Coexistent mitral stenosis and left circumflex coronary artery to left atrial fistula in a patient with severe pulmonary hypertension

Abstract Coronary artery to left atrial fistula is rare in patients with mitral stenosis. We report an interesting case of a patient with concomitant mitral valve stenosis and coronary fistulae, originating from the left circumflex artery and drained into the left atrium with two terminal orifices.

Estenosis mitral con calcificación severa del anillo mitral: reemplazo mitral con prótesis OnX Conform en posición supraanular / Mitral stenosis with severe annular calcification: mitral valve replacement with an OnX Conform prosthesis in a supra annular position

Abstract A 68-year-old man previously subjected to radiotherapy had a prior aortic valve replacement due de radiation induced calcification of the aortic valve. Presently the patient developed severe calcification of the mitral valve ring leading to critical mitral valve stenosis. A supra annular implantation of an On X Conform valve was successfully achieved. The clinical course was uneventful, and the echocardiographic evaluation demonstrated a normal function of the valve. Different alternatives for the surgical management of this complication are discussed.

Implante de marcapasso endocárdico em paciente com válvulas tricúspide e mitral metálicas / Implantation of endocardial pacemaker in a patient with metal tricuspid and mitral valves

O implante de marcapasso endocárdico transvenoso é contraindicado em pacientes com válvulatricúspide mecânica. Relata-se o caso de uma mulher de 79 anos de idade, com válvulas mitral e tricúspidemetálicas para estimulação permanente devido à fibrilação atrial crônica de baixa resposta ventricular, que passoua apresentar sintomas de pré-síncope. Um cabo-eletrodo endocárdico foi colocado no ventrículo esquerdo porvia transvenosa através do seio coronariano na veia cardíaca posterolateral. O implante foi realizado através doseio coronário. Oferece um ritmo seguro e eficaz em pacientes com válvula tricúspide mecânica, eliminandoa necessidade de toracotomia para o implante epicárdico de cabo-eletrodo. Normalmente, a colocação é feitamediante toracotomia anterolateral ou esternotomia. Nesse caso, tanto a vida do eletrodo é reduzida quanto o seulimiar de comando geralmente aumenta com o tempo. O tecido em torno dos ventrículos é friável, especialmenteapós cirurgia cardíaca, e um eventual dano para os ventrículos durante a incisão pode ser fatal...

Transvenous endocardial pacemaker implantation is contraindicated in patients with a mechanicaltricuspid valve. An endocardial lead was placed in the left ventricle by transvenous approach through thecoronary sinus in the posterolateral cardiac vein in 79 year-old woman with metal mitral and tricuspid valve forpermanent pacing due to chronic atrial fibrillation with low ventricular response and nearsyncope symptoms.It was implanted through the coronary sinus providing a safe and effective rhythm in patients with mechanicaltricuspid valve, thereby eliminating the need for thoracotomy to implant an epicardial electrode. Transvenousendocardial pacemaker implantation is not indicated in patients with a mechanical tricuspid valve. Typically, theyare implanted by anterolateral thoracotomy or sternotomy. However, in this case, the lead mean life is reduced,and its threshold will usually increase over time. The tissue around the ventricles is friable, especially after cardiacsurgery, and an eventual ventricular damage during incision may be fatal...

Giant pulmonary artery aneurysm in a patient with rheumatic mitral stenosis

Pulmonary artery [PA] aneurysm is a rare condition, frequently associated with pulmonary hypertension. However, the evolution and treatment of this pathology is still not clear. We report a case of a 45-year-old female patient with giant PA aneurysm associated with rheumatic mitral stenosis and severe pulmonary arterial hypertension. The patient had undergone balloon mitral valvotomy around 7 years back; aneurysm was first identified 3 years back during routine follow-up. The PA aneurysm size, however, had remained almost unchanged with associated severe pulmonary regurgitation. Surgical correction was advised but denied by the patient. To our knowledge, this is the first case report of such a large PA aneurysm in association with rheumatic heart disease. Although medical therapy for pulmonary hypertension was started, surgical correction of the aneurysm was advised in order to prevent the future complications

[ case report of acute myocardial infarction in a young woman with severe mitral stenosis and OCP Use]

Acute Myocardial Infarction is a rare complication of Mitral Stenosis, which in rare cases occurs following the use of oral contraceptive pills. A 28-year-old woman was admitted to emergency ward with complaint of severe chest pain typical of is chemia. Electrocardiogram showed ST segment elevation in leads II, III, avf, and ST depression in anterior leads. Also, Troponin I level elevated to 16mg/l. Diagnosis of MI of the inferior and lateral walls was established. The patient reported a history of breathlessness after physical activity over the last 2 years, and was aware of her mitral disease. Also, the patient stated that she had been taking oral contraceptive pills over the past 3 months. Transthorasic echocardiography showed severe mitral stenosis [mitral valve area<1cm], and severe hypokinesia of inferior and laterel walls. Coronary arteries were normal in cardiac angiography. It seemed that acute MI in this patient with valvular heart disease, history of OCP use, and normal angiogram was due to thromboembolism from left atrial thrombus

Taquicardia pré-excitada em paciente com estenose mitral / Pre-Excited Tachycardia in a Patient with Mitral Stenosis

Paciente masculino, 52 anos, com estenose mitral moderada, apresentou taquicardia sustentada com QRS alargado, 120bpm, com diagnóstico de taquicardia ventricular (TV) pelo algoritmo de Brugada.Eletrocardiograma (ECG) subsequente revelou flutter atrial atípico com condução atrioventricular (AV)variável, com diferentes graus de pré-excitação por via acessória lateral esquerda. Em ritmo sinusal foi possível evidenciar a pré-excitação ventricular, o que permitiu fazer o diagnóstico de arritmia atrial associada à presença de via acessória. Nesses casos, o algoritmo de Brugada sugere, erroneamente, tratar-se de taquicardia ventricular.

A 52-year-old male patient with moderate mitral stenosis developed a sustained wide QRS tachycardia of 120 bpm,diagnosed as ventricular tachycardia through the Brugada algorithm. A subsequent ECG revealed anatypical flutter with variable atrioventricular conductionat different pre-excitation levels through the left lateral accessory pathway. In sinus rhythm, it was possible tonote ventricular pre-excitation, which led to a diagnosisof atrial arrhythmia associated with the presence of anaccessory pathway. In cases of pre-excited tachycardia,the Brugada algorithm can be misdiagnosed.

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.

Associação entre doenças imunológicas e suas manifestações clínicas semelhantes / Association between immunological diseases and their similar clinical manifestations / Asociación entre enfermedades inmunológicas y sus manifestaciones clínicas semejantes

Trata-se de um caso de uma paciente de 30 anos do sexo feminino, com prótese biológica valvar mitral em razão de estenose mitral sintomática e antecedentes de infarto agudo do miocárdio, episódios de convulsões tônico-clônicas generalizadas, alucinações visuais, eventos tromboembólicos cerebrais, apresentando no momento coreia e cardite aguda. Foram diagnosticados na paciente febre reumática em atividade, lúpus eritematoso sistêmico e síndrome do anticorpo antifosfolipídeo. A combinação de três diagnósticos incomuns em um mesmo paciente torna esse caso único, modificando o tratamento e seu prognóstico.

We report on a 30-year-old female patient, with biological mitral valve prosthesis due to symptomatic mitral stenosis and a history of acute myocardial infarction and generalized tonic-clonic seizure episodes, visual hallucinations, cerebral thromboembolic events and, at present, chorea and acute carditis. The patient was diagnosed with active rheumatic fever (RF), systemic lupus erythematosus (SLE) and Antiphospholipid syndrome (APS). The combination of three unusual diagnoses in the same patient makes this a unique case, modifying patient treatment and prognosis.

Se trata de un caso de una paciente de 30 años del sexo femenino, con prótesis biológica valvular mitral en razón de estenosis mitral sintomática y antecedentes de infarto agudo de miocardio, episodios de convulsiones tónico-clónicas generalizadas, alucinaciones visuales, eventos tromboembólicos cerebrales, presentando en el momento corea y carditis aguda. Fueron diagnosticados en la paciente fiebre reumática en actividad, lupus eritematoso sistémico y síndrome del anticuerpo antifosfolípido. La combinación de tres diagnósticos infrecuentes en un mismo paciente vuelve este caso único, modificando el tratamiento y su pronóstico.

Evolução muito tardia da valvotomia percutânea por balão na estenose mitral grave / Very late follow-up of percutaneous balloon mitral valvotomy in severe mitral stenosis

INTRODUÇÃO: A valvotomia mitral percutânea por balão é um procedimento seguro e eficaz em pacientes com estenose mitral grave sintomática selecionados, com resultados imediatos e a longo prazo semelhantes aos da intervenção cirúrgica. Este estudo tem o objetivo de descrever os resultados muito tardios das primeiras valvotomias mitrais percutâneas por balão realizadas em nossa instituição e identificar os fatores preditores de reestenose. MÉTODOS: No período de 1987 a 1991, 200 pacientes consecutivos foram submetidos a valvotomia mitral percutânea por balão. Avaliações clínica e ecocardiográfica foram realizadas antes do procedimento, 48 horas após e, então, anualmente. RESULTADOS: A média de idade foi de 32 ± 12 anos, 86,5% eram do sexo feminino e 80,5% encontravam-se em classe funcional III ou IV da New York Heart Association. A média do escore de Wilkins foi de 7,6 ± 1,2 e o sucesso do procedimento ocorreu em 87,5% (175/200) dos pacientes. Durante o seguimento, foram acompanhados 129 pacientes (74%) por 140 ± 79 meses. Reestenose após o primeiro procedimento ocorreu em 46,5% (60/129) dos pacientes, sendo realizada uma segunda valvotomia mitral percutânea por balão em 25 pacientes, uma terceira em 4 pacientes, e uma quarta em 1 paciente. Em cinco anos, a probabilidade livre de reestenose foi de 85%, em 10 anos foi de 60% e em 20 anos, de 36%. O diâmetro do átrio esquerdo (P = 0,034) e o gradiente transvalvar mitral tanto pré (P = 0,013) como pós-procedimento (P = 0,038) foram preditores de reestenose. CONCLUSÕES: Em seguimento clínico muito tardio, a valvotomia mitral percutânea por balão mostrou que os resultados são duradouros em mais de um terço dos pacientes e que a repetição do procedimento pode ser realizada com segurança em pacientes selecionados. A identificação dos preditores de reestenose é útil para guiar a seleção de casos para o procedimento.

BACKGROUND: Percutaneous balloon mitral valvotomy is safe and effective in patients with severe symptomatic mitral stenosis with immediate and long-term results comparable to those of surgical intervention. This study was aimed at reporting the very late follow-up results of the first percutaneous balloon mitral valvotomies performed at our institution and at identifying predictive factors of restenosis. METHODS: From 1987 to 1991, 200 consecutive patients were submitted to percutaneous balloon mitral valvotomy. Clinical and echocardiographic evaluations were performed prior to the procedure, 48 hours after the procedure and annually thereafter. RESULTS: Mean age was 32 ± 12 years; 86.5% were female and 80.5% were in New York Heart Association functional class III or IV. Mean Wilkins score was 7.6 ± 1.2 and procedure success was observed in 87.5% (175/200) of the patients. During follow-up, 129 patients (74%) were followed up for 140 ± 79 months. Restenosis was observed after the first procedure in 46.5% (60/129) patients and a second percutaneous balloon mitral valvotomy was performed in 25 patients, a third one in 4 patients and a fourth one in 1 patient. The probability of being restenosis-free was 85% at 5 years, 60% at 10 years and 36% at 20 years. Left atrial diameter (P = 0.034), and preoperative (P = 0.013) and postoperative (P = 0.038) transvalvar gradient were predictors of restenosis. CONCLUSIONS: In a very late clinical follow-up, percutaneous balloon mitral valvotomy provided long-lasting results in over one-third of the patients and showed that repeated procedures may be performed safely in selected patients. The identification of restenosis predictors is useful for patient selection.

Valvotomia mitral percutânea: quarta dilatação / Percutaneous mitral valvotomy: fourth dilatation

Paciente do sexo feminino, 37 anos, portadora de estenose mitral de etiologia reumática, submetida, com sucesso, a valvotomia mitral percutânea em 1996, 2000 e 2005. Após 41 meses do terceiro procedimento, além de episódios de fibrilação atrial paroxística, voltou a apresentar dispneia aos moderados esforços. O estudo ecocardiográfico demonstrou gradiente transvalvar médio de 8 mmHg, pressão sistólica da artéria pulmonar de 55 mmHg, área valvar mitral de 1 cm² e escore de Wilkins de 10 pontos. Em decorrência da evidência de reestenose clínica e ecocardiográfica, indicou-se novo procedimento percutâneo, realizado sem intercorrências, porém obtendo-se resultado subótimo.

Thirty-seven-year-old female, previously diagnosed with mitral valve stenosis due to rheumatic fever, who had successfully undergone percutaneous mitral valvotomy in 1996, 2000 and 2005. Forty-one months after the thirdprocedure, in addition to episodes of paroxystic atrial fibrillation, the patient had dyspnea after ordinary exertion.Echocardiographic evaluation revealed a mean transvalvular gradient of 8 mmHg, pulmonary artery systolic pressure of 55 mmHg, a mitral valve area of 1 cm², and a Wilkins score of 10 points. With the evidence of clinical and echocardiographicmitral restenosis, a new percutaneous procedure was performed, with no periprocedural complications, however, achieving suboptimal outcome.

Comparação das medidas da área valvar mitral obtidas por parâmetros hemodinâmicos invasivos e ecocardiográfia tridimensional em tempo real pré e pós-valvoplastia mitral percutânea / Comparison of mitral valve area measurements by invasive hemodynamic parameters and real-time three-dimensional echocardiography before and after percutaneous mitral valvuloplasty

INTRODUÇÃO: A técnica de ecocardiografia tridimensional em tempo real (eco-3D) para avaliação da área valvar mitral (AVM) é uma técnica inovadora, que deve ser validada na compração com técnicas de cálculos de AVM já consagrados. O cálculo da AVM pelo método de Gorlin já foi bem estabelecido e correlaciona-se de forma adequada com a verdadeira AVM. Nosso objetivo foi comparar as medidas de AVM obtidas pelos métodos hemodinâmicos e ecocardiográfico, a partir de parâmetros hemodinâmicos invasivos (fórmula de Gorlin) e de eco-3D após valvoplastia mitral percutânea por cateter-balão (VCB). Método: Estudo prospectivo, realizado entre fevereiro de 2009 e fevereiro de 2010, em que foram selecionados 24 indivíduos (dos quais 22 mulheres, na faixa etária de 39 + ou - 12 anos) portadores de estenose mitral sintomática submetidos a VCB. Foi feita análise ecocardiográfica transtorácica bidimensonal e tridimensional antes e após VCB, em que foram aferidos a AVM (eco-3D/medida invasiva) e o gradiente...

BACKGROUND: Real time three-dimensional (3D) echocardiography (Echo) is an innovative technique to assess mitral valve area (MVA) that must be validated in comparison with established MVA calculation techniques. The calculation of valve area by Gorlin's method has been well established and correlates adequately with the true MVA. This study was aimed at comparing MVA obtained by hemodynamic and echocardiographic methods, based on invasive hemodynamic (Gorlin's formula) and 3D Echo parameters after percutaneous balloon mitral valvuloplasty (PBMV). METHODS: Prospective study carried out from February 2009 to February 2010, in 24 individuals (22 women, 39 ± 12 years) with symptomatic mitral stenosis undergoing PBMV. 2D and 3D Echo were compared before and after the PBMV, with MVA (3D Echo/invasive measurement) and mitral gradient (max/mean) measurements. RESULTS: Two patients were excluded (one because of a left atrial thrombus and another due to MVA > 1.5 cm²). Mean MVA obtained by the hemodynamic method was 1.73 cm², whereas the mean value obtained by 3D Echo was 1.72 cm². There was a significant correlation between MVA obtained by Gorlin's formula and 3D Echo pre-valvuloplasty (r: 0.7638; P < 0.001) and post-valvuloplasty (r: 0.6659; P < 0.0019). CONCLUSIONS: 3D Echo is an adequate method for the assessment and calculation of MVA before and after PBMV procedures providing accurate values when compared to the established MVA calculation obtained by Gorlin's formula.

Anesthetic management for surgical repair of Ebstein's anomaly along with coexistent Wolff-Parkinson-White syndrome in a patient with severe mitral stenosis.

Ebstein's anomaly (EA) is the most common cause of congenital tricuspid regurgitation. The associated anomalies commonly seen are atrial septal defect or patent foramen ovale and accessory conduction pathways. Its association with coexisting mitral stenosis (MS) has uncommonly been described. The hemodynamic consequences and anesthetic implications, of a combination of EA and rheumatic MS, have not so far been discussed in the literature. We report successful anesthetic management of a repair of EA and mitral valve replacement in a patient with coexisting Wolff-Parkinson-White (WPW) syndrome.

Valvotomia mitral percutânea: da primeira à terceira dilatação / Percutaneous mitral ballon valvotomy: from the first to third dilatation

Introdução: As causas de reestenose pós-valvotomia mitral percutânea dependem principalmente das características da população submetida à técnica. O objetivo deste tra balho foi comparar os resultados imediatos e tardios dos pacientes submetidos a dois ou mais procedimentos de valvotomia mitral percutânea (VMP) a um grupo de pacientes submetidos apenas a uma dilatação para o tratamento da estenose mitral grave. Método: Os pacientes foram divididos em dois grupos: o grupo A incluiu 90 pacientes submetidos a uma primeira VMP e que, em decorrência de reestenose ecocardiográfica e clínica, foram encaminhados a uma segunda intervenção, e 9 pacientes que, pelo mesmo motivo, foram submetidos a um terceiro procedimento; e grupo B, composto de 90 pacientes selecionados por amostra aleatória simples submetidos a apenas uma dilatação, todas com sucesso. As variáveis ecocardiográficas analisadas para comparação dos resultados dentro do mesmo grupo e entre os grupos A e B foram a área valvar mitral (AVM), os gradientes diastólicos máximo (GDM) e médio (GDm), o diâmetro do átrio esquerdo e a incidência de reestenose...

Background: The causes for restenosis following percutaneous balloon mitral valvotomy (PBMV) vary according to the population undergoing this technique. The aim of this study was to compare the immediate and long-term results of patients undergoing a second and third PBMV to patients submitted to a single dilatation of the mitral valve for the treatment of severe mitral stenosis. Methods: Patients were divided into two groups: group A with 90 patients who, due to clinical and echocardiographic restenosis, were submitted to more than one procedure, and group B with 90 patients, selected by a random sample who underwent a single successful procedure. The echocardiographic variables analyzed to compare the results in the same group and between groups A and B were mitral valve area (MVA), maximal and mean diastolic gradients, left atrial diameter and incidence and time to restenosis...

Valvoplastia mitral por balão: fatores de risco para insucesso, insuficiência mitral grave e complicações graves / Mitral balloon valvuloplasty: risk factors for lack of sucess, severe mitral regurgitation and major complications

A valvoplastia mitral por balão não está livre de insucesso e complicações. Determinar fatores independentes de risco para procedimento incompleto, insucesso, insuficiência mitral grave e complicações graves no procedimento de valvoplastia mitral por balão. Estudo prospectivo longitudinal de 518 procedimentos de valvoplastia mitral por balão, realizados entre 6 de julho de 1987 e 31 de dezembro de 2004, dendo 429 (82,8 por cento) pacientes do sexo feminino e 89 (17,2por cento) do sexo masculino, com idade média...

Induced mild hypothermia in post-cardiopulmonary bypass vasoplegia syndrome.

The state of vasoplegia in immediate post-cardiopulmonary bypass period is characterized by severe hypotension, supranormal cardiac output, low systemic vascular resistance (SVR), and resistance to vasoconstrictors. We could successfully use induced mild hypothermia to increase SVR, and could avoid very high doses of nor-epinephrine (>0.3 mcg/kg/min) in the background of severe pulmonary hypertension (systolic pulmonary pressure> 90 mmHg). Its effects such as decreased oxygen demand, positive inotropy and better right ventricle performance probably helped to improve oxygenation in presence of pulmonary oedema.