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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.

Surgical reconstruction of the right ventricular outflow tract (RVOT) with valved conduits in infants and children with congenital heart disease leads to re-intervention in later fife as the ensuing pulmonary regurgitation and stenosis of the degenerating conduit impacts negatively on right ventricular function. Percutaneous pulmonary valve implantation (PPVI) provides a safe alternative to early surgical re-intervention in these patients. We describe this procedure as performed on an 11-year-old boy. Difficulty may be experienced crossing the RVOT prior to PPVI. We describe several techniques that may be used to encourage the distal movement of the delivery system through the RVOT

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

Objective. To evaluate employment, career problems and job-related handicaps in adults with complex or mild congenital heart disease (CHD). Design. Cross-sectional. Method. Data were obtained from 76 patients with complex and 80 with mild CHD who were known to the Department of Paediatric Cardiology at the Leiden University Medical Center, the Netherlands. They were selected on the basis of diagnosis and year of birth (1968-1982). They completed a self-report questionnaire on education, employment and handicaps. A total of 224 patients were approached; the response from the groups was 88% and 58% respectively. Results. Participants included 83 women and 73 men. Their average age was 24.5 years (parameters: 17-32). In the study groups, 45 of 76 patients with complex CHD (59%), and 61 of 80 patients with mild CHD (76%) (p = 0.002) had paid work. Patients aged 25-29 years with complex disease had a statistically significantly lower rate of employment (64%) than the same age group in the general population (83%). Complex CHD and a lower level of education increased the risk of unemployment (odds ratios 4.8 (99% CI: 1.2-19.6) and 4.7 (99% CI: 1.3-17.2), respectively). Of the patients with complex CHD, 55% (42/76) experienced disease-related career problems, in contrast to 1% (1/80) of the patients with mild disease. Both CHD groups showed more job-related handicaps in the area of mobility than the reference group in the general population (p = 0.002). In the mild CHD group, this could be attributed to other, non-cardiac diseases. Conclusion. Patients with complex CHD had a lower level of job participation, on comparison with patients with mild CHD and their peers in the general population. Many experienced career problems or mobility problems at work. These problems may be prevented or limited by career counselling focussing on physical abilities and level of education.

In deze studie worden volwassenen met een milde of complexe aangeboren hartafwijking (17 tot 32 jaar) beschreven. Hun gezondheidsgerelateerde kwaliteit van leven (gemeten met de taaqol) en subjectieve gezondheidstoestand (gemeten met de sf-36) werden vergeleken met die van de algemene populatie. Tevens werd de relatie tussen deze uitkomstmaten en objectieve gezondheidstoestand bepaald. Deze gegevens kunnen gebruikt worden om ouders van patiënten beter voor te lichten. De gezondheidsgerelateerde kwaliteit van leven en subjectieve gezondheidstoestand van de groep volwassenen met een mild defect (n = 80) verschilden niet van die van de algemene populatie. Echter, de gezondheidsgerelateerde kwaliteit van leven van de patiënten met een complexe afwijking (n = 76) was significant slechter dan die in de algemene populatie voor de domeinen grove motoriek en vitaliteit (p < 0,01). Deze patiënten hadden tevens een significant slechtere subjectieve gezondheidstoestand dan de algemene populatie voor de domeinen fysiek functioneren, fysieke rolfunctie, vitaliteit en algemene gezondheidsperceptie (p < 0,01). Correlaties tussen de gezondheidsgerela-teerde kwaliteit van leven en subjectieve gezondheidstoestand enerzijds en de objectieve gezondheidstoestand anderzijds waren laag. Concluderend hebben volwassenen met een milde aangeboren hartafwijking een generieke kwaliteit van leven vergelijkbaar met de algemene bevolking. Speciale aandacht is echter nodig voor de gezondheidsgerelateerde kwaliteit van leven (en dan vooral de beleving van de fysieke aspecten) van de patiënten met een complexe afwijking. De relatie tussen kwaliteit van leven en de objectieve gezondheidstoestand is laag. Daarom is het bij evaluaties van kwaliteit van leven van belang specifieke kwaliteit-van-levenvragenlijsten (zoals de taaqol) te gebruiken.

OBJECTIVE: Evaluation of the first results in the Netherlands of percutaneous and transvenous closure of an ASD II in children with an Amplatzer Septal Occluder (ASO). DESIGN: Prospective. METHOD: Data were collected from children with an ASD II prior to, during and up to 24 months after the insertion of an ASO during heart catheterisation in Leiden University Hospital, the Netherlands. RESULTS: Between 1 January 1998 and 29 February 2000, 28 patients (12 girls, 16 boys; mean age: 74 months (range: 15-198 months)) underwent heart catheterisation to close an ASD II with an ASO. In 26 patients an ASO could be placed without significant complications. The size of the device varied from 9-34 mm (median 16 mm). In one patient ASD closure was not attempted because of multiple ASDs. In another patient the procedure was stopped after air embolism into the coronary arteries had occurred during preparation of ASO implantation. In 23/26 patients with an implanted ASO, no residual shunt was present after 24 hours. One child, in whom the defect was found to be closed after 24 hours and after three weeks, returned abroad and was lost to follow-up. After one year all defects (n = 22) were completely closed. CONCLUSION: Percutaneous transvenous closure of an ASD II with an ASO was possible, was not associated with any significant complications and had a high success rate, even in relatively young children with large defects

Objective: To discuss the need for continuing cardiac surveillance in adults with hemodynamically insignificant congenital cardiac disease. Methods: In 82 patients with mild congenital cardiac malformations, aged from 17 to 32 years, we investigated the subjective health status, the health-related quality of life, any difficulties encountered in daily life, the regularity of follow-up, current diagnosis, and antibiotic prophylaxis. Results: The subjective health status, and the health-related quality of life, did not differ from those of the general population. Nevertheless, patients experienced unnecessary difficulties with choice of sport, obtaining insurance cover, and education. After clinical re-evaluation, diagnosis and antibiotic regimes had to be changed in 9 patients (11%). Conclusions: Patients with a mild congenital cardiac malformation consider themselves to be in good health. So as to fine tune the diagnosis, and update the information supplied to the patients, we suggest that at least a cardiological assessment be carried out at the age of 16 to 18 years. In this way, patients might be protected from unnecessary difficulties, such as restrictions for sport or the charging of unjustifiably high rates for insurance. © Greenwich Medical Media Ltd.

The present study examined the social impediments experienced by young adults with minor congenital heart disease (CHD) and compared the health status and health-related quality of life of these CHD-patients with a matched group (age, sex) from the general population. Young adults with minor CHD, not in need of surgical correction, were sent a questionnaire. Results showed that a substantial part of the CHD-patients experienced social impediments, i.e. at school (19%), during free time (15%), in choosing (13%) or performing a job (9%), with medical examinations for job/insurance (19%), and taking out a life insurance policy (8%). Those CHD-patients that experienced social impediments reported a health status and health-related quality of life comparable with the general population. Remarkably, CHD-patients without social impediments reported a better health-related quality of life for 6 of the 12 scales, when compared with the general population. This last result might be explained by coping mechanisms resulting in a higher appreciation of health status which is expressed in a higher health related quality of life.

BACKGROUND: Abnormal atrial automaticity in young patients with structurally normal hearts is often located around the pulmonary veins and in sinus venosus-related parts of the right atrium. We hypothesize that these ectopic pacemaker sites correspond to areas of embryonic myocardium with an early phenotypic differentiation, as indicated by differences in antigen expression during normal cardiac development. METHODS AND RESULTS: In human embryos ranging in age from 42 to 54 days of gestation, the development of the cardiac conduction system was studied with the use of HNK-1 immunohistochemistry. HNK-1 stains the developing atrioventricular conduction system, ie, the bundle branches, His bundle, right atrioventricular ring, and retroaortic ring. In addition, the myocardium around the common pulmonary vein showed transient HNK-1 antigen expression. In the right atrium, 3 HNK-1-positive connections were demonstrated between the sinoatrial node and the right atrioventricular ring. An anterior tract through the septum spurium connects the sinoatrial node with the anterior right atrioventricular ring, and 2 posterior tracts connect the sinoatrial node with the posterior right atrioventricular ring through the right venous valve (future crista terminalis) and sinus septum, encircling the coronary sinus. The medioposterior part of the right atrioventricular ring connected to the His bundle and the medioanterior part form 2 node-like structures. CONCLUSIONS: In patients with abnormal atrial automaticity, the distribution of left and right atrial pacemaker foci correspond to areas of the embryonic myocardium that temporarily express the HNK-1 antigen