Background: The pulmonary vein (PV) flow pattern is influenced by the presence of mitral regurgitation (MR). After a successful reduction in MR severity, the pattern is expected to be changed. We ...aimed to evaluate the prognostic value of a change in the PV flow pattern in patients with primary MR undergoing mitral valve repair (MVR). Methods: The PV flow pattern was assessed with transthoracic echocardiography in 216 patients (age 65 IQR 56–72 years, 70% male) with primary MR before and after surgical MVR. The population was divided according to a change in the PV flow pattern following MVR into ‘improvers’ and ‘non-improvers’. Results: Non-improvers (15%) had a higher prevalence of paroxysmal AF at baseline (46% vs. 22%, p = 0.004), left ventricular dysfunction (LVEF ≤60%) (39% vs. 21%, p = 0.020), and had lower systolic pulmonary artery pressure (28IQR 25–38 vs. 35IQR 26–48 mmHg, p = 0.018) compared to improvers (85%). After a median follow-up of 83IQR 43–140 months, 26(12%) patients died. Non-improvers had higher mortality rates than improvers (p = 0.009). On multivariable Cox regression analysis, a lack of improvement in the PV flow pattern remained independently associated with all-cause mortality (HR 2.322, 95% CI 1.140 to 4.729, P = 0.020). Conclusion: A lack of improvement in the PV flow pattern is independently associated with worse long-term survival in patients with primary MR undergoing MVR.
•The pulmonary vein flow is influenced by the mitral regurgitation severity.•Surgical Mitral valve repair is a treatment for primary mitral regurgitation.•After surgery, the pattern of pulmonary vein flow is expected to be improved.•Un improvement of flow pattern, is associated with worse long-term survival.
ObjectiveMitral valve prolapse (MVP) has been associated with ventricular arrhythmias (VA), but little is known about VA in patients with significant primary mitral regurgitation (MR). Our aim was to ...describe the prevalence of symptomatic VA in patients with MVP (fibro-elastic deficiency or Barlow’s disease) referred for mitral valve (MV) surgery because of moderate-to-severe MR, and to identify clinical, electrocardiographic, standard and advanced echocardiographic parameters associated with VA.Methods610 consecutive patients (64±12 years, 36% female) were included. Symptomatic VA was defined as symptomatic and frequent premature ventricular contractions (PVC, Lown grade ≥2), non-sustained or sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) without ischaemic aetiology.ResultsA total of 67 (11%) patients showed symptomatic VA, of which 3 (4%) had VF, 3 (4%) sustained VT, 27 (40%) non-sustained VT and 34 (51%) frequent PVCs. Patients with VA were significantly younger, more often female and showed T-wave inversions; furthermore, they showed significant MV morphofunctional abnormalities, such as mitral annular disjunction (39% vs 20%, p<0.001), and dilatation (annular diameter 37±5 mm vs 33±6 mm, p<0.001), lower global longitudinal strain (GLS 20.9±3.1% vs 22.0±3.6%, p=0.032) and prolonged mechanical dispersion (45±12 ms vs 38±14 ms, p=0.003) as compared with patients without VA. Female sex, increased MV annular diameter, lower GLS and prolonged mechanical dispersion were identified as independent associates of symptomatic VA.ConclusionIn patients with MVP with moderate-to-severe MR, symptomatic VA are relatively frequent and associated with significant MV annular abnormalities, subtle left ventricular function impairment and heterogeneous contraction. Assessment of these parameters might help decision-making over further diagnostic analyses and improve risk-stratification.
Background
Initial studies have suggested the familial clustering of mitral valve prolapse, but most of them were either community based among unselected individuals or applied non-specific ...diagnostic criteria. Therefore little is known about the familial distribution of mitral regurgitation in a referral-type population with a more severe mitral valve prolapse phenotype. The objective of this study was to evaluate the presence of familial mitral regurgitation in patients undergoing surgery for mitral valve prolapse, differentiating patients with Barlow's disease, Barlow forme fruste and fibro-elastic deficiency.
Methods
A total of 385 patients (62 ± 12 years, 63% men) who underwent surgery for mitral valve prolapse were contacted to assess cardiac family history systematically. Only the documented presence of mitral regurgitation was considered to define ‘familial mitral regurgitation’. In the probands, the aetiology of mitral valve prolapse was defined by surgical observations.
Results
A total of 107 (28%) probands were classified as having Barlow's disease, 85 (22%) as Barlow forme fruste and 193 (50%) patients as fibro-elastic deficiency. In total, 51 patients (13%) reported a clear family history for mitral regurgitation; these patients were significantly younger, more often diagnosed with Barlow's disease and also reported more sudden death in their family as compared with ‘sporadic mitral regurgitation’. In particular, ‘familial mitral regurgitation’ was reported in 28 patients with Barlow's disease (26%), 15 patients (8%) with fibro-elastic deficiency and eight (9%) with Barlow forme fruste (P < 0.001).
Conclusions
In a large cohort of patients operated for mitral valve prolapse, the self-reported prevalence of familial mitral regurgitation was 26% in patients with Barlow's disease and still 8% in patients with fibro-elastic deficiency, highlighting the importance of familial anamnesis and echocardiographic screening in all mitral valve prolapse patients.
Abstract
Aims
Mitral regurgitation (MR) has a significant haemodynamic impact on the left atrium. Assessment of left atrial reservoir strain (LARS) may have important prognostic implications, ...incremental to left atrial (LA) volume, and conventional parameters of left ventricular (LV) structure and function. This study investigated whether preoperative assessment of LARS by speckle tracking echocardiography is associated with long-term outcomes in patients undergoing mitral valve repair for severe primary MR.
Methods and results
Echocardiography was performed prior to mitral valve surgery in 566 patients (age 64 ± 12years, 66% men) with severe primary MR. The study population was subdivided based on a LARS value of 22%, using a spline curve analysis. The primary endpoint was all-cause mortality. During a median follow-up of 7 (4–12) years, 129 (22.8%) patients died. Patients with LARS ≤22% showed significantly higher mortality rates at 1-, 3-, and 5-year follow-up (6%, 12%, and 15%, respectively) when compared with patients with LARS >22% (2%, 3% and 5%, respectively, P < 0.001). Age hazard ratio (HR): 1.06; 95% confidence interval (CI): 1.03–1.09; P < 0.001, LV global longitudinal strain (HR: 0.92; 95% CI: 0.87–0.98; P = 0.014), and LARS (HR: 0.96; 95% CI: 0.93–0.99; P = 0.014) were independently associated with all-cause mortality.
Conclusion
Preoperative LARS is independently associated with all-cause mortality in patients undergoing mitral valve repair for primary MR and provides incremental prognostic value over LA volume. LARS might be helpful to guide timing of mitral valve surgery in patients with severe primary MR.
Graphical Abstract
Graphical Abstract
Assessment of left atrial reservoir strain and long-term survival in patients with severe primary mitral regurgitation undergoing mitral valve repair.
In patients with severe primary mitral regurgitation (MR), the indication for surgery is currently based on the presence of symptoms, left ventricular dilatation and dysfunction, atrial fibrillation, ...and pulmonary hypertension.
The aim of this study was to evaluate the prognostic impact of the presence of extra–mitral valve cardiac involvement (including known risk factors but also severe left atrial LA dilatation and right ventricular RV dysfunction) in a large multicenter study of patients with primary MR.
Patients with severe primary MR undergoing surgery were included and categorized according to the extent (highest) of cardiac involvement: group 0, no cardiac involvement; group 1, left ventricular involvement; group 2, LA involvement; group 3, pulmonary vasculature or tricuspid valve involvement; or group 4, RV involvement. The outcome was all-cause mortality.
A total of 1,106 patients were included (mean age 63 ± 12 years, 68% male). In total, 377 patients (34%) were classified in group 0, 239 (22%) in group 1, 213 (19%) in group 2, 180 (16%) in group 3, and 97 (9%) in group 4. Kaplan-Meier curve analysis revealed significantly worse survival (log-rank chi-square = 43.4; P < 0.001) with higher group. On multivariable analysis, age, male sex, chronic obstructive pulmonary disease, kidney function, and group of cardiac involvement were independently associated with all-cause mortality. For each increase in group, a 17% higher risk for all-cause mortality was observed (95% CI: 1.051-1.313; P = 0.005) during a median follow-up time of 88 months.
In patients with severe primary MR, a novel classification system based on extra–mitral valve cardiac involvement may help refine risk stratification and timing of surgery, particularly including severe LA dilatation and RV dysfunction in the assessment.
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Posterior 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.
In 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.
At 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).
Our 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.
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To explore the presence of left ventricular (LV) and left atrial (LA) morphological and functional abnormalities in patients with Barlow's disease (BD) without significant mitral regurgitation (MR) ...and to investigate whether these abnormalities may predict MR progression.
Consecutive patients with BD were retrospectively identified from two tertiary centers; those with MR graded from trivial to mild-to-moderate were selected and matched with healthy controls in a 1:1 ratio. Conventional and speckle-tracking echocardiographic data were collected. The development of moderate-to-severe or greater MR was evaluated on follow-up echocardiograms.Patients with BD (n=231) showed increased LV dimensions and indexed LV mass (LVMi) in comparison to controls (p<0.001); LV remodeling worsened with higher MR severity and was accompanied by an increased prevalence of eccentric LV hypertrophy (eLVH). Moreover, BD patients had larger LA volumes and more impaired LA reservoir strain versus controls (p<0.001), while LV strain was similar between the two groups. Multivariable linear regression analyses in the overall population identified BD and MR grade as independent predictors of remodeling markers (LV dimensions, LVMi and LA volume), and BD as independent correlate of LA strain.MR progression was observed in 51 BD subjects (out of 170 patients with available follow-up). On Cox regression analysis, age, eLVH, mild-to-moderate MR and mitral annular disjunction (MAD) emerged as independent predictors of MR progression.
BD patients without significant MR show early LV and LA remodeling, together with reduced LA strain. MR progression was associated with eccentric LV remodeling, MAD, and MR severity.
In patients with primary mitral regurgitation (MR), concomitant tricuspid valve (TV) annuloplasty at the time of left-sided valve surgery is indicated in case of a dilated TV annulus ≥40 mm ...independent of the presence or severity of tricuspid regurgitation (TR). However, the long-term impact on right ventricular (RV) adverse remodeling is less well established and the benefit of preventive TV annuloplasty remains controversial. The aim of the study was to assess differences in long-term RV adverse remodeling and the development of significant TR in those patients. In total, 98 patients (mean age 65 ± 11 years, 85% men) with significant primary MR and TV annulus dilatation ≥40 mm without significant TR who underwent mitral valve (MV) repair with or without concomitant TV annuloplasty were included. Of the 98 patients, 28 patients underwent isolated MV repair without TV annuloplasty and 70 patients received concomitant TV annuloplasty at the time of MV surgery. The RV basal diameter (p = 0.03), RV long-axis diameter (p = 0.04), RV end-diastolic area (p <0.01), and RV end-systolic area (p = 0.03) showed less adverse remodeling at follow-up in patients with concomitant TV annuloplasty compared with patients without TV annuloplasty. Additionally, 4 patients (14%) in the subgroup without TV annuloplasty developed significant TR during follow-up in contrast to zero patients in the subgroup with TV annuloplasty (p = 0.001). In conclusion, concomitant preventive TV annuloplasty during MV surgery in patients with primary MR, no significant TR and a tricuspid annulus (≥40 mm) prevented RV adverse remodeling and the development of significant TR at long-term follow-up.
PurposeAlthough a familial distribution has been documented, the genetic aetiology of mitral valve prolapse (MVP) is largely unknown, with only four genes identified so far: FLNA, DCHS1, DZIP1 and ...PLD1. The aim of this study was to evaluate the genetic yield in known causative genes and to identify possible novel genes associated with MVP using a heart gene panel based on exome sequencing.MethodsPatients with MVP were referred for genetic counselling when a positive family history for MVP was reported and/or Barlow’s disease was diagnosed. In total, 101 probands were included to identify potentially pathogenic variants in a set of 522 genes associated with cardiac development and/or diseases.Results97 (96%) probands were classified as Barlow’s disease and 4 (4%) as fibroelastic deficiency. Only one patient (1%) had a likely pathogenic variant in the known causative genes (DCHS1). However, an interesting finding was that 10 probands (11%) had a variant that was classified as likely pathogenic in six different, mostly cardiomyopathy genes: DSP (1×), HCN4 (1×), MYH6 (1×), TMEM67 (1×), TRPS1 (1×) and TTN (5×).ConclusionExome slice sequencing analysis performed in MVP probands reveals a low genetic yield in known causative genes but may expand the cardiac phenotype of other genes. This study suggests for the first time that also genes related to cardiomyopathy may be associated with MVP. This highlights the importance to screen these patients and their family for the presence of arrhythmias and of ‘disproportionate’ LV remodelling as compared with the severity of mitral regurgitation, unravelling a possible coexistent cardiomyopathy.
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