Leaflet Prolapse Research Articles

Aortic Valve Sparing Operations in the Presence of Aortic Cusp Prolapse

Aortic Valve Sparing Operations in the Presence of Aortic Cusp Prolapse

Long-term Results of Valve-Sparing Aortic Root Replacement and Aortic Cusp Repair

BackgroundLong-term results of valve-sparing aortic root replacement (VSRR) and aortic cusp repair for aortic regurgitation are unclear. MethodsVSRR by reimplantation was performed in 363 patients. Tricuspid aortic valve (TAV) and bicuspid aortic valve were found in 285 and 71 patients, respectively. ResultsAortic cusp repair was performed in 268 patients. Of patients with TAV 129 had central plication of the Arantius node, 36 had free margin resuspension, and 71 had reinforcement. Mean follow-up was 71.4 months. Among TAV patients freedom from aortic valve reoperation at 10 and 15 years was 85.1% and 78.3%, respectively. Freedom from aortic valve reoperation at 10 years was lower in patients with cusp prolapse than without (77.4% vs 93.2%, P = .007). The overall freedom from more than mild aortic regurgitation at 10 and 15 years was 72.4% and 64.0%, respectively. It was also significantly greater in patients without cusp prolapse (78.4% vs 67.7%, P = .02). As for the cusp repair technique the freedom from aortic valve reoperation at 10 years was significantly better in patients who underwent only resuspension or reinforcement techniques compared with patients who underwent only central plication technique (100% vs 72.8%, P = .008). ConclusionsLong-term results of VSRR with aortic cusp repair were satisfactory. The resuspension technique appears to be useful for repairing aortic cusp prolapse in patients with TAV.

Microinvasive mitral valve repair with transapical mitral neochordae implantation.

Microinvasive cardiac surgery includes procedures performed off-pump, on the beating heart, with limited or absent skin incision, and those that rely on live imaging techniques. Transapical off-pump beating heart neochordae implantation allows the repair of severe mitral valve regurgitation due to leaflet prolapse or flail with live three-dimensional echo guidance. This procedure has shown good results for up to 5 years and can be considered as a valid alternative to conventional surgery in selected patients with high prediction of success based on clinical and anatomical considerations. The aim of this review is to describe the devices, indications, patient screening process, clinical and echocardiographic results, and future perspectives of this procedure.

Late results of chord transfer and other techniques for anterior leaflet repair without neochords

ObjectiveAnterior and bileaflet degenerative mitral regurgitation repairs are challenging. We examined our early and late outcomes for repair using 4 techniques, without neochord repair. MethodsBetween February 1, 2006, and June 30, 2021, a total of 2368 patients received mitral valve ± other surgery by 1 surgeon, including 1160 with degenerative mitral regurgitation. Clinical follow-up was conducted annually (mean 6.8 ± 4.4 years). ResultsRepair was performed in 1137 patients (98%) (mean age, 60.5 ± 11.9 years). Repair rate varied between groups: 99% for isolated posterior leaflet (794/799), 91% for isolated anterior leaflet (83/91), and 96% for bileaflet prolapse (260/270; P < .001). Thirty-day mortality was 0.2%. On a scale of 0 to 4+ mitral regurgitation, mean mitral regurgitation grade decreased from 3.8 ± 0.6 preoperatively to 0.07 ± 0.3 at discharge, including moderate (2+) in 0.6% (7/1137) overall and 0.9% (3/343) with anterior prolapse. None were more than 2+ at discharge. Among the 3 groups of leaflet prolapse, there was no significant difference in long-term survival (P = .26), freedom from mitral valve reintervention (P = .12; 99.4% overall), and freedom from more than moderate (2+) mitral regurgitation (P = .16; 98.3% overall). The 4 most common anterior leaflet repair techniques (chord transfer 17%; commissuroplasty 10%; Alfieri [edge-to-edge] 6%); ring with posterior resection (4.3%) had similar freedom from 10-year reintervention (99.4%, 94%, 100%, and 100%, respectively; P = .29). ConclusionsComplex anterior leaflet prolapse repairs are successful using a variety of techniques without neochord implantation. Although neochords are popular, there are other ways to repair complex valves that do not require as much judgment and experience.

Repair of Mitral Prolapse: Comparison of Thoracoscopic Minimally-invasive and Conventional Approaches.

Surgical repair remains the best treatment for severe primary mitral regurgitation. Minimally invasive mitral valve surgery is being increasingly performed, but there is a lack of solid evidence comparing thoracoscopic with conventional surgery. Our objective was to compare outcomes of both approaches for repair of leaflet prolapse. All consecutive patients undergoing surgery for severe mitral regurgitation due to mitral prolapse from 2012 to 2020 were evaluated according to the approach used. Freedom from mortality, reoperation and recurrent severe mitral regurgitation were evaluated by Kaplan-Meier method. Differences in baseline characteristics were adjusted with propensity score matched analysis (1:1, nearest neighbor). 300 patients met inclusion criteria and were divided into thoracoscopic (N = 188) and conventional (sternotomy; N = 112) groups. Unmatched patients in the thoracoscopic group were younger and had lower body mass index, New York Heart Association class and EuroSCORE-II preoperatively. After matching, thoracoscopic group presented significantly shorter mechanical ventilation (9 vs.15h), shorter intensive care unit stay (41 vs 65 h) and higher postoperative hemoglobin levels (11 vs.10.2 mg/dL) despite longer bypass and cross-clamp times (+30 and +17min). There were no differences in mortality or mitral regurgitation grade at discharge between groups nor differences in survival, repair failures and reinterventions during follow-up. Minimally invasive mitral repair can be performed in the majority of patients with mitral prolapse, without compromising outcomes, repair rate or durability, while providing shorter mechanical ventilation and intensive care unit stay and less blood loss.

Echocardiographic and clinical outcomes following beating heart NeoChord DS1000 mitral valve repair: a single centre case series.

The NeoChord DS1000 system implants artificial neochords transapically, through a left mini-thoracotomy to treat degenerative mitral valve regurgitation (MR). Performed without cardiopulmonary bypass, neochord implantation and length adjustment is guided by transesophageal echocardiography. We describe imaging and clinical outcomes for a single center case series using this innovative device platform. In this prospective series, all study patients had degenerative MR and were considered for conventional mitral valve surgery. Moderate to high-risk candidates were screened for NeoChord DS1000 eligibility based on echocardiographic criteria. Study criteria included isolated posterior leaflet prolapse, leaflet-to-annulus index greater than 1.2, and coaptation length index greater than 5 mm. Patients with bileaflet prolapse, mitral annular calcification, and ischemic MR were excluded from our early experience. Ten patients underwent the procedure, including 6 males and 4 females, with a mean age of 76 ± 9.5 years. All patients had severe chronic MR and normal left ventricular function. One patient required conversion to an open procedure for failure to deploy neochords with the device transapically. The median number of NeoChord sets was 3 (IQR 2.3-3.8). Immediate post-procedure (POD#0) degree of MR on echocardiography ranged from mild or less, and on postoperative day 1 (POD#1) from moderate or less. Average length of coaptation was 0.85 ± 0.21 cm and average depth of coaptation was 0.72 ± 0.15 cm. At 1-month follow-up echocardiography, MR was graded from trivial to moderate and left ventricular inner diameter dimensions decreased from an average of 5.4 ± 0.4 cm to 4.6 ± 0.3 cm. None of the patients who had successful NeoChord implantation required blood products. There was 1 perioperative stroke with no residual deficits. There were no device-related complications or serious adverse events. The median length of hospital stay was 3 (IQR 2.3-10) days. 30-day and 6-weeks postoperative mortality and readmission rates were 0%. We report the first Canadian case series using the NeoChord DS1000 system for off-pump, transapical, beating heart mitral valve repair, through a left mini-thoracotomy. The early surgical outcomes suggest this approach is feasible, safe, and effective in reducing MR. This novel procedure has the advantage of offering a minimally invasive, off-pump option for select patients with high surgical risk.

Aortic regurgitation in bicuspid aortic valve: know to decide

Abstract Funding Acknowledgements Type of funding sources: None. Chronic aortic regurgitation (AR) is the second cause of valve surgery in patients with bicuspid aortic valve (BAV) and has distinctive characteristics of trileaflet regurgitation. The quantification variables of aortic regurgitation do not take into account the unique characteristics of bicuspid regurgitation. Recognition of the mechanisms that lead to bicuspid AR is relevant as it allows correct treatment planning and valve repair in candidate patients. Objective a prospective study was designed to identify the mechanisms of AR and the factors (clinical and anatomical) associated with the development of significant AR (moderate/severe) in adult patients with BAV. Materials and methods We included consecutive adult patients with a confirmed diagnosis of BAV evaluated between 2014 and 2021 with at least 3 years of follow-up by the Structural Heart Disease group of our institution. Valve disease was evaluated by echocardiography and complementary methods. It was classified as mild, moderate (Mod), and severe (Sev) aortic regurgitation, according to current criteria (integral approach with quantitative and semi-quantitative criteria). The prevalence of regurgitation mechanisms, the progression of aortic regurgitation, and predictive variables of significant AR and major cardiovascular events (death, surgery) were analyzed using univariate and multivariate analyses. A p&amp;lt;0.05 was considered significant. Surgery was indicated following international recommendations (ESC guidelines): symptoms development or ventricular dysfunction. Results We included 300 patients (39.7 ± 14.9 years, 72.6% men). Regarding valvular phenotype 84% (257p) presented fused BAV (coronary leaflets) and the majority with raphe (87.5%). 71.7% of the patients had some degree of aortic regurgitation at the start of follow-up (24.1% Mod/Sev AR). Among the patients with significant AR, 50% had cusp prolapse (86% prolapse of the fused leaflet), 39.18% dilatation of the aortic root, 26.6% significant calcification of the valve, 1.3% valve perforation and 29.4% restriction of valve movement. 43% with Mod/Sev AR had mixed mechanisms of regurgitation and the combination, half represented by the combination of aortic root dilatation and cusp prolapse (Fig 2). During follow-up 18–6% of patients with Mod AR showed progression to Sev AR and 4.7% underwent surgery. On the other hand, 34.1% of patients with baseline Sev AR required aortic valve surgery (p&amp;lt;0.01). Conclusions In our cohort, AR was highly prevalent: 1 out of 4 patients had significant AR at the start of follow-up. Eccentric jets and the presence of valve prolapse were frequent in patients with significant AR. 43% of BAV patients with Mod/Sev AR exhibited mixed regurgitating mechanisms. Because mechanism affects the choice of surgical technique, comprehensive mechanism description is critical in patients with BAV and AR.

Windows, Wings and Wonder: Advanced Cardiac Imaging Via Transesophageal Two-Dimensional and Transthoracic Three-Dimensional Echocardiography for the Accurate Diagnosis of Double-Orifice Mitral Valve in the Background of Primum Atrial Septal Defect: A Case Report

The assessment of mitral valve anatomy can be difficult or even misleading on standard cardiac imaging procedures. Such is the scenario in a 40-year-old woman with an incidental finding of atrial septal defect (ASD) and consideration of a trileaflet versus double-orifice mitral valve (DOMV) on transthoracic two-dimensional echocardiography. Further interrogation using transesophageal two-dimensional and transthoracic three-dimensional (3D) echocardiography (to allow more accurate assessment and simultaneous viewing of multiple imaging planes) revealed the typical “seagull wing” configuration of the mitral valve on long-axis view and two distinct but nearly equal-sized orifices on short-axis view, one oriented anteriorly and the other posteriorly. The patient’s DOMV was classified as an incomplete bridge type as confirmed on 3D imaging, with each orifice having its own set of papillary muscles and chordal attachments. The mitral regurgitation was graded as severe, resulting from leaflet prolapse of the anterior orifice. No left ventricular outflow tract obstruction was demonstrated in this case, and the overall left ventricular systolic function was preserved. The ASD was of the primum type, with the configuration and exact location verified through 3D imaging. The accurate detection of congenital anomalies via noninvasive techniques and complementary advanced modalities is vital for preoperative planning, as well as anticipation of potential complications related to the structural anomalies. To our knowledge, this is the first local report of DOMV with primum ASD in an adult. KEYWORDS: double-orifice mitral valve, primum atrial septal defect, transesophageal and three dimensional echocardiography

Prolapse repair for aortic regurgitation in tricuspid aortic valves

ObjectiveCusp prolapse is an important cause of aortic regurgitation (AR) in tricuspid aortic valves (TAVs) and can be caused by myxomatous degeneration or cusp fenestration. Long-term data for prolapse repair in TAVs are scarce. We analyzed the results of aortic valve repair in patients with TAV morphology and AR caused by prolapse and compared the results for cusp fenestration and myxomatous degeneration. MethodsBetween October 2000 and December 2020, 237 patients (221 male; 15-83 years) underwent TAV repair for cusp prolapse. Prolapse was associated with fenestrations in 94 (group I) and myxomatous degeneration in 143 patients (group II). Fenestrations were closed using a pericardial patch (n = 75) or suture (n = 19). In myxomatous degeneration, prolapse was corrected by free margin plication (n = 132) or triangular resection (n = 11). Follow-up was 97% complete (cumulatively 1531, mean 6.5, median 5.8 years). Cardiac comorbidities were present in 111 patients (46.8%), and more frequent in group II (P = .003). ResultsTen-year survival was better in group I (I: 84.5%; II: 72.4%; P = .037), and patients without cardiac comorbidities (89.2% vs 67.0%; P = .002). Ten-year freedom from reoperation (P = .778), moderate or greater AR (P = .070), and valve-related complications (P = .977) were similar in both groups. AR at discharge was the only significant predictor for reoperation (P = .042). The type of annuloplasty did not affect repair durability. ConclusionsRepair of cusp prolapse in TAVs with preserved root dimensions can be performed with acceptable durability, even in the presence of fenestrations.

Features of Diagnostics and Course of Hypertrophic Cardiomyopathy in Real Clinical Practice

Introduction and purpose. Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) wall thickening not associated with increased afterload (hypertension and aortic stenosis), is usually caused by mutations in sarcomeric protein genes, and is inherited in an autosomal dominant manner. Unlike HCM, myocardial hypertrophy in its phenocopies is associated with the accumulation of substances such as amyloid, glycogen, etc. in the myocardium. The aim of our work was to analyze the features of the diagnosis and course of HCM in real clinical practice. Material and methods. A retrospective analysis of medical records of 80 patients (56.3 % of men) discharged with a diagnosis of HCM from a multidisciplinary hospital in Moscow in the period from 2007 to 2021 was carried out. The diagnosis of HCM in all patients was established on the basis of echocardiography data. The median age (25th and 75th percentiles are indicated in brackets) was 57 (48.5; 63) years. The duration of hospitalization was 8 (6; 12.5) days. Results. The reason for hospitalization was angina syndrome in 35 %, suspicion of acute coronary syndrome in 16.3 %, paroxysmal atrial fibrillation (AF) in 11.3 %, decompensation of chronic heart failure in 11.3 %, syncope in 7.5 % %, hypertensive crisis in 3.8 %, coronary angiography in 3.8 %, pacemaker implantation in 2.5 %, consultation with an arrhythmologist in 2.5 %, implantation of a cardioverter-defibrillator in 1.2 %, medical examination to resolve the issue of fitness for military service in 1.2 %, acute cerebrovascular accident in 1.2 %, hypotension in 1.2 %, drug bradycardia in 1.2 % of patients. Before hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension — in 53.8 %, chronic heart failure — in 77.6 %, chronic kidney disease — in 21.3 % of patients. Prior to the analyzed hospitalization, a history of myocardial infarction was diagnosed in 15 %, arterial hypertension in 53.8 %, chronic heart failure in 77.6 %, chronic kidney disease in 21.3 % of patients. LV wall thickness ≥1.5 cm was detected in 91.2 %, symmetrical form of hypertrophy — 22.1 %, apical — 5.2 %, papillary muscle hypertrophy — 1.3 %, interventricular septum — 71.4 % of patients. Permanent obstruction of the LV outflow tract (LVOTO) was detected in 62.8 % (9.0 % of patients had a history of septal reduction), transient LVOTO — in 1.3 %, non-obstructive HCM — in 35.9 %. The ejection fraction (EF) of the LV (according to Simpson) was 63 (55-70) %, CHF with reduced LV EF &lt;40 % was detected in 3.8 %, with a moderately reduced LV EF (40-49 %) — in 5 %, with preserved LV EF — in 68.8 % of patients. Anterior systolic movement of the anterior leaflet of the mitral valve occurred in 47.5 %, prolapse of the anterior leaflet of the mitral valve was described in 7.14 %. Mitral regurgitation was registered in 75 % of patients. 45 % of patients with HCM suffered from AF: permanent 15 %, paroxysmal 23.8 %, persistent 6.2 % of patients. During hospitalization, ventricular tachycardia was registered in 7.5 %, supraventricular tachycardia — 3.8 %, conduction disturbances were noted in 36.3 % of patients, of which atrioventricular block in 6.3 %, blockade of the right bundle branch block in 21.3 %, left bundle branch block in 15 %, and Wolff-Parkinson-White syndrome in 1.3 % . Implantation of a pacemaker in history was in 5 %, including in connection with MorgagniAdams-Stokes attacks — in 3.8 % of patients. During a median follow-up of 87 (interquartile range 45–131.5) months, 13.8 % of patients with HCM died. In deceased patients, LVOTO was significantly more common (in the dead 100 %, in the living 58.2 %, p = 0.006) and AF (in the dead 72.7 %, in the living 40.6 %, p = 0.047). Genetic testing and exclusion of HCM phenocopies was not performed during hospitalization and was not recommended for any patient. Conclusion. In real clinical practice, in most cases, only phenotypic diagnosis of HCM is carried out according to echocardiography, and screening for genetic mutations and HCM phenocopies is not performed. It is necessary to widely introduce genetic testing and screening for HCM phenocopies for the timely diagnosis of pathology that requires the appointment of specific pathogenetic therapy to improve the prognosis of patients

Impact of bench repair for donor mitral valve before orthotopic heart transplantation: a case report

BackgroundThe use of donor hearts with valvular disease has been considered debatable in heart transplantation for many years. However, few reports indicate successful heart transplantation using donor hearts with mitral regurgitation that underwent mitral valve repair on the back bench.Case presentationWe report two cases of a 38-year-old and a 48-year-old woman with implantable left ventricular assist devices who underwent heart transplantation at our institution. Transthoracic echocardiography of donor hearts just before the explant revealed that each donor heart had preserved cardiac function and significant mitral regurgitation due to mitral posterior leaflet prolapse and annular dilatation, respectively. Bench mitral valve repair was accomplished using triangular resection for one patient and annuloplasty for the other. This was followed by confirmation of excellent mitral leaflet coaptation without residual mitral regurgitation. Transthoracic echocardiography and right heart catheterization performed 6 months after transplantation clarified the favorable cardiac function of each transplanted heart without mitral regurgitation recurrence.ConclusionsEfficient utilization of donor hearts with mitral regurgitation may be acceptable when the cardiac function of donor hearts with mitral regurgitation is preserved and heart transplantation, including bench mitral valve repair, is feasible within an acceptable ischemic time.

What Is the Optimal Mitral Valve Repair for Isolated Posterior Leaflet Prolapse to Achieve Long-Term Durability?

Background This study assessed risk factors for mitral regurgitation (MR) recurrence or functional mitral stenosis during long-term follow-up in patients undergoing mitral valve repair for isolated posterior mitral leaflet prolapse. Methods and Results We assessed a consecutive series of 511 patients who underwent primary mitral valve repair for isolated posterior leaflet prolapse between 2001 and 2021. Annuloplasty using a partial band was selected in 86.3%. The leaflet resection technique was used in 83.0%, whereas the chordal replacement without resection was used in 14.5%. Risk factors were analyzed for MR recurrence ≥grade 2 or functional mitral stenosis with mean transmitral pressure gradient ≥5 mm Hg using a multivariable Fine-Gray regression model. The 1-, 5-, and 10-year cumulative incidence of MR ≥grade 2 was 7.8%, 22.7%, and 30.1%, respectively, whereas that of mean transmitral pressure gradient ≥5 mm Hg was 8.1%, 20.6%, and 29.3%, respectively. Risk factors for MR ≥grade 2 included chordal replacement without resection (hazard ratio [HR], 2.50, P<0.001) and larger prosthesis size (HR, 1.13, P=0.023), whereas factors for functional mitral stenosis were use of a full ring (partial band versus full ring, HR, 0.53, P=0.013), smaller prosthesis size (HR, 0.74, P<0.001), and larger body surface area (HR, 3.03, P=0.045). Both MR ≥grade 2 and mean transmitral pressure gradient ≥5 mm Hg at 1 year post surgery were significantly associated with the long-term incidence of reoperation. Conclusions Leaflet resection with a large partial band may be an optimal strategy for isolated posterior mitral valve prolapse.

Mid-term outcomes of non-resectional chordal foldoplasty for degenerative mitral regurgitation.

We aimed to evaluate the mid-term clinical and echocardiographic outcomes of chordal foldoplasty performed for non-resectional mitral valve repair in degenerative mitral valve disease with a large posterior leaflet. We reviewed 82 patients undergoing non-resectional mitral valve repair via chordal foldoplasty between October 2013 and June 2021. We analysed operative outcomes, mid-term survival rate, freedom from reoperation and freedom from recurrent moderate or severe mitral regurgitation (MR). The mean age of patients was 57.2 ± 12.4 years; 61 patients (74%) had posterior leaflet prolapse, 21 patients (26%) had bileaflet prolapse and all of them had at least 1 tall posterior leaflet scallop. Minimally invasive approach with a right mini-thoracotomy was used in 73 patients (89%). The operative mortality was zero. There was no conversion to mitral valve replacement and postoperative echocardiography revealed no more than mild residual regurgitation or systolic anterior motion. Five-year survival rate, freedom from mitral reoperation and freedom from recurrent moderate/severe MR were 93.9%, 97.4% and 94.5%, respectively. Non-resectional chordal foldoplasty is a simple and effective repair technique for select degenerative MR cases with a tall posterior leaflet.

P406 LAUBRY–PEZZI SYNDROME: EVERYTHING IS NOT AS IT SEEMS

Abstract Introduction Laubry–Pezzi syndrome is a congenital heart disease characterized by the prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD), mostly supracristal, leading to severe aortic regurgitation (AR). It has been reported nearly always in childhood, the Venturi effect is the predominant factor and onset symptoms may be variable. Case Report A 61–years–old male was admitted to our intensive case unit for chest pain radiated to interscapular region and exertional dyspnea. Hemodynamic parameters were normal. At ECG sinus rytm with heart rate 92 bpm and non–specific repolarization abnomalities were reported. Hs troponin I was 276 ng/L, myoglobin 30.2 ng/mL and BNP 325 pg/mL. Transthoracic echocardiography showed a dilated left ventricle (LV EDV 107 mL/m2) with normal ejection fraction (55%), severe AR without aortic root dilation and a suspicious shunt left to right at level of interventricular septum. Therefore, transesophageal echocardiography was performed and confirmed severe AR by probable bicuspid valve and right cusp prolapse, in absence of calcifications and/or vegetations by endocarditis, restrictive perimembranous VSD (maximum diameter 0.6 cm) and ostium secundum ASD with shunt L–R. Right chambers were not dilated and non invasive systolic pulmonary artery pressure was 43 mmHg. Coronary angiography was performed and showed a significant stenosis of distal left anterior descending (LAD) artery and of proximal circumflex artery. Right heart catetherization excluded significant high values of pulmonary pressures (mPAP=18 mmHg), of pulmonary capillary wedge pressure (9 mmHg), with a mild increase of pulmonary vascular resistances (1.16 HRU; nv &amp;lt; 96). Coronary artery bypass grafting (left internal mammary artery on LAD and Y–graft radial artery on obtuse marginal artery), aortic valve replacement with bioprothesis (Inspiris Resilia 23 mm), ASD closure by direct suture and VSD closure with pericardial patch were performed. No post–surgical complications were reported. Interestingly, during the replacement of aortic valve, it resulted tricuspid, confirming the suspicion of Laubry Pezzi syndrome. Conclusions our case was object of great debate because restrictive VSD and severe AR were not previously known, were diagnosed in middle age and until then paucisymptomatic. Laubry–Pezzi syndrome is a very rare disease that may explain controversial cases of severe AR.

P403 MITRAL VALVE PROLAPSE AND RISK OF SUDDEN CARDIAC DEATH IN A PATIENT WITH VENTRICULAR ARRHYTHMIAS: THE PROGNOSTIC ROLE OF CARDIAC MAGNETIC RESONANCE IMAGING

Abstract Introduction in most cases mitral valve prolapse is a benign condition, however in 2% of cases it has been associated with the risk of sudden cardiac death (SCD). Basso et al have demonstrated papillary muscle’s and left ventricle’s inferolateral wall fibrosis on cardiac magnetic resonance imaging (MRI), in patients who died from SCD with mitral valve prolapse. Clinical case A 59–year–old patient was admitted to the Cardiology Department of Pertini Hospital (Rome, Italy) for episodes of vertigo and not sustained ventricular tachycardia in two morphologies at 24–hour ECG monitoring. The patient had negative history for cardiovascular risk factors, but an echocardiogram had demonstrated a moderate mitral valve regurgitation, secondary to valve prolapse with mitral annular disjunction (MAD) (Fig.1). Considering patient‘s age associated with low pre–test probability of coronary artery disease, the patient underwent coronary CT that showed a coronary artery tree free from injury. Cardiac MRI showed increased volumes in the left ventricle and slightly reduced systolic function and valve prolapse of both mitral leaflets determining moderate regurgitation. In addition, intra–myocardial Late Gadolinium Enhancement (LGE) showed fibrosis of the inferior and inferolateral wall with extension to the proximal portion of the inferior papillary muscle (Fig.2). Considering such findings, an intracavitary electrophysiological study (EPS) was performed for an evaluation of the arhythmic burden, by administrating the patient a double extra stimulus from the outflow tract of the right ventricle; the treatment induced a polymorphic ventricular tachycardia, starting from the inferior papillary muscle and degenerating into ventricular fibrillation (Fig.3). Discussion considering all the above findings, a mitral valve prolapse at high risk of malignant arrhythmias has been diagnosed and a subcutaneous cardiac defibrillator was implanted. Conclusions in mitral valve prolapse associated with complex ventricular arrhythmias, evaluation with cardiac MRI increasingly plays a crucial role in prognostic stratification for defibrillator implantation in primary prevention.

P167 CARDIAC ARREST IN SUSPECTED LONG QT: APPROACH TO ARRHYTHMIC MITRAL VALVE PROLAPSE

Abstract Mitral valve prolapse is a common condition. It affects 1–3% of the population. Clinical manifestations range from the absence of symptoms to sudden cardiac death. The arrhythmogenic mechanism is related to fibrotic changes in myocardial posterior caused by valve prolapse. ECG abnormalities indicative of high risk are inverted or biphasic T waves, ST depression, QT prolongation or dispersion, and BEV originating from the left outflow tract or papillary muscles. The echocardiogram allows the identification of mitral prolapse and disjunction of the mitral annulus (MAD), while MRI shows fibrosis. ICD implantation should be considered in high–risk patients with documented ventricular arrhythmias. Clinical case A 29–year–old woman with a family history of sudden cardiac death reported a state of absolute well–being until September 2022 when she presented with syncope with referred pulse failure for which she underwent cardiac massage, without defibrillation, by family members. At the arrival of 118 the patient presented bigeminal ventricular extrasystole with superior axis and BBsx type morphology (Fig.1). When she arrived at the hospital, ECG showed long QT (484 ms) associated with hypokalemia (2.8 mmol / l). During hospitalization, the coronary angiography was normal, while the cardiac MRI showed a globular and dilated left ventricle with preserved EF, thickening of the mitral leaflets, prolapse of both leaflets more evident at the level of P2/P3, MAD more evident in P3 with separation maximum end–systolic of 12 mm and absence of edema (Fig. 2). The patient underwent ICD implantation. In the presence of hypertelorism, prognathism, and flat feet, even in the absence of anomalies affecting the aorta and epiaortic vessels, scarring defects or ease to joint sprain, genetic screening was started for channelopathies and collagen diseases. Conclusions The approach to the patient with cardiac arrest and typical facies must be based on a systemic overview of the patient, whose phenotypic characteristics may suggest involvement of connective tissue diseases. In the future, the cardiologist must develop adequate knowledge to fully collect the information that the patient provides not only during instrumental examinations but also during an adequate clinical objective examination and facies, possibly supported by artificial intelligence.

P162 ARRHYTHMIC MITRAL VALVE PROLAPSE OCCURRING AS ABORTED SUDDEN CARDIAC DEATH

Abstract Mitral valve prolapse (MVP) is the common valvular heart disease, with a prevalence about 1–3% in the population. MVP is characterized on echocardiography by an abnormal systolic displacement of mitral valve leaflets into left atrium (LA) ≥ 2 mm above the mitral annulus. We described a clinical case of a 15 years–old boy with a first clinical presentation of out–of–hospital cardiac arrest related to ventricular fibrillation (VF) with a successful resuscitation after 2 DC shocks. Retrospectively, an episode of loss of awareness reported as post–minational. No familiar history of sudden cardiac death (SCD) or MVP was noticed. In the emergency room, the patient was hemodynamically unstable and was sedated and intubated. The surface electrocardiogram (ECG) showed sinus rhythm, QRS with fragmentation, and frequent monomorphic isolated premature ventricular contractions (PVCs) with RBBB/LAFB configuration.Frequent monomorphic PVCs with bigeminy was observed during continuous ECG monitoring, so the patient was treated with magnesium sulphate and metoprolol. The patient was subsequently weaned from ventilation support, presenting good gas exchange and without neurological sequalae. The 24h Holter monitoring showed an average of 53 bpm, extremely rare supraventricular extrasystoles, high incidence of monomorphic PVCs (23% of the total beats) with 4 couplets. Transthoracic echocardiography documented normal size and thickness with systolic function (GLS –21%, mild asynchrony of posterior basal segments), bi–leaflet prolapse associated to mild–moderate mitral regurgitation, and mitral annular disjunction (MAD) of 6.2 mm. The late gadolinium enhancement cardiac magnetic resonance showed bi–leaflet prolapse with mild insufficiency and maximum MAD 9 mm, mild LA dilatation, and slightly dilated both ventricles, without regional wall motion abnormalities; no scar or fibrosis at the late gadolinium enhancement was observed. According to guidelines, the patient received a subcutaneous implantable cardiac defibrillator for the secondary prevention of SCD and maintained under betablockers in order to control the arrhythmic burden. The genetic sampling did not show any pathogenetic mutation potentially related to cardiomyopathies or channelopathies. This case show an arrhythmic MVP complex with probably posterior papillary muscle source. The early PVCs with R/T constituted a possible trigger of VF. The betablocker therapy exhibited a full suppression of extrasystolic burden.

P155 CT EVIDENCE OF PARTIAL ISCHEMIC PAPILLARY MUSCLE RUPTURE

Abstract A 77–year–old man was referred for coronary CT following an episode of prolonged chest pain that occurred about 30 days earlier, with subsequent onset of dyspnea on moderate exertion. Coronary CT demonstrated right dominance, occlusion of the right coronary artery, as well as severe stenosis of the distal segment of the left anterior descending artery and of the first obtuse marginal branch. CT scan also showed an intraventricular image consistent with partial rupture of the posteromedial papillary muscle (Figure 1). The patient was transferred to the emergency department. An ECG showed signs of inferolateral necrosis. Transthoracic echocardiography confirmed akinesia of the inferior and inferolateral walls and of the basal segment of the inferior septum, with severe mitral valve insufficiency from anterior leaflet prolapse (A3 scallop) from partial rupture of the posteromedial papillary muscle (Figure 2). Transesophageal echocardiography confirmed the diagnosis (Figure 3). The patient was admitted to the CCU and underwent coronarography, which confirmed the severe triple vessel coronary artery disease with occlusion of the right coronary artery, and then urgent mitral valve replacement with bioprosthesis and aortocoronary bypass with saphenous vein grafting to the posterior descending artery. The postoperative course was uneventful. Ischemic papillary muscle rupture is a rare cause of mitral insufficiency. Papillary rupture typically occurs within 5 days of an acute myocardial infarction, and causes mitral insufficiency by prolapse and flail of one or, more rarely, both mitral valve leaflets. The posteromedial papillary muscle is most commonly affected, since the anterolateral papillary muscle has dual supply from the anterior interventricular artery and the circumflex artery. Papillary muscle rupture can be total or partial; however, most cases of partial rupture progress to complete rupture and therefore require urgent surgical treatment. The peculiarity of this case lies in the fact that it is the first case described in the literature of a papillary muscle rupture documented on CT.

P47 MITRAL VALVE RE–REPAIR THROUGH A RIGHT MINITHORACOTOMY

Abstract Objective Recurrent mitral valve regurgitation after prior repair is usually treated with re–repair or replacement through a median sternotomy. We report our small initial experience with mitral valve re–repair through a repeated minimally invasive approach. Methods From July 2018 to November 2021, 10 patients (median age 52 years IQR 49–68; male 90%) underwent isolated mitral valve re–repair with a right mini–thoracotomy approach with peripheral arterial and venous cannulation. Median Euroscore II was 2.05 (IQR 2.03–2.54). Results The median time from the original operation to redo surgery was 4.7 (3.5–8.1) years. Median cardiopulmonary bypass and cross–clamp times were 128 (IQR 109–134) and 99.5 (IQR 81–112) minutes, respectively. Failure of previous mitral valve repair was mainly due to Gore–Tex chordal rupture (3 patients), annular detachment (3 patients), native chordal rupture (2 patients), and posterior leaflet prolapse (2 patients). Concerning the surgical terchnique, a complete mitral valve ring Corcym Memo 3D or 4D was used in 8 patients while additional repair techniques were needed 5 patients (PTFE neochords implantation, cleft or commissure closure). No conversion to full sternotomy and no reoperation for bleeding was necessary. Median ICU and hospital stay were 2.0 (IQR 2.0–2.0) and 7.5 (7.0–9.0) days respectively. Median follow up time was 3.5 (2.8–3.9) years. Neither deaths nor major postoperative complications occurred in the follow–up period. Conclusions In our experience, mitral valve re–repair can be an attractive option for patients with failure of previous mitral valve surgery and can be safely performed through a minimally invasive approach with good outcomes. Main advantages seem to be the avoidance of extensive surgical dissection, optimal valve exposure, and low risk of surgical complications.

Barlow's disease with arrhythmic phenotype: a case report

Barlow disease represents the extreme form of the degenerative mitral valve spectrum described by Carpentier. The myxoid degeneration of the mitral valve may result in a billowing leaflet or in a prolapse and myxomatous degeneration of the mitral leaflets. There are increasing evidences of the association between Barlow disease and sudden cardiac death. It is common in young women. Symptoms include anxiety, chest pain and palpitation. In this case report, the markers of risk for sudden death such as typical ECG changes, complex ventricular ectopy, a spiked configuration of the lateral annular velocities, mitral annular disjunction and evidence of myocardial fibrosis were assessed.