Disjunção do anel mitral: modalidades diagnósticas, implicações clínicas e evolução prognóstica / Mitral annulus disjunction: diagnostic modalities, clinical implications, and prognostic progression

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)

Tiger stripes sign in acute coronary syndrome / Signo manchas de tigre en síndrome coronario agudo

Abstract The incidence of mitral regurgitation in acute myocardial syndromes is variable. Echocardiographic evaluation is fundamental in making a proper diagnosis of mechanical complications and to offer timely treatment. We present a case of a 64-year-old male who was admitted to the ER in acute pulmonary edema. The electrocardiogram showed negative ST-segment deviation from V4-V6, positive ST-segment deviation in aVR. Multivessel disease with severe mitral regurgitation was seen in catheterization. A transthoracic echocardiogram revealed important mitral regurgitation showing the "tiger stripes" sign, seen in the presence of intracardial oscillating structures, in this case, suspected papillary muscle rupture. Echocardiographic evaluation is necessary in every case of myocardial infarction who present with new-onset mitral regurgitation. Treatment is complex and must be determined with an interdisciplinary group.

Evolução Doppler Ecocardiográfica das Lesões Valvares Mitral e Aórtica em Crianças e Adolescentes com Cardite Reumática Subclínica e Leve / Doppler Echocardiographic Follow-Up of Mitral and Aortic Regurgitation in Children and Adolescents with Subclinical and Mild Rheumatic Carditis

Fundamento: As cardites reumáticas leve e subclínica se diferenciam basicamente pela ausculta de sopro regurgitativo mitral. A evolução destas formas não está bem estabelecida na literatura. Objetivo: Avaliar a evolução das cardites reumáticas leve e subclínica, considerando as valvites mitral e/ou aórtica (fase aguda) e a regressão, manutenção ou piora delas ao final do seguimento (fase crônica). Métodos: Estudo retrospectivo, longitudinal, incluindo pacientes com cardites reumáticas leve e subclínica. A evolução ecocardiográfica das valvites mitral e/ou aórtica foi comparada nos dois grupos, considerando a análise ao final do seguimento. Foram utilizados o teste qui quadrado e as curvas de sobrevida de Kaplan-Meier, com nível de significância p < 0,05. Resultados: Foram incluídos 125 pacientes, sendo 69 (55,2%) com cardite reumática subclínica e 56 (44,8%) com cardite reumática leve, com média de idade na fase aguda de 10,4 ± 2,6 anos e, ao final do estudo, de 19,9 ± 4,6 anos. O tempo de seguimento variou de 2 a 23 anos (média: 9,38 ± 4,3 anos). Na fase aguda, a regurgitação mitral leve/ moderada ou moderada foi mais frequente nos pacientes com cardite reumática leve (p = 0,001). A regurgitação aórtica leve ou leve/moderada também foi mais comum no grupo de cardite reumática leve (p = 0,045). Na fase crônica, observou-se que tanto a regurgitação mitral (p < 0,0001) quanto a regurgitação aórtica (p = 0,009) foram mais frequentes nos pacientes com cardite reumática leve, e a sobrevida livre de valvopatia residual foi maior no grupo de cardite reumática subclínica (p = 0,010). A regurgitação mitral residual foi maior no grupo de cardite reumática leve p < 0,0001), e a regurgitação aórtica residual foi semelhante nos dois grupos (p = 0,099). Conclusão: A resolução da regurgitação mitral foi maior nos pacientes com cardite reumática subclínica, e a involução da regurgitação aórtica foi menos frequente e semelhante nos dois grupos

Background: Mild rheumatic carditis (MRC) and subclinical rheumatic carditis (SRC) are basically differentiated through auscultation of mitral regurgitation murmur. The evolution of these forms is not well established in the literature. Objective: To evaluate the evolution of mild and subclinical rheumatic carditis, considering mitral and aortic regurgitation (acute phase) and regression, maintenance or worsening of these diseases at the end of follow-up (chronic phase). Methods: Retrospective, longitudinal study, including patients with mild and subclinical rheumatic carditis. The echocardiographic evolution of mitral and aortic regurgitation was compared in both groups, considering the analysis at the end of follow-up. The Chi-square test and Kaplan-Meier survival curves were used, with significance level established at p < 0.05. Results: A total of 125 patients were included, 69 (55.2%) with subclinical rheumatic carditis and 56 (44.8%) with mild rheumatic carditis, with a mean age in the acute phase of 10.4 ± 2.6 years and, at the end of study, 19.9 ± 4.6 years. The time of follow-up ranged from 2 to 23 years (mean: 9.38 ± 4.3 years). In the acute phase, mild/moderate or moderate mitral regurgitation was more frequent in patients with mild rheumatic carditis (p = 0.001). Mild or mild/moderate aortic regurgitation was also more common in the mild rheumatic carditis group (p = 0.045). In the chronic phase, we observed that both mitral (p < 0.0001) and aortic regurgitation (p = 0.009) were more frequent in patients with mild rheumatic carditis, and survival free of rheumatic heart disease was higher in the subclinical rheumatic carditis group (p = 0.010). Residual mitral regurgitation was higher in the mild rheumatic carditis group p < 0.0001), and residual aortic regurgitation was similar in both groups (p = 0.099). Conclusion: Mitral regurgitation resolution was higher in patients with subclinical rheumatic carditis, and the involution of aortic regurgitation was less frequent and similar in both groups

Parameters indicative of persistence of valvular pathology at initial diagnosis in acute rheumatic carditis: the role of albumin and CD19 expression / Parâmetros indicativos de persistência de patologia valvular no diagnóstico inicial de cardite reumática aguda: o papel da albumina e da expressão de CD19

Abstract Objective: The aim of this study is to define the predictors of chronic carditis in patients with acute rheumatic carditis (ARC). Methods: Patients diagnosed with ARC between May 2010 and May 2011 were included in the study. Echocardiography, electrocardiography, lymphocyte subset analysis, acute phase reactants, plasma albumin levels, and antistreptolysin-O (ASO) tests were performed at initial presentation. The echocardiographic assessments were repeated at the sixth month of follow-up. The patients were divided into two groups according to persistence of valvular pathology at 6th month as Group 1 and Group 2, and all clinical and laboratory parameters at admission were compared between two groups of valvular involvement. Results: During the one-year study period, 22 patients had valvular disease. Seventeen (77.2%) patients showed regression in valvular pathology. An initial mild regurgitation disappeared in eight patients (36.3%). Among seven (31.8%) patients with moderate regurgitation initially, the regurgitation disappeared in three, and four patients improved to mild regurgitation. Two patients with a severe regurgitation initially improved to moderate regurgitation (9.1%). In five (22.8%) patients, the grade of regurgitation [moderate regurgitation in one (4.6%), and severe regurgitation in 4 (18.2%)] remained unchanged. The albumin level was significantly lower at diagnosis in Group 2 (2.6 ± 0.48 g/dL). Lymphocyte subset analysis showed a significant decrease in the CD8 percentage and a significant increase in CD19 percentage at diagnosis in Group 2 compared to Group 1. Conclusion: The blood albumin level and the percentage of CD8 and CD19 (+) lymphocytes at diagnosis may help to predict chronic valvular disease risk in patients with acute rheumatic carditis.

Resumo Objetivo: Definir os preditores da cardite crônica em pacientes com cardite reumática aguda (CRA). Métodos: Os pacientes diagnosticados com CRA entre maio de 2010 e maio de 2011 foram incluídos no estudo. Foram feitos os testes de ecocardiografia, eletrocardiograma, uma análise do subgrupo de linfócitos, provas de fase aguda, níveis de albumina plasmática, antiestreptolisina-O (ASO) na manifestação inicial. As avaliações ecocardiográficas foram repetidas no 6º mês de acompanhamento. Os pacientes foram divididos em dois grupos de acordo com a persistência da patologia valvular no 6º mês como Grupo 1 e Grupo 2 e todos os parâmetros clínicos e laboratoriais na internação foram comparados entre dois grupos de comprometimento valvular. Resultados: Durante o período do estudo de um ano, 22 pacientes apresentaram doença valvular; 17 (77,2%) apresentaram regressão da patologia valvular. Houve desaparecimento de regurgitação moderada inicial em oito pacientes (36,3%). Entre sete (31,8%) pacientes com regurgitação moderada inicialmente, a regurgitação desapareceu em três e quatro apresentaram melhoria para regurgitação leve. Dois pacientes com regurgitação grave inicialmente apresentaram melhoria para regurgitação moderada (9,1%). Em cinco (22,8%) pacientes o grau de regurgitação (regurgitação moderada em um [4,6%] e regurgitação grave em quatro [18,2]) continuou inalterado. O nível de albumina foi significativamente menor no diagnóstico no Grupo 2 (2,6 ± 0,48 gr/dL). A análise do subgrupo de linfócitos mostrou uma redução significativa no percentual de CD8 e um aumento significativo no percentual de CD19 no Grupo 2 em comparação com o Grupo 1. Conclusão: O nível de albumina no sangue e o percentual de linfócitos CD8 e CD19 (+) no diagnóstico podem ajudar a prever risco de doença valvular crônica em pacientes com cardite reumática aguda.

Mitral valve prolapse in pregnancy

Abstract Mitral valve prolapse is a benign condition. Mitral regurgitation is only complicated in patients with severe mitral valve prolapse. Women with mitral valve prolapse in the absence of other cardiovascular disorders tolerate pregnancy well and do not develop remarkable cardiac complications. Nevertheless, serious complications of mitral valve prolapse, including arrhythmia, infective endocarditis and cerebral ischemic events, can be present in pregnancy. Debates remain with regard to the use of prophylactic antibiotics and β-blockers in the pregnant women with mitral valve prolapse. The prognosis of the pregnant patients might be closely related to the pathological and (or) functional changes of the mitral valve. Non-myxomatous mitral valve prolapse poses no or little obstetric risks in terms of pregnancy, labor and neonatal complications; whereas myxomatous mitral valve prolapse is a major etiology of valvular heart disease in women of childbearing age. In the pregnant patients with mitral valve prolapse progressing into major complications, surgical interventions are considered. Medicinal treatment of such patients with β-blockers should be a concern for the fetal safety.

Dilatación aneurismática de la aurícula izquierda / Giant left atrium

Mujer de 48 años con síntomas progresivos de disnea, fatiga, disfagia, edema de miembros inferiores, palpitaciones y disminución de su capacidad física que le obligan a buscar atención médica. La radiografía de tórax muestra cardiomegalia grave o <

Woman 48 years old with progressive dyspnea, fatigue, dysphagia, lower limb edema, palpitations and decreased physical ability that made her to seek medical attention. The chest film showed severe cardiomegaly or <

Obstructive membrane in arch of aorta in a case of Shone’s complex.

Shone’s complex is a rare congenital heart disease consisting of multisite obstruction on the left side of the heart. The obstructive membrane in the arch of aorta is never described among these obstructions. We report echocardiographic findings in a patient with Shone’s complex with the obstructive membrane in the arch of aorta.

Geometria da valva mitral derivada da ressonância magnética cardiovascular na avaliação da gravidade da regurgitação mitral / Cardiovascular magnetic resonance imaging-derived mitral valve geometry in determining mitral regurgitation severity

FUNDAMENTO: A regurgitação mitral é a doença valvar cardíaca mais comum em todo o mundo. A ressonância magnética pode ser uma ferramenta útil para analisar os parâmetros da valva mitral. OBJETIVO: diferenciar padrões geométricos da valva mitral em pacientes com diferentes gravidades por regurgitação mitral (RM) com base na ressonância magnética cardiovascular. MÉTODOS: Sessenta e três pacientes foram submetidos à ressonância magnética cardiovascular. Os parâmetros da valva mitral analisados foram: área (mm2) e ângulo (graus) de tenting, altura do ventrículo (mm), altura do tenting (mm), folheto anterior, comprimento posterior do folheto (leaflet) e diâmetro do anulo (mm). Os pacientes foram divididos em dois grupos, um incluindo pacientes que necessitaram de cirurgia da valva mitral e o outro os que não. RESULTADOS: Trinta e seis pacientes apresentaram de RM discreta a leve (1-2+) e 27 RM de moderada a grave (3-4+). Dez (15,9%) dos 63 pacientes foram submetidos à cirurgia. Pacientes com RM mais grave tiveram maior diâmetro sistólico final do ventrículo esquerdo (38,6 ± 10,2 vs. 45,4 ± 16,8, p < 0,05) e diâmetro diastólico final esquerdo (52,9 ± 6,8 vs. 60,1 ± 12,3, p = 0,005). Na análise multivariada, a área de tenting foi a determinante mais forte de gravidade de RM (r = 0,62, p = 0,035). Comprimento do anulo (36,1 ± 4,7 vs. 41 ± 6,7, p< 0,001), área de tenting (190,7 ± 149,7 vs. 130 ± 71,3, p= 0,048) e comprimento do folheto posterior (15,1 ± 4,1 vs. 12,2 ± 3,5, p= 0,023) foram maiores em pacientes que precisaram de cirurgia da valva mitral. CONCLUSÕES: Área de tenting, anulo e comprimento do folheto posterior são possíveis determinantes da gravidade da RM. Estes parâmetros geométricos podem ser usados para individualizar a gravidade e, provavelmente, no futuro, orientar o tratamento do paciente com base na anatomia individual do aparelho mitral.

BACKGROUND: Mitral regurgitation is the most common valvular heart disease worldwide. Magnetic resonance may be a useful tool to analyze mitral valve parameters. OBJECTIVE: To distinguish mitral valve geometric patterns in patients with different severities of mitral regurgitation (MR) based on cardiovascular magnetic resonance imaging. METHODS: Sixty-three patients underwent cardiovascular magnetic resonance imaging. Mitral valve parameters analyzed were: tenting area (mm2) and angle (degrees), ventricle height (mm), tenting height (mm), anterior leaflet, posterior leaflet length and annulus diameter (mm). Patients were divided into two groups, one including patients who required mitral valve surgery and another which did not. RESULTS: Thirty-six patients had trace to mild (1-2+) MR and 27 had moderate to severe MR (3-4+). Ten (15.9%) out of 63 patients underwent surgery. Patients with more severe MR had a larger left ventricle end systolic diameter (38.6 ± 10.2 vs 45.4 ± 16.8, p<0.05) and left end diastolic diameter (52.9 ± 6.8 vs 60.1 ± 12.3, p= 0.005). On multivariate analysis, the tenting area was the strongest determinant of MR severity (r= 0.62, p=0.035). Annulus length (36.1 ± 4.7 vs 41 ± 6.7, p< 0.001), tenting area (190.7 ± 149.7 vs 130 ± 71.3, p= 0.048) and posterior leaflet length (15.1 ± 4.1 vs 12.2 ± 3.5, p= 0.023) were larger on patients requiring mitral valve surgery. CONCLUSIONS: Tenting area, annulus and posterior leaflet length are possible determinants of MR severity. These geometric parameters could be used to determine severity and could, in the future, direct specific patient care based on individual mitral apparatus anatomy.

Insuficiencia mitral severa asintomática: a propósito de un caso / Severe asymptomatic mitral regurgitation: About a case

La insuficiencia mitral es una afección común que incide generalmente en 2 porceinto de la población, el prolapso valvular es el hallazgo más frecuente por la elongación o ruptura de las cuerdas tendinosas lo que ocasiona disímiles grados de regurgitación durante la contracción ventricular. A pesar de ello, la evaluación clínica de los pacientes asintomáticos con insuficiencia mitral severa, permanece en controversia y es motivo de debate entre los principales expertos. Se presentó un caso de insuficiencia mitral severa asintomática por rotura de cuerda tendinosa, al cual se le realizó sustitución valvular mitral debido al grado de deterioro del aparato valvular y sub-valvular, se logró buen resultado en el seguimiento

Mitral failure is a common disease that generally affects 2 percent of the population. The valvular prolapse is the most frequent finding due to elongation or rupture of tendinous cords that brings about different levels of regurgitation during the ventricular contraction. Despite the above-mentioned, the clinical evaluation of asymptomatic severe mitral failure patients remains controversial and is very much debated among outstanding experts. A case of asymptomatic severe mitral failure caused by rupture of the tendinous cord was presented. The mitral valve was replaced because the valvular and subvalvular systems were much deteriorated. The follow-up of the patient showed good results

Validation of a decision-making strategy for systolic anterior motion following mitral valve repair.

Low cardiac output syndrome and hypotension are dreadful consequences of systolic anterior motion (SAM) after a mitral valve (MV) repair. The management of SAM in the operating room remains controversial. We validate a recently suggested two-step management method and classification of this complication. This was a teaching hospital-based observational study. We validated a novel two-step conservative management method, consisting in intravascular volume expansion and discontinuation of inotropic drugs (step 1), and increasing the afterload by ascending aorta manual compression while administering esmolol e.v. (step 2). We also validate a novel classification of SAM: easy-to-revert (responding to step 1), difficult-to-revert (responding to step 2), or persistent. Fifty patients had an easy-to-revert while 26 had a difficult-to-revert SAM; 4 patients had a persistent condition (promptly diagnosed through our decisional algorithm) and underwent an immediate second pump run to repeat the mitral repair surgery. We confirmed that SAM after a repair of a degenerative MV is common and validated a simple two-step conservative management method that allows to clearly identify those few patients who require immediate surgical revision.

Manifestações cardíacas em pacientes com mucopolissacaridose VI / Cardiac manifestations in patients with mucopolysaccharidosis VI

A mucopolissacaridose tipo VI (MPS VI) é uma doença rara causada pela deficiência da enzima lisossômica arilsulfatase B, com consequente acúmulo de glicosaminoglicanos (GAGs) em vários tecidos, incluindo o cardiovascular. Com o objetivo de descrever as manifestações cardiovasculares na MPS VI, uma das principais causas de óbito, seis pacientes (4 a 13 anos) foram avaliados por exame físico, eletrocardiograma e ecocardiograma. Todos os pacientes, exceto a paciente com a menor idade, apresentaram sopro cardíaco e alterações ecocardiográficas. Os 6/6 pacientes apresentaram, no eletrocardiograma, desvio do eixo cardíaco para a direita, associado à sobrecarga atrial esquerda (1/6) ou distúrbio de condução (1/6).