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)

Embolia coronariana associada a prolapso de valva mitral / Coronary embolism associated with mitral valve prolapse

Embolia coronariana é um evento raro. Neste trabalho, relatamos caso de pacientes, sexo masculino, de 64 anos, com um evento embólico coronariano associado a prolapso e rotura de valva mitral mixomatosa, situação essa extremamente incomum.

Avaliação ecocardiográfica tridimensional do mecanismo e da graduação da regurgitação mitral / Three-dimensional echocardiographic evaluation of the mechanism and severity of mitral regurgitation

A ecocardiografia tridimensional é uma modalidade de imagem adequada para avaliar a anatomia complexa da valva mitral assim como para determinar o mecanismo e também para graduar a regurgitação mitral. A ecocardiografia tridimensional pode ser realizada a partir da abordagem transtorácica como também transesofágica. Este artigo discute as formas de aquisição de dados e de manipulação de imagens com a ecocardiografia tridimensional para a avaliação da valva mitral.

Peculiaridades fisiopatológicas das valvopatias mitrais: implicações clínicas e terapêuticas / Pathophysiologic characteristics of mitral valve lesions: clinical and therapeutic implications

As disfunções primárias da valva mitral podem ter várias etiologias, entre elas doenças reumáticas, prolapso da valva mitral, doença isquêmica, calcificação do anel mitral e congênita...

Prolapso da valva mitral em mulheres adultas: características clínicas, fisiológicas e cineantropométricas / Mitral valve prolapse in adult women: clinical, physiological and kinanthropometric

Ojetivo: Identificar as características clínicas, fisiológicas e cineantropométricas que são capazes de discrimicar mulheres adultas com e sem prolapso de valva mitral (PVM), partindo-se da hipotese que determinadas caractérísticas cineantropométricas apresentam um padrão bastante típico nessas mulheres e poderial contribuir significativamente para essa diferenciação. Métodos: Foram analisados, retrospectivamente, 124 laudos consecutivos de mulheres aultas e não-atletas (idade média de 50 anos), sendo 34 delas com PVM, submetidas às avaliações cineantropométricas: somatotipo, flexibilidade, destreza para sentar e levantar do solo, clínica: ausculta, frequência cardíaca (FC) e pressão arterial (PA) em repouso, e fisiológica: FC, PA e consumo de oxigênio...

Obstrução da via de saída do ventrículo esquerdo durante ecocardiograma de estresse farmacológico - Relato de caso / Left ventricle outflow tract obstruction during pharmachological stress echocardiography - Case report

São descritas as histórias de 2 pacientes adultos encaminhados para ecocardiograma com estresse farmacológico para avaliação de dor precordial com teste ergométrico não conclusivo. Ambos apresentam prolapso da valva mitral com refluxo holossistólico discreto no ecocardiograma. Durante o estresse, quando em dose elevada de dobutamina, apresentaram movimento anterior sistólico da valva mitral com obstrução da via de saída do ventrículo esquerdo associada a dor precordial, sem alteração da contratilidade segmentar ou da função do ventrículo esquerdo. A cineangiocoronariografia dos dois pacientes demonstrou lesões coronarianas discretas, não obstrutivas.

A report case of sudden cardiac death in a young adult male from northeastern part of Thailand with mitral valve prolapse.

A case of sudden unexpected natural death in a young adult male from the north-eastern part of Thailand with clinical non Lai-Tai disease, but pathologic feature of mitral valve prolapse is discussed. The approach to the postmortem examination of the mitral valve is reviewed. Because of the sudden nature of this death, this entity is more commonly seen in Medico-legal Medicine populations than in hospital autopsies.

Autologous right atrial patch for closure of atrial septal defect.

BACKGROUND: Prosthetic or pericardial patches used for the closure of atrial septal defects are associated with infrequent but definite problems. As an alternative, we used a right atrial free-wall patch in 12 patients, 7-54 years of age. METHODS AND RESULTS: The presence of a large secundum atrial septal defect (n=2). associated mitral valve regurgitation (n=7), primum atrial septal defect (n=2) and sinus venosus defect (n=1) necessitated the use of a patch. The mitral valve was repaired in 9 patients (including 2 with a primum defect). One patient with a primum defect who was in congestive heart failure preoperatively died after 3 weeks due to refractory ventricular fibrillation. The remaining patients were discharged 5 to 7 days post procedure. No flow was detected across the septal patch on predischarge echocardiography. One patient underwent reoperation for failed mitral valve repair one month postprocedure. At reoperation, the patch was found to be intact with normal texture and without any suture dehiscence. Histopathological examination of the explanted patch revealed viable endothellum and subendothelial muscle on both surfaces of the patch. Follow-up ranged from 6 to 36 months. Echocardiography performed after 6 to 32 months post procedure showed an intact patch with no residual defect. All the patients are in sinus rhythm. Holter monitoring performed in 6 patients was normal in all of them. Electrophysiological study was performed in 2 patients using a mapping catheter 4 and 6 months post-procedure, respectively, and recorded normal atrial potentials from the site of the patch. CONCLUSIONS: The use of an autologous free right atrial wall as a patch for atrial septal defect closure is a viable option.

Pseudoaneurysm of gastroduodenal artery associated with mitral valve prolapse presenting as haemobilia.

Pseudoaneurysms of the hepatic or gastroduodenal arteries may cause Haemobilia. Mitral valve prolapse associated with mycotic pseudoaneurysm of cerebral and extracerebral arteries have been reported. We report a case of gastroduodenal artery pseudoaneurysm presenting as haemobilia. The patient was successfully treated with indigenously fabricated steel coil embolization followed by surgery.

Mitral valve prolapse with pulmonary haemosiderosis and severe anaemia: cause or association?

A 12 years boy presented with the history of pallor for one month. Two days before hospitalisation he developed fever, cough, shortness of breath. He had past history of such episode.On examination, his heart rate was found to be 120/minute, respiratory rate 40/minute and moderate anaemia was detected. Scattered creptus was audible over mid and lower lung fields and a soft systolic murmur was auscultated at apex. On investigations, Hb was found as 4.6 g/dl and HbF was less than 2%. Plasma Hb was 5 g/dl. Straight x-ray chest showed bilateral patchy opacities over mid and lower zones. His sputum was found to be blood stained and prompted the possibility of blood loss occurring in the lungs, which was confirmed by demonstrating haemosiderin laden macrophages on three consecutive sputum specimens. Echocardiography revealed a systolic displacement of mitral valve leaflets into the left atrium with co-optation superior to the plane of mitral annulus. Doppler study showed a minimal late systolic regurgitation. It was decided to treat the case as idiopathic pulmonary haemosiderosis. Oxygen inhalation, hypertonic saline nebulisation, i.v. hydrocortisone, packed cell transfusion followed by oral prednisolone improved the patient's condition. After 3 months of discontinuing prednisolone, he remained asymptomatic. Here one case of pulmonary haemosiderosis characterised by abnormal accumulation of haemosiderin in the lungs following repeated alveolar haemorrhages with the presence of mitral valve prolapse is reported.

Prolapso de la válvula mitral: complicaciones y conducta a seguir / Mitral valve prolapse: complications and behaviour to be followed

El prolapso de la válvula mitral constituye una de las afecciones valvulares que con mayor frecuencia diagnosticamos en la práctica cardiológica y es considerado, generalmente, como un proceso benigno; sin embargo, puede conducir a graves complicaciones y es la causa más frecuente de insuficiencia mitral pura, endocarditis infecciosa y arritmias cardiacas, muerte súbita, fenómenos tromboembólicos y ruptura de las cuerdas tendinosaas. En esta revisión presentamos una estratificación de los pacientes de acuerdo con su grado de riesgo que los agrupa en: bajo, intermedio y alto riesgo, lo cual nos facililtó elegir la conducta terapéutica de ellos. Se recomendó el seguimiento clínico periódico en los de bajo riesgo, el uso de betabloquedores si son muy sintomáticos y la profilaxis de la endocarditis infecciosa (cuando se realicen procederes invasivos) en los de riesgo intermedio,m y en los de alto riesgo se recomendó la profilaxis de la endocarditis siempre, tratamiento específico según la complicación y el tratamiento quirúrgico cuando la función del ventrículo izquierdo esté deteriorada

Extra-sístoles / Premature cardiac complexes

Extra-sístoles são despolarizaçöes cardíacas prematuras que podem se originar em qualquer local do coração. Se originadas nos átrios ou na junção atrio-ventricular, são denominadas extra-sístoles supraventriculares. As extra-sístoles supraventriculares são geralmente benignas, não necessitando de tratamento. Se originadas nos ventrículos, são denominadas extra-sístoles ventriculares, e seu prognóstico está diretamente correlacionado à presença e ao tipo de cardiopatia. Em coraçöes normais, o prognóstico é excelente; na cardiopatia isquêmica, a presença de extra-sístoles ventriculares freqüentes aumenta significativamente o risco de morte súbita. Requerem tratamento apenas quando relacionadas a sintomas limitantes. A supressão das extra-sístoles ventriculares com medicaçöes antiarrítmicas parece não modificar o prognóstico.

Edema pulmonar no-cardiogénico relacionado con la hidroclorotiazida / Non-cardiogenic pulmonary edema related to hidrochlorothiazide

A 54 years old female patient with a mitral valve prolapse and a rheumatic arthritis with steroids was admitted with dysnea and hypotension, that started 30 min after taking a pill containing enapril and hydroclorothiazide. Hemodynamic monitoring with a Swan-Ganz catheter showed a pulmonary capillary pressure of 5 mm Hg, a systemic vascular resistance of 887 (dyn sec)/cm5 and a cardiac output of 10 1/min. Tha patient had a history of adverse reactions to thiazides and responded to volume replacement, dopamine and steroids