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Aims and objectives To investigate parents’ experiences, coping ability and quality of life while monitoring their sick child with hypoplastic left heart syndrome at home. Background Interstage home monitoring for children with hypoplastic left heart syndrome reduces interstage mortality between Norwood stages I and II. Little is known about the psychosocial impact of interstage home monitoring. Design Prospective mixed-method study. Methods This study assessed the psychosocial impact on parents during interstage home monitoring. This contains for quantitative assessment the Short Form Health Survey questionnaire and the Impact of Family Scale administered one and five weeks following discharge before and after stage II. For qualitative assessment, semi-structured interviews focussing on the postdischarge coping strategies were conducted twice, five weeks after hospital discharge before and after stage II. Results Ten infants (eight males) with hypoplastic left heart syndrome (n = 7) or other types of univentricular heart malformations (n = 3), and their parents (nine mother/father two-parent households, one single mother) were included. There were no interstage deaths. Mental Health Composite Summary scores were low in both parents (mothers: 40·45 ± 9·07; fathers: 40·58 ± 9·69) and lowest for the item ‘vitality’ (mothers: 37·0 ± 19·46; fathers: 43·12 ± 25·9) before and after stage II. Impact of Family Scale values showed higher daily and social burdens for mothers. ‘Becoming a family’ was the most important task as coping strategy to equilibrate the fragile emotional balance. The parents judged interstage home monitoring as a protective intervention. Conclusions Although psychosocial burden before and after stage II remains high, becoming a family is an essential experience for parents and confirms their parenthood. Relevance to clinical practice Healthcare professionals must be aware of parents’ needs during this vulnerable interstage period and to provide psychosocial and nursing support.

AIMS: This study set out to assess indications, feasibility, complications, and clinical outcome of percutaneous transcatheter device closure of atrial septal defects (ASDs) in infants with a bodyweight below 10 kg. METHODS AND RESULTS: Retrospective single center chart and echocardiography review study from 8/2005-12/2013. Twenty-eight children with ASD (13 female) with a median age of 1.15 years (0.2-2.8) and a median weight of 7.2 kg (4.5-9.9) were analyzed. Indications for early ASD closure were failure to thrive (n = 15, 54%), bronchopulmonary dysplasia (BPD) with supplemental oxygen dependency (n = 7, 25%), and genetic syndromes with suspected pulmonary hypertension (n = 12, 43%). Device implantation was successful in all patients without any periprocedural mortality or major complication. Clinical outcome after a median follow-up period of 2.1 years (0.25-7.3) revealed no residual shunt and a significant decrease of right ventricular volume load. Patients with pulmonary hypertension experienced a significant reduction of pulmonary artery/RV pressure. Patients also showed decreased supplemental oxygen dependency and less cardiac medications, but no significant "catch-up growth" in those with failure to thrive. CONCLUSION: Interventional ASD closure in children weighing less than 10 kg can be performed without any additional major risks and shows a favorable outcome, especially in selected patients with significant non cardiac co-morbidities.

Objectives The aim of this study was to evaluate the effectiveness of balloon angioplasty (BAP) for recurrent aortic coarctation (ReCoA) in infants comparing simple and complex type of aortic arch pathology (Norwood I procedure). Background ReCoA is a known complication after cardiovascular surgery for coarctation of the aortic arch. Methods and Results In a single center case study, we analyzed 20 infants undergoing BAP for ReCoA comparing simple (n = 10) and complex type of aortic arch pathology (n = 10). At catherization diameter of ReCoA stenosis was 3.2 ± 0.7 mm (mean ± SD) with short localized (11/20) or long hypoplastic stenosis (9/20) before and 4.8 ± 1.2 mm after BAP (P

OBJECTIVES: The study aimed on the catheter interventional treatment of congenital portosystemic venous shunt (CPSVS) in childhood and the impact on vascular growth of hypoplastic portal veins. Clinical course and follow up of partial or complete closure of CPSVS are described. BACKGROUND: CPSVS is a very rare vascular malformation of the portal venous drainage resulting in potentially life threatening abdominal, pulmonary, cerebral, and cardiac complications. The anatomic type, clinical course, and diagnosis must be determined for optimal management. METHODS: Single centre case series. RESULTS: Eight (6 female) children with extrahepatic (n = 5) and intrahepatic (n = 3) CPSVS were diagnosed invasively by catheterization, including test balloon occlusion of the shunt and simultaneous retrograde angiography, and treated by catheter interventions (n = 5) with partial (n = 2) and complete (n = 3) occlusion of CPSVS at a median age of 3.9 years (range 0.7-21). Congenital heart disease (CHD) was the most frequent associated organ manifestation (n = 5) followed by mild to severe pulmonary arterial hypertension (n = 4), hepatopulmonary syndrome (n = 2), and portosystemic encephalopathy (n = 1). CHD was simple (n = 3) or complex type (n = 2). Three patients were untreated so far, because they were in excellent clinical condition at an age

Aim: To determine plasma B-type natriuretic peptide (BNP) levels in children with heart diseases before medical or surgical treatment for monitoring therapeutic efficacy in an observational prospective clinical trial at tertiary care centre. Methods: In 522 paediatric patients at an age of 6.4 ± 5.2 years (mean ± SD; range: 14 days–18 years) with congenital heart disease (CHD), cardiomyopathies (CMP) or pulmonary arterial hypertension (PAH), plasma BNP levels were evaluated before and under treatment. Results: Most types of heart disease are associated with increased mean plasma BNP levels before treatment, with highest values in children with CMP (BNP 6165 pg/mL in dilated CMP vs. 817 pg/mL in hypertrophic, vs. 1236 pg/mL in restrictive CMP, each p

PURPOSE: To evaluate whether regional cerebral oxygenation (rSO(2)) by near-infrared spectroscopy correlates with central venous (SvO(2)) or internal jugular (SjO(2)) oxygen saturation, and whether changes over time (Delta) in rSO(2) (DeltarSO(2)) predict changes in SvO(2) (DeltaSvO(2)) and SjO(2) (DeltaSjO(2)). METHODS: The rSO(2) values were measured using the INVOS 5100 cerebral oximeter in children undergoing interventional cardiac catheterization and were compared with the oxygen saturation of analysed central venous and internal jugular blood samples. Changes over time (Delta) were calculated as the difference between the values before and after catheter intervention for rSO(2).(DeltarSO(2)), SvO(2).(DeltaSvO(2)) and SjO(2).(DeltaSjO(2)). Simple regression and Bland-Altman analysis were performed. Data are presented as median (range). RESULTS: Sixty patients aged 4.3 (0.2-16.0) years were investigated. A closer correlation was found between rSO(2) and SvO(2) (r=0.728, P

Background: In this study, we evaluated the ratio of the cuff diameters of the Microcuff paediatric tracheal tube (Microcuff PET, Weinheim, Germany) to fluoroscopically measured internal transverse tracheal diameters in children from birth to adolescence. Methods: With Institutional Ethics Committee approval and parental consent, we measured the internal transverse tracheal diameters from fluoroscopy images in children undergoing cardiac catheterization requiring general anaesthesia with oro-tracheal intubation. Minimal tracheal sealing pressures were assessed at standardized respirator settings. Internal transverse tracheal diameters were compared with cuff diameters at 20 cmH2O cuff pressure. Linear regression analysis was employed to assess the correlation of tracheal diameters with age, height and weight, and to assess the correlation of the cuff/tracheal diameter ratio with sealing pressures. For all tests, P

The clinical diagnosis of Marfan syndrome in childhood is difficult, because symptoms may not have developed to their full expression until adulthood. The Ghent nosology for the diagnosis of Marfan syndrome classifies dural ectasia as a major diagnostic criterion. More than two thirds of adult patients with Marfan syndrome show dural ectasia, while the frequency in childhood is unknown. This prospective multicenter observational patient-control study was performed to identify pathologic changes of the lumbosacral spine in young patients with Marfan syndrome. Design: Prospective clinical trial, multicentric, cross-sectional. Setting: MRI of the lumbosacral spine. Patients: Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls. Outcome measures: Vertebral body diameter (VBD) from L1 to S1, dural sac diameter (DSD) from L1 to S1, dural sac ratio (DSR), qualitative assessment of the lumbosacral spine. Results: DSD and VBD in different age groups were higher in patients with proven or suspected Marfan syndrome than in healthy controls (DSD: L1, 6–8 years, P

A 3-year-old boy underwent interventional closure of an atrial septal defect using an Amplatzer septal occluder. After 4 weeks, an aortic sinus-to-left atrial fistula was detected by echocardiography in an asymptomatic child. The device was surgically explanted with fistula and atrial septal defect closure. Follow-up was uneventful.

Percutaneous transluminal renal angioplasty (PTRA) is a well-established method to treat renal artery stenosis (RAS) in children and adults. However, a significant number of stenoses might not be treated by interventional techniques due to the inability to dilate the RAS. Conventional balloon angioplasty with a high-pressure coronary angioplasty balloon at 20 atm was unable to dilate a significant RAS in a 12-year-old child with severe renovascular hypertension (RR 195/125 mm Hg). After using a 4 mm cutting balloon, we achieved wide patency of the renal artery and an instant normalization of blood pressure without further need of antihypertensive therapy. PTRA using the cutting balloon technique may offer an additional therapeutic option for selected patients in whom conventional balloon angioplasty was not able to dilate RAS.

Fracture of a 12 mm Palmaz stent after implantation in the left pulmonary artery for palliation of postoperative stenosis in a 9-year-old child is described. Successful management by implantation of a second Palmaz stent revealed immediate stabilization and no signs of significant restenosis during 1-year follow-up.