Mitral re-valve-in-valve as a new perspective for high-risk patients with prosthetic valve dysfunction: case reports.

Background: Mitral valve diseases are a common medical condition, and surgery is the most used therapeutic approach. The need for less invasive interventions led to the development of transcatheter valve implantation in high-risk patients. However, the treatment to the dysfunctions of these prosthetic valves is still uncertain, and the yield and safety of repeated transcatheter valve implantations remain unclear. Cases summary: A 69-year-old Caucasian woman with three previous mitral valve procedures performed due to rheumatic valve disease (currently with a biological prosthetic mitral valve) and a 76-year-old Latin woman with previous liver transplantation (due to metabolic-associated fatty liver disease) and biological mitral prosthesis due to mitral valve prolapse with severe regurgitation underwent mitral valve-in-valve (ViV) transcatheter implantation at the time of dysfunction of their surgical prostheses. Later, these patients developed prosthetic valve dysfunction and clinical worsening, requiring another invasive procedure. Due to maintained high-risk status and unfavourable clinical conditions for surgery, re-valve-in-valve (re-ViV) was performed. Discussion: Valve-in-valve transcatheter mitral valve implantation was approved in 2017, and, since then, it has been used in several countries, mainly in high-risk patients. Nevertheless, these prosthetic valves may complicate with stenosis or regurgitation, demanding reinterventions. Although there are favourable data for mitral ViV, re-ViV still lacks robust data to support its performance, with only case reports in the literature so far. It is possible that in high-risk patients, there is a greater benefit from re-ViV when compared with the surgical strategy. However, this hypothesis must be studied in future controlled trials.

Total surgical correction of Lutembacher syndrome associated with partial anomalous connection of the pulmonary veins and tricuspid regurgitation.

Lutembacher syndrome is characterized by the association of mitral stenosis and atrial septal defect (ASD), usually of the ostium secundum type. The association between superior vena cava-type ASD and partial anomalous pulmonary venous connection is unusual and there are few descriptions in the literature. We report this condition in a 24-year-old woman who was admitted to the hospital with a 1-year history of progressive dyspnea and describe the successful surgical repair following mitral commissurotomy and tunneling of the anomalous pulmonary veins to the left atrium, which determines the closure of the ASD and tricuspid repair.

Síndrome de Heyde: Estratégias Terapêuticas e Seguimento de Longo Prazo / Heyde's Syndrome: Therapeutic Strategies and Long-Term Follow-Up

Resumo Fundamentos A síndrome de Heyde é a associação de estenose aórtica importante com episódio de sangramento gastrointestinal por lesões angiodisplásicas. Pouco é conhecido sobre os fatores associados a novos sangramentos e desfechos em longo prazo. Além disso, a maioria dos dados é restrita a relatos de casos e pequenas séries. Objetivo Avaliar o perfil clínico, laboratorial e ecocardiográfico de pacientes com síndrome de Heyde submetidos a intervenção valvar ou tratamento medicamentoso. Métodos Coorte prospectiva de 24 pacientes consecutivos entre 2005 e 2018. Foram avaliados dados clínicos, laboratoriais, ecocardiográficos e relacionados à intervenção valvar e a desfechos após o diagnóstico. Valor de p<0,05 foi considerado estatisticamente significante. Resultados Metade dos 24 pacientes apresentou sangramento com necessidade de transfusão sanguínea na admissão. Angiodisplasias foram encontradas mais frequentemente no cólon ascendente (62%). Intervenção valvar (cirúrgica ou transcateter) foi realizada em 70,8% dos pacientes, e 29,2% foram mantidos em tratamento clínico. Novos episódios de sangramento ocorreram em 25% dos casos, e não houve diferença entre os grupos clínico e intervenção (28,6 vs. 23,5%, p=1,00; respectivamente). A mortalidade no seguimento de 2 e 5 anos foi de 16% e 25%, sem diferença entre os grupos (log-rank p = 0,185 e 0,737, respectivamente). Conclusões Pacientes com síndrome de Heyde tiveram alta taxa de sangramento com necessidade de transfusão sanguínea na admissão, sugerindo ser uma doença grave e com risco elevado de mortalidade. Não encontramos diferenças entre os grupos submetidos ao tratamento clínico e à intervenção valvar em relação a taxas de ressangramento e mortalidade tardia.

Abstract Background Heyde's syndrome is the association of severe aortic stenosis with episodes of gastrointestinal bleeding due to angiodysplastic lesion. Little is known about the factors associated with new episodes of bleeding and long-term outcomes. Furthermore, most data are restricted to case reports and small case series. Objective To assess the clinical, laboratory and echocardiography profile of patients with Heyde's syndrome who underwent valve intervention or drug therapy. Methods Prospective cohort of 24 consecutive patients from 2005 to 2018. Clinical, laboratory and echocardiography data were assessed, as well as those related to valve intervention and outcomes after diagnosis. A P <0.05 was used to indicate statistical significance. Results Half of the 24 patients presented with bleeding requiring blood transfusion on admission. Angiodysplasias were more frequently found in the ascending colon (62%). Valve intervention (surgical or transcatheter) was performed in 70.8% of the patients, and 29.2% remained on drug therapy. News episodes of bleeding occurred in 25% of the cases, and there was no difference between clinical and intervention groups (28.6 vs 23.5%, p = 1.00; respectively). Mortality at 2-year and 5-year was 16% and 25%, with no difference between the groups (log-rank p = 0.185 and 0.737, respectively). Conclusions Patients with Heyde's syndrome had a high rate of bleeding requiring blood transfusion on admission, suggesting that it is a severe disease with high mortality risk. No difference was found between clinical and intervention group regarding the rate of rebleeding and late mortality.

Transcatheter Valve-in-Valve Procedures for Bioprosthetic Valve Dysfunction in Patients With Rheumatic vs. Non-Rheumatic Valvular Heart Disease.

Background: Bioprosthetic heart valve has limited durability and lower long-term performance especially in rheumatic heart disease (RHD) patients that are often subject to multiple redo operations. Minimally invasive procedures, such as transcatheter valve-in-valve (ViV) implantation, may offer an attractive alternative, although data is lacking. The aim of this study was to evaluate the baseline characteristics and clinical outcomes in rheumatic vs. non-rheumatic patients undergoing ViV procedures for severe bioprosthetic valve dysfunction. Methods: Single center, prospective study, including consecutive patients undergoing transcatheter ViV implantation in aortic, mitral and tricuspid position, from May 2015 to September 2020. RHD was defined according to clinical history, previous echocardiographic and surgical findings. Results: Among 106 patients included, 69 had rheumatic etiology and 37 were non-rheumatic. Rheumatic patients had higher incidence of female sex (73.9 vs. 43.2%, respectively; p = 0.004), atrial fibrillation (82.6 vs. 45.9%, respectively; p < 0.001), and 2 or more prior surgeries (68.1 vs. 32.4%, respectively; p = 0.001). Although, device success was similar between groups (75.4 vs. 89.2% in rheumatic vs. non-rheumatic, respectively; p = 0.148), there was a trend toward higher 30-day mortality rates in the rheumatic patients (21.7 vs. 5.4%, respectively; p = 0.057). Still, at median follow-up of 20.7 [5.1-30.4] months, cumulative mortality was similar between both groups (p = 0.779). Conclusion: Transcatheter ViV implantation is an acceptable alternative to redo operations in the treatment of patients with RHD and severe bioprosthetic valve dysfunction. Despite similar device success rates, rheumatic patients present higher 30-day mortality rates with good mid-term clinical outcomes. Future studies with a larger number of patients and follow-up are still warranted, to firmly conclude on the role transcatheter ViV procedures in the RHD population.

Heyde's Syndrome: Therapeutic Strategies and Long-Term Follow-Up. / Síndrome de Heyde: Estratégias Terapêuticas e Seguimento de Longo Prazo.

BACKGROUND: Heyde's syndrome is the association of severe aortic stenosis with episodes of gastrointestinal bleeding due to angiodysplastic lesion. Little is known about the factors associated with new episodes of bleeding and long-term outcomes. Furthermore, most data are restricted to case reports and small case series. OBJECTIVE: To assess the clinical, laboratory and echocardiography profile of patients with Heyde's syndrome who underwent valve intervention or drug therapy. METHODS: Prospective cohort of 24 consecutive patients from 2005 to 2018. Clinical, laboratory and echocardiography data were assessed, as well as those related to valve intervention and outcomes after diagnosis. A P <0.05 was used to indicate statistical significance. RESULTS: Half of the 24 patients presented with bleeding requiring blood transfusion on admission. Angiodysplasias were more frequently found in the ascending colon (62%). Valve intervention (surgical or transcatheter) was performed in 70.8% of the patients, and 29.2% remained on drug therapy. News episodes of bleeding occurred in 25% of the cases, and there was no difference between clinical and intervention groups (28.6 vs 23.5%, p = 1.00; respectively). Mortality at 2-year and 5-year was 16% and 25%, with no difference between the groups (log-rank p = 0.185 and 0.737, respectively). CONCLUSIONS: Patients with Heyde's syndrome had a high rate of bleeding requiring blood transfusion on admission, suggesting that it is a severe disease with high mortality risk. No difference was found between clinical and intervention group regarding the rate of rebleeding and late mortality.

FUNDAMENTOS: A síndrome de Heyde é a associação de estenose aórtica importante com episódio de sangramento gastrointestinal por lesões angiodisplásicas. Pouco é conhecido sobre os fatores associados a novos sangramentos e desfechos em longo prazo. Além disso, a maioria dos dados é restrita a relatos de casos e pequenas séries. OBJETIVO: Avaliar o perfil clínico, laboratorial e ecocardiográfico de pacientes com síndrome de Heyde submetidos a intervenção valvar ou tratamento medicamentoso. MÉTODOS: Coorte prospectiva de 24 pacientes consecutivos entre 2005 e 2018. Foram avaliados dados clínicos, laboratoriais, ecocardiográficos e relacionados à intervenção valvar e a desfechos após o diagnóstico. Valor de p<0,05 foi considerado estatisticamente significante. RESULTADOS: Metade dos 24 pacientes apresentou sangramento com necessidade de transfusão sanguínea na admissão. Angiodisplasias foram encontradas mais frequentemente no cólon ascendente (62%). Intervenção valvar (cirúrgica ou transcateter) foi realizada em 70,8% dos pacientes, e 29,2% foram mantidos em tratamento clínico. Novos episódios de sangramento ocorreram em 25% dos casos, e não houve diferença entre os grupos clínico e intervenção (28,6 vs. 23,5%, p=1,00; respectivamente). A mortalidade no seguimento de 2 e 5 anos foi de 16% e 25%, sem diferença entre os grupos (log-rank p = 0,185 e 0,737, respectivamente). CONCLUSÕES: Pacientes com síndrome de Heyde tiveram alta taxa de sangramento com necessidade de transfusão sanguínea na admissão, sugerindo ser uma doença grave e com risco elevado de mortalidade. Não encontramos diferenças entre os grupos submetidos ao tratamento clínico e à intervenção valvar em relação a taxas de ressangramento e mortalidade tardia.

Rheumatic Myocarditis: A Poorly Recognized Etiology of Left Ventricular Dysfunction in Valvular Heart Disease Patients.

Background: Heart failure occurs in ~10% of patients with acute rheumatic fever (RF), and several studies have shown that cardiac decompensation in RF results primarily from valvular disease and is not due to primary myocarditis. However, the literature on this topic is scarce, and a recent case series has shown that recurrent RF can cause ventricular dysfunction even in the absence of valvular heart disease. Methods: The present study evaluated the clinical, laboratory and imaging characteristics of 25 consecutive patients with a clinical diagnosis of myocarditis confirmed by 18F-FDG PET/CT or gallium-67 cardiac scintigraphy and RF reactivation according to the revised Jones Criteria. Patients underwent three sequential echocardiograms at (1) baseline, (2) during myocarditis and (3) post corticosteroid treatment. Patients were divided according to the presence (Group 1) or absence (Group 2) of reduced left ventricular ejection fraction (LVEF) during myocarditis episodes. Results: The median age was 42 (17-51) years, 64% of patients were older than 40 years, and 64% were women. Between Group 1 (n = 16) and in Group 2 (n = 9), there were no demographic, echocardiographic or laboratory differences except for NYHA III/IV heart failure (Group 1: 100.0% vs. Group 2: 50.0%; p = 0.012) and LVEF (30 [25-37] vs. 56 [49-62]%, respectively; p < 0.001), as expected. Group 1 patients showed a significant reduction in LVEF during carditis with further improvement after treatment. There was no correlation between LVEF and valvular dysfunction during myocarditis. Among all patients, 19 (76%) underwent 18F-FDG PET/CT, with a positive scan in 68.4%, and 21 (84%) underwent gallium-67 cardiac scintigraphy, with positive uptake in 95.2%, there was no difference between these groups. Conclusion: Myocarditis due to rheumatic fever reactivation can cause left ventricular dysfunction despite valvular disease, and it is reversible after corticosteroid treatment.

Requerimento administrativo prévio e judicialização da saúde pública: uma análise do requisito e de suas exceções / Prior administrative request and judicialization of public health: An analysis of the requirement and its exceptions / Prior administrative request and judicialization of public health: An analysis of the requirement and its exceptions

O estudo analisou em que medida o requerimento administrativo prévio deve condicionar o acesso à justiça em demandas sobre saúde pública. Inicialmente, adotou-se o método dedutivo para, a partir da concepção de princípios presente na teoria dos direitos fundamentais de Robert Alexy, investigar a possibilidade de exigência desse pressuposto processual no contexto de colisão entre os princípios do acesso à justiça e os do devido processo legal instaurada para a proteção do direito fundamental à saúde. Em seguida, por meio do método indutivo, analisaram-se os fundamentos do julgamento do Supremo Tribunal Federal que condicionara o ajuizamento de ações previdenciárias ao requerimento administrativo prévio a fim de identificar se, consideradas as semelhanças e diferenças, essas razões seriam também aplicáveis, e em que medida, às demandas em matéria de saúde pública. Após, expuseram-se contribuições da adoção do prévio requerimento administrativo como requisito ao processamento de ações judiciais sobre saúde pública para o aprimoramento qualitativo da política pública sanitária, bem como para a prestação jurisdicional e a resolução adequada de conflitos nesse campo. Ao final, investigaram-se os limites e exceções a serem observados quanto à exigência de prévio requerimento administrativo em ações sobre saúde pública, com base na fundamentalidade do direito à saúde e nos parâmetros que vêm sendo consolidados na jurisprudência dos tribunais superiores, nos enunciados das Jornadas de Direito da Saúde e na literatura especializada.

The study analyzed to what extent the prior administrative request should condition the access to justice in public health demands. Initially, the deductive method was adopted, based on the concept of principles present in Robert Alexy's theory of fundamental rights, to investigate the possibility of requiring this procedural assumption in the context of the collision between the principles of access to justice and due process of law established for the protection of the fundamental right to health. Then, through the inductive method, we analyzed the fundamentals of Supreme Court judgment that conditioned the filing of social security lawsuits to the prior administrative request in order to identify whether, considered as similarities and differences, these possible reasons would also be applicable, and to what extent, to the demands in terms of public health. Afterwards, the contributions of the adoption of the prior administrative request as a requirement for processing lawsuits on public health for the qualitative improvement of public health policy were exposed, as well as for the provision of jurisdiction and the appropriate resolution of conflicts in this field. At the end, the limits and exceptions to be observed regarding the requirement of prior administrative request in public health lawsuits were investigated, based on the fundamentals of the right to health and on the parameters that have been consolidated in the jurisprudence of the higher courts, in the statements of the Health Law Conferences and in the specialized literature.

Non-toxigenic Corynebacterium diphtheriae infective endocarditis with embolic events: a case report.

BACKGROUND: Corynebacterium diphtheriae (C. diphtheriae) infections, usually related to upper airways involvement, could be highly invasive. Especially in developing countries, non-toxigenic C. diphtheriae strains are now emerging as cause of invasive disease like endocarditis. The present case stands out for reinforcing the high virulence of this pathogen, demonstrated by the multiple systemic embolism and severe valve deterioration. It also emphasizes the importance of a coordinated interdisciplinary work to address all these challenges related to infectious endocarditis. CASE PRESENTATION: A 21-year-old male cocaine drug abuser presented to the emergency department with a 1-week history of fever, asthenia and dyspnea. His physical examination revealed a mitral systolic murmur, signs of acute arterial occlusion of the left lower limb, severe arterial hypotension and acute respiratory failure, with need of vasoactive drugs, orotracheal intubation/mechanical ventilation, empiric antimicrobial therapy and emergent endovascular treatment. The clinical suspicion of acute infective endocarditis was confirmed by transesophageal echocardiography, demonstrating a large vegetation on the mitral valve associated with severe valvular regurgitation. Abdominal ultrasound was normal with no hepatic, renal, or spleen abscess. Serial blood cultures and thrombus culture, obtained in the vascular procedure, identified non-toxigenic C. diphtheriae, with antibiotic therapy adjustment to monotherapy with ampicillin. Since the patient had a severe septic shock with sustained fever, despite antimicrobial therapy, urgent cardiac surgical intervention was planned. Anatomical findings were compatible with an aggressive endocarditis, requiring mitral valve replacement for a biological prosthesis. During the postoperative period, despite an initial clinical recovery and successfully weaning from mechanical ventilation, the patient presented with a recrudescent daily fever. Computed tomography of the abdomen revealed a hypoattenuating and extensive splenic lesion suggestive of abscess. After sonographically guided bridging percutaneous catheter drainage, surgical splenectomy was performed. Despite left limb revascularization, a forefoot amputation was required due to gangrene. The patient had a good clinical recovery, fulfilling 4-weeks of antimicrobial treatment. CONCLUSION: Despite the effectiveness of toxoid-based vaccines, recent global outbreaks of invasive C. diphtheriae infectious related to non-toxigenic strains have been described. These infectious could be highly invasive as demonstrated in this case. Interdisciplinary work with an institutional "endocarditis team" is essential to achieve favorable clinical outcomes in such defiant scenarios.

Cogan's syndrome - A rare aortitis, difficult to diagnose but with therapeutic potential / Síndrome de Cogan - Uma aortite rara, de diagnóstico difícil, mas com potencial terapêutico

Summary The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control.

Resumo A inflamação da parede da aorta, denominada aortite, é uma condição clínica rara, que pode ser causada por diversas patologias, principalmente as de fundo inflamatório e/ou infeccioso. Nesse contexto, a ocorrência de sintomas vestibulares e oftalmológicos associados ao quadro remete ao diagnóstico de síndrome de Cogan, tornando o caso raro, mas passível de tratamento imunossupressor adequado e controle satisfatório da doença.

Rheumatic Heart Disease and Myxomatous Degeneration: Differences and Similarities of Valve Damage Resulting from Autoimmune Reactions and Matrix Disorganization.

Autoimmune inflammatory reactions leading to rheumatic fever (RF) and rheumatic heart disease (RHD) result from untreated Streptococcus pyogenes throat infections in individuals who exhibit genetic susceptibility. Immune effector mechanisms have been described that lead to heart tissue damage culminating in mitral and aortic valve dysfunctions. In myxomatous valve degeneration (MXD), the mitral valve is also damaged due to non-inflammatory mechanisms. Both diseases are characterized by structural valve disarray and a previous proteomic analysis of them has disclosed a distinct profile of matrix/structural proteins differentially expressed. Given their relevance in organizing valve tissue, we quantitatively evaluated the expression of vimentin, collagen VI, lumican, and vitronectin as well as performed immunohistochemical analysis of their distribution in valve tissue lesions of patients in both diseases. We identified abundant expression of two isoforms of vimentin (45 kDa, 42 kDa) with reduced expression of the full-size protein (54 kDa) in RHD valves. We also found increased vitronectin expression, reduced collagen VI expression and similar lumican expression between RHD and MXD valves. Immunohistochemical analysis indicated disrupted patterns of these proteins in myxomatous degeneration valves and disorganized distribution in rheumatic heart disease valves that correlated with clinical manifestations such as valve regurgitation or stenosis. Confocal microscopy analysis revealed a diverse pattern of distribution of collagen VI and lumican into RHD and MXD valves. Altogether, these results demonstrated distinct patterns of altered valve expression and tissue distribution/organization of structural/matrix proteins that play important pathophysiological roles in both valve diseases.

Cogan's syndrome - A rare aortitis, difficult to diagnose but with therapeutic potential.

The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control.