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CLINICAL DATA: Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect. Technical description: Echocardiographic findings compatible with Barlow's disease and atrial septal defect, ostium secundum type. OPERATION: She was submitted to mitral valvuloplasty with chordal shortening and prosthetic posterior ring (Gregori-Braile®) along with patch atrioseptoplasty. COMMENTS: Mitral valve regurgitation is a rare congenital heart disease and Barlow's disease is probably rarer. Mitral valve repair is the treatment of choice.

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Resumen En Costa Rica, el Reglamento de la Autopsia Hospitalaria y Médico Legal establece que la autopsia médico legal es obligatoria para toda muerte súbita. La patología cardíaca es responsable aproximadamente del 80 % de las muertes súbitas que requieren una autopsia forense; el prolapso de la válvula mitral (degeneración mixomatosa de la válvula mitral) es una de las formas más comunes de valvulopatía cardíaca, es relativamente común (2%-3% de la población general), y a menudo se considera benigno, la tasa anual de muerte cardíaca súbita (MSC) en individuos con MVP (0,2%-0,4% /año) es aproximadamente el doble que el observado en la población general (0,1%-0,2% año).

Abstract In Costa Rica, the Hospital and Legal Autopsy Regulations establish that a legal medical autopsy is mandatory for all sudden deaths. Cardiac pathology is responsible for approximately 80% of sudden deaths requiring a forensic autopsy; Mitral valve prolapse (myxomatous mitral valve degeneration) is one of the most common forms of heart valve disease, is relatively common (2%-3% of the general population), and is often considered benign, the annual rate of Sudden cardiac death (SCD) in individuals with MVP (0.2%-0.4%/year) is approximately twice that observed in the general population (0.1%-0.2%/year).

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INTRODUCTION: Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor movement across the valve, functional obstruction across the valve, and severe pulmonary hypertension secondary to chronic obstruction. A common belief among surgeons is that excision of myxoma leads to abatement of symptoms, and further valve intervention may not be warranted. METHODS: A 10-year retrospective descriptive study was designed to analyze patients who underwent excision of cardiac myxoma at our center. Data was analyzed regarding presenting features, echocardiographic findings of myxoma and valve morphology, intraoperative assessment, and postoperative outcome with/without valve repair/replacement in all patients. RESULTS: A total of 22 patients underwent surgery for myxoma. Six patients underwent successful mitral valve repair with ring annuloplasty, two had moderate mitral regurgitation, three had severe mitral regurgitation, and one patient had no mitral regurgitation on preoperative assessment, but moderate mitral regurgitation was found intraoperatively. Four of these patients had no residual mitral regurgitation in follow-up period while two had mild residual mitral regurgitation. One patient had severe mitral stenosis of concurrent rheumatic etiology and successfully underwent mitral valve replacement. CONCLUSION: Cardiac myxomas are rare benign tumors commonly associated with mitral valve insufficiency. Mitral valve should be assessed intraoperatively after excision of mass as preoperative assessment might often be insufficient. Concomitant mitral valve intervention might be needed with a case-specific tailored approach, and mitral valve repair with ring annuloplasty offers best surgical outcome in such cases.

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ABSTRACT Introduction: Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor movement across the valve, functional obstruction across the valve, and severe pulmonary hypertension secondary to chronic obstruction. A common belief among surgeons is that excision of myxoma leads to abatement of symptoms, and further valve intervention may not be warranted. Methods: A 10-year retrospective descriptive study was designed to analyze patients who underwent excision of cardiac myxoma at our center. Data was analyzed regarding presenting features, echocardiographic findings of myxoma and valve morphology, intraoperative assessment, and postoperative outcome with/without valve repair/replacement in all patients. Results: A total of 22 patients underwent surgery for myxoma. Six patients underwent successful mitral valve repair with ring annuloplasty, two had moderate mitral regurgitation, three had severe mitral regurgitation, and one patient had no mitral regurgitation on preoperative assessment, but moderate mitral regurgitation was found intraoperatively. Four of these patients had no residual mitral regurgitation in follow-up period while two had mild residual mitral regurgitation. One patient had severe mitral stenosis of concurrent rheumatic etiology and successfully underwent mitral valve replacement. Conclusion: Cardiac myxomas are rare benign tumors commonly associated with mitral valve insufficiency. Mitral valve should be assessed intraoperatively after excision of mass as preoperative assessment might often be insufficient. Concomitant mitral valve intervention might be needed with a case-specific tailored approach, and mitral valve repair with ring annuloplasty offers best surgical outcome in such cases.

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ABSTRACT Clinical data: Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect. Technical description: Echocardiographic findings compatible with Barlow's disease and atrial septal defect, ostium secundum type. Operation: She was submitted to mitral valvuloplasty with chordal shortening and prosthetic posterior ring (Gregori-Braile®) along with patch atrioseptoplasty. Comments: Mitral valve regurgitation is a rare congenital heart disease and Barlow's disease is probably rarer. Mitral valve repair is the treatment of choice.

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ABSTRACT Introduction: The impact of mitral regurgitation (MR) on valve-in-valve transcatheter aortic valve implantation (VIV-TAVI) in patients with failed bioprostheses remains unclear. The purpose of this study was to assess the prognostic impact of residual moderate MR following VIV-TAVI. Methods: We retrospectively analyzed 127 patients who underwent VIV-TAVI between March 2010 and November 2021. At least moderate MR was observed in 51.2% of patients before the procedure, and MR improved in 42.1% of all patients. Patients with postoperative severe MR, previous mitral valve intervention, and patients who died before postoperative echocardiography were excluded from further analyses. The remaining 114 subjects were divided into two groups according to the degree of postprocedural MR: none-mild MR (73.7%) or moderate MR (26.3%). Propensity score matching yielded 23 pairs for final comparison. Results: No significant differences were found between groups before and after matching in early results. In the matched cohort, survival probabilities at one, three, and five years were 95.7% vs. 87.0%, 85.0% vs. 64.5%, and 85.0% vs. 29.0% in the none-mild MR group vs. moderate MR-group, respectively (log-rank P=0.035). Among survivors, patients with moderate MR had worse functional status according to New York Heart Association (NYHA) class at follow-up (P=0.006). Conclusion: MR is common in patients with failed aortic bioprostheses, and improvement in MR-status was observed in over 40% of patients following VIV-TAVI. Residual moderate MR after VIV-TAVI is not associated with worse early outcomes, however, it was associated with increased mortality at five years of follow-up and worse NYHA class among survivors.

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In recent decades, the advances in the management of functional mitral regurgitation (MR), makes knowledge of this disease extremely important to the clinical cardiologist, as it has an increasing prevalence in patients older than 50 years. The objective of this article is to review the pathophysiological bases of MR, in order to differentiate its mechanism, and to correlate them with the clinical, hemodynamic, and imaging findings that assess the severity of the disease. These data are relevant for the adequate selection of the patient and the opportune moment of the therapeutic interventions, which involves a multidisciplinary team in decision-making to achieve the greatest benefit within the treatment options of this pathology.

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INTRODUCTION: The impact of mitral regurgitation (MR) on valve-in-valve transcatheter aortic valve implantation (VIV-TAVI) in patients with failed bioprostheses remains unclear. The purpose of this study was to assess the prognostic impact of residual moderate MR following VIV-TAVI. METHODS: We retrospectively analyzed 127 patients who underwent VIV-TAVI between March 2010 and November 2021. At least moderate MR was observed in 51.2% of patients before the procedure, and MR improved in 42.1% of all patients. Patients with postoperative severe MR, previous mitral valve intervention, and patients who died before postoperative echocardiography were excluded from further analyses. The remaining 114 subjects were divided into two groups according to the degree of postprocedural MR: none-mild MR (73.7%) or moderate MR (26.3%). Propensity score matching yielded 23 pairs for final comparison. RESULTS: No significant differences were found between groups before and after matching in early results. In the matched cohort, survival probabilities at one, three, and five years were 95.7% vs. 87.0%, 85.0% vs. 64.5%, and 85.0% vs. 29.0% in the none-mild MR group vs. moderate MR-group, respectively (log-rank P=0.035). Among survivors, patients with moderate MR had worse functional status according to New York Heart Association (NYHA) class at follow-up (P=0.006). CONCLUSION: MR is common in patients with failed aortic bioprostheses, and improvement in MR-status was observed in over 40% of patients following VIV-TAVI. Residual moderate MR after VIV-TAVI is not associated with worse early outcomes, however, it was associated with increased mortality at five years of follow-up and worse NYHA class among survivors.

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A insuficiência mitral moderada a grave é observada em 17 a 35% dos pacientes submetidos a implante transcateter de válvula aórtica. Estudo que reporta a insuficiência mitral pós- realização de implante transcateter de válvula aórtica por estenose aórtica demonstra que 50% dos pacientes com refluxo moderado a grave apresentaram melhora da regurgitação, e 8,7% evidenciam piora do quadro. Nesses pacientes com piora, houve aumento da mortalidade. Essa progressão sugere que condutas convencionais, baseadas em otimização medicamentosa, podem não ser capazes de prevenir quadros negativos. Relatamos um caso sobre a evolução da insuficiência mitral após implante transcateter de válvula aórtica e o uso do MitraClip® como alternativa de tratamento e benefícios.

Moderate to severe mitral regurgitation is observed in 17 to 35% of patients undergoing transcatheter aortic valve implantation. A study reporting mitral regurgitation after transcatheter aortic valve implantation due to aortic stenosis demontrated 50% of patients with moderate to severe reflux showed improvement in regurgitation, and 8.7% showed worsening of the condition, which led to increased mortality. This progression suggested conventional management, based on medication optimization, may not be able to prevent poor outcomes. We report a case on the clinical course of a patient with mitral regurgitation after transcatheter aortic valve implantation, and the use of MitraClip® as an alternative treatment and its benefits.

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ABSTRACT Severe functional mitral valve regurgitation should be treated in patients undergoing myocardial revascularization. When replacement is considered the best therapeutic option, preservation of the mitral subvalvular apparatus is crucial, especially in the emergency setting, because of its primary role in preserving geometry and function of left and right ventricles. Here we present a simple and quick technique, where subvalvular apparatus is preserved in toto in patients undergoing mitral valve replacement with a bioprosthesis.

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Severe functional mitral valve regurgitation should be treated in patients undergoing myocardial revascularization. When replacement is considered the best therapeutic option, preservation of the mitral subvalvular apparatus is crucial, especially in the emergency setting, because of its primary role in preserving geometry and function of left and right ventricles. Here we present a simple and quick technique, where subvalvular apparatus is preserved in toto in patients undergoing mitral valve replacement with a bioprosthesis.

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Abstract Introduction: This study examines early- and long-term outcomes of mitral valve repairs in a low-volume cardiac surgery centre in the Caribbean. Methods: Ninety-six consecutive patients underwent mitral valve repair from April 2009 to December 2018. Patients were divided into two groups: functional mitral regurgitation requiring simple mitral annuloplasty (FMR, n=63) or structural degenerative mitral regurgitation requiring more complex repair (DMR, n=33). Data collected prospectively were retrospectively analysed from the unit-maintained cardiac surgery database. Results: Thirty-day mortality in the whole series was 2.1%, with 3% in the FMR group and 0% in the DMR group. Early post-operative echocardiography in the FMR group demonstrated 51 patients (83.6%) without mitral regurgitation, 8 patients (13.1%) with trivial to mild regurgitation, and 2 patients (3.3%) with moderate regurgitation. However, at a mean follow-up of 98.2±50.8, only 21 patients (42.8%) were in NYHA class I, with 7 (14.2%) in class II, 16 (32.6%) in class III, and 5 (10.2%) in class IV. There were 9 cardiac-related deaths at final follow-up, with freedom from re-operation and survival of 98% and 75.6%, respectively. In the DMR group, early post-operative echocardiography demonstrated 29 patients (87.9%) without mitral regurgitation, 3 patients (9.1%) with trivial regurgitation and 1 patient (3.0%) with mild regurgitation. At a mean follow-up of 114.1±25.4 months, there was a good functional post-operative status in this group with 93.3% in NYHA class I, and 6.7% in class II. No patient required reintervention, 96.3% of patients had mild or no mitral regurgitation and survival was 90.9%. Conclusion: Despite challenges of maintaining skills in a low-volume centre, mitral valve repair can be performed safely with good early- and long-term results.

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RESUMEN Introducción: La insuficiencia mitral funcional (IMF) es común en pacientes con insuficiencia cardíaca (IC). La IMF moderada/ grave (M/G) se asocia a peor pronóstico. Objetivo: Describir la prevalencia de IMF y los mecanismos involucrados en su reducción en respondedores a la terapia de resincronización cardíaca (TRC) a los 6 meses comparados con 12 y 24 meses. Métodos: Entre 2009 y 2018 fueron tratados 338 pts. Respondedores: reducción de CF NYHA ≥1 grado o aumento de la fracción de eyección ventricular izquierda (FEVI) ≥5% (absoluto). La IMF se graduó en 4 puntos: No-IMF, leve, M y G, y se la relacionó con las mediciones ecocardiográficas. Características Basales: edad 64 ±10 años, hombres 71%, CF-NYHA IIIII 92%, bloqueo de rama izquierda (BRI) 67%, QRS ≥150 ms 75%, diámetro diastólico del VI (DDVI) 68 ± 9 mm, diámetro sistólico del VI (DSVI) 52 ± 12 mm, FEVI 24 ± 7%. Resultados: La prevalencia de IMF fue del 92,6%. A los 6 meses, 86% fueron respondedores y 23% de ellos mejoraron de IMF-M/G a IMF-Leve/No-IMF. Hubo un fuerte remodelado inverso: DDVI 68 ± 10 vs 63 ± 11 mm, (p = 0,0001), DSVI 55 ± 12 vs 50 ± 13 mm, (p = 0,0006) y FEVI 25 ± 11 vs 33 ± 10%, (p = 0,00001). Comparando 6 con 12 meses 89,4% fueron respondedores, 8% mejoraron de IMF-M/G a IMF-Leve/No-IMF. Comparando 6 con 24 meses 88% fueron respondedores, 14,6% mejoraron de IMF-M/G a IMF-Leve/No-IMF. Entre 6 y 12 y 6 y 24 meses no hubo remodelado inverso significativo. Conclusiones: La prevalencia de IMF fue elevada. El mayor remodelado inverso y reducción de la IMF se observaron a los 6 meses, siendo el primero el principal mecanismo en la reducción de la IMF. Esta mejoría se sostuvo a los 12 y 24 meses.

ABSTRACT Background: Functional mitral regurgitation (FMR) is common in heart failure, and moderate/severe (M/S) FMR is associated with worse prognosis. Objective: The aim of this study was to describe the prevalence of FMR and the mechanisms involved in its reduction in responders to cardiac resynchronization therapy (CRT) at 6 months compared with 12 and 24 months. Methods: Between 2009 and 2018, 338 patients received CRT. Patients showing NYHA functional class (FC) reduction ≥1 or left ventricular ejection fraction (LVEF) absolute increase ≥5% were considered responders. Functional mitral regurgitation was graded using a 4-point scale into none-, mild-, M- and S-FMR, and was related to echocardiographic measurements. Baseline patient characteristics were: age 64±10 years, men 71%, NYHA FC II-III 92%, left bundle branch block (LBBB) 67%, QRS ≥150 ms 75%, LV diastolic diameter (LVDD) 68±9 mm, LV systolic diameter (LVSD) 52±12 mm, and LVEF 24±7%. Results: The prevalence of FMR was 92.6%. At 6 months, 86% were responders, 23% improved from M/S-FMR to mild/none-FMR and there was strong reverse remodeling: LVDD 68±10 vs. 63±11 mm, (p=0.0001), LVSD 55±12 vs. 50±13 mm, (p=0.0006) and LVEF 25±11 vs. 33±10%, (p=0.00001). Comparing 6 with 12 months, 89.4% were responders and 8% improved M/S-FMR to mild/none-FMR. Comparing 6 with 24 months, 88% were responders and 14.6% improved M/S-FMR to mild/none-FMR. Between 6 and 12 and 6 and 24 months, there was no significant reverse remodeling. Conclusions: The prevalence of FMR was high. The highest reverse remodeling and FMR reduction was observed at 6 months, the former being the main mechanism of FMR reduction. This improvement persisted at 12 and 24 months.

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Apresentamos o caso debutante na América Latina de reparos transcateteres edge- to-edge nas valvas mitral e tricúspide em um único procedimento em paciente de alto risco para cirurgia. A paciente evoluía com insuficiência cardíaca e limitações em suas atividades diárias quando foi submetida ao procedimento, no qual foram realizados os reparos transcateteres das valvas mitral e tricúspide. Aos 6 meses de evolução, estava assintomática e realizando suas atividades diárias. Debatemos as recomendações mais atualizadas sobre a terapia transcateter para coexistência dessas doenças valvares, assim como expomos as lacunas no conhecimento.

We present the first case in Latin America of transcatheter edge-to-edge mitral and tricuspid valve repair in a single procedure, in a patient at high risk for surgery. The patient progressed to heart failure and limitation in her daily living activities when she was submitted to transcatheter mitral and tricuspid valve repair. Six months later, she was asymptomatic and performing her daily living activities. We discuss the most updated recommendations for transcatheter repair when both valvar conditions coexist, and show the knowledge gaps.

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INTRODUCTION: This study examines early- and long-term outcomes of mitral valve repairs in a low-volume cardiac surgery centre in the Caribbean. METHODS: Ninety-six consecutive patients underwent mitral valve repair from April 2009 to December 2018. Patients were divided into two groups: functional mitral regurgitation requiring simple mitral annuloplasty (FMR, n=63) or structural degenerative mitral regurgitation requiring more complex repair (DMR, n=33). Data collected prospectively were retrospectively analysed from the unit-maintained cardiac surgery database. RESULTS: Thirty-day mortality in the whole series was 2.1%, with 3% in the FMR group and 0% in the DMR group. Early post-operative echocardiography in the FMR group demonstrated 51 patients (83.6%) without mitral regurgitation, 8 patients (13.1%) with trivial to mild regurgitation, and 2 patients (3.3%) with moderate regurgitation. However, at a mean follow-up of 98.2±50.8, only 21 patients (42.8%) were in NYHA class I, with 7 (14.2%) in class II, 16 (32.6%) in class III, and 5 (10.2%) in class IV. There were 9 cardiac-related deaths at final follow-up, with freedom from re-operation and survival of 98% and 75.6%, respectively. In the DMR group, early post-operative echocardiography demonstrated 29 patients (87.9%) without mitral regurgitation, 3 patients (9.1%) with trivial regurgitation and 1 patient (3.0%) with mild regurgitation. At a mean follow-up of 114.1±25.4 months, there was a good functional post-operative status in this group with 93.3% in NYHA class I, and 6.7% in class II. No patient required reintervention, 96.3% of patients had mild or no mitral regurgitation and survival was 90.9%. CONCLUSION: Despite challenges of maintaining skills in a low-volume centre, mitral valve repair can be performed safely with good early- and long-term results.

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A disjunção do anel mitral (DAM) é uma inserção anormal da linha de flexão do anel mitral na parede atrial. O anel mostra uma separação (disjunção) entre a junção folheto posterior-parede atrial e a crista miocárdica ventricular esquerda.1 A DAM foi descrita pela primeira vez há mais de 30 anos em estudo de autópsia, estando relacionada com prolapso da valva mitral (PVM) em 92% dos casos.2 Desde então, foram realizados diversos estudos, sendo a prevalência de DAM em pacientes com PVM reportada de forma variável, podendo ou não estar associada à insuficiência mitral. O ecocardiograma transtorácico (ETT) faz parte da avaliação inicial do prolapso valvar mitral, permitindo o diagnóstico e a avaliação de complicações relacionadas. Com a evolução de novos métodos diagnósticos, a ressonância magnética cardíaca (RMC) e o ecocardiograma transesofágico (ETE) passaram a aprimorar a avaliação dessa patologia, bem como de sua extensão e localização. Contudo, as características fenotípicas do PVM que estão mais associadas a DAM permanecem incertas, sobretudo devido ao número limitado de pacientes, nos estudos clássicos sobre o tema. Portadores de DAM podem desenvolver sintomas relacionados a arritmias ventriculares, configurando a síndrome arrítmica da DAM (SDAM), podendo evoluir para morte súbita. Na literatura, os dados prognósticos ainda são conflitantes entre os diversos estudos acerca do tema, indo desde critérios claros de diagnóstico, o melhor método de imagem a ser aplicado, o tratamento e o prognóstico. Esta revisão descreve as características da DAM associada ou não ao prolapso valvar, auxiliando no diagnóstico e na conduta dessa importante patologia. (AU)

Mitral annulus disjunction (MAD) is an abnormal insertion of the flexion line of the mitral annulus into the atrial wall. The annulus presents a separation (disjunction) between the posterior leaflet­atrial wall junction and the left ventricular myocardial crest.1 MAD was first described more than 30 years ago in an autopsy study and is reportedly related to mitral valve prolapse (MVP) in 92% of cases.2 Since then, several studies have been conducted, and reports on the prevalence of MAD in patients with MVP have varied. Ultimately, it may or may not be associated with mitral regurgitation. Transthoracic echocardiography is part of initial MVP assessment, allowing its diagnosis and the assessment of related complications. As new diagnostic methods emerged, cardiac magnetic resonance imaging and transesophageal echocardiography improved the assessment of this pathology in terms of its diagnosis, extension, and location. However, the phenotypic characteristics of MVP that are more closely associated with MAD remain uncertain mainly due to the limited number of patients in classic studies on the subject. Patients with MAD may develop symptoms related to ventricular arrhythmias, configuring the MAD arrhythmic syndrome, which may progress to sudden death. The literature presents conflicting prognostic data among several studies on the subject from clear diagnostic criteria and best imaging method to be used to treatment and prognosis. This review describes MAD characteristics associated (or not) with valve prolapse to improve the diagnosis and management of this important pathology. (AU)

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