Morphometric evaluation of the left atrioventricular valve complex and its clinical relations.

BACKGROUND: In this study, it was aimed to evaluate morphometrically and morphologically the left fibrous ring, mitral leaflets, tendinous cords, and papillary muscles, which are the components of the left atrioventricular valve complex (LAVC), and to reveal their clinical relationships. MATERIALS AND METHODS: A total of 120 human hearts were examined at the Forensic Medicine Institute. Cases aged 30 years and older, less than 24 hours after their death, were included in the study. Heart length, width, height/width ratio, anteroposterior and mediolateral diameters of the annulus, annular area, length and width of leaflets, number and attachment sites of tendinous cords, number, shape, length, the width of papillary muscles, and distances to various points were recorded to determine their spatial configurations. As well as the measurement data of LAVC components in cases with and without cardiovascular disease (CVD), the relationships of these data with the demographic characteristics of the cases are also explained. RESULTS: In the diagnostic performance test (ROC analysis), it was determined that body mass index (> 26.7), heart weight (> 414 g), heart height/width ratio (≤ 1.24), mitral valve width (> 99.96 mm), left ventricular wall thickness (> 15.08 mm), annular area (> 619.37 mm²) and mediolateral diameter of the annulus (> 30.71 mm) are important diagnostic criteria in determining CVD if they are outside the specified reference values. CONCLUSIONS: This study provides anatomical information about LAVC, as well as recommendations for diagnosis and surgical treatment planning. We therefore believe that our findings will be useful to clinicians.

Preclinical Study of Pulsed Field Ablation of Difficult Ventricular Targets: Intracavitary Mobile Structures, Interventricular Septum, and Left Ventricular Free Wall.

BACKGROUND: Endocardial catheter-based pulsed field ablation (PFA) of the ventricular myocardium is promising. However, little is known about PFA's ability to target intracavitary structures, epicardium, and ways to achieve transmural lesions across thick ventricular tissue. METHODS: A lattice-tip catheter was used to deliver biphasic monopolar PFA to swine ventricles under general anesthesia, with electroanatomical mapping, fluoroscopy and intracardiac echocardiography guidance. We conducted experiments to assess the feasibility and safety of repetitive monopolar PFA applications to ablate (1) intracavitary papillary muscles and moderator bands, (2) epicardial targets, and (3) bipolar PFA for midmyocardial targets in the interventricular septum and left ventricular free wall. RESULTS: (1) Papillary muscles (n=13) were successfully ablated and then evaluated at 2, 7, and 21 days. Nine lesions with stable contact measured 18.3±2.4 mm long, 15.3±1.5 mm wide, and 5.8±1.0 mm deep at 2 days. Chronic lesions demonstrated preserved chordae without mitral regurgitation. Two targeted moderator bands were transmurally ablated without structural disruption. (2) Transatrial saline/carbon dioxide assisted epicardial access was obtained successfully and epicardial monopolar lesions had a mean length, width, and depth of 30.4±4.2, 23.5±4.1, and 9.1±1.9 mm, respectively. (3) Bipolar PFA lesions were delivered across the septum (n=11) and the left ventricular free wall (n=7). Twelve completed bipolar lesions had a mean length, width, and depth of 29.6±5.5, 21.0±7.3, and 14.3±4.7 mm, respectively. Chronically, these lesions demonstrated uniform fibrotic changes without tissue disruption. Bipolar lesions were significantly deeper than the monopolar epicardial lesions. CONCLUSIONS: This in vivo evaluation demonstrates that PFA can successfully ablate intracavitary structures and create deep epicardial lesions and transmural left ventricular lesions.

Automatic left ventricle volume and mass quantification from 2D cine-MRI: Investigating papillary muscle influence.

OBJECTIVE: Early detection of cardiovascular diseases is based on accurate quantification of the left ventricle (LV) function parameters. In this paper, we propose a fully automatic framework for LV volume and mass quantification from 2D-cine MR images already segmented using U-Net. METHODS: The general framework consists of three main steps: Data preparation including automatic LV localization using a convolution neural network (CNN) and application of morphological operations to exclude papillary muscles from the LV cavity. The second step consists in automatically extracting the LV contours using U-Net architecture. Finally, by integrating temporal information which is manifested by a spatial motion of myocytes as a third dimension, we calculated LV volume, LV ejection fraction (LVEF) and left ventricle mass (LVM). Based on these parameters, we detected and quantified cardiac contraction abnormalities using Python software. RESULTS: CNN was trained with 35 patients and tested on 15 patients from the ACDC database with an accuracy of 99,15 %. U-Net architecture was trained using ACDC database and evaluated using local dataset with a Dice similarity coefficient (DSC) of 99,78 % and a Hausdorff Distance (HD) of 4.468 mm (p < 0,001). Quantification results showed a strong correlation with physiological measures with a Pearson correlation coefficient (PCC) of 0,991 for LV volume, 0.962 for LVEF, 0.98 for stroke volume (SV) and 0.923 for LVM after pillars' elimination. Clinically, our method allows regional and accurate identification of pathological myocardial segments and can serve as a diagnostic aid tool of cardiac contraction abnormalities. CONCLUSION: Experimental results prove the usefulness of the proposed method for LV volume and function quantification and verify its potential clinical applicability.

Corrigendum: Anomalous papillary muscle insertion into the mitral valve leaflet in hypertrophic obstructive cardiomyopathy: a lip nevus sign in echocardiography.

[This corrects the article DOI: 10.3389/fcvm.2023.1292142.].

Resection of hypertrophic papillary muscles and mitral valve replacement in a patient with midventricular hypertrophic obstructive cardiomyopathy - a new approach.

Midventricular hypertrophic obstructive cardiomyopathy (HOCM) is characterized by hypertrophy of the interventricular septum (IVS) and - in rare cases - of the papillary muscles (PM), which subsequently can cause dynamic left ventricular outflow tract obstruction (LVOTO) and severe heart failure symptoms. We report on a rare case of a 44-year-old patient suffering from midventricular HOCM with hypertrophic anterolateral PM and an additional chorda between the PM and the IVS.We describe a new surgical approach via right anterolateral thoracotomy in 3-dimensional (3D) video-assisted minimal-invasive technique with resection of hypertrophic PMs as well as the entire mitral valve-apparatus and valve replacement avoiding septal myectomy and potentially associated complications. After an uneventful procedure clinical symptoms improved from NYHA III-IV at baseline to NYHA 0-I postoperatively and remained stable over a follow-up period of 24 months. Therefore, the presented technique may be considered as a new and alternative approach in patients with hypertrophic PMs and hypertrophic IVS as subtype of midventricular HOCM.

Anomalous papillary muscle insertion into the mitral valve leaflet in hypertrophic obstructive cardiomyopathy: a lip nevus sign in echocardiography.

Background: Anomalous papillary muscle (APM) insertion into the mitral valve leaflet is rare but clinically important in hypertrophic obstructive cardiomyopathy (HOCM). In this study, we report the detection rate of APM insertion into the mitral valve using preoperative imaging modalities and the surgical outcomes of the patients. Methods: By retrospectively reviewing the clinical records of patients with HOCM who underwent surgical treatment by a single operation group at our center from January 2020 to June 2023, patients with APM insertion into the mitral valve leaflet were identified. Baseline data, image characteristics, and surgical outcomes were analyzed. Results: The incidence of APM insertion into the mitral valve leaflet was 5.1% (8/157). The insertion site was located at A3 in six cases, which was more common than at A2 (n = 2). Preoperative echocardiography was used to identify two patients (25%) with APM insertion. We observed a particular echocardiographic feature for APM in HOCM patients, which was noted as a "lip nevus sign", with a higher detection rate (62.5%). All patients successfully underwent septal myectomy with concomitant APM excision or mitral valve replacement via the transaortic (n = 5) or transmitral (n = 3) approach. The mean age was 49.0 ± 17.4 years and seven patients (87.5%) were female. Interventricular septum thickness (17.0 mm vs. 13.3 mm, P = 0.012) and left ventricular outflow gradient (117.5 mmHg vs. 7.5 mmHg, P = 0.012) were significantly decreased after surgery. Residual outflow obstruction, systolic anterior motion, and ≥3+ mitral regurgitation were negative. During the follow-up of 26.2 ± 12.2 months, there were no reported operations, adverse events, mitral regurgitation aggravations, recurrences of outflow obstruction, or instances of SAM. Conclusions: Papillary muscles inserted into the mitral valve leaflet are a subtype of subvalvular malformation in HOCM that requires surgical correction. The lip nevus sign on echocardiography is a characteristic of APM insertion in HOCM and may improve the preoperative detection rate. Adequate myectomy with anomalous papillary muscle excision has achieved good results in reducing the outflow gradient and eliminating mitral regurgitation, with good outcomes at short-to-intermediate follow-up.

Assessment of the mechanism of mitral valve prolapse in children: An echocardiography study.

BACKGROUND: The high complexity of mitral valve anatomy and function in mitral valve prolapse (MVP) is not yet fully understood. OBJECTIVE: The purpose of this study was to analyze each part of the mitral valve apparatus in children to determine its impact on the presence of MVP and to assess the interaction between the coaptation length (CL) and mitral regurgitation severity. METHODS: We prospectively analyzed transthoracic echocardiograms of 60 patients with MVP (mean age 9.8 ± 3.1 years). We compared these patients with 60 control patients without disease. We determined length of leaflets, chordal length, tenting area, coaptation CL, the intrapapillary muscle distance (IPMD) and relation between CL and severity of mitral regurgitation (MR). RESULTS: For patients with MVP, the posterior mitral leaflet (PML) was significantly enlarged 13.9 ± 4.1 mm versus 10.7 ± 3.5 mm (p < .01), the primary chordal length was significantly decreased 15.4 ± 3.61 mm versus 17.6 ± 3.8 mm (p < .02), and IPMD was significantly greater 18.1 ± 2.7 mm versus 16.6 ± 4.3 mm (p < .03). The difference between CL for both the anterior and posterior mitral leaflets correlated positively with MR (r = .249, p < .05). A greater than 4 mm CL correlated with at least MR (sensitivity 100%, specificity 72%) and greater than 5 mm correlated with at least moderate MR (sensitivity 100%, specificity 60%). CONCLUSION: The majority of pediatric patients with mitral valve prolapse have structural abnormalities that are defined well by echocardiography. In addition to the presence of prolapse and regurgitation, routine assessment of leaflet length, thickness, chordal length and papillary muscle distance is fundamental for patients with MVP.

Combination of Intracardiac Echocardiography and Contact Force Sensing for Left Ventricular Papillary Muscle Arrhythmias.

OBJECTIVES: The catheter ablation of ventricular arrhythmias (VAs) arising from the left ventricular (LV) papillary muscles (PMs) is challenging. This study sought to address whether the combination of intracardiac echocardiography (ICE) and contact force sensing (CFS) can improve the acute and long-term ablation outcomes of left ventricular papillary muscle arrhythmias. METHODS AND RESULTS: From May 2015 to August 2022, a total of thirty-three patients underwent catheter ablation for LV PM arrhythmias: VAs were located in anterolateral PMs in 11 and posteromedial PMs in 22. A combination of intracardiac echocardiography (ICE) and contact force sensing (CFS) was used in 21 of the 33 procedures. A mean of 6.93 ± 4.91 for lesions was used per patient, comparable between the CFS/ICE and no ICE/CFS (4.90 ± 2.23 vs. 10.17 ± 5.89; p = 0.011). The mean CF achieved in the ICE/CFS group was 7.52 ± 3.31 g. Less X-ray time was used in the combination group (CFS/ICE: 165.67 ± 47.80 S vs. no ICE/CFS: 365.00 ± 183.73 S; p < 0.001). An acute success rate of 100% was achieved for the ICE/CFS group (n = 22) and 66.67% for the no ICE/CFS group (n = 8). VA recurrence at the 11.21 ± 7.21-month follow-up was 14.2% for the ICE/CFS group and 50% for the no ICE/CFS group (p = 0.04). No severe complications occurred in all patients. CONCLUSIONS: The combination of intracardiac echocardiography (ICE) and contact force sensing (CFS) could provide precise geometries of cardiac endocavitary structures and accurate contact information for the catheter during ablation, which improved acute and long-term ablation outcomes. The routine adoption of this strategy should be considered to improve the outcomes of LV PM VA ablation.

Papillary muscle abnormality as an electrocardiographic mimicker of apical hypertrophic cardiomyopathy: a case report.

Background: Apical hypertrophic cardiomyopathy (ApHCM) is often associated with characteristic giant T wave inversions (GNT) in precordial leads without septal Q waves and increased QRS voltage on 12-lead electrocardiograms (ECGs). However, these electrocardiographic findings are not specific to ApHCM and can be mimicked by papillary muscle abnormalities. Differentiation between the two is important as the disease course, treatment, and prognosis differ substantially. Case summary: We report a case report of two such patients both of which presented with abnormal ECGs concerning for ApHCM. Echocardiogram did not show characteristic findings of ApHCM. Cardiac magnetic resonance imaging (MRI) showed apically displaced, hypertrophied papillary muscles responsible for electrocardiographic abnormalities. Discussion: Papillary muscle abnormalities including hypertrophy and/or apical displacement can result in giant negative T wave and increased QRS voltage like those seen in ApHCM and should be considered especially in otherwise healthy individuals with normal or near-normal transthoracic echocardiograms. Role of cardiac MRI is critical in this context and is the imaging modality of choice for accurate diagnosis.

Abnormal Mechanics Relate to Myocardial Fibrosis and Ventricular Arrhythmias in Patients With Mitral Valve Prolapse.

BACKGROUND: The relation between ventricular arrhythmia and fibrosis in mitral valve prolapse (MVP) is reported, but underlying valve-induced mechanisms remain unknown. We evaluated the association between abnormal MVP-related mechanics and myocardial fibrosis, and their association with arrhythmia. METHODS: We studied 113 patients with MVP with both echocardiogram and gadolinium cardiac magnetic resonance imaging for myocardial fibrosis. Two-dimensional and speckle-tracking echocardiography evaluated mitral regurgitation, superior leaflet and papillary muscle displacement with associated exaggerated basal myocardial systolic curling, and myocardial longitudinal strain. Follow-up assessed arrhythmic events (nonsustained or sustained ventricular tachycardia or ventricular fibrillation). RESULTS: Myocardial fibrosis was observed in 43 patients with MVP, predominantly in the basal-midventricular inferior-lateral wall and papillary muscles. Patients with MVP with fibrosis had greater mitral regurgitation, prolapse, and superior papillary muscle displacement with basal curling and more impaired inferior-posterior basal strain than those without fibrosis (P<0.001). An abnormal strain pattern with distinct peaks pre-end-systole and post-end-systole in inferior-lateral wall was frequent in patients with fibrosis (81 versus 26%, P<0.001) but absent in patients without MVP with basal inferior-lateral wall fibrosis (n=20). During median follow-up of 1008 days, 36 of 87 patients with MVP with >6-month follow-up developed ventricular arrhythmias associated (univariable) with fibrosis, greater prolapse, mitral annular disjunction, and double-peak strain. In multivariable analysis, double-peak strain showed incremental risk of arrhythmia over fibrosis. CONCLUSIONS: Basal inferior-posterior myocardial fibrosis in MVP is associated with abnormal MVP-related myocardial mechanics, which are potentially associated with ventricular arrhythmia. These associations suggest pathophysiological links between MVP-related mechanical abnormalities and myocardial fibrosis, which also may relate to ventricular arrhythmia and offer potential imaging markers of increased arrhythmic risk.

Anatomical and biometric study of the mitral valve apparatus: application in valve repair surgery.

OBJECTIVE: Most mitral valve repair techniques provide excellent surgical results by removing regurgitation, but all of these techniques simultaneously reduce posterior valve mobility. A comprehensive biometric study of the mitral valve apparatus will provide landmarks that would help improve this posterior valve mobility. MATERIALS AND METHODS: Thirty one (31) human hearts have been studied, from 14 women and 17 men. The characteristics of the studied sample were analyzed descriptively. The difference in means of the variables between women and men were tested using a Student t test. Correlations between the different measures were determined by simple regression analysis. Mean values are shown with ± 1 standard deviation and the limit of significance was set at 0.05. RESULTS: The mean weight of the hearts was 275.3 ± 2.4 g. The anteroposterior diameter of the mitral annulus was 29.3 ± 1.22 mm, the intertrigonal distance was 25.2 ± 3.50 mm and the anterior leaflet to posterior leaflet ratio was 1.9 ± 0.10, the length of the chordae A2 = 19.4 ± 1.15 mm and P2 = 14.5 ± 0.85 mm. The length of the anterior papillary muscle averaged 30.9 ± 7.20 mm and that of the posterior one 30.0 ± 8.75 mm. The comparison of the different values measured between women and men showed no statistically significant difference (p > 0.05). There was no correlation between these different measured values (p > 0.05). CONCLUSION: A perfect knowledge of anatomy and biometry is therefore essential to offer alternative techniques that reproduce the real anatomy and physiology with a complete reconstruction of the mitral valve.

Apical papillary muscle displacement is a prevalent feature and a phenotypic precursor of apical hypertrophic cardiomyopathy.

AIMS: Papillary muscle (PM) abnormalities are considered part of the phenotypic spectrum of hypertrophic cardiomyopathy (HCM). The aim of this study was to evaluate the presence and frequency of PM displacement in different HCM phenotypes. METHODS AND RESULTS: We retrospectively analysed cardiovascular magnetic resonance (CMR) findings in 156 patients (25% females, median age 57 years). Patients were divided into three groups: septal hypertrophy (Sep-HCM, n = 70, 45%), mixed hypertrophy (Mixed-HCM, n = 48, 31%), and apical hypertrophy (Ap-HCM, n = 38, 24%). Fifty-five healthy subjects were enrolled as controls. Apical PM displacement was observed in 13% of controls and 55% of patients, which was most common in the Ap-HCM group, followed by the Mixed-HCM and Sep-HCM groups (respectively: inferomedial PM 92 vs. 65 vs. 13%, P < 0.001; anterolateral PM 61 vs. 40 vs. 9%, P < 0.001). Significant differences in PM displacement were found when comparing healthy controls with patients with Ap- and Mixed-HCM subtypes but not when comparing them with patients with the Sep-HCM subtype. T-wave inversion in the inferior and lateral leads was more frequent in patients with Ap-HCM (100 and 65%, respectively) when compared with Mixed-HCM (89 and 29%, respectively) and Sep-HCM (57 and 17%, respectively; P < 0.001 for both). Eight patients with Ap-HCM had prior CMR examinations because of T-wave inversion [median interval 7 (3-8) years], and in the first CMR study, none showed apical hypertrophy [median apical wall thickness 8 (7-9) mm], while all of them presented with apical PM displacement. CONCLUSION: Apical PM displacement is part of the phenotypic Ap-HCM spectrum and may precede the development of hypertrophy. These observations suggest a potential pathogenetic, mechanical link between apical PM displacement and Ap-HCM.

Irregular ventricular tachycardia originating from the moderator band.

Ventricular tachycardias (VT) may initially show beat to beat oscillations but rapidly stabilize into a regular tachycardia with a stable cycle length. A persistently irregular ventricular tachycardia is a rare phenomenon. We report a rare case of an "irregular" ventricular tachycardia with so pronounced oscillations in cycle length that it was initially misdiagnosed as atrial fibrillation with aberrant conduction. This ventricular tachycardia was incessant and resulted in a tachycardia induced cardiomyopathy refractory to several antiarrhythmic drugs. Mapping of the right ventricle demonstrated that the tachycardia had a focal origin in the moderator band close to its insertion into the anterior papillary muscle. Radiofrequency ablation eliminated the tachycardia with eventual normalization of left ventricular function. The moderator band and anterior papillary muscle of the right ventricle are known to be the source of short-coupled ventricular premature beats and regular ventricular tachycardias. However, an "irregular" ventricular tachycardia has not been previously reported to arise from these structures.

Papillary Muscle Involvement during Acute Myocardial Infarction: Detection by Cardiovascular Magnetic Resonance Using T1 Mapping Technique and Papillary Longitudinal Strain.

Papillary muscle (PPM) involvement in myocardial infarction (MI) increases the risk of secondary mitral valve regurgitation or PPM rupture and may be diagnosed using late gadolinium enhancement (LGE) imaging. The native T1-mapping (nT1) technique and PPM longitudinal strain (PPM-ls) have been used to identify PPM infarction (iPPM) without the use of the contrast agent. This study aimed to assess the diagnostic performance of nT1 and PPM-ls in the identification of iPPM. Forty-six patients, who performed CMR within 14-30 days after MI, were retrospectively enrolled: sixteen showed signs of iPPM on LGE images. nT1 values were measured within the infarcted area (IA), remote myocardium (RM), blood pool (BP), and anterolateral and posteromedial PPMs and compared using ANOVA. PPM-ls values have been assessed on cineMR images as the percentage of shortening between end-diastolic and end-systolic phases. Higher nT1 values and lower PPM-ls were found in infarcted compared to non-infarcted PPMs (nT1: 1219.3 ± 102.5 ms vs. 1052.2 ± 80.5 ms and 17.6 ± 6.3% vs. 21.6 ± 4.3%; p-value < 0.001 for both), with no significant differences between the nT1 of infarcted PPMs and IA and between the non-infarcted PPMs and RM. ROC analysis demonstrated an excellent discriminatory power for nT1 in detecting the iPPM (AUC = 0.874; 95% CI: 0.784-0.963; p < 0.001). nT1 and PPM-ls are valid tools in assessing iPPM with the advantage of avoiding contrast media administration.

Hypertrophic cardiomyopathy - phenotypic variations beyond wall thickness.

Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy (LVH) in the absence of another causal disease. Several morphologic and histologic changes have been described. Given the morbidity and mortality associated with HCM, understanding these anatomic variations is key to interpreting imaging. This is especially important since many patients exhibit these associated findings in the absence of LVH and prompt early detection of these variations may lead to early diagnosis and treatment. This article describes the appearance of morphologic variations seen in HCM beyond myocardial thickening including: papillary muscle and mitral valve variants, myocardial crypts, left ventricular myocardial bands, and dystrophic calcification related to increased wall tension.

Dark papillary muscles sign: a novel prognostic marker for cardiac magnetic resonance.

OBJECTIVES: The prognostic role of left ventricular (LV) papillary muscle abnormalities in patients with preserved LV systolic ejection fraction (LVEF) is unknown. We sought to evaluate the prognosis role of LV papillary muscle abnormalities by CMR in patients with ventricular arrhythmias, preserved LVEF with no cardiac disease. METHODS: A total of 391 patients with > 500/24 h premature ventricular complexes and/or with non-sustained ventricular tachycardia (NSVT), preserved LVEF, and no cardiac disease were enrolled. Different features of LV papillary muscles were considered: supernumerary muscles, papillary thickness, the attachment, late gadolinium enhancement (LGE). Dark-Paps was defined as end-systolic signal hypointensity of both papillary muscles in early post-contrast cine CMR images. Mitral valve prolapse, mitral annular disjunction (MAD), and myocardial LGE were considered. RESULTS: Dark-Paps was found in 79 (20%) patients and was more frequent in females. It was associated with higher prevalence of mitral valve prolapse and MAD. During a median follow-up of 2534 days, 22 hard cardiac events occurred. At Kaplan-Meier curve analysis, patients with Dark-Paps were at higher risk of events than those without (p < 0.0001). Dark-Paps was significantly associated with hard cardiac events in all the multivariate models. Dark-Paps improved prognostic estimation when added to NSVT (p = 0.0006), to LGE (p = 0.005) and to a model including NSVT+LGE (p = 0.014). Dark-Paps allowed a significant net reclassification when added to NSVT (NRI 0.30, p = 0.03), to LGE (NRI 0.25, p = 0.04), and to NSVT + LGE (NRI 0.32, p  = 0.02). CONCLUSIONS: In LV papillary muscles, Dark-Paps is a novel prognostic marker in patients with ventricular arrhythmias and preserved ejection fraction. KEY POINTS: • Papillary muscle abnormalities are seen in patients with ventricular arrhythmias and preserved left ventricular ejection fraction. • Early post-contrast hypointensity of papillary muscles in end-systolic cine images (Dark-Paps) is a novel prognostic marker in patients with ventricular arrhythmias and preserved ejection fraction. • Dark-Paps had an additive prognostic role over late gadolinium enhancement and non-sustained ventricular tachycardia.

Percutaneous transluminal septal myocardial ablation was effective in hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles: a case report.

Background: Abnormalities of the mitral subvalvular apparatus are not uncommon in hypertrophic obstructive cardiomyopathy (HOCM). Where invasive treatment is indicated in these patients to reduce left ventricular outflow tract (LVOT) obstruction, surgical myectomy with mitral valve repair is recommended. Case summary: In this report, we describe the case of a patient with HOCM and anomalous papillary muscle anatomy, successfully treated by percutaneous transluminal septal myocardial ablation (PTSMA). Discussion: PTSMA effectively reduced septal myocardial thickness and LVOT gradient, with only mild residual systolic anterior motion and mitral regurgitation despite anomalous papillary muscle anatomy. Upon careful anatomical evaluation, PTSMA may be a suitable therapeutic option for patients with LVOT obstruction and mitral valve abnormalities who are poor surgical candidates.

Apical Aneurysms and Mid-Left Ventricular Obstruction in Hypertrophic Cardiomyopathy.

BACKGROUND: Apical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%. OBJECTIVES: The authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms. METHODS: The authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities). RESULTS: There were 108 patients with a mean age of 57.4 ± 13.5 years; 40 (37%) were women. A total of 103 aneurysm patients (95%) had mid-LV obstruction with mid-LV complete systolic emptying. Of the patients with obstruction, 84% had a midsystolic Doppler signal void, a marker of complete flow cessation, but only 19% had Doppler systolic gradients ≥30 mm Hg. Five patients (5%) had relative hypokinesia in mid-LV without obstruction. Aneurysm size is not bimodal but appears distributed by power law, with large aneurysms decidedly less common. Comparing mid-LV obstruction aneurysm patients with all control groups, the short-axis (SAX) systolic areas were smaller (P < 0.007), the percent SAX area change was greater (P < 0.005), the papillary muscle (PM) areas were larger (P < 0.003), and the diastolic PM areas/SAX diastolic areas were greater (P < 0.005). Patients with aneurysms had 22% greater SAX PM areas compared with those with elevated LV velocities but no aneurysms (median: 3.00 cm2 [IQR: 2.38-3.70 cm2] vs 2.45 [IQR: 1.81-2.95 cm2]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying. CONCLUSIONS: The great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.

Catheter ablation of frequent monomorphic ventricular arrhythmias in Andersen-Tawil syndrome: case report and focused literature review.

BACKGROUND/PURPOSE: Andersen-Tawil syndrome type 1 is a rare autosomal dominant disease caused by a KCNJ2 gene mutation and clinically characterized by dysmorphic features, periodic muscular paralysis, and frequent ventricular arrhythmias (VAs). Although polymorphic and bidirectional ventricular tachycardias are prevalent, PVCs are the most frequent VAs. In addition, a "dominant" morphology with RBBB pattern associated with either superior or inferior axis is seen in most of the patients. Due to the limited efficacy of most antiarrhythmic drugs, catheter ablation (CA) is an alternative in patients with monomorphic VAs. Based on our experience, we aimed to review the arrhythmogenic mechanisms and substrates for VAs, and we analyzed the potential reasons for CA failure in this group of patients. METHODS: Case report and focused literature review. RESULTS: Catheter ablation has been reported to be unsuccessful in all of the few cases published so far. Most of the information suggests that VAs are mainly originated from the left ventricle and probably in the Purkinje network. Although identifying well-established and accepted mapping criteria for successful ablation of a monomorphic ventricular arrhythmia, papillary muscles seem not to be the right target. CONCLUSIONS: More research is needed to understand better the precise mechanism and site of origin of VAs in Andersen-Tawil syndrome patients with this particular "dominant" monomorphic ventricular pattern to establish the potential role of CA.