Aneurisma aórtico e insuficiencia valvular severa por sífilis terciaria en paciente joven, una enfermedad desatendida / Aortic aneurysm and severe valvular insufficiency due to tertiary syphilis in a young patient, a neglected disease / Aneurisma da aorta e insuficiência valvular grave devido à sífilis terciária em um paciente jovem, uma doença negligenciada

La sífilis es causada por Treponema pallidum, es reconocida por la variedad de sus manifestaciones clínicas; compromete el sistema nervioso, tegumentario y cardiovascular. Las alteraciones cardiovasculares ocurren en 70% de los casos con sífilis no tratada, el 71% de las aortitis sifilíticas desarrollan aneurismas aórticos y la complicación más frecuente es la insuficiencia aórtica. El tratamiento va dirigido a la enfermedad subyacente, con manejo antibiótico para la sífilis terciaria y las complicaciones tromboembólicas y si requiere reparación quirúrgica. Presentamos un caso poco frecuente de un paciente diagnosticado de infección por el virus de la inmunodeficiencia humana y complicaciones cardiovasculares típicas de la sífilis terciaria, que precisó tratamiento médico y quirúrgico, que fue exitoso. Destacamos la pericia clínica de los profesionales de nuestra institución para diagnosticar e identificar las complicaciones cardiovasculares de la sífilis terciaria.

Syphilis is caused by Treponema pallidum and is recognized by the variety of its clinical manifestations; it involves the nervous, integumentary and cardiovascular systems. Cardiovascular alterations occur in 70% of cases with untreated syphilis, 71% of syphilitic aortitis develop aortic aneurysms and the most frequent complication is aortic insufficiency. Treatment is directed at the underlying disease, with antibiotic treatment for tertiary syphilis and thromboembolic complications and if necessary surgical repair. We present a rare case of a patient diagnosed with human immunodeficiency virus infection and cardiovascular complications typical of tertiary syphilis, who required medical and surgical treatment, which was successful. We highlight the clinical experience of our institution's professionals in the diagnosis and identification of cardiovascular complications of tertiary syphilis.

A sífilis é causada pelo Treponema pallidum e é reconhecida pela variedade de suas manifestações clínicas; ela afeta os sistemas nervoso, tegumentar e cardiovascular. As alterações cardiovasculares ocorrem em 70% dos casos de sífilis não tratada, 71% dos casos de aortite sifilítica desenvolvem aneurismas aórticos e a complicação mais frequente é a insuficiência aórtica. O tratamento é direcionado para a doença subjacente, com tratamento antibiótico para sífilis terciária e complicações tromboembólicas e, se necessário, reparo cirúrgico. Apresentamos um caso raro de um paciente diagnosticado com infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que precisou de tratamento médico e cirúrgico, que foi bem-sucedido. Apresentamos um caso raro de um paciente com diagnóstico de infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que necessitou de tratamento médico e cirúrgico, o qual foi bem-sucedido. Destacamos a experiência clínica dos profissionais de nossa instituição no diagnóstico e na identificação das complicações cardiovasculares da sífilis terciária.

Kissing lesion of mitral valve - a case report

Abstract Aortic valve endocarditis can lead to secondary involvement of aorto-mitral curtain and the adjacent anterior mitral leaflet (AML). The secondary damage to AML is often caused by the infected jet of aortic regurgitation hitting the ventricular surface of the mitral leaflet, or by the pronounced bacterial vegetation that prolapses from the aortic valve into the left ventricular outflow tract. This is called 'kissing lesion'. We describe a patient with infective endocarditis of the aortic valve causing perforation of both noncoronary cusp of aortic valve and the AML, which is rare.

Rotura aguda de prótesis valvular aórtica biológica Biocor® seis años después de su implante: reporte de un caso / Acute rupture a a Biocor® aortic valve prosthesis six years after implantation

Resumen: Un hombre de 66 años portador de una prótesis valvular aórtica SJM Biocor® (Saint Jude Medical, St Paul, Minn), instalada 6 años antes, se presentó con disnea severa de rápida instalación. Mediante ecocardiografía de superficie y transesofágica se demostró insuficiencia aórtica masiva y el paciente fue reintervenido con éxito. En la operación se observó un desprendimiento parcial de la inserción del velo coronario izquierdo al anillo protésico. No había endocarditis. Se implantó una nueva prótesis biológica Trifecta® (Saint Jude Inc, St Paul, Minn) N°27. La evolución precoz y a 2 años de la intervención es muy satisfactoria.

Abstract A 66 year-old man with a SJM Biocor® (Saint Jude Medical, St Paul, Minn) implanted 6 years before developed unexpected severe dyspnea. Surface and transesophageal echocardiograms confirmed severe aortic regurgitation. The patient was rapidly re operated on. There was a partial detachment of the left coronary leaflet form the valve annulus. There was no evidence of endocarditis. A new valve (Trifecta® (Saint Jude Inc, St Paul, Minn) N°27). was inserted. Clinical course has been uneventful up to two years post surgery.

Relapsing polychondritis, a rare cause of valvulopathy: A review of the medical literature / Policondritis recurrente, una rara causa de valvulopatía: revisión de las publicaciones médicas

Abstract Relapsing polychondritis (RP) is an inflammatory disease that involves cartilaginous structures predominantly in the nose, ears, and respiratory tract. Cardiovascular involvement is not common. Despite this, they are the second cause of death in patients with RP. The structures usually affected by this disease are the heart valves, with regurgitation being the most common valvulopathy. We present the case of a patient without the previous diagnosis of RP who was referred to our institute with heart failure secondary to aortic regurgitation, initially attributed to endocarditis.

Resumen La policondritis recurrente (PR) es una enfermedad inflamatoria que afecta a estructuras cartilaginosas, predominantemente las que se encuentran en nariz, pabellones auriculares y vías respiratorias. Las manifestaciones cardiovasculares son poco comunes; sin embargo, son la segunda causa de mortalidad en pacientes con PR. Unas de las estructuras afectadas casi siempre en la PR son las estructuras valvulares y la valvulopatía más común es la insuficiencia aórtica (IA). A continuación se presenta el caso de una paciente sin diagnóstico previo de PR a quien se refirió a este instituto por insuficiencia cardíaca secundaria a IA, atribuida en un principio a endocarditis.

Válvula aórtica cuadricúspide: imágenes intraoperatorias / Quadricuspid aortic valve: intraoperative images

Resumen: Recientemente, Sepúlveda et al. publicaron en la Revista Chilena de Cardiología(1) el caso clínico de una mujer de 60 años con una válvula aórtica cuadricúspide, diagnosticada por ecocardiografía transtorácica y transesofágica, no operada, con una extensa revisión de la literatura respectiva. En esta oportunidad, nosotros presentamos los hallazgos intraoperatorios en un hombre de 72 años operado por una insuficiencia aórtica severa secundaria a una válvula aórtica cuadricúspide no diagnosticada preoperatoriamente.

Abstracts: Recently, Sepúlveda et al. published in Revista Chilena de Cardiología1 the clinical case of a 60-year-old woman with quadricuspid aortic valve diagnosed by transthoracic and transesophageal echocardiography not operated on, including an extensive review of the literature. We present the intraoperative findings of a 72-year-old man with severe aortic insufficiency and a quadricuspide aortic valve, not preoperatively diagnosed.

The Role of Quantitative Aortographic Assessment of Aortic Regurgitation by Videodensitometry in the Guidance of Transcatheter Aortic Valve Implantation / Papel da Avaliação Aortográfica Quantitativa da Regurgitação Aórtica por Videodensitometria na Orientação do Implante da Valva Aórtica Transcateter

Abstract Background: Balloon post-dilatation (BPD) is often needed for optimizing transcatheter heart valve (THV) implantation, since paravalvular leak (PVL) after transcatheter aortic valve implantation is associated with poor outcome and mortality. Quantitative assessment of PVL severity before and after BPD is mandatory to properly assess PVL, thus improving implantation results and outcomes. Objective: To investigate a quantitative angiographic assessment of aortic regurgitation (AR) by videodensitometry before and after BPD. Methods: Videodensitometric-AR assessments (VD-AR) before and after BPD were analysed in 61 cases. Results: VD-AR decreased significantly from 24.0[18.0-30.5]% to 12.0[5.5-19.0]% (p < 0.001, a two-tailed p < 0.05 defined the statistical significance). The relative delta of VD-AR after BPD ranged from -100% (improvement) to +40% (deterioration) and its median value was -46.2%. The frequency of improvement, no change, and deterioration were 70% (n = 43), 25% (n = 15) and 5% (n = 3), respectively. Significant AR (VD-AR > 17%) was observed in 47 patients (77%) before and in 19 patients (31%) after BPD. Conclusions: VD-AR after THV implantation provides a quantitative assessment of post-TAVI regurgitation and can help in the decision-making process on performing BPD and in determining its efficacy.

Resumo Fundamento: A pós-dilatação com balão (PDB) é normalmente necessária para otimização do implante da válvula cardíaca transcateter (THV), uma vez que o "escape" ou leak paravalvar (PVL) após implante de valva aórtica transcateter está associada com desfecho ruim e mortalidade. A avaliação quantitativa da gravidade do PVL antes e após a PDB é mandatória para se avaliar adequadamente o PVL e, assim, melhorar os resultados e os desfechos do implante. Objetivo: Investigar uma avalição angiográfica quantitativa da regurgitação aórtica (RA) por videodensitometria (VD-RA) antes e após a PDB. Métodos: Resultados da VD-RA antes e após a PDB foram analisados em 61 casos. Resultados Houve diminuição significativa da VD-RA de 24,0(18,0-30,5)% para 12,0(5,5-19,0)% (p < 0,001; p < 0,05 bilateral foi definido como significância estatística). O delta relativo de VD-RA após a PDB variou de -100% (melhora) a +40% (piora) e o valor mediano foi -46,2%. As frequências de melhora, ausência de mudança, e piora foram 70% (n = 43), 25% (n = 15) e 5% (n = 3), respectivamente. Observou-se RA significativo (VD-RA > 17%) em 47 pacientes (77%) antes e em 19 pacientes (31%) após a PDB. Conclusões: A VD-RA após o implante de THV possibilita a avaliação quantitativa da regurgitação pós-TAVI, e pode auxiliar na tomada de decisão quanto à realização ou não da PDB, bem como na avaliação de sua eficácia.

Mid- and Longterm Neo-Aortic Valve Regurgitation after Jatene Surgery: Prevalence and Risk Factors / Regurgitação da Valva Neo-Aórtica a Médio e Longo Prazo após Cirurgia de Jatene: Prevalência e Fatores de Risco

Abstract Background: Jatene surgery became the surgical procedure of choice to repair transposition of the great arteries (TGA) in neonates and infants. Late complications, mainly related to the pulmonary outflow tract and coronary arteries, are well known. The behavior of the neo-aortic valve is a cause of concern because of its potential for requiring late reoperation. Objectives: To assess the prevalence and risk factors of neo-aortic valve regurgitation in 127 patients in the late postoperative period of the Jatene surgery. Methods: Of the 328 survivors of the Jatene surgery at the Biocor Institute from October 1997 to June 2015, all patients undergoing postoperative follow-up were contacted via telephone, 127 being eligible for the study. The patients were divided into two groups, simple TGA and complex TGA groups, with follow-up means of 6.4 ± 4.7 years and 9.26 ± 4.22 years, respectively. Echocardiography was performed with adjusted measurements (Z-score) of the neo-aortic annulus, sinus of Valsalva, sinotubular region and ascending aorta, as well as quantification of the neo-aortic valve regurgitation grade. Results: The incidence of mild neo-aortic valve regurgitation was 29% in a follow-up of 7.4 ± 4.7 years. Moderate regurgitation was identified in 24 patients with age mean (± standard-deviation) of 9.81 ± 4.21 years, 19 of whom (79%) in the complex TGA group. Those patients had a higher aortic annulus Z-score. The reoperation rate due to neo-aortic regurgitation associated with aortic dilation was 1.5%, all patients in the complex TGA group. Conclusion: This study shows that, despite the low incidence of reoperation after Jatene surgery due to neo-aorta dilation and neo-aortic valve regurgitation, that is a time-dependent phenomenon, which requires strict vigilance of the patients. In this study, one of the major risk factors for neo-aortic valve regurgitation was the preoperative pulmonary artery diameter (p < 0.001).

Resumo Fundamento: A operação de Jatene tornou-se o procedimento cirúrgico de escolha para o reparo da transposição das grandes artérias (TGA) em neonatos e lactentes. Complicações tardias, principalmente relacionadas à via de saída pulmonar e às artérias coronarianas, já são bem reconhecidas. O comportamento da valva neo-aórtica tem sido motivo de crescente preocupação pelo seu potencial de necessidade de reoperações tardias. Objetivos: Avaliar a prevalência e os fatores de risco associados à regurgitação da valva neo-aórtica em 127 pacientes em pós-operatório tardio de cirurgia de Jatene. Métodos: Dos 328 sobreviventes da cirurgia de Jatene no Biocor Instituto operados de outubro de 1997 a junho de 2015, todos os pacientes em seguimento de pós-operatório foram contatados via ligação telefônica, sendo 127 elegíveis para o estudo. Os pacientes foram divididos em dois grupos, Grupo TGA simples e Grupo TGA complexa, com médias de follow-up de 6,4 ± 4,7 anos e 9,26 ± 4,22 anos, respectivamente. Foi realizada avaliação ecocardiográfica com medidas indexadas (escore Z) do anel da valva neo-aórtica, do seio de Valsalva, da região sinotubular e da aorta ascendente, bem como quantificação do grau de regurgitação da valva neo-aórtica. Resultados: A incidência de leve regurgitação da valva neo-aórtica em nossa casuística foi de 29% em um follow-up de 7,4 ± 4,7 anos. Regurgitação moderada foi observada em 24 pacientes, com idade média (± desvio-padrão) de 9,81 ± 4,21 anos, sendo 19 (79%) no Grupo TGA complexa. Nesses pacientes, observou-se maior escore Z do anel aórtico. A taxa de reoperação por regurgitação da valva neo-aórtica associada a dilatação da aorta foi de 1,5%, sendo todos os casos no Grupo TGA complexa. Conclusão: O estudo demonstra que, embora reoperações após cirurgia de Jatene por dilatação da neo-aorta e regurgitação da valva neo-aórtica tenham incidência baixa, esse é um fenômeno dependente de tempo, requerendo rígida vigilância desses pacientes. Na nossa casuística, um dos principais fatores de risco para regurgitação da valva neo-aórtica foi o diâmetro da artéria pulmonar no pré-operatório (p < 0,001).

Insuficiencia aórtica aguda por endocarditis infecciosa / Acute aortic regurgitation due to infective endocarditis

La insuficiencia aórtica aguda (IAOA) por endocarditis infecciosa (EI) es grave y generalmente requiere tratamiento quirúrgico. Se compararon los pacientes con IAOA grave por EI e insuficiencia cardíaca (IC) en clase funcional I-II NYHA (G1) con los pacientes en clase funcional III-IV (G2) en relación a características clínicas, ecocardiográficas, microbiológicas y evolución hospitalaria y se evaluaron los predictores de mortalidad, en un centro de alta complejidad. Desde 06/92 a 07/16, de 439 pacientes con EI, 86 presentaron IAOA: (G1, 39: 45.4% y G2, 47: 54.7%). El G1 presentó mayor EI protésica (43.6% vs. 17.0%; p < 0.01). Los 47 casos G2 presentaban disnea vs. 12 (30.8%) G1 (p < 0.0001). No hubo diferencias en cuanto a las características clínicas, ecocardiográficas y microbiológicas. El tratamiento quirúrgico fue principalmente por extensión de la infección y/disfunción valvular en el G1 y por IC en el G2. La mortalidad hospitalaria fue del 15.4% vs. 27.7% (G1 y G2 respectivamente, p NS). Fueron predictores en el análisis multivariado: la infección intrahospitalaria (p 0.001), los hemocultivos negativos (p 0.004) y la presencia de IC clase funcional III-IV (p 0.039).Una quinta parte de los pacientes con EI presentaron IAOA. Aquellos con IC grave requirieron tratamiento quirúrgico de emergencia y con IC con clase funcional I-II requirieron cirugía por extensión de la infección y/o disfunción valvular. La mortalidad quirúrgica y hospitalaria continúan siendo elevadas en ambos grupos y fueron predictores de mortalidad hospitalaria: la infección intrahospitalaria, los hemocultivos negativos y la IC avanzada.

Acute aortic regurgitation (AAR) due to infective endocarditis (IE) is a serious disease and usually requires surgical treatment. Our study aims to compare the clinical, echocardiographic, and microbiological characteristics as well as in-hospital outcome of patients with AAR according to the severity of heart failure (HF) and to evaluate predictors of in-hospital mortality in a tertiary centre. In a prospective analysis, we compared patients with NYHA functional class I-II HF (G1) vs. functional class III-IV HF (G2). From 06/92 to 07/16, 439 patients with IE were hospitalized; 86 presented AAR: (G1, 39: 45.4% y G2, 47: 54.7%). The G1 had higher prosthetic IE (43.6% vs. 17%, p 0.01). All G2 patients had dyspnoea vs. 30.8% of the G1 (p < 0.0001). There were no differences in clinical, echocardiographic and microbiological characteristics. Surgical treatment was indicated mainly due to infection extension or valvular dysfunction in G1 and HF in G2. In-hospital mortality was 15.4% vs. 27.7% (G1 and G2 respectively p NS). In multivariate analysis, health care-associated acquisition (p 0.001), negative blood cultures (p 0.004), and functional class III-IV HF (p 0.039) were in-hospital mortality predictors. One-fifth of the patients with EI had AAR. Half of them had severe HF which needed emergency surgery and the remaining needed surgery for extension of the infection and / or valvular dysfunction. Both groups remain to have high surgical and in-hospital mortality. Health care-associated acquisition, negative blood cultures and advanced HF were predictors of in-hospital mortality.

Avaliação da influência de alterações cardíacas na ultrassonografia vascular periférica de idosos / Assessment of cardiac influence on peripheral vascular Doppler in the elderly

Resumo Contexto As cardiopatias podem causar alterações no formato das ondas da ultrassonografia vascular (UV) em vasos periféricos. Essas alterações, tipicamente bilaterais e sistêmicas, são pouco conhecidas e estudadas. Objetivo Avaliar as ondas periféricas da UV de pacientes idosos para identificar alterações decorrentes de cardiopatias. Métodos Foram estudados 183 pacientes idosos submetidos a UV periférica no ano de 2014. Resultados Foram avaliados 102 mulheres (55,7%) e 81 homens (44,3%) com idade entre 60 e 91 anos (média de 70,4±7,2 anos). Encontraram-se alterações pela UV em 84 pacientes (45,9%). Foram identificadas 138 alterações de oito dos 13 tipos descritos na literatura: arritmia, onda bisferiens de pico sistólico, baixa velocidade de pico sistólico, pulsatilidade em veias femorais, bradicardia, taquicardia, onda de pulso parvus tardus e onda de pulso alternans. Houve baixa concordância entre a presença e a não presença de alterações na UV e na avaliação cardiológica. Na análise específica das alterações, os exames tiveram uma concordância variável, que foi boa para o achado de taquicardia, moderada para arritmia e baixa para bradicardia. Não houve concordância entre a UV e os exames cardiológicos para as demais alterações. Conclusões É possível identificar determinadas alterações cardíacas em idosos por meio da análise do formato das ondas periféricas da UV. É importante reconhecer e relatar a presença dessas alterações, pela possibilidade de alertar para um diagnóstico ainda não identificado nesses pacientes. Entretanto, mais estudos são necessários para que seja definida a importância das alterações no formato das ondas Doppler periféricas no reconhecimento de cardiopatias.

Abstract Background Heart diseases can cause changes to vascular ultrasonography (VUS) waveforms in peripheral vessels. These changes are typically bilateral and systemic, they have been little studied, and little is known about them. Objective To assess peripheral VUS waveforms in elderly patients in order to identify changes caused by heart diseases. Methods During 2014, a total of 183 elderly patients were examined with peripheral VUS and the results were analyzed. Results The sample comprised 102 women (55.7%) and 81 men (44.3%) with ages ranging from 60 to 91 years (mean of 70.4±7.2 years). Abnormalities were identified in VUS waveforms in 84 patients (45.9%). A total of 138 abnormalities were identified and classified into eight of the 13 categories described in the literature, as follows: arrhythmia, systolic pulsus bisferiens, low peak systolic velocity, pulsatile flow in femoral veins, bradycardia, tachycardia, pulsus tardus et parvus and pulsus alternans. There was low agreement between presence/absence of VUS abnormalities and cardiological assessments. Analysis of specific abnormalities revealed variable levels of agreement between VUS and cardiological assessments, ranging from good for tachycardia, moderate for arrhythmia, to low for bradycardia. There was no agreement between VUS and cardiological examinations for the remaining categories of abnormalities. Conclusions Certain cardiac abnormalities can be identified in elderly patients by analysis of peripheral VUS waveforms. It is important to recognize and report the presence of these abnormalities because there is a possibility that they may serve to signal hitherto unidentified diagnoses in these patients. However, further studies are needed to determine the importance of changes to peripheral Doppler waveforms to recognition of heart diseases.

Paravalvular Regurgitation: Clinical Outcomes in Surgical and Percutaneous Treatments / Regurgitação Paravalvar: Análise de Desfechos Clínicos no Tratamento Cirúrgico e Percutâneo

Abstract Background: Paravalvular regurgitation (paravalvular leak) is a serious and rare complication associated with valve replacement surgery. Studies have shown a 3% to 6% incidence of paravalvular regurgitation with hemodynamic repercussion. Few studies have compared surgical and percutaneous approaches for repair. Objectives: To compare the surgical and percutaneous approaches for paravalvular regurgitation repair regarding clinical outcomes during hospitalization and one year after the procedure. Methods: This is a retrospective, descriptive and observational study that included 35 patients with paravalvular leak, requiring repair, and followed up at the Dante Pazzanese Institute of Cardiology between January 2011 and December 2013. Patients were divided into groups according to the established treatment and followed up for 1 year after the procedure. Results: The group submitted to percutaneous treatment was considered to be at higher risk for complications because of the older age of patients, higher prevalence of diabetes, greater number of previous valve surgeries and lower mean creatinine clearance value. During hospitalization, both groups had a large number of complications (74.3% of cases), with no statistical difference in the analyzed outcomes. After 1 year, the percutaneous group had a greater number of re-interventions (8.7% vs 20%, p = 0.57) and a higher mortality rate (0% vs. 20%, p = 0.08). A high incidence of residual mitral leak was observed after the percutaneous procedure (8.7% vs. 50%, p = 0.08). Conclusion: Surgery is the treatment of choice for paravalvular regurgitation. The percutaneous approach can be an alternative for patients at high surgical risk.

Resumo Fundamento: Regurgitação ou escape paravalvar é uma complicação grave e incomum associada ao implante de prótese valvar. Estudos mostram incidência de 3% a 6% com repercussão hemodinâmica. Existem poucos estudos na literatura que comparam as abordagens cirúrgica e percutânea para sua correção. Objetivos: Comparar as abordagens cirúrgica e percutânea de correção da regurgitação paravalvar quanto a desfechos clínicos durante a internação e após 1 ano do procedimento. Métodos: Este é um estudo retrospectivo, descritivo e observacional, que incluiu 35 pacientes com escape paravalvar acompanhados no Instituto Dante Pazzanese de Cardiologia entre janeiro de 2011 e dezembro de 2013 e que necessitaram de correção. Os pacientes foram divididos de acordo com o tratamento estabelecido e acompanhados por um período 1 ano após o procedimento. Resultados: O grupo submetido ao tratamento percutâneo foi considerado como de maior risco para complicações por apresentar pacientes mais idosos, com maior prevalência de diabetes, maior quantidade de cirurgias valvares prévias e menor valor médio de clearance de creatinina. Durante a evolução intra-hospitalar, observou-se grande número de complicações nos dois grupos (74,3% dos casos), sem diferença estatística nos desfechos analisados. Após 1 ano, o grupo percutâneo teve maior número de reintervenções (8,7% vs. 20%, p = 0,57) e mortalidade maior (0% vs. 20%, p = 0,08). Uma alta incidência de escape residual mitral foi verificada após procedimento percutâneo (8,7% vs. 50%, p = 0,08). Conclusão: A cirurgia é o tratamento de escolha da regurgitação paravalvar. A abordagem percutânea pode ser uma alternativa para os pacientes com risco cirúrgico elevado.

Subaortic Membrane Late after Surgical Correction of Tetralogy of Fallot

We herein report a rare case of subaortic stenosis in association with a previous tetralogy of Fallot (TOF) surgical repair, which was not taken into account as a differential diagnosis. Echocardiography plays a pivotal role in identification of this rare combination. Therefore, echocardiography should be performed periodically during follow-up of patients with surgically corrected TOF. Given the clinical complications that can result from subaortic stenosis (i.e., aortic regurgitation and infective endocarditis), early and aggressive management of this rare combination should be performed.

Aortite sifilítica: diagnóstico e tratamento. Relato de caso / Syphilitic aortitis: diagnosis and treatment. Case report

A aortite é uma das muitas possibilidades de lesão produzida pela sífilis terciária. Dentro das lesões cardiovasculares, o acometimento da aorta é o mais comum. Apresentamos o caso de um paciente de 48 anos, diagnosticado com aortite sifilítica, sendo realizada cirurgia para substituição de raiz da aorta e valva aórtica. A hipótese diagnóstica foi devido ao aspecto do dano arterial in situ. Apesar da raridade da doença atualmente, ainda é uma possibilidade.

Aortitis is one of the many possibilities of lesion caused by tertiary syphilis. Between all the cardiovascular lesions, the aorta's injury is the most common. We report a case of a 48-year-old patient diagnosed with syphilitic aortitis who had undergone surgery for the replacement of the aortic root and aortic valve. The diagnosis hypothesis was pondered because of the in situ aspect of the arterial damage. Although the rarity of the disease, it persists.

Late aortic dilatation and regurgitation after Ross operation

The Ross operation, a procedure of replacement of the diseased aortic valve with an autologous pulmonary valve, has many advantages such as no need for anticoagulation therapy and similar valve function and growth potential as native valves. However secondary aortic disease has emerged as a significant complication and indication for reoperation. We report a 48-year-old woman who had Ross operation in 1997 for a damaged bicuspid aortic valve and severe aortic regurgitation due to subacute bacterial endocarditis complicated by aortic root abscess. In 2009, 12 years later, progressive severe aortic regurgitation with incomplete coaptation and mild dilatation of the aortic root was shown on echocardiography and contrasted CT, while the pulmonary homograft retained normal function. She subsequently underwent aortic valve replacement. Histopathological examination of the explanted neo-aortic valve and neo-arterial wall revealed pannus formation at the nodulus Arantii area of the three valve cusps, ventricularis, and arterialis. The amount of elastic fibres in the neo-aorta media was less than usual for an aorta of this patient's age but was similar to a pulmonary artery. The pathological findings were not different from other studies of specimens removed between 7 to 12 years after Ross operation. However, the pathophysiology and long-term implications of these findings remain debatable. Considering the anatomical and physiological changes induced by the procedure, separate mechanisms for aortic dilatation and regurgitation are worthy of consideration.

Effect of vegetation size on the outcome of infective endocarditis in intravenous drug users

Infective endocarditis is one of the most important complications of injection drug use, which is associated with significant mortality and morbidity. The aim of this study was to evaluate the influence of vegetation size and localization on outcome and in-hospital mortality of infective endocarditis. A total of 34 patients were admitted to a university hospital in a four-year period and analyzed prospectively. Injection drug users were defined as patients who had injected drugs intravenously within the past 3 months before admission. The diagnosis of infective endocarditis was made on the basis of modified Duke's criteria. Totally, 38 episodes of endocarditis in 34 patients were identified. Patients were all male with the mean [ +/- standard deviation] age of 30.4 +/- 7.1 years. The affected valves were as follow: tricuspid valve in 26 cases [74.3%], mitral valve in 4 [11.4%], mitral and tricuspid valve in 4 [11.4%], and mitral and aortic valve in 1 [2.9%]. The vegetation size was 10mm or less in 12 cases [34.29%], and more than 10mm in the remaining 23 cases [65.71%]. Eight patients [21.1%] underwent surgery, however, unfortunately, nine [23.7%] died. Vegetations sized more than 10mm [p<0.033], involvement of the left-sided valves [p<0.012], and presence of more than one vegetation on TTE or TEE [p<0.05] were associated with higher probability of death. In contrast to some earlier studies which concluded that vegetations larger than 20mm are associated with higher mortality, our results revealed that the mortality rate increases even with smaller vegetations. In addition, the number and the location of vegetations also could affect the prognosis. Therefore, lower threshold for surgical interventions should be applied in patients with vegetations larger than 10mm

Natural aortic valve complications of ventricular septal defect: a prospective cohort study.

OBJECTIVE: To study the incidence and onset of aortic valve prolapse (AVP) and aortic regurgitation (AR) in the ventricular septal defect (VSD). STUDY DESIGN: A prospective cohort study POPULATION: The less than one-year-old children with diagnosis of isolated VSD were studied from October 2000 to September 2006 at Queen Sirikit National Institute of Child Health. Clinical follow-up and echocardiographic studies were scheduled every 2-3 months in the first year of age and then every 6 months to evaluate the size, location, flow across VSD, aortic valve morphology and aortic regurgitation. RESULTS: Three hundred and twenty-one cases of VSD were followed up. One was excluded due to associated hypoplastic RV An overall of 2,644 echocardiograms were performed. The percentage of perimembranous, subpulmonic, muscular inlet and multiple types were 70.3%, 19.4%, 5.6%, 3.1% and 1.6%, respectively. Size of the VSD was diagnosed to be small, moderate, and large VSD in 62.5%, 15.9% and 21.6% respectively. At the end of the study, the incidence of AVP in subpulmonic VSD was 87.1% compared to 16.4% in perimembranous VSD, with a relative risk of 5.30 and the incidence of AR in subpulmonic VSD was 37.1% compared to 5.3% in perimembranous VSD, with a relative risk of 6.95. From the survival analysis, the patient with subpulmonic VSD developed AVP at 46%, 77%, 90% and 94% compare to 8%, 13%, 20% and 23% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). The patient with subpulmonic VSD developed AR at 8%, 17%, 35% and 38% compare to 2%, 4%, 5% and 7% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). At the end of the study, ninety-six cases (30%) underwent cardiac operation with the indication of heart failure or the occurrence of AR. Sixty one cases (19.1%), including two cases of subpulmonic type had spontaneous closure of VSD. Seven cases (2.2%) had lost to follow up and five cases (1.6%) died during the follow up period. CONCLUSION: The incidence of AVP and AR are high in subpulmonic VSD being much higher than perimembranous VSD with a relative risk of 5.30 and 6.95 respectively. These complications are significantly from infancy period and are an indication for early cardiac surgery.

Valva aórtica quadrivalvular por ecocardiografia transtorácica / Quadricuspid aortic valve by transthoracic echocardiography

Uma mulher de 55 anos procurou atendimento médico com queixas de palpitações, dor torácica atípica e dispnéia ao esforço, com duração de 12 meses. O eletrocardiograma revelou hipertrofia ventricular esquerda. O ecocardiograma Doppler transtorácico confirmou hipertrofia de câmaras esquerdas e detectou a presença de valva aórtica quadri, com válvulas de mesmo tamanho, e fluxo regurgitante central moderado. No ecocardiograma, a valva quadrivalvular é identificada por sua forma característica em "X" durante a diástole e aspecto retangular durante a sístole. Os sintomas de insuficiência cardíaca provavelmente são causados por insuficiência valvar, em decorrência de coaptação anormal das válvulas. Em vista da possibilidade de aumento da regurgitação, a paciente foi submetida a controle ecocardiográfico periódico. Observou-se melhora dos sintomas com o tratamento.

A 55-year-old woman seeks medical attention with palpitations, atypical chest pain and dyspnea upon exertion, lasting for 12 months. ECG showed left ventricular hypertrophy. Transthoracic Doppler echocardiogram confirmed hypertrophy of the left chambers and showed a quadricuspid aortic valve, with equal-sized cusps, in addition to a moderate regurgitant central flow. Upon echocardiography, the quadricuspid valve is identified by its characteristic "X" form during diastole and rectangular aspect during systole. Heart failure symptoms are presumably caused by valve insufficiency due to abnormal leaflet coaptation. Considering the possibility of progressive regurgitation, follow up on the patient was performed with periodic echocardiographic control. Symptoms responded to treatment.

Bartonella native valve endocarditis: the first brazilian case alive and well

Bartonella is an important cause of blood culture-negative endocarditis in recent studies. Seroprevalence studies in the States of Minas Gerais and Rio de Janeiro have shown Bartonella IgG positivity around 14 percent in healthy adults and 40 percent in HIV seropositive adults, respectively. A case report of a 46-year-old white male with moderate aortic regurgitation (AR) due to rheumatic heart disease (RHD), admitted due to worsening heart failure, is presented. Clinical features were apyrexia, anemia, polyclonal hypergammaglobulinemia, hematuria and splenomegaly. He was submitted to surgery due to worsening AR. Histopathology of the excised valve showed active bacterial endocarditis and underlying RHD. Routine blood cultures were negative. Indirect immunofluorescence (IFI) assays for Coxiella burnetii were non-reactive. Bartonella henselae IgG titer was 1:4096 prior to antibiotics and 1:512 14 months after treatment. History of close contact with a young cat during the months preceding his admission was elicited.

Myocardial dysfunction in rheumatic carditis--does it really exist?

OBJECTIVES: Acute rheumatic fever (ARF) continues to affect millions of children in developing countries. Aim of the present study was to evaluate the role of myocardial dysfunction in the genesis of heart failure in patients with rheumatic carditis. There are limited studies on this subject. METHODS AND RESULTS: In this prospective study, 108 consecutive patients of ARF were evaluated by echocardiography and assay of cardiac troponin I blood levels. The patients were divided into three groups. Group A (n = 30): patients with no evidence of carditis; Group B (n = 45): patients with first attack of carditis; and group C (n = 33): patients with recurrent attacks of carditis. Left ventricular dimensions tended to be larger in Group B and C patients. Left ventricular ejection fraction did not differ between the groups (Group A: 63 +/- 8.1%, Group B: 58 +/- 7.9%, Group C: 61.2 +/- 9%, p = ns). Heart failure was present in 37.7% patients of Group B, and in 60.6% patients of Group C (p = < 0.05). Ejection fraction was normal in majority of heart failure patients (75.7%). It was reduced in 29.4% of patients in Group B and in 20% of Group C patients with heart failure (p = ns). All patients with low ejection fraction had hemodynamically significant regurgitant valvular lesions. Mean cardiac troponin I values, an index of myocardial damage, did not differ between the three groups (Group A: 0.062 +/- 0.027 ng/ml, Group B: 0.068 +/- 0.019 ng/ml, Group C: 0.071 +/- 0.031 ng/ml, p = ns). CONCLUSION: The present study did not demonstrate any echocardiographic abnormalities or cardiac troponin I elevation suggesting significant myocardial involvement during acute rheumatic fever. This lends credence to the view that myocardial involvement does not play any significant role in the genesis of heart failure in patients with rheumatic carditis.