Incidental Diagnosis of Rheumatic Myocarditis during Cardiac Surgery-Impact on Late Prognosis.

Rheumatic fever (RF) and rheumatic heart disease (RHD) are still highly prevalent, particularly in low- and middle-income countries. RHD is a neglected and underdiagnosed disease for which no specific laboratory diagnostic test is completely reliable. This is a retrospective observational study, which included 118 patients with RHD who underwent cardiac surgery from 1985 to 2018. The aim of this investigation was to evaluate the clinical, epidemiological, echocardiographic and pathological characteristics in two cohorts of RHD patients: one cohort with Aschoff bodies present in their pathological results and the other without such histopathological characteristics. No conventional clinical and laboratory tests for RHD myocarditis were able to identify active carditis during the preoperative phase of valve repair or replacement. Patients who had Aschoff bodies in their pathological results were younger (median age of 13 years (11-24 years) vs. 27 years (17-37 years), p = 0.001) and had higher rate of late mortality (22.9% vs. 5.4%, p = 0.043). In conclusion, the presence of Aschoff bodies in pathological findings may predict increased long-term mortality, emphasizing the importance of comprehensive pathology analysis for suspected myocarditis during heart surgery.

Desfechos clínicos intra-hospitalar e em até 1 ano de seguimento da Takotsubo no Brasil: Registro Brasileiro de Takotsubo (Takosubo Br-R) / In-hospital clinical outcomes and up to 1 year of follow-up of Takotsubo in Brazil: Brazilian Takotsubo Registry (Takosubo Br-R)

FUNDAMENTO: A evolução clinica intra-hospitalar e pós alta da Cardiomiopatia por Takotsubo (CT) assim como o perfil clinico dos pacientes de maior risco prognóstico não estão bem caracterizados nos grandes registros assim como no Brasil. OBJETIVO: Determinar a taxa de mortalidade intrahospitalar (MIH), as características dos pacientes que apresentaram relação com uma maior mortalidade, e a taxa de reincidência de CT(RCT)e mortalidade ao fim de 1 ano pós alta no Brasil. Delineamento e MÉTODOS: Este é um estudo retrospectivo, observacional,multicêntrico envolvendo 25 centros dispersos geograficamente pelo Brasil. Os critérios de inclusão foram de acordo com International Takotsubo Diagnostic Criteria (InterTAK Diagnostic Criteria). A características clinicas, biomarcadores, ECG, ecocardiograma (ECO), ressonância magnética cardíaca (RMC), foram avaliados durante a fase IH. Também foram avaliados a taxa de MIH, e a taxa de RCT, readmissão por DCV e mortalidade em 30 dias, 6 meses e 1 ano pós-alta. RESULTADOS: 448 pacientes foram admitidos CT, onde foi observado uma taxa de MIH de 7,5%. Na análise univariada do perfil clinico os pacientes do sexo masculino (p=0,009), com idade menos avançada (67±14 vs 73±11; P=0,0179), com choque cardiogênico (p<0,0001), sepsis (P<0,0001), fibrilação atrial (p=0,01) apresentaram significativamente maior MIH e dor toráxica (p<0,0001) com menor MIH. Na análise do ecocardiograma, ECG, RMC e peptídeos natriuréticos e Troponina não foram observados correlações significativas com a MIH. Quanto a terapêutica utilizada, os pacientes que usaram betabloqueador (P<0,0001), IECA/BRA (p<0,001) e AAS (p=0,04), demonstraram uma menor MIH. Os pacientes que utilizaram Dobutamina (p<0,0001), NE (P<0,0001) e e Vasopressina (P < 0,0001) demonstraram maior MIH. Na regressão logística de todas a variáveis significativas, a presença de sepsis (OR:6,8;IC-95%:2,3- 19,4;p=0,0005), uso de vasopressina(OR:7,5;IC95%:1,8-31;p=0,005) definiram maior MIH, enquanto que Betabloqueador(OR:0,23;IC-95%:0,1- 0,7;p=0,009) edortoráxica (OR:0,28;IC-95%:0,1-0,8;p=0,02) demonstraram uma menor associação com MIH. No seguimento pós-alta observamos uma taxa acumulativa de RCT, readmissão por DCV e mortalidade em 30 dias (0,2%;0,4%;0,2%);6 meses (0,6%; 1,2%;0,8%) e 12meses (0,8%;2,4%;0,8%) respectivamente. CONCLUSÃO: O Registro Brasileiro de Takotsubo demonstrou características clinicas e de exames complementares semelhantes aos dos registros internacionais com predomino de dor toráxica com alteração do segmento ST, assim como nos desfechos clínicos intra-hospitalares. A Takotsubo apresenta um prognostico benigno nos 12 meses pós alta, com uma baixa taxa de recorrência, readmissão hospitalar e mortalidade. Palavras-chave: Takotsubo; cardiomiopatia neuroadrenergica.

Cardiac Rehabilitation in a Transplanted Person with Emery-Dreifuss Muscular Dystrophy. / Reabilitação Cardíaca na Pessoa Transplantada com Distrofia Muscular de Emery-Dreifuss.

Emery-Dreifuss muscular dystrophy is a rare hereditary neuromuscular disease. Its manifestations begin primarily in childhood. The most frequent manifestations are progressive muscle weakness, atrophy that usually begins in the scapula-vertebral region, extending later to the pelvic girdle, and spinal stiffness. Patients can also manifest cardiac involvement as palpitations, syncope, exercise intolerance, congestive heart failure, and variable heart rhythm disturbances. 1 - 3 The presence and severity of these manifestations can vary according to the individual and the disease's subtypes. 2 Cardiac involvement is the most worrisome feature of this disease, and there are some reports of the need for heart transplantation in this dystrophy. 4.

A distrofia muscular de Emery-Dreifuss é uma doença neuromuscular hereditária rara. Suas manifestações começam principalmente na infância. As manifestações mais frequentes são fraqueza muscular progressiva, atrofia que geralmente se inicia na região escápulo-vertebral, estendendo-se posteriormente para a cintura pélvica e rigidez da coluna vertebral. Os pacientes também podem manifestar envolvimento cardíaco como palpitações, síncope, intolerância ao exercício, insuficiência cardíaca congestiva e distúrbios variáveis do ritmo cardíaco. 1 - 3 A presença e a gravidade dessas manifestações podem variar de acordo com o indivíduo e os subtipos da doença. 2 O envolvimento cardíaco é a característica mais preocupante desta doença, havendo alguns relatos da necessidade de transplante cardíaco nesta distrofia. 4.

Reabilitação Cardíaca na Pessoa Transplantada com Distrofia Muscular de Emery-Dreifuss / Cardiac Rehabilitation in a Transplanted Person with Emery-Dreifuss Muscular Dystrophy

Resumo A distrofia muscular de Emery-Dreifuss é uma doença neuromuscular hereditária rara. Suas manifestações começam principalmente na infância. As manifestações mais frequentes são fraqueza muscular progressiva, atrofia que geralmente se inicia na região escápulo-vertebral, estendendo-se posteriormente para a cintura pélvica e rigidez da coluna vertebral. Os pacientes também podem manifestar envolvimento cardíaco como palpitações, síncope, intolerância ao exercício, insuficiência cardíaca congestiva e distúrbios variáveis do ritmo cardíaco. 1 - 3 A presença e a gravidade dessas manifestações podem variar de acordo com o indivíduo e os subtipos da doença. 2 O envolvimento cardíaco é a característica mais preocupante desta doença, havendo alguns relatos da necessidade de transplante cardíaco nesta distrofia. 4

Abstract Emery-Dreifuss muscular dystrophy is a rare hereditary neuromuscular disease. Its manifestations begin primarily in childhood. The most frequent manifestations are progressive muscle weakness, atrophy that usually begins in the scapula-vertebral region, extending later to the pelvic girdle, and spinal stiffness. Patients can also manifest cardiac involvement as palpitations, syncope, exercise intolerance, congestive heart failure, and variable heart rhythm disturbances.1 - 3 The presence and severity of these manifestations can vary according to the individual and the disease's subtypes. 2 Cardiac involvement is the most worrisome feature of this disease, and there are some reports of the need for heart transplantation in this dystrophy. 4

Photobiomodulation exerts anti-inflammatory effects on the vascular and cellular phases of experimental inflammatory models.

Photobiomodulation therapy (PBMT) is a non-thermal therapeutic procedure widely used in clinical practice. It is considered an effective modality of treatment for the control of various inflammatory conditions with fewer adverse effects as compared to conventional therapy. However, despite the clinical effects, the mechanisms of action and dosimetric parameters of PBMT are not fully understood. This study was performed to describe the effects of two different doses of PBMT on experimental models of inflammation. Male Swiss mice were administered with 0.9% of saline or phlogistic agents (carrageenan, dextran, serotonin, histamine, or bradykinin) by intra-plantar injection and were treated with PBMT at a dose of 1 or 5 J/cm2; right after, the variation of the paw volume was made, and histopathological analysis and myeloperoxidase assay of the carrageenan-induced edematous paw tissues were performed. The action of PBMT on carrageenan-induced vascular permeability was further evaluated. Our results showed that PBMT (1 J/cm2) led to an improvement in paw edema induced by the phlogistic agents and further reduced the histological scores. Inhibition of neutrophil migration was observed following the administration of 1 and 5 J/cm2 of PBMT. However, only 1 J/cm2 of PBMT showed beneficial effects on carrageenan-induced edema. Laser at a dose of 1 J/cm2 showed cellular and vascular effects since it was able to reverse all the inflammatory parameters, and laser at a dose of 5 J/cm2 probably has only cellular effects in the presence of acute inflammation.

3D Echocardiography for Rheumatic Heart Disease Analysis: Ready for Prime Time.

Rheumatic heart disease (RHD) remains to be a very important health issue worldwide, mainly in underdeveloped countries. It continues to be a leading cause of morbidity and mortality throughout developing countries. RHD is a delayed non-suppurative immunologically mediated inflammatory response to the throat infection caused by a hemolytic streptococcus from the A group (Streptococcus pyogenes). RHD keeps position 1 as the most common cardiovascular disease in young people aged <25 years considering all the continents. The disease can lead to valvular cardiac lesions as well as to carditis. Rheumatic fever valvular injuries lead most commonly to the fusion and thickening of the edges of the cusps and to the fusion, thickening, and shortening of the chordae and ultimately to calcification of the valves. Valvular commissures can also be deeply compromised, leading to severe stenosis. Atrial and ventricular remodeling is also common following rheumatic infection. Mixed valvular lesions are more common than isolated valvular disorders. Echocardiography is the most relevant imaging technique not only to provide diagnostic information but also to enable prognostic data. Further, it presents a very important role for the correction of complications after surgical repair of rheumatic heart valvulopathies. Three-dimensional (3D) echocardiography provides additional anatomical and morphofunctional information of utmost importance for patients presenting rheumatic valvopathies. Accordingly, three-dimensional echocardiography is ready for routine use in patients with RHD presenting with valvular abnormalities.

Recurrent traumatic hip dislocation in an 8-year-old boy.

Pediatric traumatic hip dislocations (PTHD) are rare and represent around 5% of all pediatric dislocations. Associated bony or soft tissue injury can occur as often as 17%-25% of the time. We report a case of an 8-year-old boy presenting a posterior hip dislocation after a low-energy trauma, which was initially managed with closed reduction and bed rest for 1 week. Two days after hospital discharge, he suffered a recurrent posterior hip dislocation. He was now managed with 4 weeks of bed rest and lower limb skin traction followed by 1 week of no weight-bearing on crutches. With 6 months of follow-up, he is asymptomatic, walking autonomously, with complete and painless range of motion of the affected hip and no major radiographic changes. Pediatric traumatic hip dislocation is a rare and challenging injury that should be managed promptly. Currently, there is no protocol concerning treatment in the literature and its largely dependent of patient and parents' cooperation.

Quadricuspid aortic valve with a hidden left ostium: Case report and literature review.

Quadricuspid aortic valve (QAV) is a rare congenital condition that frequently progresses to aortic regurgitation with clinical impact in adulthood. Surgical treatment is required in the fifth to sixth decade of life in about one fifth of patients. We describe the case of a 64-year-old woman with regular cardiological follow-up for severe aortic valve regurgitation who had suffered recent clinical and echocardiographic deterioration. Conventional open surgery was indicated. During the procedure, a QAV with leaflet retraction and central orifice was observed. The aortic valve was successfully replaced.

Tibialis Posterior Tendon Dislocation: A Case Report.

CASE: A 37-year-old man presented after a sudden snap followed by pain in the medial side of the ankle while playing football. Physical examination showed edema and pain posteriorly to the medial malleolus, and a longitudinal structure was palpable anteriorly. Ultrasound confirmed a dislocated tibialis posterior tendon (TPT). The patient underwent surgery, and the flexor retinaculum was repaired. Six months after the surgery, he was asymptomatic and had resumed his usual physical activity, with an AOFAS score of 100. CONCLUSION: TPT dislocation is rare and orthopaedic surgeons should be aware of this condition in patients with a sudden traumatic medial ankle pain.

Sublobar resection in the treatment of elderly patients with early-stage non-small cell lung cancer.

Surgical resection is the primary treatment option for early-stage non-small cell lung cancer, lobectomy being considered the standard of care. In elderly patients, physiological characteristics can limit the suitability for surgery and the extent of resection. Sublobar resection (SLR) can be offered as an alternative. The aim of this real-world analysis was to compare lobectomy and SLR in terms of recurrence and survival rates in patients over 70 years of age.

Sublobar resection in the treatment of elderly patients with early-stage non-small cell lung cancer / Ressecção sublobar no tratamento de pacientes idosos com câncer de pulmão não pequenas células em estágio inicial

ABSTRACT Surgical resection is the primary treatment option for early-stage non-small cell lung cancer, lobectomy being considered the standard of care. In elderly patients, physiological characteristics can limit the suitability for surgery and the extent of resection. Sublobar resection (SLR) can be offered as an alternative. The aim of this real-world analysis was to compare lobectomy and SLR in terms of recurrence and survival rates in patients over 70 years of age.

RESUMO A ressecção cirúrgica é a principal opção de tratamento para o câncer de pulmão não pequenas células em estágio inicial, sendo a lobectomia considerada o tratamento padrão. Em pacientes idosos, as características fisiológicas podem limitar a adequabilidade da cirurgia e a extensão da ressecção. A ressecção sublobar (RSL) pode ser oferecida como alternativa. O objetivo deste estudo de mundo real foi comparar a lobectomia e a RSL em termos de taxas de recidiva e de sobrevida em pacientes acima de 70 anos de idade.

Hereditary Neuropathy with Liability to Pressure Palsies: A Rare Condition That Presents with Common Symptoms: A Case Report.

CASE: A 13-year-old girl presented with paresthesia of the fourth and fifth fingers and the dorsal ulnar surface of the left hand that had started 3 months prior. Physical examination showed loss of sensation at the ulnar side of the fourth and fifth fingers and a positive Froment sign. Electromyography showed a severe motor conduction block in the ulnar nerve at the elbow. Eighteen months later, the patient had similar symptoms in the right hand. The diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP) was considered and confirmed with genetic testing. CONCLUSION: HNPP is a rare disease that should be considered not only in patients with multiple compressive neuropathies, but also in patients with any unexpected or unexplained neuropathy, even if it is isolated.

[Single versus multiple bypass grafts to each diseased coronary territory: impact on survival and major adverse cardiovascular and cerebrovascular events].

BACKGROUND: Complete revascularization is the gold standard of coronary artery bypass grafting (CABG). However, the rationale for revascularization of all diseased vessels is questionable. We aimed at evaluating the impact of multiple versus single grafts in each diseased coronary territory in the long-term survival and incidence of major adverse cardiac and cerebrovascular events (MACCE). METHODS: From January/00 to November/15, 5.694 consecutive patients were submitted to isolated CABG, of whom 4.243 (74.5%) had complete anatomical revascularization and constituted the study population. Patients were divided into two groups: multiple grafts to each major territory (RCA, LAD, Cx, n=755) a single graft to each territory (n=3.488). Mean follow-up time was 8.5±4.4 years and complete for 96.4% of patients. RESULTS: No differences were found concerning major immediate postoperative complications (cardiogenic shock, acute myocardial infarction or stroke) and thirty-day mortality was similar (0.7%; p=0.871). Long-term survival was 64.4±1.3% vs. 67.7±2.9%, p=0.232. Older age (HR:1.07; 1.06-1.08, p<0.001), diabetes mellitus (HR:1.44; 1.24-1-66, p<0.001), peripheral vascular disease (HR: 1.52; 1.29-1.81, p<0.001), chronic obstructive pulmonary disease (HR:1.38; 1.01-1.89, p=0.042), moderate/ severe cardiac dysfunction (HR:1.95; 1.60-2.38, p<0.001) and moderate/severe renal impairment (HR:1.65; 1.40-1.94, p<0.001) were independent predictors for late mortality. Freedom from MACCE was higher in multiple graft group (79.4±2.0% vs. 90.7±2.7%; p=0.026, respectively) at 4 years. CONCLUSION: Isolated CABG can be performed safely and with very low mortality. The number of bypass grafts did not adversely affect the perioperative results and long-term survival. However, implantation of multiple grafts was associated with lower incidence of major adverse events.

Introdução: A revascularização completa representa o gold standard para a cirurgia de revascularização miocárdica. No entanto, a pontagem de todas as artérias com doença significativa no mesmo território coronário é controversa. Deste modo, objetivamos avaliar o impacto, na sobrevivência a longo e na incidência de eventos cardio e cerebrovascular major (MACCE), da colocação de um enxerto único versus múltiplos enxertos coronários no mesmo território. Métodos: de Janeiro/00 a Novembro15, 5.694 doentes foram consecutivamente submetidos a CABG isolada, dos quais 4.243 (74.5%) tiveram revascularização anatómica completa, constituindo a população em estudo. Os doentes foram dividos em dois grupos: os que receberam enxertos múltiplos para cada território (CD, DA, CX, n=755) e os que receberam um enxerto único (n=3.488). O tempo médio de seguimento foi de 8.5±4.4 anos e completo em 96.4% dos doentes. Resultados: não foram observadas diferenças no que respeita às complicações major pós-operatórias (choque cardiogénico, enfarte agudo do miocárdio ou acidente vascular cerebral) bem como na mortalidade aos 30 dias (0.7%; p=0.871). A sobrevivência a longo prazo foi 64.4±1.3% vs. 67.7±2.9%, p=0.232. A idade avançada (HR:1.07; 1.06-1.08, p<0.001), diabetes (HR:1.44; 1.24-1-66, p<0.001), doença vascular periférica (HR:1.52; 1.29-1.81, p< 0.001), doença pulmonar crónica obstrutiva (HR:1.38; 1.01-1.89, p=0.042), disfunção cardíaca moderada/severa (HR:1.95; 1.60-2.38, p< 0.001) e disfunção renal moderada/severa (HR:1.65; 1.40-1.94, p< 0.001) foram preditores de mortalidade a longo prazo. A taxa livre de MACCE foi superior no grupo com múltiplos enxertos aos 4 anos (79.4±2.0% vs. 90.7±2.7%; p=0.026, respetivamente). Conclusão: a CABG é realizada com segurança e com baixa mortalidade. O número de enxertos não afetou os resultados perioperatorios e a sobrevivência a longo prazo. No entanto, a confecção de múltiplos enxertos foi associada a menor incidência de MACCE.

Is population-based screening for rheumatic heart disease precluded by the Cairo accord? Echocardiography...and beyond.

In the 2017, "Cairo Accord on Rheumatic Heart Disease-From Molecules to The Global Community" experts from endemic areas enumerated an approach to reduce the population burden of rheumatic heart disease. The 10 key recommendations include immediate logistical objectives as well as domains for further study. Echocardiographic population screening programs were relegated to research alone. Given the large body of supporting data, relegating echo screening to an investigational modality is an opportunity lost.

Heart transplantation: Early Results of Two Different Regimes of Immnunosuppression.

INTRODUCTION: The management of induction and maintenance immunosuppression therapy after heart transplantation (HT) remains a controversial issue. The dosage and the timing has been a changing target. We aimed at evaluate the incidence of acute cellular rejection (ACR) [≥1R grade], major infection and survival in first year after HT in patients receiving two different induction immunosuppression regimes and with a reduction in intensity of triple maintenance immunosuppression dose. METHODS: From November-2003 to June-2016, 317 patients were submitted to HT. After excluding those with pediatric age (n=8), those with previous renal or hepatic transplantation (n=2), those submitted to retransplantation (n=2), patients with early death without endomiocardial biopsy (n=10) and those in a transition maintenance regime (n=26), the study population resulted in 269 patients. These patients were divided in two groups: patients receiving the previous regime of two doses of basiliximab (group A, n=211) and those receiving a single dose of basiliximab (group B, n=58). All the patients were treated with a maintenance standard triple immunosuppressive regimen of corticosteroids, an inhibitor of calcineurin and mycophenolate mofetil but more immunosuppressive load in group A. RESULTS: Mean age of the recipients (group A vs. group B) was 54.6±10.6vs.55.0±9.8 years (p=0.808); 77.3%vs.75.9% were male (p=0.861); 28.4%vs.28.1% were diabetic (p=0.957); and ischemic etiology was present in 39.8%vs 41.0% of the patients (p=0.798), respectively. No differences were found, at first year, between the two groups concerning global ACR incidence (55.0%vs.56.9%, p=0.882, respectively) but major ACR (≥2R grade) was slightly superior in group B (16.6%vs.27.6%, p=0.080, respectively). Time-free from major ACR at 3rd, 6th and 12th months was, respectively 91.0±2.0%vs.84.5%±4.8%; 86.7±2.3%vs.74.1±5.7%; and 83.4±2.6%vs.72.4±5.9% (p=0.048). Time-free from major infection at 3rd, 6th and 12th months was, respectively 89.6±2.1%vs.82.8±5.0%; 87.7±2.3%vs.79.3±5.3%; and 84.4±2.5%vs.79.3±5.3% (p=0.253). No differences were found concerning survival at 3rd, 6th and 12th months (94.3±1.6%vs.94.8±2.9%; 92.4±1.8%vs.93.1±3.3%; and 90.0±2.1%vs.91.4±3.7%, (p=0.771) respectively). CONCLUSION: With this study, we verified that lowering doses of induction and maintenance therapy was responsible for increase cases of major ACR at first year of heart transplant. However, no differences were found concerning the incidence of major infection and early survival. Hence, effective immunosuppression induction regimen can apparently be done safely with a single dose regime without compromising survival at first year after HT.

Coronary Allograft Vasculopathy after Cardiac Transplantation: Prevalence, Prognostic and Risk Factors.

INTRODUCTION: Coronary allograft vasculopathy (CAV) is still a serious long-term complication after cardiac transplantation. PURPOSE: To evaluate the prevalence of CAV in a single institution, its impact on survival and to explore associated risk factors. METHODS: From November-2003 through June-2016, 316 patients were submitted to cardiac transplantation. After excluding those with paediatric age (n=8), those with previous renal or hepatic transplantation (n=2) and those who didn't survive the first year after cardiac transplantation (n=40), the study population resulted in 266 patients. Forty two patients (15.8%) with CAV, diagnosed by a new >50% coronary artery stenosis in any vessel during follow-up, were compared with a non-CAV group. RESULTS: Both groups share de same median age (54+10years). Recipient male sex predominated in the CAV group (93% vs. 74%), as did ischemic etiology (52% vs. 37%). Although not reaching statistical significance, CAV patients also had more dyslipidemia (60% vs. 50%), history of smoking (52% vs. 44%) and peripheral vascular disease (45% vs. 29%). The incidence of celular acute rejection 1R is more frequent in CAV group (69% vs. 60%) such as 2R or 3R (29% vs. 27%). Prolonged use of inotropic support and mechanical assistance after cardiac transplantation were comparable between both groups. The survival of this patients, who were submitted to cardiac transplantation and had lived at least 1 year, between CAV and non-CAV group was comparable at 5-year (91% vs. 85%), but tended to be lower for CAV patients in 10-year interval (52% vs. 73%). CONCLUSION: This data confirms CAV as a common long-term complication following cardiac transplantation. Although short to mid-term survival seems not to be affected by CAV, long-term survival appears lower, hence a longer follow-up is needed.