Une double discordance cardiaque complexe décompensée, à propos d'un cas et revue de la littérature / A double cardiac discordance decompensated, about a case and review of the literature

La double discordance est une cardiopathie congénitale complexe extrêmement rare, elle se caractérise par une discordance auriculo-ventriculaire suivie d'une autre discordance ventriculo-artérielle. Son diagnostic est posé généralement à l'âge adulte par des examens d'imagerie. Son pronostic dépend essentiellement des autres malformations congénitales cardiaques associées, des arythmies et des troubles de la conduction, ainsi que de la fonction systolique du ventricule droit en position systémique. Nous rapportons le cas d'un patient âgé de 23 ans, porteur d'une cardiopathie congénitale complexe cyanogène. Il s'agit d'une double discordance associée à une large communication interventriculaire sous aortique avec un shunt inversé et une hypoplasie de l'artère pulmonaire. Ce diagnostic a été confirmé par l'imagerie multimodale. Non opéré, le patient a vu son stade fonctionnel s'aggraver rapidement avec une dyspnée qui est passée d'un stade I à un stade III-IV de la New York Heart Association (NYHA), et apparition de signes hypoxiques et congestifs. A travers cette observation, nous avons précisé les caractéristiques anatomocliniques de cette cardiopathie congénitale complexe cyanogène, son exploration, ses options thérapeutiques, ainsi que le rôle péjoratif de son association avec d'autres malformations congénitales.

Double discordance is an extremely rare complex congenital heart disease; It is characterized by atrioventricular discordance followed by another ventriculo-arterial discordance. Its diagnosis is usually made in adulthood by imaging examinations. Its prognosis depends mainly on other associated congenital heart defects, arrhythmias and disorders of the conduction, as well as systolic function of the right ventricle in the systemic position. We report the case of a 23-year-old patient with a complex cyanogen heart disease.It is a double discordance associated to an interventricular communication with reverse shunt and an hypoplasia of the pulmonary artery.This Diagnosis was confirmed in multimodal imaging. Non-operated, the patient saw his functional status worsen rapidly with dyspnea which went from stage I to stage III-IV of the New York Heart Association (NYHA), and appearance of hypoxic and congestive signs. Through this observation, we have clarified the anatomoclinical characteristics of this complex congenital cyanogens heart disease, the necessary explorations and the different therapeutic options, as well as the pejorative role of its association with other congenital malformations.

Factors associated with global longitudinal strain decline in hypertensive patients with normal left ventricular ejection fraction.

Background Early detection of risk factors for left ventricular (LV) dysfunction may be useful in patients with high blood pressure (HBP). Methods Patient from an outpatient HBP clinic underwent a two-dimensional Doppler-coupled echocardiography with determination of LV global longitudinal strain (GLS) by speckle-tracking. Results Among 200 patients (mean age 61.7 ± 9.7 years), 155 were overweight, 93 had diabetes, 83 had dyslipidemia, and 109 had uncontrolled HBP. LV hypertrophy (LVH) was found in 136 patients (68%), including concentric ( n = 106) and eccentric ( n = 30) LVH. Diastolic dysfunction patterns were observed in 178 patients (89%), and increased filling pressures were observed in 37 patients (18.5%). GLS ranged from -25% to -11.6% (mean -16.9 ± 3.2%). Low GLS values (>-17%) were found in 91 patients (45.5%), 68 with and 23 without LVH. In univariate analysis, a reduced GLS was associated with HBP lasting for >10 years (odds ratio (OR) = 3.51, 95% confidence interval (CI) 1.73-7.09; p = 0.002), uncontrolled HBP (OR = 3.55, 95% CI 1.96-6.43; p < 0.0001), overweight (OR = 2.01, 95% CI 0.93-4.31; p = 0.0028), diabetes (OR = 2.21, 95% CI 1.25-3.90; p = 0.006), dyslipidemia (OR = 2.16, 95% CI 1.22-3.84; p = 0.008), renal failure (OR = 4.27, 95% CI 1.80-10.10; p = 0.001), an increased Cornell index (OR = 3.70, 95% CI 1.98-6.90; p < 0.0001), concentric LVH (OR = 9.26, 95% CI 2.62-32.73; p = 0.001), remodeling (OR = 8.51, 95% CI 2.18-33.23; p = 0.002), and filling pressures (OR = 7.1, 95% CI 2.9-17.3; p < 0.0001). In multivariable analysis, duration of HBP ( p = 0.038), uncontrolled BP ( p = 0.006), diabetes ( p = 0.023), LVH ( p = 0.001), and increased filling pressures ( p = 0.003) remained associated with GLS decline. Conclusion Early impairment of LV function, detected by a reduced GLS, is associated with long-lasting, uncontrolled HBP, overweight, related metabolic changes, and is more pronounced in patients with LVH.

Low SOD activity is associated with overproduction of peroxynitrite and nitric oxide in patients with acute coronary syndrome.

OBJECTIVE: The present study was undertaken to evaluate the variation of the oxidative/nitrosative stress status in a population of subjects; with acute coronary syndrome (ACS), and examine its possible implication in plaque rupture which is the main mechanism in the pathophysiology of ACS. PATIENTS AND METHODS: We made this study on 50 men with ACS and 50 age and sex matched healthy controls. Nitrosative/oxidative stress markers including; nitric oxide, superoxide anion levels, superoxide dismutase (SOD) activity and peroxynitrite levels were evaluated in blood samples of patients and controls. RESULTS: Compared with healthy subjects, coronary patients had significantly higher nitric oxide, peroxynitrite and superoxide anion concentrations in both plasma and erythrocytes associated to significant decrease of SOD activity. Erythrocytes peroxynitrite concentration was negatively correlated with the antioxidant enzyme activity (SOD). CONCLUSION: Our results show a significant accumulation of both intracellular and plasma pro-oxidants with a concomitant decrease in the SOD scavenging activity in ACS patients. Both seem to be associated with plaque rupture and ischemia observed in ACS.