Heart transplantation in primary amyloidosis

Cardiac amyloidosis is a disease with a gloom life expectance after the beginning of the symptomatic phase, usually with sudden death as the final event. The aggression to other organs, although, can make heart transplantation a disputable form of treatment taking into consideration the shortage of donor organs. The aim is to report the evolution with a survival of seven years after heart transplantation and in very fair condition of a patient with amyloidosis. One year after the heart transplantation, there was indication of renal transplantation also from the aggression from the disease. This patient compares' favorable with three other patients also from our service, who died early after de diagnosis. Even considering the multi systemic nature of amyloidosis, we can accept that in peculiar patients justified the heart transplantation, taking in the consideration the very bad prognosis of the diseaseA amiloidose cardíaca é doença altamente limitante da sobrevida, por morte súbita na maioria dos pacientes. Pela agressão contra outros órgãos, particularmente rins e sistema nervoso central, o transplante cardíaco tem sido opção questionável, face à escassez de órgãos. O objetivo é relatar a evolução, com sobrevivência de 7 anos, da paciente após transplante cardíaco por amiloidose, em boas condições. Um ano após o transplante cardíaco, houve indicação de transplante renal, também pela agressão da doença. Esta paciente contrasta com outros três pacientes de nosso serviço que foram a óbito, ainda na fase de avaliação. Apesar de sua natureza multisistêmica, a amiloidose cardíaca pode, em pacientes selecionados, justificar o transplante cardíaco, pela gravidade do seu potencial evolutivo após o início dos sintomasHospital Beneficência PortuguesaUNIFESPHospital Beneficência Portuguesa UTI cardiológicaUNIFESPSciEL

Pervasive gaps in Amazonian ecological research

Biodiversity loss is one of the main challenges of our time,1,2 and attempts to address it require a clear un derstanding of how ecological communities respond to environmental change across time and space.3,4 While the increasing availability of global databases on ecological communities has advanced our knowledge of biodiversity sensitivity to environmental changes,5–7 vast areas of the tropics remain understudied.8–11 In the American tropics, Amazonia stands out as the world’s most diverse rainforest and the primary source of Neotropical biodiversity,12 but it remains among the least known forests in America and is often underrepre sented in biodiversity databases.13–15 To worsen this situation, human-induced modifications16,17 may elim inate pieces of the Amazon’s biodiversity puzzle before we can use them to understand how ecological com munities are responding. To increase generalization and applicability of biodiversity knowledge,18,19 it is thus crucial to reduce biases in ecological research, particularly in regions projected to face the most pronounced environmental changes. We integrate ecological community metadata of 7,694 sampling sites for multiple or ganism groups in a machine learning model framework to map the research probability across the Brazilian Amazonia, while identifying the region’s vulnerability to environmental change. 15%–18% of the most ne glected areas in ecological research are expected to experience severe climate or land use changes by 2050. This means that unless we take immediate action, we will not be able to establish their current status, much less monitor how it is changing and what is being lostinfo:eu-repo/semantics/publishedVersio

Pervasive gaps in Amazonian ecological research

Biodiversity loss is one of the main challenges of our time,1,2 and attempts to address it require a clear understanding of how ecological communities respond to environmental change across time and space.3,4 While the increasing availability of global databases on ecological communities has advanced our knowledge of biodiversity sensitivity to environmental changes,5,6,7 vast areas of the tropics remain understudied.8,9,10,11 In the American tropics, Amazonia stands out as the world's most diverse rainforest and the primary source of Neotropical biodiversity,12 but it remains among the least known forests in America and is often underrepresented in biodiversity databases.13,14,15 To worsen this situation, human-induced modifications16,17 may eliminate pieces of the Amazon's biodiversity puzzle before we can use them to understand how ecological communities are responding. To increase generalization and applicability of biodiversity knowledge,18,19 it is thus crucial to reduce biases in ecological research, particularly in regions projected to face the most pronounced environmental changes. We integrate ecological community metadata of 7,694 sampling sites for multiple organism groups in a machine learning model framework to map the research probability across the Brazilian Amazonia, while identifying the region's vulnerability to environmental change. 15%–18% of the most neglected areas in ecological research are expected to experience severe climate or land use changes by 2050. This means that unless we take immediate action, we will not be able to establish their current status, much less monitor how it is changing and what is being lost

Cardiogenic shock due to citomegalovirus myocarditis: successful clinical treatment Choque cardiogênico devido à miocardite por citomegalovírus: terapêutica clínica com sucesso

OBJECTIVE: Cytomegalovirus (CMV) systemic disease and myocarditis in healthy persons is infrequently reported in the literature, although in increasing numbers in recent years. The importance of the recognition of the syndrome that usually has an initial picture of a mononucleosis like infection in an otherwise healthy person, is the available therapeutic agent, ganciclovir, that can cure the infectious disease. METHODS: We analyzed the clinical result of pulsotherapy with steroids in a patient with CMV myocarditis after 7 days of etiological treatment, with ganciclovir, intravenous vasodilators, and the conventional treatment for congestive heart failure. RESULTS: The clinical condition of the patient improved accordingly to the better function of the left ventricle, and the ganciclovir was kept for 21 days, most of it in an out patient basis. The patient was dismissed from the hospital, with normal myocardial function. CONCLUSION: Potentially curable forms of myocarditis, like M pneumoniae and CMV, for example, can have an initial disproportionate aggression to the myocardium, by the acute inflammatory reaction, that can by itself make worse the damage to the LV function. In our opinion, the blockade of this process by pulsotherapy with steroids can help in the treatment of these patients. We understand that the different scenario of immunosuppressive treatments for the possible auto immunity of the more chronic forms of the presumably post viral cardiomyopathy has been in dispute in the literature, and has stolen the focus from the truly acute cases.<br>OBJETIVO: Doença sistêmica por citomegalovírus (CMV) com miocardite em pessoas saudáveis é raramente referida na literatura, apesar de em maior número em anos recentes. A importância do reconhecimento da síndrome, que usualmente tem um quadro inicial "mononucleosis like" em uma pessoa sadia é a disponibilidade do agente terapêutico ganciclovir, que pode curar a infecção. MÉTODOS: Nós analisamos o resultado da pulsoterapia com esteróides em um paciente com miocardite por CMV, após 7 dias de tratamento etiológico com ganciclovir, vasodilatadores intravenosos e o tratamento convencional para insuficiência cardíaca congestiva. RESULTADOS: A condição clínica do paciente melhorou com a melhor função do ventrículo esquerdo e o ganciclovir foi mantido por 21 dias após alta hospitalar.A função miocárdica retornou ao normal. CONCLUSÃO: Formas curáveis de miocardites como M pneumonia e CMV, por exemplo, podem ter uma agressão grave ao miocárdio por uma ação inflamatória que pode piorar a função cardíaca. Em nossa opinião, o bloqueio deste processo pela pulsoterapia com esteróides pode auxiliar no tratamento destes pacientes. Entendemos que existe um cenário diferente de tratamento com imunossupressores para possível agressão auto-imune das formas mais crônicas de cardiomiopatias dilatadas e isso está em disputa na literatura, talvez mudando o foco dos casos realmente agudos

Late results of pulmonary root translocation in the correction of transposition of the great arteries Resultados tardios da translocação da raiz pulmonar na correção da transposição das grandes artérias

OBJECTIVE: The purpose of this study was to evaluate the late follow-up of pulmonary root translocation, a technique that aims to avoid complications and the need of reoperations related to the Rastelli procedure, in the repair of ventriculo-arterial connection anomalies associated to pulmonary stenosis and ventricular septal defect. METHODS: Five patients, ranging from two months to three years of age, were submitted to anterior pulmonary root translocation, from April 1994 to July 1999. The surgical technique consisted of pulmonary root removal from the left ventricle and its connection to the right ventricle, after patch diversion of the blood flow from the left ventricle to the aorta, through the ventricular septal defect. The construction of the right ventricle outflow tract was completed with autologous pericardium. RESULTS: There were neither early nor late deaths. There was no need for re-intervention. The late clinical and echocardiographic follow-ups showed some growth of the pulmonary root, with no clinically significant transpulmonary valve gradient (zero to 41 mmHg) and all patients were asymptomatic. CONCLUSION: Pulmonary root translocation showed to be efficient in the repair of ventriculo-arterial connection anomalies, with ventricular septal defect and pulmonary stenosis, and it was employed even in small children, with good early and long-term results, without the need for late reoperations in this small group of patients.<br>OBJETIVO: Este estudo tem por objetivo avaliar a evolução tardia da translocação da raiz pulmonar, técnica que visa evitar as complicações e a necessidade de reintervenções da operação de Rastelli, na correção de anomalias da conexão ventrículo arterial associados à estenose pulmonar (EP) e comunicação interventricular (CIV). MÉTODO: Cinco pacientes, com idade de dois meses a três anos, foram submetidos à translocação anterior da raiz pulmonar, no período de abril de 1994 a julho de 1999. A técnica consistiu da retirada da raiz pulmonar, com valva, do ventrículo esquerdo (VE) e a sua conexão ao ventrículo direito (VD), após o fluxo do VE ter sido direcionado para a aorta, através da CIV, com placa de teflon. A construção da via de saída do VD foi completada com pericárdio autólogo. RESULTADOS: Não houve mortalidade precoce ou tardia. Não houve necessidade de reintervenção. O seguimento clínico e ecocardiográfico tardio mostra crescimento da raiz pulmonar, sem gradiente transvalvar pulmonar clinicamente significativo (zero a 41 mmHg) e sem insuficiência valvar pulmonar, com todos os pacientes assintomáticos. CONCLUSÃO: A translocação da raiz pulmonar mostrou-se método eficaz na correção anatômica das anomalias de conexão ventrículo-arteriais associadas a CIV e EP, sendo possível sua utilização mesmo em crianças menores, com bom resultado a curto e longo prazo, demonstrando a não necessidade de reoperações, neste pequeno grupo de pacientes