Closure of coronary artery fistula in childhood: treatment techniques and long-term follow-up.

INTRODUCTION: Coronary artery fistula (CAF) is a rare congenital anomalous connection between the coronary arteries (CA) and a cardiac chamber or great vessel. Treatment options of symptomatic CAF consist of transcatheter or surgical closure. METHODS: Retrospective analysis of all patients with CAF diagnosed between 1993 and 2014 concerning treatment approaches and follow-up after closure. RESULTS: In a cohort of more than 25000 patients, 194 (<0.01 %) were diagnosed to have CAF. Median age at diagnosis was 6 months (0 day-18 years). Treatment was indicated in ten patients (5.2 %). Six patients (60 %) were treated by catheter interventional approach using Coils (three patients), Amplatzer Vascular Plugs (two patients) and Amplatzer Duct Occluder (one patient). One of these patients showed a significant residual shunt through the fistula 5 days after interventional closure, necessitating surgical removal of the device and closure of CAF. At a median follow-up of 7 (range 2-12) years, the remaining five patients showed successfully closed CAF without causing thrombosis of the CA. Control angiography in three patients showed persistent dilated CA. Surgical closure of CAF was performed in four (40 %) patients; in two as an isolated procedure and in the remaining two as a part of another congenital cardiac corrective procedure. CONCLUSIONS: CAF in paediatric cardiology patients is a very rare finding. Intervention in childhood is rarely needed; nevertheless, it is known that small fistulas may become relevant in adulthood. Transcatheter closure techniques are effective and are considered the treatment of choice, especially in isolated CAF.

Academic achievement and satisfaction in adolescents with CHD.

OBJECTIVES: To evaluate academic achievement and satisfaction in adolescents with CHD. Study design Questionnaires were sent to all adolescents, aged between 17 and 20 years with CHD, currently treated at our hospital (n=326) in order to assess the patients' education and satisfaction with their academic career. Results were compared with the official community statistics. RESULTS: A total of 207 patients completed the questionnaires (participation rate 63.5%), 113 boys and 94 girls; 50% had completed mandatory school at the highest, 37.3% at the middle, and 12.7% at the lowest educational level. The distribution in the general population was comparable: 57.6, 32.5, and 9.9%, respectively (p=0.8). Adolescents with severe CHD were less likely to attain a higher educational level than those with moderate or mild CHD (p=0.03 for school grades 7-9). None of the other examined medical or socio-demographic factors, such as socio-economic status, foreign language, severity of CHD, cyanosis, and open heart surgery, were found to be associated with lower educational attainment. After the mandatory 9 years of schooling, 21.4% (n=44) of the patients with CHD compared with 16.7% in the general population attended higher school levels heading towards university education (p=0.7). From the 165 patients who provided information on career satisfaction, 79% regarded their job or school situation as being their desired one without a difference for those with severe CHD. CONCLUSION: School education in Swiss adolescents with CHD is very similar to the normal population. In addition, the majority of adolescents are satisfied with their educational career. This fact may be due to the good educational support provided during schooling.

Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures.

OBJECTIVES: Neonates with hypoplastic left heart syndrome (HLHS) are at risk of high mortality and neurodevelopmental morbidity. As an alternative to Norwood-type stage I palliation, the hybrid procedure has been developed. It consists of bilateral pulmonary artery banding, catheter-based stenting of the arterial duct and balloon atrioseptostomy and delays open-heart surgery. Thus, it may be associated with a better outcome. The aim of this study was to determine the mortality and neurodevelopmental outcome in patients with HLHS and other univentricular heart (UVH) defects treated with hybrid or Norwood procedures. METHODS: Thirty-one children (18 males) with HLHS and other UVH defects undergoing Norwood or hybrid procedure between 2004 and 2008 were consecutively enrolled. Mortality and neurodevelopmental outcome at 1 year of age were determined. RESULTS: One-year mortality was 36% (31% in the hybrid vs. 39% in the Norwood group, P=0.71). Predictors of mortality were lower birth weight (P=0.02), older age at first procedure (P=0.02) and smaller size of ascending aorta (P=0.05). Overall, median psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scales of Infant Development II were lower than the norm of 100 [PDI 57 (49-99), P<0.001; MDI 91 (65-109), P=0.002]. No effect of surgical treatment on neurodevelopmental outcome was found. Predictors of impaired motor outcome were length of hospital stay (LOHS) (P=0.01), lower body weight at second procedure (P=0.004) and female sex (P=0.01). Predictors of impaired cognitive outcome were longer mechanical ventilation time (P=0.03), intensive care unit stay (P=0.04) and LOHS (P<0.001), respectively. CONCLUSIONS: Mortality at 1 year of age is comparable between patients undergoing hybrid and Norwood procedures. Early neurodevelopmental outcome is significantly impaired in patients with both HLHS and other UVH defects. Multicentre randomized studies are needed to determine the long-term neurodevelopmental outcome of children treated with the hybrid procedure.

Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia--usefulness of MR angiography in clinical routine.

BACKGROUND: To explore the role of contrast-enhanced magnetic resonance angiography (CE-MRA) in clinical routine for evaluating neonates with pulmonary atresia (PA) and to describe their pulmonary artery morphology and blood supply.CE-MRA studies of 15 neonates with PA (12 female; median weight: 2900 g) were retrospectively evaluated by two radiologists in consensus. Each study was judged to be either diagnostic or non-diagnostic depending on the potential to evaluate pulmonary artery morphology and pulmonary blood supply. In those cases where surgery or conventional angiocardiography was performed results were compared. RESULTS: CE-MRA was considered diagnostic in 87%. Pulmonary artery morphology was classified as "confluent with (n = 1) and without (n = 1) main pulmonary artery", "non-confluent" (n = 6) or "absent" (n = 7). Source of pulmonary blood supply was "a persistent arterial duct" (n = 12), "a direct" (n = 22) or "indirect (n = 9) aortopulmonary collateral artery (APCA)" or "an APCA from the ascending aorta" (n = 2). In no patient were there any additional findings at surgery or conventional angiocardiography which would have changed the therapeutic or surgical approach. CONCLUSIONS: CE-MRA is a useful diagnostic tool for the preoperative evaluation of the morphology of pulmonary arteries and blood supply in neonates with PA. In most cases diagnostic cardiac catheterization can be avoided.