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An 11-year-old girl, a 15-year-old boy and a 12-year-old girl all underwent percutaneous implantation of a Melody pulmonary valve prosthesis to replace a stenotic and insufficient homograft in the pulmonary artery. Preoperatively, 2 of the children suffered from fatigue and dyspnoea on exertion The homografts had been implanted between the ages of 1-2, to establish surgical continuity between the right ventricle and the pulmonary artery. The anomalies were tetralogy of Fallot, pulmonary atresia with intact ventricular septum and pulmonary atresia with a ventricular septum defect. Percutaneous pulmonary valve replacement was successful in all 3 patients. After implantation, right ventricular pressure decreased to 30% of systemic pressure and regurgitation was not observed. All patients were discharged in a good condition on the day after the implantation. Percutaneous pulmonary valve replacement is a promising technique with good short-term results. In selected patients this percutaneous technique can substitute or postpone the surgical replacement ofa stenotic or insufficient homograft.

Objective: To describe the results of surgical treatment of hypoplastic left-heart syndrome (HLHS) and HLHS-like disorders in the Amsterdam-Leiden Centre for Congenital Heart Disease, the Netherlands., Design: Retrospective, descriptive., Method: Data were collected on 43 neonates with HLHS or similar disorders who underwent surgical treatment between December 1999 and December 2005. HLHS was present in 37 patients and 6 had disorders similar to HLHS (unbalanced atrioventricular septal defect, truncus arteriosus with hypoplastic left ventricle, double inlet left ventricle). Surgery was performed in 3 steps: Norwood operation shortly after birth (n = 43), bidirectional cavopulmonary anastomosis a few months later (n = 30) and total cavopulmonary connection at the age of 2-3 years (n = 10). During the Norwood operation, the first 21 patients received a modified Blalock shunt (between the right brachiocephalic artery and pulmonary artery), whereas the following 22 patients received a Sano shunt (between the right ventricle and pulmonary artery)., Results: Of the 43 patients, 11 died: 7 within 30 days of the first operation, 2 between the first and second operation, and 2 between the second and third operation. Actuarial survival for the entire group is 74% (32/43). The mortality rate was lower with the Sano shunt (9%; 2/22) than with the modified Blalock shunt (43%; 9/21). Catheter interventions were necessary in 10 patients: 6 had balloon dilatation of the distal aortic arch and 4 had balloon dilatation/stent placement for narrowed pulmonary arteries. With a median follow-up of 22 months (range: 1-75), 2 patients had marked neurological side effects. All 32 surviving patients were in good clinical condition.

When circulatory failure occurs after paediatric cardiac surgery despite conventional therapy (inotropic medication, optimising pre- and afterload) mechanical assist devices can be used. Assist devices can be categorised in different ways: according to the type of pump used (centrifugal, axial flow, or pneumatic), according to univentricular or biventricular support and in relation to the presence of an oxygenator. Paediatric devices are non-implantable. Extra-corporeal membrane oxygenation (ECMO) consists of a roller pump and a membrane oxygenator and is a relatively simple means of biventricular cardiac support while respiratory support is provided at the same time. All other devices lack the oxygenator and thus provide only circulatory support. The main disadvantage of ECMO is that it can be used for a maximum of several weeks while modern pneumatic assist devices (MEDOS, Berlin Heart) can be used for several months. Thromboembolic events, bleeding and sepsis are the main complications of all assist devices. The use of circulatory assist devices can increase the chances of survival when severe cardio(pulmonary) failure occurs following surgical correction of congenital heart defects.

Objective: To evaluate the results of cardiosurgical treatment of children with Down's syndrome and a complete atrioventricular septal defect (cAVSD)., Design: Retrospective., Method: Data were collected from the records of all patients with Down's syndrome who had been subjected to primary corrective surgery for cAVSD in the period 1980-2003 in Leiden, The Netherlands. Exclusion criteria were: concomitant tetralogy of Fallot or interruption of the aortic arch and palliative banding of the pulmonary artery. Children with an abnormal shape of the ventricle making it impossible to correct biventricularly were not considered in the study. Data on mortality >30 days after the operation and on repeated surgery were included in the analysis if the duration of follow-up was at least 5 years., Results: The group comprised 148 children: 75 girls and 73 boys. The median age at time of surgery was 20 weeks (range: 6 weeks to 3.7 years) and showed a statistically significant decrease during the period under investigation. Of 4 children lost to clinical follow-up actual data could be obtained. The median duration offollow-up was 6 years and 7 months (range: 38 days to 23 years and 11 months). 28 children (19%) died. The mortality within 30 days after the operation decreased from 0-38% in the period 1980-1989 via 0-30% in the period 1990-1999 to 0% in 2000-2003. The percentage ofreoperations related to the correction ofcAVSD (14%; 14/98) did not seem to increase. Of the correction-related reoperations, 14% (2/14) were followed by a second reoperation. Conclusion. In the period 1980-2003, children with Down's syndrome and a cAVSD were corrected surgically at a younger and younger age. The early mortality decreased to 0% in the years 2000-2003. Echocardiography in the first weeks of life for all children with Down's syndrome makes it possible to diagnose those with a cAVSD early enough for surgical repair.

A 5-month-old female infant with Down syndrome underwent surgical repair of a complete atrioventricular septal defect and a patent ductus arteriosus. The postoperative course was complicated by life-threatening pulmonary hypertensive crises which did not respond to conventional vasodilating therapy. Nitric oxide inhalation resulted in rapid decrease in pulmonary artery pressure and improvement of haemodynamics and oxygenation without causing any untoward side effects. Inhalation of NO was continued over the next 4 days, with a maximum NO concentration of 20 ppm during a period of 12 hours. The subsequent postoperative course was uneventful and the patient was discharged from the hospital in good health on the 23rd postoperative day.