Search

INTRODUCTION mitral valve prolapse(MVP) is a common disease in women, causing chest pain and palpitation, due to structural and functional valve abnormality, sometime associated with gastro-esophageal-reflux-disease(GERD).This is a challenging clinical problem in clinical practice,requires targeted diagnostic assessment,to identify the underlying causes of the symptoms because treatment needs to be tailored,according to the causes themselves, to resolve the symptoms. Our aim is to assess the prevalence of GERD in a population of postmenopausal women affected by MVP and if there is any correlation between the two conditions. PATIENTS AND METHODS MVP diagnosis was performed by echocardiograpic examination,according to ASE(American-Society-Echocardiography) criteria. 289 consecutive MVP women,symptomatic for chest pain and palpitation,were included,250 consecutive women without MVP, symptomatic for chest pain and palpitation were control group(CG).GERD diagnosis was performed according to 2013 ACG(American-College-Gastroenterology) criteria,women affected by thyroid disorders, all heart disease, including mitral disease with moderate or severe mitral regurgitation,gastrointestinal diseases assessed by gastroscopy were excluded. RESULTS 31(11%) women were affected by GERD,among 289 with MVP, 11(4.4%) women were affected by GERD,among 250 on CG,Chi-squared 8.1,Odds ratio 2.7,c.i.95%,p

IntroductionIn patients with mitral annular disjunction (MAD), it can be difficult to assess the severity of mitral regurgitation (MR), as they present with a prolapsing volume (i.e. volume resulting from mitral valve prolapse, blood volume shift) rather than a regurgitant jet. The influence of the mitral prolapsing volume (MPV) on cardiac dimensions is unknown. We hypothesised that the severity of MR is underestimated in these patients. Our aim was to measure MPV and to investigate its influence on cardiac dimensions in patients with MAD.MethodsWe retrospectively included 131 consecutive patients with MAD from our institution’s echocardiographic database. Transthoracic echocardiography was used to assess MPV. Additionally, we established a control group of 617 consecutive patients with degenerative mitral valve disease and performed propensity score matching.ResultsMedian MPV in the MAD group was 12 ml. MPV was an independent predictor for left ventricular end-diastolic (LVEDD) and end-systolic diameter (LVESD) and left atrial volume (allp 15 ml), LVEDD (56 ± 6 mm vs 51 ± 6 mm,p p p = 0.04] were significantly increased compared to matched patients with degenerative mitral valve disease and similarly assessed severity of MR.ConclusionDue to a volume shift based on the MPV rather than an actual regurgitant jet, MR severity cannot be assessed adequately in MAD patients. Increased MPV induces ventricular and atrial enlargement. These findings warrant future studies to focus on MPV as an additional parameter for assessment of the severity of MR in MAD patients.

Mitral annular disjunction (MAD) is an abnormal insertion of the hinge line of the posterior mitral leaflet on the atrial wall: the mitral annulus shows a separation or "disjunction" between the leaflet-atrial wall junction and the crest of the left ventricle myocardium. This anomaly is often observed in patients with myxomatous mitral valve prolapse. The anatomical substrate of MAD remains unclear for the following reasons: (1) most studies are focused on the association between MAD and arrhythmias, rather than on pathomorphological aspects of MAD; and (2) the complex anatomic architecture of the posterior mitral annulus is often simply described as the posterior segment of a fibrous ring. The aims of this paper are to review the pertinent normal anatomy of the mitral valve and to propose new hypotheses on the morphological nature of MAD.

The inherited connective tissue disorders (Marfan syndrome, Loeys-Dietz syndrome [LDS], and Ehlers-Danlos syndrome [EDS]) involve connective tissue of various organ systems. These pathologies share many common features, nonetheless compared to Marfan syndrome, LDS' cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis. The EDS are currently classified into thirteen subtypes. There is substantial symptoms overlap between the EDS subtypes, and they are associated with an increased incidence of cardiovascular abnormalities, such as mitral valve prolapse and aortic dissection.

Poland syndrome (PS) is a rare congenital malformation, most commonly characterized by absence of chest wall muscles on one side of the body. It may be accompanied with other deformities of the extremities. We present the case of a 10-year-old girl with Poland syndrome and hypertrichosis of the back of the trunk and extremities. The clinical examination did not reveal the etiology of the syndrome, such as familial predisposition or some event that led to interrupted blood flow during the early embionic growth. The pregnancy was concieved with in vitro fertilization (IVF); triplets were born and our patient is one of these three girls. The hypertrychosis appeared at 8 years of life, without evidence of previous familial occurance, medications or hormonal disbalance. Other malformations that were found were: a mild form of kyphoscoliosis and mitral valve prolapse. The child was evaluated using a multidisciplinary approach, with further follow-up planned with surgical correction of the chest wall and breast augmentation., Поландовиот синдром е ретка конгенитална малформација, која најчесто се карактеризира со отсуство на мускули на градниот кош на една страна на телото. Може да биде придружена и со други деформитети на екстремитетите. Нашиот приказ на случај е женско дете старо 10 години со Поландов синдром, здружен со појава на хипертрихоза на грбот и екстремитетите. Направените иследувања кај детето не ја открија етиологијата на синдромот, како генетска предиспозиција или настан за време на бременоста на мајката кој довел до намалена циркулација за време на раниот ембрионален раст на плодот. Кај мајката е спроведена in vitro фертилизација (IVF) и ова е едно од трите женски деца кои се родени. Хипертрихозата се појавила на 8-годишна возраст, без податок за претходна фамилијарна појава, медикаментозна терапија или хормонален дисбаланс. Додатни малформации кои се најдени беа: лесна форма на кифосколиоза и пролапс на митрална валвула. Детето е водено мултидисциплинарно со план за понатамошна хируршка корекција на градниот кош и неразвиената града.

Late-onset atrial fibrillation (LOAF) after valve surgery for degenerative mitral valve disease often with underlying mitral valve prolapse is a known phenomenon. However, there is no similar data for postoperative rheumatic heart disease (RHD) patients. We sought to assess the incidence and predictors of LOAF during postoperative follow-up in RHD patients. This single-center retrospective case–control study included a total of 384 RHD patients with normal sinus rhythm (NSR) who underwent rheumatic valve surgery between 1st July 2008 and 30th June 2013. Patients detected with de novo persistent atrial fibrillation (AF) after 2 months of valve surgery were diagnosed as having LOAF. Presurgical demographic and echocardiographic parameters were compared between the LOAF and NSR groups to identify risk factors for LOAF. The incidence of de novo LOAF after rheumatic valve surgery was 9.63% at an average of 2.67 ± 1.32 years follow-up. Age ≥ 32 years [OR 2.4 (95% CI 1.2–5.1); P = 0.01] and left atrial (LA) size ≥ 51 mm [OR 5.9 (95% CI 2.8–12.4); P

To study the cardiac and visceral signs of connective tissue dysplasia in children with juvenile rheumatoid arthritis, a comprehensive ultrasound examination of the abdominal cavity organs and echocardiography examination were conducted according to the standard protocol in 52 children aged from 5 to 16 years with the diagnosis of juvenile rheumatoid arthritis. The reference group consisted of 26 patients with autonomic dysfunction (vegetative-vascular dystonia). The incidence of such visceral dysplasia types as duodenogastric reflux, gastroesophageal reflux, and gallbladder malformations among children with juvenile rheumatoid arthritis was shown higher than in the reference group. The main cardiac sign of connective tissue dysplasia in patients with juvenile rheumatoid arthritis are an excessive chord of the left ventricle.

Background An association has been identified between mitral valve prolapse (MVP) and sudden cardiac arrest (SCA), and ventricular arrhythmias (VA). This study aimed to elucidate predictive factors for SCA or VA in MVP patients. Methods MVP patients who underwent cardiac magnetic resonance (CMR) were retrospectively included. Patients with other structural heart disease or causes of aborted SCA were excluded. Clinical characteristics (sex, age, body mass index, histories of diabetes, hypertension, and dyslipidemia) and electrocardiographic (PR interval, QRS duration, corrected QT interval, inverted T wave in the inferior leads, bundle branch block, and atrial fibrillation), echocardiographic [mitral regurgitation grade, prolapsing mitral leaflet, and right ventricular systolic pressure (RVSP)], and CMR [left atrial volume index, both ventricular ejection fractions, both ventricular end-diastolic and systolic volume indexes, prolapse distance, mitral annular disjunction, systolic curling motion, presence of late gadolinium enhancement (LGE), LGE volume and proportion] parameters were analyzed. Results Of the 85 patients [age, 54.0 (41.0–65.0) years; 46 men], seven experienced SCA or VA. Younger age and wide QRS complex were observed more often in the SCA/VA group than in the no-SCA/VA group. The SCA/VA group exhibited lower RVSP, more systolic curling motion and LGE, greater LGE volume, and higher LGE proportion. The presence of LGE [hazard ratio (HR), 19.8; 95% confidence interval (CI) 2.65–148.15; P = 0.004], LGE volume (HR 1.08; 95% CI 1.02–1.14; P = 0.006) and LGE proportion (HR 1.32; 95% CI 1.08–1.60; P = 0.006) were independently associated with higher risk of SCA or VA in MVP patients together with systolic curling motion in each model. Conclusions The presence of systolic curling motion, high LGE volume and proportion, and the presence of LGE on CMR were independent predictive factors for SCA or VA in MVP patients.

Objectives The aim of this study was to assess in patients with mitral valve prolapse (MVP) mitral annular disjunction (MAD) prevalence, phenotypic characteristics, and long-term outcomes (clinical arrhythmic events and excess mortality). Background Clinical knowledge regarding MAD of MVP remains limited and controversial, and its potential link with untoward outcomes is unsubstantiated. Methods A cohort of 595 (278 women, mean age 61 ± 16 years) consecutive patients with isolated MVP, with comprehensive clinical, rhythmic, Doppler echocardiographic, and consistent MAD assessment, were examined. MAD prevalence, associated MVP phenotypes, and outcomes (survival, clinical arrhythmic events) starting at diagnostic echocardiography were analyzed. To balance important baseline differences, propensity scoring matching was conducted among patients with and those without MAD. Results The presence of MAD was common (n = 186 [31%]) in patients with MVP, generally in younger patients, and was not random but was independently associated with severe myxomatous disease involving bileaflet MVP and marked leaflet redundancy (both P ≤ 0.0002). The presence of MAD was also independently associated with a larger left ventricle (P = 0.005). Age-matched cohort survival after MVP diagnosis was not worse with MAD (10-year survival 93% ± 2% for patients without MAD and 97% ± 1% for those with MAD; P = 0.40), even adjusted comprehensively for MVP characteristics (P = 0.80) and accounting for time-dependent mitral surgery (P = 0.60). During follow-up, 170 patients had clinical arrhythmic events (ventricular tachycardia, n = 159; arrhythmia ablation, n = 14; cardioverter-defibrillator implantation, n = 14; sudden cardiac death, n = 3). MAD was independently associated with higher risk for arrhythmic events (adjusted HR: 2.60; 95% CI: 1.87-3.62; P Conclusions This large cohort with MVP comprehensively characterized shows that MAD is frequent at MVP diagnosis and is strongly linked to advanced myxomatous degeneration. The presence of MAD was independently associated with long-term excess incidence of clinical arrhythmic events. However, within the first 10 years post-diagnosis, MAD was not linked to excess mortality, and although reassurance should be provided from the survival point of view, careful monitoring for arrhythmias is in order for MAD.

Arrhythmic mitral valve prolapse syndrome is associated with a high risk of death. A 60-year-old man with arrhythmic mitral valve prolapse syndrome was monitored with an implantable loop recorder. Nine months later dyspnea developed, followed by cardiac arrest. Echocardiography showed mitral valve chordal rupture. He underwent successful surgical mitral valve repair. (Level of Difficulty: Intermediate.), Central Illustration

Eine junge Patientin prasentiert sich mit Palpitationen. Bei der Abklarung fallen im Langzeit-EKG gehaufte ventrikulare Extrasystolen (VES) auf, echokardiographisch eine hochgradige Mitralklappeninsuffizienz mit Mitralklappenprolaps. Es wurde daher eine Mitralklappenoperation geplant, nach weiterem diagnostischen Work-up der Patientin wurde jedoch eine andere Therapie durchgefuhrt, die sich retrospektiv als hocheffizient darstellt.

Parachute mitral valve (PMV) is a rare congenital cardiac valvular anomaly often associated with other congenital cardiac defects, particularly Shone's complex, but may infrequently occur in isolation. PMV and its variants are predominantly associated with mitral stenosis (MS) or rarely mitral regurgitation (MR). We present the case of a middle-aged female who was evaluated for a syncopal episode and found to have an atypical variant PMV with disproportionately long anterior mitral leaflet, bileaflet mitral valve prolapse, and mitral annular disjunction, without associated MS or MR. This is the first case report to highlight this particular constellation of findings.

La Disautonomía primaria no degenerativa es una patología con respuestas poco precisas desde los centros reguladores centrales hacia el sistema nervioso autónomo, frecuente en la población. No se aborda desde un punto de vista integral, por desconocimiento y por la sintomatología tan variada: síncope, mareos, parestesias, disnea, insomnio, palpitaciones, estreñimiento-diarrea, fatiga, cefalea, sudoración, alteraciones de ánimo, opresión torácica, etc. Ante esta sintomatología, en todas las áreas de la Medicina prácticamente se encuentra presente, etiquetada como: migraña, síndrome de colon irritable, discinesia vesicular, fatiga crónica, síndrome anémico, hipoglucemia, vejiga irritable, síndrome de prolapso de la válvula mitral, intolerancia ortostática, síncope vaso-vagal, falla autonómica pura, síndrome de respuesta disfuncional, etc. Se realizó una revisión de la literatura, con el objetivo de analizar la patología integralmente, para su correcto diagnóstico y tratamiento, ampliando horizontes diagnósticos, ya que quizá dentro del campo de la otorrinolaringología, pacientes con diagnóstico de Vértigo postural paroxístico benigno o Migraña vestibular atípicos o de difícil control, correspondan realmente a esta patología.

IntroductionMitral valve (MV) prolapse (MVP) is a primary valvular abnormality. We hypothesized that additionally there are concomitant abnormalities of the left ventricle (LV) and MV apparatus in this entity even in the absence of significant mitral regurgitation (MR).ObjectiveTo characterize MV and LV anatomic and functional features in MVP with preserved LV ejection fraction, with and without significant MR, using cardiovascular magnetic resonance (CMR).MethodsConsecutive MVP patients (n = 80, mean 52 years, 37% males) with preserved LV ejection fraction, and 44 controls (46 years, 52% males) by CMR were included, as well as 13 additional patients with “borderline” MVP. From cine images we quantified LV volumes, MV and LV anatomic measurements (including angle between diastolic and systolic annular planes, annular displacement, and basal inferolateral hypertrophy) and, using feature tracking, longitudinal and circumferential peak systolic strains.ResultsSignificant MR was found in 46 (56%) MVP patients. Compared with controls, MVP patients had LV enlargement, basal inferolateral hypertrophy, higher posterior annular excursion, and reduced shortening of the papillary muscles. LV basal strains were significantly increased, particularly in several basal segments. These differences remained significant in patients without significant MR, and many persisted in “borderline” MVP.ConclusionsIn patients with MVP and preserved LV ejection fraction there is LV dilatation, basal inferolateral hypertrophy, exaggerated posterior annular displacement and increased basal deformation, even in the absence of significant MR or overt MVP. These findings suggest that MVP is a disease not only of the MV but also of the adjacent myocardium.

Background Mitral valve prolapse (MVP) is largely considered a benign condition. However, MVP is over-represented consistently in sudden unexplained death in the young (SUDY) cohorts. Objective To determine the prevalence and potential genetic underpinnings of suspected arrhythmogenic MVP in a referral cohort of SUDY cases. Methods In this retrospective study, medical records/autopsy reports and whole exome molecular autopsy (WEMA) results for 77 SUDY victims (27 female; average age at death 20.6 ± 8.9 years) were reviewed for evidence of myxomatous MVP and left ventricle (LV) fibrosis. Variants detected in the prespecified 147 WEMA gene panel with a minor allele frequency ≤ 0.001 in public exomes/genomes were classified using the 2015 American College of Medical Genetics (ACMG) guidelines. Results Overall, 6 of 77 (7.8%; 2 female; average age at death 20.7 ± 6.9 years) SUDY cases had MVP as the lone abnormal postmortem finding. The majority had bileaflet involvement (5/6; 83%) and microscopic LV fibrosis (5/6; 83%). In 2 SUDY cases (33%), subjects were diagnosed with MVP by echocardiography prior to death. Unexpectedly, an ACMG pathogenic/likely pathogenic (P/LP) was more likely to be detected in SUDY cases with MVP than those without (3/6 [50%] vs 9/71 [13%]; P < .05). Interestingly, the 3 variants identified in MVP-positive SUDY cases localized to genes associated previously with a cardiomyopathy/channelopathy predisposition (p.E1518fsX25-DMD, p.S285N-RYR2, and p.R109X-TTN). Conclusion This WEMA series provides additional evidence that the combination of MVP and LV fibrosis underlies an unexpected number of SUDY cases. Whether P/LP variants in cardiomyopathy/channelopathy-susceptibility genes contribute to the pathogenesis of arrhythmogenic MVP requires further investigation.

The association of mitral valve prolapse (MVP) with ventricular arrhythmias has long been known and has generally been considered a benign condition. In recent years, however, a small but not negligible risk of malignant ventricular arrhythmias and sudden cardiac death has been documented in the large population of subjects with MVP. The main predictors of major arrhythmic risk identified so far include history of syncope, ventricular repolarization abnormalities in the inferior-lateral electrocardiogram leads, right bundle branch block morphology of ventricular ectopic beats, finding of areas of myocardial fibrosis on cardiac magnetic resonance, and mitral annular disjunction (MAD) on echocardiogram, as well as a possible pro-arrhythmic genetic substrate. The stratification of arrhythmic risk, with the active search for red flags and in particular of MAD, is important to identify patients with the malignant arrhythmic variant of MVP in whom to implement closer surveillance and possible therapeutic interventions.

PURPOSE Our study aims to examine the midterm outcomes of the loop technique for extended mitral valve (MV) prolapse patients. METHODS From October 2008 to August 2020, we performed MV repairs in 407 patients with severe mitral regurgitation (MR). Follow-up ranged in duration from 287 to 2899 days (median, 872 days). The prolapse extensiveness (p-score) was determined based on the ratio of prolapsing segment's areas to whole area. We divided the whole MV into 10 segments (A1, A2 medial, A2 lateral, A3, P1, P2 medial, P2 lateral, P3, AC, and PC). Patients were categorized into three groups according to the p-score: simple (0.1-0.2), intermediate (0.3-0.4), and extensive (0.5-0.9). RESULTS All patients underwent MV repair with the loop technique. The rates of freedom from significant (moderate to severe or severe) MR at 5 and 7 years after surgery were 91% and 91%, respectively. There were 252, 115, and 40 patients in simple group, intermediate group, and extensive group, respectively. The following were significantly increased in extended group: Barlow disease (23/40 patients, p

Neonatal Marfan syndrome is a rare condition with poor prognosis because of severe mitral and/or tricuspid valve insufficiency. Mitral valve replacement is sometimes required in early infancy, while tricuspid valve replacement is rarely done. We report the first infant neonatal Marfan syndrome case with a missense variant of c.3706T>C in the fibrillin-1 gene that was successfully managed by mitral and tricuspid valve replacement. Early multiple-valve replacement may sometimes be required during infant age in this genetic syndrome.

Among the visceral manifestations of NDST in pregnant women most often diagnosed mitral valve prolapse (20-25%) that accompanied by more cardiovascular and obstetric complications during pregnancy. It demonstrates the high clinical significance of the problem of connective tissue dysplasia with mitral valve prolaps for pregnancy and requires adequate treatment programs for prevention of complications and management of pregnant women with connective tissue dysplasia. Aim. Determination of the frequency of pregnancy pathology in women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia. Materials and methods. 138 pregnant women with MVP and concomitant signs of NDST and 54 healthy pregnant women were selected for analysis. Clinical manifestations of NDST, different variants of arrhythmias and the total number of complications of pregnancy and childbirth were evaluated. Results. In pregnant women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia, cases of frequent sinus extrasystole were significantly more often compared to frequent ventricular arrhythmia (47.8% vs. 18.1%, p

Background Long-term predictors of recurrent mitral regurgitation (MR) after mitral valve plasty (MVP) remain to be elucidated. This study sought to determine the prognostic factors of recurrent MR during long-term follow-up after MVP, by analysing findings of three-dimensional transoesophageal echocardiography (TEE) conducted after MVP. Methods This study analysed 207 patients who underwent MVP for A2 and/or P2 prolapse and received TEE before discharge. Recurrent MR was defined as moderate or worse regurgitation detected by annual transthoracic echocardiography. Results During a median follow-up period of 49 months after MVP, 18 patients experienced recurrent MR and six patients needed reoperation. In the recurrent group, 16 of 18 patients showed less than moderate MR before discharge. Patients in the recurrent group underwent repair for worse MR (effective orifice area, 54±19 vs 44±16 mm2; p=0.01) and had shorter A2–P2 coaptation length (5.3±1.4 vs 7.3±1.5 mm; p Conclusion Coaptation length measured by post-MVP TEE predicted the tendency of recurrent MR. Patients with short coaptation length should be carefully monitored, even when residual MR is less than moderate after MVP.

BACKGROUND Although frequently considered a benign condition, new evidence has shown that mitral valve prolapse (MVP) is associated with complex ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Therefore, we conducted a systematic review and meta-analysis of the relevant studies to investigate the parameters that could identify MVP patients at higher risk of experiencing complex VAs. METHOD We performed a systematic literature search of PubMed for potential studies between January 2010 and January 2021. Our meta-analysis included studies comparing MVP patients with complex VAs (A-MVP) and those without (NA-MVP). We used the fixed-effects model to obtain the odds ratio (OR), risk ratio (RR), or mean difference (MD) and 95% confidence interval (CI) for each analyzed parameter. RESULTS Six studies with 848 individuals were included in the meta-analysis. As compared to the NA-MVP patients, A-MVP patients had a higher prevalence of inverted T-wave (OR: 2.73; 95% CI: 1.85-4.02; p

OBJECTIVES We have previously shown in experimental settings that a leaflet foldoplasty device reduces redundant leaflet area to re-establish mitral valve (MV) coaptation. The current study investigates the in vivo device retention and functional durability following foldoplasty. METHODS The prototype is of superelastic nitinol formed into a 3-dimensional shape. It is unfolded to engage a specified area of leaflet tissue and then folded to exclude this tissue from the coaptation surface. Design modifications were made and tested in benchtop studies to determine the optimal design for durable retention within the leaflet. To evaluate in vivo performance, posterior leaflet chordae were severed in Yorkshire pigs to produce complete posterior leaflet prolapse and severe mitral regurgitation. Design modifications were then used for MV repair. Five animals that underwent repair using the optimal design were observed for 2 weeks postoperative to evaluate the functional result and implant retention. RESULTS Device position and orientation were maintained at 2 weeks while preserving the functional MV repair in all 5 animals. Coaptation height was 5.5 ± 1.5 mm, which was not significantly different from a baseline of 4.9 ± 0.8 mm. The degree of leaflet excursion was 41.0 ± 16.0 compared to a baseline of 58.7 ± 27.5. CONCLUSIONS Device foldoplasty is a new concept for MV repair based on the reduction of redundant leaflet tissue area. This study demonstrates the feasibility of safe maintenance of this repair without early dislodgement or embolization.

Introduction Extended-spectrum β-lactamase-producing Escherichia coli (ESBL-EC) reportedly accounts for >20% of E. coli infections and 2.0% of infective endocarditis cases. Nonetheless, there is a global paucity of reports on infective endocarditis caused by ESBL-EC. Case An 83-year-old Japanese man who underwent mitral annuloplasty for mitral valve prolapse 5 years ago developed a fever of 38.5°C. The patient was hospitalized the first time for pyelonephritis and bacteremia due to ESBL-EC and treated successfully with the antimicrobial meropenem for 14 days. Two days after discharge, however, the patient was re-admitted with bacteremia due to ESBL-EC. He was treated successfully with the antimicrobial cefmetazole for 14 days. The patient was admitted to our institution for a third time due to bacteremia again, a day after discharge following meropenem antibiotic therapy. Transesophageal echocardiography showed vegetation in the anterior mitral valve annulus. Magnetic resonance imaging of the head showed septic cerebral embolism. The patient was diagnosed with infective endocarditis due to ESBL-EC and underwent mitral valve replacement. After 6 weeks of antibiotic therapy with meropenem and tobramycin, the patient recovered completely. The causative E. coli strain MS6396 was identified as the E. coli clone ST131 by multilocus sequence typing and confirmed the presence of bla CTX-M-27 ESBL gene. Conclusion Only six cases of infective endocarditis associated with ESBL-EC have been reported in the past. Moreover, this is the first report of a patient with infective endocarditis bacteriologically or genetically analyzed for ESBL-EC. In future, factors that may cause infective endocarditis in ESBL-EC infections may be clarified through more thorough bacteriological/genetic analyses of ESBL-EC.

Objectives The phenomenon of systolic anterior motion (SAM) of the mitral valve (MV) was discovered 50 years ago, but to date only a few studies have identified risk factors for SAM following mitral repair. There are limited data on the necessity of surgical reintervention on the MV once SAM is discovered by intraoperative transesophageal echocardiography. We sought to identify predictors of SAM in a large cohort of consecutive patients, assess the rate of early reintervention on the MV to address SAM, and follow the progression of SAM postdischarge. Methods Analysis of electronically stored echocardiographic exams of adults who underwent MV repair in a recent decade. Results Following MV repair, the incidence of SAM immediately after cardiopulmonary bypass was 13% (98 of 761 patients). Multivariable analysis revealed several preoperative risk factors of SAM development and progression, including a lower ratio of anterior to posterior leaflets heights, younger age, lower end-systolic left ventricular volume, presence of bileaflet prolapse, and male sex. SAM was managed conservatively in 91 patients (93%) and surgically in 7 patients (7%). In a majority of patients (70 of 98 patients [71%]) SAM resolved before hospital discharge. Conclusions Transesophageal echocardiography findings associated with SAM were excessive height of posterior to anterior mitral leaflet, smaller left ventricular end-systolic volume, and bileaflet prolapse. Conservative management of SAM was usually successful, and persistent hemodynamically significant SAM was uncommon. Prophylactic modification of the surgical technique to avoid SAM seems unnecessary for all but those at highest risk for developing SAM.

Objectives This study aimed to assess whether patients with MAD also have disjunction of the tricuspid annulus. Background Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral annulus. Whether the disjunction extends to the right side of the heart is not known. Methods In a cohort of patients with MAD, we assessed the presence of tricuspid annulus disjunction (TAD) with the use of cardiac magnetic resonance. We explored the associations between TAD and MAD characteristics and the relationship to ventricular arrhythmias (nonsustained/sustained ventricular tachycardias and aborted cardiac arrest). Results We included 84 patients (mean age: 48 ± 16 years; 63% female). We observed TAD in 42 (50%). Patients with TAD were older (age 52 ± 16 years vs. 43 ± 15 years; p = 0.02), had greater circumferential extent of MAD (164 ± 57° vs. 115 ± 58°; p = 0.002), greater maximum longitudinal MAD distance (9.4 ± 2.9 mm vs. 6.2 ± 2.8 mm; p Conclusions We report for the first time the existence of right-sided annulus disjunction as a common finding in patients with MAD. TAD was associated with more severe left-sided annulus disjunction and mitral valve prolapse, but not with ventricular arrhythmias.

Detailed 3-dimensional analysis of mitral annular disjunction was undertaken in 3 comparative cases of mitral valve prolapse. A case of Barlow disease showed extensive disjunction, whereas cases of fibroelastic deficiency and forme fruste demonstrated less extensive disjunction. Considering the current controversies surrounding disjunction, these observations call for detailed research in the future. (Level of Difficulty: Advanced.), Central Illustration

Marfan syndrome (MFS) is a heritable connective tissue disorder caused by pathogenic variants in the gene coding for the extracellular matrix protein fibrillin-1. While the disease affects multiple organ systems, the most life-threatening manifestations are aortic aneurysms leading to dissection and rupture. Other cardiovascular complications, including mitral valve prolapse, primary cardiomyopathy, and arrhythmia, also occur more frequently in patients with MFS. The standard medical care relies on cardiovascular imaging at regular intervals, along with pharmacological treatment with β-adrenergic receptor blockers aimed at reducing the aortic growth rate. When aortic dilatation reaches a threshold associated with increased risk of dissection, prophylactic surgical aortic replacement is performed. Although current clinical management has significantly improved the life expectancy of patients with MFS, no cure is available and fatal complications still occur, underscoring the need for new treatment options. In recent years, preclinical studies have identified a number of potentially promising therapeutic targets. Nevertheless, the translation of these results into clinical practice has remained challenging. In this review, we present an overview of the currently available knowledge regarding the underlying pathophysiological processes associated with MFS cardiovascular pathology. We then summarize the treatment options that have been developed based on this knowledge and are currently in different stages of preclinical or clinical development, provide a critical review of the limitations of current studies and highlight potential opportunities for future research.

Aims Mitral valve prolapse (MVP) causes left ventricular (LV) remodelling even in the absence of significant mitral regurgitation. To evaluate whether apical insertion of the papillary muscle (PM) influences the pattern and severity of MVP-related LV remodelling. Methods and results All MVP patients who underwent CMR at our institution between December 2008 and December 2019 were included, thoroughly reviewed and grouped according to apical/non-apical PM insertion. Apical PM insertion was found in 53/92 patients (58%) and associated with mitral leaflet thickening (P 5%) and non-sustained ventricular tachyarrhythmias was found in patients with apical PM insertion: 53% vs. 25% (P = 0.04) and 38% vs. 18% (P = 0.04), respectively. Conclusion Apical PM insertion is part of the phenotypic spectrum of MVP, impacts significantly LV remodelling, and potentially may be related to increased ventricular arrhythmogenicity.

Background With an increased number of surgical procedures involving the mitral annular region, the risk of mitral valve prolapse (MVP) has also increased. Previous studies have reported that worsening of MVP occurred early after radiofrequency catheter ablation (RFCA) at papillary muscles in ventricular tachycardia (VT) patients with preoperative MVP. Case summary We report a case where MVP and papillary muscle rupture occurred 2 wk after RFCA in a papillary muscle originated VT patient without mitral valve regurgitation or prolapse before. The patient then underwent mitral valve replacement with no premature ventricular contraction or VT. During the surgery, a papillary muscle rupture was identified. Pathological examination showed necrosis of the papillary muscle. The patient recovered after mitral valve replacement. Conclusion Too many ablation procedures and energy should be avoided.

Mitral annular disjunction (MAD) is a structural abnormality defined as the separation of the ventricular myocardium between the mitral valve annulus and the left atrial wall. It is present in some patients with mitral valve prolapse (MVP) and is associated with papillary muscle fibrosis and ventricular arrhythmia. Although it is easy to diagnose, it can be overlooked in daily practice. This study presents the case of a 42-year-old patient who was admitted to the cardiology clinic with complaints of palpitation and syncope. The patient was diagnosed with bileaflet MVP, MAD, and severe mitral regurgitation using transthoracic echocardiography and cardiac magnetic resonance imaging, in which ventricular tachycardia disappeared following subsequent surgical repair.

Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD. This case series describes 3 patients in an adult HAD clinic who have concomitant mitral valve prolapse, MAD, and malignant arrhythmias. These cases may represent a unique disease entity or overlap syndrome, and they introduce MAD as a potential arrhythmogenic risk marker in HAD.

The purpose of the research was to study the state of autonomic regulation in prepubertal children with mitral valve prolapse during an orthostatic test Materials and methods. The study involved 2 groups: the main – 26 children aged 10-11 years with mitral valve prolapse, and a control group – 22 relatively healthy children. The adaptive mechanisms were monitored by analyzing heart rate variability. All children participated in a cardiorhythmic examination at rest lying down and during an active orthostatic test. Results and discussion. Among the indicators that had significant differences, the indicators of regulatory process adequacy index and mode amplitude should be noted. In the group of children with mitral valve prolapse, an increase of the regulatory process adequacy index indicated the predominance of the functioning of the sinus node over the activity of the sympathetic division of the autonomic nervous system. An increase in the adequacy index and mode amplitude indicates the connection of the central structures of rhythm control (subcortical rhythms) during a change in body position. Stress index also increased. This index of tension of regulatory systems shows the activity of the mechanisms of sympathetic regulation, the state of central regulation. Children in the control group had a well-coordinated response of the sympathetic nervous system to the orthostatic test: the low frequency spectrum and very low frequency indicators increased. While in main group, the value of low frequency spectrum (the work of the sinus node) increased, the value of very low frequency (the reaction of the central structures of the nervous system) decreased. This indicates dysfunction of the most important reactions, which also affects the daily activities of children, increases the risk of mitral valve prolapse complications. Conclusion. In children with mitral valve prolapse, the absence of a pronounced typical reaction to an ortho test is a reflection of an adaptive-regulatory overstrain in conditions of morphological determinacy of connective tissue dysplasia, which are trying to ensure the adequacy of intracardiac hemodynamics. The data obtained will be useful for predicting the reaction of the body of children with mitral valve prolapse to physical activity of varying intensity

Mitral valve prolapse (MVP) is the most common valvar dysfunction in children. There is emerging evidence that MVP is not always a benign entity, hence identification of underlying mechanisms is pertinent to clinical management. Our group previously identified a ventricular contraction abnormality named end-systolic basal eversion (ESBE) in adults that contributed to MVP. The aim of this study was to evaluate regional circumferential strain in pediatric patients with MVP and ESBE compared to normal controls. Left ventricular circumferential strain was assessed in 16 pediatric patients referred for clinical echocardiographic examination with MVP and ESBE (MVP group) and compared to age-gender-matched healthy subjects. ESBE has been previously described as late systolic bileaflet mitral valve prolapse, papillary traction, and concomitant late systolic outward movement of the basal inferior myocardium. The mean age of the MVP group was 13.8 ± 4.6 year and 75% were female. All patients with MVP and controls had qualitatively normal systolic cardiac function. The MVP group had significantly lower regional strain values for 11/16 of the segments including all 6 basal segments. Importantly, the basal inferior (− 17.02 ± 8.32% vs. − 26.10 ± 3.18, p = 0.001) and basal inferolateral (− 19.53 ± 9.76 vs. − 26.10 ± 3.18, p = 0.03) had the lowest strain values compared to the average of all other segments suggesting weaker contraction in the basal inferior segments. Pediatric patients with MVP and ESBE are subject to a similar left ventricular mechanical dysfunction previously described in adults. ESBE was evident by decreased basal circumferential strain values. These findings denoted weaker contraction which is believed to propagate the late systolic outward movement of the basal ventricular myocardium.

The aim of our research was to increase the effectiveness of the therapy administered to the patients with idiopathic mitral valve prolapse by pharmacological correction of magnesium deficiency. 79 patients (23 females and 56 males with average years of age 35.7±4.3 ) with undifferentiated connective tissue dysplasia and mitral valve prolapse of the 1st and 2nd degree were examined. The control group consisted of 20 healthy individuals, comparable by sex and age. A test by the UNESCO Institute for Microelements was used for the preliminary diagnostics of magnesium deficiency. Daily ECG monitoring with heart rate variability analysis, echodopplercardiography with the assessment of left ventricular diastolic function and determination of magnesium concentration in blood serum were performed. For the demonstration of autonomic dysfunction “the test for detection of the signs of vegetative changes” was used (10). For the assessment of situational and personal anxiety an “anxiety test” by Ch. D. Spielberg and Y. L. Hanin (25, 26) was used. The succeeding study was performed after 6 months. It was found that complex therapy with magnesium orotate in patients with idiopathic mitral valve prolapse helps to reduce the frequency of clinical manifestations of neurovegetative disturbances in the majority of examined patients contributing to harmonization of the autonomic nervous system function. It has a favorable effect on dysplastic changes and the state of bioelectrical activity of the heart, as well as correction of the psychoemotional state.

This study used cardiovascular magnetic resonance (CMR) to evaluate whether elevated extracellular volume (ECV) was associated with mitral valve prolapse (MVP) or if elevated ECV was a consequence of remodeling independent of primary mitral regurgitation (MR) etiology.Replacement fibrosis in primary MR is more prevalent in MVP; however, data on ECV as a surrogate for diffuse interstitial fibrosis in primary MR are limited.Patients with chronic primary MR underwent comprehensive CMR phenotyping and were stratified into an MVP cohort (2 mm leaflet displacement on a 3-chamber cine CMR) and a non-MVP cohort. Factors associated with ECV and replacement fibrosis were assessed. The association of ECV and symptoms related to MR and clinical events (mitral surgery and cardiovascular death) was ascertained.A total of 424 patients with primary MR (229 with MVP and 195 non-MVP) were enrolled. Replacement fibrosis was more prevalent in the MVP cohort (34.1% vs. 6.7%; p 0.001), with bi-leaflet MVP having the strongest association with replacement fibrosis (odds ratio: 10.5; p 0.001). ECV increased with MR severity in a similar fashion for both MVP and non-MVP cohorts and was associated with MR severity but not MVP on multivariable analysis. Elevated ECV was independently associated with symptoms related to MR and clinical events.Although replacement fibrosis was more prevalent in MVP, diffuse interstitial fibrosis as inferred by ECV was associated with MR severity, regardless of primary MR etiology. ECV was independently associated with symptoms related to MR and clinical events. (DeBakey Cardiovascular Magnetic Resonance Study [DEBAKEY-CMR]; NCT04281823).

BACKGROUND The present study is based on the World Heart Federation (WHF) echocardiographic criteria to assess the prevalence of subclinical rheumatic heart disease (RHD) and elucidate evolution of the disease when the cases were placed on appropriate antibiotic prophylaxis and regular follow-up. The prevalence of subclinical RHD reported by previous active surveillance studies among asymptomatic school children is not comparable to our study because of major differences in screening methods. METHODS A random inclusion strategy was adopted to recruit urban and rural school children of Bikaner district in the state of Rajasthan, India. The diagnosis of RHD was based on the echocardiographic criteria proposed by the WHF. All studies were reported on-site by a single experienced cardiologist and the digitally preserved studies were reported by a second cardiologist off-site. The final diagnosis was made by consensus. The second echocardiogram was performed for cases diagnosed with RHD after two years from start of study to document early evolution of the disease with ongoing antibiotic prophylaxis. RESULTS A high prevalence of subclinical RHD was observed in the study population. Pathological mitral and/or aortic valve regurgitation was the commonest lesion, and a significant proportion of cases improved while on regular antibiotic prophylaxis. No case showed fixity of leaflets/ stenosis. CONCLUSION The prevalence of subclinical RHD is high in the study population, and the disease seems to regress over time in the presence of appropriate antibiotic prophylaxis.

Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus, which has been described by pathologists to be associated with mitral leaflet prolapse. Mitral annular disjunction is a common finding in patients with myxomatous mitral valve diseases. The prevalence of mitral annular disjunction should be checked routinely during presurgical imaging. Otherwise, mitral annular disjunction itself might be an arrhythmogenic entity, irrespective of the presence of mitral valve prolapse (MVP). Therefore, we should check echocardiography keeping in mind mitral annular disjunction. Further prospective studies are needed to address whether a causative mechanistic link exists between mitral annular disjunction and arrhythmic MVP.

Patients with mitral valve prolapse (MVP), undergoing early surgery for severe regurgitation, are usually characterized by a low degree of right chambers’ remodeling. In this selected population, the mechanisms leading to tricuspid annular (TA) dilatation (TAD) are not well understood. In this setting, we aimed to evaluate, using three-dimensional echocardiography (3DE), how right chambers affect TA size and might contribute to functional tricuspid regurgitation (FTR) progression. We studied 159 patients treated with early isolated surgery for MVP, characterized by: sinus rhythm; normal biventricular function; normal or elevated pulmonary artery pressure; tricuspid regurgitation (TR) ≤ mild; no concomitant cardiac disease. All patients reached a 3-year echocardiographic follow-up. Based on two-dimensional echocardiography, patients were divided in Group 1 (N = 68, 43%, TAD, TA ≥ 21 mm/m2) and Group 2 (N = 91, 57%, no TAD, TA

The incidence of supraventricular arrhythmia (SVA) is high in patients with mitral valve prolapse (MVP). The purpose of our study was to determine the role of parameters showing atrial conduction heterogeneity such as P‑wave dispersion (PWD) and atrial electromechanical delay (AEMD) in predicting the development of SVA in MVP patients.A total of 76 patients with MVP (56 female, 20 male) were included in the study. The patients were divided into two groups according to the presence or absence of SVA: 36 patients were allocated to the non-SVA group and 40 patients to the SVA group. Heart rate variability (HRV), PWD, and AEMD values were determined and compared.The PWD was found to be higher in the SVA group. Interatrial EMD was 32.00 ms (25.00-35.00) in patients with SVA while it was 18.00 ms in patients without SVA (11.00-23.75); the intra-atrial EMD was 17.0 ms (10.00-20.00) in patients with SVA whereas it was 10.00 ms (4.00-14.00) in patients without SVA. Lower HRV was found in the SVA group.In the SVA group, PWD and AEMD were increased while HRV values were decreased. Noninvasive parameters may help predict the presence and incidence of SVA during the follow-up of this group of patients.HINTERGRUND: Die Inzidenz supraventrikulärer Arrhythmien (SVA) ist bei Patienten mit Mitralklappenprolaps (MVP) hoch. Ziel der vorliegenden Arbeit war es, für die Vorhersage der Entwicklung von SVA bei MVP-Patienten die Bedeutung von Parametern zu ermitteln, welche den Nachweis einer Heterogenität der Vorhofüberleitung liefern, z. B. die P‑Wellen-Dispersion (PWD) und die atriale elektromechanische Verzögerung (AEMD).Dazu wurden 76 Patienten mit MVP (56 Frauen, 20 Männer) in die Studie einbezogen. Die Patienten wurden – je nach Vorliegen von SVA oder nicht – in 2 Gruppen unterteilt: Der Non-SVA-Gruppe wurden 36 Patienten zugewiesen und der SVA-Gruppe 40 Patienten. Herzfrequenzvariabilität (HRV), PWD und AEMD-Werte wurden ermittelt und verglichen.In der SVA-Gruppe erwies sich die PWD als höher denn in der Vergleichsgruppe. Die interatriale EMD betrug 32,00 ms (25,00–35,00) bei Patienten mit SVA, während sie 18,00 ms bei Patienten ohne SVA betrug (11,00–23,75); die intraatriale EMD lag bei 17,0 ms (10,00–20,00) bei Patienten mit SVA, dagegen bei 10,00 ms (4,00–14,00) für Patienten ohne SVA. In der SVA-Gruppe wurde eine niedrigere HRV festgestellt.In der SVA-Gruppe waren PWD und AEMD erhöht, während die HRV-Werte niedriger waren. Noninvasive Parameter können möglicherweise zur Vorhersage des Vorliegens und der Inzidenz von SVA im Rahmen der Nachuntersuchung bei dieser Patientengruppe beitragen.

Degenerative mitral valve disease (myxomatous degeneration or fibroelastic deficiency) is the most common indication for surgical referral to treat mitral regurgitation. Mitral valve repair is the procedure of choice whenever feasible and when the results are expected to be durable. Posterior leaflet prolapse is the commonest lesion, found in up to two-thirds of patients. It is the easiest to repair, particularly when limited to one segment. In these cases, rates of repairability and procedural success approach 100%, and there is now ample evidence that the immediate and long-term results are better than those of valve replacement. Notably, minimally invasive valvular procedures, surgical or interventional, have attracted increasing interest in the last decade. When performed by experienced groups, mitral valve repair is unrivaled irrespective of the severity of lesions, from simple to complex, which leaflets are involved, and the type of degenerative involvement (myxomatous or fibroelastic). Its results should be viewed as the benchmark for other present and future technologies. By contrast, percutaneous mitral valve repair is still in its infancy and its results so far fall short of those of surgical repair. Nevertheless, continued investment in transcatheter procedures is of great importance to enable development and improved accessibility, particularly for patients who are considered unsuitable for surgery. In this review, we analyze the current status of management of degenerative mitral valve disease, discussing mitral valve anatomy and pathology, indications for intervention, and current surgical and transcatheter mitral valve procedures and results. Resumo: A doença valvar mitral degenerativa – deficiência mixomatosa ou fibroelástica – é a indicação mais comum de referência cirúrgica para tratamento da regurgitação mitral. A plastia da válvula mitral é o procedimento de escolha sempre que possível e os resultados são duradouros. O prolapso do folheto posterior é a lesão mais comum, encontrada em cerca de dois terços dos pacientes. É mais fácil de reparar, principalmente quando limitado a um segmento. Nesses casos, as taxas de reparabilidade e de sucesso aproximam-se dos 100% e agora há ampla evidência de que os resultados imediatos e a longo prazo são melhores do que os da substituição valvular. Os procedimentos valvares minimamente invasivos, cirúrgicos ou de intervenção, têm ganhado crescente interesse na última década. Quando realizada por grupos experientes, a plastia da valva mitral não tem rival, independentemente da gravidade das lesões, das simples às complexas, de quais os folhetos envolvidos e do tipo de patologia degenerativa (deficiência mixomatosa versus fibroelástica). Os resultados devem ser usados como referência para outras tecnologias presentes e futuras. Por outro lado, o tratamento percutâneo da válvula mitral ainda é incipiente e os resultados, até ao momento, ficam aquém dos da correção cirúrgica. No entanto, o investimento contínuo em procedimentos transcateter é de extrema importância para possibilitar a evolução e acessibilidade aos pacientes, principalmente àqueles aos quais é recusada a cirurgia. Nesta revisão, analisamos o estado atual do tratamento da doença degenerativa da válvula mitral, discutindo a anatomia e patologia valvular, as indicações para a intervenção, os procedimentos e resultados cirúrgicos e transcateter atuais.

Background and Objectives: The anatomical characteristics of patients with mitral valve prolapse (MVP) and mitral regurgitation (MR) have rarely been investigated demographically to determine the applicability of transcatheter intervention. Therefore, the study objective was to analyze potential candidates and their prognosis. Predictors determining the prognosis were also investigated. Methods: Patients diagnosed with MVP and MR severity of ≥2+ were screened from our echocardiography database from 2010 to 2012. All clinical and echocardiogram information was retrieved from electronic medical records. The endpoint was all-cause mortality analyzed by a proportional hazards model. Results: A total of 1268 patients (mean age 57.50 ± 14.88 years, 47.16% female) with MVP and MR severity of ≥ 2+ were included. Isolated P2 (n = 239, 18.85%) appeared as the most common site of leaflet prolapse. The incidence of MR jet solely from middle scallop (A2 and/or P2) was 31.07% (n = 394). If a nonsignificant jet from other locations was also accepted, the incidence of MR jet derived from mainly the middle scallop (A2 and/or P2) was 52.10% (n = 659). For MVP patients with MR R + 3, the conservative therapy group had higher mortality than the early surgery group (31.45% vs. 5.25%, P < 0.001) after 4.5 ± 1.0 years of follow-up, multiple analysis showed that surgical treatment (hazard ratio [HR]: 0.202, P < 0.001), systolic pulmonary artery pressure of o60 mmHg (HR: 6.816, P < 0.001), age of ≥ 60 years (HR: 3.838, P < 0.001), and pericardial effusion (HR: 1.915, P = 0.003) were independent predictors of all-cause mortality. Conclusions: In patients with MVP, one-fifth leaflet prolapse located solely in P2 and one-half of MR jet derived from the middle scallop were anatomically eligible for transcatheter chordal repair and edge-to-edge repair therapy, respectively. Initial conservative therapy, pericardial effusion, pulmonary hypertension, and advanced age were independent predictors of a higher mortality rate in MVP patients with MR severity of ≥ 3+.

Objectives Barlow´s disease represents a wide spectrum of mitral valve pathologies associated with regurgitation (MR), excess leaflet tissue, and prolapse. Repair strategies range from complex repairs with annuloplasty plus neochords through resection to annuloplasty-only. The latter requires symmetric prolapse patterns and central regurgitant jets. We aimed to assess repair success and durability, survival, and intraoperative outcomes with symmetric and asymmetric Barlow’s disease. Methods Between 09/10 and 03/20, 103 patients (of 1939 with mitral valve surgery) presented with Barlow´s disease. All received surgery through mini-thoracotomy with annuloplasty plus neochords (n = 71) or annuloplasty-only (n = 31). One valve was replaced for endocarditis (repair rate: 99%). Results Annuloplasty-only patients were older (64 ± 16 vs. 55 ± 11 years, p = 0.008) and presented with higher risk (EuroSCORE II: 4.2 ± 4.9 vs. 1.6 ± 1.7, p = 0.007). Annuloplasty-only patients had shorter cross-clamp times (53 ± 18 min vs. 76 ± 23 min, p p p = 0.455). In three patients, annuloplasty-only caused intraoperative systolic anterior motion (SAM), which was fully resolved by neochords to the posterior leaflet. There were no conversions to sternotomy or deaths at 30-days. Three patients required reoperation for recurrent MR (at 25 days, 2.8 and 7.8 years). At the latest follow-up, there was no MR in 81.4%, mild in 14.7%, and moderate in 2.9%. Three patients died due to non-cardiac reasons. Surviving patients report the absence of relevant symptoms. Conclusions Minimally-invasive Barlow’s repair is safe with good durability. Annuloplasty-only may be a simple solution for complex but symmetric pathologies. However, it may carry an increased risk of intraoperative SAM.

BACKGROUND: Cardiac fibrosis in mitral valve prolapse (MVP) is implicated in the development of sudden cardiac death (SCD); however, the pattern remains poorly characterized. OBJECTIVE: The purpose of this study was to systematically quantify left and right ventricular fibrosis in individuals with isolated MVP and SCD (iMVP-SCD), whereby other potential causes of death are excluded, compared to a control cohort. METHODS: Individuals with iMVP-SCD were identified from the Victorian Institute of Forensic Medicine, Australia, and matched for age, sex, and body mass index to control cases with noncardiac death. Cardiac tissue sections were analyzed to determine collagen deposition in the left ventricular free wall (anterior, lateral, and posterior portions), interventricular septum, and right ventricle. Within the iMVP-SCD cases, the endocardial-to-epicardial distribution of fibrosis within the left ventricle was specifically characterized. RESULTS: Seventeen cases with iMVP-SCD were matched 1:1 with 17 controls, yielding 149 samples and 1788 histologic regions. The iMVP-SCD group had increased left ventricular (anterior, lateral, and posterior; all P

Mitral commissural prolapse or flail, either isolated or combined with more extensive degenerative valve disease imposes several challenges both on its diagnosis and management whilst being a risk factor for valve reoperation after mitral valve repair. Accurate identification of the prolapsing segment is often not feasible with transthoracic 2D echocardiography, with transesophageal 3D imaging then required for correct diagnosis and surgical planning. Various surgical techniques employed alone or in combination, have yielded good results in the repair of commissural prolapse. Herein, we analyze the specific characteristics of commissural disease focusing our attention on 2D and 3D echocardiographic findings and we briefly comment on techniques employed for surgical correction of the disease.

The aim. The aim of the study was to conduct a comparative analysis of fibroblast growth factor-2, the total content and fractions of sulfated glycosoaminoglycans as well as free and peptide-bounding oxyproline as the markers of connective tissue metabolism in young patients with mitral valve prolapse, type 1 diabetes mellitus and the combination of both indications. Materials and methods. 93 patients between 19 and 33 years old with either mitral valve prolapse, or type 1 diabetes or the combination of both indications were examined. Group 1 was represented by 36 patients with the monomorbid type 1 diabetes mellitus. Group 2 consisted of 33 patients with type 1 diabetes mellitus and mitral valve prolapse. The comparison group included 24 people with diagnosed mitral valve prolapse. The concentration of fibroblast growth factor-2 in blood plasma was determined by the enzyme immunoassay using a Quantikine reagent kit (Human FGF basic Immunoassay), manufactured by R&D Systems, Inc. (USA) and expressed in pg/ml. The total content and fractions of glycosaminoglycans in blood serum were determined by the method of N. G. Stern et al. and expressed in units of optical density. The level of free and peptide-bounding oxyproline in blood serum were determined by the method of P. N. Sharaev and expressed in µmol/L. Results. Fibroblast growth factor-2 was higher in group 2, where it was 23.7±0.25 pg/ml compared to the control group – 14.20±0.22 pg/ml (p

In order to study the influence of the main fibroblast growth factor on the indicators of the state of the main substance of the extracellular matrix, a comparative analysis of their levels in the blood serum was carried out in persons with mitral valve prolapse and type 1 diabetes mellitus. The data obtained showed that the highest level of FGF-2 was observed in patients with a combination of MVP and type 1 diabetes mellitus compared with isolated MVP and diabetes, the control group (p

Objectives This study hypothesized that left ventricular (LV) enlargement in Barlow disease can be explained by accounting for the total volume load that consists of transvalvular mitral regurgitation (MR) and the prolapse volume. Background Barlow disease is characterized by long prolapsing mitral leaflets that can harbor a significant amount of blood—the prolapse volume—at end-systole. The LV in Barlow disease can be disproportionately enlarged relative to MR severity, leading to speculation of Barlow cardiomyopathy. Methods Cardiac magnetic resonance (CMR) was used to compare MR, prolapse volume, and heart chambers remodeling in patients with Barlow disease (bileaflet prolapse [BLP]) and in single leaflet prolapse (SLP). Results A total of 157 patients (81 with BLP, 76 with SLP) were included. Patients with SLP were older and more had hypertension. Patients with BLP had more heart failure. Indexed LV end-diastolic volume was larger in BLP despite similar transvalvular MR. However, the prolapse volume was larger in BLP, which led to larger total volume load compared with SLP. Increasing tertiles of prolapse volume and MR both led to an incremental increase in LV end-diastolic volume in BLP. Using the total volume load improved the correlation with indexed LV end-diastolic volume in the BLP group, which closely matched that of SLP. A multivariable model that incorporated the prolapse volume explained left heart chamber enlargement better than a MR-based model, independent of prolapse category. Conclusions The prolapse volume is part of the total volume load exerted on the LV during the cardiac cycle and could help explain the disproportionate LV enlargement relative to MR severity noted in Barlow disease.