Mitral Annular Disjunction-A New Disease Spectrum.

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.

Mitral annular disjunction in patients with primary severe mitral regurgitation and mitral valve prolapse.

BACKGROUND: Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus which is often associated with mitral leaflet prolapse. However, few reports have described mitral annular disjunction in mitral valve prolapse (MVP). This study aimed to investigate the characteristics of mitral annular disjunction in patients with severe mitral regurgitation (MR) caused by MVP. METHODS: We reviewed 185 consecutive patients with severe MR caused by fibroelastic deficiency (FED) and Barlow's syndrome from March 2009 to December 2010. The upper limit of the disjunction was defined at the level of the posterior scallop's insertion into the left atrial wall, whereas the lower limit was defined at the level of the left atrium's connection to the ventricular myocardium. The distance between the two levels was called mitral annular disjunction. Prolapse sites in FED patients were categorized into anterior leaflet, posterior leaflet, and commissure groups. Patients with a disjunction distance of ≥2 mm were diagnosed with mitral annular disjunction. RESULTS: Annular disjunction was found in 45 patients (24%). Among them, the most common site of prolapse was the posterior leaflet (n = 35, 77.8%). During a median follow-up of 20.3 years, arrhythmic events and sudden death occurred in seven patients (3.8%). CONCLUSIONS: Mitral annular disjunction was detected in 24% of patients with severe MR and in 90% of the patients with Barlow's syndrome. There were significant differences at its sites of prolapse in FED patients. The presence and site of prolapse with mitral annular disjunction should be actively determined in FED patients.

Significance of isolated papillary muscle hypertrophy: A comparison of left ventricular hypertrophy diagnosed using electrocardiography vs echocardiography.

AIMS: The clinical significance of papillary muscle hypertrophy and its electrocardiography (ECG) findings has not been fully elucidated. This study aimed to investigate the relationship between ECG findings and papillary muscle hypertrophy and to confirm the importance of papillary muscle measurements on transthoracic echocardiography (TTE). METHODS AND RESULTS: Of the 237 consecutive patients who showed left ventricular hypertrophy (LVH) pattern on a 12-lead ECG, TTE data were available for 101 patients. The patients were divided into the two groups according to the presence (ECG-LVH [strain pos] group, n = 60) or absence (ECG-LVH [strain neg] group, n = 41) of LV strain pattern (study 1). The prevalence of papillary muscle hypertrophy using TTE was significantly higher in the ECG-LVH (strain neg) group than in the ECG-LVH (strain pos) group (P = .0002). Of the 42 cases with papillary muscle hypertrophy, 5 cases (12%) showed isolated papillary muscle hypertrophy with normal geometry. ECG data were prospectively analyzed for 36 patients who were diagnosed with papillary muscle hypertrophy by TTE (study 2). The prevalence of LV strain pattern was significantly higher in patients with LV wall hypertrophy than in those without LV wall hypertrophy (P = .04). Of the 25 cases with papillary muscle hypertrophy, 6 cases (24%) showed normal geometry and 4 cases (16%) showed ECG abnormality. CONCLUSIONS: Isolated papillary muscle hypertrophy can cause ECG abnormalities such as LV high voltage and LV strain pattern. Particular attention must be paid to the papillary muscle during echocardiographic examinations.

Primary and metastatic cardiac tumors: echocardiographic diagnosis, treatment and prognosis in a 15-years single center study.

BACKGROUND: The frequency of primary cardiac tumors is rare at about 0.3% by autopsy. Our objective was to investigate the characteristics and locations of cardiac tumors and to provide a prognostic analysis in our hospital. METHODS: We collected data on 95 patients with echocardiographic diagnosis or detection of cardiac tumors in a prospective analysis from 1999 to 2014. The median follow-up period was 43 months (0.5-183 months). RESULTS: The subjects included 56 men and 39 women with a mean age of 65 years. Clinical diagnosis revealed primary tumors in 61 patients (64%) and secondary metastatic tumors in 34 patients (36%). In the 61 patients, 41 patients (67%) underwent surgery and tissue samples were obtained. Of these 41 patients, benign tumors were found in 30 cases (73%). One patient (2%) was diagnosed with thrombus. Among the benign tumors, myxoma (67%) was the most common type followed by papillary fibroelastoma (23%). The most common site was the left atrium (35%) followed by the right atrium (25%). Primary malignant tumors were diagnosed in 10 cases (24%), including 6 angiosarcomas, 3 lymphomas, and 1 leiomyosarcoma. The diagnostic accuracy of echocardiography was 80%. The patients with benign tumors were all alive at the end of the follow-up period. In contrast, 7 patients with malignant tumors died (70%) (p < 0.0001). CONCLUSIONS: Our data is in line with previous literature. Our study also suggests the necessity of extending our knowledge of the characteristics of cardiac tumors for diagnosis.

The analysis of mitral annular disjunction detected by echocardiography and comparison with previously reported pathological data.

BACKGROUND: Mitral annular disjunction is a structural abnormality of the mitral annulus fibrosus and is pathologically defined by a separation between the atrial wall-mitral valve junction and the left ventricular attachment. Mitral annular disjunction can cause hypermobility of the mitral valve apparatus and is often associated with mitral valve prolapse (MVP). The aim of this study was to investigate the frequency and characteristics of mitral annular disjunction in the patients referred to an echocardiography laboratory and to compare these with previously reported pathological data. METHODS AND RESULTS: We retrospectively studied 1439 patients (mean age 65 ± 17 years, 58% male) referred to our echocardiography laboratory from 6 January 2014 to 31 March 2014. The echocardiographic parameters were compared between the patients with and without mitral annular disjunction. There were 125 cases (8.7%) with mitral annular disjunction, of which 15 (12%) also had MVP. The number of MVP patients in the group with mitral annular disjunction was significantly larger than in the group without mitral annular disjunction (p < 0.0001). The grade of mitral regurgitation was not significantly different between the two groups. CONCLUSIONS: Mitral annular disjunction was detected not only in patients with a myxomatous mitral valve but also in normal cases. The number of MVPs was significantly larger in patients with mitral annular disjunction than patients without mitral annular disjunction. Further investigation is needed to clarify the clinical significance of the mitral annular disjunction detected by routine echocardiography.

A rare cardiac tumor: Bronchogenic cyst of interatrial septum.

Intracardiac bronchogenic cysts are uncommon congenital tumors, which rarely become symptomatic. We describe a rare case of bronchogenic cyst in the atrioventricular node. A 36-year-old man with third-degree atrioventricular block was referred to our hospital. Transthoracic echocardiography revealed a cystic mass at the right atrial aspect of the low interatrial septum. He underwent surgical resection of the mass, and a permanent epicardium pacemaker was implanted. His postoperative course was uneventful. Microscopic examination showed a cyst surrounded by ciliated columnar epithelium and partially smooth muscle, and the histopathological diagnosis was bronchogenic cyst.

Associations of residual left ventricular and left atrial remodeling with clinical outcomes in patients after aortic valve replacement for severe aortic stenosis.

BACKGROUND: Aortic valve replacement (AVR) is currently the standard therapy for severe aortic stenosis (AS), and regression of left ventricular (LV) hypertrophy after AVR has been reported. However, data regarding a temporal relation between LV mass and left atrial (LA) volume are limited, and their prognostic impacts have not been fully elucidated. We aimed to clarify the temporal patterns of LA and LV reverse remodeling and their associations with clinical outcomes. METHODS: We retrospectively reviewed 198 consecutive patients who underwent AVR for severe AS. After excluding patients with prior cardiac surgery, atrial fibrillation, concomitant moderate to severe aortic regurgitation, or concurrent mitral valve surgery, 83 patients with echocardiographic LV mass index (LVMI) and LA volume index (LAVI) data before and 1 year after AVR were eligible for the outcome analysis and 29 patients with these 2 measures before surgery, 1 month, 1 year, and 3 years after surgery were eligible for the analysis of time-dependent change of LVMI and LAVI. RESULTS: Significant reductions in LVMI and LAVI (both p<0.001) after surgery were observed over time. LA dilatation improved and reached a plateau 1 month after surgery, whereas LV hypertrophy improved more gradually and reached a plateau at 1 year. The presence of both LV hypertrophy and LA dilatation 1 year after surgery was associated with significantly higher mortality (patients with both conditions vs. patients with neither or one condition=22.6% vs. 7.3% at 3 years; p=0.031) and major adverse cardiac and cerebrovascular events (38.9% vs. 12.6% at 3 years; p=0.021). CONCLUSIONS: LA reverse remodeling occurred rapidly after AVR for severe AS, and regression of LV hypertrophy was more gradual. The presence of both residual LV hypertrophy and LA dilatation 1 year after AVR was associated with poor long-term outcomes.

Reversible severe mitral regurgitation caused by systolic anterior motion of the mitral valve in the absence of left ventricular hypertrophy: A case report.

A 67-year-old woman with exertional dyspnea was referred to our hospital. Transthoracic echocardiography revealed severe mitral regurgitation and significant left ventricular (LV) outflow tract obstruction due to prominent systolic anterior motion (SAM) of the mitral valve without LV hypertrophy. Oral bisoprolol remarkably attenuated SAM. Two- or three-dimensional echocardiographic analysis demonstrated the elongation of anterior and posterior mitral leaflets and interventricular septum (IVS) bulging due to narrow aorto-mitral angle. In the present case, elongation of mitral leaflet and hyperkinetic motion of left ventricle, and IVS bulging due to narrow aorto-mitral angle possibly play important roles in the development of SAM. .

Mitral annular morphology in mitral valve disease with three-dimensional transesophageal echocardiography.

BACKGROUND AND AIM OF THE STUDY: Three-dimensional (3D) transesophageal echocardiography (TEE) is useful for the quantification of mitral valve structures. The study aim was to investigate, in quantitative manner, any differences in mitral valve anatomy among patients with mitral valve prolapse (MVP) or functional mitral regurgitation (FMR), compared to normal control subjects. METHODS: 3D-TEE was performed in 20 MVP patients, 10 FMR patients and in 15 control subjects. Analyses of the full-volume 3D mitral valve data sets were performed offline, using Q-Lab software. RESULTS: Distinctive patterns were identified in annular geometric changes in normal subjects compared to patients with MVP or FMR. Patients with FMR showed significant annular anterior to posterior dilatation (34.6 +/- 8.3 mm versus 28.4 +/- 2.9 mm, p < 0.04: FMR versus control), whereas in patients with MVP dilatation in the anterolateral to posteromedial diameter was more prominent (41.0 +/- 5.9 mm versus 36.6 +/- 2.4 mm, p < 0.03; MVP versus control). CONCLUSION: 3D-TEE represents a useful method for the evaluation of mitral valve geometry.

Timing of valve repair for severe degenerative mitral regurgitation and long-term left ventricular function.

OBJECTIVES: Current guidelines recommended surgery for patients with severe degenerative mitral regurgitation (MR) when specific left ventricular (LV) dimensions or ejection fraction (EF) are reached, based on previous postoperative survival studies. The aim of this study was to evaluate the incidence and predictors of long-term postoperative LV dysfunction, and investigate the preoperative parameters necessary to maintain or recover long-term LV function in the era of mitral valve (MV) repair. METHODS: We retrospectively reviewed 473 consecutive patients undergoing MV repair for severe degenerative MR for whom both preoperative and 3-year postoperative echocardiographic data were available in our institution. Preoperative and 3-year postoperative echocardiographic data and clinical outcomes were evaluated. RESULTS: Receiver operating characteristic analysis identified preoperative LVEF 63% or less (area under curve [AUC], 0.725; P<.001) and LV end-systolic dimension (ESD) 39 mm or greater (AUC, 0.724; P<.001) as cut-off values for predicting LVEF less than 50% 3 years after surgery. On multivariate analysis, both preoperative LVEF and LVESD were not significant predictors of 3-year postoperative LV dysfunction among patients with preoperative LVEF greater than 63% and LVESD less 39 mm, whereas preoperative LVESD (odds ratio [OR], 2.22; P=.004), higher age (OR, 1.03; P=.04), and atrial fibrillation (OR, 2.68; P=.01) were independent predictors among patients with preoperative LVEF 63% or less or LVESD 39 mm or greater. CONCLUSIONS: Early MV repair with LVEF greater than 63% and LVESD less than 39 mm preserved long-term postoperative LV function well, and smaller preoperative LVESD was associated with long-term LV function recovery, even in patients with preoperative LV dysfunction.

Interpapillary muscle distance independently affects severity of functional mitral regurgitation in patients with systolic left ventricular dysfunction.

OBJECTIVES: Surgical papillary muscle (PM) imbrication has been reported to be effective to relieve leaflet tethering in patients with functional mitral regurgitation (FMR). However, the mechanism that contributes to improvement of FMR by shortening the interpapillary muscle distance (IPMD) has not been well investigated. The purpose of the present study was to investigate whether IPMD can affect MR severity independently of PM tethering distance in patients with left ventricular dysfunction (LVD) using multislice computed tomography. METHODS: We analyzed volumetric multislice computed tomography images of mitral apparatus in 83 patients with LVD (ejection fraction <50%): 37 patients with FMR and 46 patients without FMR. By using the original software, we assessed the 3-dimensional geometry of mitral apparatus including IPMD, tethering distances, and mitral tenting volume at end-systole. The severity of FMR was assessed using vena contracta (VC) width by 2-dimensional echocardiography. RESULTS: Posterior and anterior tethering distance and IPMD were increased significantly in patients with FMR than in those without FMR. Patients with IPMD in the highest tertile had a significantly higher degrees of MR (mean ± standard error VC width, 4.5 ± 0.3 mm) compared with patients in the lowest and the middle tertiles, adjusting for PM tethering distance (mean ± standard error VC width, 3.0 ± 0.4 and 2.9 ± 0.3 mm; P < .001, respectively). Multivariate analysis showed that anteroposterior annular diameter and IPMD were the strongest determinants of FMR severity and mitral tenting volume. CONCLUSIONS: IPMD, which affects leaflet tethering independently of PM tethering distance, was the major determinant of mitral tenting volume and FMR severity in patients with LVD.

Prognostic value of sustained elevated C-reactive protein levels in patients with acute aortic intramural hematoma.

OBJECTIVES: The appropriate management of aortic intramural hematoma is still controversial, because a variety of aortic events can arise during follow-up in some patients. However, simplified identification of these patients remains challenging. The present study aimed to determine the prognostic significance of serial C-reactive protein measurements for the prediction of adverse events in patients with acute aortic intramural hematoma. METHODS: A total of 180 patients with aortic intramural hematoma were retrospectively reviewed. The C-reactive protein data were obtained at admission and 2 days, 1 week, and 2 weeks from the onset, and the maximum value was obtained during the acute phase. Adverse aorta-related events were defined by a composite of aortic rupture, aortic aneurysm, and surgical or endovascular aortic repair. RESULTS: The C-reactive protein value was 3.0 ± 4.6, 8.7 ± 5.9, 9.0 ± 5.5, and 5.7 ± 4.5 mg/dL on admission and 2 days, 1 week, and 2 weeks from the onset, respectively. The maximal value of C-reactive protein was 12.4 ± 6.3 mg/dL at a mean of 4 days from the onset. Patients with elevated C-reactive protein levels (≥7.2 mg/dL) at 2 weeks had significantly greater rates of aorta-related events (P < .001). On multivariate analysis, an elevated C-reactive protein level at 2 weeks (hazard ratio, 3.16; P < .001) and the development of an ulcer-like projection (hazard ratio, 2.68; P = .002) were independent predictors of adverse aorta-related events. In addition, an elevated C-reactive protein level at 2 weeks had incremental value compared with the development of an ulcer-like projection (chi-square, 16.94 for ulcer-like projection only vs 34.32 with the addition of C-reactive protein at 2 weeks, P < .001). CONCLUSIONS: C-reactive protein was a simple and useful marker providing incremental prognostic information compared with the development of an ulcer-like projection in patients with aortic intramural hematoma.

Sex-based differences in clinical practice and outcomes for Japanese patients with acute myocardial infarction undergoing primary percutaneous coronary intervention.

BACKGROUND: Limited data are available for sex-based differences in Japanese patients with acute myocardial infarction (AMI) undergoing primary percutaneous coronary intervention (PCI). METHODS AND RESULTS: The study patients comprised 1,197 women and 3,182 men who underwent primary PCI for AMI in 2005-2007. Compared with the men, the women were significantly older, and had significantly longer onset-to-balloon time and lower rate of follow-up coronary angiography. In-hospital mortality was higher among women than men (8.7% vs. 4.9%, P<0.001). Although the cumulative incidence of all-cause death at 3 years was also higher for women (17.7% vs. 10.7%, P<0.001), the adjusted risk for all-cause death was comparable [hazard ratio (HR, women vs. men)=0.94, 95% confidence interval (CI): 0.71-1.24, P=0.66]. The incidence (12.1% vs. 12.4%, P=0.77) and the adjusted risk (HR=0.99, 95% CI 0.78-1.24, P=0.92) for any clinically-driven coronary revascularization were both comparable. However, regarding any non-clinically-driven coronary revascularization, the incidence (19.6% vs. 27.8%, P<0.001) and the adjusted risk (HR=0.79, 95% CI 0.65-0.95, P=0.012) were both lower in women relative to men. CONCLUSIONS: In current Japanese clinical practice for AMI, onset-to-balloon time was significantly longer in women than in men. Female sex was associated with lower follow-up coronary angiography rate and lower incidence of any non-clinically-driven coronary revascularization, whereas the incidence of any clinically-driven coronary revascularization was comparable between the sexes.

Progression of left main coronary artery disease 3 years after Bentall operation in a young female with Marfan syndrome.

A 39-year-old woman with Marfan syndrome presented to our hospital with chest oppression on effort. She underwent aortic root remodeling combined with aortic valve replacement 14 years ago and Bentall operation for enlargement of remaining native Valsalva sinus 3 years ago. A coronary computed tomography and a coronary angiography showed left main coronary artery stenosis, which was subsequently treated with percutaneous coronary intervention using a bare-metal stent. Follow-up coronary angiography performed 1 year after stenting revealed no restenosis.

Impact of aortic regurgitation on the prognosisof severe aortic stenosis.

OBJECTIVE: Many patients with aortic stenosis (AS) have coexisting aortic regurgitation (AR). However, few data exist regarding its clinical significance and prognostic value. The aim of this study was to examine the effect of concomitant significant AR on clinical outcomes in patients with non-surgically treated severe AS. DESIGN: A single centre, retrospective cohort study. PATIENTS AND METHODS: We retrospectively reviewed 306 consecutive patients (age, 72±11 years) with severe AS in whom non-surgical management was primarily planned at our institution between January 1999 and December 2011. There were 74 patients with moderate or severe AR (ASR) and 232 patients without significant AR (isolated AS). Clinical outcomes were compared between the two groups. MAIN OUTCOME MEASURES: All-cause mortality and valve-related events, were defined by a composite of cardiac death and hospitalisation because of heart failure. RESULTS: The mean follow-up period was 4.5±3.3 years. Although the overall survival was comparable between the groups (p=0.07), the event-free survival, defined as survival without cardiac death or hospitalisation because of heart failure, was significantly worse in ASR than in isolated AS (p=0.02). Concomitant AR was an independent predictor of adverse events in patients with severe AS (HR, 2.10; p=0.003). Among patients who did not eventually undergo aortic valve replacement, ASR was associated with significantly worse survival and event-free survival than isolated AS (p=0.002 and p=0.03, respectively). CONCLUSIONS: Concomitant AR might worsen the prognosis of severe AS. Greater consideration of surgery might be beneficial in patients with ASR.

Early surgery for asymptomatic mitral regurgitation: importance of atrial fibrillation.

BACKGROUND AND AIM OF THE STUDY: It remains controversial whether early mitral valve (MV) repair should be performed for severe degenerative mitral regurgitation (MR) without symptoms, left ventricular (LV) dilatation or dysfunction, atrial fibrillation (AF) or pulmonary artery hypertension (PH), even at experienced surgical centers. The study aim was to reconsider the optimal timing of intervention for asymptomatic patients with severe degenerative MR at experienced surgical centers. METHODS: Clinical outcomes were reviewed retrospectively for 298 consecutive asymptomatic patients (mean age 57 +/- 12 years) who underwent MV surgery for degenerative MR. The patients were allocated to two groups based on the following comorbid conditions: LVEF < or = 60%, LV end-systolic dimension 40 mm, AF, and PH. Group A comprised 122 patients with none of these conditions, while group B comprised 176 patients with any one of the conditions. The clinical outcomes were compared between the two groups at a mean of 7.0 +/- 4.5 years after surgery. RESULTS: MV repair had been attempted in all patients, with a success rate of 100%. At 10 years, survival among group B patients was poorer than in group A (93% and 81%, respectively; p = 0.02), and there was a lower freedom from valve-related events (89% and 71%, respectively; p < 0.01). The independent predictors of valve-related events were preoperative AF (hazard ratio 3.34; p < 0.001) and age > 60 years (hazard ratio 2.50; p < 0.01). CONCLUSION: Early MV repair is a reasonable option in asymptomatic patients, while preoperative AF may be a more appropriate predictor of an adverse outcome than LV function, as is currently recommended.

Mitral valve repair for double-orifice mitral valve with flail leaflet: the usefulness of real-time three-dimensional transesophageal echocardiography.

We describe a 66-year-old man who required an operation for severe mitral regurgitation associated with a double-orifice mitral valve. Real-time 3-dimensional transesophageal echocardiography clearly demonstrated a double-orifice mitral valve with a central fibrous bridge. A flail posterior leaflet was observed on the anterolateral mitral valve orifice. Mitral valve repair using P1 triangular resection, anterolateral commissure plication, and ring annuloplasty with Duran band (Medtronic, Minneapolis, MN) was successfully performed. Postoperative real-time 3-dimensional transesophageal echocardiography demonstrated a double-orifice mitral valve without regurgitation or stenosis.

Left ventricular systolic dyssynchrony index by three-dimensional echocardiography in patients with decreased left ventricular function: comparison with tissue Doppler echocardiography.

BACKGROUND: Three-dimensional echocardiography (3DE) can simultaneously assess left ventricular (LV) regional systolic motion and global LV mechanical dyssynchrony. METHODS: We used 3DE to measure systolic dyssynchrony index (SDI) (standard deviation of the time from cardiac cycle onset to minimum systolic volume in 17 LV segments) in 100 patients and analyzed the association of SDI with other parameters for LV systolic function or dyssynchrony. Eighteen patients who underwent cardiac resynchronization therapy (CRT) were also evaluated at 6 months after CRT, and the association of baseline SDI and tissue Doppler imaging (TDI) dyssynchrony index (Ts-SD) with the change of LV end-systolic volume (ESV) analyzed. Ts-SD was calculated using the standard deviation of the time from the QRS complex to peak systolic velocity. RESULTS: There was a significant inverse correlation between LVEF and SDI (r =-0.686, P < 0.0001). QRS duration was also significantly correlated to SDI (r = 0.407, P < 0.0001). There was a significant positive correlation between baseline SDI and the decrease in LVESV after CRT (r = 0.42). Baseline SDI was significantly greater in responders (10 patients) than in nonresponders (16.4 ± 5.1 vs. 7.9 ± 2.4%, P < 0.01), but there was no significant difference in Ts-SD. SDI > 11.9% predicted CRT response with a sensitivity of 90% and a specificity of 75%. CONCLUSIONS: SDI derived from 3DE is a useful parameter to assess global LV systolic dyssynchrony and predict responses to CRT.