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The high morbidity and mortality rate due to illegal abortions in South Africa necessitated the implementation of abortion legislation in February 1997. Abortion legislation stipulates that registered nurses who had undergone the proposed abortion care training — certified nurses — may carry out abortions within the first 12 weeks of pregnancy. Currently it seems that an inadequate number of nurses are being trained in the Western Cape to provide pregnant women with counselling, to perform abortions and/or refer problem cases. No real attempts have since been made by higher education institutions in the Western Cape to offer abortion care training for nurses. This case study explores the situation of certified nurses and the context in which they provide abortion care in different regions of the Western Cape. The sampling included a random, stratified (non-proportional) number of designated state health care facilities in the Western Cape, a non-probability purposive sampling of nurses who provided abortion care, a non-probability convenience sample of women who had received abortion care, and a non-probability purposive sampling of final-year pre-registration nursing students. Data was generated by means of questionnaires, a checklist and semi-structured interviews. The main findings of this study indicate that the necessary infrastructure required for legal abortion is in place. However, the ongoing shortage of trained health care practitioners hampers abortion care services. Deficiencies were identified in the existing provincial protocol as some of the guidelines were either not in use or had become obsolete. Certified midwives who had been trained by the regional offices of the Department of Health: Western Cape were skilled in carrying out the abortion procedure, but other aspects of abortion care mainly carried out by other categories of nurses required more attention. This article suggests a training framework that should provide focus for the development of a formal programme or programmes for the training of nurses in abortion care at higher education institutions in the Western Cape.

Objective. Few empirical studies have addressed the impact of trauma exposure and posttraumatic stress disorder (PTSD) on treatment utilisation and outcome in South African youth. This study was undertaken to document demographic, clinical, and treatment characteristics of child and adolescent inpatients with PTSD. Design. A retrospective chart study of all patients presenting to a child and adolescent inpatient unit was conducted between 1994-1996. For children and adolescents diagnosed with PTSD; demographic, diagnostic and treatment variables, including trauma type, family history, and delays in treatment seeking, were documented. Setting. Child and Adolescent Psychiatric Inpatient Unit, Tygerberg Hospital, Cape Town. Subjects. Children and adolescents (2 to 18 years) presenting to an inpatient unit (n=737). Results. 10.3% (n=76) met diagnostic criteria for PTSD. Gender differences were clearly evident: PTSD was six times more prevalent in girls (65 with PTSD were female and 11 were male); girls were most likely to have experienced rape or sexual abuse while boys were most likely to have witnessed a killing. Psychotherapy was the most common intervention for PTSD, followed by treatment with a tricyclic antidepressant. 97.4% of children and adolescents who were treated demonstrated significant improvement. Delays in seeking treatment and problems with the primary support group were highly prevalent. Conclusion. PTSD is a common disorder that is responsive to treatment with psychotherapy and/or tricyclic antidepressants in child and adolescent inpatients. These findings underscore the importance of early identification and treatment of childhood PTSD in mental health settings, in particular tertiary service institutions.

Background: Mechanical factors may cause bottlenecks in a Fontan circuit. Extracardiac conduits (ECC) are placed at a young age, but the materials do not allow growth. Restriction in ECC dimensions may deteriorate the function of the circuit., Aims: This study aimed to evaluate the feasibility and safety of stent expansion of an ECC to the nominal dimension at the time of implant and, if possible, beyond nominal., Methods: Retrospective, single-center observational review of all ECC Fontan patients who received a stent to expand a previously placed surgical conduit., Results: A total of 44 restrictive conduits were stented over a 14-year study period with a median of 11.8 (interquartile ranges [IQR]: 9.1-13.8) years after ECC placement. Cross-sectional areas were a median of 30% (IQR: 21-42) smaller than the originally placed ECC; there was no gradient in 23/44 patients and in 21/44, a minimal gradient of 1.3 ± 0.5 (range 1-3 mmHg). All conduits could be enlarged with a significant (p < 0.0001) increase in diameter from 13.6 ± 1.8 to 19.2 ± 1.2 mm, corresponding to a median cross-sectional area increase of 171% (IQR: 153-220). In three patients where the conduits were not contracted, expansion of between 127% and 165% was obtained. There were no conduit ruptures and only one minor complication., Conclusions: ECC in some Fontan patients become smaller than nominal over time, usually without overt symptoms. The dimensions of ECC's can be safely and significantly increased to nominal or even beyond employing stenting. It allows adjustment of ECC dimensions to compensate for somatic growth., (© 2022 Wiley Periodicals LLC.)

Aims: Percutaneous pulmonary valve implantation (PPVI) has proven good hemodynamic results. As infective endocarditis (IE) remains a potential complication with limited available clinical data, we reviewed our patient records to improve future strategies of IE prevention, diagnosis and treatment., Methods: Medical records of all patients diagnosed with Melody® valve IE according to the modified Duke criteria were retrospectively analyzed in three Belgian tertiary centers., Results: 23 IE episodes in 22 out of 240 patients were identified (incidence 2.4% / patient year) with a clear male predominance (86%). Median age at IE was 17.9 years (range 8.2-45.9 years) and median time from PPVI to IE was 2.4 years (range 0.7-8 years). Streptococcal species caused 10 infections (43%), followed by Staphylococcus aureus (n = 5, 22%). In 13/23 IE episodes a possible entry-point was identified (57%). IE was classified as definite in 15 (65%) and as possible in 8 (35%) cases due to limitations of imaging. Echocardiography visualized vegetations in only 10 patients. PET-CT showed positive FDG signals in 5/7 patients (71%) and intracardiac echocardiography a vegetation in 1/1 patient (100%). Eleven cases (48%) had a hemodynamically relevant pulmonary stenosis at IE presentation. Nine early and 6 late percutaneous or surgical re-interventions were performed. No IE related deaths occurred., Conclusions: IE after Melody® valve PPVI is associated with a relevant need of re-interventions. Communication to patients and physicians about risk factors is essential in prevention. The modified Duke criteria underperformed in diagnosing definite IE, but inclusion of new imaging modalities might improve diagnostic performance., Competing Interests: Conflict of interest statement Marc Gewillig is proctor for Numed, Medtronic and Edwards. All other authors and study collaborators have no conflict of interest to declare., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Introduction: Results and outcomes of ductus arteriosus stenting vary widely. The aim of this study was to determine whether ductus morphology is associated with different procedural outcome., Methods: Over an 18-year period, 123 patients presented with ductal dependent pulmonary blood flow. Results were retrospectively assessed based on radiographic anatomic features of the ductus arteriosus: Group 1: "straight" ductus arteriosus, typically seen in patients with Pulmonary atresia with intact septum (PA-IVS), Group 2: "intermediate" ductus arteriosus as seen in severe pulmonary stenosis (PS)-single ventricle, Group 3: "vertical" ductus arteriosus typically seen in patients with pulmonary atresia-ventricular septal defect, Group 4: ductus arteriosus arising from the aorta to a single lung, Group 5: ductus arteriosus arising from the innominate/subclavian artery to a single lung, Group 6: ductus arteriosus from innominate/subclavian artery to both lungs., Results: Ductal stenting (DS) was attempted in 98 patients with 99 ducts. Successful stenting was possible in 83 patients. Success of DS was significantly different among the groups (p = .04, F = 5.41). Groups 1, 4, and 5 were "easy" with good success while Groups 2, 3, and 6 were complex and demanding. There were two deaths (after 5 and 7 days, respectively) that could be ascribed to DS. Elective re-interventions were performed in 34 ductuses (40%). Fifty three percent (n = 44/83) of successful ductus stents proceeded to further surgery and 20 ducts closed spontaneously in asymptomatic patients over time., Conclusions: Ductus arteriosus morphology influences technique and determines complexity, safety, and final outcome of ductus arteriosus stenting., (© 2020 Wiley Periodicals, Inc.)

Background: To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein-losing enteropathy (PLE) or plastic bronchitis (PB)., Methods: Dilated lymph vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae. After flushing with 10% glucose solution, occlusion of hepatoduodenal or paratreacheal lymphatics was effected by injection of 1-4 cc mixture 4/1 of Lipiodol/n-butyl cyanoacrylate (n-BCA; Histoacryl)., Results: Seven patients with proven PLE were treated with periportal lymphatic embolization 10.7 (range: 6.6-13.5) years after the Fontan operation. The Fontan operation was performed at a median age of 3.7 (range: 2.9-5.7) years and PLE started a median of 3.1 (range: 0.9-4.7) years later. Five patients required a second procedure 2-8 months later. Complications were limited (spillage of glue in portal branch, transient cholangitis, and caustic duodenal bleeding). Six of seven patients reported significant improvement in quality of life and normalization of albumin levels after limited follow-up (p < .01). One patient (Fontan at 2.9 years; age 16.4 years) had PB for 2 years. Selective transthoracic cone-beam-directed puncture of left and right paratracheal lymphatics with n-BCA embolization of distal lymphatic fistulae resulted in lasting absence of tracheal casts (11 months)., Conclusions: Embolization of periportal/peritracheal lymphatics is a promising technique in Fontan patients with PLE/PB. Larger series are required to determine incidence and reasons of success/failure, with long-term results and effects on liver function., (© 2019 Wiley Periodicals, Inc.)

Background and Aims: Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices., Methods and Results: Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.6 months (range: 2.7-51.7). Right ventricle coronary dependent flow to the left ventricular myocardium was excluded. All four patients had dual perfusion with competitive flow from the RV through the sinusoids to the coronary arteries. Devices used were: Amplatzer vascular plug II of 10-16 mm; 27 coils (diameter 5-15 mm) in the oldest patient. Right ventricular angiography after cavity obliteration showed no more significant coronary perfusion through the sinusoids. There were no complications or deaths. Only minor and transient changes in the levels of troponin were observed. Coronary angiography at pre-Fontan evaluation showed no progress of coronary abnormalities in two patients., Conclusion: In selected patients with functionally single left ventricle, obliteration of the hypertensive RV cavity by percutaneous devices is safe and abolishes the systolic pressure wave in coronary sinusoids. When performed early, this may halt coronary damage and avoid excess mortality., (© 2019 Wiley Periodicals, Inc.)

Background: Infective endocarditis (IE) remains a diagnostic and therapeutic challenge associated with high morbidity and mortality. We evaluated the microbial profile and clinical manifestation of IE in children., Methods: A retrospective study examining pediatric IE cases treated between 2000 and 2017 at the Department of Pediatric Cardiology, KU Leuven, was conducted. Clinical presentation, treatment, complications, outcome of IE, underlying microorganisms and congenital heart defects were reviewed., Results: Fifty-three patients were diagnosed with IE. Overall, 19 patients (36%) required cardiac surgery. Seven patients (13%) died. Eighty-seven percent of patients had an underlying congenital cardiac defect. Eighteen (34%) children presented with prosthetic graft IE. A causative organism was found in 49 (92%) cases: viridans group streptococci were identified in 17 (32%), Staphylococcus aureus in 13 (25%) and coagulase-negative staphylococci in 11 (20%) children. Community-acquired (CA) IE increased significantly from 8 (33%) cases in 2000-2007 to 20 (74%) cases in 2008-2017 (P < 0.01). Even with viridans streptococci being significantly more prevalent in the CA group (P < 0.01), we did not observe an increase of streptococcal IE from 2008 to 2017. Seventeen (32%) patients presented with hospital-acquired IE during the first year of life with 14 (82%) children after surgery and a prevalence of coagulase-negative staphylococci (53%)., Conclusions: The incidence of pediatric IE was similar over the investigated time period with a shift toward CA IE. Streptococci and staphylococci accounted for the majority of cases in both periods. Awareness of IE and its prevention is crucial in patients after implantation of prosthetic grafts.

Aims: The aim of the study was to report up to 11 years of follow-up after Melody valve implantation in the pulmonary position., Methods and Results: This was a single-institution non-randomised prospective observational study of all Melody valves in the pulmonary position after discharge between 2006 and 2017 (n=188). Mean age was 19.4 years (SD 13.2). The indication was stenosis (45%), regurgitation (33%) and mixed (22%). Pre-stenting was performed in all except the initial four patients. In stenotic lesions the peak gradient was 36 mmHg PIG (SD 12.0) after 11 years and in regurgitant lesions the maximal regurgitation was 2/4. Stent fractures were observed in 8.6%; only one grade III fracture required redo PPVI. Surgical removal was carried out in seven (3.7%), redo PPVI in five (2.7%). Endocarditis was diagnosed in 19 (10.2%) patients at a median of 2.3 years (0.7-8.8) after Melody implantation. Three were surgically removed early because of persistent infection, 16 were sterilised; six required replacement (three surgical, three redo PPVI). There were no valve- or procedure-related deaths., Conclusions: The Melody valve shows a good preserved leaflet function up to 11 years after implantation. The main reason for graft failure was endocarditis, although in half of those patients no reintervention was needed. After pre-stenting, stent fractures led very exceptionally to reintervention.

Background: In adults, xenon has only minimal hemodynamic side effects when compared with other anesthetics. Moreover, in preclinical experiments, xenon has been demonstrated to possess cardio- and neuroprotective properties. Altogether, the favorable hemodynamic profile combined with its potential for organ-protection could render xenon an attractive option for anesthesia in children with cardiovascular compromise., Aims: The aim of this study was to explore safety and feasibility of sevoflurane-augmented xenon anesthesia in school-aged children and to assess early postoperative neurocognitive effects of xenon-sevoflurane and sevoflurane anesthesia when compared to a control group that did not have anesthesia., Methods: Forty children aged 4-12 years, suffering from congenital heart disease, undergoing diagnostic or interventional cardiac catheterization were randomized to either xenon-augmented sevoflurane anesthesia or sevoflurane alone. Safety was assessed by the incidence of intraprocedural hemodynamic instability and feasibility by anesthetic depth and respiratory profile. In addition, neurocognitive performance was assessed preoperatively, 2 hours after discharge from PACU and at 24 hours after anesthesia using the Amsterdam Neuropsychological Tasks system. A healthy control group of 22 age- and gender-matched children not exposed to anesthesia underwent an identical neurocognitive test battery, at comparable time intervals., Results: Overall hemodynamics did not differ between groups. Xenon-sevoflurane anesthesia resulted in decreased intraoperative ephedrine requirements (median [IQR]) (0.00 mg/kg [0.00-0.00] vs 0.00 mg/kg [0.00-0.01], P = 0.047). Only neurocognitive tests in the domain of alertness were significantly impaired 2 hours postoperatively in both anesthesia groups in comparison with the control group (alertness variability: P = 0.02, odds ratio 5.8), but recovered at 24 hours. For working memory, inhibition, cognitive flexibility, and motor coordination tasks, no significant interaction effects of anesthesia were found in the early postoperative period., Conclusion: In this pilot trial, xenon-augmented sevoflurane anesthesia in school-aged children was feasible, and associated with decreased ephedrine requirements. All children exposed to anesthesia showed impaired neurocognitive performance in the immediate postoperative period when compared to control children; however, without significant differences between both treatment groups., (© 2018 John Wiley & Sons Ltd.)

Background: The aim of this study was to assess the resistance to compression (stiffness) of frequently used stents for right ventricular outflow tract prestenting. In addition, to assess the corrosion potential when different types of stent alloys come into contact with each other., Method: Different stents were tested in vitro in various combinations at specialized metallurgic laboratories. A bench compression test was used to assess resistance to compression of singular and joined combinations of stents. Corrosion was evaluated by standardized electrochemical galvanic tests in physiological solutions at 37°C. Single stents and combinations of stents were evaluated over a period of 4-12 weeks., Results: Relative stiffness of the stents Optimus/Andrastent XXL/Intrastent LD Max/8zig Cheatham-Platinum, expressed as load per length to deform the stent for 1 mm at 22 mm was 100/104/161/190. Adding additional stents to a single stent significantly strengthened the joined couples (P < 0.001). The lowest galvanic corrosion rates (about 0.000001 mm/year) were observed for the joined CP-Andrastent, Andra-Sapien, and Andra-SapienXT. The corrosion rate for coupled CP-Sapien and CP-SapienXT was somewhat higher (about 0.000003 mm/year). The materials with the highest corrosion rates resulted in material losses of, respectively, 17 and 24 µg/year, which is negligible over a lifetime., Conclusion: Adding stents to a single stent significantly increases stiffness which will reduce the risk of metal fatigue failure. Corrosion of individual stents or stent combinations occurs, but is negligible over a human lifetime with low risk of biological effects. No mechanical integrity problems are thus expected as there is only 0.3% of the initial diameter of the struts of a stent that will be lost as a consequence of corrosion after 100 years., (© 2017 Wiley Periodicals, Inc.)

Objectives: To define the optimal timing for percutaneous pulmonary valve implantation (PPVI) in patients with severe pulmonary regurgitation (PR) after Fallot's Tetralogy (ToF) correction., Background: PPVI among the aforementioned patients is mainly driven by symptoms or by severe right ventricular (RV) dilatation/dysfunction. The optimal timing for PPVI is still disputed., Methods: Twenty patients [age 13.9 ± 9.2 years, (range 4.3-44.9), male 70%] with severe PR (≥3 grade) secondary to previous correction of ToF, underwent Melody valve (Medtronic, Minneapolis, MN) implantation, after a pre-stent placement. Full echocardiographic assessment (traditional and deformation analysis) and cardiovascular magnetic resonance evaluation were performed before and at 3 months after the intervention. 'Favorable remodelling' was considered the upper quartile of RV size decrease (>20% in 3 months)., Results: After PPVI, indexed RV effective stroke volume increased from 38.4 ± 9.5 to 51.4 ± 10.7 mL/m 2 , (P = 0.005), while RV end-diastolic volume and strain indices decreased (123.1 ± 24.1-101.5 ± 18.3 mL/m 2 , P = 0.005 and -23.5 ± 2.5 to -21 ± 2.5%, P = 0.002, respectively). After inserting pre-PPVI clinical, RV volumetric and deformation parameters in a multiple regression model, only time after last surgical correction causing PR remained as significant regressor of RV remodelling [R 2  = 0.60, beta = 0.387, 95%CI(0.07-0.7), P = 0.019]. Volume reduction and functional improvement were more pronounced in patients treated with PPVI earlier than 7 years after last RV outflow tract (RVOT) correction, reaching close-to-normal values., Conclusions: Early PPVI (<7 years after last RVOT operation) is associated with a more favorable RV reverse remodelling toward normal range and should be considered, before symptoms or RV damage become apparent. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Objective The aim of the study was to assess the feasibility of using commonly available catheterization laboratory equipment for radiofrequency perforation of the pulmonary valve in patients with pulmonary atresia and intact ventricular septum. Methods The system (off-label use for all items) is made up of a co-axial telescopic arrangement consisting of a 0.014" PT 2 ™ coronary guidewire, for insulation inside a 2.7-F microcatheter which has an inner lumen of 0.021". The microcatheter was passed via a standard 4-F right coronary catheter to just below the atretic pulmonary valve. Radiofrequency (RF) energy was delivered using a standard electrosurgical system. In vitro testing had been performed and indicated that 5-10 W for 2-5 s would be sufficient for valve perforation. Results Radiofrequency perforation was successfully performed in all (n = 5, 100%) patients at a median age of 3 days (range: 1-36) and weight 2.7 kg (range 2.3-3.0). In one patient the pericardium was entered during the initial attempt; the generator was put on coagulation mode during retrieval of the guidewire and no haemopericardium occurred. The pulmonary valve was dilated in all; in three patients (n = 3) the ductus arteriosus was stented during the same session. Conclusion Results of the study show that it is feasible to perforate the pulmonary valve safely using this system. Availability, simplicity and cost are noteworthy benefits.

Objectives: To determine the outcome of a bailout procedure using the right ventricle to re-assist the left ventricle in neonates after technically adequate coarctectomy but a failing borderline left heart., Methods: The surgical procedure was performed on bypass. A 'reversed' 6-mm surgical shunt was inserted between the pulmonary trunk and the descending aorta together with bilateral branch pulmonary artery banding., Results: Over a 10-year period, 89 neonates presented with coarctation and small left hearts. In 9 neonates, a hybrid procedure was performed at the outset. The remaining 80 underwent extended end-to-end coarctectomy. Two of these, despite adequate coarctectomy, developed retrograde cardiac failure with supra-systemic pulmonary hypertension, dilating right ventricles and progressive cardiogenic shock. The progressively dilating right ventricles inhibited left ventricular filling. Reversed surgical shunts were performed at 9 and 7 days post-coarctectomy. Both infants recovered rapidly and could be extubated after 4 and 7 days, respectively. Patient 1 proceeded to a univentricular repair and Patient 2 to a biventricular repair., Conclusions: Reversed surgical shunt with bilateral banding of the branch pulmonary arteries after neonatal coarctectomy can be successfully employed as a bailout procedure in cases where a borderline left heart with growth potential cannot tolerate a biventricular circulation. It may act as an acute life-saving measure as well as a bridge to later repair. If high risk for backward failure exists in a borderline left heart with catch-up growth potential, a primary hybrid procedure is probably a more elegant and predictable strategy., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Introduction: Data on long term function of the Melody valve are scarce. Patients and methods: single institution; results of percutaneous pulmonary valve implantation (PPVI) from 2006 to 2014. The function of the valved conduit was analyzed by Doppler echocardiography. Annual Chest X-ray after implant and permanent screening for events (e.g. Endocarditis)., Results: 112 Melody valves were implanted in 111 patients; mean age 19.3 years (4.5-81.6). No pre-stenting of the RVOT was performed (n = 4) at first. In the next 107 patients pre-stenting was always performed. In 82 patients 1 pre-stent, 18 patients 2, in 6 patients 3 stents and 1 patient 4 stents were used. The Melody stent was dilated up to 24 mm (n = 4), 22 mm (n = 72), 20 mm (n = 28) and 18 mm (n = 6). When stenotic, the Doppler gradient reduced from 67.0 mm Hg (SD 13.9) to 18.9 mm Hg (SD 10.4) (p < 0.001); pulmonary regurgitation (PR) was reduced from median 3.5/4 (range 0-4/4) to none or trivial (p < 0.001). There was no significant change in RVOT peak velocity at 5 years (p = 0.122) nor PR (p = 0.835). Type 1 stent fractures were observed in 1/4 non-pre-stented patients and in 5/107 pre-stented (p < 0.05). Endocarditis occurred in 8/112 valves; freedom of endocarditis was 85% at 5 years. In 2 patients early surgical replacement was necessary. Six were sterilized with antibiotic treatment; 2 patients required re-stenting and re-PPVI due to residual gradient., Conclusion: Adequate pre-stenting of the RVOT before PPVI nearly abolishes or delays stent fracture. Cusp function is well preserved in mid-term follow-up; endocarditis is a threat.

Background: Xenon has minimal haemodynamic side effects when compared to volatile or intravenous anaesthetics. Moreover, in in vitro and in animal experiments, xenon has been demonstrated to convey cardio- and neuroprotective effects. Neuroprotection could be advantageous in paediatric anaesthesia as there is growing concern, based on both laboratory studies and retrospective human clinical studies, that anaesthetics may trigger an injury in the developing brain, resulting in long-lasting neurodevelopmental consequences. Furthermore, xenon-mediated neuroprotection could help to prevent emergence delirium/agitation. Altogether, the beneficial haemodynamic profile combined with its putative organ-protective properties could render xenon an attractive option for anaesthesia of children undergoing cardiac catheterization., Methods/design: In a phase-II, mono-centre, prospective, single-blind, randomised, controlled study, we will test the hypothesis that the administration of 50% xenon as an adjuvant to general anaesthesia with sevoflurane in children undergoing elective cardiac catheterization is safe and feasible. Secondary aims include the evaluation of haemodynamic parameters during and after the procedure, emergence characteristics, and the analysis of peri-operative neuro-cognitive function. A total of 40 children ages 4 to 12 years will be recruited and randomised into two study groups, receiving either a combination of sevoflurane and xenon or sevoflurane alone., Discussion: Children undergoing diagnostic or interventional cardiac catheterization are a vulnerable patient population, one particularly at risk for intra-procedural haemodynamic instability. Xenon provides remarkable haemodynamic stability and potentially has cardio- and neuroprotective properties. Unfortunately, evidence is scarce on the use of xenon in the paediatric population. Our pilot study will therefore deliver important data required for prospective future clinical trials., Trial Registration: EudraCT: 2014-002510-23 (5 September 2014).

Background: Two siblings from consanguineous parents of Turkish descent presented with isolated dilated cardiomyopathy, leading to early death in infancy. The diagnosis of mitogenic cardiomyopathy was made histologically., Methods and Results: Linkage analysis combined with exome sequencing identified a homozygous deleterious mutation in the ALMS1 gene as the cause of this phenotype., Conclusions: Alström syndrome is characterized by a typically transient dilating cardiomyopathy in infancy, suggesting that mitogenic cardiomyopathy represents the extreme phenotype, resulting in demise before the other clinical symptoms become evident. This observation further illustrates the role of ALMS1 and cell cycle regulation., (Copyright © 2014. Published by Elsevier Masson SAS.)

Introduction: The management of small infants with tetralogy of Fallot (TOF) with pulmonary atresia (PA) and hypoplastic pulmonary arteries can be very challenging., Methods: In three small infants (weight range 2,200-3,600 g, pulmonary trunk 2.0-3.2 mm), initial palliation consisted of sternotomy, transventricular puncture of the right ventricular outflow tract and atretic pulmonary valve, followed by balloon dilation (n = 1) or stent deployment (n = 2) from the right ventricle into the pulmonary trunk (stent diameter 5-6 mm, length 16 mm)., Results: The procedure resulted in adequate palliation with good anterograde flow to the pulmonary arteries and near normal saturations in all three patients (>92%); there was no associated morbidity. Additional transvenous stenting was required in all patients because of progressive muscular infundibular stenosis after a median of 3 months. Two patients evolved to full repair at the age of 5 months and one patient with multiple hilar stenoses requires additional percutaneous procedures through the stented RV outflow tract., Conclusion: Transventricular balloon dilation and stenting of the RVOT through medial sternotomy as initial palliation strategy appears a safe and well tolerated alternative treatment in small infants with TOF with PA and a hypoplastic pulmonary trunk., (© 2013 Wiley Periodicals, Inc.)

Coarctation of the aorta includes a wide array of anatomical and pathophysiological variations that may cause important long-term morbidity and mortality. Percutaneous techniques, such as balloon dilation and stenting, allow clinicians to safely decrease or abolish most gradients along the aorta, albeit with limitations. Proper patient selection and interventional technique allow clinicians to obtain an adequate stretch or therapeutic tear of the vessel wall, but should avoid complications, such as an excessive tear, dissection, aneurysm formation or rupture. The interventional technique is tailored by patient characteristics such as age, size and growth potential, by characteristics of the lesion such as degree of narrowing, length, angulation(s) and by local regulations.

The prenatal diagnosis of fetal coarctation is still challenging. It is mainly suspected by ventricular disproportion (smaller left ventricle than right ventricle). The sensitivity of ventricular discrepancy is however moderate for the diagnosis of coarctation and there is a high false positive rate. Prenatal diagnosis of coarctation is important because the delivery can be arranged in a centre with a pediatric cardiac intensive careand this reduces postnatal complications and longterm morbidity. For many years the prenatal diagnosis of coarctation has been investigated to improve specificity and sensitivity by several of measurements. This article reviews all relevant articles from 2000 until 2011 searching pubmed and the reference list of interesting articles. An overview of specific measurements and techniques that can improve the diagnosis of coarctation has been made, such as the isthmus diameter, ductal diameter, isthmus/ductal ratio, z-scores derived from measurements of the distal aortic isthmus and arterial duct, the presence of a shelf andisthmal flow disturbance. Also 3-dimensional (3D) and 4-dimensional (4D) imaging with or without STIC has been -suggested to be used as newer techniques to improve diagnosis of coarctation in fetal life. Although more methods regarding prenatal diagnosis of coarctationare being investigated, the ultrasound specialist remains challenged to correctly diagnose this cardiac anomaly in prenatal life.

Objective: This study evaluated the efficiency and feasibility of dilatable bands in selected patients., Methods: Two types of dilatable handmade bands were retrospectively evaluated and divided into two groups: main pulmonary artery bands and bilateral branch pulmonary artery bands (hybrid stage I palliation). Stepwise balloon angioplasty (BA) was performed, increasing the diameter either to completely dilate with total release of the band, or in others, to partially dilate the bands in order to improve flow and/or saturation., Patients and Results: Balloon angioplasty was performed in 20 patients (median birth weight 2.9 kg, range: 1.3-4.5 kg). Main pulmonary artery: Partial dilation: Six patients: Large ventricular septal defects (VSDs) and complex lesions requiring additional surgery. Progressive dilation allowed postponement of surgery in four children and allowed percutaneous VSD closure in one. Complete dilation: Eight patients: Spontaneous restriction of VSDs occurred in six patients; the bands were subsequently percutaneously completely released after a median of 39 weeks (7-91 weeks). The median gradient decreased from 90 to 38 mmHg (p<0.0001). Bilateral branch pulmonary artery: An average 8.5% increase in saturations was achieved in five patients, and in one patient, a hybrid procedure with borderline left ventricle, complete dilation allowed successful percutaneous biventricular repair., Conclusions: Dilation of both main and bilateral branch pulmonary artery bands is possible, effective and safe. Dilatable main pulmonary artery bands allow for progressive dilation with postponement of surgery or complete release of the bands. Bilateral dilatable branch pulmonary bands offer palliative benefit, especially in hybrid cases where pulmonary blood flow may be limited by the bands before the ideal conditions for a stage II procedure exist., (Copyright 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

A newborn presented with severe aortic valve stenosis and a borderline hypoplastic left ventricle due to disproportionate left ventricular hypertrophy (maternal diabetes). The aortic valve was balloon dilated and the infant tolerated a biventricular circulation. However, severe retrograde pulmonary hypertension and mitral regurgitation developed, indicating that biventricular circulation was not possible at that stage. A hybrid approach with ductal stenting, atrial septostomy and bilateral dilatable pulmonary artery band placement was followed on day 25. This allowed the left ventricle several months to adapt to lower pressure and normoglycemic conditions. At re-evaluation after 8 months biventricular repair appeared possible: the ductus was closed with Amplatzer occluders and the pulmonary artery bands were opened up with bilateral balloon angioplasty of the dilatable bands. At the age of 3 years, the infant is doing well with a biventricular circulation and normal pulmonary artery pressure. The hybrid approach allowed adequate time (months) for careful consideration and acted as a bridge to biventricular repair in this infant.

Objective: To evaluate the efficiency and safety of clopidogrel treatment in children with heart disease., Study Design: We conducted single center retrospective chart review of children with heart disease at the University Hospital, Leuven, Belgium, in whom clopidogrel was used. The indication, dosage, duration of therapy, and adverse events were examined. Clinical efficacy was defined by an absence of thrombotic events., Results: 46 children were identified. The mean age of first clopidogrel dose was 4.9 +/- 4.1 years. The study dosage ranged from 0.1 to 0.7 mg/kg/day clopidogrel. Almost all patients received concomitant aspirin therapy. No thrombotic events developed. Skin bruising developed in almost every patient, suggesting that clopidogrel has an anti-platelet effect. 2 patients who were treated with concomitant warfarin had bleeding complications (severe epistaxis and gastrointestinal bleeding). Hematological abnormalities were documented in 1 patient who received clopidogrel for 1 year; they reversed with medication cessation., Conclusion: Clopidogrel therapy in a pediatric population appears to be relatively safe and effective; however, randomized, controlled prospective studies are needed to determine the true efficacy and safety of clopidogrel in children.

Objective: To assess safety and efficacy of transcatheter atrial septal defect (ASD) closure in small children., Background: Percutaneous closure of ASD is a well accepted alternative to surgery. Reported experience in small children remains, however, scarce., Methods: Fifty-two children underwent percutaneous ASD closure at a weight < or =15 kg, in four Belgian tertiary referral paediatric cardiology centers. Indication for treatment, device implantation rate, procedural details, complication rate, residual shunt on echocardiography, and impact of procedure on symptoms were retrospectively analyzed., Results: Mean age at procedure was 36 months (7-60), mean weight 13 kg (4.7-15). Associated cardiac lesions were seen in 21% of the patients and noncardiac in 32.7%. Most patients (69.2%) were symptomatic. Mean ASD size was 12 mm (range 5-20 mm). A device could be inserted in 49 patients (94%). No major complications occurred. Minor complications occurred in 8 patients (15.4%) including device embolization in 2, successfully treated with transcatheter retrieval and second device insertion. Mean follow-up reached 27 months (0.03-61.2). Residual shunt was absent, trivial, or small in 93% (n = 42) at latest follow-up. Clinical improvement was noted in 91.7% of the symptomatic patients. Minor complications were more frequent in the presence of large ASDs (>15 mm) but not in smaller babies (<10 kg)., Conclusion: Patients < or =15 kg requiring ASD closure form a special population with high incidence of associated anomalies and medical conditions. For this particular set of patients, percutaneous closure is a valid alternative. Asymptomatic or mildly symptomatic children should be treated at a later age., ((c) 2006 Wiley-Liss, Inc.)

Objective: To determine the safety, feasibility and effectiveness of stent expansion of hypoplastic aortic segments with pressure gradients in patients with arterial hypertension., Design: Non-randomised prospective clinical trial., Setting: Tertiary referral centre, congenital cardiac unit., Patient Selection: 20 consecutive patients (median age 14.5 years, range 11.6-38.8 years) with arterial hypertension and a hypoplastic segment of the aorta. Seventeen patients had successful previous arch interventions in a coarctation site., Interventions: Stent deployment in hypoplastic arch segments., Main Outcome Measures: Gradient across the aortic arch; complications early and during follow up; residual hypertension., Results: 23 stents were deployed: 13 in the cross and 10 in the isthmus. The mean gradient across the aortic arch decreased from 16 (SD 6) (median 17) to 3 (4) (median 1) mm Hg (p < 0.001). In a few patients a mild gradient persisted just distal to the left carotid artery due to residual orificial narrowing or acute angulation. No complications occurred during or after the procedure. During follow up of 2.2 years (range 0.2-4.8 years) arterial hypertension resolved in 10 patients and 10 required residual drug treatment with better control of blood pressures., Conclusions: Pressure loss due to residual hypoplastic aortic segments can be treated effectively and safely with stent expansion. Some patients remain mildly hypertensive and require additional drug treatment.

Aims: Stenting has become an established interventional cardiology procedure for congenital heart disease. Although most stent procedures are completed successfully, complications may occur. This multicentre study evaluated early complications after stenting in patients with congenital heart disease, including potential risk factors., Methods and Results: In this combined Dutch-Belgian retrospective study, 309 consecutive patients had undergone 366 catheterizations and received 464 stents in 13 different anatomical positions (418 sites). Seventy-two stenting-related complications (19%) occurred, of which 24 (5.7%) were major. Seven procedure-related deaths were documented (2.3%). Stent malpositioning and embolization were most common (7.7%). The use of non-premounted stents tended to be associated with higher complication rates. Centre inexperience with stenting and stenting of native vs. post-surgical stenosis tended to be associated with increased major complication rates., Conclusion: After stenting, complications are common for congenital heart disease. The vast diversity of stenotic sites combined with relatively small patient populations makes these procedures sensitive to complications. Combining operator experience may reduce the risks of stenting in congenital heart disease. The availability of premounted stents for greater vessel diameters will likely reduce incidences of stent migration and embolization.

Introduction: Although summer and sports camps for children with congenital heart disease are organized in many countries and regions, empirical data on the effects of such camps is limited., Objectives: The aim of the present study was to investigate changes in the perceived health status and habitual physical activities in children attending a special sports camp., Materials and Methods: In this longitudinal study, 25 children with congenital heart disease who participated in a three-day multi-sports camp were included. The perceived health status was measured using the Child Health Questionnaire-Child Form, CHQ-CF87, completed by the child at the start of the camp (T1), at the end of the camp (T2), and 3 months after the camp concluded (T3). Habitual physical activities were assessed by means of a modified version of the Baecke questionnaire, which was completed by one of the parents at T1 and T3., Results: During the sports camp, we observed significant improvements in the children's perception of their physical functioning, role-physical functioning, general health, role-emotional functioning, self-esteem, mental health, and general behavior. For physical functioning, role-emotional functioning, and family activities, high scores persisted three months after the sports camp concluded. The habitual physical activities (sport and leisure time) of the children remained unchanged., Discussion and Conclusion: In conclusion, we propose that a special sports camp for children with congenital heart disease may improve specific dimensions of subjective health status. Our study confirms a previous report on the benefits of such camps for afflicted children. If these findings can be further corroborated in other settings, participation in sport camps should be advocated as a simple, noninvasive means to promote healthier children.

This study examined the influence of preload alterations on systolic right ventricular (RV) velocities and deformation indices in patients with atrial septal defect (ASD). A total of 21 patients with ASD underwent 2-dimensional echocardiography and color Doppler myocardial imaging scan before and 24 hours after transcatheter ASD closure. The data were compared with those obtained from 30 healthy age-matched children. Longitudinal peak systolic velocities, peak systolic strain rate (SR), and end-systolic strain (epsilon) were calculated in the midsegment of the RV free wall and the septum. In patients with ASD, higher RV peak systolic velocities were recorded, which returned to normal values after closure of the defect. In contrast, RV deformation indices in the patients with ASD were not different from normal values. These RV indices did not change significantly after ASD closure. We found a significantly decreased septal function after percutaneous ASD closure. This may be attributed to the device within the atrial septum, affecting deformation of the interventricular septum. In conclusion, RV longitudinal systolic deformation indices were not significantly influenced by chronic ventricular volume overload and sudden abolishment of this volume overload. These findings suggest that SR/epsilon are relatively load-stable measures of contractile function in the clinical setting. In contrast, myocardial velocities are clearly load dependent.

Recent Fontan circuits frequently involve an extracardiac conduit. We report on a new technique to create a late fenestration in such an extracardiac circuit by sequential flaring of a stent in the fenestration., (2006 Wiley-Liss, Inc.)

Closure of a Potts' aortopulmonary anastomosis during repair of a congenital heart malformation has been associated with high morbidity and mortality, especially due to technical problems encountered at closure of the shunt. A case is presented of a 40-year-old patient with tetralogy of Fallot and pulmonary atresia after palliation during infancy with a Potts' shunt. The anastomosis was successfully occluded using an Amplatzer ventricular septal defect occluder, immediately followed by complete surgical repair of his tetralogy of Fallot.

Objective: We sought to evaluate the incidence and nature of pulmonary stenosis after implantation of the bovine jugular vein graft (Contegra; Medtronic, Inc, Minneapolis, Minn) in the right ventricular outflow tract., Methods: Between May 2000 and September 2002, 58 Contegra conduits (8-22 mm) were implanted during primary (n = 27) or redo operations (n = 31) in 57 patients, with ages ranging from 2 days to 48 years (mean, 9 years). Indications were truncus arteriosus (n = 16), tetralogy of Fallot (n = 28), pulmonary replacement in the Ross operation (n = 10), and Rastelli-type repair for double-outlet right ventricle (n = 4). Echocardiography was prospectively performed by a fixed team of investigators during follow-up (mean, 22.7 +/- 10 months). A peak gradient of greater than 50 mm Hg was considered severe stenosis., Results: Two patients died from Staphylococcus aureus -induced septicemia and enterococcal endocarditis after 12 days and 12 weeks, respectively. One patient died of heart failure caused by endocardial fibroelastosis after 1 year. Freedom from severe stenosis at the distal anastomosis was 91% +/- 3% at 3 months, 68% +/- 6% at 12 months, and 49% +/- 8% at 24 months. The risk of development of stenosis does not change over time. Younger age and its derivatives (graft size and indication) are significantly related to the occurrence of severe stenosis ( P < .0001). Seventeen (29%) conduits required an endovascular intervention (balloon dilatation or stent). Seven (12%) conduits were explanted (endocarditis, 2; stenosis, 5). Histologic analysis of the explanted conduits showed excessive proliferation of neointima at the level of the distal anastomosis. Valve regurgitation was observed in 9 (16%) conduits and was always secondary to dilatation in the presence of severe distal stenosis., Conclusion: The Contegra conduit induces a neointimal proliferation at the level of the pulmonary anastomosis. This leads to a high incidence of severe stenosis at intermediate-term follow-up.

Objective: Few empirical studies have addressed the impact of trauma exposure and posttraumatic stress disorder (PTSD) on treatment utilisation and outcome in South African youth. This study was undertaken to document demographic, clinical, and treatment characteristics of child and adolescent inpatients with PTSD., Design: A retrospective chart study of all patients presenting to a child and adolescent inpatient unit was conducted between 1994-1996. For children and adolescents diagnosed with PTSD; demographic, diagnostic and treatment variables, including trauma type, family history, and delays in treatment seeking, were documented., Setting: Child and Adolescent Psychiatric Inpatient Unit, Tygerberg Hospital, Cape Town., Subjects: Children and adolescents (2 to 18 years) presenting to an inpatient unit (n = 737)., Results: 10.3% (n = 76) met diagnostic criteria for PTSD. Gender differences were clearly evident: PTSD was six times more prevalent in girls (65 with PTSD were female and 11 were male); girls were most likely to have experienced rape or sexual abuse while boys were most likely to have witnessed a killing. Psychotherapy was the most common intervention for PTSD, followed by treatment with a tricyclic antidepressant. 97.4% of children and adolescents who were treated were improved at treatment endpoint. Delays in seeking treatment and problems with the primary support group were highly prevalent., Conclusion: PTSD is a common disorder that is responsive to treatment with psychotherapy and/or tricyclic antidepressants in child and adolescent inpatients. These findings underscore the importance of early identification and treatment of childhood PTSD in mental health settings, in particular tertiary service institutions.