Aborted sudden cardiac death in a young patient with epilepsy and the Gorlin Goltz syndrome.

Epilepsy is one of the most common chronical neurological conditions affecting over 50 million people worldwide. In addition to the stigma and discrimination, individuals with epilepsy suffer from a nearly three-fold increased risk of premature death compared to the general population. Although these premature deaths occur due to multiple causes, sudden unexpected death in epilepsy (SUDEP) still challenges neurologists and clinicians dealing with individuals with epilepsy. Recently, an increased interest in cardiac outcomes related to acute seizures and chronic epilepsy resulted in the groundbreaking development of the "epileptic heart" concept, and sudden cardiac death in individuals with epilepsy, which is 4.5 times as frequent as SUDEP according to some observational data, has gained more attention. As we gather information and learn about possible comorbidities and consequences of seizures and/or chronic epilepsy, we present a clinical case of a young patient with an unusual association of epilepsy, the Gorlin Goltz syndrome, and a cardiac fibroma with Wolf-Parkinson-White (WPW), who had multiple aborted cardiac arrests. Diagnostic challenges and multiple possible causes of sudden cardiac death in this single patient report are discussed.

The epileptic heart: Cardiac comorbidities and complications of epilepsy. Atrial and ventricular structure and function by echocardiography in individuals with epilepsy - From clinical implications to individualized assessment.

Epilepsy is an increasing global neurological health issue. Recently, epidemiological and mechanistic studies have raised concern about cardiac involvement in individuals with epilepsy. This has resulted in the "epileptic heart" concept. Epidemiological data linking epilepsy to cardiovascular disease indicate an increased risk for ventricular and atrial arrhythmias, myocardial infarction, heart failure, and sudden death among individuals with epilepsy. Pathways of this interaction comprise increased prevalence of traditional cardiac risk factors, genetic abnormalities, altered brain circuitry with autonomic imbalance, and antiseizure medications with enzyme-inducing and ionic channel-blocking proprieties. Pathophysiological findings in the atria and ventricles of patients with epilepsy are discussed. Echocardiographic findings and future applications of this tool are reviewed. A risk stratification model and future studies on cardiac risk assessment in individuals with epilepsy are proposed.

Desproporção prótese-paciente em indivíduos submetidos à troca valvar aórtica / Prosthesis-patient mismatch in Individuals undergoing aortic valve replacement

Fundamentos: A desproporção prótese-paciente (DPP) em indivíduos submetidos à cirurgia de troca valvar aórtica é objeto de muitas pesquisas e está associada a piora do prognóstico em longo prazo. Objetivos: Avaliar a incidência da DPP após cirurgia de troca valvar aórtica e caracterizar o perfil clínico dos pacientes submetidos a esta cirurgia. Métodos: Estudo transversal, observacional, retrospectivo com 53 pacientes hospitalizados para tratamento cirúrgico de estenose valvar aórtica grave, no período de janeiro de 2014 a junho de 2015. Foram utilizados três modelos de biopróteses e um modelo de prótese metálica. A área de orifício efetivo indexado (AOEi) foi calculada a partir da divisão da área de orifício efetivo fornecida pelo fabricante da prótese pela área de superfície corporal do paciente receptor. Definiu-se a presença de DPP quando AOEi <0,90cm²/m². Resultados: A média de idade dos pacientes foi 65,8±9,9 anos, predominando o sexo masculino. A mortalidade geral foi 11,3%. A DPP ocorreu em 32,0% dos pacientes, sendo a maioria de grau moderado. A prótese Biocor apresentou 70,5% de DPP enquanto a prótese Braile não demonstrou nenhum caso. Não houve diferença na redução dos gradientes transvalvares aórticos no período pós-operatório entre os grupos com e sem DPP. Conclusões: O perfil dos pacientes assemelha-se ao descrito na literatura, a incidência de DPP é elevada, variando muito entre os modelos de próteses. Não houve diferença na redução dos gradientes pós-operatórios entre os grupos com ou sem DPP.

Background: Prosthesis-patient mismatch (PPM) in patients undergoing aortic valve replacement surgery is the subject of many research studies and is associated with worse prognosis in the long term. Objectives: To evaluate the incidence of PPM after aortic valve replacement surgery and characterize the clinical profile of patients undergoing this surgery. Methods: Cross-sectional observational retrospective study of 53 patients hospitalized for surgical treatment of severe aortic valve stenosis from January 2014 to June 2015. Three models of bioprosthesis and a metal prosthesis model were used. Indexed effective orifice area (iEOA) was calculated by dividing the effective orifice area provided by the prosthesis manufacturer by the body surface area of the recipient patient. The presence of PPM was defined when iEOA <0.90 cm²/m². Results: The average age of patients was 65,8±9,9. Male sex prevailed. Overall mortality rate was 11.3%. PPM occurred in 32.0% of patients, mostly of which were moderate degree. The prosthesis Biocor showed 70.5% of PPM while the Braile prosthesis showed no case. There was no difference in the reduction of transvalvular aortic gradients in the postoperative period between the groups with and without PPM. Conclusions: The patients profile is similar to that described in the literature, the incidence of PPM is high, varying greatly between the prosthesis models. There was no difference in the reduction of postoperative gradients between the groups with or without PPM.

Revisiting the J-curve phenomenon. An old new concept?

Cohort studies have demonstrated the association between blood pressure and increased cardiovascular events. There are different therapeutic strategies to achieve goals of systolic and diastolic blood pressure. For a long time, therapeutic targets were not well defined and the concept of "smaller is better" was used diffusely. However, clinical trials have shown the presence of a "J-curve" in different clinical situations: below a certain level of blood pressure, more aggressive reductions may not represent benefit and increase the incidence of adverse events in elderly patients, patients with coronary artery disease, patients with diabetes or chronic renal failure.