Scimitar syndrome is a rare malformation defined as the partial or total anomalous pulmonary venous return of the right lung veins to the inferior vena cava just above or below the diaphragm. Severe forms of the disease are diagnosed in infancy and childhood. However, because of the mild form of the syndrome in adult patients, they remain asymptomatic and few cases are reported in the literature. We report two adults cases of this syndrome with one presenting with chest discomfort.
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.
Aortopulmonary window (APW) is an uncommon congenital cardiac malformation, accounting for 0.1% of all congenital cardiac diseases. It is a defect between the ascending aorta and the trunk of the pulmonary artery. Such abnormality may occur as an isolated lesion or it can be associated with other cardiac abnormalities in one third to one half of cases. Clinical and hemodynamic presentation of this condition depends on the size of the defect and on the associated lesions. It is usually fatal in infancy or childhood if untreated with the development of irreversible pulmonary hypertension. Once the diagnosis is made, surgery must be performed quickly to avoid irreversible pulmonary vascular disease. We report the case of a 5-month-old infant with APW who was referred to our center by respiratory symptoms and heart murmurs.
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