Válvula aórtica bicúspide: ¿qué debo conocer? Revisión actualizada de sus aspectos clínicos y fisiopatológicos / Bicuspid aortic valve: what should I know? Updated review of its clinical and pathophysiological aspects

Resumen La válvula aórtica bicúspide es la cardiopatía congénita más frecuente en la población general. Lejos de ser solo una malformación valvular inocua, supone una enfermedad compleja y heterogénea. A menudo es identificada como un hallazgo incidental en personas sanas, cursando de manera asintomática. Sin embargo, en un alto porcentaje de pacientes conduce a lo largo de su vida a complicaciones valvulares (estenosis, insuficiencia, endocarditis) o aórticas (dilatación o disección). Con frecuencia estas manifestaciones suceden a una edad temprana y causan una elevada morbimortalidad. A pesar de que en los últimos años se ha producido una intensa investigación en este campo, la fisiopatogenia de la enfermedad no es del todo conocida y muchas preguntas siguen abiertas. En este artículo se revisan de forma actualizada los aspectos clínicos y fisiopatológicos más novedosos y relevantes sobre esta cardiopatía congénita.

Abstract The most common congenital heart disease in the general population is the bicuspid aortic valve. Far from being just a harmless valve malformation, it is a complex and heterogeneous disease. It is often identified as an incidental finding in healthy people. However, in a high percentage of patients it leads throughout their life towards valvular (stenosis, insufficiency, endocarditis) or aortic (dilatation or dissection) complications. Frequently, manifestations occur at an early age, being responsible for high morbidity and mortality. Even though in recent years intense research has been carried out in this field, the pathophysiogenesis of the disease is not fully known and many questions remain open. In this article, we review the most innovative and relevant clinical and pathophysiological aspects of this congenital heart disease.

Echocardiographic assessment of right ventricular function by two-dimensional strain in patients with left-sided valvular heart disease: comparison whit three-dimendional echocardiography / Avaliação ecocardiográfica da função ventricular direita por strain bidimensional em pacientes com valvopatia à esquerda: comparação com ecocardiografia tridimensional

Background: Right ventricular (RV) dysfunction is a well-known predictor of mortality in patients with valvular heart disease (VHD). The assessment of RV function is often difficult due to complex geometry and hemodynamic factors. Objective: We aim to analyze RV function in patients with severe mitral and/or aortic valve disease using two-dimensional strain (2DS) imaging and conventional echocardiographic parameters, comparing it with right ventricular ejection fraction (RVEF) measured by three-dimensional echocardiography (3DE). Methods: Fifty-three patients with severe mitral and/or aortic VHD underwent complete transthoracic echocardiogram in the preoperative setting for cardiac surgery, including conventional echocardiographic parameters of RV function and speckle-tracking derived 2DS indices: RV global longitudinal strain (RVGS) and RV free wall longitudinal strain (RVFWS). Conventional echocardiographic and 2DS parameters were compared with real-time 3DE RVEF using Spearman correlation test. For comparison between two groups of patients based on the presence of RV dysfunction (normal RVEF ≥ 44% - A, abnormal RVEF < 44% - B), we used nonparametric Mann-Whitney U test. ROC (receiver operating characteristic) curve analysis was used to assess the clinical utility of all RV function variables in defining RV dysfunction. P values <0,05 were considered statistically significant.Results: We found a significant correlation between all parameters and RVEF (p<0.05), with best results for RV fractional area change (FAC), RVGS, and RVFWS. Dividing the population into two-groups based on RVEF, we found 14 patients with RV dysfunction (27.4%), and significant differences between the groups for all RV function variables. For detection of RV dysfunction defined by 3DE, ROC curve analysis showed the best area under the curve (AUC) for RVGS (0.872), RVFWS (0.851) and FAC (0.932). Conclusions: We observed significant correlation between RVGS, RVFWS and RVEF, with good accuracy in detecting RV dysfunction, comparable to FAC and better than other conventional parameters of RV function assessment. The evaluation of RV myocardial deformation with 2DS may have additional diagnostic and prognostic value in patients with severe left-sided VHD

Valvular disorders in carcinoid heart disease

Abstract Carcinoid heart disease is a rare but important cause of intrinsic right heart valve disorders leading to right heart failure. Occasionally, left-sided heart valves may also be involved. The characteristic cardiac pathological findings of carcinoid heart disease are endocardial thickening as a result of fibrous deposits on the endocardium. Echocardiographic examination and right heart catheterization are very useful for the diagnosis of the lesion. If more cardiac valves are affected, multiple valve replacement should be considered. The management of the pulmonary valve lesion depends on the extent of the diseased valve, either by valvulotomy, valvectomy, or valve replacement. Percutaneous valve implantations in the pulmonary and in the inferior vena cava positions have been advocated for high-risk patients.

Importância dos métodos diagnósticos na estratificação da gravidade da estenose aórtica / Importance of diagnostic tests to evaluate the severity of aortic stenosis

A quantificação da gravidade da estenose aórtica é de fundamental importância na avaliação diagnóstica e prognóstica. É realizada mais frequentemente com os gradientes transvalvares, a velocidade máxima do fluxo através da valva e o cálculo da área valvar aórtica por meio da ecocardiografia Doppler. Outros métodos de imagem, como a tomografia computadorizada e ressonância magnética cardíaca, permitem obter informações complementares à ecocardiografia na avaliação da gravidade da doença. Nos últimos anos, com o melhor entendimento da doença, foram identificadas novas condições hemodinâmicas na estenose aórtica, como as situações de baixo fluxo transvalvar, com ou sem disfunção sistólica ventricular esquerda. Estes aspectos e a possibilidade de implante de prótese valvar por via percutânea trouxeram novas necessidades e desafios na avaliação diagnóstica de pacientes sintomáticos ou assintomáticos com estenose aórtica, os quais têm sido progressivamente superados com auxílio dos métodos de imagem.

The severity of aortic valve stenosis is fundamental for diagnosis and prognosis of the disease. This evaluation is most commonly performed by measuring transvalvar pressure gradients, peak aortic valve velocity and aortic valve area. Other cardiovascular imaging methods such as tomography and cardiac magnetic resonance al/ow additional information for evaluating the severity of the disease. In the last years with the improved knowledge of the disease, new hemodynamic conditions in aortic stenosis were identified, such as transval var low flow with or without left ventricular systolic dysfunction. The possibility of percutaneous prosthetic valve implantation is a challenge for new information in both symptomatic and asymptomatic patients.

Clinical and epidemiological profile of patients with valvular heart disease admitted to the emergency department / Aspectos clínicos e epidemiológicos de portadores de doença valvar atendidos em unidade de pronto atendimento

Objective To evaluate the clinical and epidemiological profile of patients with valvular heart disease who arrived decompensated at the emergency department of a university hospital in Brazil. Methods A descriptive analysis of clinical and echocardiographic data of 174 patients with severe valvular disease, who were clinically decompensated and went to the emergency department of a tertiary cardiology hospital, in the State of São Paulo, in 2009. Results The mean age of participants was 56±17 years and 54% were female. The main cause of valve disease was rheumatic in 60%, followed by 15% of degenerative aortic disease and mitral valve prolapse in 13%. Mitral regurgitation (27.5%) was the most common isolated valve disease, followed by aortic stenosis (23%), aortic regurgitation (13%) and mitral stenosis (11%). In echocardiographic data, the mean left atrial diameter was 48±12mm, 38±12mm for the left ventricular systolic diameter, and 54±12mm for the diastolic diameter; the mean ejection fraction was 56±13%, and the mean pulmonary artery pressure was 53±16mmHg. Approximately half of patients (44%) presented atrial fibrillation, and over one third of them (37%) had already undergone another cardiac surgery. Conclusion: Despite increased comorbidities and age-dependent risk factors commonly described in patients with valvular heart disease, the clinical profile of patients arriving at the emergency department represented a cohort of rheumatic patients in more advanced stages of disease. These patients require priority care in high complexity specialized hospitals. .

Objetivo Avaliar o perfil clínico e epidemiológico dos pacientes com doença valvar que chegaram descompensados no pronto atendimento de um hospital universitário de referência nacional. Métodos Análise descritiva de dados clínicos e ecocardiográficos de 174 pacientes consecutivos portadores de doença valvar grave, que apresentaram descompensação clínica e procuraram atendimento no pronto-socorro de um hospital terciário de cardiologia do Estado de São Paulo no ano de 2009. Resultados Dos 174 pacientes avaliados, a média etária foi de 56±17 anos e 54% eram do gênero feminino. A principal etiologia da doença valvar foi a reumática (60%), seguida pela doença degenerativa aórtica (15%) e pelo prolapso mitral (13%). A valvopatia mais comumente observada de forma isolada foi a insuficiência mitral (27,5%), seguida por estenose aórtica (23%), insuficiência aórtica (13%) e estenose mitral (11%). Nos dados ecocardiográficos, a média do diâmetro do átrio esquerdo foi de 48±12mm, do ventrículo esquerdo na sístole foi de 38±12mm, e diástole foi de 54±12mm; a média da fração de ejeção foi de 56±13% e a pressão pulmonar média foi de 53±16mmHg. Aproximadamente metade dos pacientes (44%) estava em fibrilação atrial, e mais de um terço dos pacientes (37%) já havia sido submetido a outra cirurgia cardíaca. Conclusão Apesar do aumento das comorbidades e dos fatores de risco idade dependentes comumente descritos nos portadores de cardiopatia valvar, o perfil clínico dos sujeitos que chegaram ao pronto atendimento representou uma coorte de pacientes reumáticos em estágios mais avançados de doença. Esses pacientes requerem atendimento prioritário em serviço ...

Rotura espontánea de válvula tricúspide en un paciente con hipertensión pulmonar secundaria a VIH / Spontaneous rupture of tricuspid valve papillary muscle in pulmonary hypertension secondary to HIV infection.Report of one case

Acute primary tricuspid regurgitation (TR) secondary to papillary muscle rupture is an extremely rare clinical situation. We report a 42-year-old male with pulmonary artery hypertension (PAH) secondary to HIV infection, who presented with an acute TR due to spontaneous papillary muscle rupture. He remained in cardiogenic shock despite therapy with inotropic drugs and pulmonary vasodilator therapy. He was subjected to a tricuspid valve replacement. In the postoperative period the patient had severe PAH, which was successfully controlled with inhaled nitric oxide. Tricuspid valve replacement and adjunctive use of pulmonary vasodilator therapy can be a life saving and useful approach in this condition.

Endocardite marântica e adenocarcinoma de sítio primário oculto / Marantic endocarditis and adenocarcinoma of unknown primary site / Endocarditis marántica y adenocarcinoma de sitio primario desconocido

A endocardite trombótica não bacteriana é caracterizada pela deposição de trombos e fibrina sobre valvas cardíacas normais ou degeneradas na ausência de germes. A patologia está relacionada a estados inflamatórios crônicos, com maior incidência de fenômenos embólicos quando comparada à endocardite infecciosa. Os autores relatam o caso de um paciente masculino, 63 anos, com adenocarcinoma de sítio primário oculto e embolia sistêmica.

Nonbacterial thrombotic endocarditis (NBTE) is a disease characterized by deposition of thrombi and fibrin on normal or degenerated cardiac valves in the absence of microorganisms. This condition is more commonly seen in chronic inflammatory states, and is associated with higher incidence of thromboembolic events than infective endocarditis. We report the case of a 63-year old male patient with adenocarcinoma of unknown primary site and systemic embolism.

La endocarditis trombótica no bacteriana se caracteriza por el depósito de trombos de fibrina en las válvulas cardíacas normales o degenerados en ausencia de gérmenes. La patología se relaciona con estados inflamatorios crónicos, con una mayor incidencia de fenómenos embólicos cuando comparada a endocarditis infecciosa. Los autores refieren el caso de un paciente varón, de 63 años con adenocarcinoma de sitio primario desconocido y la embolia sistémica.

Quiste hidatídico hepático complicado con invasión a vena cava inferior y embolización de membranas hidatídicas a aurícula derecha: caso clínico / Hepatic hydatid cyst invading inferior vena cava with embolic membranes to right atrium: report of one case

We report a 30 year old female admitted with a story of right upper quadrant pain and previous hepatic surgery for hydatid disease. A thoracoabdominal angio CAT sean and transesophageal echocardiogram revealed a mass located in the right atrium. The patient was operated and the right atrial mass was excised. Pathological study of the surgical piece confirmed a hydatid cyst. Post operative recovery was uneventful.

La hidatidosis es una zoonosis parasitaria, de mayor incidencia en el sur de Chile. Su localización más frecuente es hepática y pulmonar. Sus complicaciones derivan de su crecimiento y afección de estructuras adyacentes o de su ruptura a cavidades. Presentamos un caso de quiste hidatídico hepático complicado, en una paciente que debutó con cuadro de dolor abdominal localizado en hipocondrio derecho producto de una lesión quística hepática con comunicación a vena cava inferior, siendo intervenida en su hospital de origen. Posteriormente AngioTAC y ecocardiografía transesofágica confirmaron masa intra auricular. Se realizó extracción de la masa bajo paro circulatorio con hipotermia profunda, correspondía a membranas hidatídicas y trombos adheridos a ella. La paciente evolucionó satisfactoriamente.

Distúrbios cardiovasculares na acromegalia: [revisão] / Cardiovascular disturbances in acromegaly: [review]

A acromegalia acarreta uma série de distúrbios ao sistema cardiovascular, decorrentes da exposição crônica a níveis elevados de GH e IGF-1. Estes distúrbios são os principais responsáveis pelo aumento da mortalidade de acromegálicos. Entre as várias formas de acometimento cardiovascular, destaca-se a miocardiopatia acromegálica, entidade caracterizada, inicialmente, pelo estado hiperdinâmico, seguido de hipertrofia ventricular esquerda concêntrica e disfunção diastólica por déficit de relaxamento, culminando com disfunção sistólica e, por vezes, insuficiência cardíaca franca. Além disso, são também relevantes as arritmias, as valvulopatias, sobretudo mitral e aórtica, a cardiopatia isquêmica, a hipertensão e os distúrbios dos metabolismos glicêmico e lipídico. Nesta revisão são abordados os principais aspectos clínicos e prognósticos destas entidades, os efeitos do tratamento da acromegalia sobre elas e as repercussões correspondentes sobre a sobrevida dos pacientes.

Acromegaly causes a number of disorders in the cardiovascular system, resulting from chronic exposure to high levels of GH and IGF-1. Such disorders are the main responsible for increased mortality rates among acromegalic patients. Among several forms of cardiovascular impairment is acromegalic cardiomyopathy, an entity that is initially characterized by a hyperdynamic state, followed by concentric left ventricular hypertrophy and diastolic dysfunction due to relaxation deficit, culminating in systolic dysfunction and sometimes heart failure. In addition, arrhythmias and heart valve diseases are also relevant, especially mitral and aortic, ischemic heart disease, hypertension, and glucose and lipid metabolism disorders. This review approaches the main clinical and prognostic aspects of these entities, the effects of acromegaly treatment on them, and the respective consequences on patient survival.

Troca valvar aórtica com diferentes próteses: existem diferenças nos resultados da fase hospitalar? / Aortic valve replacement with different types of prosthesis: are there differences in the outcomes during hospital phase?

OBJETIVOS: Analisar dados intra-operatórios e possíveis diferenças na evolução clínica da fase hospitalar de pós-operatório da troca valvar aórtica com diferentes próteses. MÉTODOS: Análise de 60 pacientes, divididos em três grupos: os submetidos a troca valvar por prótese biológica (20); por prótese mecânica (20); e finalmente, por valva homóloga (20). A média da idade foi de 51,1 anos; 60 por cento eram do sexo masculino e 40 por cento do feminino; 86,7 por cento estavam em NYHA II ou III; 63,3 por cento eram hipertensos, 18,3 por cento diabéticos; a etiologia valvar foi degenerativa em 39 por cento, reumática em 36 por cento e endocardite em 15 por cento. RESULTADOS: A mortalidade hospitalar foi de 5 por cento; não houve diferenças entre os grupos na incidência de choque séptico ou cardiogênico, insuficiência renal aguda, arritmias no centro cirúrgico e na unidade de terapia intensiva (UTI), assim como para o tempo de internação na UTI e tempo de ventilação mecânica. Houve diferença estatística nos tempos de circulação extracorpórea (P=0,02) e pinçamento aórtico (P<0,0001) desfavorável aos pacientes com valva homóloga. O tempo de internação na enfermaria foi maior para os pacientes com valva mecânica (P=0,05), assim como o tempo total de internação hospitalar tende a ser maior neste grupo, porém sem significância estatística. Pacientes com hematócrito pré-operatório abaixo de 38,1 por cento utilizaram 2,73 unidades de hemoderivados; e com hematócrito no pós-operatório abaixo de 32 por cento utilizaram 1,79 unidades. Controle ecocardiográfico mostrou mínimas diferenças evolutivas. CONCLUSÃO: A utilização de diferentes próteses, nesta amostra, não gerou diferenças nos resultados da fase hospitalar de pós-operatório; O homoenxerto é uma opção viável e com boa aplicabilidade clínica.

OBJECTIVE: To analyze intraoperative data and possible differences in clinical evolution during postoperative hospital phase for aortic valve replacement surgery using different types of prosthesis. METHODS: Analysis of 60 patients divided into three groups. Valve replacement with bioprosthesis (20), mechanical prosthesis (20) and homologous valve (20). The mean age was 51.1, 60 percent were male and 40 percent female patients; 86.7 percent were in NYHA II or III; 63.3 percent presented arterial hypertension and 18.3 percent had diabetes. Aetiology of valve disease was degenerative for 39 percent, rheumatic for 36 percent and endocardits for 15 percent. RESULTS: The hospital mortality was 5 percent; there were no differences in the incidence of septical or cardiogenic shock, acute renal failure, rhythms disorders during surgery or intensive care, neither for total time in intensive care and mechanical ventilation. However, there was statistical differences as regards the cardiopulmonary bypass total time (P=0.02) and the aortic clamping time (P<0.0001) unfavorable to homograft valve group. The ward admission time was greater for mechanical valve group (P=0.05) as well as for total admission time, but without statistical significance. It was observed that patients with preoperative hematocrit smaller than 38.1 percent used 2.73 units of blood components, and with postoperative hematocrit smaller than 32 percent used 1,79 units of blood components. Echocardiography control showed minimal evolutional differences. CONCLUSION: The use of different types of prosthesis for this study does not cause differences in the results of postoperative hospital phase. The use of homograft valve is a feasible option with good clinical applicability.

Enfermedad reumática cardiovascular: observaciones anatomopatológicas en biopsias valvulares de jóvenes con menor e igual a 20 años de edad / Cardiovascular rheumatic disease: pathologic´s observations in biopsies valvulares of young people minor and equal 20 years of age

La fiebre reumática es una enfermedad endémica en los países en desarrollo como Venezuela y es causa importante de valvulopatía crónica progresiva, siendo primeramente afectado el aparato valvular mitral. Evaluar el aspecto morfológico de la valvulitis crónica en su etapa inicial, en individuos quienes han sido sometidos a reemplazo valvular mitral o mitro-aórtico. Se seleccionaron 20 biopsias de válvulas mitrales y mitro/aórticas diagnosticadas como valvulopatía crónica reumática, representativas de la etapa inicial, en pacientes con edad igual o menos de 20 años. Los diagnósticos pre-operatorios fueron: disfunción valvular severa mitral (insuficiencia (60%), doble lesión (10%) y estenosis (5%) y mitro-aórtica (25%). Las biopsias (valvas y aparatos valvulares mitrales) fueron evaluadas macroscópicamente. El estudio histológico se hizo con secciones teñidas con Hematoxilina-eosina e inmunomarcadas con anticuerpos CD 34 y antimúsculo liso. Todos los casos mostraron lesiones morfológicas propias de la valvulitis crónica reumática inicial: inflamación grado 1 (81,6%), con nódulos de Aschoff (35%), fibrosis de grado 2 (40%) y 3 (60%) Con una alta densidad de neovascularización (14,78 ± 1,92). Se hizo el análisis de los hallazgos morfológicos y se consideró que este trabajo puede servir de base para investigaciones comparativas con casos de etapas más avanzadas de la enfermedad valvular en relación a la inflamación, remodelado colágeno y angiogénesis.

Rheumatic fever is an endemic disease in developing countries such as Venezuela and is an important cause of progressive chronic valve disease, the mitral valve apparatus being affected in the first place. To evaluate the morphologic aspect of chronic valvulitis in its initial stage among individuals that have gone through mitral and mitro-aortic valve replacement. Twenty (20) biopsies of mitral and mitro-aortic valves diagnosed with rheumatic chronic valvulopathy in its initial stage were selected from patients aged 20 years or younger. Preoperating diagnoses were as follows: severe mitral valve dysfunction (insufficiency: 60%, double injury: 10%, stenosis: 5%, and mitro-aortic: 25%). The biopsy material (valve apparatuses) was macroscopically evaluated. A histological study was made on haematoxylin-eosin stained and CD34 antibody immunomarked sections and smooth antimuscle. All cases showed morphologic lesions typical of initial chronic rheumatic valvulitis: grade 1 inflammation (81,6%), with Aschoff's bodies or nodules (35%), grade 2 fibrosis (40%), and grade 3 fibrosis (60%) with high density neovascularization (14,78 ± 1,92). Morphologic findings were analyzed, thus concluding that this work can serve as a basis for comparative investigations on case of valvular disease at more advanced stages in terms of inflammation, collagen remodeling and angiogenesis.

Doença arterial coronariana em pacientes com valvopatia reumática e não-reumática acompanhados em hospital público do Rio de Janeiro / Coronary artery disease in patients with rheumatic and non-rheumatic valvular heart disease treated at a public hospital in Rio de Janeiro

OBJETIVO: Avaliar a prevalência de doença arterial coronariana (DAC) na valvopatia de etiologia reumática e não-reumática, examinando possíveis fatores preditivos da presença da doença. MÉTODOS: Estudo transversal, de série de casos obtidos em população pré-definida. Foram avaliados 1.412 pacientes com indicação de cirurgia cardíaca por qualquer etiologia. Destes, foram encontrados e estudados 294 casos com valvopatia primária de etiologias reumática e não-reumática, com idade > 40 anos, submetidos a coronariografia. RESULTADOS: Os valvopatas reumáticos apresentaram menor prevalência de DAC (4 por cento) que os não-reumáticos (33,61 por cento) (p < 0,0001). O modelo de regressão logística evidenciou que idade, dor torácica típica, hipertensão arterial sistêmica (HAS), diabete melito e dislipidemia estavam significativamente relacionados à DAC, e que a etiologia reumática não era determinante da doença. Tabagismo e sexo revelaram-se de importância clínica na DAC, embora sem significância estatística. No grupo total, o modelo de análise Log linear demonstrou que, independentemente da etiologia, sexo, idade > 55 anos, HAS, dor torácica típica, diabete e dislipidemia se relacionavam diretamente com a DAC, sendo as três últimas as variáveis de maior peso para a doença. CONCLUSÃO: A prevalência de DAC é baixa entre valvopatas reumáticos e mais alta entre não-reumáticos; a etiologia reumática não parece exercer efeito protetor sobre a prevalência de DAC; e as variáveis sexo, idade, HAS, dor torácica típica, dislipidemia e diabete melito foram identificadas como fortemente associadas à presença de DAC. É possível definir critérios de indicação de coronariografia pré-operatória nas trocas valvares, podendo-se evitar a indicação rotineira a partir dos 40 anos.

OBJECTIVE: to estimate the prevalence of coronary artery disease (CAD) in valvular heart disease of rheumatic (RVHD) and non-rheumatic (NVHD) etiology, assessing possible predictive factors for the presence of CAD. METHODS: This is a cross-sectional study of a series of cases obtained from a pre-defined population, wherein 1,412 patients referred for heart surgery of any etiology were evaluated. Of these, 294 primary heart disease patients aged >40 submitted to cinecoronary arteriography (CA) were identified and studied. RESULTS: patients with RVHD presented lower prevalence of CAD (4 percent) when compared to NVHD (33.61 percent), p<0.0001. The logistic regression analysis showed that age, typical angina-like chest pain (TACP), systemic arterial hypertension (SAH), diabetes and dyslipidemia were significantly related to CAD, and that the rheumatic etiology was not a disease determinant. Smoking and gender were clinically important in CAD, although not statistically significant. In the whole group, the Log-linear analysis showed that, regardless of the etiology, gender, age >55, SAH, TACP, diabetes and dyslipidemia were all related directly to CAD, with the latter three being the most important variables for the disease. CONCLUSION: the prevalence of CAD among RVHD patients is low, whereas it is high among NVHD patients; the rheumatic etiology does not seem to have any beneficial effects on the prevalence of CAD; gender, age, SAH, TACP, dyslipidemia and diabetes were identified as being strongly associated with the presence of CAD. It is possible to define the criteria that indicate the need for pre-surgical CA in heart valve replacements, so that the standard indication after the age of 40 years can be avoided.

Compromiso cardiovascular en el lupuseritematoso sistémico / Cardiovascular involvement in patient with systemic lupus erythematosus

Lupus erythematosus is a multisystemic disease that compromises principally women in fertile age. The principal affected organs are kidney, SNC, bone marrow and serous membranes. Cardiovascular affection includes pericardium, conduction system, myocardium, valves and coronary arteries. The most frequent valve disease is Libman-Sacks endocarditis, although valvulitis or valve dysfunction can exist as well. The mitral valve is the most affected, followed by the aortic valve. The most frequent valve abnormality is slight to moderate aortic insufficiency, while serious insufficiency or valve disruption is very rare. A physical examination has limited efficacy in the diagnosis of valve disease. A high degree of suspicion associated with echocardiography helps to establish the diagnosis. If surgery is not needed, antiplatelet therapy is recommended for asymptomatic patients, and oral anticoagulation treatment is advised for those with valve disease and evidence of thromboembolic phenomena. Recurrence of the disease has been observed in biological grafts, which makes the use of mechanical prostheses advisable when valve replacement indication exists. The following case shows the clinical evolution of a female patient with a rare but very serious lupus erythematosus complication.

Low Molecular Weight Heparin Treatment in Pregnant Women with a Mechanical Heart Valve Prosthesis

No definitive recommendation is available concerning optimal antithrombotic therapy in pregnant women with a mechanical heart valve. The purpose of the current study was to evaluate the clinical results of nadroparin treatment with respect to pregnancy outcome and maternal complications. From 1997 to 2005, 31 pregnancies were reviewed in 25 women. Nadroparin (7,500 U, twice daily) was used in 23 pregnancies between 6 and 12 weeks of gestation and close-to-term only, and coumarin derivatives were used with aspirin at other times. Eight pregnant women treated with coumarin derivatives throughout pregnancy were compared to evaluate the safety and efficacy of nadroparin. No maternal death or bleeding complication occurred in either of the two groups, and frequencies of maternal thromboembolism including valve thrombosis (8.7% vs. 12.5%, p>0.05) were similar. However, the frequencies of live born (91.3% vs. 50%, p=0.01) and healthy babies (90.4% vs. 25%, p<0.01) were significantly higher, and the fetal loss rate was significantly lower (8.7% vs. 50%, p=0.01) in the nadroparin-treated group. Regarding the efficacy and safety of antithrombotic treatment in pregnant women with prosthetic heart valves, nadroparin treatment during the first trimester is an acceptable regimen and produces better results than coumarin derivatives.

Echocardiogram: Screening for valvular heart disease in an out- patient clinic

Congenital and acquired Valvular heart diseases [VHD] remain a frequent cause of morbidity and mortality especially in the developing countries. It presents a diagnostic challenge in all age groups and often occurs in association with other types of heart diseases. Echocardiogram remains a gold standard for the diagnosis and periodic assessment of patients with VHD. Early recognition is important in the management of VHD. Therefore, this study was conducted to describe the pattern of various VHD in patients presenting at the National Institute of Cardiovascular Diseases [NICVD] Karachi, Pakistan. Among 525 patients studied we found 403 patients with Mitral Regurgitation [MR], 187 Tricuspid Regurgitation [TR], 182 Aortic Regurgitation [AR], 134 Mitral Stenosis [MS], 60 Aortic Stenosis [AS], 11 Pulmonic Stenosis [PS], 8 Tricuspid Stenosis [TS] and 8 Pulmonic Regurgitation [PR]. Combined mitral valve disease [both MS and MR] 90 [17.1%], combined aortic valve disease [both AS and AR] 50 [9.5%], mixed valvular heart disease i-e both aortic and mitral valves disease were 85 [16.2%]. Rheumatic heart disease [RHD] was present in 189 patients [36%] with mean age of 28.4 +/- 11.8 years

Pathology and etiology of 110 consecutively removed aortic valves.

OBJECTIVES: To study the pathology and determine the etiology and prevalence of aortic valve disease from surgically removed aortic valve specimens. MATERIAL AND METHOD: All the native surgically excised aortic valves (AV) received from June 1997 to March 1999 (22 months) were studied macroscopically including cuspal measurements and microscopically. By preoperative echocardiographic and macroscopic studies, they were classified into functional disorders of predominant aortic stenosis (AS), aortic stenosis with regurgitation (AS-AR) and predominant aortic regurgitation (AR). The patients' medical records were reviewed and the clinical information was extracted. The etiology was determined according to the macroscopic, microscopic and clinical findings. RESULTS: Among 110 AV (76 isolated AV and 34 with concomitant mitral valves from patients aged 15-96 years, mean age 47.54 years; male:female = 1.39:1) there were 25 AS (22.73%), 34 AS-AR (30.91%) and 51 AR (46.36%) cases. Eighty-four (76.36%) were tricuspid, 16 (14.54%) were bicuspid and 10 were undetermined. Cuspal measurements of each disease were provided and compared. All AS specimens were related to moderate to severe calcification and causes included postinflammatory disease (14 cases, 56%; age range 38-67 years, mean age 53.29 years, male:female = 0.56:1), degenerative calcific change (11 cases, 44%, age range 56-76 years, male:female = 1.2:1; mean age 69 years of 5 tricuspid AV and 60.83 years of 6 bicuspid AV). In AS-AR, 29 cases (85.29%; mean age 47.10 years; male:female = 1.23:1) were attributable to postinflammatory disease and 5 cases (mean age 70.20 years; male:female = 1.5:1) to degenerative calcific change. In pure AR, there were 21 cases (age range 15-65 years, mean age 29.76 years) of postinflammatory disease, 14 cases of infective endocarditis (IE) and postIE (age range 20-63 years, mean age 42.21 years; all 10 IE cases contained gram positive cocci), 1 case (age 55 years) of bicuspid calcific change, 8 cases of AV with dilated valve ring, 5 cases of miscellaneous causes and 2 cases of indeterminate etiology. Aschoff bodies were found in 3 AR cases. Four of 18 postinflammatory AS-AR and 4 of 14 postinflammatory disease AR cases had past history of rheumatic fever. One postinflammatory AS also had infective endocarditis from gram positive cocci without clinical sign. Severe degenerative calcific change had a higher incidence of underlying diabetes (3 of 15 cases, 20%), hypertension (8 of 14 cases, 57.14%) and dyslipoproteinemia (9 of 13 cases, 69.23%) in comparison with 3.37% (3/89) for diabetes, 9.09% (8/88) for hypertension and 30.99% (22/71) for dyslipoproteinemia in other AV diseases in combination. CONCLUSION: The three common causes of severe AV functional disorders were postinflammatory disease (58.18%), degenerative calcific change (15.45%) and IE-postIE (12.72%). Underlying diseases of severe degenerative calcific change included hypertension, dyslipoproteinemia and diabetes. Macroscopic and microscopic examinations together with clinical information, echocardiographic findings and operative details are important in evaluating the etiology of valvular diseases especially in severely calcified specimens.