test, remaining in a low risk category and on a WHO class I.

This case reports a very rare congenital abnormality identified in an adult patient. Despite the complex anatomy and severe pulmonary hypertension, the patient is reasonably well under medical therapy and close follow up.

Abstract P1728 Figure. Cardiac MRI Cine Sequences
P1730 Multimodality assessment of complex ventricular septal defect
Authors: Morrison M L , Kabir S , Salih C , Valverde I , Tometzki A , Simpson J M
Published: January 2020

Case Summary

A 16 month old, 8.2 kg patient came forward for evaluation of complex cardiac anatomy with multimodality imaging assessment to ascertain suitability for biventricular repair. A large ventricular septal defect was diagnosed before birth but possible straddling of the tricuspid valve identified postnatally.

The patient developed symptoms of congestive cardiac failure and was palliated with a pulmonary artery band. In view of the difficult nature of the defect they were reviewed with 3D-transthoracic echo, transoesophageal echo and cardiac MRI.

Their transthoracic echo confirmed situs solitus with levocardia, atrioventricular and ventriculoarterial concordance. There was a well placed pulmonary artery band with peak velocity of 5 m/s. Biventricular systolic function appeared good. There was a large ventricular septal defect at the inlet extending to the muscular septum. There were 2 prominent muscle bundles arising from the ventricular apex and it was difficult to distinguish on echocardiography which of these formed the true ventricular septum (Figures A & B). Although the tricuspid valve opened normally, there were multiple chords extending to overlie the right ventricular aspect of the ventricular septal defect (Figure C), some of which appeared to cross the through defect (*) and attach to the more leftward of the apical trabeculations (Figure D white dotted line illustrates the true plane of ventricular septum which overlies the attachments. Red dotted line represents the plane followed by the leftward apical trabeculation). Cardiac MRI showed that the trabeculation positioned to the left was the true ventricular septum, as it seemed to be in line with the plane of the atrial septum at the crux of the heart (Figure E & F). At surgery her heart was found to be unseptatable due to multiple straddling chords from the tricuspid valve inserting into multiple papillary muscle heads with the left ventricle.

Conclusions

The key issue in this case is which of the muscular structures positioned at the ventricular apex is considered to be the true ventricular septum as this determines whether on not there is straddle of the tricuspid valve. In addition the complex and multiple nature of the chordal attachments below the valve made accessing and closing the defect not feasible.

Even in the present era with wide availability of advanced, multimodality imaging techniques demonstrating anatomy can still prove challenging in planning surgical repair, especially within the setting of complex congenital heart disease. Many aspects of such cases still only become apparent at the time of surgery and this remains a key issue when counselling parents.

Abstract P1730 Figure.
P1735 Jellyfish sign, not all echocardiographic findings are related to heart disease
Authors: Lambarri Izaguirre A , Codina Prat M , Ugedo Alzaga K , Candina Urizar R , Castellanos Alcalde M , Aurrekoetxea Bajeneta G , Bravo Martinez I , Mendoza Cuartero P M , Asla Ormaza C , Caballero Alvarez D , Manzanal Rey A , Ibarrondo Garcia N , Ramirez-Escudero Ugalde G , Ruiz Gomez L , Ormaetxe Merodio J M
Published: January 2020

We report the case of a 71-year-old man, without any previous history of cardiovascular disease, who was derived to our echocardiography laboratory because of a six-month period of dyspnoea.

In the echocardiographic examination, various pleural masses which depended from the left visceral pleura were found, surrounded by, what it seemed, an abnormally located severe anterior pleural effusion, which caused left lung atelectasis (jellyfish sign, complete atelectasis of a lung lobe which floats above a massive pleural effusion) with a less important pericardial effusion (Figure 1). After the echocardiographic findings, a total body Computed Axial Tomography (CAT) scan and a Positron Emission Tomography (PET) were performed, confirming various left pleural implants and a severe left pleural effusion causing a big left lung atelectasis with widespread cervical, thoracic and abdominopelvic adenophaties, suggestive of advanced lymphoma. The cytological exam of the pleural liquid obtained by the thoracentesis procedure, showed a monotonous population of predominantly medium size cells with signs of nuclear indentation compatible with a pleural affection by a germinal center follicular lymphoma.

Pleural effusion is a common complication of lymphomas (20-30%) and is considered as one of the factors adversely influencing overall survival, as in most of the cases, serous cavity involvement is part of a disseminated disease process. The thoracic duct obstruction and impaired lymphatic drainage appear to be the primary mechanism for pathogenesis of pleural effusion in Hodgkin´s disease and direct pleural infiltration is the predominant cause in non-Hodgkin´s lymphoma. Because the descending thoracic aorta is interposed between the pericardium anteriorly and the pleura posteriorly, echocardiography may be a useful landmark in the differentiation of posterior effusions. However, sometimes due to the abnormal position of the effusion, it might be difficult to differentiate between pericardial and pleural effusions, with the need, as in our case, to perform a multi-modality imaging study with a CAT and PET scan, followed by a cytological exam, to confirm the diagnosis and to guide the posterior treatment. Finally, we would like to underline the importance of familiarizing ourselves on the basics of pulmonary echocardiography, as these findings are frequent during the performance of echocardiography in our daily practice.

Abstract P1735 Figure. Jellyfish sign in pleural effusion
P1759 How an echocariologist can work off-line in a personal computer on the echocardiography images at the level of mitral valve?
Authors: Karvandi M , Ranjbar S
Published: January 2020

Background

The objective of this presentation is to provide an educational survey on the mitral valve geometrical indices measurements using MATLAB software offline in a personal computer as an echo desk for further information and recommendation before mitral valve repairs or the other treatments.

Methods and Results:

Utilizing mathematical equation and MATLAB software (a simple echo desk) to measure the geometrical mitral valve variables offline based on 2D or 3D conventional echocardiographic images.

MATLAB software as an echocardiography desk

Open the MATLAB software. Write down the command pic = imread(‘’) in the command window. You can use this command to read an echocardiographic image from your computer and in a variable like a put. You must write the image address, image name and photo extensions in parentheses. For example, your echocardiographic image is in the following address: C:\Users\USER\Desktop\echo image 1.jpg. You should write the above command like this: pic = imread(‘C:\Users\USER\Desktop\echo image 1.jpg’); Finally, call that image by imshow(pic) command and then save the program and click the RUN bottom. You will see that a new window is opened and the image you are looking for is displayed. You can edit or measure the distances of the image using the menus above this observed image (Figure A). You can also extract frames from an echocardiography video clip of a patient by writing down the command Avi2frame (‘1.avi’, ‘OutputFolder’,’jpg’) in the command window and then you can work offline on echocardiography images frame by frame to calculate quantitative data (Figures B and C).

Conclusion

In spite of the introduced methods applied for echocardiographic datasets at the level of mitral valve for practical applications and clinical verifications. The most important applications, however, may be pedagogical. A computer desk (online or offline) can help an Echocardiologist to work on the echocardiographic images to evaluate the data.

Abstract P1759 Figure. working offline on echo images
P1762 Natural history and outcome of adults with bicuspid aortic valve: a middle-aged french cohort of 541 patients
Authors: Bohbot Y , Cheng C , Fay F , Elmkies F , Gugeinheim A L , Tribouilloy C
Published: January 2020

Funding Acknowledgements

none

Background

No data are available regarding the natural history and outcomes of middle-aged European patients with bicuspid aortic valve(BAV), as the only 2 large studies were conducted on the other side of the Atlantic.

Purpose

To determine the frequency of cardiovascular events and to assess the survival relative to that of the general population(GP) in a large population of adults diagnosed with BAV according to the presence or the absence of a surgical indication at baseline.

Methods

Between 2005 and 2017, 350 patients without surgical indication (mean age 53, 71% men) and 191 patients with surgical indication (mean age 59, 71% men) at baseline were included. Median follow-up was 80 months.

Results

In the medical group, 34 deaths(9.7%) were recorded(10 cardiovascular). The 10-years survival rate was 88 ± 2% with a relative survival compared to the age-and-sex matched population of 98.8%. Nine patients(2.6%) were diagnosed with infective endocarditis (IE) and no aortic dissection occurred. Aortic valve and/or ascending aorta surgery was performed in 102 patients(29%) during follow-up. In the surgical group, 12 deaths(6.3%) were recorded(5 cardiovascular). The 10-years survival rate was 90 ± 3% with a relative survival compared to the matched population of 99.2%. Five patients(2.6%) were diagnosed with IE and no aortic dissection occurred.

Conclusion

Middle-aged adults with BAV have a high likelihood of surgery but a survival comparable to the GP. The rate of IE is low and no aortic dissection occurred in our study.

Abstract P1762 Figure. a) survival curves B) surgery incidence
P1763 Impact of disproportionate functional mitral regurgitation
Authors: Bartko P E , Heitzinger G , Arfsten H , Pavo N , Spinka G , Prausmueller S , Andreas M , Mascherbauer J , Hengstenberg C , Huelsmann M , Goliasch G
Published: January 2020

Background

Application of the effective regurgitant orifice area (EROA) and regurgitant volume (RegVol) is potentially limited as such lesion-focused metrics inevitably lack flexibility to account for the heterogeneity of left ventricular size and function. A recently proposed conceptual framework seeks to rearrange EROA and RegVol cut-offs according to left ventricular end-diastolic volume (LVEDV) and left ventricular ejection fraction (LVEF), introducing the novel term "disproportionate FMR" to describe clinically meaningful FMR.

Methods

To test the impact of disproportionate FMR, we embedded data of 291 heart failure patients with reduced ejection fraction (HFrEF) under guideline directed therapy (GDT) into this framework. Regurgitant Volume and EROA were plotted against LVEDV using bubble plots that also account for the heterogeneity of EF (Figure 1 A and C). The black lines depict a regurgitant fraction (RegFrac) of 50% at the median EF (25%) or Vmax (4.3m/s) of the study population. Thus, above individual center lines (illustrated by different bubble sizes) FMR severity is disproportionate, within the area of measurement uncertainty it is proportionate to LV dilation and below, it is likely non-severe. The degree of uncertainty of proportionate FMR is determined by the imprecision of the measurements defined as 2SDs of regurgitant fraction (±6.6%) per Bland-Altmann analysis.

Results

During a median follow-up of 84 months (IQR 84-136), 166 patients died. Disproportionate FMR was associated with excess mortality (RegVol: HR 1.97, 95%CI 1.38-2.81, P < 0.001; EROA: HR 2.22, 95%CI 1.52-3.22), whereas proportionate FMR was not associated with increased long-term mortality (RegVol: HR 1.04, 95%CI 0.71-1.53, P = 0.83; EROA: HR 1.06, 95%CI 0.71-1.58, P = 0.79; Figure 1B&D).

Conclusions

In this contemporary HFrEF cohort every fifth patient has disproportionate FMR which conveys a two-fold increased risk of mortality which provides evidence for the validity of the conceptual framework. Advancement of the proposed framework to clinical practice has several implications: 1)EROA and RegVol are metrics that do not account for the contextual variability of LVEDV and EF. 2)The RegFrac -not incorporated in ESC guidelines but integrated in AHA/ACC definitions- provides a metric proportionated to left ventricular size and function supporting its use to define relevant FMR. However, technical limits suggest its complementary use on top of more robust metrics such as EROA and RegVol. Future studies need to clarify whether disproportionate FMR reflects the subgroup of patients that benefit from mitral valve repair, and provide a robust algorithm that integrates the metrics of FMR severity in a complementary manner.

Abstract P1763 Figure.
P1764 A unifying concept for the quantitative definition of functional mitral regurgitation
Authors: Bartko P , Arfsten H , Heitzinger G , Pavo N , Toma A , Strunk G , Hengstenberg C , Huelsmann M , Goliasch G
Published: January 2020

Background

Diverging guideline definitions for the quantitative assessment of severe secondary mitral regurgitation (sMR) reflect the lacking link of the sMR spectrum to mortality and has introduced a source of uncertainty and continuing debate.

Objectives

The current study aimed to define improved risk-thresholds specifically tailored to the complex nature of sMR that provide a unifying solution to the ongoing guideline-controversy.

Methods

We enrolled 423 heart failure patients under guideline directed medical therapy and assessed sMR by effective regurgitant orifice area (EROA), regurgitant volume (RegVol) and regurgitant fraction (RegFrac).

Results

Measures of sMR severity were consistently associated with 5-year mortality with a HR for a 1-SD increase of 1.42 (95%CI 1.25-1.63, P < 0.001) for EROA, 1.37 (95%CI 1.20-1.56, P < 0.001) for RegVol and 1.50 (95%CI 1.30-1.73, P < 0.001) for RegFrac. Results remained statistically significant after bootstrap- or clinical confounder-based adjustment. Spline-curve analyses (Figure 1A-C) showed a linearly increasing risk enabling to stratify in low-risk (EROA < 20mm2 and RegVol < 30ml), intermediate-risk (EROA 20-30mm2 and RegVol 30-45ml) and, high-risk (EROA≥30mm2 and RegVol≥45ml). In the intermediate-risk group, a RegFrac ≥50% as indicator for hemodynamic severe sMR was associated with poor outcome (P = 0.017). A unifying concept based on combined assessment of the EROA, the RegVol, and the RegFrac (Figure 1D) showed a significantly better discrimination compared to the currently established algorithms (Table 1).

Conclusions

Risk-based thresholds tailored to the pathophysiological concept of sMR provide a unifying solution to the ongoing guideline controversy. An algorithm based on the combined assessment of the unifying cut-offs for EROA, RegVol and RegFrac improves risk prediction compared to currently established grading.

Table 1 Definition of severe sMR Cox regression analysis ROC analysis IDI analysis HR (95%CI) P-Value ROC P-Value-for-comparison IDI P-Value Unifying concept 3.76 (2.71-5.23) <0.001 0.63 –- –- –- ACC/AHA definition 3.20 (2.14-4.78) <0.001 0.57 <0.001 0.06 <0.001 ESC/EACTS definition 1.52 (1.10-2.09) 0.01 0.55 <0.001 0.13 <0.001 ACC/ASE expert consensus 1.89 (1.40-2.56) <0.001 0.59 0.04 0.08 <0.001 Comparison of the unifying concept with the ACC/AHA, ESC/EACTS and ACC/ASE expert consensus definitions of sMR by Cox regression, ROC, and IDI demonstrated the most powerfull prediction by the unifying concept with significantly higher ROC area under the curve and better discriminatory power by IDI.

Abstract P1764 Figure 1 A-D
P1767 Abnormal longitudinal peak systolic strain in asymptomatic patients with psoriatic arthritis
Authors: Tsigaridas N , Mantzoukis S , Mantzoukis S , Toli E , Toli E , Tsimos K , Tsimos K , Varsamis N , Varsamis N , Gerasimou M , Gerasimou M , Gianniki M , Gianniki M , Patsouras D , Patsouras D , Tsifetaki N , Tsifetaki N
Published: January 2020

Background/Introduction: Psoriatic arthritis (PsA) is a systemic inflammatory disease affecting 15-30% of patients with psoriasis. Patients with PsA present 43% higher risk of cardiovascular disease compared to non-psoriatic patients. However, the probable existence of subclinical myocardial dysfunction in these patients has not been fully elucidated.

Purpose

Purpose of this study is to evaluate systolic and diastolic myocardial function in asymptomatic patients with PsA and no cardiac comorbidities and to reveal the value of speckle tracking echocardiography in this object.

Methods

Sixty patients (29 males, mean age 52.55) and 34 healthy controls (18 males, mean age 50.79) participated the study. They were subjected to TTE to evaluate left ventricular systolic function with conventional indexes such as ejection fraction (EF) and with novel indexes such as global longitudinal strain (GLS); diastolic dysfunction was also assessed in both groups. Blood exams were conducted including crp, esr. Severity scales of the disease such as PASI and DAS 28 scores, and the duration of the disease were also recorded.

Results

Linear regression analysis showed significantly impaired global longitudinal peak systolic strain (GLS) in PsA patients (mean =-19.79, s.d. =4.54) compared to the controls (mean =-23.67, s.d. =3.27). Ejection fraction, on the contrary, did not show any significant difference between the two groups. Left ventricular diastolic function did not present significant difference between the two groups. Comparison of GLS with PASI and DAS 28 scores, disease duration, esr and crp showed no association.

Conclusions

PsA patients present a higher risk of left ventricular systolic dysfunction. GLS is a useful tool in revealing myocardial impairment. Moreover, this high risk for systolic dysfunction did not appear to correlate with disease severity markers.

Systolic and diastolic function indexes GroupStatistics group N Mean Std. Deviation Std. ErrorMean p-value E/A Cases 60 1,1510 ,49319 ,06367 0,971 Controls 34 1,1471 ,53555 ,09185 E/E" (average) Cases 60 8,21 3,016 ,389 0,703 Controls 32 7,95 3,096 ,547 GLS (%) Cases 52 -19,795 4,5495 ,6309 <0,001 Controls 34 -23,671 3,2668 ,5602 EF(%) Cases 59 59,24 3,919 ,510 0,986 Controls 34 59,26 10,880 1,866 Differences between PsA patients and control group

Abstract P1767 Figure. GLS in PsA patients and control group