RESUMEN

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.

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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.

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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

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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

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