Mid-term outcomes of right ventricular papillary muscle approximation for severe functional tricuspid regurgitation.

OBJECTIVES: Recurrence of tricuspid regurgitation (TR) after tricuspid annuloplasty can occur in cases where a dilated right ventricle exists and subsequent leaflet tethering follows. We previously reported a new technique of the right ventricular papillary muscle approximation (RV-PMA) for functional TR associated with leaflet tethering. The objective of this study is to elucidate the mid-term outcomes and evaluate the durability of RV-PMA. METHODS: Between January 2014 and March 2023, we applied RV-PMA in 20 patients of advanced functional TR with severe leaflet tethering. The indication of the technique was severe TR with leaflet tethering height >8 mm, and/or a right ventricular end-diastolic diameter >45 mm. The patients were followed up with echocardiography before discharge and at annual interval thereafter. RESULTS: There was no perioperative mortality. In the echocardiography performed before discharge, TR was decreased to mild or less in 85%, and a significant improvement in right ventricular end-diastolic diameter and tethering height were achieved (53-45 mm and 11.1-4.4 mm, respectively). Furthermore, during the median 3-year follow-up period, TR was kept controlled mild or less in 80% of the cases. CONCLUSIONS: RV-PMA is considered to be a safe, effective and durable technique as an additional approach for tricuspid annuloplasty.

Interpapillary muscle distance independently predicts recurrent mitral regurgitation.

OBJECTIVE: Ischaemic secondary mitral regurgitation (ISMR) after surgery is due to the displacement of papillary muscles resulting from progressive enlargement of the left ventricle end-diastolic diameter (LVEDD). Our aim was to prove that if the interpapillary muscle distance (IPMD) is surgically stabilized, an increase in LVEDD will not lead to a recurrence of ischaemic mitral regurgitation (MR). METHODS: Ninety-six patients with ISMR, who underwent surgical revascularisation and annuloplasty, were randomly assigned in a 1:1 ratio to undergo papillary muscle approximation (PMA). At the 5-year follow-up, we assessed the correlation between PMA and echocardiographic improvements, the effect size of PMA on echocardiographic improvements, and a prediction model for recurrent MR using inferential tree analysis. RESULTS: There was a significant correlation between PMA and enhancements in both the α and ß angles (Spearman's rho > 0.7, p < 0.01). The α angle represents the angle between the annular plane and either the A2 annular-coaptation line or the P2 annular-coaptation line. The ß angle indicates the angle between the annular plane and either the A2 annular-leaflet tip line or the P2 annular-leaflet tip line. PMA led to substantial improvements in LVEDD, tenting area, α and ß angles, with a large effect size (Hedge's g ≥ 8, 95% CI ORs ≠ 1). The most reliable predictor of recurrent MR grade was the interpapillary distance, as only patients with an interpapillary distance greater than 40 mm developed ≥ 3 + grade MR. For patients with an IPMD of 40 mm or less, the best predictor of recurrent MR grade was LVEDD. Among the patients, only those with LVEDD greater than 62 mm showed moderate (2+) MR, while only those with LVEDD less than or equal to 62 mm had absent to mild (1+) MR. CONCLUSION: Prediction of recurrent ischaemic MR is not independent of progressive LVEDD increase. PMA-based surgical procedure stabilises IPMD.

Multimodality imaging for the diagnosis of left ventricular apical hypoplasia: A case presentation and review of the literatures.

Left ventricular apical hypoplasia is a rare malformation recently described congenital abnormality characterized by: (1) truncation of the left ventricle, with the septum projecting toward the right ventricle; (2) abnormal papillary muscle originating from the flattened left ventricular apex; (3) a narrow right ventricle encompassing the periapical area of the left ventricle; (4) fatty infiltration of the apex of the left ventricle. We reported a case of LVAH and reviewed the patient's clinical presentation. And its morphologic characteristics were revealed by multimodality imaging, including echocardiography and cardiac magnetic resonance imaging. Additionally, we reviewed 41 cases from 32 reports to summarize the pathogenesis and analyzed the imaging manifestations of LVAH in this study, aiming to provide new ideas for the diagnosis and clinical management of LVAH patients.

The active papillary muscle sign in 18F-FDG PET/CT cardiac sarcoidosis exams and its relationship with myocardial suppression.

OBJECTIVE: Papillary muscle (PM) activity may demonstrate true active cardiac sarcoidosis (CS) or mimic CS in 18FDG-PET/CT if adequate myocardial suppression (MS) is not achieved. We aim to examine whether PM uptake can be used as a marker of failed MS and measure the rate of PM activity presence in active CS with different dietary preparations. MATERIALS AND METHODS: We retrospectively reviewed PET/CTs obtained with three different dietary preparations. Diet-A: 24-h ketogenic diet with overnight fasting (n = 94); Diet-B: 18-h fasting (n = 44); and Diet-C: 72-h daytime ketogenic diet with 3-day overnight fasting (n = 98). Each case was evaluated regarding CS diagnosis (negative, positive, and indeterminant) and presence of PM activity. MaxSUV was measured from bloodpool, liver, and the most suppressed normal myocardium. Linear mixed-effects models were used to compare these factors between those with PM activity and those without. RESULTS: PM activity was markedly lower in the Diet-C group compared with others: Diet-C: 6 (6.1%), Diet-A: 36 (38.3%), and Diet-B: 26 (59.1%) (p < 0.001). MyocardiumMaxSUV was higher, and MyocardiummaxSUV/BloodpoolmaxSUV, MyocardiummaxSUV/LivermaxSUV ratios were significantly higher in the cases with PM activity (p < 0.001). Among cases that used Diet-C and had PM activity, 66.7% were positive and 16.7% were indeterminate. If Diet-A or Diet-B was used, those with PM activity had a higher proportion of indeterminate cases (Diet-A: 61.1%, Diet-B: 61.5%) than positive cases (Diet-A: 36.1%, Diet-B: 38.5%). CONCLUSION: Lack of PM activity can be a sign of appropriate MS. PM activity is less common with a specific dietary preparation (72-h daytime ketogenic diet with 3-day overnight fasting), and if it is present with this particular preparation, the likelihood that the case being true active CS might be higher than the other traditional dietary preparations.

Hypertrophic Cardiomyopathy: Preadolescence, Mitral Valve Disease, and Midventricular Obstruction.

Septal myectomy is indicated in patients with obstructive hypertrophic cardiomyopathy (HCM) who have persistent symptoms despite medical therapy, intolerance of medication side effects, or severe resting or provocable gradients. Septal myectomy at high volume centers is safe, with low operative mortality (1%) and low rates of complications such as complete heart block or ventricular septal defect (3% and 0.5%, respectively). Additionally, improved survival following myectomy has been observed when compared to patients with obstructive HCM managed medically or those with nonobstructive HCM. As a longstanding, quaternary referral center for septal myectomy, our institution has built significant experience and expertise in the surgical and medical management of HCM, including atypical HCM, defined as preadolescent patients, those with mitral valve disease, and those with isolated midventricular obstruction. The most important factor of septal myectomy in achieving complete resolution of obstruction and avoiding recurrence is the apical extent of the myectomy trough, which must extend to the septum opposite the papillary muscles. If this cannot be fully achieved via a transaortic exposure, especially in preadolescents and patients with midventricular obstruction, then a transapical approach may be needed. Mitral valve repair is rarely necessary as SAM-mediated MR resolves with adequate myectomy alone, but mitral repair is performed in cases of intrinsic valvular disease. In this manuscript we provide a summary of current operative techniques and outcomes data from our institution on the management of these various categories of HCM.

Minimally Invasive Mitral Valve Repair Using Transcatheter Chordal Attachments.

The advent of transcatheter mitral chordal replacement techniques has offered an alternative approach that is less invasive and may be more suitable for select patients compared with surgical repair. These systems involve introducing artificial chordae, via catheter, to replace or supplement damaged or elongated natural chordae. These artificial chordae are anchored at one end to the mitral leaflet and the other end to the papillary muscle or directly to the left ventricular apex, restoring the leaflet's coaptation and reducing regurgitation. Early trials and studies suggest promising results in terms of safety and efficacy in reducing MR severity and improving symptoms.

Transcatheter edge-to-edge repair in papillary muscle injury complicating acute myocardial infarction.

AIMS: Acute mitral regurgitation (MR) in the setting of myocardial infarction (MI) may be the result of papillary muscle rupture (PMR). This condition is associated with high morbidity and mortality. We aim to evaluate the feasibility of transcatheter edge-to-edge mitral valve repair (TEER) in this acute setting. METHODS AND RESULTS: We analysed data from the International Registry of MitraClip in Acute Mitral Regurgitation following acute Myocardial Infarction (IREMMI) of 30 centres in Europe, North America, and the middle east. We included patients with post-MI PMR treated with TEER as a salvage procedure, and we evaluated immediate and 30-day outcomes. Twenty-three patients were included in this analysis (9 patients suffered complete papillary muscle rupture, 9 partial and 5 chordal rupture). The patients' mean age was 68 ± 14 years. Patients were at high surgical risk with median EuroSCORE II 27% (IQR 16, 28) and 20 out of 23 (87% were in cardiogenic shock). All patients were treated with vasopressors, and 17 out of 23 patients required mechanical support. TEER procedure was performed on the median 6 days after the index MI date IQR (3, 11). Procedural success was achieved in 87% of patients. The grade of MR was significantly decreased after the procedure. MR reduction to 0 or 1 + was achieved in 13 patients (57%), to 2 + in 7 patients (30%), P < 0.01. V-Wave was reduced from 49 ± 8 mmHg to 26 ± 10 mmHg post-procedure, P < 0.01. Sixteen out of 23 patients (70%) were discharged from hospital and 5 of them required reintervention with surgical mitral valve replacement. No additional death at 1 year was documented. CONCLUSIONS: TEER is a feasible therapy in critically ill patients with PMR due to a recent MI. TEER may have a role as salvage treatment or bridge to surgery in this population.

Effects of the Heterodimeric Neurotoxic Phospholipase A2 from the Venom of Vipera nikolskii on the Contractility of Rat Papillary Muscles and Thoracic Aortas.

Phospholipases A2 (PLA2s) are a large family of snake toxins manifesting diverse biological effects, which are not always related to phospholipolytic activity. Snake venom PLA2s (svPLA2s) are extracellular proteins with a molecular mass of 13-14 kDa. They are present in venoms in the form of monomers, dimers, and larger oligomers. The cardiovascular system is one of the multiple svPLA2 targets in prey organisms. The results obtained previously on the cardiovascular effects of monomeric svPLA2s were inconsistent, while the data on the dimeric svPLA2 crotoxin from the rattlesnake Crotalus durissus terrificus showed that it significantly reduced the contractile force of guinea pig hearts. Here, we studied the effects of the heterodimeric svPLA2 HDP-1 from the viper Vipera nikolskii on papillary muscle (PM) contractility and the tension of the aortic rings (ARs). HDP-1 is structurally different from crotoxin, and over a wide range of concentrations, it produced a long-term, stable, positive inotropic effect in PMs, which did not turn into contractures at the concentrations studied. This also distinguishes HDP-1 from the monomeric svPLA2s, which at high concentrations inhibited cardiac function. HDP-1, when acting on ARs preconstricted with 10 µM phenylephrine, induced a vasorelaxant effect, similar to some other svPLA2s. These are the first indications of the cardiac and vascular effects of true vipers' heterodimeric svPLA2s.

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.

Meta-Analysis of Normal Reference Values for Right and Left Ventricular Quantification by Cardiovascular Magnetic Resonance.

BACKGROUND: Cardiovascular magnetic resonance (CMR) reference values are relied upon to accurately diagnose left ventricular (LV) and right ventricular (RV) pathologies. To date, reference values have been derived from modest sample sizes with limited patient diversity and attention to 1 but not both commonly used tracing techniques for papillary muscles and trabeculations. We sought to overcome these limitations by meta-analyzing normal reference values for CMR parameters stemming from multiple countries, vendors, analysts, and patient populations. METHODS: We comprehensively extracted published and unpublished data from studies reporting CMR parameters in healthy adults. A steady-state free-precession short-axis stack at 1.5T or 3T was used to trace either counting the papillary muscles and trabeculations in the LV volume or mass. We used a novel Bayesian hierarchical meta-analysis model to derive the pooled lower and upper reference values for each CMR parameter. Our model accounted for the expected differences between tracing techniques by including informative prior distributions from a large external data set. RESULTS: A total of 254 studies from 25 different countries were systematically reviewed, representing 12 812 healthy adults, of which 52 were meta-analyzed. For LV parameters counting papillary muscles and trabeculations in the LV volume, pooled normative reference ranges in men and women, respectively, were as follows: LV ejection fraction of 52% to 73% and 54% to 75%, LV end-diastolic volume index of 60 to 109 and 56 to 96 mL/m2, LV end-systolic volume index of 18 to 45 and 16 to 38 mL/m2, and LV mass index of 41 to 76 and 33 to 57 g/m2. For LV parameters counting papillary muscles and trabeculations in the LV mass, pooled normative reference ranges in men and women, respectively, were as follows: LV ejection fraction of 57% to 74% and 57% to 75%, LV end-diastolic volume index of 60 to 97 and 55 to 88 mL/m2, LV end-systolic volume index of 18 to 37 and 15 to 34 mL/m2, and LV mass index of 50 to 83 and 38 to 65 g/m2. For RV parameters, pooled normative reference ranges in men and women, respectively, were as follows: RV ejection fraction of 47% to 68% and 49% to 71%, RV end-diastolic volume index of 64 to 115 and 57 to 99 mL/m2, RV end-systolic volume index of 23 to 52 and 18 to 42 mL/m2, and RV mass index of 14 to 29 and 13 to 25 g/m2. CONCLUSIONS: Our Bayesian hierarchical meta-analysis provides normative reference values for CMR parameters of LV and RV size, systolic function, and mass, encompassing both tracing techniques across a diverse multinational sample of healthy men and women.

Morphological Assessment and Possible Clinical Implications of the Left Ventricle Papillary Muscles. Study in a Colombian Population Sample

SUMMARY: Papillary muscles in the left ventricle present multiple anatomic expressions that are relevant for medical fields focusing on the understanding of clinical events involving these structures. Here, the aim was to perform a morphological characterization of the left ventricle papillary muscles in a sample of Colombian population. In the study were included eighty-two hearts from male individuals who underwent autopsy at the Institute of Legal Medicine and Forensic Sciences in Bucaramanga, Colombia. In each heart was carefully performed a longitudinal incision on the obtuse margin to visualize the papillary muscles. Data set was registered, and analysis of the continuous and categorical variables was carried out. Single anterior papillary muscle was observed in 74 samples (90.2 %) whereas this represented only 48 specimens (58.5 %) for the posterior papillary muscle (p = 0.3). Mean length and breadth of the anterior muscle were 29.9 ± 4.94 and 11.74 ± 2.75 mm, and those for the posterior muscle were 27.42 ± 7.08 and 10.83 ± 4.08 mm. Truncated apical shape was the most frequent type observed on the papillary muscles, anterior 41 (50 %) and posterior 37 (45.1 %), followed by flat-topped in the anterior 25 (30.5 %) and bifurcated in posterior muscle 14 (17.1 %). A mean of 9.04 ± 2.75 chordae raised from the anterior and 7.50 ± 3.3 from posterior papillary muscle. In our study we observed a higher incidence of single papillary muscles and slightly larger dimensions than information reported in the literature. The anatomic diversity of the papillary muscles should be considered for the correct image interpretation, valve implantation and performance evaluation on myocardial ischemic events.

Los músculos papilares del ventrículo izquierdo presentan múltiples expresiones anatómicas que son relevantes para las áreas médicas que se centran en la comprensión de los eventos clínicos que involucran estas estructuras. El objetivo fue realizar una caracterización morfológica de los músculos papilares del ventrículo izquierdo en una muestra de población colombiana. En el estudio se incluyeron ochenta y dos corazones de individuos masculinos a los que se les realizó autopsia en el Instituto de Medicina Legal y Ciencias Forenses de Bucaramanga, Colombia. En cada corazón se realizó cuidadosamente una incisión longitudinal en el margen obtuso para visualizar los músculos papilares. Se registró el conjunto de datos y se realizó el análisis de las variables continuas y categóricas. Se observó un solo músculo papilar anterior en 74 muestras (90,2 %), mientras que este rasgo se presentó en 48 muestras (58,5 %) para el músculo papilar posterior (p = 0,3). La longitud y anchura media del músculo anterior fueron 29,9 ± 4,94 y 11,74 ± 2,75 mm, y las del músculo posterior fueron 27,42 ± 7,08 y 10,83 ± 4,08 mm. La forma apical truncada fue el tipo más frecuente observado en los músculos papilares, anterior 41 (50 %) y posterior 37 (45,1 %), seguido de la forma plana en los 25 anteriores (30,5 %) y bifurcada en el músculo posterior 14 (17,1 %). Una media de 9,04 ± 2,75 cuerdas elevadas desde el músculo papilar anterior y 7,50 ± 3,3 desde posterior. En nuestro estudio observamos una mayor incidencia de músculos papilares únicos y dimensiones ligeramente mayores que la información reportada en la literatura. La diversidad anatómica de los músculos papilares debe ser considerada para la correcta interpretación de imágenes, implantación valvular y evaluación del desempeño en eventos isquémicos miocárdicos.

Echocardiographic quantification of mitral apparatus morphology and dynamics in patients with dilated cardiomyopathy.

Mitral regurgitation is among the most common valvular heart diseases. Mitral regurgitation in patients with dilated cardiomyopathy is a complex pathology involving annular dilatation, papillary muscle displacement, systolic leaflet tethering, and left ventricular remodeling. Quantification of mitral apparatus damage in these patients is essential for successful interventional and surgical therapy. Mitral regurgitation in the presence of dilated cardiomyopathy is classified as Carpentier type IIIB, with restricted leaflet mobility as a standard feature. Echocardiography allows accurate evaluation of the complex anatomy and function of the mitral apparatus. Updated guidelines recommend two-dimensional followed by systematic three-dimensional echocardiographic evaluation in patients with mitral regurgitation. New three-dimensional echocardiographic software packages provide many parameters that help identify the precise morphology and function of the various components of the mitral apparatus, helping to determine the etiology of mitral regurgitation and evaluate disease severity. This review provides the first point-by-point approach to the assessment of all old and new echocardiographic methods, from the simplest to the most complex, used to examine the components of the mitral valve apparatus in patients with dilated cardiomyopathy. Although these parameters are still under research, this information will be helpful for establishing therapeutic procedures in a disease with a poor prognosis.

The effects of nickel chloride on papillary muscle contractility under normothermic and hypothermic conditions: Comparison of active and hibernating ground squirrels (Urocitellus undulatus) with Wistar rats.

Extracellular Ca2+ plays a pivotal role in the regulation of cardiac contractility under normal and extreme conditions. Here, by using nickel chloride (NiCl2), a non-specific blocker of extracellular Ca2+ influx, we studied the input of extracellular Ca2+ on the regulation of papillary muscle (PM) contractility under normal and hypothermic conditions in ground squirrels (GS), and rats. By measuring isometric force of contraction, we studied how NiCl2 affects force-frequency relationship and the rest effect in PM of these species at 30 °C and 10 °C. We found that at 30 °C 1.5 mM NiCl2 significantly reduced force of contraction across entire frequency range in active GS and rats, whereas in hibernating GS force of contraction was reduced at low and high frequency range. Additionally, NiCl2 evoked spontaneous contractility in rats but not GS PM. The rest effect was significantly reduced by NiCl2 for active GS and rats but not hibernating GS. At 10 °C, NiCl2 fully reduced contractility in active GS and, to a lesser extent, in rats, whereas in hibernating GS it was significant only at 0.3 Hz. The rest effect was significantly reduced by NiCl2 in both active and hibernating GS, whereas it was unmasked in rats that had high contractility under hypothermic conditions in control. Our results show a significant contribution of extracellular Ca2+ to myocardial contractility in GS not only in active but also in hibernating states, especially under hypothermic conditions, whereas limitation of extracellular Ca2+ influx in rats under hypothermia can play protective role for myocardial contractility.

Insights into malignant mitral valve degenerative disease from a sudden cardiac death cohort highlighting significant measurement differences from normal.

AIMS: Mitral valve prolapse (MVP) is an accepted cause of sudden cardiac death (SCD) in most autopsy series. Diagnosis at autopsy relies upon subjective assessment with no established objective pathological criteria. This study set out to establish objective measurements to help pathologists dealing with SCD. METHODS: We diagnosed 120 (1.5%) cases of MVP in 8108 cases of SCD. We measured the mitral annulus, anterior and posterior leaflets, rough zone and mitral annular disjunction (MAD) in 27 MVP cases and compared them to 54 age- and sex-matched normal mitral valves. RESULTS: Age of death was 39 ± 16 years, with 59 females and 61 males. History of mild MV disease was present in 19 (16%). Eleven (9%) died associated with exertion. Left ventricular hypertrophy was present in nine (15%) females and 10 (16%) males. Both MV leaflets showed thickening and ballooning in all individuals. MVP showed highly significantly increased annular circumference, elongation and thickening of both leaflets as well as increased MAD (all P < 0.001). Left ventricular fibrosis was present in 108 (90%), with interstitial fibrosis in the posterolateral wall and papillary muscle in 88 (81%) and coexisting replacement fibrosis in 40 (37%). CONCLUSION: This is the largest MVP associated with SCD series highlighting a young cohort with equal representation of males and females. There is involvement of both leaflets with significant annular dilatation, elongation and thickening of both leaflets with MAD. Left ventricular fibrosis explains arrhythmia. Our quantitative measurements should serve as a reference for pathologists assessing post-mortem hearts for MVP.

Mid-term outcome of catheter ablation of idiopathic non-outflow tract ventricular arrhythmias.

BACKGROUND: Catheter ablation is recommended in patients with frequent and symptomatic ventricular arrhythmias (VAs) in an otherwise normal heart. Right or left outflow tract (OT) are the most common origins, and catheter ablation is highly effective with low complication rates. However, outcome of catheter ablation of VAs other than the OT (non-OTVAs) is limited. The aim of this single-center study was to assess the safety and mid-term outcome of catheter ablation for non-OTVAs. METHOD AND RESULTS: From 2013 to 2018, 251 patients who underwent catheter ablation for idiopathic non-OTVAs were enrolled and grouped according to the origins including His-Purkinje system (HPS, n = 108), papillary muscle / moderator band (PM/MB, n = 47), tricuspid annulus (TA, n = 70), and mitral annulus (MA, n = 26), 244 (97.2%) had acute elimination of VAs. The time of VAs recurrence of the single procedure was 1.69 (0.12,9.72) months, with 66% occurring within the first 3 months. The recurrence rate was significantly higher in the PM/MB group than in the TA (p = 0.025) and MA groups (p = 0.023). The single procedure success rate in all patients was 70.1%, in which 66.7%, 59.6%, 80%, and 76.9% were achieved in the HPS, PM/MB, TA, and MA groups, respectively (p = 0.284). After multiple procedures, the total success rate was 76.5% at the follow-up of 4.38 ± 2.42 years. The rate was significantly lower in the PM/MB group than in the TA group (p = 0.035). In subgroup analysis, no significant difference was observed in the recurrence rate of single procedure in patients with different VA origins within the PM/MB (log-rank test, p = 0.546). CONCLUSION: Despite a certain percentage of recurrences observed in the mid-term follow-up, catheter ablation remained feasible and effective for idiopathic non-OTVAs.

[Clinical and genetic manifestations of left ventricular non-compaction in children]. / Caratteristiche cliniche e genetiche del ventricolo sinistro non compatto in età pediatrica.

Left ventricular non-compaction (LVNC) is a myocardial disease characterized by a two-layered structure typically seen at the apical and lateral left portions of the ventricular myocardium, distal to the papillary muscles. While considered a rare disease, its prevalence in children is increasing due to the increased awareness of this condition and improved resolution of imaging techniques. The etiology is heterogeneous, ranging from inherited conditions to acquired diseases. Although many patients are asymptomatic, some patients may experience adverse events, including heart failure, arrhythmias, or thromboembolic events. Several echocardiographic or cardiac magnetic resonance imaging diagnostic criteria have been proposed for diagnosing LVNC. However, their application in children is significantly limited. This review aims to describe the clinical and genetic characteristics of children with LVNC and discuss the role of the proposed diagnostic criteria.

Posterior Papillary Muscle Suspension for Treating Systolic Anterior Motion in Hypertrophic Obstructive Cardiomyopathy.

Systolic anterior motion of the anterior mitral leaflet can persist after ventricular septal myectomy for obstructive hypertrophic cardiomyopathy, resulting in residual pressure gradients and mitral regurgitation. However, additional procedures for systolic anterior motion involving mitral valve leaflet suturing and resection may lead to future valve disease. Therefore, we adopted posterior papillary muscle suspension, a subvalvular procedure for functional mitral regurgitation, to treat systolic anterior motion without directly intervening in the mitral valve leaflets. Papillary muscle suspension toward the posterior mitral annulus moved the papillary muscles away from the interventricular septum and successfully eliminated the systolic anterior motion and midventricular pressure gradient. In terms of avoiding direct mitral interventions, this procedure is a viable option for systolic anterior motion, especially in cases of very mild mitral regurgitation.

Left ventricular fibrosis and CMR tissue characterization of papillary muscles in mitral valve prolapse patients.

PURPOSE: Mitral valve prolapse (MVP) is associated with left ventricle (LV) fibrosis, including the papillary muscles (PM), which is in turn linked to malignant arrhythmias. This study aims to evaluate comprehensive tissue characterization of the PM by cardiovascular magnetic resonance (CMR) imaging and its association with LV fibrosis observed by intraoperative biopsies. METHODS: MVP patients with indication for surgery due to severe mitral regurgitation (n = 19) underwent a preoperative CMR with characterization of the PM: dark-appearance on cine, T1 mapping, conventional bright blood (BB) and dark blood (DB) late gadolinium enhancement (LGE). CMR T1 mapping was performed on 21 healthy volunteers as controls. LV inferobasal myocardial biopsies were obtained in MVP patients and compared to CMR findings. RESULTS: MVP patients (54 ± 10 years old, 14 male) had a dark-appearance of the PM with higher native T1 and extracellular volume (ECV) values compared with healthy volunteers (1096 ± 78ms vs. 994 ± 54ms and 33.9 ± 5.6% vs. 25.9 ± 3.1%, respectively, p < 0.001). Seventeen MVP patients (89.5%) had fibrosis by biopsy. BB-LGE + in LV and PM was identified in 5 (26.3%) patients, while DB-LGE + was observed in LV in 9 (47.4%) and in PM in 15 (78.9%) patients. DB-LGE + in PM was the only technique that showed no difference with detection of LV fibrosis by biopsy. Posteromedial PM was more frequently affected than the anterolateral (73.7% vs. 36.8%, p = 0.039) and correlated with biopsy-proven LV fibrosis (Rho 0.529, p = 0.029). CONCLUSIONS: CMR imaging in MVP patients referred for surgery shows a dark-appearance of the PM with higher T1 and ECV values compared with healthy volunteers. The presence of a positive DB-LGE at the posteromedial PM by CMR may serve as a better predictor of biopsy-proven LV inferobasal fibrosis than conventional CMR techniques.

Arrhythmic mitral valve prolapse: a practical approach for asymptomatic patients.

Mitral valve prolapse (MVP) is usually regarded as a benign condition though the proportion of patients with a life-threatening arrhythmic MVP form remains undefined. Recently, an experts' consensus statement on arrhythmic MVP has proposed approaches for risk stratification across the spectrum of clinical manifestation. However, sudden cardiac death may be the first presentation, making clinicians focused to early unmasking this subset of asymptomatic patients. Growing evidence on the role of cardiac imaging in the in-deep stratification pathway has emerged in the last decade. Pathology findings have suggested the fibrosis of papillary muscles and inferobasal left ventricular wall as the malignant hallmark. Cardiac magnetic resonance, while of limited availability, allows the identification of this arrhythmogenic substrate. Therefore, speckle-tracking echocardiography may be a gateway to prompt referring patients to further advanced imaging investigation. Our review aims to summarize the phenotypic features linked to the arrhythmic risk and to propose an image-based algorithm intended to help stratifying asymptomatic MVP patients.

Diagnostic value of papillary muscle hypertrophy and mitral valve thickness to discriminate cardiac amyloidosis and Fabry disease.

BACKGROUND: Cardiac amyloidosis (CA) and Fabry disease (FD) cause myocardial damage but may also affect the valvular and subvalvular apparatus. We aimed to evaluate the diagnostic accuracy of new echocardiographic indices including mitral valve thickness and papillary muscle (PM) hypertrophy to differentiate CA and FD. METHODS: In patients with confirmed CA and FD, a detailed assessment of valvular function, mitral valve leaflet thickness and PM area as well as PM left ventricular area ratio (PM/LV-ratio) was performed in offline analyses. Receiver operating characteristic curve analyses were conducted to determine the diagnostic accuracy of mitral valve thickness, PM hypertrophy, and PM/LV-ratio to distinguish CA from FD. RESULTS: We retrospectively analyzed a cohort of 129 patients (FD n = 49, CA n = 80). CA patients showed significantly more thickened mitral valve leaflets (4.1 ± 1.3 mm vs. 2.9 ± 1.1 mm, p < 0.001) and a higher PM area [4.0 (3.1-4.6) mm2 vs. 2.8 (2.1-4.6) mm2, p = 0.009] with a comparable PM/LV-ratio in both groups. Mitral valve thickness showed the highest diagnostic accuracy to discriminate CA [AUC 0.77 (95% CI 0.67-0.87)]. The prevalence of aortic, tricuspid, and pulmonary valve regurgitation was significantly higher in CA (aortic regurgitation ≥ II° 13% vs. 4%, tricuspid regurgitation≥ II° 19% vs. 8%, p < 0.001). CONCLUSION: Our results suggest that the assessment of mitral valve thickness may be a new useful echocardiographic parameter to differentiate CA and FD, whereas papillary muscle hypertrophy and PM/LV-ratio showed a limited diagnostic performance to discriminate CA. German clinical trials registry: DRKS00027403.