[Mitral valve prolapse and mitral annulus disjunction: be aware of a potential arrhythmogenic substrate]. / Prolasso valvolare mitralico e disgiunzione mitro-anulare. La consapevolezza di un possibile substrato aritmico.

Mitral valve prolapse is a relatively common disease with a good overall prognosis. However, in specific clinical and instrumental contexts, patients at high risk of ventricular arrhythmias and sudden cardiac death can be identified. Female sex, history of palpitations or syncope, bi-leaflet myxomatous valve, ECG repolarization abnormalities in the inferior leads, complex ventricular arrhythmias, left ventricular fibrosis detected by cardiac magnetic resonance correlate with a higher risk clinical profile. Additionally, morpho-functional abnormalities of the mitral valve annulus, particularly mitral annulus disjunction, may cause a mechanical stretch at the inferior basal ventricular wall and posterior papillary muscles, predisposing to myocardial fibrosis and arrhythmias. A risk stratification strategy is needed to identify patients with mitral valve prolapse and/or mitral annulus disjunction at high risk of arrhythmias; however, few data are available. Further prospective multicenter studies are warranted, focusing on medical therapy, the role of implantable cardioverter-defibrillators for primary prevention, efficacy of targeted catheter ablation or mitral valve surgery.

Surviving cardiac arrest from mitral annular disjunction: A case report.

A 38-year-old man with no previous medical history presented to hospital after having an out-of-hospital cardiac arrest. He was found to have a ventricular fibrillation and was successfully resuscitated after receiving cardiopulmonary resuscitation and three shocks. Extensive investigations were performed which included an electrocardiogram that showed no significant abnormality, coronary angiogram which showed unobstructed arteries, and a flecainide challenge test which was negative for Brugada syndrome. A resting echocardiogram showed a myxomatous mitral valve with mild bi-leaflet bowing, trivial mitral regurgitation, normal left ventricular systolic function, and no other structural abnormalities. A cardiac magnetic resonance imaging showed no significant late gadolinium enhancement to suggest infarct or myocardial scarring. He was subsequently diagnosed with idiopathic ventricular fibrillation and treated with a subcutaneous internal cardioverter-defibrillator for secondary prevention. A follow-up echocardiogram was performed which revealed the presence of mitral annular disjunction which has been recently shown to be associated with significant life-threatening arrhythmias and sudden cardiac death. This case highlights the importance of improving awareness of mitral annular disjunction which is not often considered as a cause for adverse patient outcomes.

Management and outcomes in mitral valve prolapse with ventricular arrhythmias undergoing ablation and/or implantation of ICDs.

BACKGROUND: While there is an association between isolated mitral valve prolapse (MVP) and sudden cardiac arrest (SCA), the baseline characteristics and outcomes of patients with isolated MVP who experience ventricular arrhythmias (VAs) and then subsequently undergo catheter ablation and/or implantable cardioverter defibrillator (ICD) implantation are unknown. METHODS: We performed a retrospective review of all patients at the Cleveland Clinic with isolated MVP between 1997 and 2016 who underwent VA catheter ablation or secondary prevention ICD implantation. RESULTS: Of 617 screened patients, we identified 43 patients with isolated MVP and significant VA who underwent ICD placement (n = 13, 30%) or catheter ablation (n = 30, 70%). Both leaflets were most commonly involved (n = 22, 52%) with posterior MVP being next most common (n = 15, 36%). The most common foci of VA origin was the left ventricular papillary muscle (n = 9, 27%). Ablation was successful in the majority of cases (n = 20, 65%). At a mean follow-up of 2.5 years, 11 patients (26%) had recurrent VT. CONCLUSIONS: Patients with isolated MVP and VA were more likely to have bileaflet prolapse and at least moderate mitral regurgitation. VA originated more commonly from left-sided foci. While ablation was acutely successful in the majority of cases, there was still a moderate rate of VA recurrence. There is still more study needed on factors that will predict malignant VAs and management of these VAs in the MVP population.

Mitral valve prolapse.

Introduction: Mitral valve prolapse (MVP) is a common valve pathology with a spectrum of disease from isolated prolapse to myxomatous, multi-scallop Barlow's disease. The main complications relate to progression of mitral regurgitation, endocarditis, sudden death, and stroke. The timing of intervention in patients with asymptomatic severe mitral regurgitation is controversial. Areas covered: This article reviews the pathophysiology, genetics, clinical features, diagnostic imaging, complications, long-term outcomes, and indications for intervention in MVP. Expert commentary: Several key dilemmas in the management of MVP remain. Factors which influence progression of mitral regurgitation are unclear and therefore, we have no therapeutic targets to prevent progression. Evidence-based methods to reduce the risk of sudden death, stroke, and endocarditis have not been identified. In symptomatic patients with severe mitral regurgitation valve surgery is recommended. In asymptomatic patients, careful risk stratification incorporating markers of left ventricular dysfunction, atrial fibrillation, pulmonary hypertension, and valve reparability is required to identify the optimal timing of intervention.

Clinical Outcome of Degenerative Mitral Regurgitation: Critical Importance of Echocardiographic Quantitative Assessment in Routine Practice.

BACKGROUND: Echocardiographic quantitation of degenerative mitral regurgitation (DMR) is recommended whenever possible in clinical guidelines but is criticized and its scalability to routine clinical practice doubted. We hypothesized that echocardiographic DMR quantitation, performed in routine clinical practice by multiple practitioners, predicts independently long-term survival and thus is essential to DMR management. METHODS: We included patients diagnosed with isolated mitral valve prolapse from 2003 to 2011 and any degree of mitral regurgitation quantified by any physician/sonographer in routine clinical practice. Clinical/echocardiographic data acquired at diagnosis were retrieved electronically. The end point was mortality under medical treatment analyzed by Kaplan-Meier method and proportional hazard models. RESULTS: The cohort included 3914 patients (55% male) mean age (±standard deviation) 62±17 years with left ventricular ejection fraction 63±8% and median after routinely-measured effective regurgitant orifice area (EROA) [interquartile range], 19 [0-40] mm2. During follow-up (6.7±3.1 years), 696 patients died under medical management, and 1263 underwent mitral surgery. In multivariate analysis, routinely-measured EROA was associated with mortality (adjusted hazard ratio, 1.19; 95% confidence interval, 1.13-1.24; P<0.0001 per 10 mm2) independently of left ventricular ejection fraction and end-systolic diameter, symptoms, and age/comorbidities. The association between routinely-measured EROA and mortality persisted with competitive risk modeling (adjusted hazard ratio, 1.15; 95% confidence interval, 1.10-1.20; P<0.0001 per 10 mm2), or in patients without guideline-based class I/II surgical triggers (adjusted hazard ratio, 1.19; 95% confidence interval, 1.10-1.28; P<0.0001 per 10 mm2) and in all subgroups examined (all P<0.01). Spline curve analysis showed that, compared with general population mortality, excess mortality appears for moderate DMR (EROA ≥20 mm2), becomes notable at EROA ≥30 mm2, and steadily increases with higher EROA levels (eg, higher EROA levels beyond the 40 mm2 threshold). CONCLUSIONS: Echocardiographic DMR quantitation is scalable to routine practice and is independently associated with clinical outcome. Routinely-measured EROA is strongly associated with long-term survival under medical treatment. Excess mortality versus the general population appears in the moderate DMR range and steadily increases with higher EROA. Hence, individual EROA values should be integrated into therapeutic considerations, in addition to categorical DMR grading.

[Malignant bileaflet mitral valve prolapse syndrome in otherwise idiopathic ventricular fibrillation]. / Malignes Mitralklappenprolapssyndrom bei "idiopathischem" Kammerflimmern.

A 32-year-old, otherwise healthy woman was admitted after successful out-of-hospital resuscitation due to ventricular fibrillation. Established cardiac, pulmonary, metabolic, and toxicological causes were excluded. However, persisting (biphasic) negative T waves in the inferior ECG leads and premature ventricular contractions (PVC) were noted. PVC morphology indicated a focus alternating between the posterior papillary muscle/the left posterior fascicle and the left ventricular outflow tract region/anterior papillary muscle. Echocardiography revealed a bileaflet mitral prolapse with mild mitral valve regurgitation. This case is a typical presentation of the recently described malignant bileaflet mitral valve prolapse syndrome. The patient was discharged without overt neurological deficit after implantation of a cardioverter-defibrillator.

STRUCTURAL HEART ANOMALIES (REVIEW).

Structural heart anomalies (SHA) are congenital abnormalities of cardiovascular system, characterized by various anatomical departures of heart and great vessels from normal conditions. SHA are a part of connective tissue dysplasia syndrome (CTDS), one of the most common congenital autosomal-dominant diseases in people of young and middle age. The most common SHA are a mitral valve prolapse, abnormal chords of left ventricle and their combinations. The clinical significance of these anomalies depends on a degree of severity and impact on intracardial hemodynamics, as described in the article. The most prognostically dangerous are multiple abnormal chords of left ventricle, which can be a sign of serious hereditary disease - a left ventricular non-compaction.

P210Long- term outcome of primary mitral valve prolapse: results from a population of 250 patients referred to a tertiary cardiovascular center.

BACKGROUND: Outcome of Mitral Valve Prolapse (MVP) was controversial for long time. Many studies reported great differences in the incidence of cardiovascular events due, above all, to heterogeneous and small studied populations. Most of theme were also published to late '80 of the last century till early '00. PURPOSE: To make a contemporary survey on the incidence of cardiovascular events in a selected population of patients affected by primary MVP referred to a tertiary cardiovascular center for the medical and surgical care of valvular heart disease. METHODS: We reviewed our MVP database; patients with at least 2 cardiologic evaluations inclusive of echocardiographic examination and at least 6 months follow up were enrolled. A total of 250 patients (126 F) were selected. Their mean age was 52.1 years (ranging from 13 to 88 yo). The average follow-up time was 100 months (8,33 yrs). RESULTS: At the first medical and echocardiographic examination 8 patients (3,2%) had no mitral regurgitation (MR), 104 (41.6%) have a trace/mild MR, 93 (37,2%) a moderate MR and 46 (18,4%) a severe MR. They were widely asymptomatic (NYHA I 205-82%, NYHA II 44-17.60%, NYHA III 1- 0.40%). Most of theme presented a bileaflet (140-55.8%) or a posterior MVP (94 - 37.6%); an isolated anterior MVP was rare (16 - 6,4%). Flail leaflet was present in 8 (3,2%) and 25 (10%) had a chordal rupture. Respectively 165 (65,6%) and 115 (46,1%) patients had thick and redundant leaflets. Mean antero-posterior mitral annulus diameter was 37 mm. During the follow up 7 patients died of non-cardiac cause and 5 (2%) of suspected cardiac cause (2 because of acute coronary syndrome and 3 died suddenly). MR progresses in 43 (17,2%) patients and finally we observed 81 (32,4%) moderate/severe and severe MR. 12 new chordal rupture occurred during the follow up in most cases concerning mitral chordae linked to posterior mitral leaflet (10 cases-83,3%). The worsening of MR provoked an evolution of the clinical condition of 48 patients (19.2%) which developed Dyspnea On Excertion (DOE) with 42 new NYHA II and 6 new NYHA III. At the end of the follow up the amount of patients symptomatic for DOE was 93 (37.2% vs 18% at the initial evaluation). A total of 45 patients (18%) underwent mitral valve surgery. 40 needed in-hospital treatment in most cases due to the development of atrial fibrillation (19 -7.6%) or heart failure ( 8- 3,2%). Endocarditis occurred in 4 patients (1.6%) and cerebrovascular accidents/cardioembolic event in 6 (2.4%). The overall cardiovascular event rate was 4,33/100 patients-year, significantly higher than reported in community based studies. CONCLUSIONS: The prognosis of a MVP population referred to a tertiary cardiovascular center is not benign. The most frequent complications are progression of MR and MV surgery. Sudden death is also more frequent than in general population. More studies are needed to identify what patients with MVP are at risk for it.

Transcatheter Mitral Valve Repair, MitraClip, in a Nonagenarian Patient with Acute Regurgitation / Reparo Mitral Percutâneo pelo MitraClip em Paciente Nonagenária com Insuficiência Mitral Aguda

O implante transcateter de MitraClip é uma opção promissora de tratamento da insuficiência mitral (IM) em pacientes com risco cirúrgico elevado. Neste relato, descreve-se o caso de paciente nonagenária com IM aguda por ruptura de cordoalha que, devido a risco cirúrgico proibitivo, foi submetida a implante transcateter de MitraClip com sucesso...

MitraClip transcatheter implant is a promising option for treating mitral regurgitation (MR) in patients with high surgical risk. This report describes the case of a nonagenarian patient with acute MR for chordae rupture that, due to prohibitive surgical risk, underwent a successful MitraClip transcatheter implant...

Unique experiences with intercontinental trials in stroke - part II.

Supported by the Canadian Medical Research Council we performed a randomized trial extending from Newfoundland to British Columbia. With others a number of observations showed that aspirin will reduce stroke. With National Institute of Neurological Disorders and Stroke support we learned who would benefit and not from surgery in these stroke threatened carotid diseased patients. We evaluated the upper limits of acceptability of complications beyond which harm was done. Amassing this large data base of approximately 5000 individuals, followed for five years, previously unknown carotid phenomena were observed: 1. Ischemic stroke occurs in patients with prolapsing mitral valves; 2. There is risk of stroke in patients with residual thrombi in the occluded stump of the carotid artery; 3. We detected a lower risk than expected in patients with nearly occluded carotid arteries. We support the contention of Yusuf and Cairns' that Canada needs to give more financial support to purely clinical research. It pays off !

The role of 3-dimensional echocardiography in the diagnosis and management of mitral valve disease: myxomatous valve disease.

Myxomatous mitral valve (MV) disease is a spectrum that ranges from fibroelastic deficiency to Barlow's disease. Diagnosis has been greatly aided by the use of 3-dimensional echocardiography, which improves not only the accuracy of lesion localization but also the quantification of the associated mitral regurgitation. These improvements in turn have altered MV surgical repair techniques and percutaneous interventions.

Management of systolic anterior motion after mitral valve repair: an algorithm.

OBJECTIVE: To evaluate the effectiveness and outcomes of an intraoperative and postoperative algorithm for managing systolic anterior motion (SAM) after mitral valve repair (MVRr). METHODS: All consecutive patients who underwent MVRr for degenerative disease from January 2002 to June 2011 were included, with the data collected retrospectively. Patients who underwent MVRr for primary SAM were excluded from the study. Patients who developed SAM after the repair were systematically treated according to the algorithm. The intraoperative algorithm first involved medical management techniques, followed by surgical correction for significant SAM (mild or greater mitral regurgitation, left ventricular outflow tract gradient > 50 mm Hg). The postoperative algorithm focused on medical management and symptoms to guide the treatment decisions. RESULTS: The overall in-hospital incidence of SAM was 6.6% (52/785). In 41 patients, SAM was identified in the operating room, and in 11 patients, it was found postoperatively on the predischarge echocardiogram. Of the 41 patients with intraoperative SAM, 35 (85.4%) had resolution with medical management and 6 (14.6%) required surgical repeat repair while in the operating room. No patient required mitral valve replacement for persistent SAM. Postoperatively, 11 new cases were identified, and 7 cases of resolved intraoperative SAM recurred. These postoperative cases of SAM were managed according to the postoperative SAM algorithm. At last follow-up, 17 (94.4%) of 18 patients had resolution of SAM and 1 (5.6%) patient had mild SAM (less than mild mitral regurgitation, peak left ventricular outflow tract gradient < 50 mm Hg) and were asymptomatic. No patients with postoperative SAM required reoperation after their initial surgery. The median echocardiographic follow-up was 1.3 years. During follow-up, 1 early death (noncardiac) and 2 late deaths (1 noncardiac, 1 of unknown etiology) occurred. CONCLUSIONS: SAM is a relatively frequent complication after MVRr and can occur intraoperatively or postoperatively. A systematic approach addressing perioperative SAM after MVRr yields excellent mid-term results.

Folding mitral valvuloplasty without posterior leaflet resection for calcified mitral annulus.

Mitral valve annular calcification has long been a challenge in repairing posterior mitral valve prolapse. Folding valvuloplasty of the posterior leaflet without resection provides a means of circumventing common procedural complications. This report demonstrates the success of folding valvuloplasty without resection in the treatment of mitral valve prolapse and severe annular calcification.

Current concepts in mitral valve prolapse--diagnosis and management.

Although mitral valve prolapse as a disease entity has been recognized for over 50 years, its precise definition has been elusive. Initial reports based the diagnosis on auscultatory findings (late systolic click - murmur), with left ventricular angiography as a confirmative test. Echocardiography, first the M-mode, and subsequently the two-dimensional, became the dominant diagnostic modality. However, the early reports did not distinguish between billowing valve and flail valve. The advent of surgical repair techniques provided a different perspective; the surgical definition of mitral valve prolapse is often different from that of cardiologists. Intraoperative echocardiography gained wide acceptance necessitating a common language to describe precise terminology of the leaflet anatomy and definition of valve prolapse. The present report proposes a terminology and definitions of valve prolapse with relevance to surgical mitral valve repair. The addition of real-time 3D transesophageal echocardiography now provides highly accurate localization of lesions and the multi segment assessment of valve pathology. The etiologic considerations and surgical repair techniques with the role of echo - surgery team in improved patient outcome are described.

Feasibility of myxomatous mitral valve repair using direct leaflet and chordal radiofrequency ablation.

OBJECTIVE: Minimally invasive repair of mitral valve prolapse (MVP) causing severe mitral regurgitation (MR) should reduce MR and have chronic durability. Our ex vivo, acute in vivo, and chronic in vivo studies suggest that direct application of radiofrequency ablation (RFA) to mitral leaflets and chordae can effect these repair goals to decrease MR. METHODS: A total of seven canines were studied to assess the effects of RFA on mitral valve structure and function. RFA was applied ex vivo (n = 1), acutely in vivo using a right lateral thoracotomy and cardiopulmonary bypass (n = 3), and chronically in vivo using percutaneous access to the heart (n = 3). RFA was applied to the mitral valve and its associated chordae. Mitral valve structure and function (in vivo preparations) were then assessed. RESULTS: Ex vivo application of RFA resulted in qualitative reduction in mitral leaflet surface area and chordal length. Acute in vivo application of RFA to canines found to have MVP causing severe MR demonstrated a 43.7-60.7% statistically significant (P = 0.039) reduction in postablation MR. Chronic, in vivo, percutaneous application of RFA was found to be feasible and the engendered alterations durable. CONCLUSION: These data suggest that myxomatous mitral valve repair using radiofrequency energy delivered via catheter is feasible.

Initial clinical experience with Myxo-ETlogix mitral valve repair ring.

OBJECTIVE: Complexity of mitral valve repair for myxomatous disease has led to low adoption. We report initial experience with a new ring designed specifically for myxomatous disease, the Myxo-ETlogix (Edwards Lifesciences LLC, Irvine, Calif). METHODS: From March 15, 2006, through November 19, 2007, 129 patients underwent mitral valve surgery for pure myxomatous disease, and 124 valves (96.1%) were repaired. The Myxo-ETlogix ring was used in 100 cases and the Physio ring (Edwards) in 24. The Myxo-ETlogix design includes a 3-dimensional shape to reduce systolic anterior motion and a larger orifice to accommodate elongated leaflets and decrease need for sliding plasty. Direct mitral valve measurements were made. Sizing was based on A2 height, and choice of ring type was based on unresected leaflet heights. RESULTS: There was no operative mortality or lasting perioperative morbidity. The Myxo-ETlogix group had taller A2, P1, P2, and P3 leaflet segments than the Physio group (P < or = .003). Only 1 sliding plasty was performed for asymmetry in the Myxo-ETlogix group. Predischarge and follow-up echocardiograms (n = 338 in 124 patients) disclosed transient nonobstructive chordal systolic anterior motion in 3 echocardiograms in 3 patients. No patients had 2+ or greater mitral regurgitation. At discharge, 5.7% had 1+ mitral regurgitation; this proportion was 17.3% at last follow-up (mean 6.1 +/- 4.4 months). CONCLUSION: In initial experience with the Myxo-ETlogix ring, nonobstructive systolic anterior motion has been rare and obstructive systolic anterior motion not observed. Ongoing prospective echocardiographic and clinical studies will elucidate the role of this etiology-specific ring.

[Mitral valve prolapse: identification of high-risk patients and therapeutic management]. / Mitralklappenprolaps: Identifikation von Patienten mit erhöhtem Risiko und therapeutisches Management.

Mitral valve prolapse shows a wide spectrum from a benign anatomic variant to a progressive disease with severe cardiovascular morbidity and mortality. Echocardiography is the most important tool for diagnosis and risk stratification. Predictors for high risk are significant thickening of mitral leaflet of > 5 mm ("classic" prolapse), moderate to severe mitral regurgitation and reduced left ventricular function. These patients have an increased risk of infective endocarditis, cerebral ischemia and sudden cardiac death. Because of the risk for the development of severe mitral regurgitation requiring surgery short follow-up intervals are necessary. In mitral prolapse syndrome cardiac clinical signs (palpitation, rhythm disorders, syncope, etc.) are associated with a prolapse that can be treated symptomatically with drugs after exclusion of other causes and significant mitral regurgitation requiring surgery.