Covered CP stents may be used as the therapy of choice in patients with complications after CoA repairs, whereas they provide a safe alternative to conventional stenting in patients with severe and complex CoA lesions or advanced age.

Objective-To determine the early results of balloon expandable stent implantation for aortic coarctation or recoarctation. Design-Prospective observational study. Setting-Two paediatric cardiology tertiary referral centres. Patients-17 patients, median age 17 years (range 4.4 to 45) and median weight 61 kg (17 to 92). Six had native aortic coarctation and 11 had aortic recoarctation; 14 had upper limb systolic hypertension. Of those with recoarctation, eight had had at least one previous balloon dilatation attempt and two of these patients also had further surgical interventions. Intervention-Balloon expandable Palmaz iliac stent implantation. Main outcome measures-Systolic pressures gradients, minimum aortic diameter, upper limb blood pressures, and incidence of aneurysm formation. Results-18 stents were implanted during 18 procedures in the 17 patients. Mean peak systolic pressure gradient fell from 26 mm Hg (95% confidence interval (CI), 21 to 31 mm Hg) before to 5 mm Hg (2 to 8 mm Hg) after stent implantation (p < 0.001), and mean minimum aortic diameter increased from 7 mm (95% CI, 6 to 8 mm) before to 11.3 mm (10 to 12.6 mm) after implantation (p < 0.001). Complications occurred in five patients (bleeding in two, stent migration in two, and aneurysm formation in one). Two patients remained borderline hypertensive and eight were receiving antihypertensive treatment at most recent assessment. Conclusions-Stent implantation for aortic recoarctation and native coarctation gives good immediate results. Careful follow up is necessary to evaluate complications and the long term eVect on blood pressure. (Heart 1999;82:600-606)

Modified Blalock-Taussig (B-T) shunt occlusion results in a sudden reduction of pulmonary vascular perfusion, causing dramatic saturation drop and cyanosis which pose a direct hazard to a child's life. The results of percutaneous local r-tpa infusion, balloon angioplasty and additionally stent implantation in obstructed modified B-T shunts were studied to assess their role as an alternative to a re-do surgery. We outline two pediatric centers' experience (period 2004-2008) regarding the effectiveness of various emergency treatment methods for occlusion or critical stenosis of systemic-to-pulmonary arterial shunts in 23 children. Local r-tpa infusion via catheter was performed in 12/23 patients, balloon angioplasty in 22/23 and additionally stent implantation in 3/23 children. Procedures were successful in 22/23 patients (96%), with an increase in arterial saturation [average: 30%; standard deviation (S.D.) 15%; Shapiro-Wilk test; dependent t-test-P<0.01]. Unrestricted contrast flow was achieved in 18 patients, reduced central flow in three and minimal flow in one child. Neither local nor systemic complications occurred. Our experience demonstrates the possibility of successful early shunt recanalization with the use of local thrombolytic therapy combined with the balloon angioplasty. The presence of old fixed thrombus with neointimal hypertrophy in the shunt constitutes an indication for endovascular stent implantation.

We inserted covered Cheatham-Platinum stents in 4 patients, ranging in age from 12 to 19 years, who weighed between 45 and 94 kg. All the patients had aortic coarctation, with surgical repair having been attempted previously in one, and with balloon dilation having been performed as the primary treatment in two, resulting in formation of aneurysms. The fourth patient had not received any treatment. The gradients were reduced from 10 to 40 mmHg before insertion of the stent to 0 to 5 mmHg after stenting. No complications were encountered. All the patients are well at an interval of 3 to 14 months after stenting.

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The relative decrease of the size of pulmonary arteries in the inter-stage period (between bidirectional Glenn anastomosis and Fontan completion) and after Fontan completion may indicate that pulmonary artery sizes should probably not bean absolute limiting factor in the decision on treatment of functionally UVH patients, especially at the stage of Fontan approach.

A b s t r a c tBackground: Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfavourable prognosis. Aim:To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004-2013. Methods:The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH -n = 23 (42%), PAH associated with systemic-to-pulmonary shunts -n = 17 (31%), and PAH after corrective cardiac surgery -n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy.Results: During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03).Conclusions: Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.

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