Severe Mitral Valve Regurgitation Research Articles

TMVR after TA-TAVR: a re-redo surgery—case report

IntroductionTranscatheter mitral valve replacement (TMVR) is a valuable treatment option in patients with severe mitral regurgitation. Prior transapical transcatheter aortic valve replacement (TA-TAVR) may complicate the procedure and is therefore considered a relative contraindication. In this case report, the authors describe the successful TMVR as a tertiary cardiac surgery and transapical redo procedure.Case SummaryAn 83-year-old male patient, suffering from dyspnoea and angina, was diagnosed with severe mitral valve regurgitation (MR). He had already undergone cardiac surgery in the form of coronary artery bypass grafting at the age of 64 and TA-TAVR at 79 years. After a failed attempt at mitral valve transcatheter edge-to-edge repair, he opted for TMVR. Pre-TMVR computed tomography simulation was used to analyse possible interactions between the prostheses and to predict the neo-left ventricular outflow tract (neo-LVOT). The operation was carried out without complications. There was no bleeding and the LV function remained unchanged. On MRI, the valves were perfectly aligned without any signs of paravalvular leakage or LVOT obstruction. The patient was discharged seven days postoperatively. At the one-year follow up, there was no need for rehospitalisation and the patient had clinically improved (from NYHA IV to II). Echocardiography demonstrated a mean transvalvular gradient of under 5 mmHg and no residual MR.ConclusionA redo transapical access for TMVR as a tertiary cardiac operation can be easily performed. Pre-operative CT suggested good alignment of the aortic and mitral valved stent which was confirmed postoperatively.

CHRONIC INFECTION AND SEVERE MITRAL REGURGITATION: A TRICKY CASE REPORT

Abstract Background Techniques of mitral valve repair has improved in the last decades. Percutaneous approach is now a reliable and safe therapy in those patients with high surgical risk. Of note, the presence of implanted prothesis may increase the risk of endocarditis, especially in those patients experiencing recurrent infection. Case Summary We describe a case of a 75–year–old with medical history of chronic lymphedema. It was characterized by periodical episode (yearly) of cellulitis, with a rapid onset of fever (38.5–39° C), lasting for a few days after paracetamol therapy. Patients had severe mitral valve regurgitation due to prolapse of the posterior leaflet (P2). In December 2022, he had percutaneous edge–to–edge mitral valve. Three months later the patient was admitted to the emergency department of our hospital due to fever without any specific symptoms. The patient was well compensated, normal blood pressure, heart rate and oxygen saturation. He had emocolture which revealed the presence of Staphilococcus lugdunensis. Transesophageal echocardiogram confirmed the presence endocarditis, showing a huge (10x12 mm) vegetation at the level of mitral device (Fig 1 and 2). Patients was then referred to cardiac surgery for urgent mitral valve replacement with mitral bioprothesis (Fig 3). He was treated with ev antibiotic for six weeks after cardiac surgery. Additionally, chronic treatment with amoxicillin 500 mg/day was also prescribed at discharge. After six months follow–up, patients had no signs of endocarditis. Conclusion The management and treatment of chronic infection is of paramount importance to reduce the risk of prothesis infection procedure in patients needing a prothesis implantation. The last European Society of Cardiology guidelines on endocarditis do not provide any recommendation regarding a long–term treatment of patients with high risk of infection (i.e. due to chronic infection) and with an implanted cardiac device/prothesis. We believe this represents an important unmeet need.

Staged biventricular repair in a premature neonate with critical aortic stenosis, severe mitral regurgitation, and fetal hydrops: a case report

BackgroundThe surgical management of critical aortic stenosis, mitral regurgitation, and left ventricular dysfunction is a significant clinical challenge. Whether left ventricular function will recover to support systemic circulation after the relief of aortic stenosis is a concern. In this setting, surgical or balloon aortic valvotomy combined with bilateral pulmonary artery banding and atrial septectomy may allow time for left ventricular adaptation, while the systemic circulation is supported by the right ventricle through the ductus arteriosus. We describe the case of a premature neonate with critical aortic stenosis, severe mitral regurgitation, and fetal hydrops who successfully underwent staged biventricular repair after bilateral pulmonary artery banding, atrial septectomy, balloon aortic valvuloplasty, and stent implantation for ductus arteriosus.Case presentationA 29-year-old female was referred to our hospital at 25 weeks of gestation with fetal echocardiography findings of critical aortic stenosis, severely impaired left ventricular function, severe mitral regurgitation, and restrictive foramen ovale. At 33 weeks of gestational age, the baby was born via cesarean delivery. Prostaglandin E1 infusion was immediately initiated, and the neonate underwent emergecy bilateral pulmonary artery banding and atrial septectomy. On the second day, a balloon aortic valvuloplasty was performed. The neonate underwent stent implantation to open the ductus arteriosus and multiple-balloon aortic valvuloplasty. At 4 months of age, he underwent biventricular repair consisting of surgical aortic valvuloplasty, atrial septal defect closure, bilateral pulmonary artery debanding, and ductus arteriosus ligation. At 1 year of age, he underwent the Ross –Konno procedure. Six years after the operation, the patient’s general condition was stable, and the patient is doing well.ConclusionsStaged biventricular repair was successfully achieved in a premature neonate with fetal hydrops and critical aortic stenosis associated with severe mitral valve regurgitation and left ventricular dysfunction.

Predicting outcomes in patients with low ejection fraction undergoing coronary artery bypass graft

IntroductionReduced left ventricular ejection fraction (LVEF) is a well-known predictor of adverse events after cardiac surgery. We aimed to assess the outcomes in patients with low LVEF undergoing coronary artery bypass graft. MethodsIn this retrospective cohort, we included all patients with left ventricular ejection fraction ≤ 40 who underwent coronary artery bypass grafting between March 2007 and March 2016 (with a median follow-up of nine years) at Tehran Heart Center. Demographics and clinical characteristics were extracted from the data registry. Akaike information criterion (AIC) was used. The univariate Cox regression was performed. We investigated the predictors of mortality and major adverse cardiac and cerebrovascular events (MACCE) using Cox multivariable regression. ResultsIn total, 5,532 cases (79 % male) with a mean age of 65.58 were included in the study. The nine-year overall survival was calculated at 68 %, and more than half of the patients had MACCE (55 %). In adjusted multivariable Cox regression analysis, moderate to severe mitral valve regurgitation, glomerular filtration rate ≤ 60, mild right ventricular dysfunction, and valvular heart disease independently predicted higher mortality. The abovementioned predictors and peripheral vascular disease significantly increased MACCE. ConclusionOur study indicates the clinical significance of mitral regurgitation, valvular heart disease, and renal function in patients with low ejection fraction treated by coronary artery bypass grafting surgery. Identifying predictors of adverse events can help with clinical decision-making and risk stratification, ultimately improving patient outcomes.

Early and late Results after Surgical Mitral Valve Repair: A High-Volume Center Experience.

Surgical mitral valve repair is the gold standard treatment of severe primary mitral valve regurgitation (MR). In the light of rapidly evolving percutaneous technologies, current surgical outcome data is essential to support heart team-based decision making. This retrospective, high-volume, single-center study analyzed in 1779 patients with primary MR early morbidity and mortality, postoperative valve function, and long-term survival after mitral valve repair. Surgeries were performed between 2009 and 2022. Surgical approaches included full sternotomy (FS) and right-sided mini-thoracotomy (MIC). Of the surgeries (mean age: 59.9 (SD:11.4) years; 71.5% males), 85.6% (n=1527) were mini-thoracotomies. Concomitant procedures were performed in 849 patients (47.7%), including tricuspid valve and/or atrial septal defect repair, cryoablation and atrial appendage closure. The majority of patients did not need erythrocyte concentrates. Mediastinitis and re-thoracotomy for bleeding rates were 0.1 and 4.3%, respectively. Reoperation before discharge for failed repair was necessary in 12 patients (0.7%). Freedom from more than moderate MR was >99%. Thirty-day mortality was 0.2% and did not differ significantly between groups (P=0.37). Median follow-up was 48.2 months with a completeness of 95.9%. Long term survival was similar between groups (P=0.21). In the FS and MIC groups, 1-year, 5-year and 10-year survival was 98.8 and 98.8%, 92.9 and 94.4%, and 87.4 and 83.1%, respectively. Mitral valve surgery, both minimally-invasive and via sternotomy, is associated with high repair rates, excellent perioperative outcomes, and long-term survival. Data underscore the effectiveness of surgical repair in managing MR, even in the era of advancing interventional techniques.

Unusual origins of cardiac insufficiency: a case of iliac arteriovenous fistula post-spinal disc surgery

In this case report, we present the unique and intriguing case of a 57-year-old man who experienced exertional palpitations and shortness of breath for 5 years. He was diagnosed with idiopathic heart failure three years ago, leading to diuretic treatment. Physical examination revealed notable left lower extremity swelling, severe varicose veins, and cardiac murmurs. Echocardiography showed significant cardiac enlargement and severe functional mitral and tricuspid valve regurgitation. Computed tomography (CT) imaging uncovered a 10 mm left common iliac arteriovenous fistula, causing abnormal early filling of the inferior vena cava (IVC) and marked IVC dilation. Open surgical repair of the arteriovenous fistula resulted in symptom relief and improved cardiac function. This case underscores the importance of considering unusual causes in heart failure patients and highlights the value of early diagnosis and intervention in complex cardiac-vascular interactions.

Risk of infective endocarditis after hybrid melody mitral valve replacement in infants: the French experience.

Surgical management of mitral valve disease is challenging in infants <1 year old. We aimed at reviewing the French experience with Melody mitral valve replacement in critically ill infants. A retrospective cohort study reporting the French experience with Melody mitral valve replacement. Seven symptomatic infants [complete atrioventricular septal defect (n = 4, Down syndrome: n = 3), hammock valve (n = 3)] underwent Melody mitral valve replacement [age: 3 months (28 days to 8 months), weight: 4.3 kg (3.2-6.4 kg)] because of severe mitral valve regurgitation (6) or mixed valve disease (1) and 14 mm (11-16 mm) mitral valve annulus. In 2 patients whose valve was felt irreparable, Melody mitral valve replacement was performed straightaway. The others underwent 2 (1-3) previous attempts of valve repair; 3 were on extracorporeal membrane oxygenation. Melody mitral valve replacement led to competent valve and low gradient [3 mmHg, (1-4 mmHg)]. One patient died 3 days post-implant from extracorporeal membrane oxygenation-related stroke. Of the 6 discharged home patients, 3 (50%) were readmitted for a definite diagnosis (1) or high suspicion (2) of infective endocarditis, of which 2 died. Over the follow-up, 1 underwent balloon expansions of the valve at 9- and 16-months post-implant, and mechanical mitral valve replacement at 2 years; another is currently planned for transcatheter Melody valve dilation. Melody mitral valve replacement may be considered in selected infants with small mitral valve annulus as an alternative to mechanical mitral valve replacement. Our experience highlights a high-risk of late infective endocarditis that deserves further consideration.

Prognostic value of mitral valve regurgitation in patients with heart failure with mildly reduced ejection fraction

BackgroundAlthough mitral valve regurgitation (MR) is a common valvular heart disease in patients with heart failure (HF), there is a paucity of data on the characterization and outcomes of patients with HF with mildly reduced ejection fraction (HFmrEF) and concomitant MR. MethodsFrom 2016 to 2022, consecutive patients hospitalized with HFmrEF (i.e., left ventricular ejection fraction from 41% to 49% and signs and/or symptoms of HF) were retrospectively included at one institution. Patients with MR were compared with patients without MR. Further risk stratification was performed according to MR severity and etiology (i.e., primary vs. secondary MR). The primary end point was all-cause mortality at 30 months (median follow-up), and the key secondary end point was hospitalization for worsening HF. ResultsOf 2181 patients hospitalized with HFmrEF, 59% presented with mild, 10% with moderate, and 2% with severe MR. MR was associated with increased all-cause mortality at 30 months (HR = 1.756; 95% CI 1.458–2.114; p = 0.001), with higher risk in more advanced stages. Furthermore, MR patients had higher risk of HF-related re-hospitalization at 30 months (HR = 1.560; 95% CI 1.172–2.076; p = 0.002). Even after multivariable adjustment, mild, moderate, and severe MR were still associated with all-cause mortality. Finally, the risk of all-cause mortality was lower in patients with secondary MR compared with patients with primary MR (HR = 0.592; 95% CI 0.366–0.956; p = 0.032). ConclusionMR is common in HFmrEF and independently associated with higher risk of all-cause mortality and HF hospitalization.

Long-term outcomes and quality of life following implementation of dedicated mitral valve Heart Team decisions for patients with severe mitral valve regurgitation in tertiary cardiovascular care center.

This study was purposed to investigate which treatment strategy was associated with the most favourable prognosis for patients with severe mitral regurgitation (MR) following Heart Team (HT)-decisions implementation. In this retrospective study, long-term outcomes of patients with severe MR qualified after HT discussion to: optimal medical treatment (OMT) alone, OMT and MitraClip (MC) procedure or OMT and mitral valve replacement (MVR) were evaluated. The primary endpoint was defined as cardiovascular (CV) death and the secondary endpoints included all-cause mortality, myocardial infarctions (MI), strokes, hospitalizations for heart failure exacerbation and CV events during a mean (standard deviation [SD]) follow-up of 29 (15) months. From 2016 to 2019, 176 HT meetings were held and a total of 157 participants (mean age [SD] = 71.0 [9.2], 63.7% male) with severe MR and completely implemented HT decisions (OMT, MC or MVR for 53, 58 and 46 patients, respectively) were included into final analysis. Comparing OMT, MC and MVR groups statistically significant differences between the implemented procedures and occurrence of primary and secondary endpoints with the most frequent in OMT-group were observed (p < 0.05). However, for interventional strategy MC was non-inferior to MVR for all endpoints (p > 0.05). General health status assessed at the end of follow-up were significantly the lowest for MVR, then for MC and the highest for OMT-group (p < 0.01). In the present study it was demonstrated that after careful HT evaluation of patients with severe MR at high risk of surgery, percutaneous strategy (MC) can be considered as equivalent to surgical treatment (MVR) with non-inferior outcomes.

Totally endoscopic concomitant aortic and mitral valve surgery in junctional epidermolysis bullosa: a case report

BackgroundJunctional epidermolysis bullosa is a rare skin and mucosal disorder characterized by blister formation in response to minor trauma and extracutaneous manifestations. There have been no reports of cardiac surgery and prognostication in patients with epidermolysis bullosa due to skin and mucosal fragility.Case presentationA 55-year-old man presented with congenital junctional epidermolysis bullosa, hypertension, and vasospastic angina. He complained of dyspnea on exertion, and transthoracic echocardiography revealed severe aortic valve regurgitation, moderate aortic valve stenosis (tricuspid valve), and severe mitral valve regurgitation. Considering that the skin condition in the right chest wall was relatively healthy, the right thoracotomy approach was preferred and totally endoscopic concomitant mitral valve repair and aortic valve replacement were performed using a sutureless bioprosthetic valve (Perceval™ (Corcym, Group, Milan, Italy)). Polyurethane and silicon dressing foams were used to protect the skin at the site of contact with the bag valve mask, arterial pressure catheter, intravenous catheter, and the tracheal intubation tube. Vertical mattress sutures were used for the skin sutures. The postoperative course was uneventful, and the patient was discharged nine days after the operation. There was no indication for reoperation until three years follow-up period.ConclusionsThe totally endoscopic concomitant aortic and mitral valve surgery using Perceval™ prosthesis can be performed safely in patients with junctional epidermolysis bullosa by adequate protection of the skin and mucosa.

An Unusual Presentation of Partial Atrioventricular Canal Defect in an Elderly Woman

A partial atrioventricular canal defect (PAVCD) is a form of endocardial cushion defect. It’s a rare congenital cardiac malformation, few reported patients have survived into the seventh decade of life. A 71-year-old female with a history of diabetes and hypertension was admitted for heart failure (HF) symptoms. Transthoracic echocardiography revealed a left to right shunt in the basal portion of the interatrial septum, 19mm ostium primum atrial septal defect (ASD), a mitral valve cleft, and severe mitral valve regurgitation. PAVCD consists of an ostium primum ASD and the dividing of the common atrioventricular (AV) valve into 2 separate AV orifices, resulting in a so-called cleft in the mitral valve. The ventricular septum is intact. Echocardiography is the key tool for diagnosis and anatomic classification. Surgical repair is recommended in adults with primum ASDs causing impaired functional capacity, right atrial or right ventricular enlargement, and left-to-right shunt without cyanosis, as long as systolic pulmonary artery pressures are lower than 50% of systemic pressures. Reconstructive surgery includes, in addition to the closure of the interatrial septum, a suturing of the mitral cleft. The estimated mean age at death of the patients with ostium primum ASD is about 40 years. The objective of this clinical case is to highlight the good clinical tolerance of atrial shunts and the anatomical particularities in the PAVCD.

Predictor of mitral valve regurgitation severity and left ventricular dilatation using amino-terminal pro-brain natriuretic peptide marker in pediatric rheumatic heart disease.

Early rheumatic heart disease (RHD) is characterized by valve regurgitation, leading to ventricular distention and possible elaboration of amino-terminal pro-brain natriuretic peptide (NT-proBNP). Thirty-one children with RHD were entered in the study. Transthoracic echocardiography was performed in all patients to assess the severity of the valve disease and cardiac function, thus will be classified into three groups: mild, moderate, and severe mitral valve regurgitation (MR), with and without left ventricular (LV) dilatation. At the time of echo, venous blood samples were drawn; thus, NT-proBNP levels were measured using sandwich immunoassay. The median NT-proBNP levels in mild, moderate, and severe MR were 32.34, 120.75, and 7094 pg/ml, respectively. The median NT-proBNP levels in patients with and without LV dilatation were 3045 and 30.82 pg/ml. There was a significant correlation between the severity of MR and NT-proBNP levels (P < 0.001), thus a significant correlation between LV dilatation and NT-proBNP levels (P = 0.013). A cutoff value of 2598.50 pg/ml was obtained with a sensitivity value of 90% and a specificity of 90.5% for NT-proBNP levels against severe MR. A cutoff value of 199.35 pg/ml was obtained with a sensitivity value of 73.3% and a specificity of 75% for NT-proBNP levels on LV dilatation. A cutoff value of 2598.50 pg/ml was obtained with a sensitivity value of 85.7% and a specificity of 79.2% for NT-proBNP levels against severe MR with LV dilatation. There was a significant relationship between NT-proBNP levels and the severity of MR and LV dilatation in children with RHD.

Kiosk 6R-TA-01 - Impact of Mitral Valve Repair on Left Atrial Fibrosis in Patients with Severe Mitral Valve Regurgitation: Insights FBom the ALIVE Trial

Kiosk 6R-TA-01 - Impact of Mitral Valve Repair on Left Atrial Fibrosis in Patients with Severe Mitral Valve Regurgitation: Insights FBom the ALIVE Trial

Increased Left Ventricular End-Diastolic Volume Index Is Associated With Increased Adverse Events After MitraClip Implantation

This study investigated the association between the left ventricular end-diastolic volume index (LVEDVI) and the incidence of adverse clinical events in patients after MitraClip implantation. In this retrospective, observational study, 123 patients who underwent the MitraClip procedure were enrolled. Participants were divided into 2 groups according to the LVEDVI cut-off level, calculated using receiver operating characteristic curve analysis, to predict the primary end point and the occurrence of cardiovascular events was compared between the groups. The primary end point was all-cause mortality and hospitalization because of heart failure. The receiver operating characteristic curve analysis for the composite primary end point revealed an LVEDVI cut-off point of 118ml/m2. Based on this threshold, 61 patients (49.6%) were categorized into the LVEDVI <118 ml/m2 group, whereas 62 (50.4%) fell into the LVEDVI ≥118 ml/m2 group. Over a median follow-up period of 336days (interquartile range 80 to 667), the primary end points occurred in 15 and 26 patients in the LVEDVI <118 and LVEDVI ≥118 ml/m2 groups, corresponding to incidence rates of 24.6% and 41.9%, respectively. Patients in the LVEDVI ≥118ml/m2 group demonstrated a significantly higher risk of adverse clinical events than those in the LVEDVI <118ml/m2 group (hazard ratio 2.24, 95% confidence interval 1.17 to 4.28, p=0.01). This trend persisted even after adjusting for several confounders (p=0.02). In conclusion, increased LVEDVI values were associated with increased adverse clinical events after MitraClip implantation in patients with severe mitral valve regurgitation.

Urgent Transcatheter Edge-to-Edge Repair (TEER) of severe mitral regurgitation in patients with cardiogenic shock: A systematic review and meta-analysis of 5,428 patients

Abstract Background Patients presenting with severe mitral valve regurgitation (MR) and cardiogenic shock (CS) demonstrate a poor prognosis and high mortality rate. Transcatheter edge-to-edge repair (TEER) of the mitral valve regurgitation with MitraClip may alter patient management while alleviating hemodynamic instability. Aim To perform a systematic review of the published literature and meta-analysis on the use of MitraClip in patients with severe MR and CS. Methods We performed a systematic review and meta-analysis according to the PRISMA guidelines. We systematically explored published literature in MEDLINE (Pubmed) before January 8, 2023, for studies assessing urgent TEER with MitraClip in patients presenting with severe MR and CS. CS was defined as 1) CS according to ICD-10; 2) inotrope use; or 3) mechanical circulatory support before TEER. The primary outcome was device success and all-cause mortality rates while the secondary outcome was the incidence of myocardial infarction (MI), stroke, and hospitalization for heart failure (HF), during in-hospital/30-days and long-term periods. We calculated I² to test heterogeneity. We used random-effects modeling for the meta-analyses to assess the primary and secondary outcomes. Results In total 25 studies, including 5,428 patients met the predefined eligibility criteria and were included in this meta-analysis. During the in-hospital/30-day period, device success was achieved in 86% (95% CI: 80%-91%, I2 =49%) with MR ≤2+ to be described in 87% (95% CI: 79%-93%, I2 =75.8%) of the cases, while the overall all-cause mortality was 14% (95% CI: 10%-19%, I2 =84%). The incidence of MI and stroke hovered at 4% (95% CI: 1%-13%, I2 =92%) and 2% (95% CI: 1%-2%, I2 =0%), respectively. During a mean follow-up of 10.6 ± 6.4 months, the all-cause mortality was increased to 31% (95% CI: 24%-39%, I2 =92%), while hospitalization for HF is needed in 18% (95% CI: 9%-29%, I2 =80%) of the patients. In the subgroup analysis of patients in CS due to acute MI who underwent urgent TEER, 11 studies and 352 patients were included. Device success was achieved in 92% (95% CI: 75%-100%, I2 =77.8%) and the in-hospital/30-day mortality was 13% (95% CI: 5%-21%, I2 =60%). In a mean follow-up of 10.2 ± 8.3 months, long-term all-cause mortality fluctuated at the same rate of around 11% (95% CI: 2%-23%, I2 =70%). Conclusion The use of MitraClip in patients with MR and concurrent CS is associated with improved in-hospital/30-day and long-term survival. Our findings indicate that using MitraClip in the acute phase is a safe approach that can significantly reduce all-cause mortality, MI, stroke, and re-hospitalization for HF in this patient population.

Clinical genetic testing in arrhythmogenic mitral valve prolapse syndrome highlights a potential overlap with familial dilated cardiomyopathy

Abstract Background/Introduction Arrhythmogenic mitral valve prolapse syndrome (AMVPS) is a female-predominant sudden cardiac death (SCD)-predisposing disorder characterized clinically by myxomatous mitral valve prolapse (MVP), inferolateral T-wave inversions and complex ventricular ectopy. However, insights into the pathogenesis and potential genetic basis of AMVPS are currently lacking. Purpose Considering recent case reports that suggest a potential role for genetic cardiomyopathies in the pathogenesis of AMVPS, we sought to determine the yield of pathogenic/likely pathogenic (P/LP) genetic variants in strong evidence cardiomyopathy-susceptibility genes amongst AMVPS patients who underwent clinical genetic testing. Methods In this retrospective study, the medical records of 4,907 patients referred to our cardiovascular genetics clinic was used to identify those patients with a clinical phenotype consistent with AMVPS. After exclusion of individuals with severe mitral valve regurgitation, pertinent clinical, electrocardiographic, imaging and genetic testing data was extracted from the electronic record. For those AMVPS that underwent a form of genetic testing that encompassed 23 Clinical Genome Resource-defined strong/definitive evidence cardiomyopathy-susceptibility genes, ultra-rare (minor allele frequency ≤ 0.00005 in the Genome Aggregation Database [n=141,456 individuals]) non-synonymous variants identified within these genes were adjudicated independently as P, LP or variant of uncertain significance with the assistance of Varsome. The frequency of P/LP variants was then compared to a previously published cohort of 973 ostensibly healthy control exomes. Results Overall, 56 unrelated patients with suspected AMVPS were identified (82% female; average age at presentation of 31 ± 13 years; 32% with a family history of MVP and/or SCD). Of these, 24/56 (43%) underwent a form of clinical genetic testing that encompassed the pre-defined panel of cardiomyopathy-susceptibility genes. In comparison to control exomes (n=973), P/LP variants in strong/definitive evidence cardiomyopathy-susceptibility genes were over-represented in AMVPS patients [4/24 (16.7%) vs 20/973 (2.1%); p=0.002]. Interestingly, this enrichment was driven in large part by an excess number of A-band-localizing P/LP truncating variants in TTN-encoded titin in AMVPS cases [3/24 (12.5%) versus 4/973 (0.4%); p=0.0004]. Conclusion This study provides further evidence that a subset of AMVPS may arise secondary to a "two-hit" model whereby an underlying cardiomyopathic genetic substrate is exacerbated by the mechanical force generated by genetically unrelated myxomatous MVP resulting in maladaptive and ventricular arrhythmia-predisposing fibrosis within the inferobasal left ventricle and posteromedial papillary muscles. Larger studies are needed to better understand the potential role of genetic testing in the diagnosis, risk-stratification and management of patients with suspected AMVPS.

Acute changes of right ventricular longitudinal strain after transcatheter edge-to-edge repair in patients with functional mitral regurgitation

Abstract Background Transcatheter edge-to-edge repair (TEER) is a valid therapeutic option in symptomatic patients with severe secondary mitral valve regurgitation (SMR) at high surgical risk. While right ventricular (RV) dysfunction is a predictor of poor outcomes in this context, TEER may lead to an improvement of RV function which portends a better outcome. Purpose The aim of this study was to investigate acute change in RV function and its prognostic implication after successful TEER in patients with severe SMR. Methods Patients with severe SMR who underwent TEER from 2013 to 2021 at our center were retrospectively screened. Only patients with successful procedure (MR ≤ grade 2) and adequate echocardiographic images for longitudinal strain assessment were enrolled. RV function was estimated at baseline and pre-discharge by echocardiographic evaluation; RV global longitudinal strain (RVGLS) and RV free wall longitudinal strain (RVFWLS) were assessed before and after TEER. Longitudinal strain improvement was defined as an increase of post procedural strain greater than median value variation. Population was then divided in two groups based on RVGLS significant improvement. The primary outcome was a composite of all-cause death, heart transplantation (HT) and left ventricular assist device (LVAD) implantation during follow-up, assessed with Kaplan-Maier curves and compared by log-rank test. Results A total of 61 patients were included. Mean age was 70±10 years, 89% patients were symptomatic with NYHA class 3-4, mean furosemide daily dose was 200±146 mg. Pre-TEER median left ventricle ejection fraction (LVEF) was 29% [IQR, 26; 35%], RV fractional area change (FAC) was 32% [IQR 26; 37%] and tricuspid anulus plan systolic excursion (TAPSE) was 17 mm [IQR 14; 20 mm]. Median RVGLS and RVFWLS at baseline were respectively -11% [IQR -13; -9%] and -14% [IQR -17; -11%]. After TEER a significant variation of RVGLS and RVFWLS was observed (RVGLS: -13% [IQR -15; -10%] p 0.012; RVFWLS: -16% [-19; - 13%] p 0.001). Moreover, a significant reduction in estimated systolic pulmonary artery pressure (sPAP) between baseline (45 mmHg [IQR 35; 55mmHg]) and post TEER (40 mmHg [IQR 30;45 mmHg] p &amp;lt;0.001) was observed. Median improvement of RVGLS was 1.3%. After mean follow up time of 55±7 months event rate for the combined primary endpoint was numerically lower in the improved RVGLS group, although the difference was not statistically significant (p 0.243). Interestingly, rate of HT was significantly lower in RVGLS-improved group (p 0.032). Conclusions This study showed significant pre-discharge improvement of RV longitudinal strain in patient with severe SMR after successful TEER. At follow-up this improvement did not affect the outcome in terms of freedom from composite endpoint of all-cause death, HT or LVAD implantation but events were numerically lower in patients with strain improvement. Further data on larger population are needed to confirm our observations.Table 1,2Figure 1,2

Incidence and predictors of the novo significant mitral and tricuspid regurgitation in patients with permanent atrial fibrillation

Abstract Introduction Atrial fibrillation (AF) is related to atrial remodeling and annular enlargement, which leads to functional mitral and tricuspid regurgitation. These two entities can derive in heart failure with preserved left ventricular ejection fraction (LVEF), the reason why it is becoming a rising concern in recent years. However, its incidence and predictors have not been well established. Purpose This study aimed to assess the incidence and predictors of significant atrial functional mitral and tricuspid regurgitation in patients with permanent AF. Methods The study population comprised 547 patients from a Spanish health area diagnosed with permanent AF between 2014 and 2019 (CardioCHUVI-AF registry; ClinicalTrials.gov identifier: NCT04364516). Individuals who presented with atrial enlargement at the diagnosis, primary moderate to severe valve disease, previous cardiac surgery (including valve replacement or repair), decreased LVEF, pulmonary hypertension, or right ventricular pacing that could interfere with tricuspid valve closure were excluded. The median age was 78.0 (IQ 70.4-85.6), and men represented 43% of the population (235 patients) compared to women, which represented 57% (312 patients). During a median follow-up of 3.5 years (IQ 2.03-4.95) we assessed the incidence of moderate to severe de novo mitral and tricuspid valve regurgitation and its predictors. Results The development of moderate to severe mitral and tricuspid valve regurgitation was higher after three years of diagnosis. The incidence of mitral regurgitation was 3.8 (3.0-4.9) per 100 persons/year. The incidence of tricuspid regurgitation was 5.5 (4.5-6.8) per 100 persons/year. In addition, multivariate analysis showed an association between the development of mitral regurgitation and older age (HR 1.05 [CI 95% 1.01-1.09]), female sex (HR 1.82 [CI 95% 1.1-3]) and prior admission for HF (HR 2.35 [CI 95% 1.18-4.7]). On the other hand, the development of tricuspid regurgitation did not show an association with age (HR 1.02 [CI 95% 0.97-1.05]), but it did with prior admission for HF (HR 2.11 [CI 95% 1.17-3.79]) and female sex (HR 2.17 [CI 95% 1.43-3.3]). Conclusion Permanent atrial fibrillation was associated with an increased risk of developing atrial functional mitral and tricuspid regurgitation, especially after three years of the diagnosis and in the female sex. Older age was associated with a higher incidence of mitral regurgitation. Prior admission for HF was associated with a higher incidence of tricuspid regurgitation. Based on the preceding, monitoring a specific profile of patients with atrial fibrillation may be especially important in preventing and early treating valve disease that can compromise their prognosis.Incidence of mitral regurgitationIncidence of tricuspid regurgitation

Abstract 17855: Improvement of Mitral Valve Regurgitation Severity Following Catheter Ablation for Atrial Fibrillation

Background: The presence of concomitant mitral valve regurgitation (MR) in patients with atrial fibrillation (AF) is well known, yet the degree of MR improvement after catheter ablation is not well studied. We investigated the effect of catheter ablation on the degree of MR in patients with baseline ≥moderate MR and AF. Methods: We retrospectively reviewed charts of AF catheter ablation patients at Montefiore Medical Center (2018-2019). Patients with prior or subsequent mitral valve surgeries were excluded. Degree of MR, assessed through echocardiograms, was compared at baseline and follow-up. Early follow-up was defined as 6 months post-ablation. Patients were stratified by left atrial size and left ventricular ejection fraction (LVEF) to assess the influence of these parameters on MR severity after ablation. Results: A total of 202 patients were included in our analysis with an average age of 64 years. At baseline there were 149 patients (74%) with &lt;moderate MR and 44 patients with ≥moderate MR (22%). There was a statistically significant reduction in the number of patients with ≥moderate MR following ablation both at early and late follow-up (p&amp;lt;0.0001, Figure 1). Our results remained statistically significant after stratification by LVEF (&lt;40% vs ≥ 40%) and LA dimension (&lt;4.5cm vs ≥ 4.5cm) (p&amp;lt;0.001, Figure 2). Conclusions: Catheter ablation of AF improved the degree of MR severity in patients with baseline ≥moderate MR and AF. Our findings were preserved across stratification by left atrial size and LVEF subgroups. Further investigation is necessary to validate our findings.

Abstract 15479: High Output Cardiac Failure Associated With Arteriovenous Fistula

Introduction: High output heart failure (HOHF) secondary to arteriovenous fistula (AVF) is known. However, having a high index of suspicion for the latter when encountering diuretic resistance in patients with a remote history of hemodialysis is key. Case A 68-year-old female with a history of kidney transplant in 2012 with no recent dialysis, hypertension, hyperlipidemia, pulmonary hypertension, heart failure with reduced ejection fraction, and atrial fibrillation presented with decompensated heart failure and acute kidney injury (AKI). Physical exam was remarkable for anasarca, and no apparent fistulas were noted. She was started on milrinone and furosemide drips. Clinical decision making Initial transthoracic echocardiogram (TTE) revealed an ejection fraction (EF) of 20%, which declined from 35%, along with new mitral and tricuspid regurgitation. Her hospitalization was complicated by altered mental status, worsening AKI requiring hemodialysis, atrial fibrillation with rapid ventricular response, and resistance to multiple diuretics and after-load reduction combinations. Diuretics and inotropic support were discontinued due to worsening clinical status. Given concerns for valvular disease, a transesophageal echocardiogram was obtained, confirming moderate to severe mitral and tricuspid valve regurgitation, bi-atrial enlargement, and reduced biventricular function. However, the patient was not clinically optimized for Mitra-clip. Further hemodynamics and volume status assessment with RHC revealed a mean PAP 44, PCWP 26, and CO 11 L/min. RHC results raised suspicion for HOHF. Interestingly, the patient was noted to have a right inguinal surgical scar with a palpable thrill, and an arteriovenous graft duplex revealed high volume flow. The patient underwent ligation of the right inguinal fistula. Repeat TTE two weeks post AVF ligation showed EF of 40% and improved functional mitral and tricuspid valve regurgitation. The patient ultimately was taken off hemodialysis and had clinically improved heart failure. GDMT was optimized, and outpatient evaluation for mitral valve intervention was scheduled. Conclusion Longstanding AVF can cause HOHF. Early index of suspicion is warranted for AVF ligation as it results in rapid EF recovery.