Considerations & challenges of mitral valve repair in females: diagnosis, pathology, and intervention.

PURPOSE OF REVIEW: Disparities in mitral valve (MV) repair outcomes exist between men and women. This review highlights sex-specific differences in MV disease aetiology, diagnosis, as well as timing and type of intervention. RECENT FINDINGS: Females present with more complicated disease: anterior or bileaflet prolapse, leaflet dysplasia/thickening, mitral annular calcification, and mixed mitral lesions. The absence of indexed echocardiographic mitral regurgitation (MR) severity parameters contributes to delayed intervention in women, resulting in more severe symptom burden at time of surgery. The sequelae of chronic MR also necessitate concomitant procedures (e.g. tricuspid repair, arrhythmia surgery) at the time of mitral surgery. Complex MV pathology, greater patient acuity, and more complicated procedures collectively pose challenges to successful MV repair and postoperative recovery. As a consequence, women receive disproportionately more MV replacement than men. In-hospital mortality after MV repair is also greater in women than men. Long-term outcomes of MV repair are comparable after risk-adjustment for preoperative status; however, women experience a greater incidence of postoperative heart failure. SUMMARY: To address the inequity in MV repair outcomes between sexes, indexed diagnostic measurements, diligent surveillance of asymptomatic MR, increased recruitment of women in large clinical trials, and mandatory reporting of sex-based subgroup analyses are recommended.

Effects of Mitral Valve Surgery on Ventricular Arrhythmia in Mitral Valve Prolapse Patients: Five-Year Eollow-up.

Aim      To evaluate the effect of mitral valve (MV) repair and replacement on the incidence of ventricular arrhythmias (VA) and to identify risk factors for the persistence of VA in patients with MV prolapse and severe mitral regurgitation (MR) during a mid-term follow-up.Material and methods  A single-site observational, prospective study successively enrolled 30 patients (mean age, 55.2±9.9 years, 60% men) who underwent MV repair or replacement for severe MR due to MV prolapse or chordal avulsion. Transthoracic echocardiography and Holter monitoring were performed in all patients before and annually after surgery. A pathomorphological study of MV fragments excised during surgery was performed.Results During the five-year follow-up period (144 person-years), one case of sudden cardiac death outside a health care facility was recorded. MR severity progressed in three patients after MV repair. The total number of all VAs decreased during the follow-up period, with a significant decrease in the number of paroxysms of unstable ventricular tachycardia during the first two years after surgery. The presence of VA in the postoperative period was correlated with the severity of postoperative left ventricular (LV) remodeling: end-diastolic volume (EDV) (rs=0.69; p=0.005), LV ejection fraction (EF) (rs = -0.55; p=0.004) and severity of MV myxomatous alterations according to histological study data (rτ=0.58; p=0.045). The beta-blocker treatment did not influence the VA frequency and severity (rs= -0.18; p=0.69). According to a univariate regression analysis only EDV (p = 0.001), LVEF <50% (p = 0.003), and myxomatous MV degeneration (p = 0.02) were risk factors for persistent ventricular tachycardia in the postoperative period.Conclusion      Surgical intervention on MV in patients with MV prolapse and severe MR decreased the number of cases of malignant VAs and was correlated with the postoperative changes in LV volume and function, as well as the severity of MV myxomatous alterations.

Acute mitral regurgitation with and without acute heart failure.

Acute severe mitral regurgitation (MR) is rare, but often leads to cardiogenic shock, pulmonary edema, or both. Most common causes of acute severe MR are chordae tendineae (CT) rupture, papillary muscle (PM) rupture, and infective endocarditis (IE). Mild to moderate MR is often seen in patients with acute myocardial infarction (AMI). CT rupture in patients with floppy mitral valve/mitral valve prolapse is the most common etiology of acute severe MR today. In IE, native or prosthetic valve damage can occur (leaflet perforation, ring detachment, other), as well as CT or PM rupture. Since the introduction of percutaneous revascularization in AMI, the incidence of PM rupture has substantially declined. In acute severe MR, the hemodynamic effects of the large regurgitant volume into the left atrium (LA) during left ventricular (LV) systole, and in turn back into the LV during diastole, are profound as the LV and LA have not had time to adapt to this additional volume. A rapid, but comprehensive evaluation of the patient with acute severe MR is essential in order to define the underline cause and apply appropriate management. Echocardiography with Doppler provides vital information related to the underlying pathology. Coronary arteriography should be performed in patients with an AMI to define coronary anatomy and need for revascularization. In acute severe MR, medical therapy should be used to stabilize the patient before intervention (surgery, transcatheter); mechanical support is often required. Diagnostic and therapeutic steps should be individualized, and a multi-disciplinary team approach should be utilized.

How many clues make an evidence? An unusual case of aborted cardiac arrest due to mitral valve prolapse.

There is an increasing awareness on the association between mitral valve prolapse (MVP) and sudden cardiac death. Mitral annular disjunction (MAD) is a phenotypic risk feature that can help in risk stratification. We present a case of a 58-year-old woman who experienced an out-of-hospital cardiac arrest caused by ventricular fibrillation interrupted by a direct current shock. No coronary lesions were documented. Echocardiogram showed myxomatous MVP. Nonsustained ventricular tachycardia have been registered during hospital stay. Interestingly, cardiac magnetic resonance revealed MAD and a late gadolinium enhancement area in inferior wall. Finally, a defibrillator has been implanted. For arrhythmic risk stratification of MVP with MAD, multimodality imaging is the diagnostic tool to find out the disease behind many cardiac arrests of unknown cause.

Can cartilage intermediate layer protein 1 (CILP1) use as a novel biomarker for canine myxomatous mitral valve degeneration levels or not?

BACKGROUND: Myxomatous mitral valve degeneration (MMVD) is the most common degenerative heart disease in dogs and is associated with irreversible changes in the valve tissue. Although traditional cardiac biomarkers are efficient for diagnosing MMVD, there are limitations, therefore, it is important to find novel biomarkers. Cartilage intermediate layer protein 1 (CILP1), an extracellular matrix-derived protein, acts as a transforming growth factor-ß antagonist and is involved in myocardial fibrosis. This study aimed to evaluate serum CILP1 levels in canines with MMVD. Dogs with MMVD were staged according to the American College of Veterinary Internal Medicine consensus guidelines. Data analysis was performed using the Mann-Whitney U test, Spearman's correlation, and receiver operating characteristic (ROC) curves. RESULTS: CILP1 levels were elevated in dogs with MMVD (n = 27) compared to healthy controls (n = 8). Furthermore, results showed that CILP1 levels were significantly higher in stage C group dogs compared to healthy controls. The ROC curve of CILP1 and NT-proBNP were good predictors of MMVD, although no similarity was observed between the two. Left ventricular end-diastolic diameter normalized to the body weight (LVIDdn) and left atrial to aorta dimension (LA/Ao) showed a strong association with CILP1 levels; however, no correlation was observed between CILP1 levels and vertebral heart size (VHS) and vertebral left atrial score (VLAS). The optimal cut-off value was selected from the ROC curve and dogs were classified according to the cut-off value (1.068 ng/mL, sensitivity 51.9%, specificity 100%). Results showed a significant association of CILP1 with cardiac remodeling indicators, such as VHS, VLAS, LA/Ao, and LVIDdn. CONCLUSIONS: CILP1 can be an indicator of cardiac remodeling in canines with MMVD and therefore, can be used as an MMVD biomarker.

Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome.

Importance: Mitral annular disjunction (MAD) has received particular interest in patients with mitral valve prolapse, ventricular tachycardia, and sudden cardiac death. The clinical significance of MAD for patients with Marfan syndrome (MFS) remains largely unexplored. Objective: To define the prevalence of MAD and examine its association with cardiovascular outcomes and arrhythmia among patients with MFS. Design, Setting, and Participants: This retrospective, single-center cohort study included 142 patients with a diagnosis of MFS based on the revised Ghent criteria and a confirmed (likely) pathogenic variant in the FBN1 gene who underwent regular follow-up between January 1, 2004, and December 31, 2019. Main Outcomes and Measures: The presence of MAD was assessed by echocardiography, and the extent of MAD was categorized in tertiles. Patients also underwent resting electrocardiography and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (defined as sustained ventricular tachycardia or sudden cardiac death), and mitral valve surgery. Results: A total of 142 patients (72 female patients [51%]; median age at first examination, 25 years [range, 2-64 years]) were evaluated. Forty-eight patients (34%) had MAD. Patients with MAD had larger aortic root z scores than patients without MAD (4.1 [interquartile range, 2.8-5.7] vs 3.0 [interquartile range, 1.8-4.0]; P < .001) and more often had mitral valve prolapse (34 of 48 [71%] vs 14 of 94 [15%]; P < .001), ventricular ectopy (14 of 33 [42%] vs 15 of 70 [21%]; P = .03), and nonsustained ventricular tachycardia (13 of 33 [39%] vs 12 of 70 [17%]; P = .01). During follow-up, aortic events occurred at similar rates among patients with vs without MAD (15 of 43 [35%] vs 21 of 84 [25%]; P = .24), but patients in the upper MAD tertile (>10 mm) showed a higher occurrence of aortic events compared with patients with MAD of 10 mm or smaller (9 of 15 [60%] vs 6 of 28 [21%]; P = .01). Patients with arrhythmic events (n = 5) and patients requiring mitral valve surgery (n = 7) were observed exclusively in the group displaying MAD. Conclusions and Relevance: This study suggests that MAD among patients with MFS is associated with the occurrence of arrhythmic events, a higher need for mitral valve intervention, and, among patients with extensive MAD, more aortic events. Cardiac imaging for patients with MFS should consider the assessment of MAD as a potential marker for adverse outcomes.

Minimally-invasive mitral valve repair of symmetric and asymmetric Barlow´s disease.

OBJECTIVES: Barlow´s disease represents a wide spectrum of mitral valve pathologies associated with regurgitation (MR), excess leaflet tissue, and prolapse. Repair strategies range from complex repairs with annuloplasty plus neochords through resection to annuloplasty-only. The latter requires symmetric prolapse patterns and central regurgitant jets. We aimed to assess repair success and durability, survival, and intraoperative outcomes with symmetric and asymmetric Barlow's disease. METHODS: Between 09/10 and 03/20, 103 patients (of 1939 with mitral valve surgery) presented with Barlow´s disease. All received surgery through mini-thoracotomy with annuloplasty plus neochords (n = 71) or annuloplasty-only (n = 31). One valve was replaced for endocarditis (repair rate: 99%). RESULTS: Annuloplasty-only patients were older (64 ± 16 vs. 55 ± 11 years, p = 0.008) and presented with higher risk (EuroSCORE II: 4.2 ± 4.9 vs. 1.6 ± 1.7, p = 0.007). Annuloplasty-only patients had shorter cross-clamp times (53 ± 18 min vs. 76 ± 23 min, p < 0.001) and received more tricuspid annuloplasty (15.5% vs. 48.4%, p < 0.001). Operating times were similar (170 ± 41 min vs. 164 ± 35, p = 0.455). In three patients, annuloplasty-only caused intraoperative systolic anterior motion (SAM), which was fully resolved by neochords to the posterior leaflet. There were no conversions to sternotomy or deaths at 30-days. Three patients required reoperation for recurrent MR (at 25 days, 2.8 and 7.8 years). At the latest follow-up, there was no MR in 81.4%, mild in 14.7%, and moderate in 2.9%. Three patients died due to non-cardiac reasons. Surviving patients report the absence of relevant symptoms. CONCLUSIONS: Minimally-invasive Barlow's repair is safe with good durability. Annuloplasty-only may be a simple solution for complex but symmetric pathologies. However, it may carry an increased risk of intraoperative SAM.

Endocarditis infecciosa con hemocultivos negativos en prolapso de la válvula mitral. Presentación de caso / Infective endocarditis with negative blood cultures in mitral valve prolapse. Case presentation

Introducción: La Endocarditis infecciosa sigue desafiando a la Medicina moderna a pesar de no ser una entidad frecuente. Objetivo: Se presenta un caso con una lesión valvular previa no diagnosticada antes, y sin síntomas, y que se consideró el diagnóstico tempranamente de endocarditis en el nivel hospitalario. Presentación del caso: Paciente de 20 años, mujer, con antecedentes de salud referidos, fumadora. Ingresa en sala del Servicio de Medicina el 21 de enero de 2020 por fiebres que se mantienen todo el día de 38-38,50 C, con picos que alcanzan los 400 C con escalofríos en determinados momentos. Desde hace un mes presenta esta sintomatología. Ruidos cardiacos rítmicos, taquicárdicos, de buena intensidad. Clic sistólico con arrastre sistólico fuerte de regurgitación IV/VI audible en foco mitral con frémito que se irradia a la axila, anemia, VSG acelerada, leucocitosis con desviación izquierda, hemocultivos negativos y en ecocardiograma prolapso de válvula mitral, valva anterior y posterior, con regurgitación que ocupa toda la aurícula izquierda hasta el techo de la misma. Múltiples vegetaciones en cara auricular de valva posterior de válvula mitral, la mayor de 7 x 3 mm. Conclusiones: El método clínico es fundamental en el proceso diagnóstico en la práctica clínica secundado por los medios diagnósticos como en la enfermedad que nos ocupa(AU)

Introduction: Infective endocarditis continues to be a great challenge for modern medicine although it is not a frequent entity. Objective: We present a case of an undiagnosed previous valve lesion without symptoms. The early diagnosis of endocarditis was made at the hospital level. Case Presentation: A 20-year-old woman, smoker, with previous history of good health was admitted to the medical ward on January 21, 2020. The patient reported continuous fever (38-38,50 C) throughout the day, with spikes up to 400 C and intermittent chills. She has been having these symptoms for a month. Rhythmic heart sounds and high intensity tachycardia and systolic click with strong systolic displacement of regurgitation grade IV/VI audible in mitral area with fremitus radiating to the armpit were heard. Anemia, accelerated ESR, leukocytosis with left deviation, and negative blood cultures were confirmed. The echocardiogram showed a mitral valve prolapse with regurgitation of anterior and posterior valves that occupies all the left atrium until its top. There was multiple vegetation in the atrial side of the posterior leaflet of the mitral valve; the greatest is 7 x 3 mm. Conclusions: The clinical method is essential in the diagnostic process performed in clinical practice supported by diagnostic means, as in the current case(AU)

Identification of a High-risk Subgroup With Malignant Mitral Valve Prolapse Who Are Predisposed to Sudden Cardiac Death: A Review.

Mitral valve prolapse (MVP) affects approximately 170 million people worldwide; however, phenotypically, there is a wide variety of heterogeneity. In particular subsets, the incidence of sudden cardiac death is calculated to be 998 per 100,000 person-years, which is significantly increased when compared with the general population of MVP patients. Individuals with high-risk features have been identified as young females with bileaflet MVP and electrocardiogram findings of frequent complex ectopy, ST-T wave changes, and inferior T wave inversions. Supplemental imaging modalities in this subgroup demonstrate redundant leaflets and chordae on 2-dimensional transthoracic echocardiography along with varying severity of mitral annular disjunction. Detailed morphologic assessment by 3-dimensional echocardiography provides a quantitative assessment of annular disjunction along with left ventricular longitudinal and basal circumferential strain patterns. Late gadolinium enhancement on cardiac magnetic resonance imaging identifies diffuse and isolated left ventricle fibrosis involving the fascicles and papillary muscles, which has been visualized in isolation during autopsy. Findings of this review propose that sudden cardiac death as a result of malignant arrhythmias arises from automaticity, complex ectopy, and reentry at the level of the fascicles and papillary muscles. The repetitive mechanical stress provides a nidus for the development of both micro- and macrofibrosis easily identified by late gadolinium enhancement on cardiac magnetic resonance imaging. Escalation to electrophysiology studies and early intervention could provide new targeted lifesaving therapies.

Mitral valve dysplasia in eight English Springer Spaniels.

INTRODUCTION: To describe the signalment, physical examination, and echocardiographic findings of a series of English Springer Spaniels (ESSs) diagnosed with congenital mitral valve dysplasia (MD). ANIMALS: Eight client-owned ESSs with congenital MD referred for murmur investigation and/or suspected congestive heart failure (CHF). MATERIALS AND METHODS: Retrospective case series. Medical records and echocardiograms were reviewed to collect relevant data. Echocardiograms were assessed for the following abnormalities consistent with MD: thickened valve leaflets or leaflet tips, a 'hockey stick' appearance to the valve leaflets, abnormal length of one leaflet with respect to the other, and tethering of one or both leaflets to the papillary muscles. RESULTS: All eight dogs showed the typical echocardiographic lesions associated with MD: thickened leaflet tips (5/8), 'hockey stick' appearance (5/8), elongated anterior leaflet (4/8), tethering of one or both leaflets (7/8). Seven of the eight dogs presented in CHF. Six of the eight dogs had left ventricular dilation in both systole and diastole. Two of the eight dogs had reduced systolic function as assessed by ejection fraction/fractional shortening; however end-systolic volume index was increased in 6/8 dogs. Two dogs subsequently developed atrial fibrillation. CONCLUSIONS: Congenital MD should be considered in ESSs with a left-sided apical systolic murmur, particularly in younger dogs. The valve changes seen are similar to those reported in other breeds with MD (thickened leaflet tips, hockey stick appearance to open leaflet tips, abnormal leaflet tethering, abnormally shaped leaflets) and may result in marked remodeling and CHF.

Features of degenerative mitral valve prolapse in the North East of China: repair characteristics, and short-term follow-up results.

BACKGROUND: We aimed to assess the clinical and echocardiographic characteristics of patients who underwent surgery for degenerative mitral valve prolapse (MVP) in our center, and its relation to outcomes. METHODS: We enrolled 117 consecutive patients from North-East China with an echocardiographic diagnosis of MVP related mitral regurgitation (MR) between April 2018 and November 2019. A complexity scoring system was used for valve anatomy, and patients were re-evaluated at 3-6 months after surgery. RESULTS: Most patients (57.3%) were 40-59 years old. Ejection fraction was <60% in one third, and pulmonary hypertension was present in 64.3% of operated patients. Etiology was myxomatous in 58.9%, with flail as main lesion. Leaflet involvement was posterior in 59.8% patients, anterior in 32.5%, bileaflet in 6%, and commissural in 25.6%. Lesion score was intermediate in >50% of patients, and myxomatous lesions scored higher compared to fibroelastic deficiency (FED). Degree of MR left atrial volume and estimated wedge pressure were significantly higher in intermediate and complex lesions. Repair was performed in 93/101 patients (95.8% success rate). No in-hospital major adverse events, nor deaths at follow-up were reported. Residual MR was ≤ mild in 86.7% of patients at follow-up and was associated with FED etiology and complex lesions. CONCLUSIONS: Compared to Western countries, in our sample of Chinese population degenerative severe MR occurred in younger patients. The MVP lesion characteristics are similar, can be accurately detected by non-invasive preoperative evaluation, allowing predictable results. Advanced tailored repair techniques allow excellent immediate and short-term results regardless of the underlying complexity.

Malignant mitral valve prolapse: an uncommon variation of a common condition.

Mitral valve prolapse affects up to 3% of the population, with an annual risk of death of up to 2% a year. Presentation is often in the third to fourth decade of life. This report details an adolescent with mitral valve prolapse with non-specific symptoms but investigative findings of ventricular ectopy at higher heart rates. These patients warrant close surveillance to monitor for progressive arrhythmias and may progress to need an ICD.

Hybrid Positron Emission Tomography/Magnetic Resonance Imaging in Arrhythmic Mitral Valve Prolapse.

Importance: Myocardial replacement fibrosis has been reported to occur in one-third of patients with mitral valve prolapse (MVP) and significant mitral regurgitation (MR). However, it remains unknown whether there are detectable changes in myocardial metabolism suggestive of inflammation or ischemia that accompany the development of fibrosis. Objectives: To characterize the burden and distribution of fluorine 18-labeled (18F) fluorodeoxyglucose (FDG) uptake and late gadolinium enhancement (LGE) in patients with degenerative MVP and ventricular ectopy. Design, Setting, and Participants: Prospective observational study of 20 patients with MVP and significant primary degenerative MR who were referred for mitral valve repair and underwent hybrid positron emission tomography/magnetic resonance imaging (PET/MRI). Ventricular arrhythmias were categorized as either complex (n = 12) or minor (n = 8). Coregistered hybrid 18F FDG-PET and MRI LGE images were assessed and categorized. Recruitment occurred in the new patient clinic of a mitral valve repair reference center. This study was conducted from January 11, 2018, to June 26, 2019. Exposures: Simultaneous cardiac 18F FDG-PET and MRI with LGE imaging on a hybrid PET/MRI system and ambulatory rhythm monitoring. Main Outcomes and Measures: Patients were categorized by the presence and pattern of FDG uptake and LGE, the severity of ventricular arrhythmias, and the indication for mitral valve surgery. Results: In the cohort of 20 patients, the median age was 59.5 years (interquartile range, 52.5-63.2 years). Focal, or focal-on-diffuse uptake, of 18F-FDG (PET positive) was detected in 17 of 20 patients (85%). The FDG uptake coexisted with areas of LGE (PET/MRI positive) in 14 patients (70%). Of the 5 asymptomatic patients with normal ventricular indices and absence of any surgical indications, all were PET/MRI positive. Conclusions and Relevance: In this pilot study, we demonstrate a novel association between degenerative MVP and FDG uptake, a surrogate for myocardial inflammation and/or ischemia. Such evidence of myocardial injury, even in asymptomatic patients, suggests an ongoing subclinical disease process. These findings warrant further investigation into whether imaging for myocardial inflammation, ischemia, and scar has a role in arrhythmic risk stratification and whether it provides incremental prognostic value in patients with chronic severe mitral regurgitation undergoing active surveillance.

Association Between Malignant Mitral Valve Prolapse and Sudden Cardiac Death: A Review.

Importance: Malignant arrhythmic mitral valve prolapse (MVP) phenotype poses a substantial risk of sudden cardiac death (SCD), and an estimated 26 000 individuals in the United States are at risk of SCD per year. Thus, identifying risk-stratification strategies for SCD is imperative. Observations: Patients with MVP have a heterogenous clinical spectrum, ranging from a benign course to a devastating complication such as SCD. Some of the high-risk markers of MVP, which are identified electrocardiographically, include inverted or biphasic T waves, QT dispersion, QT prolongation, and premature ventricular contractions originating from the left ventricular outflow tract and papillary muscles. Morphofunctional characteristics of SCD are leaflet thickness of 5 mm or greater, mitral annulus disjunction, paradoxical systolic increase of the mitral annulus diameter, increased tissue Doppler velocity of the mitral annulus, and higher mechanical dispersion on echocardiography and fibrosis identified by late gadolinium enhancement on cardiac magnetic resonance imaging. Conclusions and Relevance: Findings from this review suggest that SCD can occur earlier in the course of MVP from complex arrhythmias that are triggered by the repeated tugging and traction of the chordopapillary muscle unit and basal mid-myocardium, even before macrofibrosis can be identified in these regions by late gadolinium enhancement on cardiac magnetic resonance imaging. Some of the newer markers identified by speckle-tracking Doppler, such as mechanical dispersion, myocardial work index, and postsystolic shortening, need further validation in a larger population.

Presentation and Outcome of Arrhythmic Mitral Valve Prolapse.

BACKGROUND: Mitral valve prolapse (MVP) is often considered benign but recent suggestion of an arrhythmic MVP (AMVP) form remains incompletely defined and uncertain. OBJECTIVES: This study determined ventricular arrhythmia prevalence, severity, phenotypical context, and independent impact on outcome in patients with MVP. METHODS: A cohort of 595 (age 65 ± 16 years; 278 women) consecutive patients with MVP and comprehensive clinical, arrhythmia (24-h Holter monitoring) and Doppler-echocardiographic characterization, was identified. Long-term outcomes were analyzed. RESULTS: Ventricular arrhythmia was frequent (43% with at least ventricular ectopy ≥5%), most often moderate (ventricular tachycardia [VT]; 120 to 179 beats/min) in 27%, and rarely severe (VT ≥180 beats/min) in 9%. Presence of ventricular arrhythmia was associated with male sex, bileaflet prolapse, marked leaflet redundancy, mitral annulus disjunction (MAD), a larger left atrium and left ventricular end-systolic diameter, and T-wave inversion/ST-segment depression (all p ≤ 0.001). Severe ventricular arrhythmia was independently associated with presence of MAD, leaflet redundancy, and T-wave inversion/ST-segment depression (all p < 0.0001) but not with mitral regurgitation severity or ejection fraction. Overall mortality after arrhythmia diagnosis (8 years; 13 ± 2%) was strongly associated with arrhythmia severity (8 years; 10 ± 2% for no/trivial, 15 ± 3% for mild and/or moderate, and 24 ± 7% for severe arrhythmia; p = 0.02). Excess mortality was substantial for severe arrhythmia (univariate hazard ratio [HR]: 2.70; 95% confidence interval [CI]: 1.27 to 5.77; p = 0.01 vs. no/trivial arrhythmia), even after it was comprehensively adjusted, including for MVP characteristics (adjusted HR: 2.94; 95% CI: 1.36 to 6.36; p = 0.006) and by time-dependent analysis (adjusted HR: 3.25; 95% CI: 1.56 to 6.78; p = 0.002). Severe arrhythmia was also associated with higher rates of mortality, defibrillator implantation, VT ablation (adjusted HR: 4.68; 95% CI: 2.45 to 8.92; p < 0.0001), particularly under medical management (adjusted HR: 5.80; 95% CI: 2.75 to 12.23; p < 0.0001), and weakly post-mitral surgery (adjusted HR: 3.69; 95% CI: 0.93 to 14.74; p = 0.06). CONCLUSIONS: In this large cohort of patients with MVP, ventricular arrhythmia by Holter monitoring was frequent but rarely severe. AMVP was independently associated with phenotype dominated by MAD, marked leaflet redundancy, and repolarization abnormalities. Long-term severe arrhythmia was independently associated with notable excess mortality and reduced event-free survival, particularly under medical management. Therefore, AMVP is a clinical entity strongly associated with outcome and warrants careful risk assessment and well-designed clinical trials.

Mitral Valve Prolapse Patients with Less than Moderate Mitral Regurgitation Exhibit Early Cardiac Chamber Remodeling.

BACKGROUND: Mild physiologic mitral regurgitation (MR) is common in normal individuals. Patients with primary MR due to mitral valve prolapse (MVP) may also exhibit less than moderate MR. We sought to determine whether MVP patients with less than moderate MR displayed early cardiac chamber remodeling or factors related to early remodeling and whether early remodeling predicted MR progression. METHODS: Consecutive MVP patients with less than moderate MR by proximal isovelocity surface area-derived effective regurgitant orifice < 20 mm2 and regurgitant volume < 30 mL, were matched for age and sex with non-MVP patients (controls) having less than moderate MR. Patients with moderate or greater dysfunctional left- or right-sided valves and left ventricular ejection fraction < 50% were excluded. We evaluated left ventricle (LV) and left atrium (LA) remodeling parameters (LV end-diastolic and end-systolic indexed diameters, LA volume-index, and LV mass-index) as well as determinants of remodeling. The last available transthoracic echocardiography was reviewed to identify progression to moderate-severe MR or more. RESULTS: A total of 253 MVP patients with less than moderate MR were matched to 344 controls (P for age and sex, ≥.18) with less than moderate MR. Patients with MVP (mean effective regurgitant orifice and regurgitant volume, 12 ± 4 mm2 and 18 ± 6 mL, respectively) had more premature ventricular contractions (PVCs), larger LV and LA remodeling parameters, and more mild-to-moderate MR (all P < .0001). Multivariate linear regression models showed that larger LV remodeling parameters were independently associated with MVP and female sex but not MR severity (all P < .0001). The LA volume index was independently associated with MVP, age, and E/e' (all P < .0001). The LV mass index was associated with MVP, age, and hypertension (all P ≤ .002). Presence of PVCs was associated with LV end-systolic diameter ≥ 40 mm and indexed ≥ 22 mm2 (P = .005). Among 323 (54%) patients having subsequent transthoracic echocardiography, 17 patients (all MVP) progressed to moderate-severe MR or more at a median of 4.3 (interquartile range, 1.7-6.4) years. Isolated posterior leaflet prolapse was the single factor associated with MR progression (adjusted hazard ratio, 2.70; 95% CI, 0.99-7.34; P = .048) after adjustment for MR severity. At a median of 5.9 (interquartile range, 4.6-7.2) years of follow-up, female sex and MVP (vs controls) were protective factors for mortality. CONCLUSIONS: Patients with less than moderate MR due to MVP exhibit early LV and LA remodeling, which does not predict MR progression or mortality. Left ventricle remodeling is associated with MVP, female sex, and presence of PVCs. Early chamber remodeling associated with MVP may be the phenotypical expression of a genetically mediated process and is at least partially related to PVCs.

The Average Pixel Intensity Method and Outcome of Mitral Regurgitation in Mitral Valve Prolapse.

BACKGROUND: Mitral regurgitation (MR) is a frequent consequence of mitral valve prolapse (MVP). However, the echocardiographic grading of MR is challenging, and the recommended grading parameters have several limitations. The authors developed a novel echocardiographic parameter to grade MR, the average pixel intensity (API) method, on the basis of pixel intensity analysis of the continuous-wave Doppler signal. METHODS: Transthoracic echocardiography was performed prospectively in consecutive patients with MVP (N = 149). MR was quantitatively assessed using the API method, vena contracta width, effective regurgitant orifice area, and regurgitant volume. The primary clinical events were cardiovascular mortality, mitral valve surgery, percutaneous mitral intervention, and heart failure hospitalization. RESULTS: The API method was feasible in 90% of all patients with MVP, which was significantly higher than vena contracta width, effective regurgitant orifice area, and regurgitant volume. During a median follow-up period of 17 months, 44 patients (32%) had major adverse cardiac events, and the majority of events occurred in the holosystolic MVP subgroup. The degree of MR severity by the API method was highly significant for the prediction of events. An API cutoff of 111 arbitrary units was defined as "severe" MR due to MVP, with overall superior sensitivity and specificity compared with cutoffs for established MR grading parameters. In patients who did not have major adverse cardiac events during the follow-up period (n = 92), no significant changes in measures of MR severity were found on follow-up echocardiography. CONCLUSIONS: The API method is predictive of clinical events and outcomes in MR due to MVP. Therefore, the API method may be considered for grading the severity of MR due to MVP in clinical practice.

Use of a photo-realism 3D rendering technique to enhance echocardiographic visualization of the anatomical details during beating-heart mitral valve repair.

The NeoChord procedure is an innovative microinvasive, transventricular, beating-heart chordal replacement technique for patients with severe degenerative mitral valve regurgitation resulting from prolapsed or flail leaflets. Use of three-dimensional (3D) transoesophageal echocardiographic imaging is crucial to the physician operator for device navigation during the procedure and to assess the functional results of the repair at the end of the procedure. Photo-realistic, 3D rendering techniques have been recently developed for medical use. Philips TrueVue (Philips Healthcare, Eindhoven, NL) is a photo-realism technique that employs the use of a virtual light source that simulates the interaction of light on 3-dimensional surfaces. Use of photo-realism techniques, in conjunction with 3D echocardiography, improves the visualization of morphological characteristics of the mitral valve before, during, and after beating-heart mitral valve repair procedures.

Recurrent mitral regurgitation after mitral valve repair for bileaflet lesions in the modern era.

BACKGROUND: Good mid-term durability of mitral valve repair of bileaflet lesions has been reported; however, patients may develop failure during follow-up. This study assessed late outcomes and mechanisms of failure associated with mitral valve repair of bileaflet lesions. METHODS: Fifty-six patients (mean age 67 ± 12 years) underwent mitral valve repair of bileaflet lesions due to degenerative disease in 2011-2018. Mitral annuloplasty was added to all procedures except for 1 patient with annular calcification. Mitral valve lesions were identified by surgical inspection. Mean clinical and echocardiography follow-up occurred at 2.7 ± 2.1 and 2.5 ± 1.9 years, respectively. RESULTS: Additional mitral valve repair techniques involved triangular resection (n = 15 patients), quadrangular resection with sliding plasty (n = 12), neochordoplasty (n = 52), and commissural plication (n = 26). Prolapse of ≥2 anterior and posterior leaflet scallops occurred in 22 (39%) and 30 (54%) patients, respectively. During follow-up, 10 (17.8%) patients developed moderate or severe mitral regurgitation. Whereas prolapse or tethering was observed early after neochordoplasty or quadrangular resection, recurrent regurgitation occurred late after commissural repair. Five-year freedom from recurrent moderate or severe mitral regurgitation rates was 71.1 ± 11.0%. CONCLUSIONS: Seventeen percent of patients developed recurrent mitral regurgitation during follow-up. Repair failure in the early phase occurred owing to aggressive resection of the posterior mitral leaflet or maladjustment of the artificial neochordae. Recurrent mitral regurgitation might occur in the late phase even after acceptable commissural repair. A sequential approach may be useful to improve the quality of mitral valve repair in bileaflet lesions.