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.

Plastia mitral en endocarditis infecciosa: reporte de un caso / Mitral valve repair in infective endocarditis: a case report / Plastia mitral em endocardite infecciosa: relato de um caso

La endocarditis infecciosa es una patología heterogénea con una alta mortalidad y requiere tratamiento quirúrgico en al menos la mitad de los casos. Cuando asienta en posición mitral, la reparación valvular en lugar de su sustitución, si bien representa un desafío técnico, ha ido ganando terreno en los últimos años. Describimos el caso de un paciente que se presentó con una endocarditis sobre válvula nativa mitral en quien se realizó una plastia valvular exitosa. Revisaremos la evidencia acerca de su beneficio.

Infective endocarditis is a heterogeneous disease with a high mortality and that requires surgical treatment in at least half of cases. When seated in mitral position, valve repair rather than replacement, while technically challenging, has been gaining popularity in recent years. We describe the case of a patient who presented with a mitral valve endocarditis in whom a successful valve repair was performed. Evidence supporting its use will be reviewed.

A endocardite infecciosa é uma doença heterogênea com alta mortalidade que requer tratamento cirúrgico em pelo menos metade dos casos. Quando sentado na posição mitral, o reparo da válvula, em vez da substituição da válvula, embora seja um desafio técnico, tem ganhado espaço nos últimos anos. Descrevemos o caso de um paciente que apresentou endocardite valvar mitral nativa, no qual foi realizada plastia valvar com sucesso. Vamos revisar as evidências sobre o seu benefício.

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.

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.

Insuficiencia Mitral Severa secundaria a "Cleft" Mitral Aislado: revisión a partir de un caso clínico / Severe Mitral Insufficiency secondary to Isolated Mitral Cleft: Review from a clinical case

Resumen El "cleft" mitral es un defecto congénito raro que se encuentra comúnmente asociado a otras patologías no obstante, en raras ocasiones puede encontrarse como un defecto aislado. Dicho hallazgo es de suma importancia desde el punto de vista clínico ya que es una causa reparable de insuficiencia mitral, sin necesidad de someterse a reemplazo valvular. Su diagnóstico es primariamente mediante ecocardiografía, en la que el uso del 3D como técnica permite la correcta delimitación de la extensión de la lesión, así como la valoración a detalle del resto del aparato valvular mitral. Presentamos un caso de Insuficiencia Mitral severa en la infancia de causa no clara en un inicio, en la cual, por medio de ecocardiografía transtorácica 3D se logró identificar una hendidura aislada de la valva anterior como etiología y descartar otras posibles etiologías y/o mecanismos.

Abstract Severe Mitral Insufficiency secondary to Isolated Mitral Cleft: Review from a clinical case Severe mitral valve reguritacion secondary to an Isolated cleft of the mitral valve: a review based on a clinical case Mitral Cleft is a rare congenital defect that is commonly associated with others pathologies; nevertheless, in rare occasions it can be found as an isolated defect. This finding is paramount from clinical perspective as it is a reparable defect and not necessarily ends in valvular replacement. Its assessment is primarily by transthoracic echocardiography with the addition of 3D technology that provides a more detailed delimitation of the extent of the lesion, as well as detailed assessment of the whole mitral valve apparatus. In this article, we present a case of severe mitral regurgitation without a clear ethiology at glance, but posteriorly identified with the aid of transthoracic 3D echocardiography, as an isolated mitral cleft and also, allowed to rule out other possible etiologies and mechanisms.

Efectos clínicos de la corrección del valor de la fracción de expulsión del ventrículo izquierdo en pacientes con insuficiencia valvular mitral / Clinical impact of the correction of the value of left ventricular ejection fraction in patients with mitral valve regurgitation

Resumen Introducción y objetivos: La insuficiencia valvular mitral provoca el vaciado simultáneo hacia la aorta y la aurícula izquierda durante la sístole ventricular, lo que produce una disminución del volumen hacia la circulación sistémica. En este estudio se busca obtener un dato preciso del porcentaje de volumen expulsado en sentido anterógrado en pacientes con insuficiencia mitral. Métodos: Se aplica una fórmula ecocardiográfica de “corrección” de la fracción de expulsión del ventrículo izquierdo (FEVI) en 114 pacientes con insuficiencia mitral, con base en la medición de la fracción regurgitante. Resultados: La corrección de la FEVI demostró que el 44.7% de los casos (n = 51) debe reclasificarse en cuanto a la calidad de su función sistólica ventricular izquierda. De 79 sujetos con FEVI normal (≥ 50%) sólo se mantuvieron 32 en la misma categoría; en el grupo con FEVI moderadamente reducida (intervalo intermedio, 40-49.9%) se pasó de 6 a 23 casos y, en aquéllos con FEVI reducida (< 40%), el grupo aumentó de 29 a 59; el subgrupo de pacientes con FEVI < 30% se incrementó de 21 a 41 sujetos. Conclusiones: Puesto que en la mayoría de las guías de tratamiento la FEVI se usa para estratificar riesgos e indicaciones terapéuticas, los autores creen que la ponderación de la insuficiencia mitral puede incrementar la precisión del tratamiento y la posibilidad de incluir a pacientes que no están considerados en esos tratamientos en el momento actual.

Abstract Introduction and objectives: Mitral valve regurgitation causes simultaneous emptying to the aorta and left atrium during ventricular systole, generating a decrease in volume supply to the systemic circulation. In this study we seek to obtain an accurate data on the percentage of volume expelled in the anterograde direction in patients with mitral regurgitation. Methods: An echocardiographic formula for “correction” of the left ventricular ejection fraction (LVEF) was applied in 114 patients with mitral regurgitation, based on the measurement of the regurgitant fraction. Results: Correction of the LVEF showed that 44.7% of cases (n = 51) should be reclassified in terms of the quality of their left ventricular systolic function. Of 79 subjects with normal LVEF (≥ 50%) only 32 remained in the same category; in the group with moderately reduced LVEF (medium range, 40-49.9%) it went from 6 to 23 cases and, in those with reduced LVEF (< 40%), the group increased from 29 to 59; the subgroup of patients with LVEF < 30% increased from 21 to 41 subjects. Conclusions: Given that in most treatment guidelines LVEF is used to stratify risks and therapeutic indications, the authors believe that the weighting of mitral regurgitation can increase the accuracy of treatment, and the possibility of including patients who, at this current moment, are not considered for these therapies.

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.

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.

Síndrome de Ehlers-Danlos hipermóvel: um relato de caso / Hypermobile Ehlers-Danlos syndrome: a case report

A síndrome de Ehlers-Danlos é estabelecida por distúrbios hereditários do tecido conjuntivo que tem como manifestações principais a hipermobilidade articular, a hiperextensibilidade da pele e a fragilidade de tecidos, como articulações, ligamentos, pele, vasos sanguíneos e órgãos internos. São reconhecidos 13 subtipos, de acordo com Classificação Internacional de 2017. Dentre estes, abordamos o hipermóvel, cujo diagnóstico é eminentemente clínico, com manifestações sistêmicas distintas. Esse artigo refere-se ao caso de uma paciente diagnosticada com síndrome de Ehlers-Danlos hipermóvel, tendo como intuito a atualização acerca dos novos critérios diagnósticos, assim como o diagnóstico precoce de tal raropatia.

Ehlers-Danlos syndrome is established through hereditary disorders of connective tissue, and has as its manifestations: joint hypermobility, skin hyperextensibility, and fragility of tissues such as joints, ligaments, skin, blood vessels, and internal organs. Thirteen subtypes have been recognized according to the 2017 International Classification. Among these, the hypermobile type, the diagnosis of which is eminently clinical, with distinct systemic manifestations, will be addressed. This article refers to the case of a patient diagnosed with hypermobile Ehlers-Danlos syndrome, with the objective of updating the new diagnostic criteria, as well as the early diagnosis of such a rare disease.