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      Aspects écho-cardiographiques au cours de la drépanocytose en Guadeloupe Translated title: Echocardiographic aspects in sickle cell disease in Guadeloupe

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          Abstract

          Introduction

          L'atteinte cardiovasculaire représente le facteur pronostique majeur de la drépanocytose.

          Méthodes

          C’était une étude transversale et descriptive dans le service de cardiologie du centre hospitalier et universitaire de Pointe-à-Pitre.

          Résultats

          Nous avons inclus 82 patients, 23 hommes (28%) et 59 femmes (72%), âgés en moyenne de 40,0±12 ans. On avait 39 patients porteurs d'hémoglobinose SS (49,4%) et 40 porteurs d'hémoglobinose SC (50,6%). Le taux d'hémoglobine de base était en moyenne de 9,8±1,8 g/dl et celui de l'hémoglobine fœtale était en moyenne de 5,5±5,3 g/dl. La fraction de raccourcissement était de 36±4,9% en moyenne, la FeVG moyenne était de 65±5,9%. On avait 68 patients avec un profil mitral normal (82,9%) et 14 patients avec un profil pseudo normal (17,1%). On avait une dilatation ventriculaire gauche chez 26 patients (31,7%), ventriculaire droite chez 14 patients (17.1%), une dilatation auriculaire gauche chez 36 patients (43.9%), auriculaire droite chez 11 patients (13.4%). On avait une hypertrophie ventriculaire gauche chez 13 patients soit 15.9%. L'hypertension artérielle pulmonaire a été retrouvée chez 43 patients soit 52,4%. Après régression logistique, les facteurs suivants ont été associés à la présence d'une HTAP: âge (p=0,0024), le taux hémoglobine de base (p=0,032), le taux d'hémoglobine fœtale (p=0,011), le type SS (p=0,040), la dilatation de l'oreillette gauche (p=0,0012) et la dilatation de ventricule droit (p=0,047). Après comparaison, L'hémoglobinose SS s’était avérée plus sévère.

          Conclusion

          La drépanocytose est une pathologie sévère dont le pronostic est aggravé par l'atteinte cardiaque.

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          Most cited references31

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          Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.

          The prevalence of pulmonary hypertension in adults with sickle cell disease, the mechanism of its development, and its prospective prognostic significance are unknown. We performed Doppler echocardiographic assessments of pulmonary-artery systolic pressure in 195 consecutive patients (82 men and 113 women; mean [+/-SD] age, 36+/-12 years). Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity of at least 2.5 m per second. Patients were followed for a mean of 18 months, and data were censored at the time of death or loss to follow-up. Doppler-defined pulmonary hypertension occurred in 32 percent of patients. Multiple logistic-regression analysis, with the use of the dichotomous variable of a tricuspid regurgitant jet velocity of less than 2.5 m per second or 2.5 m per second or more, identified a self-reported history of cardiovascular or renal complications, increased systolic blood pressure, high lactate dehydrogenase levels (a marker of hemolysis), high levels of alkaline phosphatase, and low transferrin levels as significant independent correlates of pulmonary hypertension. The fetal hemoglobin level, white-cell count, and platelet count and the use of hydroxyurea therapy were unrelated to pulmonary hypertension. A tricuspid regurgitant jet velocity of at least 2.5 m per second, as compared with a velocity of less than 2.5 m per second, was strongly associated with an increased risk of death (rate ratio, 10.1; 95 percent confidence interval, 2.2 to 47.0; P<0.001) and remained so after adjustment for other possible risk factors in a proportional-hazards regression model. Pulmonary hypertension, diagnosed by Doppler echocardiography, is common in adults with sickle cell disease. It appears to be a complication of chronic hemolysis, is resistant to hydroxyurea therapy, and confers a high risk of death. Therapeutic trials targeting this population of patients are indicated. Copyright 2004 Massachusetts Medical Society
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            Use and Limitations of E/e' to Assess Left Ventricular Filling Pressure by Echocardiography

            Measurement of left ventricular (LV) filling pressure is useful in decision making and prediction of outcomes in various cardiovascular diseases. Invasive cardiac catheterization has been the gold standard in LV filling pressure measurement, but carries the risk of complications and has a similar predictive value for clinical outcomes compared with non-invasive LV filling pressure estimation by echocardiography. A variety of echocardiographic measurement methods have been suggested to estimate LV filling pressure. The most frequently used method for this purpose is the ratio between early mitral inflow velocity and mitral annular early diastolic velocity (E/e'), which has become central in the guidelines for diastolic evaluation. This review will discuss the use the E/e' ratio in prediction of LV filling pressure and its potential pitfalls.
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              Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes.

              Screening for pulmonary hypertension (pHTN) has not yet become routine in sickle cell disease (SCD), despite clinical evidence of its high prevalence and associated mortality. Our objectives are to identify clinical conditions and laboratory findings predictive of/or associated with pHTN. One hundred twenty-five adult outpatients with Hb SS, SC, SOArab, Sbeta(0), or Sbeta(+) thalassemia, who underwent echocardiography and/or right heart catheterization due to cardiorespiratory symptoms, were studied. pHTN was identified in 36% (28/77) of SS/Sbeta(0) and in 25% (12/48) of SC/SOArab/Sbeta(+) patients studied. In SS/Sbeta(0) patients, pHTN was associated with low hemoglobin, low GFR, increasing age, no history of treatment with hydroxyurea and a history of leg ulcers, with trends for associations with higher total bilirubin, LDH levels, systolic systemic blood pressure, history of avascular necrosis, seizures, and cerebrovascular events. Twelve (40%) of the SS/Sbeta(0) patients with pHTN had >or= 1+ proteinuria. (P<0.039). The presence of proteinuria correlated with lower GFR and had a high positive predictive value (0.60) for pHTN in subjects with SS/Sbeta(0). The data also provided evidence that pHTN in this population is associated with right heart failure, with echocardiographic evidence of right ventricle enlargement and pericardial effusion. This study confirmed that even relatively mild elevations in pulmonary pressure are associated with high prospective mortality (hazard ratio: 15.9). We concluded that pHTN has a high prevalence in all Hb S related syndromes and is associated with increased mortality in SS/Sbeta(0). Kidney dysfunction, as indicated by proteinuria or decreased GFR, also represents sufficient reason to screen for pHTN.
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                Author and article information

                Journal
                Pan Afr Med J
                Pan Afr Med J
                PAMJ
                The Pan African Medical Journal
                The African Field Epidemiology Network
                1937-8688
                13 May 2014
                2014
                : 18
                : 45
                Affiliations
                [1 ]Service de Cardiologie et Médecine Interne, CHU de Brazzaville, Brazzaville, Congo
                [2 ]Centre caribéen de la drépanocytose, CHU de Pointe-à-Pitre/ Abymes, Pointe-à-Pitre/ Abymes, Guadeloupe
                [3 ]Service de Cardiologie, CHU de Pointe-à-Pitre/ Abymes, Pointe-à-Pitre/ Abymes, Guadeloupe
                Author notes
                [& ]Corresponding author: Ondze Kafata Louis Igor, Service de Cardiologie et Médecine Interne, Centre Hospitalier et Universitaire de Brazzaville, Congo
                Article
                PAMJ-18-45
                10.11604/pamj.2014.18.45.3820
                4215370
                7b05828a-9ec8-4923-a776-7cf2ebe2cca5
                © Louis Igor Ondze-Kafata et al.

                The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 03 January 2014
                : 07 May 2014
                Categories
                Research

                Medicine
                drépanocytose,échocardiographie,hypertension artérielle pulmonaire,sickle cell disease,echocardiography,pulmonary hypertension

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