Influence of centre expertise on the diagnosis and management of hypertrophic cardiomyopathy: A study from the French register of hypertrophic cardiomyopathy (REMY).

BACKGROUND: Our knowledge of hypertrophic cardiomyopathy (HCM) mainly originates from quarternary centres. The objective is to assess the current management of HCM patients in a large multicentre French register according to the level of expertise. METHODS AND RESULTS: A total of 1431 HCM patients were recruited across 26 (11 expert and 15 non-expert) centres in REMY, a prospective hospital-based register of adult HCM patients. A sarcomeric origin was suspected in 1284 (89.7%) patients [261 (20.3%) with a reported gene mutation, 242 (18.8%) genotype-negative], while 107 (7.5%) had a diagnosis of non-sarcomeric HCM. Patients managed in non-expert centres were older (P < 0.01) and presented more often with NYHA III/IV class dyspnoea (P < 0.01), congestive heart failure (P < 0.01), low LEVF (P < 0.01), less often with a syncope history (P < 0.01) and lower LV obstruction (P < 0.01) than patients in expert centres. Genotype positive sarcomeric aetiologies were less frequent in non-expert centres (P < 0.01). The use of diagnostic and prognostic tests as cardiac MRI (P < 0.001), genetic (P < 0.001) and alpha-galactosidase A enzyme level testing (P < 0.001), Holter ECG (P < 0.001), and exercise test (P < 0.001), was lower in non-expert centres. Septal ablation procedures using alcohol (P < 0.001) or myectomy (P < 0.001) were more frequent in expert centres. CONCLUSION: In real life practice, only a minority of HCM patients are identified as sarcomere positive as per genetic testing. The management of HCM patients varies according to the centre's level of expertise, with less access to diagnostic and prognostic tests in non-expert centres. Non-sarcomeric HCM may therefore be overlooked despite specific treatment in some aetiologies.

Differential Effect of Acute Iron Overload on Oxidative Status and Antioxidant Content in Regions of Rat Brain.

The hypothesis of this study is that the cerebral cortex, hippocampus, and striatum of the rat brain are differentially affected in terms of oxidative stress and antioxidant capacity by acute Fe overload because Fe is distributed in a heterogeneous fashion among different regions and cells of the brain. The effects on the lipophilic and hydrophilic cellular environment were compared between regions and with the whole brain. A single dose of Fe-dextran increased Fe deposits, reaching a maximum after 6 hr. Both in whole brain and in cortex region, the ascorbyl/ascorbate content ratio was increased after 6 hr of Fe administration, while in striatum and hippocampus, there was no significant changes after Fe overload. Total thiol content decreased in whole brain and cortex, while there were no significant changes in striatum and hippocampus after Fe overload. The content of α-tocopherol (α-T), whether measured in the whole brain or in the isolated regions, did not change following Fe treatment. Lipid radical (LR•) generation rate after Fe-dextran overload only increased in the cortex region. The LR•/α-T content ratio was increased by Fe treatment in cortex but not in the whole brain, striatum, or hippocampus, in agreement with the study tested hypothesis.

Hipertensión matinal y comportamiento non-dipper en embarazadas con hipertensión de guardapolvo blanco / Morning Hypertension and Non-dipper Behavior in Pregnant Women with White Coat Syndrome

Introducción: La hipertensión de guardapolvo blanco (HGB) es común en el embarazo, aunque su evolución clínica durante la gestación se conoce poco. La hipertensión matinal y el comportamiento non-dipper, medidos por monitoreo ambulatorio de la presión arterial (MAPA), se asocian con mayor riesgo de eventos cardiovasculares; no obstante ello, son escasos los estudios en la gestación. Objetivos: El objetivo primario del estudio fue evaluar la hipertensión matinal y el comportamiento non-dipper en embarazadas con HGB versus un grupo control de normotensas. Se planteó como objetivo secundario evaluar si la HGB, la hipertensión matinal y el comportamiento non-dipper en el segundo trimestre del embarazo se relacionan con mayor hipertensión sostenida en el tercer trimestre. Material y métodos: Estudio prospectivo en el que se incluyeron 95 primigestas en la semana 20 de gestación, 50 con HGB y 45 normotensas como grupo control. Se registraron laboratorio de rutina, presión de consultorio y MAPA en la inclusión y a las 32 semanas de gestación. La hipertensión matinal y el comportamiento non-dipper se evaluaron por MAPA. Resultados: La edad, la glucemia y la presión diurna y nocturna por MAPA fueron similares en el examen basal en ambos grupos. Por el contrario, las pacientes con HGB presentaron valores significativamente superiores de hipertensión matinal y comportamiento non-dipper, lo cual se asoció en forma independiente con hipertensión sostenida en el tercer trimestre del embarazo. Conclusiones: Las gestantes con HGB en la semana 20 de embarazo presentaron mayor hipertensión matinal y comportamiento non-dipper y evolucionaron con más frecuencia a hipertensión sostenida que el grupo control de normotensas.

Background: White coat syndrome (WCS) is common during pregnancy, although little is known about its clinical outcome during gestation. Morning hypertension and the non-dipper behavior, measured by ambulatory blood pressure monitoring (ABPM), are associated with greater risk of cardiovascular events. However, there are few studies during pregnancy. Objectives: The primary aim of the study was to evaluate morning hypertension and the non-dipper behavior in pregnant women with WCS versus a control normotensive group. A secondary objective was to evaluate whether WCS, morning hypertension and the non-dipper behavior in the second trimester of pregnancy were associated with greater hypertension in the third trimester. Methods: This prospective study included 95 primiparae in the 20th week of gestation, 50 with WCS and 45 as normotensive control group. Routine lab tests, office blood pressure and ABPM at inclusion and in the 32th week of gestation were recorded. Morning hypertension and the non-dipper behavior were evaluated by ABPM. Results: Age, and baseline blood glucose level and daytime and nighttime blood pressure by ABPM were similar in both groups. Conversely, patients presenting WCS had significantly higher values of morning hypertension and non-dipper behavior, which were independently associated with sustained hypertension in the third trimester of pregnancy. Conclusions: Pregnant women with WCS in the 20th week of gestation presented greater morning hypertension and non-dipper behavior and progressed more frequently to sustained hypertension than the control normotensive group.

A technique for chronically recording the EKG of the lymph hearts in the toad Bufo arenarum (Hensel)

A simple technique for recording the EKG of the posterior lymphatic hearts of the toad Bufo arenarum (Hensel) in free moving unanesthetized specimens is described. This technique permits long term chronic recordings in varied physiological and behavioral conditions whereas it overcomes some of the technical difficulties encountered in obtaining reliable recordings.