Efficacy of amantadine on behavioural problems due to acquired brain injury: A systematic review.

Objectives: To systematically review literature on efficacy of amantadine on behavior (irritability/aggression/agitation, emotional lability, apathy, impairment of executive functioning), participation, quality-of-life (QoL), and safety, in patients with acquired brain injury (ABI). Amantadine is widely used clinically, so comprehensive information on efficacy, participation, QoL and safety is relevant. Methods: We used PRISMA Guidelines. We searched PubMed/EMBASE/CINAHL (last search 28-8-2018) Two independent reviewers performed selection and data-extraction. Quality of studies was assessed, using CONSORT and Quality Assessment Tool for Quantitative Studies (QATFQS). Results: Eleven out of 500 studies were included. Of five RCTs, two reported significant effects on irritability/aggression, and one no effect. One RCT on cognition no effect. One prospective cohort study showed a significant effect on executive functioning. One retrospective study was inconclusive. One single-case experimental design (SCED) study reported significant effect on apathy and three case-reports indicated effects on behavior. QoL and societal participation were not measured. No safety issues emerged. Conclusion: Amantadine may be efficacious on irritability and aggression after ABI. Amantadine is a safe drug in the presence of adequate creatinine clearance. Future studies should use designs, suitable for the heterogeneous ABI population, like randomized SCEDs, and should include the effect on societal participation and QoL.

Transition from paediatric to adult care of adolescent patients with congenital heart disease: a pathway to optimal care.

INTRODUCTION: Adolescents with congenital heart disease transition from a paediatric to an adult setting. This is associated with loss-to-follow-up and suboptimal care. Increasing numbers of patients justify a special program. In this study we evaluated the cooperative program between paediatric and adult cardiology departments in a tertiary referral centre. METHODS: In this retrospective study, patients with congenital heart disease with at least one appointment scheduled at the transition program between January 2010 and January 2015 were included. They were seen by a paediatric cardiologist at the age of 15 years in the paediatric department and from age 18 to 25 in the adult department. Demographic and medical data were collected from the electronic patient files. RESULTS: A total of 193 patients (105 males, 88 females) were identified. Sex distribution was almost equal. Most patients were 18-21 years of age. The largest group, 128 patients (67 %), lived within 50 kilometres of our hospital. Paediatric cardiologists referred 157 (81 %) of patients. General practitioners and cardiologists from outside our centre were important referrers for patients lost to follow-up, together accounting for 9 %. A total of 34 (18 %) patients missed an appointment without notification. Repeat offenders, 16 of 34 patients, formed a significant minority within this group. A total of 114 (59 %) patients were attending school, 46 (24 %) were employed, and 33 (17 %) patients were inactive. Activities are in line with capabilities. A nurse practitioner was involved with the 7 % with complex and psychosocial problems. Moderately severe congenital heart defects formed the largest patient category of 102 (53 %) patients. In 3 % of patients the diagnosis had to be revised or was significantly incomplete. In 30 (16 %) patients, cardiac diagnosis was part of a syndrome. Of the 193 patients, 117 (92 %) were in NYHA class I, with 12 (6 %) and 4 (2 %) patients falling into classes II and III, respectively. CONCLUSIONS: A viable transition program can be built by collaboration between paediatric and adult cardiology departments with the same treating physician taking care of patients between 15 and 25 years of age. General practitioners are important in returning lost-to-follow-up patients to specialised care. Nurse practitioners are essential in the care for patients with complex congenital heart disease.

Mobile health in adults with congenital heart disease: current use and future needs.

OBJECTIVE: Many adults with congenital heart disease (CHD) are affected lifelong by cardiac events, particularly arrhythmias and heart failure. Despite the care provided, the cardiac event rate remains high. Mobile health (mHealth) brings opportunities to enhance daily monitoring and hence timely response in an attempt to improve outcome. However, it is not known if adults with CHD are currently using mHealth and what type of mHealth they may need in the near future. METHODS: Consecutive adult patients with CHD who visited the outpatient clinic at the Academic Medical Center in Amsterdam were asked to fill out questionnaires. Exclusion criteria for this study were mental impairment or inability to read and write Dutch. RESULTS: All 118 patients participated (median age 40 (range 18-78) years, 40 % male, 49 % symptomatic) and 92 % owned a smartphone. Whereas only a small minority (14 %) of patients used mHealth, the large majority (75 %) were willing to start. Most patients wanted to use mHealth in order to receive more information on physical health, and advice on progression of symptoms or signs of deterioration. Analyses on age, gender and complexity of defect showed significantly less current smartphone usage at older age, but no difference in interest or preferences in type of mHealth application for the near future. CONCLUSION: The relatively young adult CHD population only rarely uses mHealth, but the majority are motivated to start using mHealth. New mHealth initiatives are required in these patients with a chronic condition who need lifelong surveillance in order to reveal if a reduction in morbidity and mortality and improvement in quality of life can be achieved.

Platypnoea-orthodeoxia syndrome, an underdiagnosed cause of hypoxaemia: four cases and the possible underlying mechanisms.

Cardiac platypnoea-orthodeoxia syndrome (POS) is a position-dependent condition of dyspnoea and hypoxaemia due to right-to-left shunting. It often remains unrecognised in clinical practice, possibly because of its complex underlying pathophysiology. We present four consecutive patients with POS and patent foramen ovale (PFO) who underwent a successful percutaneous PFO closure, describe the mechanism of their POS and provide a review of the literature.

Unroofed coronary sinus newly diagnosed in adult patients after corrected congenital heart disease.

Patients with congenital heart disease corrected in early childhood may later in life present with cardiac symptoms caused by other associated congenital anomalies that were initially not diagnosed. Nowadays, several noninvasive imaging modalities are available for the visualisation of cardiac anatomy in great detail. We describe two patients with an unroofed coronary sinus, a rare congenital anomaly which could be diagnosed using a combination of modalities including echocardiography, cardiac CT and cardiac MRI.

Two cases of aorto-right atrial tunnel: clinical presentation, imaging and percutaneous closure.

Communication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagnosed in children and young adults. We describe two cases with a tunnel between the aortic sinus and right atrium, discuss diagnostic modalities, and review the literature on this anomaly.

Per-operative stent placement in the right pulmonary artery; a hybrid technique for the management of pulmonary artery branch stenosis at the time of pulmonary valve replacement in adult Fallot patients.

After having undergone surgical correction at an early age, many patients with tetralogy of Fallot develop long-term complications including progressive pulmonary regurgitation and peripheral pulmonary stenosis. A high percentage of these patients need to undergo a second operation in their adolescence or early adulthood. If simultaneous treatment of both pulmonary regurgitation and peripheral pulmonary stenosis is warranted, a complete surgical approach has several disadvantages. We describe four cases of Fallot patients with severe pulmonary regurgitation and peripheral pulmonary stenosis who were treated using a hybrid approach involving surgical implantation of a pulmonary homograft and peroperative stenting of the pulmonary artery.

Percutaneous treatment of native aortic coarctation in adults.

Aortic coarctation is a common congenital cardiac defect, which can be diagnosed over a wide range of ages and with varying degrees of severity. We present two cases of patients diagnosed with aortic coarctation in adulthood. Both patients were treated by an endovascular approach. These cases demonstrate the variety of indications in which percutaneous treatment is an excellent alternative for surgical treatment in adult native coarctation patients.

Patent ductus arteriosus in adults - indications and possibilities for closure.

Patent ductus arteriosus (PDA) is a rare diagnosis in adults, since symptoms and signs usually occur in infancy and most cases are treated shortly after diagnosis. We present two patients who were first diagnosed with PDA during adulthood. The first case represents a more severe form of PDA, where the need for closure of the PDA is obvious. In the second case the sequelae of the PDA are less clear. In both patients, closure of the PDA (surgically in one case, percutaneously in the other) was successful.

Acute Cardiac Failure due to Intra-Atrial Mass Caused by Zygomycetes in an Immunocompromised Paediatric Patient.

Cardiac zygomycosis can be a critical condition with sudden onset of severe congestive heart failure followed by severe hemodynamic deterioration. We report a fatal course of disseminated fungal infection with a massive intra-atrial thrombosis caused by a zygomycete, in a five year old boy treated for acute lymphoblastic leukaemia. In addition, we discuss the literature concerning infections caused by zygomycetes involving the heart. Prognosis is poor. A high index of suspicion and an aggressive diagnostic and therapeutic approach with the prompt start of preemptive antifungal therapy are key factors to improve outcome.

Cluster headache and oxygen: is it possible to predict which patients will be relieved? A retrospective cross-sectional correlation study.

Most cluster headache patients respond to oxygen therapy, but approximately 20% do not. The aim of the present study was to assess which factors differ between cluster headache patients who respond to oxygen therapy and those who do not. We included patients from the headache clinic of Atrium Medical Centre Heerlen (n = 53) and patients who responded to a cluster headache web-site (n = 62). Participants completed a questionnaire with questions on cluster headache and factors that might be of significance with respect to the response to oxygen. Non-responders had less often smoked in the past (p = 0.014), had longer cluster headache attacks (p = 0.049), and more often reported interictal headache (p = 0.02) than responders. Logistic regression analysis showed these variables to be independent risk factors for not responding to oxygen and a clinical prediction model is provided. The area under the receiver operating characteristic curve was 0.75. We conclude that cluster headache patients who smoked in the past, had shorter attacks and were pain-free interictally respond best to oxygen inhalation. The results did not provide clues for the mechanism of action of oxygen therapy.

Early cardiac involvement in children carrying the A3243G mtDNA mutation.

UNLABELLED: The phenotypic spectrum of the mitochondrial A3243G DKA mutation is highly variable, particularly when occuring in childhood. In contrast to the classical presentation in adulthood (MELAS syndrome; mitochondria! myopathy, encephalopathy, lactic acidosis and stroke-like episodes) children show a different pattern of symptoms, often without the typical encephalopathy or psychomotor regression. We present six children carrying the A3243G mtDNA mutation with a heteroplasmy above 50 % in muscle tissue. The age of diagnosis ranged from 2 weeks up to 14.5 years. The clinical presentation was rather non-specific including muscle weakness, developmental delay and epilepsy. In this small pediatric group we detected presymptomatic cardiac involvement in five out of six children already at an early stage of disease. The cardiac pathology included cardiomyopathy and biventricular hypertrophy with rhythm disturbances (for example long QT-syndrome). The observed cardiac changes do not always increase the risk of cardiac deterioration; however, two of our patients died early on. CONCLUSION: We hypothesize that the A3243G mutation might be underdiagnosed, as patients could suffer from an unexplained cardiac death before the diagnosis is made. We advise performing regular repeated ECGs and echocardiography in all children carrying a A3243G mtDNA mutation independently from the presence of cardiac symptoms.

[Results of extracorporeal membrane oxygenation following cardiac surgery in 16 children in the St Radboud University Medical Centre, 1996-2004]. / Resultaten van extracorporele membraanoxygenatie na een hartoperatie bij 16 kinderen in het Universitair Medisch Centrum St Radboud, 1996-2004.

OBJECTIVE: Description of the results with extracorporeal membrane oxygenation (ECMO) in infants with cardiorespiratory failure after open-heart surgery. DESIGN: Prospective observational study. METHOD: Data on the indications, survival, and complications were collected from all infants treated with ECMO for cardiorespiratory failure after open-heart surgery in the period 1996-2004 in the St Radboud University Medical Centre in Nijmegen, the Netherlands. RESULTS: 17 ECMO treatments were carried out in 16 paediatric patients: 7 girls and 9 boys. All patients had severe circulatory failure, sometimes combined with respiratory insufficiency. The patients' average age was 8 months (range: 1 day-3 years and 10 months). The average duration of treatment was 137 hours (range: 8-433). In 12 of the 17 ECMO treatments, the patient was successfully weaned from ECMO and 7 patients were discharged home. 5 patients died in hospital after successful ECMO treatment. 10 of the 16 patients died. A functional univentricular heart was associated with a high mortality. Haemorrhage, renal insufficiency and; sepsis were the most frequent complications. Major causes of death were sepsis, multi-organ failure and pulmonary hypertension.