Posterior Mitral Valve Leaflet Research Articles

Incidental Findings of Congenital Unileaflet Mitral Valves in Young Patient Presenting Complete Atrioventricular Block.

The singular leaflet mitral valve is a rare and complex entity that has been described in only a few case reports. It is commonly observed in infancy and combined with symptomatic mitral regurgitation (MR). In most asymptomatic individuals, this complication is attributed mainly to a hypoplastic posterior mitral leaflet and is usually revealed in adulthood. We present the case of a 30-year-old man who presented with chest pain and dyspnea for two days. The electrocardiogram showed complete atrioventricular block with a beat rate of 30 bpm.Echocardiography revealed an elongated anterior mitral valve leaflet and an almost completely absent posterior mitral valve leaflet with no MR. Our case is unusual presentation of a unileaflet mitral valve that should be investigated even in adulthood as these patients are likely to be at risk of developing or worsening MR later in life; hence, performing periodic echocardiograms is important for detecting the progression of MR early.

Mitral valve repair with leaflet resection versus preservation for degenerative posterior leaflet prolapse

ObjectivesThe optimal technique for repairing posterior mitral valve leaflet prolapse remains undetermined. We aimed to compare leaflet resection and neochordae implantation in patients undergoing mitral valve repair for posterior leaflet prolapse, focusing on transmitral pressure gradient and recurrence of mitral regurgitation. MethodsWe enrolled patients undergoing mitral valve repair using either leaflet resection or neochordae implantation for single-segment prolapse of posterior mitral valve leaflet between 2000 and 2021 at our institution. Longitudinal outcomes were evaluated after adjustments with inverse-probability-of-treatment weighting. Repeat echocardiographic measurements (n = 3473, 5.4/patient) of transmitral pressure gradient and significant (moderate or severe) mitral regurgitation recurrence were estimated using nonlinear mixed-effect models. Subgroup analyses were conducted based on the size and type of prosthesis. ResultsAmong 639 patients, leaflet resection was used in 479 (75.0%) and neochordae implantation was used in 160 (25.0%). In the inverse-probability-of-treatment weighting adjusted cohort, the risk of death (P = .623) and mitral valve reoperation (P = .340) did not significantly differ between the 2 groups during a median follow-up of 97.3 months. Echocardiographic data showed comparable mean (at 5 years, 3.8 vs 4.0 mm Hg; P = .442) and peak (9.6 vs 10.4 mm Hg; P = .131) pressure gradients between groups, which persisted in most subgroup analyses. However, neochordae implantation was associated with a higher probability of significant mitral regurgitation recurrence compared with leaflet resection (at 5 years, 16.1% vs 7.0%; P < .001). ConclusionsLeaflet resection yielded similar clinical outcomes and transmitral pressure gradients compared with neochordae implantation after mitral valve repair, with a lower mitral regurgitation recurrence rate. These findings underscore the need to reassess the efficacy of neochordae implantation relative to leaflet resection.

A Man with an Usual Cause of Mitral Insufficiency

Cardiac calcified amorphous tumors (CAT) are rarely encountered nonneoplastic tumors that may arise within any cardiac chamber. Involvement of the mitral valve causing pathology is even less frequently reported. We report the case of a CAT arising on the posterior mitral valve leaflet resulting in chordal rupture and severe mitral insufficiency. Multi-modality imaging was performed before definitive diagnosis through histology. We review the morphological features encountered in our case to highlight the diagnostic difficulties and raise awareness of this peculiar pathology.

Changes in left ventricular-aortic angulation are associated with the development of obstruction in hypertrophic cardiomyopathy

ObjectiveTo characterize changes in ventricular morphology in patients with hypertrophic cardiomyopathy who develop left ventricular (LV) outflow tract obstruction. MethodsWe reviewed patients with hypertrophic cardiomyopathy with LV outflow tract obstruction who underwent septal myectomy from May 2012 to June 2023. Among 68 patients initially without obstruction documented up to 7.6 years (interquartile range, 6.3-9.4 years) before the operation, a comparison was made with 78 patients with nonobstructive hypertrophic cardiomyopathy over a similar period. Patients who did not develop obstruction were matched with those who did on sex, age, and maximum septal wall thickness during the initial echocardiography, identifying 41 matched pairs. Echocardiographic data, including 5 measures of angulation, were compared between the groups. ResultsThe median interval between echocardiographic assessments was 7.5 years (interquartile range, 6.3-8.1 years) among patients with obstruction versus 7.3 years (interquartile range, 6.2-9.0 years) in patients without nonobstruction. Patients with obstruction were more likely to have hypertension at both times. The maximum septal wall thickness increased within both groups (both P values < .001), but the magnitude of increase was not different between groups (P = .130). Patients with obstruction exhibited a greater increase in LV mass (P < .001) compared with patients without obstruction (P = .004). Aortic angulation significantly increased in 4 of the 5 measurements (all P values < .001) in patients with obstruction, whereas patients with no obstruction showed no change. Anterior and posterior mitral valve leaflet lengths and coaptation lengths remained similar in both groups over time. ConclusionsThe development of LV outflow tract obstruction in patients with hypertrophic cardiomyopathy was associated with progressive LV outflow tract angulation and increased LV hypertrophy, as reflected by LV mass. Progression to obstruction was not related to changes in the mitral valve leaflet morphology.

Mutation in PDGFRbeta: a potential new pathogenic variant for acquired mitral valve prolapse

Abstract Funding Acknowledgements None. Background Mitral valve prolapse (MVP) due to myxomatous degeneration is characterized by familial clustering, but few pathogenic genes have been identified. In a genetic screening program for MVP patients, we identified a platelet-derived growth factor receptor β (PDGFRβ)-E162K missense variant in a family with a bi-leaflet MVP phenotype in four affected family members. PDGFRβ is a receptor tyrosine kinase, which plays an important role in vascular development. Purpose In this study, we investigated whether the E162K substitution in PDGFRβ could lead to mitral valve abnormalities. Methods To assess the functional effects of the missense variant, proliferation and migration assays were performed using HeLa cells overexpressing wild-type (WT) or mutant PDGFRβ. Immunostainings were performed to determine PDGFRβ expression in the diseased valves from a family member undergoing surgical operation and from additional patients operated for MVP. Mouse models were created with the mouse equivalent of the human PDGFRβ-E162K variant (PDGFRβ-E161K) using CRISPR/CAS9. The variant was not lethal and transthoracic echocardiography was performed on adult homozygous PDGFRβE161K/E161K mice, heterozygous PDGFRβE161K/- mice, and WT mice . In addition, histology was performed in adult and neonatal mice to detect potential valvular defects. Results HeLa cells expressing PDGFRβ-E162K and stimulated with PDGFD showed reduced proliferation and migration, suggesting a loss of function of the mutant receptor. PDGFRβ was expressed in human diseased mitral valves. Echocardiographic evaluation revealed a significant larger mitral valve annulus diameter in both PDGFRβE161K/- and PDGFRβE161K/E161K compared to WT (WT: 1.97 mm; PDGFRβE161K/-:2.13, p=0.031; PDGFRβE161K/E161K:2.42 mm, p&amp;lt;0.0001). Histological data in PDGFRβE161K/E161K mice showed significant leaflet thickening in the free edge (FE) of the posterior mitral valve leaflet (PMVL) compared to WT (WT: 72.34 ɥm; PDGFRβE161K/E161K: 109.6 ɥm, p=0.040) and a larger leaflet area of the PMVL compared to WT (WT: 25280 ɥm²; PDGFRβE161K/E161K: 44494 ɥm², p=0.020). Moreover, an altered organization of the extracellular matrix reminiscent of the myxomatous degeneration in MVP was observed. These alterations were not observed in mutant neonatal hearts. Conclusions The PDGFRβ-E162K variant is associated with familial MVP and alters the function of PDGFRβ. Mice harboring this mutation display acquired mitral valve defects.

HOW TO MANAGE MITRAL VALVE POSTERIOR LEAFLET HYPOPLASIA

Abstract Background Mitral valve posterior leaflet hypoplasia is a relatively rare and often congenital anatomical malformation. In this condition, achieving a good and durable mitral valve repair still represents a challenge for surgeons. Methods We present the case of a 43 years old man presenting with exertional dyspnoea due to severe mitral regurgitation. Transthoracic and transesophageal echocardiograms revealed a severe hypoplasia of the posterior mitral leaflet with an significant prolapse of the anterior leaflet (particularly A2–A3), which determined massive regurgitation. The anteroposterior annular diameter was 38 mm with a 4 mm mitro–anular disjunction, left ventricular telediastolic diameter was 59 mm and ejection fraction 68%. No other cardiac malformations were evident; in particular tricuspid valve was normal. Results Surgery was performed through a right mini–thoracotomy with femoral cannulation, using three–dimensional video–assisted technology. The mitral valve was approached from the left atrium, behind the interatrial groove. Surgical inspection revealed a severely hypoplasic posterior leaflet and a wide anterior leaflet prolapsing near the postero–medial commissure (A2–A3) (Figure 1). Based on our previous experience, we decided to repair the valve performing a posterior para–commissural edge–to–edge (A3–P3) followed by an atypical annuloplasty using a bovine pericardium band, shaped on a 40 mm ring sizer, only in the postero–medial portion of the mitral annulus (Figure 2). On the hydrostatic test, the valve showed a satisfactory continence, that was confirmed on the intraoperative TEE. Postoperative course was uneventful and the patient was discharged home on postoperative day 7. Transthoracic echocardiography at discharge showed a good result of the mitral valve repair with no residual regurgitation a medium gradient across the mitral valve of 3 mmHg. Conclusions In our experience, reductive annuloplasty isn’t the optimal treatment for hypoplasic mitral valve posterior leaflet because it lowers the already small posterior leaflet, thus reducing the coaptation surface. To avoid this, we introduced this technique that improved our surgical outcomes.

An unusual presentation of subacute Haemophilus parainfluenzae endocarditis in a low-risk woman treated by minimally invasive mitral valve repair: a case report

BackgroundHACEK endocarditis is usually insidious and can often be difficult to diagnose due to the slow-growing nature of the organisms. This report presents our experience in treating a patient with Haemophilus parainfluenzae endocarditis.Case presentationWe describe the case of a previously fit and well 23 year-old woman who presented to her local emergency department with a four-week history of persistent febrile illness. She had associated nausea, vomiting, and lethargy. This was preceded by an episode of mucopurulent rhinorrhoea. She was treated empirically with oral amoxicillin for a putative diagnosis of rhinosinusitis. Initially, her symptoms abated, however, she was readmitted with high fevers and a new pansystolic murmur. Transthoracic echocardiography revealed a large, mobile, echogenic mass, tethered to the posterior mitral valve leaflet (PMVL) and mild mitral regurgitation (MR). On examination, she had multiple non-tender, erythematous macules on the plantar surface of her feet, consistent with Janeway lesions. Two separate blood cultures grew H. parainfluenzae. Infectious diseases recommended a four-week course of intravenous ceftriaxone. Transesophageal echocardiography demonstrated a perforation within the P3 segment of the PMVL. Subsequently, the patient underwent mitral valve repair surgery with an uneventful recovery.ConclusionsOur case highlights the importance of promptly diagnosing HACEK endocarditis. A prolonged course of antibiotic therapy can be lifesaving, and surgery is often necessary to address complications such as perforation within the mitral valve leaflets. In our patient, we were able to perform a sliding P2 leaflet plasty for good quality repair of the mitral valve, through a minimally invasive right anterior thoracotomy.

E031 Retinal vasculitis with Libman-Sacks endocarditis as presenting features of a systemic lupus erythematosus with antiphospholipid antibody syndrome patient

Abstract Background/Aims Ocular manifestations of systemic lupus erythematosus (SLE) are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with the active generalised disease. We report a case of a 22-year-old lady who was diagnosed with SLE with antiphospholipid antibody syndrome (APS) as the initial presentation of retinal vasculitis and Libman-Sacs endocarditis. Methods A 22-year-old lady with a background history of benign hypermobility spectrum disorder, presented to the eye casualty with a 4-week history of right-sided visual disturbances and was diagnosed with retinal vasculitis associated with uveitis, central retinal vein occlusion with multiple emboli in the retinal artery of the right eye and a single embolus in the left eye. In terms of family history, both father and brother were diagnosed with spondyloarthropathy, and the maternal uncle had Sjogren’s syndrome. She was then referred to the medical team for proper evaluation. On query, she mentioned photosensitive rash on her face getting worse in sunlight, a history of Raynaud’s phenomenon, significant hair loss, and one miscarriage before the 10 weeks of gestation. On examination, mild alopecia and a faint butterfly rash on the face were noted. Cardiac auscultation revealed pansystolic murmur. The rest of the systemic examinations were unremarkable. Results The blood test showed thrombocytopenia (130 u/L), positive antinuclear antibody (1:80, speckled pattern) with anti-double stranded DNA (26 IU/L), low complement (C3-0.74, C4- 0.12), markedly raised anticardiolipin antibody IgG (2016 u/ml), anti beta 2 glycoprotein 1 IgG ( &amp;gt;6100 u/ml) including presence of lupus anticoagulant. Her urinary protein creatinine ratio was 66mg/mmol. 3 sets of blood cultures came back negative. Transthoracic echo showed vegetation on the posterior mitral valve leaflet (0.95x 1.2cm) with moderate mitral regurgitation while transesophageal echo additionally found the possibility of perforation at the junction of leaflets of the mitral valve which was later confirmed by cardiac magnetic resonance imaging (MRI). The head MRI scan did not show any central nervous system abnormality. She was initially treated with 500mg intravenous methylprednisolone for 3 days followed by 20mg oral prednisolone daily with gradual tapering. Hydroxychloroquine and mycophenolate mofetil were added. Her case was discussed in the cardiology multidisciplinary team meeting and an opinion from the cardiothoracic surgery was taken to consider valve replacement in the future. Due to aspirin allergy, only warfarin was considered for APS. She had 2 sessions of pan-retinal photocoagulation as well. In subsequent follow-up, it was noted significant improvements in terms of her symptoms. Conclusion Vaso-occlusive retinopathy is a rare, potentially vision-threatening initial manifestation of SLE, which can be an indicator of active disease. Early identification of patients is necessary for timely intervention and multidisciplinary team involvement is required for comprehensive treatment plans. Disclosure M. Islam: None. S. Quddus: None. M. Hughes: None. R. Kumar: None.

Mutation in PDGFRbeta: a potential new pathogenic variant for mitral valve prolapse

Abstract Background Mitral valve prolapse (MVP) due to myxomatous degeneration is characterized by familial clustering, but few pathogenic genes have been identified. In a genetic screening program for MVP patients, we identified a platelet-derived growth factor receptor β (PDGFRβ)-E162K missense variant in a family with a bi-leaflet MVP phenotype in four affected family members. PDGFRβ is a receptor tyrosine kinase, which plays an important role in vascular development. Purpose In this study, we investigated whether the E162K substitution in PDGFRβ could lead to mitral valve abnormalities. Methods To assess the functional effects of the missense variant, proliferation and migration assays were performed using HeLa cells overexpressing wild-type (WT) or mutant PDGFRβ. Immunostainings were performed to determine PDGFRβ expression in the diseased valves from a family member undergoing surgical operation and from additional patients operated for MVP. Mouse models were created with the mouse equivalent of the human PDGFRβ-E162K variant (PDGFRβ-E161K) using CRISPR/CAS9. The variant was not lethal and a total of 12 homozygous PDGFRβ-E161K-/- mice, 11 heterozygous PDGFRβ-E161K+/- mice, and 7 WT mice were subjected to transthoracic echocardiography. In addition, histology was performed in all 30 mice to detect potential valvular defects. Results Stimulated HeLa cells expressing PDGFRβ-E162K showed reduced proliferation and migration, suggesting a loss of function of the mutant receptor. PDGFRβ was expressed in human diseased mitral valves. Echocardiographic evaluation revealed a significant larger mitral valve annulus diameter in both PDGFRβ-E161K+/- and PDGFRβ-E161K-/- compared to WT (WT: 1.97 mm; PDGFRβ-E161K+/-:2.13, p=0.031; PDGFRβ-E161K-/-: 2.42 mm, p&amp;lt;0.0001). Histological data in PDGFRβ-E161K-/- mice showed significant leaflet thickening in the free edge (FE) of the posterior mitral valve leaflet (PMVL) compared to WT (WT: 72.34 ɥm; PDGFRβ-E161K-/-: 109.6 ɥm, p=0.040) and a larger leaflet area of the PMVL compared to WT (WT: 25280 ɥm²; PDGFRβ-E161K-/-: 44494 ɥm², p=0.020). Moreover, an altered organization of the extracellular matrix reminiscent of the myxomatous degeneration in MVP was observed. Conclusions The PDGFRβ-E162K variant is associated with familial MVP and alters the function of PDGFRβ. Mice harboring this mutation display mitral valve defects and can be used as a model for mitral valve disease.

Abstract 16003: A Case of Ventricular Fibrillation Arrest in a Healthy Young Female With Newly Discovered Mitral Annular Disjunction

Introduction: Mitral annular disjunction (MAD) is an under-recognized cause of ventricular arrhythmias. Case Presentation: A previously healthy, athletic 43-year-old female suddenly collapsed during a staff meeting at a medical center. Cardiopulmonary resuscitation was initiated and she was found to be in ventricular fibrillation, requiring three defibrillation shocks to achieve ROSC. EKG was notable for prolonged QTc without signs of pre-excitation or Brugada syndrome. Patient denied any personal or family cardiac history but endorsed frequent palpitations in the past. No electrolyte derangements were identified. Coronary angiography revealed normal origins of the coronary arteries and no evidence of obstructive disease. Transthoracic echocardiogram demonstrated normal left ventricular systolic function, normal wall thickness, and no regional wall motion abnormalities. There was mild bileaflet mitral valve prolapse with mild mitral regurgitation and mitral annular disjunction measuring 0.7 cm. Cardiac MRI was obtained and demonstrated biventricular dilation (consistent with athlete’s heart) with evidence of MAD. The myocardium was normal with no evidence of late enhancement. She underwent dual-chamber cardioverter-defibrillator implantation for secondary prevention. Discussion: Mitral annular disjunction is a structural abnormality involving atrial displacement of the mitral annulus, leading to an abnormal separation between the posterior mitral valve leaflet and basal ventricular myocardium during systole. Etiology of MAD remains unclear. It is estimated to be present in 8.7% of the general population and in over 30% of individuals with mitral valve prolapse. This case presents an example of a previously healthy patient with sudden cardiac arrest. After extensive workup, MAD was identified as the most likely trigger for her new onset arrhythmia. This case highlights the severe, and potentially fatal, arrhythmias that can arise from MAD. It is important to recognize MAD seen on echocardiograms and counsel patients on potential complications. Patients with MAD and increased risk factors should undergo further evaluation with cardiac MRI and/or electrophysiology studies.

Post-cardiac catheterization infective endocarditis with flail mitral valve leaflets: an interesting and rare case

Infectious endocarditis (IE) occurs after the colonization of the cardiac endocardium by virulent microorganisms, usually bacteria. Infective endocarditis causes significant morbidity and mortality, even in this era of antibiotics. Here we present a 52-year-old male patient with hypertension and ischemic heart disease who presented with shivering, fever, and confusion for two days, fourteen days after PCI. On further assessment, transesophageal echocardiography was done due to the patient’s fulfillment of 3 minor and 1 major of Duke's criteria. Flail posterior mitral valve leaflet of 1.1x0.60cm vegetation, and severe eccentric mitral regurgitation were observed in the transesophageal echocardiography.&#x0D; Post PCI infective endocarditis is rare and missed diagnosis so it should be haven high suspension for those present with signs and symptoms compatible with infective endocarditis.&#x0D; Keywords: PCI, infective endocarditis, vegetation, echocardiography, PCI, mitral regurgitation&#x0D;

A Dual-Pathogen Mitral Valve Endocarditis Caused by Coxiella burnetii and Streptococcus gordonii-Which Came First?

Infective endocarditis (IE) is still a life-threatening disease with high morbidity and mortality. While usually caused by a single bacterium, poly-microbial infective endocarditis (IE) is rare. Here, we report a (blood-culture-negative) dual pathogen mitral valve IE caused by Coxiella burnetii and Streptococcus gordonii: A 53-year-old woman was presented to an internal medicine department with abdominal pain for further evaluation. Within the diagnostic work up, transthoracic echocardiography (TTE) revealed an irregularly shaped echogenic mass (5 × 13 mm) adherent to the edge of the posterior mitral valve leaflet and protruding into the left atrium. As infected endocarditis was suspected, blood cultures were initially obtained, but they remained negative. Chronic Q fever infection was diagnosed using serologic testing. After the occurrence of cerebral thromboembolic events, the patient was admitted for mitral valve surgery. Intraoperatively, a massively destructed mitral valve with adhering vegetations was noted. Examination of the mitral valve by broad-range bacterial polymerase chain reaction (PCR) and amplicon sequencing confirmed Coxiella burnetii infection and yielded Streptococcus gordonii as the second pathogen. Based on the detailed diagnosis, appropriate antibiotic therapy of both pathogens was initiated, and the patient could be discharged uneventfully on the 11th postoperative day after a successful minimal-invasive mitral valve replacement.

Neochordae implantation versus leaflet resection in mitral valve posterior leaflet prolapse and dilated left ventricle: a propensity score matching comparison with long-term follow-up.

Uncorrected severe mitral regurgitation (MR) due to posterior prolapse leads to left ventricular dilatation. At this stage, mitral valve repair becomes mandatory to avoid permanent myocardial injury. However, which technique among neochoardae implantation and leaflet resection provides the best results in this scenario remains unknown. We selected 332 patients with left ventricular dilatation and severe degenerative MR due to posterior leaflet (PL) prolapse who underwent neochoardae implantation (85 patients) or PL resection (247 patients) at our institution between 2008 and 2020. A propensity score matching analysis was carried on to decrease the differences at baseline. Matching yielded 85 neochordae implantations and 85 PL resections. At 10 years, freedom from cardiac death and freedom from mitral valve reoperation were 92.6 ± 6.1% vs 97.8 ± 2.1% and 97.7 ± 2.2% vs 95 ± 3% in the neochordae group and in the PL resection group, respectively. The MR ≥2+ recurrence rate was 23.9 ± 10% in the neochordae group and 20.8 ± 5.8% in the PL resection group (P = 0.834) at 10 years. At the last follow-up, the neochordae group showed a higher reduction of left ventricular end-diastolic diameter (44 vs 48 mm; P = 0.001) and a better ejection fraction (60% vs 55%; P < 0.001) compared to PL resection group. In this subgroup of patients, both neochordae implantation and leaflet resection provide excellent durability of the repair in the long term. Neochordae implantation might have a better effect on dilated left ventricle.

Perforated posterior mitral valve leaflet in mitral annular disjunction and infective endocarditis.

We herein presented a 17-year-old female with history of mild mitral valve prolapse who was admitted for methicillin-sensitive Staphylococcus aureus endocarditis and diagnosed with mitral annular disjunction and perforated posterior mitral valve leaflet on two-dimensional and three-dimensional echocardiography (P1-P2). A perforation in the posterior leaflet was confirmed and repaired during surgical intervention. This is a rare presentation of leaflet perforation in the area of mitral annular disjunction.

Reparación de la válvula mitral sin circulación extracorpórea con neocuerdas por vía transapical. Experiencia inicial

IntroductionTransapical Off-Pump mitral valve repair with transapical neochord implantation (TOP-MINI), is safe and effective for the treatment of primary mitral regurgitation. The propose of this study is to present our initial experience with this procedure. MethodsAll patients who underwent TOP-MINI between march 2017 and January 2022 were included. Perioperative safety and efficacy outcomes were evaluated. Short-term survival and freedom from mitral regurgitation at follow-up were also analyzed. Results55 patients were included, all with degenerative mitral regurgitation. Procedural success was achieved in 87,3%. At 24 months of follow-up, survival was 92,6%. In patients with procedural success, 77,1% had recurrent MR ≤ II/IV; 6,3% III/IV and 16,7% IV/IV. A significant reduction in left ventricular telesystolic diameter was also observed (36,07mm vs. 32,84mm; p= 0.035). ConclusionsTransapical neochord implantation is safe and effective for the treatment of degenerative mitral regurgitation with limited disease of the posterior mitral valve leaflet.

Valve disease in cardiac amyloidosis: an echocardiographic score

Cardiac amyloidosis (CA) may affect all cardiac structures, including the valves. From 423 patients undergoing a diagnostic workup for CA we selected 2 samples of 20 patients with amyloid transthyretin (ATTR-) or light-chain (AL-) CA, and age- and sex-matched controls. We chose 31 echocardiographic items related to the mitral, aortic and tricuspid valves, giving a value of 1 to each abnormal item. Patients with ATTR–CA displayed more often a shortened/hidden and restricted posterior mitral valve leaflet (PMVL), thickened mitral chordae tendineae and aortic stenosis than those with AL–CA, and less frequent PMVL calcification than matched controls. Score values were 15.8 (13.6–17.4) in ATTR–CA, 11.0 (9.3–14.9) in AL–CA, 12.8 (11.1–14.4) in ATTR–CA controls, and 11.0 (9.1–13.0) in AL–CA controls (p = 0.004 for ATTR- vs. AL–CA, 0.009 for ATTR–CA vs. their controls, and 0.461 for AL–CA vs. controls). Area under the curve values to diagnose ATTR–CA were 0.782 in patients with ATTR–CA or matched controls, and 0.773 in patients with LV hypertrophy. Patients with ATTR–CA have a prominent impairment of mitral valve structure and function, and higher score values. The valve score may help identify patients with ATTR–CA among patients with CA or unexplained hypertrophy.

An Unusual Case of Corynebacterium simulans causes Infective Endocarditis with Embolic Events

Corynebacterium species are often treated as contaminants. However, there were several recent outbreaks involving Corynebacterium species reported across the globe. We report a rare case of Corynebacterium simulans causes infective endocarditis which led to debilitating embolic events. A 39-year-old lady presented with a prolonged fever for three weeks. She was in septic shock and had dense right hemiplegia, a pansystolic murmur at the mitral area and hepatosplenomegaly. There were no peripheral stigmata of infective endocarditis. An urgent computed tomography (CT) of the brain showed an infarct at the left corona radiata extending to the left parietal, left basal ganglia and left temporal lobe. She deteriorated and required mechanical ventilation and intensive care. Echocardiogram showed vegetation at both her anterior and posterior mitral valve leaflets leading to severe mitral regurgitation. Splenic infarction was seen in her contrasted computed tomography (CECT) of her abdomen. Her three samples of blood culture were positive for Corynebacterium simulans. She deteriorated rapidly and went into multi-organ failure and succumbed. Corynebacterium species should be taken seriously and should not be neglected as a contaminant as it can cause potentially fatal complications as described in this case report.

Surgery for mitral annular caseous calcification-related calcified amorphous tumor: a case report

BackgroundA calcified amorphous tumor (a non-neoplastic tumor) with caseous calcification of the mitral annulus is a rare pathology that causes severe embolic events. We present a rare case of mitral valve surgery for a mitral annular caseous calcification-related calcified amorphous tumor found in cerebral infarction.Case presentationA 69-year-old man was diagnosed with a mitral valve calcified amorphous tumor with mitral annular caseous calcification found in cerebral infarction. He was admitted because of acute multiple embolic cerebral infarctions. A search for the embolic source through transesophageal echocardiography revealed a mitral valve tumor raised from the posterior mitral valve leaflet on the side of the left ventricle. Computed tomographic cardiac angiography revealed a calcified mitral valve tumor invading the posterior mitral valve annulus and left ventricular muscle. Intraoperative findings revealed a mitral annular calcification-related calcified amorphous tumor with caseous calcification of the posterior leaflet and annulus, which was suspected. Therefore, we performed radical debridement of the mitral annular calcification and bioprosthetic mitral valve replacement with patch repair of the posterior mitral valve annulus 2 weeks after the onset of cerebral infarction. The patient recovered well post operation, without any embolic events.ConclusionsA calcified amorphous tumor with caseous mitral annulus calcification may be highly associated with embolic events. In this case, mitral valve replacement with annular patch repair may be a favorable procedure for preventing embolic events.

Posterior leaflet of mitral valve—Is it really tri-scalloped? —A morphological and morphometric study in North Indian cadaveric hearts

Posterior leaflet of mitral valve—Is it really tri-scalloped? —A morphological and morphometric study in North Indian cadaveric hearts

809 A CASE OF CASEOUS MITRAL ANNULUS CALCIFICATION

Abstract Caseous mitral annular calcification (CMAC) is a rare variant of degenerative mitral annular calcification (MAC) with an echocardiographic prevalence of 0.6% of all MACs and an overall prevalence of up to 0.07% in the general population. It primarily affects older patients with hypertension and chronic kidney disease. Due to the general benign prognosis, conservative management of this lesion is recommended in most cases. Herein we describe the case of a 78-year-old female patient admitted for sustained ventricular tachycardia treated with Amiodaron infusion followed by electrical cardioversion for hemodynamically instability. Her past medical history was remarkable for hypertension, HFpEF and chronic kidney disease. Trans-thoracic echocardiogram showed the presence of an echo-dense mass with central echo-lucency surrounded by calcifications at the posterior peri-annular and leaflet region of the mitral valve, not associated with valvular disfunction. To define the etiology of the arrythmia, a coronary angiography was performed and excluded significant coronary artery disease. Nevertheless, a round-shape hyperdense mass was observed in the left atrium near the atrio-ventricular groove (figure 1): during injection through coronary vessels the contrast was not visible in the lesion. In order to better define the nature of the mass, a Trans-esophageal Echocardiogram was performed and showed a “toothpaste-like” echo-dense mass with central echo-lucency surrounded by a calcified envelope at the posterior peri-annular region of the mitral valve suggestive for CMAC (figure 2); 3D zoom imaging clarified its relationship with P2 scallop of posterior mitral valve leaflet (figure 3). For the best tissue characterization and to definitely exclude other mass-like lesions, such as myxoma, papillary fibroelastoma, myocardial abscess, vegetations, lipomatous hypertrophy, Cardiac Magnetic Resonance was performed and showed low-signal intensity in both T1- and T2-weighted sequences, reflecting its calcium content. First-pass perfusion sequence reveals no contrast enhancement, whereas late gadolinium enhancement depicts a peripheral rim of enhancement confirming our suspect. In Conclusion, CMAC is a rare disorder requiring a multimodal imaging approach to differentiate it with other intra-cardiac masses. Its course is generally benign and whether not associated with valvular or hemodynamic significance, it could be considered as a bystander. Figure 1 Figure 2 Figure 3