Evaluation of left ventricular systolic function in children with sickle cell anemia: contribution of 2D strain.

BACKGROUND: Cardiovascular involvement is not well studied in children with sickle cell disease. The aim of this study was to evaluate the echocardiographic parameters and to investigate speckle tracking echocardiography (STE) interest in detecting subclinical myocardial impairment of children with sickle cell disease. METHODS: The study was directed in the echocardiographic laboratory in the military hospital of Tunis between July 2018 and December 2018. 30 patients with sickle cell anemia (SCA) and 30 controls were compared. The echocardiographic measurements were indexed according to body surface. Cardiac output, left ventricular ejection fraction, wall thickness, as well as LV 2-D longitudinal systolic strain were assessed. RESULTS: The SCA Group included 30 patients (11.8 ± 2yrs, sex ratio: 1.31) with homozygous SCA and the C Group included 30 healthy controls (12.7 ± 1,2yrs, sex ratio: 1.27). According to the findings, SCA Group showed significantly larger LV diameter (36.2±2.5mm/m2 vs 29.3±1.3mm/m2, p=0.005). SCA Group also showed lower LV ejection fraction (62%±0.5 vs 65%±5, p=0.001). No significant difference was observed for cardiac output (p=0.4). Otherwise, two-dimensional longitudinal strain of LV was higher in SCA group (-21%±3.07 vs -25%±2.98; p<0.01). CONCLUSIONS: Our study highlights several cardiac abnormalities in children with SCA, which could represent a marker of disease severity and point out the importance of the cardiologic screening of these patients.

Sudden death complicating a coronary arteritis: polyarteritis nodosa (case report).

Coronary artery aneurysms are uncommon, are usually associated with atherosclerosis, and rarely involve all three major coronary arteries. Data on the optimal choice of acute myocardial infarction (AMI)´s revascularization in the context of polyarteritis nodosa (PAN) is limited to case reports and is still an open question. The present report describes a rare case of a young male patient followed for PAN presenting with acute myocardial infarction (AMI). Coronary angiography revealed multiple severe aneurysmal and stenotic changes. Based on clinical feature and angiographic findings, it was strongly suspected that the AMI was a complication of his vasculitis. This case indicates that coronary artery involvement should be carefully monitored during the chronic phase of PAN. The pathophysiology of AMI in PAN patients should be kept in mind and the interventional approach must be performed according to the angiographic findings to avoid complications.

Reoperation after modified Björk procedure for tricuspid atresia.

Rarely performed nowadays, the Björk procedure is an alternative to the Fontan palliation for biventricular repair in patients with tricuspid atresia. The right atrium to right ventricle conduit failure leads to serious long-term complications. The management of such patients at high surgical risk remains challenging. We report a successful reoperation in a young adult 25 years after the modified Björk intervention, who presented with severely narrowed and calcified valved conduit.

Cardiac tamponade: an uncommon presenting feature of systemic lupus erythematosus (a case-based review).

Although pericarditis is the most prevalent cardiac involvement in systemic lupus erythematosus (SLE), cardiac tamponade is extremely infrequent notably as the first manifestation of the disease. Here we report the case of a 22-year-old woman presenting with cardiac tamponade as the initial presentation of SLE.

Intercostal lung herniation secondary to thoracotomy: a case report.

Intercostal lung herniation is defined as a protrusion of the lung parenchyma through a defect in the intercostal muscles between adjacent ribs. The authors report a case of intercostal pulmonary hernia in a 45-year-old male patient, with smoking habit (30 packs-year), presented to the emergency department with dyspnea. He had the history of pulmonary emphysema complicated with a total right pneumothorax in 2015 treated by mini-thoracotomy with bullectomy and pleural abrasion. In 2019, he was admitted to hospital for left chest pain. The computed tomography (CT) scan of the chest revealed a bilateral emphysema with intercostal lung hernia through the fourth intercostal space the patient underwent, a left thoracotomy with repair of the intercostal muscle defect. He was discharged from hospital free of complications.

Particularities of neonatal isthmic aortic coarctation (A Tunisian study).

METHODS: This is a retrospective study including 47 patients with isthmic coarctation (CoA) diagnosed in ante et postnatal périod, hospitalized in pediatric cardiology departement of la Rabta Hospital-Tunisia during the period from 2000 to 2017. RESULTS: They were 36 girls and 11 boys with an average age of 14 days. The diagnosis of CoA was suspected during the anténatalperiod in eight cases. In postnatal period heart failure was observed in (38.5%), abolition of femoral pulse (74%), a tension asymetry was found in all patients. Antenatal echocardiography suspected indicators of fetal coarctation especially ventriculo-arterial asymmetryechocardiography confirmed the diagnosis of CoA ,The ejection fraction was impaired in 31% of the cases.The abnormalities associated with coarctation were dominated by the patent ductus arteriosus (68%), atrial septal defect (55.5%), aortic hypoplasia, bicuspidia in respectively in 34% and 31% coarctation syndrome in (23.4%). Prostaglandin wasneccessary in (89.3%). forty two patients were operated with good immédiaterésults. the early postoperative mortality was 12.5%. In the long term, we deplored two late deaths, six cases of recoarctation with a follow-up of 14 months treated by percutaneous angioplasty with a result considered good in all cases. CONCLUSION: Coarctation of the aorta in neonates is a special form of aorticcoarctaion that differs from the child and adult forms in clinical, echocardiographic, and therapeutic characteristics . His clinical diagnosis must be early, if possible in antenatal , to asssure optimal management.

Contribution of cardiac magnetic resonance imaging in the diagnosis of acute coronary syndrome with normal coronary angiography.

Background - Acute coronary syndrome with high level of troponin is a common pattern for emergency consultation. In 10% of cases, coronary angiography concluded that there were no significant coronary lesions. The contribution of cardiac magnetic resonance imaging (MRI) in the etiological investigation is increasing in these conditions. Aim - We analyzed the diagnostic value of cardiac MRI in case of acute coronary syndromes with elevated troponin and normal coronary angiography. Methods - It's a retrospective analytical study including 31 patients presenting with acute coronary syndrome with positive troponins and normal coronary angiography. All these patients underwent cardiac MRI. Results - The average age was 44.94 years. Cardiovascular risk factors were present in 38.70%. The average level of troponin was 4.85 ng/ml. Modification in the ST segment was noted in 87.1% of which 51.6% had ST elevated segment. Cardiac MRI was performed in the average of 8 days. MRI has contributed to the diagnosis in 77.4%: a myocardial infarction (MI) with no significant coronary lesions in 38.7% of cases, myocarditis in 29% of cases, Tako-Tsubo syndrome in 6.5% of cases and apical HCM in 3.2% of cases. MRI was normal in 22.6% of cases. Conclusions - The contribution of cardiac MRI is growing in the diagnostic management of patients with chest pain, elevated level of troponin and normal coronary angiography. The differential diagnoses have discriminating characteristics in MRI, allowing their identification with excellent diagnostic accuracy. The two main etiologies are myocardial necrosis and myocarditis.

Descriptive epidemiology of childhood central nervous system tumours in Tunisia. experience of a single institution over a 15-year period (1990-2004).

INTRODUCTION: Central nervous system tumours represent 20% of all childhood cancers, and are the second most common group of neoplasms after leukaemias. OBJECTIVE: To describe epidemiological characteristics of central nervous system tumours in a paediatric Tunisian population. PATIENTS AND METHODS: A retrospective study of 492 childhood central nervous system tumours operated between 1990 and 2004 was undertaken. We investigated the age-related location, gender distribution and the histology of all tumours, and adopted the latest WHO classification (2007) in grouping all the tumours. RESULTS: There were 488 primary and 4 secondary tumours; 426 (86.6%) were intracranial and 66 (13.4%) were intraspinal. Of the 426 intracranial tumours, 214 (50.24%) were supratentorial and 212 (49.76%) were infratentorial. The median age at diagnosis was 8 years, with a male:female ratio of 1.14:1. Low-grade tumours (WHO I/II) constituted 67.3% of all lesions and the rest (32.7%) were high-grade tumours (WHO III/IV). The most common tumour found in our series was astrocytoma (38%), followed by medulloblastoma (16.2%), then ependymoma (6.9%), cystic tumours (6.3%) and craniopharyngioma (5.3%). The overall 5-year survival rate was 45% with a mean follow-up period of 36 months. CONCLUSION: In our patient population, the incidence and distribution of central nervous system tumours were similar to those reported in literature. Overall survival rates varied according to tumour location and histopathology.