scholarly journals Mitral annular disjunction in patients with syndromic hereditary aorthopaties

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
M L Servato ◽  
A Lopez-Sainz ◽  
F Valente ◽  
R Fernandez-Galera ◽  
G Casas-Masnou ◽  
...  

Abstract Background Mitral annular disjunction (MAD) is a structural abnormality of the mitral annular fibrosus characterized by a separation between the atrial wall-mitral valve junction, and the left ventricular attachment (1). It has been associated with mitral valve prolapse (MVP) (2) but also, with arrhythmias and sudden cardiac death (SCD) (3). There is no evidence of its prevalence and clinical significance in patients with syndromic hereditary aortopathies. Purpose To evaluate the prevalence of MAD, PMV, and the combination of both in patients with syndromic hereditary thoracic aortic disease (HTAD) including Marfan (MFS), Loeys-Dietz (LDS) and vascular Ehlers-Danlos syndromes (vEDS), and its relationship with arrhythmias, SCD, mitral regurgitation (MR) severity and the need for mitral surgery at the follow-up. Methods Adult patients with syndromic HTAD seen at our specialized unit were retrospectively included. The presence of MAD, MVP, and significant MR at first echocardiogram were evaluated. Electronic medical records were reviewed to register the occurrence of arrhythmic events and the need of mitral surgery. Last echocardiogram available was also assessed to evaluate MR progression. Results A total of 295 patients were included (235 MFS, 42 LDS and 18 vEDS). Mean age at baseline was 39.0±14.4 and 52.9% were female. MAD was present in 87 (37.0%) of MFS, 6 (14.3%) of LDS and was not present in vEDS (p<0.001). MVP was found in 105 (44.7%) of MFS, 6 (14.3%) of LDS and 0 in vEDS (p<0.001). In MFS, the presence of MAD was significantly associated with MVP (p≤0.001) (Table 1). However, 14 (6.0%) of patients had isolated MAD (Table 2). At baseline, significant MR was observed in 18 (24.7%) of patients with concurrent MAD and MVP and was not present in patients with isolated MAD (Table 2). MVP (OR 16.85 IC 4.43 – 64.07) but not MAD (p=0.607), was associated with significant MR in the multivariate analysis. A second echocardiogram was available in 220 patients at ≥1 year (mean 4.1±1.4 years). Overall, 25 (11.4%) presented significant progression of MR, 0 in the isolated MAD group, 13 (19.4%) in the MAD/MVP group and 6 (20.0%) of the isolated MVP (p=0.007). After a mean clinical follow-up of 7.5±3.2 years, 10 patients required mitral surgery (6 prosthesis, 4 valvuloplasty), 22 (9.4%) presented atrial fibrillation, flutter or supraventricular tachycardia (SVT), and 2 (0.9%) SCD. After adjustment for the presence of MVP and time of follow-up, MAD was not associated with progression of MR (p=0.529) need for mitral surgery (p=0.096), atrial fibrillation-flutter or SVT (p=0.510) nor SCD. (p=0.997). Conclusions The prevalence of MAD in syndromic HTAD is high, especially in Marfan syndrome, and absent in vEDS. In this retrospective observational study, the presence of MAD in Marfan was not associated with mitral regurgitation evolution or arrhythmic events. FUNDunding Acknowledgement Type of funding sources: None. Characteristics of MFS patients Presence of significant MR by groups.

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Benito Gonzalez ◽  
X Freixa ◽  
C Godino ◽  
M Taramasso ◽  
R Estevez-Loureiro ◽  
...  

Abstract Background Limited information has been reported regarding the impact of percutaneous mitral valve repair (PMVR) on ventricular arrhythmic (VA) burden. The aim of this study was to address the incidence of VA and appropriate antitachycardia implantable cardiac defibrillator (ICD) therapies before and after PMVR. Methods We retrospectively analyzed all consecutive patients with heart failure with reduce left ventricular ejection fraction, functional mitral regurgitation grade 3+ or 4+ and an active ICD or cardiac resynchronizer who underwent PMVR in any of the eleven recruiting centers. Only patients with complete available device VA monitoring from one-year before to one year after PMVR were included. Baseline clinical and echocardiographic characteristics were collected before PMVR and at 12-months follow-up. Results 93 patients (68.2±10.9 years old, male 88.2%) were enrolled. PMVR was successfully performed in all patients and device success at discharge was 91.4%. At 12-months follow-up, we observed a significant reduction in mitral regurgitation severity, NT-proBNP and prevalence of severe pulmonary hypertension and severe kidney disease. Patients also referred a significant improvement in NYHA functional class and showed a non-significant trend to reserve left ventricular remodeling. After PMVR a significant decrease in the incidence of non-sustained ventricular tachycardia (VT) (5.0–17.8 vs 2.7–13.5, p=0.002), sustained VT or ventricular fibrillation (0.9–2.5 vs 0.5–2.9, p=0.012) and ICD antitachycardia therapies (2.5–12.0 vs 0.9–5.0, p=0.033) were observed. Conclusion PMVR was related to a reduction in arrhythmic burden and ICD therapies in our cohort. Proportion of patients who presented ven Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
AM Caggegi ◽  
P Capranzano ◽  
S Scandura ◽  
S Mangiafico ◽  
G Castania ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background – Although percutaneous mitral valve repair is an attractive alternative treatment option for patients with severe mitral regurgitation (MR) at high surgical risk, residual MR is commonly observed after the procedure and little is known about its impact on outcomes after MitraClip therapy, expecially in patients with severe left ventricular (LV) impairment. Purpose – The aim of this prospective, observational study was to evaluate the impact of residual MR (MR ≤1+ vs. MR >1+) on long-term outcomes of mitral valve repair with the MitraClip System in high surgical risk patients presenting with moderate-to-severe or severe MR and with severe reduction of LV ejection fraction (EF). Methods – Patients enrolled in the prospective Getting Reduction of Mitral Insufficiency by Percutaneous Clip Implantation (GRASP) with functional MR and EF ≤30% who were eligible at almost five-year follow-up were included in the present analysis.  The primary endpoint was death at 5-year follow-up.  Also echocardiographic parameters at baseline and 5-year follow-up and rehospitalization rates were assessed. Results – A total of 139 patients were included: 92 (66.2%) with post-procedural residual MR ≤1+ and 47 (33.8%) with residual MR > 1+ (41 patients with residual MR 2+, 5 with residual MR 3+, 1 with residual MR 4+).  Comparable clinical and echocardiographic baseline characteristics were observed between the two groups except for NYHA functional class IV and implanted pace-maker (more frequent in patients with residual MR >1+) and previous myocardial infarction (more frequent  in patients with residual MR ≤1+). At 5-year follow-up, no significant differences were reported in the primary endpoint (49.6% in patients with residual MR ≤ 1+ vs. 65.3% in patients with residual MR > 1+, p 0.203) and in cardiac death (37.8% in patients with residual MR ≤ 1+ vs. 42.6% in patients with residual MR > 1+, p 0.921). Cox regression analysis identified residual MR > 1+ as an independent predictor of re-hospitalization (HR 0.51, 95% CI 0.28-0.92, p =0.026). At 5-year follow-up,  a significant reduction in left ventricular end-systolic volume was  observed in patients with residual MR ≤ 1+. Conclusions – At 5-year follow no significant differences in survival emerged in patients with severe  LV dysfunction undergoing MitraClip therapy regardless residual MR. Nevertheless residual MR > 1+ emerged as an indipendent predictor of re-hospitalization.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
H Horinouchi ◽  
T Nagai ◽  
Y Ohno ◽  
T Murakami ◽  
J Miyamoto ◽  
...  

Abstract Background Transcatheter aortic valve implantation (TAVI) results in an immediate and greater aortic pressure gradient improvement in patients with severe aortic valve stenosis (AS), and induces early left ventricular (LV) mass regression, which may be related to favorable effects on the mid to long term outcomes. However, the extent of LV mass regression after unloading of chronic pressure overload is varying, and its determinants are still unknown. Thus, the study aims to identify echocardiographic determinants of LV mass regression following TAVI. Methods We retrospectively screened all TAVI procedures in symptomatic AS from 2017 to 2019, and selected 74 successful TAVI cases that had serial echocardiographic studies both at the baseline and at the mid-term follow-up (4 to 6 months after the procedure). Through the digitalized medical records, clinical and echocardiographic data as well as angiographic grading (0-3) of post-procedure paravalvular leakage (PVL) were obtained. LV mass was calculated by using Cube formula. Thus, the extent of LV mass regression was defined as the differences of left ventricular mas index (LVMI) between at the baseline and at the follow-up (ΔLVMI). Quantification of the baseline mitral valve regurgitant volume was performed by stroke volume method with pulmonic site measurement on the assumption of no pre-existing intra/extra cardiac shunt. Cases with prior mitral valve replacement were excluded. Results At the post-procedure angiogram, only 3 cases had significant PVL (grade 2≤). At the mid-term follow–up, average LVMI decreased significantly from the baseline (165 ± 38 g/m2vs 140 ± 37 mg/ m2, P < 0.0001) and 57 cases (70%) experienced the reduction of LVMI, although average relative wall thickness (2 × posterior wall thickness/left ventricular diastolic dimension) did not change (0.565 ± 0.135 vs 0.586 ± 0.168, P = 0.314). Among the baseline clinical and echocardiographic variables, the baseline peak A wave velocity, E/A ratio, mitral valve regurgitant volume and LVMI revealed simple correlation with ΔLVMI (γ=-0.298, p = 0.0188;γ=0.251, P = 0.0417;γ=0.354, p = 0.0041;γ=0.375, p < 0.0010; respectively), whereas no correlation was observed in angiographic PVL grade. Stepwise multiple regression analysis demonstrated baseline mitral valve regurgitant volume and LVMI as the determinants of ΔLVMI (β=0.344, p = 0.032; β=0.335 P < 0.0001; respectively). Conclusions Pre-existing mitral regurgitation has an impact on the mid–term left ventricular mass regression following TAVI. In severe AS, mitral regurgitation might be functioning as an afterload adjuster, and thus, produces protective effects on LV structure.


2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
TP Craven ◽  
PG Chew ◽  
M Gorecka ◽  
LAE Brown ◽  
A Das ◽  
...  

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


2021 ◽  
Vol 13 (1) ◽  
pp. 60-64
Author(s):  
J. Blade Hargiss ◽  
Joseph A. Dearani ◽  
Elizabeth H. Stephens ◽  
Nathaniel W. Taggart

Background: Isolated anterior mitral valve clefts (MVC) are rare congenital heart defects, and data are limited regarding the natural history and surgical outcomes for such isolated MVCs. Methods: We conducted a retrospective review of patients with congenital MVC who were evaluated at Mayo Clinic in Rochester, Minnesota between 1993 and 2020. Patients were separated into two cohorts: those who underwent surgical repair of the MVC and those who had not yet undergone repair. Baseline and postoperative clinical and echocardiographic data were analyzed. Results: Fourteen patients were included in the nonsurgical cohort and eight patients in the surgical cohort. Surgical repair was via primary median sternotomy (n = 6) or robot-assisted, minimally invasive (n = 2). All cleft repairs were performed by simple suture closure. Intraoperative evaluation of the clefts did not reveal additional structural factors that could account for the mitral regurgitation (MR). At latest follow-up of the surgical cohort, the median grade of MR was 1 (range 0-1), and median left ventricular ejection fraction was 65% (IQR 59%-67%), both similar to the immediate postoperative result. At latest follow-up, all patients in the nonsurgical cohort were NYHA Class 1, and median MR grade was 1. All patients were asymptomatic (NYHA Class 1). Conclusions: Our findings corroborate prior reports that MVC repair is safe and successful and is followed by a low rate of recurrent mitral valve dysfunction. Durable surgical repair of isolated, congenital MVC can be performed safely in select patients. The decision to intervene should be based on the severity of mitral regurgitation and patient symptoms rather than the presence of the MVC alone.


2016 ◽  
Vol 19 (4) ◽  
pp. 160
Author(s):  
Montaser Elsawy Abd elaziz ◽  
Islam Moheb Ibrahim

<strong>Background:</strong> Mitral valve regurgitation leads to deterioration of left ventricular functions if not treated early. We aimed to study the effect of mitral valve replacement on normalization of ejection fraction, remodeling of left ventricular dimensions, and left atrial reduction in patients with chronic mitral regurgitation.<br /><strong>Methods:</strong> Between December 2012 and August 2014, <br />45 patients with chronic mitral regurgitation underwent isolated mitral valve replacement. None of the patients had any other severe valvular or concomitant disease or severe coronary heart  disease. The patients were evaluated by echocardiography (preoperative, 1-week, and 1-year postoperative). The results were statistically analyzed by paired t test.<br /><strong>Results:</strong> Forty-five patients who underwent mitral valve replacement in our hospital were included in  the study. The group comprised 20 men and 25 women; the mean age was 31.8 ± 6.76 years. The mean  left ventricular ejection fraction was 61.09 ± 7.6 and decreased significantly to 59.04 ± 6.65 and 59.67 ± 6.56, 1-week and 1-year postoperative follow up, respectively. The left atrium showed significant reduction in size (4 ± 0.54 cm) at 1-year postoperative follow up, from (4.51 ± 0.57 cm) one-week postoperative, and from (5.55 ± 0.88 cm) preoperatively. The mean left ventricular end systolic diameter significantly decreased from 4.06 ± 0.65 cm preoperatively to 3.4 ± 0.4 cm, 1-week postoperative (P = .01), and also decreased significantly to 3.45 ± 0.51 cm at 1-year follow up postoperatively, but was higher than that at 1-week follow up. Also, the mean left ventricular end diastolic diameter decreased  significantly during periods of follow up (P &lt; .001).<br /><strong>Conclusion:</strong> Reversal of left ventricular functions and reduction of left-sided chamber dimensions are possible if early mitral valve replacement is considered in chronic mitral regurgitation before worsening of the condition.<br /><br />


2019 ◽  
Vol 40 (27) ◽  
pp. 2206-2214 ◽  
Author(s):  
Annelieke H J Petrus ◽  
Olaf M Dekkers ◽  
Laurens F Tops ◽  
Eva Timmer ◽  
Robert J M Klautz ◽  
...  

Abstract Aims Recurrent mitral regurgitation (MR) has been reported after mitral valve repair for functional MR. However, the impact of recurrent MR on long-term survival remains poorly defined. In the present study, mortality-adjusted recurrent MR rates, the clinical impact of recurrent MR and its determinants were studied in patients after mitral valve repair with revascularization for functional MR in the setting of ischaemic heart disease. Methods and results Long-term clinical and echocardiographic outcome was evaluated in 261 consecutive patients after restrictive mitral annuloplasty and revascularization for moderate to severe functional MR, between 2000 and 2014. The cumulative incidence of recurrent MR ≥ Grade 2, assessed by competing risk analysis, was 9.6 ± 1.8% at 1-year, 20.3 ± 2.5% at 5-year, and 27.6 ± 2.9% at 10-year follow-up. Cumulative survival was 85.8% [95% confidence interval (CI) 81.0–90.0] at 1-year, 67.3% (95% CI 61.1–72.6%) at 5-year, and 46.1% (95% CI 39.4–52.6%) at 10-year follow-up. Age, preoperative New York Heart Association Class III or IV, a history of renal failure, and recurrence of MR expressed as a time-dependent variable [HR 3.28 (1.87–5.75), P < 0.001], were independently associated with an increased mortality risk. Female gender, a history of ST-elevation myocardial infarction, a preoperative QRS duration ≥120 ms, a higher preoperative MR grade, and a higher indexed left ventricular end-systolic volume were independently associated with an increased likelihood of recurrent MR. Conclusion Mitral valve repair for functional ischaemic MR resulted in a low incidence of recurrent MR with favourable clinical outcome up to 10 years after surgery. Presence of recurrent MR at any moment after surgery proved to be independently associated with an increased risk for mortality.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Daisuke Kaneyuki ◽  
Hiroyuki Nakajima ◽  
Toshihisa Asakura ◽  
Akihiro Yoshitake ◽  
Chiho Tokunaga ◽  
...  

Abstract Background Annular dilation by left atrial remodeling is considered the main cause of atrial function mitral regurgitation. Although acceptable outcomes have been obtained using mitral ring annuloplasty alone for atrial functional mitral regurgitation, data assessing outcomes of this procedure are limited. Therefore, we aimed to assess midterm outcomes of mitral valve repair in patients with atrial functional mitral regurgitation. Methods We retrospectively studied 40 patients (mean age: 69 ± 9 years) who had atrial fibrillation that persisted for > 1 year, preserved left ventricular ejection fraction of > 40%, and mitral valve repair for atrial functional mitral regurgitation. The mean clinical follow-up duration was 42 ± 24 months. Results Mitral ring annuloplasty was performed for all patients. Additional repair including anterior mitral leaflet neochordoplasty was performed for 22 patients. Concomitant procedures included maze procedure in 20 patients and tricuspid ring annuloplasty in 31 patients. Follow-up echocardiography showed significant decreases in left atrial dimensions and left ventricular end-diastolic dimensions. Recurrent mitral regurgitation due to ring detachment or leaflet tethering was observed in five patients and was seen more frequently among those with preoperative left ventricular dilatation. Three patients without tricuspid ring annuloplasty or sinus rhythm recovery by maze procedure developed significant tricuspid regurgitation. Five patients who underwent the maze procedure showed sinus rhythm recovery. Rates of freedom from re-admission for heart failure at 1 and 5 years after surgery were 95 and 86%, respectively. Conclusions Mitral valve repair is not sufficient to prevent recurrent atrial functional mitral regurgitation in patients with preoperative left ventricular dilatation. Tricuspid ring annuloplasty may be required for long-term prevention of significant tricuspid regurgitation.


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