Insuficiencia mitral congénita en el adulto: reporte de un caso y revisión de la literatura / Congenital mitral regurgitation in adults: a case report and literature review

Congenital mitral valve disease is a rare form of mitral regurgitation. The etiological diagnosis is often challenging. The transthoracic echocardiogram is presented as a good initial approach method. The case of a 29-year-old patient referred for the diagnosis of severe congenital mitral regurgitation in parachute is presented. This report aims to illustrate the clinical and echocardiographic presentation of congenital mitral regurgitation.

Left Ventricular Remodeling Shortly after Open Mitral Valve Replacement for Rheumatic Mitral Stenosis

Abstract Introduction: Left ventricular dysfunction after surgical treatment of mitral stenosis is uncommon. We intend to determine the pattern of left ventricular remodeling, shortly after open mitral valve replacement for rheumatic mitral stenosis, with in-hospital postoperative outcomes and the determinants of postoperative worsening of left ventricular ejection fraction. Methods: From January 2008 to January 2015, 107 adult patients with rheumatic mitral stenosis were submitted to open mitral valve replacement. Their mean age was 45±11 years and 93 (86.9%) were women. Left ventricular morphology and function were studied longitudinally with echocardiography. The end point was postoperative worsening of left ventricular ejection fraction, defined by a decrease of 10% compared to preoperative basal assessment. Determinants of worsening left ventricular ejection fraction were determined by multivariable logistic regression analysis. Results: The end point occurred in 18 patients (16.8%). We tested clinical and echocardiographic parameters to verify independent variables related to the decrease in postoperative ejection fraction. Lower body weight (P=0.005; odds ratio [OR]=0.89) and smaller preoperative mitral valve area (P=0.02; OR=0.02) were independent predictors of left ventricular dysfunction. These patients presented higher mortality and morbidity rates. Conclusion: Left ventricular remodeling patterns differed among patients with predominant rheumatic mitral stenosis undergoing open mitral valve replacement. Lower preoperative body weight and mitral valve area were independent determinants of deteriorating ejection fraction with increased end-systolic volumes, indicating that this specific problem may occur in anthropometric smaller patients with more extensive rheumatic disease.

Mitral Valve Fenestration as a Rare Cause of Congenital Mitral Insufficiency Successfully Repaired

Abstract A rare case of congenital mitral insufficiency characterized by a fenestration in the anterior leaflet of mitral valve is reported. At operation, the mitral valve was successfully repaired by closure of unusual valvular tissue orifice with bovine pericardium and suture of the free edge between A1 and A2 without a ring annuloplasty.

Caracterização Histológica das Lesões da Valva Mitral de Pacientes com Cardiopatia Reumática / Histopathological Characterization of Mitral Valvular Lesions from Patients with Rheumatic Heart Disease

Resumo Fundamentos: Os mecanismos subjacentes pelos quais a doença cardíaca reumática (DCR) levam à disfunção valvar grave não são totalmente compreendidos. Objetivo: O presente estudo avaliou as alterações histopatológicas nas valvas mitrais (VM) buscando uma associação entre o padrão de disfunção valvar predominante e os achados histopatológicos. Métodos: Em 40 pacientes submetidos à troca da VM devido a DCR e em 20 controles submetidos a transplante cardíaco, foram analisados os aspectos histológicos da VM excisada. Dados clínicos e ecocardiográficos também foram coletados. As análises histológicas foram realizadas usando coloração com hematoxilina-eosina. Determinou-se inflamação, fibrose, neoangiogênese, calcificação e metaplasia adiposa. Valores de p<0,05 foram considerados estatisticamente significativos. Resultados: A idade média dos pacientes com DCR foi de 53±13 anos, sendo 36 (90%) do sexo feminino, enquanto a idade média dos controles foi de 50±12 anos, semelhante aos casos, sendo a maioria do sexo masculino (70%). O endocárdio valvar reumático apresentou espessura maior que os controles (1,3±0,5 mm versus 0,90±0,4 mm, p=0,003, respectivamente), e infiltrado inflamatório mais intenso no endocárdio (78% versus 36%; p=0,004), com predominância de células mononucleares. Ocorreu fibrose moderada a acentuada mais frequentemente em válvulas reumáticas do que em válvulas controle (100% vs. 29%; p<0,001). Ocorreu calcificação em 35% das valvas reumáticas, principalmente entre as valvas estenóticas, associada à área valvar mitral (p=0,003). Conclusões: Apesar do intenso grau de fibrose, o processo inflamatório permanece ativo na valva mitral reumática, mesmo em doença tardia com disfunção valvar. A calcificação predominou em valvas estenóticas e em pacientes com disfunção ventricular direita.

Abstract Background: The underlying mechanisms by which rheumatic heart disease (RHD) lead to severe valve dysfunction are not completely understood. Objective: The present study evaluated the histopathological changes in mitral valves (MV) seeking an association between the pattern of predominant valvular dysfunction and histopathological findings. Methods: In 40 patients who underwent MV replacement due to RHD, and in 20 controls that underwent heart transplant, histological aspects of the excised MV were analyzed. Clinical and echocardiographic data were also collected. Histological analyses were performed using hematoxylin-eosin staining. Inflammation, fibrosis, neoangiogenesis, calcification and adipose metaplasia were determined. A p value<0.05 was considered to be statistically significant. Results: The mean age of RHD patients was 53±13 years, 36 (90%) were female, whereas the mean age of controls was 50±12 years, similar to the cases, with the majority of males (70%). The rheumatic valve endocardium presented greater thickness than the controls (1.3±0.5 mm versus 0.90±0.4 mm, p=0.003, respectively), and a more intense inflammatory infiltrate in the endocardium (78% versus 36%; p=0.004), with predominance of mononuclear cells. Moderate to marked fibrosis occurred more frequently in rheumatic valves than in control valves (100% vs. 29%; p<0.001). Calcification occurred in 35% of rheumatic valves, especially among stenotic valves, which was associated with the mitral valve area (p=0.003). Conclusions: Despite intense degree of fibrosis, the inflammatory process remains active in the rheumatic mitral valve, even at late disease with valve dysfunction. Calcification predominated in stenotic valves and in patients with right ventricular dysfunction.

Kissing lesion of mitral valve - a case report

Abstract Aortic valve endocarditis can lead to secondary involvement of aorto-mitral curtain and the adjacent anterior mitral leaflet (AML). The secondary damage to AML is often caused by the infected jet of aortic regurgitation hitting the ventricular surface of the mitral leaflet, or by the pronounced bacterial vegetation that prolapses from the aortic valve into the left ventricular outflow tract. This is called 'kissing lesion'. We describe a patient with infective endocarditis of the aortic valve causing perforation of both noncoronary cusp of aortic valve and the AML, which is rare.

Comparison of early and long-term follow-up results of percutaneous mitral balloon valvuloplasty and mitral valve replacement

SUMMARY BACKGROUND: Percutaneous mitral balloon valvuloplasty and mitral valve replacement have been the treatment options for mitral stenosis for several years, however, studies that compare these two modalities are very rare in the literature. ObjectIve: In this article, we aim to investigate the comparison of clinical results of percutaneous mitral balloon valvuloplasty and mitral valve replacement. Methods: 527 patients with rheumatic mitral stenosis, treated with percutaneous mitral balloon valvuloplasty or mitral valve replacement (276 patients with percutaneous mitral balloon valvuloplasty and 251 patients with mitral valve replacement) from 1991 to 2012 were evaluated. The demographic characteristics, clinical, echocardiographic and catheterization data of patients were evaluated retrospectively. The results of early and late clinical follow-up of patients after percutaneous mitral balloon valvuloplasty and mitral valve replacement were also evaluated. Results: The mean follow-up time of the percutaneous mitral balloon valvuloplasty group was 4.7 years and, for the mitral valve replacement-group, it was 5.45 years. The hospital stay of the percutaneous mitral balloon valvuloplasty group was shorter than that of the mitral valve replacement group (2.02 days vs 10.62 days, p<0.001). The hospital mortality rate of percutaneous mitral balloon valvuloplasty and mitral valve replacement were 0% and 2% respectively (p=0.024). In the percutaneous mitral balloon valvuloplasty group, early postprocedural success rate was 92.1%. The event-free survival of percutaneous mitral balloon valvuloplasty and mitral valve replacement was found to be similar. While reintervention was higher in percutaneous mitral balloon valvuloplasty-group (p<0.001), mortality rate was higher in mitral valve replacement-group (p<0.001). Conclusion: Percutaneous mitral balloon valvuloplasty seems to be more advantageous than mitral valve replacement due to low mortality rates, easy application of the procedure and no need for general anesthesia.

Functional mitral regurgitation combined with increased early diastolic transmitral velocity to early mitral annulus diastolic velocity ratio is associated with a poor prognosis in patients with shock / 中华医学杂志(英文版)

BACKGROUND@#Functional mitral regurgitation (FMR) is common in critically ill patients and may cause left atrial (LA) pressure elevation. This study aims to explore the prognostic impact of synergistic LA pressure elevation and FMR in patients with shock.@*METHODS@#We retrospectively screened 130 consecutive patients of 175 patients with shock from April 2016 to June 2017. The incidence and impact of FMR and early diastolic transmitral velocity to early mitral annulus diastolic velocity ratio (E/e') ≥ 4 within 6 h of shock on the prognosis of patients were evaluated. Finally, the synergistic effect of FMR and E/e' were assessed by combination, grouping, and trend analyses.@*RESULTS@#Forty-four patients (33.8%) had FMR, and 15 patients (11.5%) had E/e' elevation. A multivariate analysis revealed FMR and E/e' as independent correlated factors for 28-day mortality (P = 0.043 and 0.028, respectively). The Kaplan-Meier survival analysis revealed a significant difference in survival between patients with and without FMR (χ2 = 7.672, P = 0.006) and between the E/e' ≥ 14 and E/e' < 14 groups (χ2 = 19.351, P < 0.010). Twenty-eight-day mortality was significantly different among the four groups (χ2 = 30.141, P < 0.010). The risk of 28-day mortality was significantly higher in group 4 (E/e' ≥ 14 with FMR) compared with groups 1 (E/e' < 14 without FMR) and 2 (E/e' < 14 with FMR) (P = 0.001 and 0.046, respectively).@*CONCLUSIONS@#Patients with shock can be identified by the presence of FMR. FMR and E/e' are independent risk factors for a poor prognosis in these patients, and prognosis is worst when FMR and E/e' ≥ 14 are present. It may be possible to improve prognosis by reducing LA pressure and E/e'.@*TRIAL REGISTRATION@#ClinicalTrials.gov, NCT03082326.

Massa Valvar Mitral em Paciente com Suspeita de Lúpus Sistêmico: Tumor, Endocardite ou Ambos? / Mitral Valve Mass in a Patient Suspected of Systemic Lupus: Tumor, Endocarditis or Both?

Resumo Apresentamos o relato de caso de uma paciente com mixoma valvar mitral infectado e uma revisão da literatura sobre o assunto. Uma mulher de 33 anos apresentou histórico de febre e dispneia com evolução de alguns dias. Na hospitalização, ela apresentava uma síndrome semelhante ao lúpus, com hemoculturas positivas para Haemophilus spp . O ecocardiograma revelou uma massa gigante envolvendo ambos os folhetos mitrais associada à regurgitação grave, necessitando de troca valvar mitral biológica. A microscopia revelou mixoma infectado e a paciente recebeu alta assintomática após o término da antibioticoterapia. Ela apresentou bons resultados no seguimento. Este é o sexto caso de mixoma valvar mitral infectado relatado na literatura e o terceiro caso de mixoma cardíaco infectado pelo grupo HACEK. Devido à alta incidência de eventos embólicos, a antibioticoterapia precoce aliada à pronta intervenção cirúrgica são decisivos para a redução da morbimortalidade. O tempo para o diagnóstico foi muito mais breve do que o geralmente relatado em casos de endocardite por HACEK. A troca valvar foi a intervenção mais comum e todos os pacientes em relatos de caso anteriores apresentaram bons resultados no seguimento.

Abstract We present a case report of a patient with an infected mitral valve myxoma and a literature review on the subject. A 33-year-old female presented with a history of fever and dyspnea evolving over a few days. On admission, she had a lupus-like syndrome with positive blood cultures for Haemophilus species . Echocardiogram revealed a giant mass involving both mitral leaflets causing severe regurgitation, requiring biological mitral valve replacement. Microscopy showed an infected myxoma and the patient was discharged asymptomatic upon completion of antibiotics. She did well on follow-up. This is the sixth case of an infected mitral valve myxoma reported in the literature and the third case of a cardiac myxoma infected by the HACEK group. Exceedingly high incidence of embolic events makes prompt imaging, antibiotic therapy and surgery crucial for better outcomes. Time to diagnosis was much briefer than usually reported in other cases of HACEK endocarditis. Valve replacement was the most common surgical procedure and all patients from previous reports did well on follow-up.

Implante Percutâneo Transeptal de Bioprótese em Disfunção de Prótese Valvar Cirúrgica Mitral - Experiência Multicêntrica Brasileira / Percutaneous Transseptal Bioprosthetic Implantation in Failed Prosthetic Surgical Mitral Valve - Brazilian Multicenter Experience

Resumo Fundamento A intervenção percutânea em pacientes com disfunção de prótese biológica mitral apresenta-se como uma alternativa ao tratamento cirúrgico convencional. Objetivo Relatar a primeira experiência brasileira de implante transcateter de bioprótese valve-in-valve mitral via transeptal (TMVIV-via transeptal). Métodos Foram incluídos pacientes portadores de disfunção de bioprótese cirúrgica submetidos ao TMVIV-transeptal em 12 hospitais brasileiros. Foram considerados estatisticamente significativos valores de p<0,05. Resultados Entre junho/2016 e fevereiro/2019, 17 pacientes foram submetidos ao TMVIV-via transeptal. A mediana de idade foi 77 anos (IIQ,70-82), a mediana do escore STS-PROM foi 8,7% (IIQ,7,2-17,8). Todos os pacientes tinham sintomas limitantes de insuficiência cardíaca (CF≥III), tendo 5 (29,4%) sido submetidos a mais de uma toracotomia prévia. Obteve-se sucesso do TMVIV-via transeptal em todos os pacientes. A avaliação ecocardiográfica demonstrou redução significativa do gradiente médio (pré-intervenção, 12±3,8 mmHg; pós-intervenção, 5,3±2,6 mmHg; p<0,001), assim como aumento da área valvar mitral (pré-intervenção, 1,06±0,59 cm2; pós-intervenção, 2,18±0,36 cm2; p<0,001) sustentados em 30 dias. Houve redução significativa e imediata da pressão sistólica de artéria pulmonar, com redução adicional em 30 dias (pré-intervenção, 68,9±16,4 mmHg; pós-intervenção, 57,7±16,5 mmHg; 30 dias, 50,9±18,7 mmHg; p<0,001). Durante o seguimento, com mediana de 162 dias (IIQ, 102-411), observou-se marcada melhora clínica (CF≤II) em 87,5%. Um paciente (5,9%) apresentou obstrução de via de saída de ventrículo esquerdo (VSVE), evoluindo para óbito logo após o procedimento, e outro morreu aos 161 dias de seguimento.Conclusão: A primeira experiência brasileira de TMVIV-transeptal demonstra a segurança e a efetividade dessa nova técnica. A obstrução da VSVE é uma complicação potencialmente fatal, reforçando a importância da seleção dos pacientes e do planejamento do procedimento. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0)

Abstract Background Percutaneous intervention in patients with bioprosthetic mitral valve dysfunction is an alternative to conventional surgical treatment. Objectives To report the first Brazilian experience with transseptal transcatheter bioprosthetic mitral valve-in-valve implantation (transseptal-TMVIV). Methods Patients with surgical bioprosthetic dysfunction submitted to transseptal-TMVIV in 12 Brazilian hospitals were included. The significance level adopted was p<0.05. Results From June/2016 to February/2019, 17 patients underwent transseptal-TMVIV. Their median age was 77 years (IQR,70-82) and median Society of Thoracic Surgeons predicted risk of mortality (STS-PROM) score was 8.7% (IQR,7.2-17.8). All patients had limiting symptoms of heart failure (FC≥III) and 5 (29.4%) had undergone more than one previous thoracotomy. Transseptal-TMVIV was successful in all patients. Echocardiographic assessment showed a significant reduction in mean mitral valve gradient (pre-intervention, 12±3.8 mmHg; post-intervention, 5.3±2.6 mmHg; p<0.001), in addition to an increase in mitral valve area (pre-intervention, 1.06±0.59 cm2; post-intervention, 2.18±0.36 cm2; p<0.001) sustained for 30 days. There was a significant and immediate reduction in the pulmonary artery systolic pressure, with an additional reduction in 30 days (pre-intervention, 68.9±16.4 mmHg; post-intervention, 57.7±16.5 mmHg; 30 days, 50.9±18.7 mmHg; p<0.001). During follow-up (median, 162 days; IQR, 102-411), significant clinical improvement (FC≤II) was observed in 87.5% of the patients. One patient (5.9%) had left ventricular outflow tract (LVOT) obstruction and died right after the procedure, and another died at 161 days of follow-up. Conclusion The first Brazilian experience with transseptal-TMVIV shows the safety and effectivity of the new technique. The LVOT obstruction is a potentially fatal complication, reinforcing the importance of patients' selection and of procedural planning. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0)

Evaluation of left atrial function in asymptomatic dogs with chronic myxomatous mitral valve disease: two-dimensional feature-tracking echocardiography and Simpsons monoplanar methods / Avaliação da função atrial esquerda em cães assintomáticos com degeneração mixomatosa crônica de valva mitral: métodos ecocardiográficos bidimensional feature tracking e monoplanar de Simpson

The present study evaluated the volume and function of the left atrium by two-dimensional echocardiographic feature-tracking imaging (2D-FTI) and Simpson's monoplanar modeling in dogs with asymptomatic degenerative mitral valve disease (DMVD). The study consisted of 80 dogs that were divided into the following three groups: Group 1, 21 dogs (A); Group 2, 30 dogs (B1) and Group 3, 29 dogs (B2). The variable strain (contraction phase) was significantly lower in Group 3 than in Group 1 (12.92±4.54 x 16.69±5.74, p=0.014), and significant differences in the contraction strain index (CSI) were observed between all of the groups that were evaluated (1 = 46.82±8.10, 2 = 39.88±8.03, 3 = 35.25±5.64, p<0.0001). The atrial diastolic volume index (AdVi) that was measured by 2D-FTI was significantly higher in Group 3 than in Group 1 (1.31±0.95 x 0.96±0.31, p=0.038), and the atrial cardiac index (ACI) was also higher in Group 3 than in Group 1 (102.38±80.18 x 78.19±33.38, p=0.030). Atrial function was assessed by Simpson's monoplanar method, which demonstrated an increase in the left atrial systolic volume, while the contractile function decreased with an increasing disease severity (Group 1 0.21±0.06; Group 2 0.25±0.06; Group 3 0.32±0.08, p<0.0001). The intraobserver and interobserver assessments showed low to moderate variability; most of the values for the coefficient of variation for the variables that were analysed with each method were below 25%. Thus, DMVD was determined to cause an alteration in atrial function, especially in the contraction phase, and even in asymptomatic animals, and the methods of 2D-FTI echocardiography and Simpson's monoplanar evaluation are sensitive and early methods for the detection of left atrial dysfunction.(AU)

O presente estudo avaliou o volume e a função atrial esquerda obtidos por meio da ecocardiografia bidimensional feature tracking (2D-FTI) e pelo método monoplanar de Simpson em cães saudáveis e cães com DMVD assintomáticos. Foram avaliados 80 cães distribuídos em três grupos: Grupo 1, 21 cães (classe A); Grupo 2, 30 cães (classe B1) e Grupo 3, 29 cães (classe B2). A variável strain (fase de contração) foi significativamente menor no Grupo 3 que no Grupo 1 (12,92±4,54 x 16,69±5,74, p=0,014) e para a variável índice de strain de contração (CSI), houve diferença estatística entre todos os grupos avaliados (1 = 46,82±8,10; 2 = 39,88±8,03; 3 = 35,25±5,64, p<0,0001). O índice de volume diastólico atrial (iVdA) mensurado por meio do 2D-FTI foi significativamente maior no Grupo 3 que no Grupo 1 (1,31±0,95 x 0,96±0,31, p=0,038), assim como para o índice cardíaco atrial (iCA) também foi maior no Grupo 3 (102,38±80,18 x 78,19±33,38, p=0,030). A função atrial avaliada pelo método monoplanar de Simpson demonstrou um aumento do volume atrial esquerdo e do volume sistólico do átrio esquerdo, enquanto que a função contrátil diminuiu com o aumento da gravidade da doença (Grupo 1 0,21±0,06; Grupo 2 0,25±0,06; Grupo 3 0,32±0,08; p<0,0001). A avaliação intraobservador e interobservador, demonstrou variabilidade baixa a moderada, uma vez que a maioria dos valores de coeficiente de variação se concentraram abaixo de 25% para as variáveis analisadas em ambos os métodos. Dessa forma, conclui-se que a DMVD causa alteração na função atrial, principalmente na fase de contração, mesmo em animais assintomáticos e que a ecocardiografia 2D-FTI e o método monoplanar de Simpson são métodos sensíveis e precoces na detecção da disfunção atrial esquerda.(AU)

Cardiac magnetic resonance analysis of mitral annular dynamics after mitral valve repair

OBJECTIVES: The aim of this study was to analyze mitral annulus (MA) dynamics using cardiac magnetic resonance (CMR) in patients with degenerative mitral insufficiency who underwent mitral valve repair (MVR). METHODS: Mitral valve imaging was performed by CMR in twenty-nine patients with degenerative mitral insufficiency who underwent MVR between July 2014 and August 2016, with quadrangular resection of the posterior leaflet without ring annuloplasty. They were prospectively followed up from the preoperative period up to 2 years postoperatively. RESULTS: We observed a significant reduction in all measurements of the MA after surgery. The mean systolic circumference of the MA was reduced from 13.28±1.95 cm to 11.50±1.59 cm, and the diastolic circumference was reduced from 12.51±2.01 cm to 10.66±2.09 cm in the immediate postoperative period, measures that remained stable 2 years after MVR (p<0.001). The mean maximum area of the MA was significantly reduced from 14.34±4.03 to 10.45±3.17 cm2 when comparing the immediate postoperative period and the 2 year follow-up (p<0.001). The same occurred with the mean minimum area of the MA, which was reduced from 12.53±3.68 cm2 to 9.23±2.84 cm2 in the same period, and this reduction was greater in the antero-posterior diameter than in the mid-lateral diameter. The mobility of the MA was preserved after surgery, ranging between 19.6% and 25.7% at 2-year follow-up. CONCLUSION: We observed a significant reduction in the MA size after MVR, with preservation of the MA mobility at the 2-year follow-up.