Shannon entropy as a reliable score to diagnose human fibroelastic degenerative mitral chords: A micro-ct ex-vivo study.

This paper is aimed at identifying by means of micro-CT the microstructural differences between normal and degenerative mitral marginal chordae tendineae. The control group is composed of 21 normal chords excised from 14 normal mitral valves from heart transplant recipients. The experimental group comprises 22 degenerative fibroelastic chords obtained at surgery from 11 pathological valves after mitral repair or replacement. In the control group the superficial endothelial cells and spongiosa layer remained intact, covering the wavy core collagen. In contrast, in the experimental group the collagen fibers were arranged as straightened thick bundles in a parallel configuration. 100 cross-sections were examined by micro-CT from each chord. Each image was randomized through the K-means machine learning algorithm and then, the global and local Shannon entropies were obtained. The optimum number of clusters, K, was estimated to maximize the differences between normal and degenerative chords in global and local Shannon entropy; the p-value after a nested ANOVA test was chosen as the parameter to be minimized. Optimum results were obtained with global Shannon entropy and 2≤K≤7, providing p < 0.01; for K=3, p = 2.86·10-3. These findings open the door to novel perioperative diagnostic methods in order to avoid or reduce postoperative mitral valve regurgitation recurrences.

Right atrioventricular valve dysplasia in a meerkat.

A 7-y-old, captive, intact female meerkat (Suricata suricatta) was presented with lethargy, decreased appetite, dyspnea, and distended abdomen. At autopsy, the right atrium was markedly dilated, and the right atrioventricular valve (RAV) was dysplastic with shortened or absent chordae tendineae and direct attachment of the valve to the papillary muscles, which, in turn, were fused and abnormally positioned. The right ventricle was considered to be hypertrophied. Also present were hydrothorax, ascites, atelectasis, and hepatic congestion. A diagnosis of RAV dysplasia was made. Histologic findings included hypertrophy of cardiomyocytes and marked centrilobular hepatic congestion and hemorrhage, which were consistent with right-sided heart failure.

Artificial chordae for mitral valve repair.

BACKGROUND: Mitral valve repair using expanded polytetrafluoroethylene sutures to replace mitral chordae tendineae is a well-established procedure. However, the incidence of neo-chordae failure causing recurrent mitral regurgitation is not well defined. METHODS: We have reviewed the reported cases of complications after mitral valve repair related to the use of neo-chordae. This study was mainly carried out through PubMed, Medline, and Google Chrome websites. RESULTS: We have identified a total of 26 patients presenting with rupture of polytetrafluoroethylene neo-chordae, mostly being described as isolated cases. Few other cases of recurrent mitral regurgitation with hemolysis were found, where reoperation was not caused by neo-chordal failure but most likely by technical errors. At pathological investigation the findings were substantially similar in all reported cases. The neo-chordae retained their length and pliability, became covered with host tissue and rupture was mainly related to suture size. Mild calcification was observed not interfering with chordal function; chordal infection did never occur. CONCLUSIONS: The use of artificial neo-chordae provides excellent late results with durable mitral valve repair stability. Chordal rupture may occur late postoperatively leading to reoperation because of recurrent mitral regurgitation. Despite its rarity, this potential complication should not be overlooked during follow-up of patients after mitral valve repair using artificial neo-chordae.

Acute Mitral Regurgitation Due to Chordae Tendineae Rupture: A Rare Presentation of Cardiac Amyloidosis.

BACKGROUND In cardiac amyloidosis (CA), misfolded proteins deposit in the extracellular space of cardiac tissue. These deposits classically cause restrictive cardiomyopathy with diastolic dysfunction. Although there are at least 30 proteins known to cause amyloid aggregates, 2 main types make up most diagnosed cases: light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Since CA is considered a rare condition, it is often underdiagnosed or recognized in the advanced stages. Once amyloid deposits involve the heart tissue, they are associated with a worse outcome and higher mortality rates, especially in patients presenting symptoms of heart failure. CASE REPORT We report a case of a 22-year-old man presenting with acute severe mitral regurgitation, secondary to posterior mitral leaflet chordae tendineae rupture (CTR). Surgical mitral valve replacement with a mechanical prosthesis was performed, and cardiac tissue biopsy samples were obtained. After surgery, the patient improved significantly but suddenly presented with hemodynamic deterioration, until he died due to severe hemodynamic compromise and multiorgan failure. Although the etiology of the CTR was not established before surgical intervention, the histopathological analysis suggested CA. CONCLUSIONS CA diagnosis can be complex, especially in a 22-year-old-man with atypical clinical and imaging manifestations. In this patient, other differential diagnoses were considered, since CA presenting in a young patient is a rare phenomenon and acute mitral regurgitation secondary to CTR presents more frequently in other heart conditions. Furthermore, rapid postoperative deterioration resulted in the patient's death before biopsy samples were available because suspicion of amyloidosis had not been raised until that point.

Acute aortic regurgitation due to fibrous strand rupture.

A 60-year-old male presented with sudden onset chest pain and pulmonary edema. The investigation confirmed torrential aortic regurgitation of a bicuspid valve. At surgery, a ruptured fibrous strand was identified which had been supporting the left-right cusp commissure with loss of attachment to the aortic wall. This case demonstrates that fibrous strands may be present as a supporting structure of the aortic valve, and rupture can be a rare cause of torrential aortic regurgitation, similar in pathogenesis to how it may be associated with acute severe mitral regurgitation and chordae tendineae rupture.

Morphology of the papillary muscles and the chordae tendineae of the ventricles of adult human hearts.

INTRODUCTION: The papillary muscles (PM) play a vital role in atrioventricular (AV) valve function. The PM and their chordae tendineae (CT) regulate the closure of the AV valve during systole. The present study was undertaken to categorize the PM based on their shapes and variant patterns and CT based on their types and the branching pattern. METHODS: This study included formalin-fixed ten adult cadaveric heart specimens. We observed the number, shape, length, breadth, pattern, and presence of extra PM. The number of chordae attached to the tip of each PM was quantified. We classified the types and branching patterns of the chordae and their pattern of attachment to the cusps. RESULTS: In the right ventricle, conical, truncated, and flat-topped PM were observed. The anterior PM had 5.3 ± 1.9, the posterior PM had 2.7 ± 2.1, and the septal PM had 3.5 ± 2.3 CT attached to it. In the left ventricle, we observed conical, truncated, flat-topped, bifurcate, and trifurcate shapes of PM. The anterior and the posterior PM had 7.7 ± 2.8 and 7.7 ± 2.7 CT attached to them, respectively. The true CT were cusp, cleft, and commissural and the false CT were pillar-wall, inter-pillar, and strut. We also found 3 branching patterns for the chordae (single, fan-shaped, and web forming). CONCLUSION: The study explored the comparative morphology of PM and chordae in the right and left ventricles. The knowledge of the morphological pattern of PM and CT would contribute to the valvular function and aid in diagnosing conditions such as valve prolapse or regurgitation.

Aortic Chordae Tendineae Strands with Significant Aortic Regurgitation.

The aortic chordae tendineae strands are suggested to be embryonic remnants of the cusp formation process. We herein describe a 70-year-old male who was admitted to our hospital for shortness of breath and chest tightness. During echocardiographic examination, severe aortic regurgitation with a ruptured fibrous strand was detected. Moreover, another fibrous strand was found by three-dimensional transesophageal echocardiography (TEE). To our knowledge, this is the first literature review of aortic chordae tendineae strands, including diagnosis, management, and mechanisms of aortic regurgitation due to such informal strands.

Transcatheter mitral valve repair: an overview of current and future devices.

The field of transcatheter mitral valve repair (TMVr) for mitral regurgitation (MR) is rapidly evolving. Besides the well-established transcatheter mitral edge-to-edge repair approach, there is also growing evidence for therapeutic strategies targeting the mitral annulus and mitral valve chordae. A patient-tailored approach, careful patient selection and an experienced interventional team is crucial in order to optimise procedural and clinical outcomes. With further data from ongoing clinical trials to be expected, consensus in the Heart Team is needed to address these complexities and determine the most appropriate TMVr therapy, either single or combined, for patients with severe MR.

A percutaneous approach to create tricuspid regurgitation in swine: a model for transcatheter tricuspid valve therapy assessment.

PURPOSE: The purpose of this study was to develop a simple and effective percutaneous approach to create tricuspid regurgitation in swine. METHODS: Eleven pigs (71.68 ± 7.70 kg, 3 male) were involved in this study. A grasping forceps was introduced into the right ventricle through a steerable sheath under fluoroscopic guidance and used to disrupt the tricuspid valve apparatus by avulsing leaflet or chordae tendineae repeatedly. Transthoracic echocardiography and right ventricular angiography were used to evaluate the degree of tricuspid regurgitation created. RESULTS: Ten of the 11 pigs (90.91%) achieved severe tricuspid regurgitation and 1 (9.09%) obtained moderate tricuspid regurgitation immediately after the procedure. Heart rate of the pigs significantly increased immediately after tricuspid regurgitation creation compared to baseline (88.64 ± 23.24 vs. 76.00 ± 15.30 bpm, P = 0.02), but recovered to normal level at one month follow-up (77.09 ± 11.97 bpm, P = 0.85). The right atrium, tricuspid valve annulus, and right ventricle dilated obviously one month after tricuspid regurgitation creation (dimension changes: 3.01 ± 0.35 vs. 3.56 ± 0.40 cm, P = 0.02; 2.92 ± 0.36 vs. 3.37 ± 0.39 cm, P = 0.01; 3.06 ± 0.42 vs. 3.60 ± 0.47 cm, P = 0.03 respectively). Autopsy findings showed that rupture of leaflet and/or chordae tendineae finally led to the tricuspid regurgitation. CONCLUSIONS: Severe tricuspid regurgitation can be created by a simple and effective percutaneous approach with a grasping forceps in swine model and right heart dilation can be observed consistently at one-month follow-up. This model will be valuable in pre-clinical studies for developing new tricuspid valve repair or replacement technique to treat severe tricuspid regurgitation.

Interactive editing of virtual chordae tendineae for the simulation of the mitral valve in a decision support system.

PURPOSE: Decision support systems for mitral valve disease are an important step toward personalized surgery planning. A simulation of the mitral valve apparatus is required for decision support. Building a model of the chordae tendineae is an essential component of a mitral valve simulation. Due to image quality and artifacts, the chordae tendineae cannot be reliably detected in medical imaging. METHODS: Using the position-based dynamics framework, we are able to realistically simulate the opening and closing of the mitral valve. Here, we present a heuristic method for building an initial chordae model needed for a successful simulation. In addition to the heuristic, we present an interactive editor to refine the chordae model and to further improve pathology reproduction as well as geometric approximation of the closed valve. RESULTS: For evaluation, five mitral valves were reconstructed based on image sequences of patients scheduled for mitral valve surgery. We evaluated the approximation of the closed valves using either just the heuristic chordae model or a manually refined model. Using the manually refined models, prolapse was correctly reproduced in four of the five cases compared to two of the five cases when using the heuristic. In addition, using the editor improved the approximation in four cases. CONCLUSIONS: Our approach is suitable to create realistically parameterized mitral valve apparatus reconstructions for the simulation of normally and abnormally closing valves in a decision support system.

A rare case report: tricuspid valve prolapse due to spontaneous chordae rupture in a congenitally corrected transposition of the great arteries patient.

BACKGROUND: Congenitally corrected transposition of great arteries (CCTGA) is caused by atrioventricular and ventriculoarterial discordance. Cases of CCTGA with spontaneous chordae rupture of tricuspid valve have not been reported before. CASE PRESENTATION: Here we diagnosed a 38-year-old man, who was found CCTGA 14 years ago, as spontaneous chordae rupture by real-time three dimentional transesophageal echocardiography (RT-3D-TEE). The present case is the first report to describe a CCTGA patient combine with spontaneous chordae rupture in tricuspid valve. After tricuspid valve replacement, the patient was uneventful after 6 years' follow-up. CONCLUSION: We reported a rare case with spontaneous chordae rupture of tricuspid valve in a CCTGA patient and explored its etiology here. RT-3D-TEE is an important supplement to 2-dimentional transthoracic echocardiography and can provide more accurate detections in tricuspid valve diseases in CCTGA.

Papillary fibroelastoma entangled with tendinous chords: gap between echocardiographic and operative findings.

This papillary fibroelastoma case showed the imaging gap between echocardiography and operative findings. Use of 4K endoscopy enabled us to visualize the details of the tumor and to excise it safely.

New insights into mitral heart valve prolapse after chordae rupture through fluid-structure interaction computational modeling.

Mitral valve (MV) dynamics depends on a force balance across the mitral leaflets, the chordae tendineae, the mitral annulus, the papillary muscles and the adjacent ventricular wall. Chordae rupture disrupts the link between the MV and the left ventricle (LV), causing mitral regurgitation (MR), the most common valvular disease. In this study, a fluid-structure interaction (FSI) modeling framework is implemented to investigate the impact of chordae rupture on the left heart (LH) dynamics and severity of MR. A control and seven chordae rupture LH models were developed to simulate a pathological process in which minimal chordae rupture precedes more extensive chordae rupture. Different non-eccentric and eccentric regurgitant jets were identified during systole. Cardiac efficiency was evaluated by the ratio of external stroke work. MV structural results showed that basal/strut chordae were the major load-bearing chordae. An increased number of ruptured chordae resulted in reduced basal/strut tension, but increased marginal/intermediate load. Chordae rupture in a specific scallop did not necessarily involve an increase in the stress of the entire prolapsed leaflet. This work represents a further step towards patient-specific modeling of pathological LH dynamics, and has the potential to improve our understanding of the biomechanical mechanisms and treatment of primary MR.

Spontaneous chordae tendineae rupture during peripartum.

Acute cardiopulmonary distress in pregnancy always carries exceptionally arduous challenge for physicians. Here we report a patient who sustained spontaneous chordae tendineae rupture complicated with severe mitral regurgitation and acute pulmonary edema during peripartum period. Probable causes of chordae tendineae rupture include mitral valve prolapse, infectious endocarditis, congenital heart disease, rheumatic heart disease, ischemic heart disease, connective tissue diseases, previous mitral valve surgery or pregnancy itself. The pathophysiology of spontaneous chordae tendineae rupture due to pregnancy remains unclear. However, certain physiological stress, including hormone changes related matrix remodeling, increased cardiac output during pregnancy or labor pain may precipitate to this condition. Literature reviews from previously reported cases showed that those who were diagnosed chordae tendineae rupture at very preterm period all had preterm delivery.

Systematic review and meta-analysis of chordal replacement versus leaflet resection for posterior mitral leaflet prolapse.

OBJECTIVES: To compare outcomes of chordal replacement versus leaflet resection techniques for repair of isolated posterior mitral leaflet prolapse. METHODS: We searched MEDLINE and EMBASE databases for studies that compared chordal replacement ("neo-chord" group) versus leaflet resection ("resection" group) techniques for the treatment of posterior mitral leaflet prolapse. Data were extracted by 2 independent investigators and subjected to a meta-analysis using a random-effects model. RESULTS: One randomized controlled trial (RCT), 1 propensity-matched study, and 6 unadjusted observational studies, with a total of 1926 patients, met our inclusion criteria. Two studies reported only perioperative outcomes; mean follow-up ranged from 1.0 to 5.9 years in the remaining studies. In pooled data from unadjusted observational studies, annuloplasty ring diameter was higher in the neo-chord group (+1.5 mm; P = .0003), but with high heterogeneity (I2 = 91%). Based on limited data, postprocedural left ventricular ejection fraction may be greater in the neo-chord group, but this difference reached statistical significance only in the RCT (+3.4%; P = .03), and not in 2 observational studies that reported this outcome (+2.7%; P = .10). There was no difference in recurrent mitral regurgitation at follow-up between the resection and neo-chord groups; however, patients in the neo-chord group had a lower rate of mitral valve reoperation at follow-up in the unadjusted observational studies (incidence rate ratio, 0.22; P = .0008 [I2 = 0%; 4 studies, 1331 patients]). CONCLUSIONS: Chordal replacement may be associated with greater freedom from reoperation and may lead to improved postoperative left ventricular function compared with leaflet resection. However, these conclusions are supported primarily by data from unadjusted observational studies, and high-quality RCTs of chordal replacement versus leaflet resection are needed.

Mitral Valve Replacement After Failed Mitral Ring Insertion With or Without Leaflet/Chordal Repair for Pure Mitral Regurgitation.

Mitral repair operations for correction of pure mitral regurgitation (MR) are generally quite successful. Occasionally, however, the reparative procedure incompletely corrects the MR or the MR recurs. From March 1993 to January 2016, twenty nine patients had mitral valve replacement after the initial mitral repair operation, and observations in them were analyzed. All 29 patients at the repair operation had an annular ring inserted and later (<1 year in 6 and >1 year in 21) mitral valve replacement. The cause of the MR before the repair operation appears to have been prolapse in 16 patients (55%), secondary (functional) in 12 (41%) (ischemic in 5), and infective endocarditis which healed in 1 (3%). At the replacement operation the excised anterior mitral leaflet was thickened in all 29 patients. Some degree of stenosis appeared to have been present in 16 of the 29 patients before the replacement operation, although only 10 had an echocardiographic or hemodynamic recording of a transvalvular gradient; at least 11 patients had restricted motion of the posterior mitral leaflet; 10, ring dehiscence; 2, severe hemolysis; and 2, left ventricular outflow obstruction. In conclusion, there are multiple reasons for valve replacement after earlier mitral repair. Uniformly, at the time of the replacement, the mitral leaflets were thickened by fibrous tissue. Measurement of the area enclosed by the 360° rings and study of the excised leaflet suggest that the ring itself may have contributed to the leaflet scarring and development of some transmitral stenosis.

Comparative Histopathological Analysis of Mitral Valves in Barlow Disease and Fibroelastic Deficiency.

Whether Barlow disease (BD) and fibroelastic deficiency (FED), the main causes of mitral valve prolapse (MVP), should be considered 2 distinct diseases remains unknown. Mitral valves from patients who required surgery for severe mitral regurgitation due to degenerative nonsyndromic MVP were analyzed. Intraoperative diagnosis of BD or FED was based on leaflet redundancy and thickness, number of segments involved, and annular dimension. The removed medial scallop of the posterior leaflet and attached chordae were used for histopathological and immunohistological assessment. Histologically, compared to normal controls (n = 3), BD (n = 14), and FED (n = 9) leaflets demonstrated an altered architecture and increased thickness. Leaflet thickness was greater and chordae thickness lower in BD than FED (P < 0.0001). In BD, increased thickness was owing to spongiosa expansion (proteoglycan accumulation) and intimal thickening on fibrosa and atrialis; in FED, local thickening was predominant on the fibrosa side, with accumulation of proteoglycan-like material around the chordae. Collagen accumulation was observed in FED leaflets and chords and decreased in BD. Fragmented elastin fibers were present in BD and FED; elastin decreased in BD but increased in FED leaflets and around chordae. Activated myofibroblasts accumulate in both diseased leaflets and chords, but more abundantly in FED chordae (P < 0.0001), independently of age, suggesting a role of these cells in chordal rupture. There were more CD34-positive cells in BD leaflets and in FED chordae (P < 0.01). In BD leaflets (but not chordae) proliferative Ki67-positive cells were more abundant (P < 0.01) and matrix metalloproteinase 2 levels were increased (P < 0.01) indicating tissue remodeling. Upregulation of transforming growth factor beta and pERK signaling pathways was evident in both diseases but more prominent in FED leaflets (continued on next page)(P < 0.001), with pERK upregulation in FED chordae (P < 0.0001). Most cellular and signaling markers were negligible in control valves. Quantitative immunohistopathological analyses demonstrated distinct changes between BD and FED valves: predominant matrix degradation in BD and increased profibrotic signaling pathways in FED, indicating that BD and FED are 2 different entities. These results may pave the way for genetic studies of MVP and development of preventive drug therapies.