Immunoglobulin G4 Non-Related Sclerosing Disease with Intracardiac Mass Mimicking Mitral Stenosis: Case Report

The cardiovascular system may be one of the target organs of both immunoglobulin G4 related and non-related systemic multifocal fibrosclerosis. We present a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis on echocardiography. For a more detailed differential diagnosis, we used multimodal imaging techniques. After surgical biopsy around the abdominal aortic area in the retroperitoneum, histological examination revealed IgG4 non-related systemic multifocal fibrosclerosis. We describe the multimodal imaging used to diagnose IgG4 non-related systemic multifocal fibrosclerosis and a positive response to steroid treatment. There have been no previous case reports of IgG4 non-related systemic multifocal fibrosclerosis with intracardiac involvement. Here, we report a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis.

Simplifying proximal isovelocity surface area as an assessment method of mitral valve area in patients with rheumatic mitral stenosis by fixing aliasing velocity and mitral valve angle

We aimed to test the ability of a simple equation using proximal isovelocity surface area method [PISA], created by fixing the angle to 100° and the aliasing velocity to 33 cm/s, to calculate mitral valve area [MVA] and assess severity in patients with rheumatic mitral stenosis [MS]. In a series of 51 consecutive patients with rheumatic MS, MVA was assessed by four methods, conventional PISA equation PISA[conventional], simple PISA equation PISA[simple], pressure half time [PHT], and planimetry [PLN] which was taken as the reference method. All methods correlated significantly with PLN with the highest correlation found in case of PISA[conventional] and PISA[simple] [r = 0.97, 0.96, p < 0.001], while the correlation in case PHT was relatively weaker [r = 0.69, p < 0.001]. Bland-Altman analysis revealed that the level of agreement with PLN was better in case of both PISA methods than PHT and, moreover, were close to each other. The number of cases that showed agreement of severity grade with planinetry was better in case of PISA[conventional] [42 cases] and PISA simple [44 cases] than that in case of PHT [34 cases, p = 0.037]. Finally, the measure of agreement with Cohen's Kappa test was better in case of PISA[conventional] and PISA[simple] than that in case of PHT. Provided that aliasing velocity is fixed at 33 cm/s, PISA can effectively predict mitral valve area and severity of MS by a simple equation, with the advantage of easy and accurate calculation over other methods

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.

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.

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.

Surgical treatment of primary cardiac rhabdomyosarcoma / Tratamento cirúrgico de rabdomiosarcoma primário do coração

The authors report a case of a 16-year-old man who presented progressive dyspnea. At that time the diagnosis of rheumatic fever with mitral valve involvement was performed. The bidimensional echocardiogram showed presence of mobile mass inside the left atrium. The tumor presented lobules, projecting into the left ventricle during the diastole and provoking turbulence. The patient underwent surgical resection with postoperative course needing re-operation for mitral valve replacement. Histopathology has proven that such tumor was a primary cardiac rhabdomyosarcoma and the early clinical diagnosis of rheumatic mitral valve disease was very difficult.

Os autores reportam caso de jovem de 16 anos, o qual apresentou dispnéia progressiva. No momento do atendimento foi feito diagnóstico de febre reumática com comprometimento da valva mitral. Ecocardiograma bidimensional demonstrou a presença de massa móvel dentro do átrio esquerdo. O tumor apresentava lobos, se projetando para o interior do ventrículo esquerdo durante a diástole, provocando turbulência. O paciente foi submetido a ressecção cirúrgica, complicada com reoperação e troca valvar mitral. A histopatologia demonstrou tratar-se de rabdomiosarcoma primário e o diagnóstico clínico diferencial com febre reumática e doença valvar mitral foi muito difícil desde o seu início.

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...

Percutaneous transvenous mitral commissurotomy in mitral restenosis with situs inversus

A woman, aged 30 years with situs inversus, dextrocardia and severe rheumatic mitral restenosis was referred to AFIC/NIHD for commissurotomy. A Closed Mitral Valvotomy [CMV] for severe mitral stenosis had already been done 8 years earlier, and her symptoms had reappeared. She was evaluated with transthoracic and transesophageal echocardiography and found to have a mitral valve area of 0.6 cm2, dextrocardia along with atrioventricular [AV] and ventriculo-atricular [VA] concordance was demonstrated. Successful percutaneous transvenous mitral commissurotomy [PTMC] was done. Transseptal catheterization was done via the left femoral vein. Pigtail catheter was placed in the noncoronary aortic sinus; interatrial septal puncture was done with the transseptal needle rotated to a 7 O`clock position. There were no procedural complications. Reduction in trans-mitral pressure gradient on cardiac catheterization data, and standard echocardiographic parameters confirmed a successful procedure. PTMC can be accomplished safely in patients with this unusual cardiac anatomy with a few modifications in the standard technique, even if surgical treatment has already been carried out

Mitral leaflet separation index-a simple novel index to assess the severity of mitral stenosis.

BACKGROUND: Mitral leaflet separation (MLS) index is a recently recognized method to assess the severity of mitral stenosis. The aim of this study is to assess the severity of mitral stenosis (MS) using the MLS index. METHODS AND RESULTS: One hundred and fifty consecutive patients with MS who underwent 2D echo were studied. In each patient the severity of MS was assessed using 2D mitral valve area, pressure half time and MLS index. The mitral leaflet separation was measured in diastole, as the maximal separation at the tips in long axis and apical 4 chamber views. There were 34 (22.7%) patients with mild MS, 52 (34.7%) patients with moderate MS and 64 (42.7%) patients with severe MS. One hundred and ten (73.3%) patients were in sinus rhythm and 40 (26.7%) patients were in atrial fibrillation. The MLS index correlated very well with MVA by planimetry (r = 0.87, P < 0.0001) and pressure half time (r = 0.86, P < 0.0001). Good correlation was found even in atrial fibrillation (r = 0.87 and r = 0.87, respectively). ROC curve demonstrates that MLS index of 0.80cm or less identified severe MS with 92% sensitivity and 92% specificity. MLS index of >1.11 cm identified mild MS with 97% sensitivity and 97% specificity. CONCLUSION: The MLS index can be used as a screening method to semi quantify patients with mitral stenosis. This should be added into yet another method to assess the severity. This index will complement the other methods in our practice.

Analysis and diagnosis of heart sounds

Auscultation [the interpretation by a physician of heart sounds] is a fundamental component in cardiac diagnosis. It is, however, a difficult skill to acquire. In this paper, we present study for a system Intended to aid in heart sound analysis and diagnosis based on detection of different events [main and additional sounds, murmurs and clicks] present in the cardiac cycle with resolution at the level of sampling period in time and in frequency at the level of FFT points used and for each event set of parameters in time and frequency domains have been extracted for normal and abnormal cases without using ECG signal as reference. These parameters could be easily used by the doctor for analyzing the extracted parameters for normal, mitralstenosis, and s2 split sounds have been extracted from recorded sounds

Valvular heart disease: diagnosis and management.

Valvular heart disease is a leading cause of morbidity and mortality in India. Advances in both surgical and percutaneous techniques and a better understanding of timing for intervention accounts for the current increased rates of survival. Echocardiography remains the gold standard for diagnosis and periodic assessment of patients with valvular heart disease. Generally, patients with stenotic valvular lesions can be monitored clinically until symptoms appear and most can now benefit from percutaneous techniques. In contrast, patients with regurgitant valvular lesions require careful echocardiographic monitoring for left ventricular function and may require surgery even if no symptoms are present. Percutaneous therapy of valvular regurgitant lesions is yet to evolve fully.

Mitral stenosis in a girl of five years.

Mitral stenosis(MS) detected below the age of 20 years is called juvenile mitral stenosis (JMS). JMS constitute 25-40 % of all cases of isolated mitral stenosis, though overall incidence of rheumatic fever and rheumatic heart disease (RHD) within mixed population is 7.5- 7.8 per thousand. The patient was 5 years old girl hailing from Modhupur, Tangail got herself admitted into CCU Mymensingh Medical College Hospital with the complaints of low grade fever, shortness of breath and also associated with failure to thrive. She was ill looking, mildly anaemic. Precordial examination revealed apex beat was in the left 5th intercostals space, tapping in nature, palpable P(2). There was left parasternal heave. 1st heart sound loud, pulmonary component of the 2nd heart sound was accentuated, opening snap with mid diastolic murmur with pre systolic accentuation. Routine blood examination reveals leucocytosis with raised ESR. C-reactive protein (CRP) and ASO titre were significantly raised. X-ray chest P/A view showing the features of mitral stenosis. Echocardiography showing MS (moderate) with pulmonary hypertension. She was treated with antibiotics and other relevant drugs and discharge with an advice for follow up and take preparation for cardiac intervention.