Left Ventricular Systolic Dysfunction and Cardiovascular Outcomes in Tetralogy of Fallot: Systematic Review and Meta-analysis

BackgroundAlthough there are robust data about the pathophysiology and prognostic implications of left ventricular (LV) systolic dysfunction in patients with acquired heart disease, similar prognostic data about LV systolic dysfunction are sparse in the tetralogy of Fallot (TOF) population. The purpose of this study was to perform a meta-analysis of all studies that assessed the relationship between LV ejection fraction (LVEF) and cardiovascular adverse events (CAEs) defined as death, aborted sudden death, or sustained ventricular tachycardia.MethodsWe used random-effects models to calculate hazard ratios (HRs) and 95% confidence intervals (CIs).ResultsOf the 1,809 citations, 7 studies with 2,854 patients (age 28 ± 4 years) were included. During 5.6 ± 3.4 years' follow-up, there were 82 deaths, 17 aborted sudden cardiac deaths, and 56 sustained ventricular tachycardia events. Overall, CAEs occurred in 5.1% (144 patients). As a continuous variable, LVEF was a predictor of CAE (HR 1.29, 95% CI, 1.09-1.53, P = 0.001) per 5% decrease in LVEF. Similarly, LVEF < 40% was also a predictor of CAE (HR 3.22, 95% CI, 2.16-4.80, P < 0.001).ConclusionsLV systolic dysfunction was an independent predictor of CAE, and we observed a 30% increase in the risk of CAE for every 5% decrease in LVEF, and a 3-fold increase in the risk of CAE in patients with LVEF <40% compared with other patients. These findings underscore the importance of incorporating LV systolic function in clinical risk stratification of patients with TOF and the need to explore new treatment options to address this problem.     

Uhthoff's symptom in multiple sclerosis

A case report is presented in which a patient's initial complaint is of blurred vision after exercise (Uhthoffs symptom). Visual acuity and colour vision were found to be reduced after exercise. Additional neurological signs included homonymous scotomata and delayed visual evoked and somatosensory responses. Magnetic resonance imaging demonstrated multiple abnormal lesions in the brain. The significance of this symptom and its relationship to multiple sclerosis are discussed.     

Treatment of ptosis in chronic progressive external ophthalmoplegia.

Seventeen patients with ptosis as a feature of chronic progressive external ophthalmoplegia were managed in accordance with a new protocol. An anterior approach levator advancement was performed on seven patients (13 lids) with more than 4 mm of levator function and a brow suspension procedure on eight patients (14 lids) with minimal levator function, in whom the frontalis muscle was relatively spared. Ptosis props were prescribed for two patients with a very weak orbicularis and poor lid closure. Six patients required long term lubricants, and one developed a postoperative corneal abscess associated with a poor Bell's phenomenon. Satisfactory elevation of the lids was achieved in 16 patients (25 lids).     

Increased Sensitivity to the Anticonvulsant Effect of Valproate in Aging BN/BiRij Rats

The aim of the present investigations was to study the influence of increasing age on the pharmacodynamics of valproate in BN/BiRij rats, applying a threshold for electrically induced localized seizure activity as a measure of the anticonvulsant effect. Seven groups of healthy male BN/BiRij rats were used, aged 3, 6, 12, 19, 25, 31, and 37 months. Individual plasma concentration versus anticonvulsant effect relationships were determined during a continuous intravenous infusion of sodium valproate at a rate of 5.5 mg/min/kg. The infusion was terminated when the anticonvulsant effect intensity had reached the maximum attainable level or at a total infusion time of three hours. A nonlinear relationship between valproate concentration and anticonvulsant effect intensity was observed with no maximal effect in the concentration range up to 1200 mg · L^–1. With increasing age a parallel shift in the concentration versus anticonvulsant effect relationships toward lower concentrations occurred. Thus increasing age appears to be associated with an increased sensitivity to the anticonvulsant effect of valproate.Suzanne Hovinga: Deceased January 30, 1991.     

Prevention of prematurity: Haguenau perinatal survey, 1971-1985

A preterm birth prevention program was instituted in France in the early 1970s. Its effectiveness has been assessed through a perinatal study in Haguenau. A relationship between prenatal care improvement and preterm birth rate decrease was noted, but a causal interpretation cannot be derived from such an observational study. However, some arguments do support this interpretation: no satisfactory alternative explanation (such as biases in pregnancy duration measurement, change in the composition of the population of pregnant women, or secular trend), a plausible causal pattern, and a dose-response relationship between prenatal care and preterm birth rate. The Haguenau study results can be applied to other French regions, but extrapolation to other countries would depend on their social, medical and cultural contexts.