Myxomatous Tissue Research Articles

Aortic leaflet remodeling for the repair of congenitally malformed aortic valve

ObjectiveAortic valve repair techniques are still evolving with better understanding of aortic root and leaflet imaging. This study seeks to present the results of an additional new technique in aortic valve repair of patients with congenital heart disease. The technique entails remodeling of the leaflet by intentional peeling of the myxomatous tissue to remodel the thickened leaflets (from the ventricular side of the aortic valve along with thinning and plication of the central region of the leaflets). Other repair techniques are added to complete the repair. MethodsWe performed a retrospective chart analysis of 19 patients who underwent aortic valve remodeling for aortic regurgitation and received advanced imaging preoperative assessment from January 2022 to February 2024. Institutional Review Board approval was obtained under expedited review for retrospective studies. ResultsAll patients with a wide range of congenital pathologies underwent leaflet remodeling; 9 patients (47%) underwent additional valve-sparing root replacement, and 6 patients (32%) received subaortic annuloplasty. Fifteen patients (79%) had moderate or severe aortic insufficiency at the time of presentation. Mean clinical follow-up was 10 months (range, 1.9-31). At follow-up, 7 patients did not have aortic regurgitation on echocardiogram, and the remaining 12 patients had mild regurgitation. None of the patients had more than mild regurgitation. All patients assessed for left ventricular ejection fraction at follow-up had an ejection fraction greater than 50%. None of the patients required reoperation. ConclusionsLeaflet remodeling by intentional peeling of myxomatous tissue expands leaflet dimensions by freeing tethered portions and improves mobility and coaptation. This additional technique will preserve more valves from replacement. However, further follow-up is needed.

A balanced mitral leaflet and large ring strategy avoids systolic anterior motion in Barlow's disease.

Mitral valve repair for Barlow's disease offers good outcomes but excessive and myxomatous valvular tissue is associated with systolic anterior motion. Although valvular disease might progress after repair and cause long-term systolic anterior motion, few reports focus on this aspect. Herein, we will review our 16-year experience with mitral valve repair for Barlow's disease and systolic anterior motion incidence. We retrospectively reviewed surgical outcomes of 92 cases of mitral valve repair using a balanced leaflet/large ring strategy plus median sternotomy for Barlow's disease (median age 45.1 ± 12.7years old [19-72], 37 females) from 2004 to 2019. Concomitant surgeries, except for tricuspid valve or anti-arrhythmic surgeries, were excluded. The follow-up period was 5.8 ± 4.4years with no deaths. Patients had mitral regurgitation of grade 3/4 (15 cases) or 4/4 (77 cases) due to anterior leaflet (3 cases), posterior leaflet (75 cases), or bileaflet (14 cases) prolapse, with chord elongation (39 cases), chord rupture (22 cases), or a combination of both (14 cases). All cases required ring annuloplasty (median size of 33.0 ± 5.4mm) combined with leaflet resection (91 cases), chord intervention (12 cases), or indentation closure (2 cases). No case had short- or long-term SAM. The freedom-from-mitral-regurgitation (of greater than grade 2/4) rate was 94.1% over 5years and 76.0% over 10years without reoperation. Our two-pronged strategy for mitral valve repair in Barlow's disease avoids systolic anterior motion over the long-term, with good outcomes.

Abstract 12214: Large Left Atrial Infected Myxoma in a Healthy Patient With Intermittent Fever

Introduction: Infected myxomas represent a rare subset of cardiac myxomas. The diagnosis is classified as definite, probable, or possible based on microbiological and pathological findings. Prognosis is often favorable with early diagnosis and intervention. Case description: A 39-year-old female with a history of iron-deficiency anemia presented with three months of fever, poor exercise tolerance, 15-lb unintentional weight loss, and episodes of amaurosis fugax associated with upper extremity paresthesias. Her physical exam revealed tachycardia and a grade 3/6 apical holosystolic murmur. Her laboratory workup was notable for microcytic anemia, elevated inflammatory markers, and Streptococcus sanguinis bacteremia . Echocardiography showed left ventricular and left atrial dilation with normal systolic function and a 6.4x4.3 cm heterogenous left atrial mass adherent to the interatrial septum with prolapse across the mitral valve plane (Figure 1, panel A-B) associated with eccentric mitral regurgitation. She was managed with surgical resection and antibiotics. Pathology revealed myxomatous tissue and prominent plasma cell infiltrates, confirmatory of definite infected cardiac myxoma. Follow-up echocardiography showed residual mitral regurgitation due to mitral annular stretch from myxoma for which surgical intervention was deferred in the acute setting. Discussion: A definite diagnosis of infected myxoma is established with biopsy-proven myxoma, along with microorganism visualization or positive blood cultures with inflammatory cells on histopathology. Rare in occurrence, infected myxomas have been associated with a higher incidence of embolic events, sepsis, and shock compared to uninfected myxomas. Prognosis is favorable with prompt intervention. Increased awareness leading to earlier diagnosis and management is a priority for this subset of patients.

FRI548 A Case Of Periureteric Myxedema

Abstract Disclosure: J.M. Ferris: None. J. Merrimen: None. S.M. Kaiser: Advisory Board Member; Self; Amgen Inc. Speaker; Self; Amgen Inc. Background: Graves’ disease is the most common cause of hyperthyroidism. Classic extrathyroidal manifestations include Graves orbitopathy and pretibial myxedema (also known as thyroid dermopathy). Both involve cytokine-induced hyaluronic acid secretion by fibroblasts, likely related to TSH-receptor antibodies. Pretibial myxedema is now rare, but nearly always coexists with orbitopathy. It classically involves the shins, but has been described in other locations. Here we present a case of myxedema changes affecting the periureteric soft tissue of a patient with Graves’ disease. Case: A 46-year-old male was seen by endocrinology for symptomatic hyperthyroidism. His clinical presentation was consistent with Graves’ disease and the diagnosis was confirmed with a strongly positive TSH receptor antibody of 25 IU/L (normal <1 IU/L). He had findings of moderate thyroid eye disease and had bilateral skin changes suspicious for pretibial myxedema. The skin findings preceded his hyperthyroidism by a few years. Dermatology was consulted and a skin biopsy confirmed pretibial myxedema, which was treated with triamcinolone injections with good effect. His thyroid function normalized with methimazole. Coincidentally, while being treated for Graves’ disease he altruistically donated a kidney to a transplant recipient. During the nephrectomy, it was noted that part of the periureteric tissue appeared thickened and firm, and further dissection revealed a soft tissue, lipoma-like mass surrounding the ureter. A frozen section sent from the operating room was found to contain myxomatous adipose tissue with very little cellularity. The final pathology reported adipose tissue with myxoid degeneration and reactive lymphoid aggregates. The histomorphology was consistent with a reactive process and there was no evidence of malignancy. In the context of the patient’s history the findings were concluded to be related to his Graves’ disease. Discussion: While pretibial myxedema most commonly affects the shins, it has also been described at other dermatologic sites, often associated with areas of trauma. Our patient, who had biopsy proven thyroid dermopathy, was also found to have myxedema affecting the ureter. To our knowledge this is only the third reported case of ureteral myxedema, and the first in a kidney donor. The patient’s renal function before surgery was normal, and post-nephrectomy his eGFR is mildly decreased, but stable. His thyroid function remains normal on methimazole. Presentation: Friday, June 16, 2023

The anatomical basis of third-degree atrioventricular block in dogs with atrioventricular valve endocardiosis

The cardiac conduction system was examined histologically in 13 canine cases of atrioventricular (AV) valve endocardiosis with third-degree AV block. In all cases, gross examination revealed marked thickening and distortion of the base of the central fibrous body (CFB) and varying degrees of endocardial thickening of the upper portion of the ventricular septum (VS) as well as marked thickening of the mitral and tricuspid valve leaflets due to myxomatous degeneration. Microscopically, the thickened and distorted CFB had encased or trapped, either partly or totally, the underlying penetrating and branching portions of the AV bundle. The myxomatous and/or fibrofatty tissue, which had proliferated at the base of the extensive CFB, protruded into or encroached on the AV bundle, causing severe (51–75%) to very severe (76% or more) reduction of the conduction fibres. The upper portions of the left and right bundle branches were involved in the endocardial thickening due to degenerative and fibrotic changes at the uppermost VS; however, both bundle branches were much less severely affected than the AV bundle, the degree of reduction of the conduction fibres ranging from mild (25% or less) to moderate (26–50%). These observations suggest that the sites most vulnerable to lesions in the AV conduction system are the penetrating and branching portions of the AV bundle, which would represent the anatomical basis for third-degree AV block in canine cases of AV valve endocardiosis.

Case presentation of oral soft tissue lesions by using ultrasound, terahertz and histopathology

<h3>Background</h3> In recent years, there has been an intense interest in biomedical imaging applications based on terahertz (THz) wavelengths due to its advantages such as non-invasiveness and non-ionizing radiation. <h3>Case Presentation and Differential Diagnosis</h3> A total of 10 intraoral lesions were evaluated by using an ACUSON S 2000 (Siemens, Munchen, Germany) color Doppler ultrasonography unit with a 15 MHz intraoperative probe. The borders of the lesion, vascular supply, and internal structure were evaluated. After ultrasonography, excision of lesions was performed under local anesthesia and histopathological examination and spectroscopy by using Terahertz Attenuated Total Reflection (ATR) in the light were performed. On ultrasonographic examination, 6 of the lesions showed a heterogeneous hyperechogenicity with well-defined margins and posterior acoustic shadow with no vascularity. The lesions were diagnosed histopathologically as fibroma with hyperkeratosis, hyperplasia of epidermis, proliferation of fibroblasts, and hyaline degeneration in partially hyperplastic collagen. Three of these lesions were hypoechoic with well-defined hyperechoic borders, with some isoechoic areas indicating internal vascularization. These lesions were histopathologically diagnosed as peripheral giant cell granuloma showing epithelioid cells and macrophages. One lesion showed a heterogeneous echogenic internal structure with clear hyperechoic borders without vascularization on ultrasonographic evaluation. This was histopathologically diagnosed as oral focal mucinosis, appearing as well-circumscribed but non-encapsulated myxomatous connective tissue. Imaging characteristics of the 10 lesions were shown by using the terahertz method with our laboratory settings. <h3>Conclusion</h3> Terahertz images were found to be promising in terms of assessing intraoral soft tissue lesions. <b>Statement of Ethical Review</b> Written consent and permission was obtained from the patients

Assessment of Valvular Oxidative Stress in a Young Patient with Primary Severe Mitral Regurgitation

Mitral valve regurgitation (MR) is the most common valvular heart disease. Diagnosing and managing mitral regurgitation is often challenging and requires a structured approach, integrating findings on history, physical examination and imaging. A common cardiac anomaly is myxomatous mitral valve prolapse. Excess myxomatous leaflet tissue, bileaflet prolapse or billowing, chordae elongation and annular dilatation are all features of Barlow’s disease. Currently, there is no successful pharmacological treatment available to prevent or slow its progression. Here, we report the case of a 30-year-old male patient, with no previous significant medical background and no medication at home, who was diagnosed with severe mitral regurgitation at a regular check-up and was admitted to our clinic with mild exertion dyspnea and fatigue. Transthoracic echocardiography showed intensely thickened mitral leaflets with a myxomatous appearance, prolapse of the middle scallop of the posterior leaflet (P2), apparently with ruptured chordae, and severe mitral regurgitation with a holosystolic eccentric jet. While oxidative stress is a central pathomechanism of cardiovascular disease, information regarding valvular oxidative stress in the literature is rather scarce. In this respect, we assessed oxidative stress through confocal microscopy in a sample of a mitral valve harvested during valvular surgery. We found an increased production of reactive oxygen species in the mitral valve sample that was alleviated after incubation with the angiotensin 2 receptor type I (AT1) antagonist irbesartan. This case is worth mentioning as a starting point for a prospective study aimed at assessing the role of valvular oxidative stress and mitochondrial dysfunction in patients with various degrees of primary and secondary mitral regurgitation.

A Study on Fetal Intracardiac Echogenic Foci

Objective: The objective of this study is to determine the frequency, location, and delineation of the echogenicity of intracardiac echogenic foci (ICEF) and to ponder the possible etiology of ICEF. Materials and Methods: Retrospective study of 2445 referred cases in second and third trimester for fetal cardiac ultrasound scan from 2010 to 2020, targeted for ruling out congenital heart disease were evaluated and analyzed in detail for ICEF. Further postnatal follow-up was done in 64 cases of ICEF till 7 years of age to see the natural course. Histopathology was performed from one case of fetal autopsy at 19 weeks of gestation for further correlation. Results: Among the fetal cardiac ultrasound cases, 323 (13.2%) showed ICEF (95% confidence interval 11.9–14.6). Majority of these ICEF (96%) were in the left ventricular cavity, and then both ventricles (3%) and remaining in right ventricle and all were located within the papillary muscles. On follow-up of 66 postnatal cases, ICEF had resolved in 97% by 5 years of age. Only two cases reported to have congenital heart disease. No chromosomal anomalies was associated in any case. Histopathology of fetal heart specimen in one case revealed myxomatous degeneration at the apices of the papillary muscle. Conclusion: Frequency of single ICEF is much higher in the left ventricle, and it is invariably located within the upper third of the anterior papillary muscle in such cases. As the gestational age advances, ICEF can resolve in utero, and further complete resolution is seen by 1–7 years of postnatal life. Variability in the echogenicity could be part of transformation of myxomatous tissues to fibrosis or microcalcification. In this study, ICEF have no particular association with congenital heart disease or chromosomal association.

GINGIVAL FOCAL MUCINOSIS: A CASE REPORT AND IMMUNOHISTOCHEMICAL ANALYSIS

Oral focal mucinosis (OFM) is a rare connective tissue disorder characterized by myxoid degeneration due to the overproduction of hyaluronic acid. Sometimes, other mesenchymal lesions with myxoid differentiation may create diagnostic difficulties when assessing OFM. Here, we report a case of gingival OFM. A 58-year-old woman presented in our service complaining of a painless gingival lesion 10 months ago. Intra-oral examination revealed a nodular, sessile and smooth surface lesion, located in the buccal gingiva, at level of the teeth #22 and #24. An excisional biopsy was performed, and microscopic analysis showed a well-defined, nonencapsulated area of myxomatous connective tissue surrounded by collagenous connective tissue. Alcian blue stain was positive. Immunohistochemical analysis revealed positivity only for vimentin and α-smooth muscle actin. The final diagnosis was OFM. The lesion did not present recurrence after 4 months’ of follow-up. OFM should be included in the differential diagnosis of gingival nodular lesions.

Percutaneous Edge-to-Edge Mitral Valve Repair with the Mitraclip System in Barlow’s Disease

Percutaneous mitral valve repair (PMVR) with the Mitraclip system (Abbott Vascular, Santa Clara, CA) has evolved from a novel technology to a safe therapy for inoperable or high-risk patients with severe symptomatic mitral regurgitation (MR). However, patients with Barlow’s disease were not enrolled in the pivotal randomized trial of Mitraclip.1 Different surgical techniques can be considered for the treatment of Barlow’s disease, including annuloplasty, resection, sliding plasty or edge-to-edge repair, but conventional surgery still remains particularly burdensome in this condition due to several anatomical challenges (e.g., dilated annulus, excess myxomatous tissue, diffuse chordal elongation and/or isolated or multiple chordal rupture).

Repair of bileaflet prolapse in Barlow syndrome: The 4-chord technique.

Barlow syndrome is a form of degenerative mitral valve (MV) disease found in a subset of patients with bileaflet prolapse. The hallmark of Barlow's disease includes excessive and billowing leaflet tissue caused by myxomatous tissue proliferation, elongated chordae, and pronounced annular dilatation. Surgical repair of patients with Barlow's disease is challenging due to the extent of the leaflet and annular abnormalities. Several techniques have been described to repair Barlow's MV including currently popular "non-resectional" approaches. Repair with neochordae has been associated with excellent results and includes the advantage of preserved leaflet mobility and a large surface of coaptation. We describe a simple approach to the use of neochordae to repair bileaflet prolapse in patients with Barlow syndrome and avoid systolic anterior motion.

Oral Focal Mucinosis on the Palatal Mucosa: A Rare Case Report

Oral focal mucinosis (OFM) is a soft tissue lesion commonly characterized by swelling in the gingiva; its occurrence in the palatal mucosa is rare. Differential diagnosis of OFM includes mucocele, nerve sheath myxoma, and myxoma, and the histologic and immunohistochemical findings are important for diagnosis. A 62-year-old man presented with a nodular lesion on the hard palate; excisional biopsy was performed. The histopathologic examination revealed a loose, myxomatous connective tissue stroma, with ovoid, fusiform, or stellate-shaped fibroblasts. The stroma was positive for Alcian blue and negative for periodic acid–Schiff staining, indicating overproduction of hyaluronic acid. Immunohistochemical analysis demonstrated that spindle cells were negative for S-100, vimentin, and CD34. After surgical excision, the patient is under regular follow-up, and no recurrence has been observed. This case highlights the importance of immunohistochemical analysis to confirm the final diagnosis.

Genomic analysis in patients with myxomatous mitral valve prolapse: current state of knowledge

BackgroundMyxomatous mitral valve prolapse is a common cardiac abnormality. Morbus Barlow is characterized by excess myxomatous leaflet tissue, bileaflet prolapse or billowing, chordae elongation and annular dilatation with or without calcification. Extensive myxoid degeneration with destruction of the normal three-layered leaflet tissue architecture is observed histologically in such patients. Autosomal dominant inheritance with an age and sex-dependent expression has long been recognised. This review explores the current understanding of the genetics of bileaflet prolapse, with a focus on genetic analysis and the role for echocardiographical screening of the first degree relatives of affected patients.MethodsSystematic literature searches were performed using PubMed and Embase up to September 2017. In Disse et al.’s study (study one) first degree relatives of 25 patients with Morbus Barlow who underwent mitral valve repair were screened for bileaflet valve prolapse. In Nesta et al.’s study one family with three living generations of 43 individuals with 9 confirmed cases of MVP was screened. Genotyping was performed in four families for 344 microsatellite markers from Chromosome 1 to 16.ResultsIn study one, autosomal dominant inheritance was shown in four pedigrees. Genome-wide linkage analysis of the most informative pedigree (24 individuals, three generations) showed a significant linkage for markers mapping to chromosome 16p. Linkage to this locus was confirmed in a second family within the same study, but was excluded in the remaining two pedigrees. In study two an autosomal dominant locus was mapped to chromosome 13. 8 of the 9 individuals affected were found to suffer from bileaflet prolapse.ConclusionsBarlow’s disease is a heritable trait but the genetic causes remain largely elusive. Ch16p11.2-p12.1 is the only locus proven to be associated with bileaflet prolapse. Locus 13.q31.3-q32.1 was shown to cause bileaflet as well as posterior leaflet prolapse. This review intends to make physicians aware of genetic causes of myxomatous mitral valve prolapse, thereby emphasising the importance of cardiological examination of first-degree relatives of patients with Morbus Barlow. Integrated and more comprehensive studies are needed for identification of genes involved in this heterogenic disease. Further genomic studies may facilitate more individualised and accurate risk assessment and may help to develop possible preventive stategies for patients in the future.

Infective Endocarditis With the Billowing Mitral Leaflet Syndrome

SESSION TITLE: Global Case Report Poster - Miscellaneous SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Billowing mitral leaflet syndrome, one of the degenerative mitral valve diseases, is the cause of mitral regurgitation. Valvular heart disease predispose to infective endocarditis. In this case, bulky and redundant mitral leaflets of the billowing mitral leaflet syndrome became misunderstood as sessile vegetation. CASE PRESENTATION: A 52-year-old Japanease man presented with a 1-month history of fever, general malaise, edema of legs and low back pain. He had no significant medical history. We performed transthoracic echocardiography, and detected sessile vegetations, below 10mm in size, on the posterior mitral valve leaflets (P2) and anterior mitral valve leaflet (A2). In addition, echocardiography showed a mitral severe regurgitation caused by the posterior mitral valve leaflet (P2) prolapse. A definitive blood culture revealed the presence of streptococci mutans. Based on the above findings, we diagnosed infective endocarditis with relatively large sessile vegetation. The patient was started on a 2-week course of intravenous antibiotics, benzylpenicillin + gentamicin after Sulbactam / Ampicillin + gentamicin. But clinical response was not good, persistent sessile vegetation and constant high C-reactive protein level. Thereby, we planned mitral valve operation. Surgery was performed with cardiopulmonary bypass and mild hypothermia after achievement of general anesthesia. There was no sessile vegetation that pointed by preoperative echocardiography, but mitral valve leaflets were billowing with excess tissue. In addition, there were granular vegetations on bi-mitral valve leaflets, and P2 chordae was ruptured. Hence, infected bi-Mitral valve were resected, we operated mitral valve replacement by a mechanical valve. Ampicillin was administered for 6-weeks after surgery. Control of infection after operation was satisfactory. The patient discharged hospital on 42-days after surgery. DISCUSSION: Degenerative mitral regurgitation such as billowing mitral leaflet syndrome is the most frequent etiology for severe mitral regurgitation. mitral regurgitation has the potential for causing infective endocarditis. In this case, an excess of myxomatous mitral tissue, characteristic of billowing mitral leaflet syndrome, was diagnosed with sessile vegetation, because he had no significant valvular disease history, systolic murmur or heart failure. CONCLUSIONS: In summary, we have reported infective endocarditis with the billowing mitral leaflet syndrome. An excess of myxomatous mitral tissue was misjudged sessile vegetations. Reference #1: Br Heart J. 1975 Mar;37(3):326-30. Infective endocarditis in the billowing mitral leaflet syndrome. Lachman AS, Bramwell-Jones DM, Lakier JB, Pocock WA, Barlow JB. DISCLOSURE: The following authors have nothing to disclose: Ayako Nagasawa No Product/Research Disclosure Information

Pulmonary Myxoma: A Rare Benign Tumor of the Lung

SESSION TITLE: Student/Resident Case Report Poster - Lung Cancer II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary myxoma is a rare benign tumor of the lung. Identification of this entity is important due to unknown incidence of the lesion and to avoid unnecessary invasive procedures. Pulmonary myxomas have been reported as sporadic case reports and can been seen arising in the lungs, pleura and trachea. As the use of computed tomography (CT) scan is increasing we are likely to see more of these benign lesions. CASE PRESENTATION: Patient is a 62 year-old active smoker who was recently diagnosed with cholangiocarcinoma. She has a past medical history of adenomatous goiter, hypertension and asthma. During the work up she was found to have a 1 cm nodule in the right upper lobe. Positron emission tomography scan showed minimal uptake in the lesion which was then biopsied and showed lobular myxo-mucinous blobs and benign slightly inflamed bronchio-alveolar tissue, negative for malignancy. She underwent cholecystectomy and right liver lobectomy. Following this she was referred to pulmonary clinic for follow up of the pulmonary nodule and had a repeat CT scan done which showed increased volume of the mass and hence it was re-biopsied and showed similar pathology suggestive of pulmonary myxoma. DISCUSSION: The earliest case of pleuro pulmonary myxoma was reported by Barkley and Cardozo1 in 1957 where they described a 54 year-old female active smoker who had presented with a consolidation and after treatment was found to have a left sided rounded opacity. She underwent thoracotomy due to increasing size on the radiograph and was found to have multiple pleural based lesions and a larger 3 cm mass in the lingula which was resected. This was shown to be made up of benign myxomatous tissue on histology. In addition to pleuropulmonary myxomas, there are case reports describing myxomas arising in the pulmonary artery, trachea2 and endobronchially. The differential diagnosis for a myxoma can be myxoid liposarcoma, myxoid chondrosarcoma, myxoid malignant fibrous histiocytoma, pulmonary hamartoma, angiomyolipoma and pulmonary lipoma. From these case reports it is observed that the myxomas tend to increase in size with time and are prone to recur after resection. Cases where complete resection was done had no recurrence during the documented follow up period. CONCLUSIONS: 1. Pulmonary myxoma is a rare benign tumor of the lung. 2. Myxomas tend to increase in size with time and are prone to recur after resection. 3. Myxomas can be seen independently and may co-exist with other malignancies3 making their identification important. Reference #1: Barkley H, Cardozo RH. A myxomatous tumour of pleuro-pulmonary origin. Thorax 1957;12:264-7. Reference #2: Nadrous HF, Krowka MJ, Myers JL, Allen MS, Sabri AN. Tracheal myxoma: a rare benign tracheal tumor. Mayo Clinic proceedings 2004;79:931-3. Reference #3: Hasleton PS, Langdale-Brown B, Rahman A, Smyrniou N, Barber PV. Pleural myxoma associated with a pulmonary squamous cell carcinoma. Respiratory medicine 1989;83:443-4. DISCLOSURE: The following authors have nothing to disclose: Sophie Korzan, Rahul Mutneja, Omar Ibrahim No Product/Research Disclosure Information

33. Moderate ischemic mitral regurgitation: Revascularization alone versus revascularization and mitral valve repair

Ischemic mitral regurgitation (IMR) can be defined as mitral valve (MV) insufficiency caused by coronary artery disease and excluding other causes of mitral pathology as rheumatic, myxomatous, infectious, congenital, or connective tissue diseases ,it usually occurs with right or circumflex coronary infarction that involves the posterior ventricular wall, posterior papillary muscle, and adjacent mitral annulus (1). The management of IMR represents a therapeutic challenge. Although most patients are treated medically, many patients are referred for surgery. Some authors claimed that revascularization alone is sufficient for managing those patients (3), whereas others have recommended revascularization combined with mitral valve repair (2). There is a general agreement that patients with mild mitral regurge (1+) are treated with coronary artery bypass surgery (CABG) alone and those with severe (3+ or 4+) IMR should undergo mitral valve surgery at the time of CABG surgery. However, the importance of moderate IMR (2+) is still controversial. A prospective controlled randomized study includes (60 patients with IHD undergoing CABG with ischemic mitral regurge aged from 40 to 65 years of both sexes). They will be divided into two groups of patients: Group I: 30 patients with IHD and moderate IMR undergoing on pump CABG for revascularization only. Group II: A 30 patients with IHD and moderate IMR undergoing on pump CABG for revascularization and mitral valve repair. Study made from January, 2014 to August, 2015, at Medina Cardiac Centre that the presence of moderate (2+) ischaemic mitral regurgitation in ischaemic heart disease patients undergoing revascularization alone does not add any additional burden to the operative risk nor does it affect the immediate and early outcome of these patients. That revascularization alone can ameliorate moderate ischaemic mitral regurgitation in most patients postoperatively. This improvement is translated into an improvement in the functional class and the quality of life postoperatively there is no statistical difference between two groups.Also a procedure to address the mitral valve in moderate IMR should be considered in patients with a worse preoperative left ventricular profile.

Abstract 19746: Identification of Novel microRNA Profiles in Patients With Myxomatous Mitral Valve Disease

Introduction: Myxomatous mitral valve disease (MMVD) is a degenerative condition characterized by tissue fibrosis and matrix remodeling which ultimately progresses to leaflet prolapse. While recent work has shown that transforming growth factor beta (TGF-β) signaling contributes to the pathogenesis of MMVD, upstream regulators of this and other pathways remain poorly understood. Hypothesis: We sought to use high-throughput RNA sequencing coupled with microRNA (miRNA) sequencing to identify novel molecular targets as well as upstream regulators contributing to MMVD. Methods: We conducted mRNA and miRNA sequencing on normal (n = 10) and myxomatous human mitral valve samples (n = 10). Differential expression was identified using linear modeling and parallel random forest analyses. Canonical pathways were identified by Ingenuity Pathway Analyses (IPA). Predicted miRNA targets were identified using TargetScanHuman 6.2. Results: We found 2784 mRNAs that were differentially expressed between normal and myxomatous mitral valves, which IPA largely categorized in to pro-fibrotic, matrix remodeling and cellular proliferation signaling. In miRNA sequenced from the same samples, 67 miRNAs were differentially-expressed between normal and myxomatous mitral valves. Increased expression of TGF-β ligands, collagen isoforms, and matrix metalloproteinases were associated with reductions in miRNAs predicted to target them. Conversely, mRNA levels of the “protective” genes TGFβ-induced factor homeobox 1, salt-inducible kinase 1, TIMP metallopeptidase inhibitor 4, and cyclin-dependent kinase inhibitor 1C mRNA levels were decreased in myxomatous tissue, and miRNAs predicted to target these genes (e.g., miR-656, miR-379-3p, miR-664a-3p, and miR-34c-5p) were significantly increased. Conclusions: Collectively, these data not only identify novel genes that are differentially regulated in MMVD, but also suggest miRNAs may play an active role in suppressing key protective molecules in MMVD. Thus, anti-mIRs therapy may be a viable therapeutic target to restore anti-fibrotic and anti-proliferative molecules in the valve and slow progression of MMVD. Future mechanistic studies will lay a critical foundation for translational work in these areas.

Increased Infiltration of Extra-Cardiac Cells in Myxomatous Valve Disease.

Mutations in the actin-binding gene Filamin-A have been linked to non-syndromic myxomatous valvular dystrophy and associated mitral valve prolapse. Previous studies by our group traced the adult valve defects back to developmental errors in valve interstitial cell-mediated extracellular matrix remodeling during fetal valve gestation. Mice deficient in Filamin-A exhibit enlarged mitral leaflets at E17.5, and subsequent progression to a myxomatous phenotype is observed by two months. For this study, we sought to define mechanisms that contribute to myxomatous degeneration in the adult Filamin-A-deficient mouse. In vivo experiments demonstrate increased infiltration of hematopoietic-derived cells and macrophages in adolescent Filamin-A conditional knockout mice. Concurrent with this infiltration of hematopoietic cells, we show an increase in Erk activity, which localizes to regions of MMP2 expression. Additionally, increases in cell proliferation are observed at two months, when hematopoietic cell engraftment and signaling are pronounced. Similar changes are observed in human myxomatous mitral valve tissue, suggesting that infiltration of hematopoietic-derived cells and/or increased Erk signaling may contribute to myxomatous valvular dystrophy. Consequently, immune cell targeting and/or suppression of pErk activities may represent an effective therapeutic option for mitral valve prolapse patients.

Primary cardiac malignant fibrous histiocytoma with abdominal wall metastasis

We present a rare case of cardiac malignant fibrous histiocytoma (MFH; undifferentiated pleomorphic sarcoma); to date, fewer than 100 cases of cardiac MFH have been reported. In this case, transthoracic echocardiography revealed cardiac tumors in the left atrium (LA) of a 53-year-old woman with a 3-month history of worsening dyspnea; the largest tumor was found to protrude through the mitral valve in diastole, causing stenosis. Three of the four tumors were resected during emergency surgery; however, the residual tumor extension into the left pulmonary vein could not be removed. Histological findings of the resected tumors, such as organized thrombus and myxomatous tissue changes, indicated that the tumors were benign. After 3 months, the patient underwent total resection for a small mass that developed on her right abdominal wall, which was revealed histologically to be MFH; additionally, the residual mass in the LA had enlarged progressively. After undergoing radiation therapy without further surgery, she died of cerebral bleeding 6 months after cardiac surgery. Postmortem examination revealed that the tumor in the LA was an MFH. Thus, cardiac MFH should be considered as a differential diagnosis for tumors on the posterior wall of the LA.<Learning objective: Primary cardiac malignant fibrous histiocytoma (MFH), which is easily mistaken for atrial myxoma, is a rare type of cardiac sarcoma. MFH occurs most commonly on the posterior wall of the left atrium (LA), and total resection is currently the only effective therapy; however, the prognosis is poor. Therefore, a high level of suspicion is required to facilitate early diagnosis. Cardiac MFH should be considered as a differential diagnosis for tumors on the posterior wall of the LA.>

Association between mitral valve prolapse and open-angle glaucoma

ObjectiveProteoglycans and glycosoaminoglycans play many roles in connective tissue formation, their accumulation in myxomatous tissues may influence the associated mechanical behaviour of the mitral valve, as well as the extracellular...