Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital

Abstract Background: Coarctation of the aorta is a congenital segmental narrowing of the aortic arch with severe hemodynamic repercussions and increased cardiovascular mortality. Early surgical correction and life-time echocardiographic follow-up must be performed to improve prognosis. However, this goal has been challenged by high rates of underdiagnosis, which delay surgical correction, and by recoarctation in up to one third of operated patients. Objectives: The objectives of this study were: (i) to register the frequency of common clinical signs at diagnosis of coarctation of the aorta; (ii) to describe the course of echocardiographic parameters before and during the follow-up of coartectomized subjects; (iii) to analyze the clinical prognosis of patients according to baseline characteristics, occurrence of recoarctation and associated malformations. Methods: Case-series of 72 patients coarctectomized between June 1996 and November 2016 in a tertiary care hospital. Clinical, echocardiographic and surgical variables were considered. All patients were submitted to coarctectomy by posterolateral thoracotomy and end-to-end anastomosis. Data were classified as parametric or non-parametric by Kolmogorov-Smirnov test. Parametric data were expressed as mean and standard deviation, and non-parametric data as median and interquartile range. Continuous variables were analyzed using paired t-tests, and categorical variables were compared by chi-square test. For all analysis, a p-value of less than 0.05 was considered statistically significant. Statistical analysis was performed using SPSS, version 20.0 (IBM, Chicago, IL, USA). Results: The mean follow-up time was 5.8 years (range: 0-20 years). At diagnosis, most patients had heart murmur (88%), non-palpable pulse in the lower limbs (50%), left ventricular hypertrophy (78%), and bicuspid aortic valve (33%), with a mean aortic peak gradient of 55 mmHg. After surgical correction, those without recoarctation were less symptomatic (60 vs 4.5%; p < 0.001), had lower aortic peak gradient (54 ± 3.8 vs 13 ± 0.8; p = 0.01) and left ventricle mass (95 ± 9.2 vs. 63 ± 11; p = 0.01), and the most common complications were late hypertension (39.2%), and recoarctation (27.6%). Recoarcted patients did not show improvement of neither clinical nor echocardiographic variables. Age at repair and bicuspid aortic valve groups had comparable results with controls. Surgical procedure was safe; mean time of hospitalization was 10 days and mean surgery time 2.3 hours. Conclusions: Coarctectomy improves cardiac symptoms and left ventricular hypertrophy, with a slight effect on the incidence of hypertension. Recoarctation occurs in one-third of patients and draws attention for the need of lifelong surveillance by echocardiography.

Central role of obesity in endothelial cell dysfunction and cardiovascular risk

SUMMARY Atherosclerosis is the leading cause of mortality in the contemporary world. The critical role of the endothelial cells (EC) in vascular homeostasis, the metabolic changes that take place when the cell is activated, and the elements involved in these processes have been widely explored over the past years. Obesity and its impact, promoting a rise in blood levels of free fatty acids (FAs) are often associated with atherosclerosis and cardiovascular mortality. However, the mechanisms that promote cardiovascular structural changes and adaptive changes in the ECs, particularly in the context of obesity, are little known. Here, we reviewed studies that assessed the metabolic adaptations of healthy and dysfunctional ECs during exposure to FAs, as well as the epidemiological perspectives of cardiovascular structural changes in obesity. Finally, we explored the role of new agents - sphingolipids, dietary unsaturated fatty acids and sodium-glucose cotransporter-2 inhibitors (iSGLT2) - in atherosclerosis and their relationship with obesity.

RESUMO A aterosclerose é a causa líder de mortalidade no mundo contemporâneo. O papel central da célula endotelial (EC) na homeostase vascular, as alterações metabólicas que ocorrem quando a célula se torna ativada e os elementos envolvidos nesses processos vêm sendo bastante explorados nos últimos anos. A obesidade e o seu impacto, promovendo uma elevação dos níveis sanguíneos de ácidos graxos (FAs) livres, é bastante associada à aterosclerose e à mortalidade cardiovascular. Entretanto, os mecanismos que promovem alterações estruturais cardiovasculares e alterações adaptativas nas ECs, particularmente no contexto da obesidade, são pouco conhecidos. Aqui, nós revisamos estudos que avaliaram as adaptações metabólicas das ECs normais e disfuncionais durante exposição a FAs, bem como as perspectivas epidemiológicas das alterações cardiovasculares estruturais na obesidade. Finalmente, exploramos o papel de novos atores — esfingolípides, ácidos graxos insaturados da dieta e inibidores do cotransportador de sódio-glucose 2 (iSGLT2) — na aterosclerose e sua relação com a obesidade.

Diabetic cardiomyopathy: factual or factoid?

SUMMARY Although long ago described, there is no established consensus regarding the real existence of Diabetic Cardiomyopathy (CMPDM). Due to its complex pathophysiology, it has been difficult for clinical and experimental research to establish clear connections between diabetes mellitus (DM) and heart failure (HF), as well as to solve the mechanisms of the underlying myocardial disease. However, the epidemiological evidence of the relationship of these conditions is undisputed. The interest in understanding this disease has intensified due to the recent results of clinical trials evaluating new glucose-lowering drugs, such as sodium-glucose transporter inhibitors 2, which demonstrated favorable responses considering the prevention and treatment of HF in patients with DM. In this review we cover aspects of the epidemiology of CMPDM and its possible pathogenic mechanisms, as well as, present the main cardiac phenotypes of CMPDM (HF with preserved and reduced ejection fraction) and implications of the therapeutic management of this disease.

RESUMO Apesar de há muito tempo descrita, não existe consenso estabelecido quanto à real existência da cardiomiopatia diabética (CMPDM). Devido à sua complexa fisiopatologia, tem sido árduo à pesquisa clínica e experimental estabelecer conexões claras entre diabetes mellitus (DM) e insuficiência cardíaca (IC), assim como solucionar os mecanismos da doença subjacente do miocárdio. No entanto, as evidências epidemiológicas da relação dessas condições são incontestáveis. O interesse em compreender melhor essa doença tem recrudescido devido aos recentes resultados de ensaios clínicos avaliando novos fármacos hipoglicemiantes, como os inibidores do transportador de sódio-glicose 2, que demonstraram respostas favoráveis, considerando-se a prevenção e tratamento da IC em pacientes portadores de DM. Nesta revisão, percorremos aspectos da epidemiologia da CMPDM e de seus possíveis mecanismos patogênicos, além de apresentarmos os principais fenótipos cardíacos da CMPDM (IC com fração de ejeção preservada e reduzida) e implicações do manejo terapêutico desta doença.

Infliximab reduces cardiac output in rheumatoid arthritis patients without heart failure / Infliximabe reduz débito cardíaco em pacientes com artrite reumatoide sem insuficiência cardíaca

OBJECTIVE: Human anti-tumor necrosis factor (TNF-α) monoclonal antibody (infliximab) is used to treat autoimmune diseases such as rheumatoid arthritis (RA). Although the risk of worsening heart failure has been described in patients under chronic treatment, the acute cardiovascular effects of this drug are unknown in RA patients without heart failure. METHODS: 14 RA patients with normal echocardiography and no history of heart failure were evaluated during the 2-hour infliximab (3-5 mg/kg) infusion period, using a noninvasive hemodynamic beat-to-beat system (Portapres). Stroke volume (SV); systolic, diastolic and mean blood pressures (SBP, DBP and MBP, respectively); cardiac output (CO); heart rate (HR); and total peripheral vascular resistance (PVR) were recorded. All patients also received saline infusion instead of infliximab as a control. Significant differences in hemodynamic parameters were determined using Tuckey's test. All values were expressed as mean ± standard deviation (SD). RESULTS: Fourteen RA patients (6M/8F) with mean age of 47.2 ± 8.8 years were evaluated. A significant decrease was found in cardiac output and stroke volume (7.04 ± 2.3 to 6.12 ± 2.1 l/min and 91 ± 29.0 to 83 ± 28.8 mL/beat, respectively) after infliximab infusion. Although not statistically significant, a progressive increase was detected in SBP, DBP and total PVR during infusion. Saline infusion did not cause significant hemodynamic changes in the same group of RA patients. No adverse effects were observed during the infusion period. CONCLUSION: Acute infliximab administration decreased cardiac output due to low stroke volume in RA patients without heart disease. The results also demonstrated that, in spite of its negative inotropic effect, infliximab enhanced BP, probably by increasing PVR.

OBJETIVO: O inibidor de fator de necrose tumoral (TNF-α) infliximabe é usado no tratamento de doenças autoimunes como a artrite reumatoide (AR). Embora o risco de piora de insuficiência cardíaca em pacientes submetidos a tratamento crônico tenha sido descrito, os efeitos cardiovasculares agudos da infusão desta droga em pacientes com AR sem insuficiência cardíaca são desconhecidos. MÉTODOS: Pacientes com AR e ecocardiogramas normais e sem antecedentes de insuficiência cardíaca foram avaliados durante o período de infusão de infliximabe (3-5mg/kg), de 2 horas, utilizando um sistema de monitoramento hemodinâmico não invasivo batimento-a-batimento (Portapres). As variáveis avaliadas foram: volume sistólico (VS), pressão arterial sistólica, diastólica e média (PAS, PAD e PAM, respectivamente), débito cardíaco (DC), frequência cardíaca (FC) e resistência vascular periférica total (RVPT). Todos os voluntários também receberam infusão de soro fisiológico (SF) como estudo controle. Estatísticas foram avaliadas usando o teste de Tuckey. Os valores estão expressos em média ± desvio-padrão. RESULTADOS: Catorze pacientes (6M/8F), com idade média de 47,2 ± 8,8 anos, foram avaliados. Reduções significativas no débito cardíaco e volume sistólico foram encontradas após a infusão do infliximabe (7,04 ± 2,3 a 6,12 ± 2,1 L/min e 91 ± 29,0 a 83 ± 28,8 mL/batimento, respectivamente). Embora não estatisticamente significante, detectaram-se aumentos progressivos na PAS, PAD e RVPT durante a infusão. A infusão controle de SF não causou mudanças hemodinâmicas significativas nos pacientes estudados. Não foram observados efeitos adversos no período de infusão. CONCLUSÃO: A administração de infliximabe reduz agudamente o débito cardíaco devido a redução no volume sistólico em pacientes com AR sem insuficiência cardíaca. Nossos resultados mostram que, apesar do efeito inotrópico negativo, o infliximabe elevou a pressão arterial, provavelmente devido ao aumento na RVPT.