Segmented coronary artery aneurysms and kawasaki disease

Kawasaki disease [KD] is an acute vasculitis syndrome of unknown etiology. It occurs in infants and young children, affecting mainly small and medium-sized arteries, particularly the coronary arteries. Generalized microvasculitis occurs in the first 10 days, and the inflammation persists in the walls of medium and small arteries, especially the coronary arteries, and changes to coronary artery aneurysms. We report the case of a 10-month-old girl referred to our center three months after the onset of disease due to the aneurysms of the coronary arteries. During the acute phase of her illness, she received 2 gr/kg intravenous gamma globulin; and after her referral to us, the patient was treated by antiaggregant doses of acetylsalicylic acid [ASA] [5 mg/kg] and Warfarin [1 mg/daily]. At three months' follow-up, the aneurysms still persisted in the echocardiogram

[ evaluation of late outcome of balloon pulmonary valvuloplasty in children]

Isolated pulmonary valve stenosis represents 8-10% of congenital cardiac anomalies. This study was performed to evaluate the late outcomes of Balloon Pulmonary Valvuloplasty [BPV] in children with residual infundibular stenosis. Seventy-eight patients underwent BPV in Rajaee Heart Center in Tehran Iran, from 2008-2010. The patients were divided into two groups: with and without infundibular stenosis. The group with infundibular stenosis was subdivided into two groups: with and without propranolol administration. Gradient measurement follow-ups by Doppler echocardiography were done on the first day and 1, 3, 6 and 12 months afterwards. Thirty five [44.9%] patients were male and 43 [55.1%] were female. The mean age of participants was 4.29 +/- 3.5 years and the mean weight 16.18 +/- 8.8 kg 33 patients [43%] did not show residual infundibular stenosis but 45 [57%] did so in the evaluations. There were no significant differences between the two groups regarding age, weight, sex, kind of balloon valvuplasty, balloon to body surface area ratio and pulmonary regurgitation [PR]. Propranolol was administered to 27 [60%] patients with residual stenosis for six months but 18 [40%] did not receive the medication. In both groups, the gradient significantly decreased immediately after BPV [P<0.0001]. In the propranolol group a constant decrease in residual gradient was seen. There was a significant relationship between balloon to body surface area ratio [387 +/- 94mm2/m[2]] with moderate to severe PR [P<0.015]. BPV is a safe and effective procedure to treat PS. Residual infundibular gradient after BPV decreases over time and propranolol helps decrease the gradient