Balloon pulmonary valvuloplasty in children: Jordanian experience

Aim: To describe retrospectively our experience of balloon pulmonary valvuloplasty [BPV] as a treatment for children with pulmonary valve stenosis in Queen Alia Heart Institute. Patients and methods: Over a 7-year period [1995-2001], pulmonary valvuloplasty was carried out in 68 patients [30 males, 38 females] their median age was 6 [range 1.2-14] years. 34 patients were a symptomatic, 24 had effort intolerance, and 10 had cyanosis with critical Pulmonary Stenosis [PS]. A balloon diameter 1.2-1.4 times the angiographic pulmonary valve annulus was used in our experience. BPV was considered successful by our criteria if the residual peak systolic transpulmonary pressure gradient was less than 25 mm Hg and the right ventricular /aortic pressure ratio was less than 0.6 by hemodynamic measurements. Patients were evaluated in the paediatric cardiology clinic biannually by 2D Doppler echocardiography 2-3 years following the BPV along with clinical examination and standard surface ECG. The procedure was successful, well tolerated and free of complications in 57[84%]. The median maximum instantaneous peak systolic pressure across the pulmonary valve as assessed by continuous wave Doppler echocardiography was 100 mm Hg [range 45-145 mm Hg] before the procedure; this gradient dropped to 26 mm Hg [18-82] [P value <0.01] within 24 hours after the procedure, 4 [6%] patients developed apnoea with bradycardia, another 4 [6%] patients needed another valvuloplasty and 3[4.4%] patients underwent surgical valvotomy. After 2-3 years follow up 2D-echo Doppler showed a median instantaneous gradient of 22 [12-40 mm Hg] [P<0,01]. 10 patients had immediate gradient more than 40 mm Hg, which dropped to less than 40 mm Hg during the follow up period. During follow-up and as assessed by Doppler echocardiography, pulmonary valve regurgitation was mild in 20 patients [29%] and moderate in 8 patients [12%] who had balloon annulus ratio more than 1.3:1. The mortality was 0%. The immediate and short-term results of percutaneous balloon pulmonary valvuloplasty in children are excellent. The gradient shows further decrease during follow-up due to regression of the infundibular muscular stenosis. We noticed that patients who developed moderate pulmonary valve regurgitation had higher balloon /annulus ratio of 1.3:1, than those patients who had smaller ratios. Failure of the procedure is attributed to dysplastic valves and these patients may require a second valvuloplasty or surgical approach

Report of two cases of scimitar syndrome: the classical one with subaortic membrane and the scimitar variant

Scimitar syndrome is characterized by an anomalous pulmonary vein draining into the inferior vena Cava, visible roentgenographically as a crescentic shadow of vascular density along the right border of the cardiac silhouette and dextroposition of the heart. We are reporting two cases of Scimitar sydrome; one being classical associated with subaortic membrane and the other a Scimitar variant. The clinical and anatomical spectrum for each case is described