Publications by authors named "Hakim Kaouthar"

Double-chambered left ventricle (DCLV) is a rare congenital cardiac lesion. It is usually an incidental finding in children and young adults. Diagnosis during fetal life is exceedingly rare.

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Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. It is generally diagnosed and surgically repaired early in life, with good overall outcomes.

Case Presentation: We report the case of a patient incidentally diagnosed with paucisymptomatic TOF at the age of 56 years old, during investigations for carbon monoxide poisoning.

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Background Glycogen storage disease type III (GSDIII), due to a deficiency of glycogen debrancher enzyme (GDE), is particularly frequent in Tunisia. Phenotypic particularities of Tunisian patients remain unknown. Our aim was to study complications of GSDIII in a Tunisian population and to explore factors interfering with its course.

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A 04-year-old boy was referred to our institution with severe, progressive heart failure of 4-months duration associated with a persistent wide QRS tachycardia with left bundle branch block and severe left ventricular dysfunction. Because of incessant wide QRS tachycardia refractory to antiarrhythmic drugs, he was referred for electrophysiological study. The ECG was suggestive of VT arising from the right ventricle near the His area.

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Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small- and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies, related reports on peripheral arterial and aortic aneurysms are scarce. We report the occurrence of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28-month-old boy diagnosed with KD.

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Costello syndrome is a rare syndrome associated with de novo mutations in the HRAS gene. It is mostly revealed during in the first months of life by growth retardation, facial dysmorphic features, skin and cardiac abnormalities and subsequent cognitive deficit of varying severity. We report a case of Costello syndrome in a 3-month-old infant.

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Ebstein's disease with functional pulmonary atresia is a severe neonatal presentation of Ebstein's anomaly where the therapeutic management is typically based on the prescription of prostaglandins. The circular shunt is a serious "hemodynamic" complication which is often undiagnosed leading to the discontinuation of prostaglandins. We report a severe neonatal form of Ebstein's anomaly with hemodynamic deterioration relatted to a circular shunt.

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Cantrell's pentalogy is a very rare syndrome associating varying degrees of midline wall defects and congenital cardiac anomalies. It is characterized by a combination of five anomalies that are: a midline supra umbilical abdominal wall defect, a sternal defect, an anterior diaphragmatic defect, a diaphragmatic pericardial defect and a congenital intra cardiac defect. Ectopia cordis, defined as a developmental defect in which the heart is abnormally located partially or totally outside the thorax, is in some cases a part of this syndrome.

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Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented, related reports on peripheral arterial and aortic aneurysms are scarce. We report an original case of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28 months- old patient diagnosed with Kawasaki Disease.

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