scholarly journals 228 Management and treatment of an hypertrophic cardiomyopathy phenotype

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Leonardo Ciurlanti ◽  
Antonella Accietto ◽  
Alessandro Giovannetti ◽  
Alessio Aloisio ◽  
Nazzareno Galiè ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Hypertrophic Cardiomyopathy ◽  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Genetic Analysis ◽  
Pressure Gradient ◽  
Left Ventricular ◽  
Heart Team ◽  
Clinical Conditions

Abstract Methods and results We are presenting the case of a 75-year-old man who was received in our Cardiomyopathy unit in 1986 with echocardiography showing evidence of left ventricular concentric hypertrophy and a subsequent diagnosis of a sarcomeric hypertrophic cardiomyopathy. During the follow-up, a progressive increase in mid-ventricular pressure gradient was reported and increasing pulmonary arterial pressure, even though the patient was asymptomatic. In 2013, the patient was hospitalized after a resuscitated cardiac arrest due to VF. The coronary angiogram did not show any significant coronary obstructions. The echocardiography showed a systolic anterior motion (SAM) of the mitral valve causing a dynamic pressure gradient in the left ventricular outflow tract reaching 90 mmHg and pulmonary hypertension (PAPs 60 mmHg). A double-chamber ICD was implanted as a secondary prevention of SCD and after a discussion with the Heart-Team, a surgical myectomy with the Morrow technique was performed on the patient. A total of 16 grams of myocardium was removed from the basal interventricular septum, three II order chordae tendineae were dissected from the AML and a mitral valve repair was performed on the patient. The myocardium was reported to show the typical aspects of infiltration; for this reason, a wide genetic analysis was performed which led to the diagnosis of Anderson–Fabry disease (a hemizygous GLA mutation and a homozygous MYBPC3 mutation). Therefore, specific enzymatic therapy was started. The genetic analysis was extended to the patient’s relatives leading to the that the patient’s brother and daughter were both carriers of the mutation. Starting in 2019, the patient began to develop symptoms of cardiac failure (mainly dyspnoea). An echocardiographic investigation showed a moderate to severe aortic regurgitation, a moderate mitral regurgitation, moderate left ventricular dilation, and pulmonary hypertension. The patient’s case was submitted to the Heart-Team’s discussion: due to the patient’s age and clinical conditions, heart transplantation was rejected and medical therapy was decided to be the best option. In 2021 the patient presented with worsening of clinical conditions including dyspnoea during daily activities (NYHA III). An echographic investigation found severe mitral regurgitation with a dilated and hypokinetic LV (EF 30%). The patient was hospitalized for decompensated HF: the coronary angiography did not show CAD and the cardiac catheterization showed low cardiac output without high vascular pulmonary resistances. After the Heart-Team re-evaluation, we decided to perform a percutaneous correction of the mitral regurgitation and in July 2021 Mitraclip implantation was performed on the patient without peri- and post-procedural complications. At the 3-month evaluation the patient was in better clinical conditions with improvement in his functional status (NYHA II). This patient is now continuing outpatient follow-up and we are considering the possibility of a future transcatheter correction of aortic valve regurgitation. Conclusions we submitted this clinical report with the aim to show how, thanks to the development of increasingly advanced diagnostic and therapeutic tools, it is nowadays possible to manage these complex phenotypes and their complications.

scholarly journals Ventricular arrhythmias in patients with functional mitral regurgitation and implantable cardiac devices: implications of mitral valve repair with Mitraclip

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Benito Gonzalez ◽  
X Freixa ◽  
C Godino ◽  
M Taramasso ◽  
R Estevez-Loureiro ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Mitral Valve Repair ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Functional Mitral Regurgitation ◽  
Valve Repair ◽  
One Year ◽  
The Impact

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

scholarly journals Residual mitral regurgitation impact on outcomes after mitraclip therapy: five-year follow-up from the GRASP registry

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
AM Caggegi ◽  
P Capranzano ◽  
S Scandura ◽  
S Mangiafico ◽  
G Castania ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Mitral Valve Repair ◽  
Primary Endpoint ◽  
Left Ventricular ◽  
Valve Repair ◽  
Surgical Risk ◽  
High Surgical Risk ◽  
Mitraclip Therapy

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.

scholarly journals P228 Mitral valve aneurysm in the context of post-infective endocarditis in hypertrophic cardiomyopathy: an issue of inflammation or pressure gradients?

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Pereira Oliveira ◽  
D Seabra ◽  
A Neto ◽  
I Cruz ◽  
G Abreu ◽  
...  
Keyword(s):  
Case Report ◽  
Infective Endocarditis ◽  
Hypertrophic Cardiomyopathy ◽  
Mitral Valve ◽  
Late Complication ◽  
Fatal Outcome ◽  
Left Ventricular ◽  
Pressure Gradients ◽  
Optimal Approach

Abstract Mitral valve aneurysms (MVA) are uncommon and usually develop acutely in the setting of infective endocarditis (IE). We present a case report of a patient with a ruptured aneurysm of the mitral valve (MV) leaflet and obstructive hypertrophic cardiomyopathy (HCM), previously treated for IE. Echocardiography is essential for diagnosis, highlighting the importance of imaging for early identification and timely intervention. CASE REPORT 68-year-old male patient with type 2 diabetes mellitus and dyslipidemia was admitted to hospital with a 3-week history of malaise, fever and recent left-sided abdominal pain. No past relevant history. Physical examination revealed a grade II/VI systolic heart murmur at the cardiac apex, fever, abdominal tenderness in the left upper quadrant and purpuric lesions in the inferior limbs. Neutrophilia, CPR 211mg/L. Positive blood cultures for Staphylococcus aureus methicillin-sensitive. Spleen embolization, with no abcess on abdominal CT. Transthoracic (TTE) and transesophageal echocardiography (TEE) disclosed a highly mobile polypoid mass in the atrial side of the anterior MV leaflet, septal left ventricular hypertrophy and systolic anterior motion (SAM) of the MV. Mild mitral regurgitation (MR). No evidence of abcess, aneurysm or valve perforation. The diagnosis of IE was established and the patient completed 42 days of Flucloxaciline. Favorable clinical evolution, residual lesions on the MV. TTE and TEE were repeated on follow-up. Besides HCM and SAM of the MV, an aneurysm of the anterior leaflet of the MV was identified and two regurgitant jets: one due to incomplete coaptation of the leaflets; other through the perforated aneurysm. Mild global MR. A strategy of close follow-up was adopted. Beta blocker dose was increased. Maintenance of the characteristics of the aneurysm. DISCUSSION MVA are rare, with perforation and significant MR development as the most serious complications. They mostly develop in the acute setting of IE of the aortic valve (AV), due to the "jet lesion" from the regurgitant jet or direct extension of the infection. In this case, MVA developed as a late complication of IE of the MV. Previous infection and inflammation lead to increased susceptibility of the valve leaflet, with possible persistent chronic inflammation. In the setting of obstructive HCM, the lesioned endothelium is exposed to significant intraventricular pressure gradients, which have probably raised its propensity to bulge towards the atrium, resulting in aneurysm formation and perforation. Optimal approach to MVA has not been defined. If the setting of perforation with severe MR, surgery must be performed in order to avoid a fatal outcome. In small aneurysms with mild MR, a conservative approach seems reasonable. The purpose of this case is to highlight potential complications of IE, which should be actively investigated, with echocardiography playing a central role in the diagnosis and follow-up.

scholarly journals P1320 Percutaneous mitral valve leaflet plication to reduce systolic anterior motion and mitral regurgitation using the transcatheter mitral clip system

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Drakopoulou ◽  
S Soulaidopoulos ◽  
G Oikonomou ◽  
K Stathogiannis ◽  
K Aggeli ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Significant Coronary Artery Disease ◽  
Mitral Valve Regurgitation ◽  
Left Ventricular ◽  
Heart Team ◽  
Severe Mitral Regurgitation ◽  
Systolic Anterior Motion ◽  
Anterior Leaflet ◽  
Mitral Valve Leaflet

Abstract A 72-year-old female patient with a past medical history of severe mitral regurgitation, atrial fibrillation and embolic cerebrovascular events was admitted to our institution. The patient was under optimal medical therapy and complained for progressive worsening of activity-related dyspnea with limitation of physical activity (NYHA III). Transthoracic echocardiography showed the presence of severe mitral regurgitation with a central jet. There was prolapse of both mitral valve leaflets and interestingly the anterior leaflet presented systolic anterior motion (SAM) at the same time. There was no significant left ventricular outflow tract obstruction (LVOT). Further evaluation of the regurgitant mitral valve with a transesophageal echocardiography (TOE) confirmed the above findings and the mechanism of MV regurgitation was attributed to prolapse in addition to SAM of an elongated anterior leaflet. Laboratory test showed elevated NT-pro-BNP levels. A coronary angiography was performed and excluded significant coronary artery disease. The findings were assessed by our institution’s HEART TEAM and, in the presence of high surgical risk (LogEuroscore 32,76%), a decision for transcatheter mitral valve repair with a Mitral Clip implantation was taken. The Mitral Clip was succesfully implanted with immediate significant reduction of the regurgitant jet and no signs of stenotic behavior of the repaired valve. There was only mild mitral valve regurgitation. Notably, after the procedure there was elimination of the SAM and no LVOT obstruction (Figure). In accordance to the echocardiography findings, the patient demonstrated a significant clinical improvement and was discharged home 1 day after the procedure. Mitral clip implantation in this case showed improvement of the MR by reducing the SAM of the mitral valve. Abstract P1320 Figure.

scholarly journals P295 Impact of Pre-existing Mitral Regurgitation on the Mid-Term Left Ventricular Mass Regression following Transcatheter Aortic Valve Implantation for Aortic Valve Stenosis

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
H Horinouchi ◽  
T Nagai ◽  
Y Ohno ◽  
T Murakami ◽  
J Miyamoto ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Aortic Valve ◽  
Mitral Regurgitation ◽  
Left Ventricular ◽  
Regurgitant Volume ◽  
Transcatheter Aortic Valve ◽  
Post Procedure ◽  
Lv Mass ◽  
Valve Implantation

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.

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

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
TP Craven ◽  
PG Chew ◽  
M Gorecka ◽  
LAE Brown ◽  
A Das ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Ejection Fraction ◽  
Left Ventricular ◽  
Valve Leaflet ◽  
Reference Standard ◽  
Mitral Valve Leaflet ◽  
Reverse Remodelling ◽  
Primary Mitral Regurgitation

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.

Management of Isolated, Congenital Anterior Mitral Valve Cleft

2021 ◽  
Vol 13 (1) ◽  
pp. 60-64
Author(s):  
J. Blade Hargiss ◽  
Joseph A. Dearani ◽  
Elizabeth H. Stephens ◽  
Nathaniel W. Taggart
Keyword(s):  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Surgical Repair ◽  
Nyha Class ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Postoperative Result ◽  
Ventricular Ejection Fraction ◽  
Class 1

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.

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