Editorial of the Special Issue: Cellular Mechanisms of Cardiovascular Disease.

Cardiovascular diseases (CVD) remain the major cause of mortality and disability worldwide, having contributed to 19 [...].

Interventions for Caregivers of Heart Disease Patients in Rehabilitation: Scoping Review.

Map the interventions/components directed to the caregivers of heart disease patients in cardiac rehabilitation programs that promote their role and health. METHODS: The Joanna Briggs Institute method was used to guide this scoping review. Two independent reviewers assessed articles for relevance and extracted and synthesized data. Inclusion criteria comprised articles published in English, Spanish, and Portuguese since 1950. The following databases were searched: CINAHL Complete (Via EBSCO), Medline (via PubMed), Scopus, PEDro, and Repositórios Científicos de Acesso Aberto de Portugal (RCAAP). RESULTS: From 351 articles retrieved, 10 were included in the review. The interventions identified directed to the caregiver were: educational interventions and lifestyle changes; physical exercise; psychological interventions/stress management; and a category "Other" with training interventions in basic life support, elaboration of guidelines/recommendations, and training for the role of caregiver. CONCLUSIONS: It was found that most of the related cardiac rehabilitation interventions are aimed at the dyad heart failure patient and their caregivers/family. Including specific interventions targeting caregivers improves the caregiver's health and empowers them. Patient care planning should include interventions specifically aimed at them that result in health gains for caregivers and patients, striving to improve the quality of care. This study was not registered.

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.

El proceso de transición y capacitación de la persona trasplantada al corazón y familia: ensayo teórico / The process of transition and training of the person transplanted to the heart and family: theoretical essay

La persona trasplantada de corazón y la familia experimentan diferentes tipos de transición, y un acompañante facilitador de su formación para una transición saludable es fundamental.La Enfermería de Rehabilitación (ER), debido a sus habilidades, puede ayudar a las personas a recuperar la estabilidad y el bienestar, sin embargo, existe poca difusión de su fundamento en lasteorías de enfermería. Objetivo: Analizar el aporte de la Teoría de las Transiciones en la práctica dela enfermería rehabilitadora orientada al empoderamiento de la persona trasplantada al corazón ya la familia. Metodología: Estudio crítico-reflexivo guiado por la Teoría de las Transiciones de AfafMeleis y por la legislación reguladora para la práctica del enfermero rehabilitador. Resultado: Sepercibe que la teoría de las Transiciones sustenta la intervención de Enfermería de Rehabilitaciónen las áreas de conocimiento y aprendizaje de habilidades de la persona trasplantada cardíaca y sufamilia, conduciendo a su empoderamiento, toma de decisiones y acción, haciendo que la personase sienta empoderada para superar los retos diarios derivados de su proceso de transición. Conclusión: Es fundamental demostrar la conceptualización en la práctica de enfermería, siendo la transición un foco significativo para la Enfermería de Rehabilitación.(AU)

Post-infarction ventricular septal defect surgery in Portugal.

INTRODUCTION AND OBJECTIVE: We performed a collective analysis of a dedicated national post-myocardial infarction ventricular septal defect (VSD) registry to further elucidate controversial areas of this clinical entity's surgical treatment. METHODS: A descriptive statistical analysis was carried out and cumulative survival using the Kaplan-Meier method and multivariate logistic regression of risk factors for 30-day mortality are presented. RESULTS: Median survival of the cohort (n=76) was 72 months (95% CI 4-144 months). Better cumulative survival was observed in patients who underwent VSD closure more than 10 days after myocardial infarction (log-rank p=0.036). Concomitant coronary artery bypass grafting (CABG), different closure techniques, location of the VSD, extracorporeal membrane oxygenation as bridge to closure, or intra-aortic balloon pump as bridge to closure showed no statistically significant differences at Kaplan-Meier analysis. Multivariate binary logistic regression for independent factors affecting status at 30 days showed a statistically significant effect of age (OR 1.08; 95% CI 1.01-1.15) and concomitant CABG (OR 0.23; 95% CI 0.06-0.90). CONCLUSIONS: Our results are comparable with previous reports regarding mortality, risk factors and concomitant procedures. Timing of surgery remains a controversial issue. Later closure seems to be advantageous, however, there is significant observational bias.

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

Innominate artery post-traumatic pseudoaneurysm presenting with Horner's syndrome.

Isolated innominate artery trauma after blunt thoracic contusion is rare and occurs mostly at its origin. We report a case of a post-traumatic distal innominate artery pseudoaneurysm presenting with Horner's syndrome treated by conventional surgical approach.

Current status of the treatment of degenerative mitral valve regurgitation.

Degenerative mitral valve disease (myxomatous degeneration or fibroelastic deficiency) is the most common indication for surgical referral to treat mitral regurgitation. Mitral valve repair is the procedure of choice whenever feasible and when the results are expected to be durable. Posterior leaflet prolapse is the commonest lesion, found in up to two-thirds of patients. It is the easiest to repair, particularly when limited to one segment. In these cases, rates of repairability and procedural success approach 100%, and there is now ample evidence that the immediate and long-term results are better than those of valve replacement. Notably, minimally invasive valvular procedures, surgical or interventional, have attracted increasing interest in the last decade. When performed by experienced groups, mitral valve repair is unrivaled irrespective of the severity of lesions, from simple to complex, which leaflets are involved, and the type of degenerative involvement (myxomatous or fibroelastic). Its results should be viewed as the benchmark for other present and future technologies. By contrast, percutaneous mitral valve repair is still in its infancy and its results so far fall short of those of surgical repair. Nevertheless, continued investment in transcatheter procedures is of great importance to enable development and improved accessibility, particularly for patients who are considered unsuitable for surgery. In this review, we analyze the current status of management of degenerative mitral valve disease, discussing mitral valve anatomy and pathology, indications for intervention, and current surgical and transcatheter mitral valve procedures and results.

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.

EHD1 Modulates Cx43 Gap Junction Remodeling Associated With Cardiac Diseases.

RATIONALE: Efficient communication between heart cells is vital to ensure the anisotropic propagation of electrical impulses, a function mainly accomplished by gap junctions (GJ) composed of Cx43 (connexin 43). Although the molecular mechanisms remain unclear, altered distribution and function of gap junctions have been associated with acute myocardial infarction and heart failure. OBJECTIVE: A recent proteomic study from our laboratory identified EHD1 (Eps15 [endocytic adaptor epidermal growth factor receptor substrate 15] homology domain-containing protein 1) as a novel interactor of Cx43 in the heart. METHODS AND RESULTS: In the present work, we demonstrate that knockdown of EHD1 impaired the internalization of Cx43, preserving gap junction-intercellular coupling in cardiomyocytes. Interaction of Cx43 with EHD1 was mediated by Eps15 and promoted by phosphorylation and ubiquitination of Cx43. Overexpression of wild-type EHD1 accelerated internalization of Cx43 and exacerbated ischemia-induced lateralization of Cx43 in isolated adult cardiomyocytes. In addition, we show that EHDs associate with Cx43 in human and murine failing hearts. CONCLUSIONS: Overall, we identified EHDs as novel regulators of endocytic trafficking of Cx43, participating in the pathological remodeling of gap junctions, paving the way to innovative therapeutic strategies aiming at preserving intercellular communication in the heart.

[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.

Long-term results after concomitant mitral and aortic valve surgery: repair or replacement?

OBJECTIVES: The reported superiority of mitral valve (MV) repair for isolated MV regurgitation has not been confirmed in mitroaortic valve surgery. Our goals were to evaluate the feasibility of repair in patients undergoing mitral and aortic valve surgery and to identify factors predisposing to MV replacement, to compare long-term outcomes (survival and MV reoperation) of repair and replacement and to perform a subgroup analysis in patients with rheumatic MV disease. METHODS: From January 1992 through December 2016, 1122 consecutive patients were submitted to concomitant aortic and MV surgery in 2 different centres (Coimbra and Santiago). Of these, 837 patients underwent MV repair (74.6%) and 285 patients had MV replacement (25.4%). Rheumatic aetiology was predominant (666 patients; 59.4%). Cumulative follow-up was 9522.6 patient-years (25th-75th percentile 2.6-13.2 years) and was complete for 95.6% of patients. Propensity score matching (1:1) was performed in 232 patients for comparing each treatment option (MV repair and MV replacement). RESULTS: Previous MV intervention, rheumatic aetiology, chronic obstructive pulmonary disease, higher degrees of tricuspid and mitral regurgitation and pulmonary hypertension were independently correlated with MV replacement. The 30-day mortality rate was higher in patients with MV replacement (4.2% vs 1.8%, P = 0.021) and was confirmed in the propensity score matching (4.7% vs 1.7%, P = 0.06). Late survival was lower in the MV replacement group (53.3 ± 4.5% vs 61.7 ± 2.0% at 12 years; P = 0.026) and was confirmed in the propensity score matching (54.6 ± 4.9% vs 63.2 ± 3.8%, P = 0.062) and rheumatic subgroup (57.9 ± 4.8% vs 68.0 ± 2.5%, P = 0.018). Freedom from MV reoperation at 12 years was higher in the MV repair group (94.7 ± 1.1% vs 89.0 ± 3.1%, P = 0.004) but similar in patients with rheumatic MV disease. CONCLUSIONS: MV repair can be performed in most patients undergoing aortic valve replacement. It should be the procedure of choice whenever feasible, because it is associated with lower early and late mortality rates and with freedom from reoperation in non-rheumatic patients.

Mitral valve repair for degenerative mitral valve disease: surgical approach, patient selection and long-term outcomes.

Mitral valve repair (MVRepair) has become the procedure of choice to correct severe degenerative mitral regurgitation (MR), due to its documented superiority to valve replacement regarding long-term survival, freedom from valve-related adverse events and preservation of left ventricular (LV) function. The refinement of MVRepair techniques has rendered almost all valves (more than 95%) amenable to repair with a 15-year freedom from reoperation of 90%. The concept of 'centres of excellence for MVRepair' has emerged, encouraging referring doctors to select the most experienced institutions or individual surgeons to deal with the most complex cases, based on repair volume, appropriate peri-procedural imaging and data regarding expected outcomes (repair, mortality and durability of repair). Based on the good results, operating on asymptomatic patients with severe MR is now widely accepted, prophylactically avoiding the dire consequences of chronic MR, such as LV function deterioration/enlargement, and development of atrial fibrillation and pulmonary hypertension. In reference centres, where the repair rate is over 95% for all types of disease with <1% mortality, it has become standard practice in nearly 50%-60% of all patients submitted to MVRepair. Finally, recent advances in the surgical treatment with the purpose of reducing invasiveness and surgical trauma, through partial sternotomy or mini-thoracotomy (video-assisted with or without robotics), are now being increasingly performed in 20%-30% of centres, claiming comparable results to conventional surgery. In addition, transcatheter technology, particularly the MitraClip, is evolving and treading its way in the treatment of high-risk patients with severe MR, but the results are still short of ideal.