Anticoagulation in Atrial Fibrillation Associated with Mitral Stenosis.

Rheumatic valve disease is present in 0.4 % of the word population, mainly in lowincome countries. Rheumatic mitral stenosis affects more women and between 40 to 75 % of patients may have atrial fibrillation (AF), more frequently in upper-middle income countries. This rhythm disturbance is due to increased atrial pressure, chronic inflammation, fibrosis, and left atrial enlargement. There is also an increase in the prevalence of AF with age in patients with mitral stenosis. The risk of stroke is 4 % per year. Success rates for cardioversion, Cox-Maze procedure, and catheter ablation are low. Therefore, anticoagulation with vitamin K antagonist is mandatory for Evaluated Heart valves, Rheumatic or Artificial (EHRA) classification type 1. However, this anticoagulation is used by less than 80 % of those eligible and less than 30 % have the international normalized ratio in the therapeutic range. The safety and efficacy of using rivaroxaban, a direct factor Xa inhibitor anticoagulant, were demonstrated in the RIVER trial with a sample of 1005 patients with AF and bioprosthetic mitral valve. The indication for valve replacement, that is, if severe mitral stenosis or severe mitral regurgitation, was not specified. A randomized, open-label study (DAVID-MS) is underway to compare the effectiveness and safety of dabigatran and warfarin therapy for stroke prevention in patients with AF and moderate or severe mitral stenosis. Thus, the applicability of the use of direct anticoagulants in patients with AF and mitral stenosis and also in those undergoing mitral bioprostheses surgery will be the subject of further studies. The findings may explain if specific atrial changes of mitral stenosis even after the valve replacement will influence thromboembolic events with direct anticoagulants.

Pulmonary artery pressure response to percutaneous mitral valvuloplasty: Associated factors and clinical implications.

BACKGROUND: Pulmonary hypertension (PH) is a marker of poor outcome in mitral stenosis (MS), which improves after percutaneous mitral valvuloplasty (PMV). However, mechanical interventions for relief of valve obstruction often but not always reduce pulmonary pressures. This study aimed to assess the parameters associated with abnormal pulmonary artery pressure (PAP) response immediately after a successful PMV, and also its impact on long-term outcome. METHODS: A total of 181 patients undergoing PMV for rheumatic MS were prospectively enrolled. Invasive hemodynamic and echocardiographic measures were examined in all patients. Abnormal PAP response was defined as the mean PAP (mPAP) values unchanged at the end of the procedure. Long-term outcome was a composite endpoint of death, mitral valve replacement, repeat PMV, new onset of atrial fibrillation (AF), or stroke. RESULTS: The mean age was 44.1 ± 12.6 years, and 157 patients were women (86.7%). In the overall population, mPAP decreased from 33.4 ± 13.1 mmHg pre to 27.6 ± 9.8 mmHg post (p < 0.001). Following PMV, 52 patients (28.7%) did not have any reduction of mPAP immediately after the PMV. Multivariable analysis adjusting for baseline values of PAP and mitral valve area revealed that AF (Odds ratio [OR] 2.7, 95% [confidence interval] CI 1.3 to 6.7), maximum mitral valve leaflets displacement (OR 0.8, 95% CI 0.7 to 0.9), and post-procedural left ventricular compliance (OR 0.7, 95% CI 0.5 to 0.9) were predictors of a lack of improvement in mPAP. During a median follow-up of 4.4 years, the endpoint was reached in 56 patients (31%). The pulmonary pressure response to PMV was not an independent predictor of long-term events. CONCLUSIONS: In patients with MS undergoing PMV, pulmonary pressures may not reduce immediately after the procedure, despite adequate opening of the valve. Abnormal PAP response can be predicted from baseline clinical and valvular characteristics as well as post-procedural left ventricular compliance. The lack of any immediate reduction in mPAP is not associated with long-term adverse outcomes.

Coexistent Mitral Stenosis and left Circumflex Coronary Artery to left Atrial Fistula in a Patient with Severe Pulmonary Hypertension.

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.

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.

Shone's syndrome: Insights from three-dimensional echocardiography.

Shone's syndrome is a rare congenital anomaly defined as the presence of at least two of the following heart obstructions: a mitral supravalvular ring, a "parachute" mitral valve stenosis, subaortic stenosis, and aortic coarctation. A 58-year-old man presented with a mitral ring and a "parachute" mitral valve on two-dimensional transthoracic echocardiography, raising suspicion of Shone's syndrome. Three-dimensional transesophageal echocardiography revealed a subannular mitral ring inserted directly on the mitral leaflets, thus acting as a "valvar ring." This distinction can have therapeutic implications as a "valvar" mitral ring could require valve repair or replacement, instead of simple resection.

Pulmonary artery ablation to treat pulmonary arterial hypertension: a case report.

INTRODUCTION: Idiopathic pulmonary arterial hypertension is defined as a group of diseases characterized by a progressive increase in pulmonary vascular resistance that results in right heart failure and premature death. Although therapies exist to improve hemodynamic instability and symptoms, there is no cure for pulmonary arterial hypertension and it remains a life-threatening condition. A recent study performed in China reported, for the first time, the effect of pulmonary arterial denervation on functional capacity and hemodynamics in patients with refractory idiopathic pulmonary arterial hypertension. CASE PRESENTATION: We report a case of a 60-year-old white Brazilian man, with controlled hypertension and stage 2 obesity who complained of progressive fatigue with moderate to light exertion of approximately 1 year's duration. During this period, he underwent myocardial perfusion scintigraphy without evidence of obstructive ischemic disease. He had no clinical evidence of systolic heart failure. He had undergone biological mitral valve replacement 3 years previously for mitral valve stenosis and ablation of atrioventricular nodal reentry tachycardia 18 months previously. At the time of valve replacement, he had no reported evidence of pulmonary arterial hypertension. His echocardiogram showed normal function of a mitral prosthesis, normal global left ventricular systolic function (left ventricular ejection fraction 62 % measured using the Teichholz method), stage I diastolic dysfunction, and a mean systolic pulmonary arterial blood pressure of 50 mmHg. In the 6-minute walk test, the patient walked 104 meters. Catheterization of his right heart chambers and pulmonary arteries confirmed the diagnosis of pulmonary hypertension. Electroanatomic reconstruction of the right ventricular outflow tract and pulmonary artery was performed under direct fluoroscopic visualization, and a merger was made with a formatted image of cardiac computed tomography angiography. Then we performed irrigated cardiac catheter ablation of the pulmonary trunk. CONCLUSIONS: At the patient's 3-month follow-up, he showed improvement in functional class for fatigue on major exertion, increased distance walked in the 6-minute walk test, and reductions in pressure of both the right cavities and the pulmonary artery. Currently, with 6 months of clinical follow-up, the patient has maintained his functional classification and is pedaling his bicycle.

Role of LA shape in predicting embolic cerebrovascular events in mitral stenosis: mechanistic insights from 3D echocardiography.

OBJECTIVES: This study was designed to assess the role of left atrial (LA) shape in predicting embolic cerebrovascular events (ECE) in patients with mitral stenosis (MS). BACKGROUND: Patients with rheumatic MS are at increased risk for ECE. LA remodeling in response to MS involves not only chamber dilation but also changes in the shape. We hypothesized that a more spherical LA shape may be associated with increased embolic events due to predisposition to thrombus formation or to atrial arrhythmias compared with an elliptical-shaped LA of comparable volume. METHODS: A total of 212 patients with MS and 20 control subjects were enrolled. LA volume, LA emptying fraction, and cross-sectional area were measured by 3-dimensional (3D) transthoracic echocardiography. LA shape was expressed as the ratio of measured LA end-systolic volume to hypothetical sphere volume ([4/3π r(3)] where r was obtained from 3D cross-sectional area). The lower the LA shape index, the more spherical the shape. RESULTS: A total of 41 patients presented with ECE at the time of enrollment or during follow-up. On multivariate analysis, LA 3D emptying fraction (adjusted odds ratio [OR]: 0.96; 95% confidence interval [CI]: 0.92 to 0.99; p = 0.028) and LA shape index (OR: 0.73; 95% CI: 0.61 to 0.87; p < 0.001) emerged as important factors associated with ECE, after adjustment for age and anticoagulation therapy. In patients in sinus rhythm, LA shape index remained associated with ECE (OR: 0.79; 95% CI: 0.67 to 0.94; p = 0.007), independent of age and LA function. An in vitro phantom atrial model demonstrated more stagnant flow profiles in spherical compared with ellipsoidal chamber. CONCLUSIONS: In rheumatic MS patients, differential LA remodeling affects ECE risk. A more spherical LA shape was independently associated with an increased risk for ECE, adding incremental value in predicting events beyond that provided by age and LA function.

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...

Impact of net atrioventricular compliance on clinical outcome in mitral stenosis.

BACKGROUND: Net atrioventricular compliance (Cn) has been reported to be an important determinant of pulmonary hypertension in mitral stenosis (MS). We hypothesized that it may be useful in assessing prognosis because Cn reflects hemodynamic consequences of MS. To date, limited data with an assumed Cn cutoff have indicated the need for larger prospective studies. This prospective study was designed to determine the impact of Cn on clinical outcome and its contribution to pulmonary pressure in MS. In addition, we aimed to identify a cutoff value of Cn for outcome prediction in this setting. METHODS AND RESULTS: A total of 128 patients with rheumatic MS without other significant valve disease were prospectively enrolled. Comprehensive echocardiography was performed and Doppler-derived Cn estimated using a previously validated equation. The end point was either mitral valve intervention or death. Cn was an important predictor of pulmonary pressure, regardless of classic measures of MS severity. During a median follow-up of 22 months, the end point was reached in 45 patients (35%). Baseline Cn predicted outcome, adding prognostic information beyond that provided by mitral valve area and functional status. Cn ≤4 mL/mm Hg best predicted unfavorable outcome in derivation and validation sets. A subgroup analysis including only initially asymptomatic patients with moderate to severe MS without initial indication for intervention (40.6% of total) demonstrated that baseline Cn predicted subsequent adverse outcome even after adjustment for classic measures of hemodynamic MS severity (hazard ratio, 0.33; 95% confidence interval, 0.14-0.79; P=0.013). CONCLUSIONS: Cn contributes to pulmonary hypertension beyond stenosis severity itself. In a wide spectrum of MS severity, Cn is a powerful predictor of adverse outcome, adding prognostic value to clinical data and mitral valve area. Importantly, baseline Cn predicts a progressive course with subsequent need for intervention in initially asymptomatic patients. Cn assessment therefore has potential value for clinical risk stratification and monitoring in MS patients.

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.

Association between immunological diseases and their similar clinical manifestations.

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.

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.