Posterior Mitral Leaflet Prolapse Research Articles

Mitral valve repair with leaflet resection versus preservation for degenerative posterior leaflet prolapse

ObjectivesThe optimal technique for repairing posterior mitral valve leaflet prolapse remains undetermined. We aimed to compare leaflet resection and neochordae implantation in patients undergoing mitral valve repair for posterior leaflet prolapse, focusing on transmitral pressure gradient and recurrence of mitral regurgitation. MethodsWe enrolled patients undergoing mitral valve repair using either leaflet resection or neochordae implantation for single-segment prolapse of posterior mitral valve leaflet between 2000 and 2021 at our institution. Longitudinal outcomes were evaluated after adjustments with inverse-probability-of-treatment weighting. Repeat echocardiographic measurements (n = 3473, 5.4/patient) of transmitral pressure gradient and significant (moderate or severe) mitral regurgitation recurrence were estimated using nonlinear mixed-effect models. Subgroup analyses were conducted based on the size and type of prosthesis. ResultsAmong 639 patients, leaflet resection was used in 479 (75.0%) and neochordae implantation was used in 160 (25.0%). In the inverse-probability-of-treatment weighting adjusted cohort, the risk of death (P = .623) and mitral valve reoperation (P = .340) did not significantly differ between the 2 groups during a median follow-up of 97.3 months. Echocardiographic data showed comparable mean (at 5 years, 3.8 vs 4.0 mm Hg; P = .442) and peak (9.6 vs 10.4 mm Hg; P = .131) pressure gradients between groups, which persisted in most subgroup analyses. However, neochordae implantation was associated with a higher probability of significant mitral regurgitation recurrence compared with leaflet resection (at 5 years, 16.1% vs 7.0%; P < .001). ConclusionsLeaflet resection yielded similar clinical outcomes and transmitral pressure gradients compared with neochordae implantation after mitral valve repair, with a lower mitral regurgitation recurrence rate. These findings underscore the need to reassess the efficacy of neochordae implantation relative to leaflet resection.

IATROGENIC ACUTE MITRAL INSUFFICIENCY FOLLOWING IMPELLA–ASSISTED INTERVENTIONAL PROCEDURE

Abstract Background Short–term left ventricle mechanical assistance device could be useful in assist interventional cardiologist during high–risk procedures. Abiomed Impella is one of the most frequently used device in this respect due to the high flow available with a low rate of reported complications. Here we report a rare but potentially catastrophic complication related to the Impella device. Case Description A 78 years old patient was referred to our Division by a peripheral hospital for a severe, symptomatic (NYHA III) mitral regurgitation (MR) due to posterior mitral leaflet prolapse (with focal eversion of P3) and chordal rupture (Fig 1a). Medical history included a previous admission in the some hospital due to a scheduled Impella–assisted PTCA (left main/left anterior descending artery) . Pre–procedural trans–thoracic ECHO (TTE) did not show significant MR (Fig 1b). Results Patient underwent urgent surgical procedure. At operation postero–medial mitral commissure was seen to be completely everted with multiple rupture of P3 and A3 chordae. Surgical procedure (Fig 1c) included: P3 plication + posterior–medial commissure edge–to–edge plasty + complete mitral semi–rigid ring (Simulus) n.30. Left atrial appendage was also closed using an external Atriclip. Postoperative course was without significant complications except a superficial wound dehiscence which delayed hospital discharge till 20th postoperative day. Conclusion Despite being a feasible and attractive option for short–term mechanical circulation assistance during high–risk interventional procedure, Impella device insertion could cause significant mitral valve damage leading to clinical deterioration. Such potentially catastrophic complication should be, therefore, mandatory excluded before patient discharge following interventional procedure.

What Is the Optimal Mitral Valve Repair for Isolated Posterior Leaflet Prolapse to Achieve Long-Term Durability?

Background This study assessed risk factors for mitral regurgitation (MR) recurrence or functional mitral stenosis during long-term follow-up in patients undergoing mitral valve repair for isolated posterior mitral leaflet prolapse. Methods and Results We assessed a consecutive series of 511 patients who underwent primary mitral valve repair for isolated posterior leaflet prolapse between 2001 and 2021. Annuloplasty using a partial band was selected in 86.3%. The leaflet resection technique was used in 83.0%, whereas the chordal replacement without resection was used in 14.5%. Risk factors were analyzed for MR recurrence ≥grade 2 or functional mitral stenosis with mean transmitral pressure gradient ≥5 mm Hg using a multivariable Fine-Gray regression model. The 1-, 5-, and 10-year cumulative incidence of MR ≥grade 2 was 7.8%, 22.7%, and 30.1%, respectively, whereas that of mean transmitral pressure gradient ≥5 mm Hg was 8.1%, 20.6%, and 29.3%, respectively. Risk factors for MR ≥grade 2 included chordal replacement without resection (hazard ratio [HR], 2.50, P<0.001) and larger prosthesis size (HR, 1.13, P=0.023), whereas factors for functional mitral stenosis were use of a full ring (partial band versus full ring, HR, 0.53, P=0.013), smaller prosthesis size (HR, 0.74, P<0.001), and larger body surface area (HR, 3.03, P=0.045). Both MR ≥grade 2 and mean transmitral pressure gradient ≥5 mm Hg at 1 year post surgery were significantly associated with the long-term incidence of reoperation. Conclusions Leaflet resection with a large partial band may be an optimal strategy for isolated posterior mitral valve prolapse.

Successful bailout clipping for rare complication of anterior mitral leaflet dissection during transcatheter edge-to-edge mitral valve repair

A 91-year-old woman underwent transcatheter edge-to-edge mitral valve repair (TEER) due to severe mitral regurgitation (MR) with the central posterior mitral leaflet (PML) prolapse (Figure 1A; Supplementary data online, Movie S1). The XTW size of MitraClip G4 (Abbott Vascular, USA) was chosen considering the larger length of PML. Before inserting the MitraClip into left ventricle, a newly developed membranous structure was suddenly detected on the central anterior mitral leaflet (AML) by transoesophageal echocardiography (TEE) (Figure 1B). The structure appeared to spread widely from the tip of the central AML toward lateral side (Figure 1C; Supplementary data online, Movie S2) and colour jet flow of TEE penetrated in-between the AML and mobile layered structure (Figure 1D; Supplementary data online, Movie S3). Based on the TEE findings, we suspected AML dissection caused by the proximal edge of MitraClip during one of the procedural steps. The dissection area was localized in the central to lateral side of AML but did not advance further. Therefore, the bailout-TEER was performed to stabilize for both the PML prolapse and iatrogenic AML dissection. The clip was placed under the mitral valve and the clippers grasped both the leaflets (Figure 1E). After successful TEER, the part of AML dissection was minimized (Figure 1F; Supplementary data online, Movie S4). The abnormal colour jet disappeared and trivial amount of MR was present after the procedure (Figure 1G and H). Postprocedural enhanced computed tomography also confirmed absence of abnormal findings in the left atrium around mitral valve. Although extremely rare complication, caution against the occurrence of mitral leaflet dissection during TEER should be exercised.

Surgical Results of Mitral Valve Repair for Mitral Regurgitation in Pediatric Patients with Mitral Valve Prolapse.

The rare incidence of mitral regurgitation (MR) caused by mitral valve prolapse in children lacks clinical management experience. In this study, we present our experience in surgical management and the difference between isolated anterior mitral leaflet (AML), posterior mitral leaflet (PML), and bileaflet prolapse in pediatric patients. Between January 2014 and February 2021, 52 pediatric patients, aged 7.04 ± 4.02years (ranged from 0.17 to 14years), were diagnosed as moderately severe (11, 21.2%) or severe (41, 78.8%) MR, of which, 34 (65.4%) were owing to AML prolapse, one (1.9%) was PML prolapse, and 17 (32.7%) caused by bileaflet prolapse. All had received mitral valve repair, and were divided into the group of AML prolapse and bileaflet prolapse. Demographic and clinical characteristics of all patients were retrospectively collected and analyzed. The left ventricular diameter in the bileaflet group was significantly larger than the AML group (4.04 ± 0.73cm vs. 3.45 ± 0.75cm, P = 0.026). The median follow-up time was 33months (ranged from 6 to 88months). The postoperative and follow-up results had no significant difference between two groups. And only 2 patients received reoperation due to posterior annuloplasty ring dehiscence. For pediatric patients, the majority of the patients had isolated AML prolapse instead of PML prolapse. There was no difference in recurrent regurgitation or reoperation between isolated AML and bileaflet prolapse after MV repair, and we suggest more aggressive surgical strategy should be considered in pediatric patients with bileaflet prolapse than AML prolapse.

P3 mitral valve prolapse in a young patient, an uncommon case

 A 33-year-old patient with no cardiovascular risk factors and no history presented with shortness of breath, palpitations and fatigue. The cardiovascular examination showed an apex beat lateral and inferior displacement and a mitral systolic murmur. The electrocardiogram showed both left ventricular hypertrophy and left atrial hypertrophy. Inflammatory markers were within normal range and blood cultures were sterile. Transthoracic echocardiography showed a severe mitral regurgitation and posterior mitral leaflet prolapse and both left ventricular and auricular dilatation. The transoesophageal echocardiography showed a P3 prolapse with a mobile structure on the posterior mitral valve atrial side suggesting a chordal rupture. The patient had a surgical cure (posterior valve plasty) with good outcomes.

Mitral valve repair with a device for artificial chordal implantation at 2 years

ObjectivesThis study examines the early and midterm safety, efficacy, and durability of mitral valve repair for primary mitral regurgitation (MR) using the ChordArt device (CoreMedic) for chordal replacement. MethodsFive patients with symptomatic severe primary degenerative MR due to isolated central posterior leaflet prolapse/flail were treated with the ChordArt device in a transseptal surgical approach and followed prospectively with periodical clinical and echocardiographic assessments for 2 years. ResultsReduction of MR immediately after the implantation of artificial chords was achieved in all patients showing no or trace MR (<1+/4+). In all patients, MR <1+ was maintained during 24 months of follow-up. No dehiscence, detachment, or dislocation of the implanted ChordArt devices was observed. Transthoracic echocardiography showed that left ventricle end diastolic diameter significantly decreased during the whole follow-up period in comparison to baseline condition, especially at discharge and 1-month follow-up. Left ventricle end systolic diameter also significantly decreased during the whole follow-up period in comparison to baseline condition. Left atrial volume significantly decreased during the follow-up period in comparison to discharge. No major adverse events, as defined per protocol, were observed during the intervention or during the follow-up period. ConclusionsThe ChordArt device allows successful treatment of primary degenerative MR due to posterior mitral leaflet prolapse or flail, with a good safety profile and promising immediate clinical and echocardiographic benefits that are confirmed up to 24 months.

Successful percutaneous mitral valve leaflet repair for primary mitral regurgitation results in functional improvement and positive cardiac reverse remodelling

Abstract Funding Acknowledgements Type of funding sources: None. Background Percutaneous mitral valve leaflet repair can be an effective treatment for primary mitral regurgitation (MR) patients deemed high-risk for surgery. Accurate assessment of cardiac reverse remodelling is essential to optimise future patient selection. Cardiovascular magnetic resonance (CMR) is the reference standard for cardiac volumetric assessment and compared to transthoracic echocardiography (TTE) provides superior reproducibility in MR quantification. Prior CMR studies have analysed cardiac reverse remodelling following percutaneous intervention in combined cohorts of primary and secondary MR patients. However, as aetiology of MR can significantly impact outcomes, focused studies are warranted. Purpose Assess cardiac reverse remodelling and quantify changes in MR following percutaneous mitral valve leaflet repair for primary MR using the reference standard (CMR). Methods 12 patients with at least moderate-severe MR on TTE were prospectively recruited to undergo CMR imaging and 6-minute walk tests (6MWT) at baseline and 6 months following percutaneous mitral valve leaflet repair (MitraClip). CMR protocol involved: left-ventricular (LV) short axis cines (bSSFP, SENSE-2, 10mm, no gap), transaxial right-ventricular (RV) cines (bSSFP, SENSE-2, 8mm, no gap), two and four chamber cines and aortic through-plane phase contrast imaging, planned at the sino-tubular junction. MR was quantified indirectly using LV and aortic stroke volumes. Results 12 patients underwent percutaneous mitral valve leaflet repair (MitraClip) for posterior mitral valve leaflet prolapse, however 1 patient declined follow up after single-leaflet clip detachment resulting in 11 patients (age 83 ± 5years, 9 male) completing follow up imaging. At 6-months: significant improvements occurred in New York Heart Association functional class (Table 1) and 6MWT distances (223 ± 71m to 281 ± 65m, p = 0.005) and significant reductions occurred in indexed left ventricular end-diastolic volumes (LVEDVi) (118 ± 21ml/m2 to 94 ± 27ml/m2, p = 0.001), indexed left ventricular end-systolic volumes (58 ± 19ml/m2 to 48 ± 21ml/m2, p = 0.007) and quantitated MR volume (55 ± 22ml to 24 ± 12ml, p = 0.003) and MR fraction (49 ± 9.4% to 29 ± 14%, p= &amp;lt;0.001). There were no statistically significant changes in left ventricular ejection fraction (LVEF), right ventricular dimensions/ejection fraction or bi-atrial dimensions (Table 1). All patients demonstrated decreased LVEDVi and quantified MR (Figure 1). Conclusion Successful percutaneous mitral valve leaflet repair for primary MR results in reduction in MR, positive LV reverse remodelling, preservation of LVEF, and functional improvements. Larger CMR studies are now required to further guide optimal patient selection.

Atrial Septal Aneurysm Associated with Mitral Valve Prolapse Syndrome

A 68-year-old male presented to the out-patient clinic with history of intermittent palpitations and fatigability for past seven months. On cardiovascular system examination, mid-systolic click and pan-systolic murmur grade III/VI were audible at the apex with radiation to the base of heart. Echocardiographic study revealed atrial septal aneurysm (Figure 1, Figure 2, Video 1 and Video 2), along with prolapse of Posterior Mitral Leaflet (PML) into left atrium (Figure 3 and Figure 4).

Mitral valve repair for isolated posterior mitral valve leaflet prolapse: The effect of respect and resect techniques on left ventricular function

ObjectivePosterior mitral valve leaflet prolapse repair can be performed by leaflet resection or chordal replacement techniques. The impact of these techniques on left ventricular function remains a topic of debate, considering the presumed better preservation of mitral-ventricular continuity when leaflet resection is avoided. We explored the effect of different posterior mitral valve leaflet repair techniques on postoperative left ventricular function. MethodsIn total, 125 patients were included and divided into 2 groups: leaflet resection (n = 82) and isolated chordal replacement (n = 43). Standard and advanced echocardiographic assessments were performed preoperatively, directly postoperatively, and at late follow-up. In addition, left ventricular global longitudinal strain was measured and corrected for left ventricular end-diastolic volume to adjust for the significant changes in left ventricular volumes. ResultsAt baseline, no significant intergroup difference in left ventricular function was observed measured with the corrected left ventricular global longitudinal strain (resect: 1.76% ± 0.58%/10 mL vs respect: 1.70% ± 0.57%/10 mL, P = .560). Postoperatively, corrected left ventricular global longitudinal strain worsened in both groups but improved significantly during late follow-up, returning to preoperative values (resect: 1.39% ± 0.49% to 1.71% ± 0.56%/10 mL, P < .001 and respect: 1.30% ± 0.45% to 1.70% ± 0.54%/10 mL, P < .001). Mixed model analysis showed no significant effect on the corrected left ventricular global longitudinal strain when comparing the 2 different surgical repair techniques over time (P = .943). ConclusionsOur study showed that both leaflet resection and chordal replacement repair techniques are effective at preserving postoperative left ventricular function in patients with posterior mitral valve leaflet prolapse and significant regurgitation.

Quadrangular resection versus chordal replacement for degenerative posterior mitral leaflet prolapse.

BackgroundThe aims of the present study was to compare midterm results of quadrangular leaflet resection versus chordal replacement for the repair of degenerative posterior mitral leaflet (PML) prolapse, and to explore the risk factors for recurrent severe mitral regurgitation (MR).MethodsFrom January 2012 to December 2018, 1,423 consecutive patients underwent mitral valve (MV) repair. A total of 317 had degenerative PML prolapse and constituted the study population. Of these, 74 (23.3%) underwent quadrangular leaflet resection, and 243 (76.7%) underwent chordal replacement. Outcomes were compared by using unadjusted data and propensity score-matched analyses.ResultsPatients with multiple leaflet prolapse were more likely to undergo chordal replacement (18.4% vs. 41.9%, P<0.001), and performed as a minimally invasive approach (47.3% vs. 61.7%, P=0.027). Of the entire cohort, 1 death (0.3%) occurred due to intraoperative aortic dissection, and 1 patient who had undergone chordal replacement required reoperation before discharge for posterior leaflet tearing. There was no significant difference in the probability of freedom from recurrent severe MR at 82 months between the resection and neochordae groups in both the pre-matched (95.6% vs. 88.8%, P=0.105) and matched (95.2% vs. 88.5%, P=0.170) cohorts, which was consistent across all of the examined subgroups (P>0.05). Multivariate Cox regression indicated that dilated left ventricular end-systolic diameter (LVESD) was an independent risk factor for recurrent severe MR [<40 vs. >40 mm, hazards ratio (HR): 3.17, 95% confidence interval (CI): 1.20–8.39, P=0.020]; however, surgical technique was not (resection vs. neochordae, HR: 0.31, 95% CI: 0.07–1.37, P=0.122).ConclusionsChordal replacement for the repair of degenerative posterior MV prolapse yields similar satisfactory outcomes when compared with quadrangular resection, and is promising in minimally invasive cardiac surgery for various lesions. However, it is also associated with more recurrent severe MR, albeit non-significant, for which patients with dilated LVESD are at high risk.

PULMONARY ARTERY PSEUDOANEURYSM DISGUISED AS MALIGNANT LESION

SESSION TITLE: Fellows Procedures Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary artery pseudoaneurysms (PAPs) are rare, associated with cardiovascular disease, and have potentially life-threatening complications such as exsanguination. They may be iatrogenic, or related to trauma, infection, neoplasm, pulmonary hypertension, or vasculitis. Patients with PAPs may be asymptomatic or present with hemoptysis. CASE PRESENTATION: Patient is a 67-year-old female with a significant medical history of PAF, HFrEF, DM, HTN, breast cancer s/p lumpectomy, & colon cancer s/p partial colectomy was admitted for generalized weakness & shortness of breath on exertion. She was admitted about two weeks prior for pneumonia and streptococcus agalactiae bacteremia, developed new onset atrial fibrillation, and was discharged home on Eliquis and Augmentin. Vitals were: T 98 F, BP 110/50, HR 80, RR 16, O2 sat 98 % on RA. Physical exam was unremarkable except trace pitting edema bilaterally. Lab studies including metabolic panel, cell counts, & troponins were unremarkable. BNP was elevated at 290. Echocardiogram showed EF 35%, moderate LVH paradoxical septal motion, posterior mitral valve leaflet prolapse, & moderate circumferential pericardial effusion. Chest x-ray was negative for any infiltrate & CT scan of the chest without contrast showed a right lower lung lobe opacity suspicious for malignancy Her symptoms were thought to be as a result of multifactorial etiologies including possible underlying pneumonia & exacerbation of CHF. Patient was started on broad spectrum antibiotics. Patient was scheduled for CT chest angiogram with IV contrast before proceeding with a biopsy of the right lower lobe mass, which in turn showed a 3 cm pseudoaneurysm with connection to the branch of right lower lobe pulmonary artery. Following this discovery on imaging studies, patient underwent IR-guided ablation & coiling. The first attempt at embolization was aborted after hemodynamically significant extravasation from the right lung was noted after angiographic runs. Patient was intubated for airway protection. While intubated, patient underwent a second successful attempt of IR-guided procedure. Eventually she was extubated and discharged home. DISCUSSION: Pulmonary Pseudoaneurysm (PA) is rare and potentially life threatening. It may be idiopathic or traumatic, infection, primary or metastatic lung neoplasm, Pulmonary HTN, or vasculitis. Most cases are asymptomatic, but may present with dry cough & hemoptysis. MDCT angiogram of the chest remains the investigation of choice in the diagnosis of PA. These cases are managed with Urgent endovascular procedure (Coil Embolization) or Surgery [ Open thoracotomy and aneurysm resection with lobectomy for involved lobes] CONCLUSIONS: Pulmonary artery pseudoaneurysms should be on the differential diagnosis for any lung mass or nodule. Prior to obtaining a biopsy of a suspected lung mass or nodule, patients should always undergo a CT chest with IV contrast. Reference #1: Pulmonary Artery Pseudoaneurysms: Clinical Features and CT Findings Ying Chen1, Matthew D. Gilman1, Kathryn L. Humphrey2, Gloria M. Salazar1, Amita Sharma1, Ashok Muniappan3, Jo-Anne O. Shepard1 and Carol C. Wu1 Reference #2: E. Jean-Baptiste, “Clinical assessment and management of massive hemoptysis,” Critical Care Medicine, vol. 28, no. 5, pp. 1642–1647, 2000. View at: Publisher Site | Google Scholar Reference #3: Lafita V, Borge MA, Demos TC. Pulmonary artery pseudoaneurysm: etiology, presentation, diagnosis, and treatment. Semin Intervent Radiol. 2007;24(1):119-123. DISCLOSURES: No relevant relationships by Sharath Bellary, source=Web Response No relevant relationships by Sudipa Chowdhury, source=Web Response No relevant relationships by William Meng, source=Web Response No relevant relationships by Richard Miller, source=Web Response No relevant relationships by SUMITANAND MISHRA, source=Web Response No relevant relationships by Rutwik Patel, source=Web Response No relevant relationships by Hari Sharma, source=Web Response

The MANTA Vascular Closure Device for Percutaneous Femoral Vessel Cannulation in Minimally Invasive Surgical Mitral Valve Repair.

A 65-year-old Caucasian male was referred to our institution with severe mitral regurgitation due to posterior mitral leaflet prolapse. The patient underwent minimally invasive surgical mitral valve repair. Here we present the application of a new vascular closure device (MANTA) for percutaneous arterial access and closure.

Computer simulations of transapical mitral valve repair with neochordae implantation: Clinical implications.

ObjectivesTransapical beating heart neochordae implantation is an innovative mitral valve repair technique that has demonstrated promising clinical results in patients with primary mitral regurgitation. However, as clinical experience continues to increase, neochordae implantation criteria have not been fully standardized. The aim of this study was to investigate the biomechanical effects of selecting an antero-lateral apical access site compared with a postero-lateral site, and suboptimal neochordae length compared with optimal suture length, on restoring physiologic left heart dynamics.MethodsTransapical neochordae implantation using 3 and 4 sutures was computer simulated under 3 posterior mitral leaflet prolapse conditions: isolated P2, multiscallop P2/P3 and multiscallop P2/P1. Physiologic, pre- and postrepair left heart dynamics were evaluated using a fluid-structure interaction modeling framework.ResultsDespite the absence of residual mitral regurgitation in all postrepair models with optimal neochordae length, selecting an antero-lateral apical entry site for the treatment of P2/P3 prolapse generated a significant increase (>80%) in neochordae tension and P2 peak stress, with respect to a postero-lateral entry site. During isolated P2 prolapse repair, although neochordae overtension by 5% led to minimal hemodynamic changes in the regurgitant volume compared with using an optimal suture length, a significant increase in systolic and diastolic neochordae tension (>300%) and posterior leaflet average stress (70%-460%) was quantified. On the other hand, neochordae undertension by 5% led to worsening of regurgitation severity.ConclusionsThis parametric computer study represents a further step toward an improved understanding of the biomechanical outcomes of transapical neochordae technologies.

Posterior mitral leaflet prolapse with the posteriorly directed jet: feasibility of the MitraClip procedure.

Posterior mitral leaflet prolapse with the posteriorly directed jet: feasibility of the MitraClip procedure.

Very long-term outcomes of twisted auto-pericardial mitral annuloplasty.

During mitral annuloplasty, we twisted the harvested auto-pericardium to enable adequate ring sizing and implanted it to prevent ring-related complications. Indication for twisted auto-pericardial band (APB) was limited to patients with less than severe annular dilation to ensure high reproducibility and durability. The aim of this study was to investigate the long-term outcomes of twisted APB annuloplasty. From 1999 to 2009, 107 patients (62 ± 12years, degenerative 92 and infective endocarditis 15) with isolated posterior mitral leaflet (PML) prolapse with inter-commissural distance of 32mm or smaller underwent twisted APB annuloplasty. Of these, 104 patients without early leaflet repair failure were studied. Leaflet repairs were predominantly performed by quadrangular resection. Median APB size was 28mm. Follow-up rate was 98.1% and duration was 10.9 ± 4.8years. There were two early and 34 late deaths. Survival and freedom from cardiac-related death at 15years were 61.7% ± 5.6% and 83.8% ± 4.7%, respectively. Age and male sex were independent predictors of mortality. There were four late reoperations for recurrent severe mitral regurgitation (MR), three of which were due to new chordal rupture. Freedom from reoperation and freedom from moderate or severe MR at 15years were 93.1% ± 3.5% and 81.3% ± 6.2%, respectively. No patients developed hemolysis, ring dehiscence, or infective endocarditis. The long-term outcomes of twisted APB for isolated PML prolapse without severe annular dilation are satisfactory. Twisted APB annuloplasty may be a preferable option to reduce ring-related complications with sufficient durability.

Comparative quantification of primary mitral regurgitation by computer modeling and simulated echocardiography.

Clinical investigations have demonstrated that mitral regurgitation (MR) quantification using echocardiography (echo) may significantly underestimate or overestimate the regurgitant volume, especially for two-dimensional (2D) echo. Computer modeling and simulated echo were conducted to evaluate the fundamental assumptions in the echo quantification of primary MR that is due to posterior mitral leaflet prolapse. The theoretical flaw of the proximal isovelocity surface area (PISA) method originates from the assumption that the MR flow rate is the product of the isovelocity surface area and aliasing velocity, which is only valid when the velocity vectors are perpendicular to the isovelocity surface. Other factors such as the Doppler angle effect, the view planes of 2D echo, and the single time instant of PISA were also analyzed. We find that the hemielliptic PISA method gives the smallest error for moderate and severe MR cases compared with other PISA methods. Compared with the PISA method, the volumetric technique (VT) is theoretically more robust. By considering correction factors that are caused by nonflat velocity profiles and the closing volume of the aortic valve, the accuracy of the VT method can be significantly improved. The corrected volumetric technique provides more accurate results compared with the PISA methods, especially for mild MR.NEW & NOTEWORTHY We evaluate the accuracy of common echocardiography techniques for the quantification of primary mitral regurgitations using computer modeling. The hemielliptic proximal isovelocity surface area (PISA) method gives the smallest error (within 15%) for moderate and severe mitral regurgitation cases compared with other PISA methods. The volumetric method is theoretically more robust than the PISA method. The accuracy of the volumetric method can be improved by a correction factor around 0.7 because of the nonflat velocity profiles and the closing volume of the aortic valve.

P1724 Bidimensional echocardiographical radial strain abnormalities mandate differential diagnosis between athlete heart syndrome and incipient genetic cardiomyopathy

Abstract Background Left ventricular dilation and mild systolic dysfunction at rest in young endurance athletes may mandate differential diagnosis between adaptive physiology and various forms of incipient non-ischemic dilated cardiomyopathies, especially if family history is positive. Multimodal imaging (i.e. bidimensional echocardiography (TTE), speckle tracking echography (STE) and cardiac magnetic resonance (CMR)) can accurately diagnose "true" underlying heart disease. Purpose We sought to outline the efficacy of multimodality imaging in dilated left ventricular phenotypes with mild systolic dysfunction of athletes. Methods We present the case of a 45 year old Caucasian male, rock climbing athlete (14 hours of training per week), with no prior personal medical history, who was admitted for fast palpitations leading to syncope immediately after a training session. Family history revealed maternal non-ischemic cardiomyopathy with complete atrioventricular (AV block requiring permanent pacing at a similar age. Stable, clinically asymptomatic, nodal complete AV block ∼ 40 bpm was diagnosed by ECG. TTE revealed a dilated LV (61 mm left ventricular end-diastolic diameter (LVIDd)) with no hypertrophy, with decreased diffuse contractility (LVEF ∼ 43%). Exercise echocardiography showed no improvement in LVEF, despite transition from complete AV block to first AV grade block. Moreover, moderate mitral regurgitation by posterior mitral leaflet (PML) prolapse was discovered. STE demonstrated normal global longitudinal strain (GLS), but decreased interventricular septum (IVS) radial strain (RS) (figure A). Late gadolinium enhancement was evident along the interventricular septum and the right ventricular insertion points in CMR (figure B), with no edema. Results The association of LV dilation (with multiple apparent causes – endurance training, genetic substrate, PML prolapse) with mild systolic dysfunction that persisted during echocardiographic exercise testing and a reduced IVS RS (with physiological GLS) was observed. Subsequently, mid-myocardial septal scarring in CMR was consistent with a diagnosis of "true" dilated cardiomyopathy. A cardiac resynchronization defibrillator device was implanted and genetic testing is in progress (lamin disease suspicion). Conclusions Athlete’s heart syndrome phenotypes may overlap with forms of genetic dilated cardiomyopathy. 1. The diagnostic suspicion of structural heart disease derived from echocardiography (radial strain &amp; exercise echocardiography abnormalities), with subsequent confirmation by late gadolinium enhancement myocardial fibrosis. 2. The prospective sudden cardiac death risk requires careful assessment. The presence of clinical arrhythmic syncope, septal CMR fibrosis and specific genetic mutation lead to the decision of ICD (internal cardioverter defibrillator) implantation. Abstract P1724 Figure. A - 2D Radial strain B - LGE CMR

Noonan Syndrome in a 14-year-old Female with Heart Failure

Noonan syndrome is a genetic disorder leading to multiple congenital abnormalities and other health complications, with an incidence of one in 1,000 to 2,500 live births. It is difficult to identify because of its various clinical manifestations. This is the case of a 14-year old female admitted for cardiac failure. The patient was born with normal birth weight but had stunted growth and started walking at the age of five years old. She had speech as well as learning difficulties. She had curly hair, deeply grooved philtrum, ptosis, hypertelorism, micrognathia, low set ears, webbed neck, pectus carinatum, mild thoracic scoliosis and short stature. Her bone age was in line with that of an 8–10 months old infant. Her luteinizing hormone level was below normal. Echocardiography showed severe infundibular pulmonary stenosis, secundum atrial septal defect (ASD), bidirectional shunt with a diameter of 2.1 cm, an anterior rim of 3.71 cm and a posterior rim of 1.17 cm. She also had severe tricuspid regurgitation due to severe infundibular pulmonary stenosis, and moderate mitral regurgitation due to posterior mitral leaflet prolapse. She received hormonal therapy, an angiotensin-converting enzyme inhibitor, a beta-blocker, spironolactone and furosemide. Resection of the infundibular muscle, ASD closure and repair mitral valve was planned. KEYWORDS: diagnosis, management, Noonan syndrome

A RARE CASE OF CULTURE NEGATIVE WHIPPLE’S ENDOCARDITIS

SESSION TITLE: Cardiovascular Cases SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/22/2019 3:45 PM - 4:45 PM INTRODUCTION: Whipple’s disease is a rare opportunistic infection (1 out of 1,000,000). The classical presentation of Whipple’s disease involves joint arthropathy (migratory oligoarticular or polyarticular) and digestive disorders (diarrhea and weight loss). Cardiovascular disease involvement is noted about one third of the time (pericarditis, congestive heart failure [CHF], or rarely culture-negative endocarditis). This case highlights the importance of a high clinical index of suspicion for Tropheryma whipplei culture-negative endocarditis, in the setting of rheumatologic disease and abrupt worsening of cardiac function, without overt gastrointestinal symptoms. CASE PRESENTATION: An 84-year-old man with past medical history of prostate cancer, polymyalgia rheumatica (PMR), cryptogenic stroke, and mild mitral valve prolapse of posterior mitral valve leaflet was admitted for severe mitral insufficiency with recent acute CHF symptoms. Documented diagnosis of PMR occurred 18 months prior to acute CHF symptoms, for which the patient was treated with continuous, extensive prednisone taper, starting at 15 mg daily. Clinical presentation of acute CHF and transthoracic echocardiography showing myxomatous degeneration of posterior mitral leaflet, partially flail, with severe mitral regurgitation, prompted evaluation by cardiothoracic surgery. Patient underwent mitral valve repair and pathology was suggestive of nonbacterial thrombotic endocarditis with vegetative studding of mitral valve anterior leaflet. Serum polymerase chain reaction for T. whipplei was negative and the patient was initially treated with intravenous vancomycin and cefepime. Antibiotic regimen transitioned to intravenous ceftriaxone 2gm daily and ampicillin-sulbactam 3 gm every 6 hours for 6 weeks for seronegative endocarditis of unknown etiology. Genome sequencing of valvular tissue detected T. whipplei deoxyribonucleic acid (DNA) with 16s ribosomal ribonucleic acid (rRNA) gene primer set and Periodic acid-Schiff (PAS) staining was positive. This case of Whipple’s endocarditis is currently being treated with trimethoprim-sulfamethoxazole for an anticipated 12 months. DISCUSSION: This case illustrates the importance of considering T. whipplei as a causal agent of culture-negative endocarditis, especially in the presence of rheumatologic disease and absence of overt gastrointestinal symptoms. Furthermore, in cases of recently diagnosed rheumatologic disease with iatrogenic immunosuppression, risk factors for T. whipplei exposure should be evaluated. The limitations of serum polymerase chain reaction assay as a diagnostic tool for T. whipplei further supports a high suspicion should be maintained in culture-negative endocarditis. CONCLUSIONS: A history of culture-negative endocarditis, in the presence of recently diagnosed and treated rheumatologic disease, without gastrointestinal symptoms should raise suspicion for Whipple's Endocarditis. Reference #1: Fenollar, Florence, et al. “Whipples Disease.” New England Journal of Medicine, vol. 356, no. 1, 2007, pp. 55–66., https://doi.org/10.1056/nejmra062477. Reference #2: Alozie, Anthony, et al. “Arthralgia and Blood Culture-Negative Endocarditis in Middle Age Men Suggest Tropheryma Whipplei Infection: Report of Two Cases and Review of the Literature.” BMC Infectious Diseases, vol. 15, no. 1, 2015, https://doi.org/10.1186/s12879-015-1078-6. Reference #3: Gabus, Vincent, et al. “Tropheryma Whipplei Tricuspid Endocarditis: a Case Report and Review of the Literature.” Journal of Medical Case Reports, vol. 4, no. 1, 2010, https://doi.org/10.1186/1752-1947-4-245. DISCLOSURES: No relevant relationships by SIJAN BASNET, source=Web Response no disclosure on file for Ronald Herb; No relevant relationships by Abigayle Sullivan, source=Web Response No relevant relationships by Emily Zagorski, source=Web Response