Objective-To assess the possibility of maintaining ductal patency in neonates with complex pulmonary atresia by percutaneous implantation of balloon expandable stents.Patients-Two duct-dependent neonates with long segment pulmonary atresia, right sided aortic arch, and left sided arterial duct.Results-Stents with final diameter of 3 5 or 4 mm and initial length of 7 or 15 mm were successfully positioned in the arterial duct. Two stents were required in one child and four in the other in order to stent the entire length of the duct. After dependent congenital heart disease has not, however, been attempted. We report the successful maintenance of ductal patency by implantation of expandable stainless steel stents. The technical procedure (performed in each case under general anaesthesia), its potential complications, and its very considerable future promise in the palliation of duct dependent congenital heart disease are discussed. Patients and methods CASE 1A 2-6 kg boy presented with cyanosis and a soft continuous murmur when he was four hours old. He had an extensive cleft lip and palate and a bifid right thumb. Echocardiography and later cardiac catheterisation and angiography showed severe hypoplasia of the right ventricle and tricuspid valve with an intact ventricular septum, right ventricular sinusoids, long segment pulmonary atresia, small but confluent pulmonary arteries, a right sided aortic arch, and a left sided arterial duct arising at the origin of the left subclavian artery. He was maintained on an infusion of prostaglandin E and at the age of five days underwent a Waterston aortopulmonary anastomosis. The right pulmonary artery was found to be approximately 2-5 mm in diameter behind the ascending aorta. Postoperatively the child remained duct dependent. Angiography showed that the shunt was completely occluded. In view of the failed attempt at surgical palliation and with informed consent from the child's parents, we believed the alternative approach of stenting the arterial duct was justified.
Background-The technical aspects of ductal stenting have been reported, but little is known of the fate of the duct after stent implantation. Methods and Results-Nineteen patients underwent stent implantation to maintain ductal patency. Eight had hypoplastic left heart (HLH) syndrome, 10 had pulmonary atresia, and 1 had tricuspid atresia. Median survival with HLH was 57 (12 to 907) days. Stent implantation was successful in all cases of HLH, but there were no long-term survivors. Two well-palliated infants died at transplantation. Median survival with duct-dependant pulmonary flow was 183 (0 to 1687) days, with 3 patients well at latest follow-up (56, 55, and 9 months, respectively). There were 2 operative deaths due to ductal spasm and 4 late deaths, 1 due to duct thrombosis, 1 due to chronic lung disease, and 2 of unknown cause. Stent implantation failed in 4 of the 11 cases. Assessment of endothelialization was possible in 13 cases; the stent was partially covered in 3 and fully endothelialized in all 10 cases assessed Ͼ8 weeks after implantation. In patients stented for inadequate pulmonary flow, ductal intimal hyperplasia occurred by 9 months in all 3 survivors but responded to repeated dilation. Conclusions-Ductal stenting cannot be recommended. In patients with HLH, it provides only short-term palliation even when combined with pulmonary artery banding. With duct-dependent pulmonary blood flow, the procedure carries high risk, and duration of palliation is poor. In patients with bilateral ducts and absent central pulmonary arteries, good palliation may be achieved, but repeated angioplasty is necessary to counteract intimal hyperplasia. (Circulation. 1999;99:2621-2625.)
New data are emerging on the use of palivizumab as prophylaxis against infection with the respiratory syncytial virus in infants with congenital cardiac disease. Following a 4-year multicentre randomised trial, it was shown that prophylactic injections with palivizumab were effective and safe for such children. Prophylaxis consists of 5, monthly, intramuscular injections of palivizumab, at a dose of 15 mg/kg, given during the season for infection with the respiratory syncytial virus. Timing is at the discretion of the physician, depending on the onset of the season locally. It is suggested that, in the United Kingdom, this should be commenced in mid-September. To help clinicians to identify appropriate candidates for palivizumab, a working group of the British Paediatric Cardiac Association has developed recommendations.Infants, namely those under 1 year old, with congenital cardiac disease likely to benefit from prophylaxis include those with haemodynamically significant lesions, particularly increased pulmonary blood flow with or without cyanosis; pulmonary venous congestion, pulmonary hypertension or long-term pulmonary complications, residual haemodynamic abnormalities following medical or surgical intervention (patients who have undergone cardiopulmonary bypass should receive an injection as soon as they are medically stable), cardiomyopathy requiring treatment, and congenital cardiac disease likely to need hospital admission for medical or surgical intervention during the season of infection with the virus. Prophylaxis with palivizumab may also be indicated, at the discretion of the physician, in some children with complex cardiac disease over the age of 1 year. Children less likely to benefit from prophylaxis are those with haemodynamically insignificant disease, or those with lesions adequately corrected by medical or surgical intervention.
Objective-To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum.
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