Valvotomia mitral percutânea em paciente com síndrome de Lutembacher como ponte para intervenção definitiva / Percutaneous mitral valvotomy in a patient with Lutembacher Syndrome as a bridge to definitive surgical intervention

Paciente com 45 anos de idade, do sexo feminino, testemunha de Jeová, portadora de síndrome de Lutembacher, com insuficiência cardíaca congestiva (ICC) grau funcional IV da New York Heart Association (NYHA) e desnutrição grave. O ecocardiograma revelou comunicação interatrial tipo ostiumsecundum, com 38 mm de diâmetro; área valva mitral de 0,5 cm², com escore de Wilkins de 10; hipertensão pulmonar grave, com pressão sistólica estimada em 96 mmHg; ventrículodireito com significativa dilatação e disfunção grave; e insuficiência valvar tricúspide grave. Apesar do tratamentoclínico otimizado, não houve melhora do quadro de ICC nem do estado geral, motivando a mudança da conduta detratamento cirúrgico inicial para tratamento em dois tempos,primeiramente por meio de valvotomia mitral por balão, como ponte para a cirurgia. A área valvar mitral pós-procedimento aumentou para 1,34 cm². A paciente evoluiu com significativamelhora clínica, sendo realizada cirurgia 120 dias após, com substituição da válvula mitral por uma prótese mecânica e atriosseptoplastia com patch de pericárdio bovino, além de cerclagem da válvula tricúspide. A alta hospitalar ocorreu 11 dias após a cirurgia. Atualmente, encontra-se no sexto mêspós-procedimento cirúrgico, evoluindo com estabilidade clínica e melhora da qualidade de vida.

A 45-year-old female patient, Jehovah's Witness, was diagnosed with Lutembacher syndrome, New York Heart Association (NYHA) class IV congestive heart failure (CHF) and severe malnutrition. Echocardiogram showed an ostium secundum atrial septal defect, 38 mm in diameter, mitral valve area of 0.5 cm2, Wilkins score of 10, severe pulmonary hypertension, estimated systolic pressure of 96 mmHg, right ventricle with significant dilation and severe dysfunction and severe tricuspid valve insufficiency. Despite optimal clinical treatment, there was no improvement of CHF or the patient's overall condition, which led to a change in the initial conduct of surgical treatment to a two-stage therapy, starting with balloon mitral valvotomy, as a bridge to surgery. Postoperative mitral valve area increased to 1.34 cm2. The patient evolved with significant clinical improvement, and surgery was performed 120 days later with mitral valve replacement by a mechanical valve and atrioseptoplasty using a bovine pericardial patch in addition to tricuspid valve cerclage. Patient was discharged 11 days after the surgery and is currently on the sixth postoperative month, evolving with clinical stability and improvement in quality of life.

Oclusión transitoria de comunicación interauricular en el síndrome de Lutembacher / Temporary occlusion of atrial septal defect in the Lutembacher syndrome

Se describe una paciente de 82 años de edad, con síntomas de insuficiencia cardíaca avanzada e hipertensión arterial pulmonar. Un ecocardiograma transtorácico mostró una comunicación interauricular tipo ostium secundum y estenosis valvular mitral concomitante (síndrome de Lutembacher). La valoración ecocardiográfica de la enfermedad mitral se vio dificultada por la presencia del defecto interauricular. Se realizó test de oclusión percutánea transitoria de la comunicación interauricular, observándose la aparición de estenosis valvular mitral grave. El tamaño del defecto interauricular modificó las manifestaciones clínicas y el test de oclusión transitoria ayudó a decidir la conducta terapéutica.

We report the case of an 82 year-old woman with symptoms of advanced heart failure and pulmonary arterial hypertension. An echocardiogram showed an ostium secundum type atrial septal defect and concomitant mitral valve stenosis (Lutembacher syndrome). Echocardiographic assessment of mitral pathology was hampered by the interatrial septal defect. Transient percutaneous occlusion test of the atrial septal defect was performed and severe mitral valve stenosis was detected. Atrial septal defect size modified the clinical manifestations and the transient occlusion test helped to decide the therapeutic strategy.

rare case of situs inversus with dextrocardia, Lutembacher syndrome, and pericardial effusion

Incidence of congenital cardiac anomalies in dextrocardia with situs inversus is low as compared to congenital cardiac anomalies in isolated dextrocardia. We describe the first ever case of situs inversus with dextrocardia, Lutembacher's syndrome, and pericardial effusion. The pericardial effusion in our case was acquired and was tubercular in etiology

Lutembacher's Syndrome.

A 56 years old farmer from Churkhai, Mymensingh was admitted in Cardiology unit of Mymensingh Medical College Hospital 24 October, 2004 with the complaints of progressive breathlessness on exertion with the repeated respiratory tract infection. He had 3 episodes of multiple large joints swelling involving knee, ankle, wrist, during his childhood with spontaneous recovery without any residual deformity. At the age of 45 years, he was incidentally diagnosed as enlarged heart by a medical board, when applied for Foreign Service. In cardiology unit he was diagnosed as a case of Lutembacher's syndrome on the basis of history, physical examination and it was confirmed by X-Ray, ECG and Echocardiography study. As the patient developed pulmonary hypertension with calcified mitral valve leaflet so percutaneous transseptal mitral commissurotomy and or surgery is not indicated. So the patient was managed by medical therapy alone.

Síndrome de Lutembacher em paciente portadora de Doença de Chagas / Lutembacher syndrome in patient with Chagas' Disease

Relata-se neste artigo o caso de uma paciente, mulher de 58 anos, sabidamente portadora de Doença de Chagas à 35 anos, com síndrome de Lutembacher, sem apresentar características clínicas e laboratoriais da comunicação interatrial do tipo ostium secundum, ou hipertensão pulmonar de monta. Observa-se que, apesar de tantos motivos para descompensação cardíaca, sobretudo das câmaras direitas, a paciente apresenta-se clinicamente bem, tendo permanecido hemodinamicamente estável já por vários anos com a medicação empregada, sem apresentar sinais de deterioração cardíaca importante devida à concomitância das doenças. Não foi encontrado na literatura qualquer relato da concomitância dessas duas entidades clínicas num mesmo paciente.

Síndrome de Lutembacher / Lutembacher's Syndrome

A 48-years-old woman with Lutembacher syndrome with a clinical and laboratory finding of atrial septal defect, of the ostium secundum type and without pulmonary hypertension was reported. We observed, from diagnostic viewpoint, that the electrocardiographic finding of left atrial overload and the echocardiographic pattern of mitral stenosis where the most important diagnostic sign the mitral valve involvement. The patient underwent surgery and the atrial septal defect was closed with a patch. After the surgery the signs of atrial septal defect where very poor and the signs of mitral stenosis dominated the clinical picture of the patient. Although we did not make any intervention at the mitral valve, the patient had a good outcome and until the present date she is in functional class I

Síndrome de Lutembacher / Lutembacher's syndrome

Mulher de 48 anos, com Síndrome de Lutembacher, onde predominavam as características clínicas e laboratoriais da CIA, do tipo "ostium secundum", sem hipertensäo pulmonar de monta. Os autores chamam atençäo para a dificuldade diagnóstica da síndrome, no pré-operatório, onde as pistas diagnósticas, da EM, foram dadas pela curva eletrocardiográfica de sobrecarga atrial esquerda, pelo padräo ecocardiográfica de estenose mitral e de aumento do átrio esquerdo. No pós-operatório enfatizam a grande atenuaçäo dos sinais da CIA e a intensificaçäo dos da EM. Embora näo se tenha tocado na valva mitral, durante o ato cirúrgico, a paciente teve boa evoluçäo e atualmente acha-se em classe funcional I

Situs inversus total asociado a síndrome de Lutembacher / Total situs inversus associated with Lutembacher's syndrome

Se informa el caso paciente de 19 años de edad con situs inversus total asociado a sindrome de Lutembacher (comunicación interauricular y estenosis mitral). Se presentan los hallazgos de los estudios que le fueron practicados, corroborados durante la correlación quirúrgica. En nuestra revisión de la literatura médica no hemos encontrado informes acerca de esta rara asociación por lo que hemos creido útil su publicación