Giant left atrium is a condition characterized by an extreme enlargement of the left atrium with a diameter more than 80 mm and it is usually associated with long standing rheumatic mitral valve disease. We present a case of giant left atrium in a 47-year-old female patient who had a history of rheumatic heart disease, severe mitral disease, permanent atrial fibrillation and causing the compression of adjacent intra-thoracic structures. The patient underwent a successful mitral valve replacement with reduction of the enlarged sized left atrium with good outcomes.
Giant coronary artery aneurysms that occur in 0.5 to 1% of patients with Kawasaki disease can be fatal if associated with thrombosis. Some patients may show persistent inflammation and fever despite treatment with repeated doses of intravenous immunoglobulin (IVIG), steroids, and aspirin. This report describes an infant boy with resistant Kawasaki disease who presented with extensive coronary artery involvement and coronary thrombosis. His inflammation was not controlled with multiple doses of IVIG, parenteral and oral steroids, or high-dose aspirin, and he finally needed infliximab, a monoclonal antibody against tumor necrosis factor alpha.
Scimitar syndrome or Felsons veno-lobar syndrome is a very rare congenital disease characterized by a combination of cardiopulmonary abnormalities, including partial right-sided pulmonary venous drainage to the inferior vena cava, the inferior cavo-atrial junction, or low on the right atrium. We report the case of a 53-year-old female patient who presented with recent gradually worsening dyspnea. The diagnosis was suspected on the chest x-ray and confirmed on Cardiac echography andComputed Tomography scan that showed a wide collector gathering the three right superior pulmonary veins that joins the lower part of the superior vena cava, thus joining the right atrium while the right inferior pulmonary vein is drained into the inferior vena cava. The patient was treated surgically by performing a derivation of the right superior pulmonary venous collector to the left atrium with a tricuspid annuloplasty with a good outcome.
Von willbrand syndrome remains a rare pathology. Its etiologies and clinical manifestations are varied. This syndrome can be inherited or acquired. The acquired form is associated with various pathologies (Gastric, mechanical support system…) including cardiovascular diseases such as aortic stenosis, HCM, and Mitral regurgitation. Nevertheless, the association of acquired Willebrand syndrome with coronary pathology remains a rare association. In this literature review, we have made a review of the various articles addressing this subject. The management of ACS in patients with AvWS follows that of normal patients in terms of interventional procedure, however therapeutic drug management remains delicate given the hemorrhagic risk that these drugs present. The use of DAPT and heparin is possible while thrombolysis remains at very high risk for the patient. The use of desmopressin can be done with caution and also factor VIII and vWF concentrates have their place to reduce the risk of bleeding. Overall, the collaboration of the cardiologist and hematologist remains essential for better management of these patients.
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