Echocardiographic characteristics of cardiac myxoma

Abstract Background Accurate imaging differentiation of various cardiac masses is pivotal due to differences in clinical management and treatment. The most common primary cardiac tumor is cardiac myxoma (CM), which is typically located in the left atrium attached to the interatrial septum. Although benign, serious clinical manifestations may occur and surgical treatment of CM is warranted in most cases. Echocardiography is the most common imaging modality, with a reported sensitivity of 90–96%, however accurate diagnosis can be challenging due to the heterogeneous morphological presentation of CM. Purpose The aim of this study was to determine the utility of echocardiography in CM diagnosis. Methods We retrospectively analyzed the echocardiographic and pathohistological findings of all consecutive patients admitted to our cardiology department for possible CM between 2005 and 2020. Results During the 15-year period, 73 patients were admitted for diagnostic evaluation of a possible CM. Subsequently, 54 patients (74%) were diagnosed with CM or another non-myxomal (NM) cardiac tumor, while in others cardiac masses of other etiologies (thrombus, infective endocarditis, etc.) were diagnosed (Figure 1). All but one patient with CM or NM cardiac tumor were treated surgically at our institution and pathohistological specimens were obtained from the resected tumor. There was a significant female preponderance (n=34, 63%) and the mean age at the time of surgery was 64±14 years. Based on the preoperative echocardiographic findings, 45 (85%) tumors were diagnosed as CM and 8 as NM cardiac tumors (Figure 1). Evaluation of pathohistological specimens revealed CM in 39 of 53 (74%) operated patients. Patohistiologically, a NM cardiac tumor was diagnosed in 7 patients who were preoperatively classified as CM. The sensitivity and specificity of preoperative echocardiography for the detection of CM were 97% and 50%, respectively (Figure 1). The echocardiographic characteristics of pathohistologically confirmed CM were compared to cases of NM cardiac tumors. The comparison between the two groups revealed statistically significant differences in localization and tumor size. All NM cardiac tumors were located in an atypical position and 72% of CM were found in a typical position within the left atrium (p&amp;lt;0.001). NM cardiac tumors were also significantly smaller than CM (25.1±12.6 mm vs. 37.5±18.5 mm, p=0.029). Conclusion Our single-center study confirms the excellent sensitivity of echocardiography for CM diagnosis. The specificity of echocardiography was modest, thus caution is warranted due to a wide differential diagnosis of CM. The diagnosis of CM seems to be less likely with atypical tumor location and small tumor size. Other non-invasive imaging modalities such as cardiac computed tomography or magnetic resonance imaging should be considered in such cases. Funding Acknowledgement Type of funding sources: None. Figure 1. Flow chart showing the number of patients diagnosed with CM on preoperative echocardiography. </jats:sec

Left ventricular strain analysis-the importance of being expert

Abstract Funding Acknowledgements Type of funding sources: None. OnBehalf - Background Myocardial strain imaging using speckle-tracking echocardiography is widely used in both research and clinics. Left ventricular global longitudinal strain (GLS) has proven to be a reproducible and useful tool in clinical practice.  However, data about the variability in global and segmental strain among novice are limited. Purpose The aim of this study was to evaluate how the variability of strain measurements depends on the experience of the physician performing the analysis. Furthermore, we tried to assess how image quality and presence of pathology influence the diagnostic performance of the analysis. Methods Twenty novice (no or limited experiences with strain analysis) and 5 experts analysed offline 3 cases: a healthy adult and a patient with ischemic cardiomyopathy, both with high image quality, and a healthy adult with suboptimal image quality (insufficient tracking in two segments). Frame rates were 65, 51, and 70 fps, respectively. Left ventricular GLS and segmental longitudinal peak systolic strain were quantified using the automated function imaging protocol from vendor-specific offline analysis software. The absolute difference in GLS and segmental strain between each novice readers and experts was calculated. Mean strain measurements from the experts were used as a comparator. Results Absolute values of GLS ranged from -13.6% to -20.1% in the novice group, while GLS ranged from -15.6% to -18.8% in the expert group. The absolute difference in GLS was significantly higher in the novice group compared to the expert group (P &amp;lt; 0.001). Absolute differences in GLS varied significantly among cases, with low variability in healthy adult with high image quality (Panel A) and with significantly higher variability between novices and experts in case with suboptimal image quality and ischemic cardiomyopathy (Panel B-C). The absolute differences in segmental longitudinal peak systolic strain were up to 11.9% strain unit in novice group and up to 6,8% strain unit in experts (P &amp;lt; 0.001 between groups). In novice group, highest absolute differences in segmental strain were observed in the region with suboptimal tracking and in infarct region in patient with ischemic cardiomyopathy (Panel D-E). Conclusion Left ventricular strain analysis by a reader with no or limited experiences severely affects the diagnostic potential of this method. Significant variability in strain measurements should be considered especially in cases with suboptimal image quality and in cases with regional left ventricular pathology. Abstract Figure. </jats:sec

P639 Dehiscence of Bio-Bentall aortic graft after repeated staphylococcus aureus infective endocarditis

Abstract Introduction Echocardiography is the primary imaging modality in prosthetic valve endocarditis (PVE). It is characterised by a lower incidence of vegetations and a higher incidence of perivalvular complications, including valve dehiscence and must be suspected in case of a new periprosthetic regurgitation, even without vegetation or abscess. Multimodality approach is mandatory to detect penetration of the process into the valve ring, aortic root or ascending aorta for complete operative preparation. Case presentation A 22-year old male, with history of Bio-Bentall procedure due to Staphylococcus aureus (S.aureus) infective endocarditis on the mechanical aortic valve (AV) two years priorly, presented to the emergency department with sudden dyspnea without fever. He was treated for pneumonia due to increased inflammatory parameters and bilateral pulmonary infiltrates on X-Ray. Due to complete regression of infiltrates over the night, transthoracic echocardiography (TTE) was performed, revealing almost complete dehiscence of the AV graft with most of the antegrade and retrograde flow through the pseudoaneurismatic sac, communicating with the ascending aorta at the distal graft dehiscence, no clear vegetation was seen. With clearly visible valve and supravalvular pathology of the AV on TTE, we proceeded to computed tomography angiography (CTA) of the thoracic aorta, which showed dissection and delineated rupture of Bio-Bentall graft. The pseudoaneurismatic sac surrounding bulbar portion of Bentall graft, communicating with the left outflow tract and sinus Valsave was seen, compressing ostial portion of the left main and right coronary artery. Re-Bio-Bentall procedure and venous grafting of the left anterior descendant and right coronary artery were performed. Intraoperative transthoracic transesophageal echocardiography (TEE) confirmed the findings, already provided by TTE and CTA. Hemocultures as well as sonication of the removed graft remained negative for bacteria. Postoperatively, left ventricular failure developed, requiring VA ECMO. On postoperative CTA, changes were consistent with usual postprocedural changes. After prolonged rehabilitation, the patient was released home, clinically stable, but with severely reduced ejection fraction of the left ventricle, severe diastolic dysfunction and mild mitral regurgitation. Conclusion TTE is a very useful, non-invasive imaging method in diagnosing PVE and its complications, which can be upgraded with TEE or CTA to provide additional information on the ascending aorta. In a patient, with the past history of repetitive S. aureus infective endocarditis, presenting with Bio-Bental dehiscence, PVE cannot be excluded completely. Even though the timing for follow-up imaging is not well defined in current guidelines, patients with dehiscence of prosthetic valve or graft present a high risk group, demanding individual follow-up planning and lower threshold for imaging referral. Abstract P639 Figure. Dehiscence of aortic valve graft </jats:sec

P627 Step by step valve destruction in infective endocarditis with no apparent vegetations

Abstract Background Infective endocarditis can present without evident vegetation, diagnosis is challenging and prognosis very poor. We present an illustrative case where natural evolution of the mitral valve destruction with no evident vegetations was followed with frequent consecutive transthoracic (TTE) and transesophageal echocardiography (TOE). Case presentation 71-year old male with known dilated cardiomyopathy presented with dyspnoea, ankle swelling and severe kidney failure with hyperkalemia. During short hospitalization he was recompensated with haemodialysis, parenteral diuretics and inotropes. TTE showed dilated left ventricle with severe systolic dysfunction and no evidence of valvular disease. Few days after discharge he was readmitted with malaise and febrile state with no obvious site of infection. Blood cultures were positive for Staphylococcus aureus and antibiotic therapy was initiated immediately. Weekly TTEs and TOEs were performed (Figure 1, column A-D): Week 1: TTE was performed due to congestive heart failure. There was no suspicion on disease and TTE showed no obvious mitral valve pathology. Week 3: Second TTE showed only light thickening of posterior mitral leaflet with mild mitral regurgitation. Week 4: Follow-up TOE was performed showing posterior leaflet discontinuity with small eccentric regurgitation jet and no vegetation. Week 6: Symptoms of congestive heart failure persisted despite antibiotic treatment. A progressive destruction of posterior leaflet with evident perforation of P1 scallop and consequent severe mitral regurgitation. Patient was referred for urgent mitral valve replacement. Conclusions Staphylococcus aureus is a destructive pathogen and can cause severe destruction of native valve even without obvious vegetations. This case presents echocardiographic features of natural course of infective endocarditis on mitral valve. Despite antibiotic therapy progressive valve destruction is possible. Abstract P627 Figure. </jats:sec

P1690 Dynamic mitral stenosis and Liebman-Sacks endocarditis associated with primary antiphospholipid syndrome

Abstract Primary antiphospholipid syndrome (PAPS) is a rare immune-mediated acquired thrombophilia defined by vascular thrombosis, and/or pregnancy morbidity associated with the presence of persistent antiphospholipid antibodies (aPL) in the absence of any other related disease. Although common cardiac involvement predominantly with heart valve disease is clearly defined in secondary APS associated with systemic lupus erythematosus (SLE), its prevalence in PAPS is still a matter of debate. We report a clinical case of a 33 year old female with PAPS. She was diagnosed at her age of 23 after suffering from severe preeclampsia with HELLP syndrome and stillbirth in the 26th week of pregnancy. She was treated with aspirin and was later able to carry 2 full-term pregnancies. Subsequently, some features of SLE: proteinuria, high aPL titers, presence of antinuclear antibodies, hepatosplenomegaly and thrombocytopenia, were observed and treatment with chloroquine and perindopril were added. At 32 years of age she developed symptomatic epilepsy due to chronic ischemic changes of deep white brain matter (visible on MRI). Because of an audible heart murmur an echocardiogram was performed which revealed a thickened mitral valve (MV) annulus and distal free margins of mitral leaflets. A small (5 x 6 mm) verrucous vegetation on the ventricular side of the MV was also discerned. These changes resulted in mild to moderate mitral stenosis (MS) (MV area 1.8 cm2, mean pressure gradient - PG 8 mmHg), mild valvular regurgitation and mild postcapillary pulmonary hypertension (right ventricular systolic pressure - RVSP 40 mmHg). With negative blood cultures vegetations were attributed to Liebmann Sacks endocarditis (LSE). The patient refused a transesophageal echocardiogram, however a stress echocardiogram was performed which revealed moderately decreased physical capacity (90 W; 58 % of expected). At 25 W we noted a disproportional increase of mean trans-mitral PG from 11 mmHg to 25 mmHg and RVSP rise from 40 mmHg to 60 mmHg, indicating dynamic, possibly clinically significant MS. While, according to European and American guidelines, interventional treatment (valve repair or replacement) is reserved for severe symptomatic MS there are no recommendations for borderline severe/dynamic MS with concomitant, possibly symptomatic LSE. In our patient discerning the cause of ischemic brain lesions remains a challenge since they might be of cardiac origin or due to vascular thrombogenicity of the PAPS itself. Nonetheless with clear evidence of disease progression in a patient who is currently refusing surgical treatment, a combined treatment with coumarin and aspirin in combination with statin, chloroquine, perindopril and an uptitratable dose of β-blocker was started. We decided for initial monthly echocardiographic follow-up to assess disease dynamics and to ensure possible timely surgical treatment. Significant dynamic MS due to LSE is a rare and challenging complication of PAPS.</jats:p

Disproportionate mitral regurgitation: new entity or reflection of measurment errors?

Abstract Funding Acknowledgements Type of funding sources: None. Background The results of recent studies of transcatheter mitral valve repair proposed a new conceptual framework that categorized mitral regurgitation (MR) into proportionate (propMR) or disproportionate (dispropMR) according to the relationship between effective regurgitant orifice area (EROA) and left ventricular (LV) end-diastolic volume (EDV). Purpose To determine the prevalence of dispropMR in consecutive heart failure patients with reduced ejection fraction (HFrEF) undergoing clinically indicated echocardiography over one year period and to examine characteristics of this new entity. Methods We retrospectively identified 179 patients(age:69 ± 12 years, male:132[74%]) with HFrEF who were classified more than mild MR by performing echocardiographer. Following parameters of MR severity were analysed: regurgitant volume(PISA-based regurgitant volume[RVol-PISA] and RVol calculated by the difference of total LV stroke volume by LV planimetry and Doppler-estimated effective LV stroke volume[RVol-SV]), PISA-based EROA and regurgitant fraction (RF). Grading of MR severity based on RVol was performed (mild:&amp;lt;30 ml, mild-moderate:30-44ml, moderate-severe:45-59 ml, severe:≥60 ml). The distinction between propMR and dispropMR was determined by using a proportionality scheme by Grayburn, considering ratio EROA/LVEDV. DispropMR was identified by the ratio greater than 0.14, while the others were classified as propMR. Results In our cohort, 49(27.4%)patients had dispropMR. Both MR groups were comparable in age and gender. DispropMR group had significantly smaller LV dimensions(LV end-diastolic diameter:59 ± 9mm vs. 65 ± 8mm,p &amp;lt; 0.001; LVEDV:164 ± 54ml vs. 222 ± 60ml,p &amp;lt; 0.001) and higher EF(41 ± 11% vs. 34 ± 9%, p &amp;lt; 0.001). Higher proportion of primary MR was noted in dispropMR group(15[31%] vs. 4[3.3%] patients, p &amp;lt; 0.001). Significant differences were observed in PISA-based quantification of MR between both groups (p &amp;lt; 0.001, for all), whereas RVol-SV was comparable(p = 0.667;Figure A). Discrepant grading in MR severity between RVol-PISA and RVol-SV methods was observed(p &amp;lt; 0.001), with significant high discordance in dispropMR(p &amp;lt; 0.001) and no significant differences in propMR(p = 0.187;Figure B). Additionally, difference in RVol assessed by PISA method and SV method were more prominent in dispropMR (RVol difference: dispropMR:27 ml[17-46] vs. propMR:13 ml[-4 to 24],p &amp;lt; 0.001). MR severity would be reclassified in a substantial proportion of dispropMR when considering RVol-SV. Conclusion Our results suggest that dispropMR may be found in roughly one fourth of echocardiographic studies in patients with HFrEF. DispropMR patients have less extensive LV remodelling and more severe MR based on PISA parameters compared to propMR. However, inconsistencies between parameters of MR severity in dispropMR might suggest echocardiographic limitations of quantitative grading of the MR severity or/and LV volume assessment rather than a new pathophysiological concept of disproportionate MR. Abstract Figure A, B </jats:sec