Congenital heart disease: addressing the need for novel lower-risk percutaneous interventional strategies.

INTRODUCTION: With the advent of improved neonatal care, increasingly vulnerable higher-risk patients with complex congenital heart anomalies are presenting for intervention. This group of patients will always have a higher risk of an adverse event during a procedure but by recognizing this risk and with the introduction of risk scoring systems and thus the development of novel lower risk procedures, the rate of adverse events can be reduced. AREA COVERED: This article reviews risk scoring systems for congenital catheterization and demonstrates how they can be used to reduce the rate of adverse events. Then, novel low risk strategies are discussed for low-weight infants e.g. patent ductus arteriosus (PDA) stent insertion; premature infants e.g. PDA device closure; and transcatheter pulmonary valve replacement. Finally, how risk is assessed and managed within the inherent bias of an institution is discussed. EXPERT OPINION: There has been a remarkable improvement in the rate of adverse events in congenital cardiac interventions, but now, as the benchmark of mortality rate is switched to morbidity and quality of life, continued innovation into lower risk strategies and understanding the inherent bias when assessing risk will be key to continuing this improvement.

Cardiac troponin is associated with cardiac outcomes in men and women with atrial fibrillation, insights from the ARISTOTLE trial.

BACKGROUND: Cardiac troponin T (cTnT) and I (cTnI) concentrations provide strong prognostic information in anticoagulated patients with atrial fibrillation (AF). Whether the associations between cardiac troponin concentrations and mortality and morbidity differ by sex is not known. OBJECTIVES: To assess whether men and women have different concentrations and prognostic value of cTnT and cTnI measurements in anticoagulated patients with AF. METHODS: cTnT and cTnI concentrations were measured with high-sensitivity (hs) assays in EDTA plasma samples obtained from the multicentre ARISTOTLE trial, which randomized patients with AF and at least one risk factor for stroke or systemic embolic event to warfarin or apixaban. Patients were stratified according to sex and the associations between hs-troponin concentrations, and all-cause death, cardiac death, myocardial infarction, stroke or systemic embolic event and major bleeding were assessed in multivariable regression models. RESULTS: We found higher cardiac troponin concentrations in men (n = 9649) compared to women (n = 5331), both for hs-cTnT (median 11.8 [Q1-3 8.1-18.0] vs. 9.6 [6.7-14.3] ng L-1 , P < 0.001) and hs-cTnI (5.8 [3.4-10.8] vs. 4.9 [3.1-8.8] ng L-1 , P < 0.001). Adjusting for baseline demographics, comorbidities and medications, men still had significantly higher hs-troponin concentrations than women. C-reactive protein and N-terminal pro-B-type natriuretic peptide concentrations were higher in female patients. Both hs-cTnT and hs-cTnI concentrations were associated with all clinical outcomes similarly in men and women (p-value for interaction >0.05 for all end-points). CONCLUSION: Men have higher hs-troponin concentrations than women in AF. Regardless of sex, hs-troponin concentrations remain similarly associated with adverse clinical outcomes in anticoagulated patients with AF.

Comparison of bleeding risk scores in patients with atrial fibrillation: insights from the RE-LY trial.

BACKGROUND: Oral anticoagulation is the mainstay of stroke prevention in atrial fibrillation (AF), but must be balanced against the associated bleeding risk. Several risk scores have been proposed for prediction of bleeding events in patients with AF. OBJECTIVES: To compare the performance of contemporary clinical bleeding risk scores in 18 113 patients with AF randomized to dabigatran 110 mg, 150 mg or warfarin in the RE-LY trial. METHODS: HAS-BLED, ORBIT, ATRIA and HEMORR2 HAGES bleeding risk scores were calculated based on clinical information at baseline. All major bleeding events were centrally adjudicated. RESULTS: There were 1182 (6.5%) major bleeding events during a median follow-up of 2.0 years. For all the four schemes, high-risk subgroups had higher risk of major bleeding (all P < 0.001). The ORBIT score showed the best discrimination with c-indices of 0.66, 0.66 and 0.62, respectively, for major, life-threatening and intracranial bleeding, which were significantly better than for the HAS-BLED score (difference in c-indices: 0.050, 0.053 and 0.048, respectively, all P < 0.05). The ORBIT score also showed the best calibration compared with previous data. Significant treatment interactions between the bleeding scores and the risk of major bleeding with dabigatran 150 mg BD versus warfarin were found for the ORBIT (P = 0.0019), ATRIA (P < 0.001) and HEMORR2 HAGES (P < 0.001) scores. HAS-BLED score showed a nonsignificant trend for interaction (P = 0.0607). CONCLUSIONS: Amongst the current clinical bleeding risk scores, the ORBIT score demonstrated the best discrimination and calibration. All the scores demonstrated, to a variable extent, an interaction with bleeding risk associated with dabigatran or warfarin.

Left atrial appendage closure: outcomes and challenges.

Whereas the left atrial appendage plays a rather minor role under physiological circumstances, it gains an importance in patients with atrial fibrillation. Compelling evidence has revealed that the left atrial appendage is implicated as the source of thrombus in the vast majority of strokes in atrial fibrillation. Oral anticoagulation remains the standard of care for stroke prevention in atrial fibrillation; nevertheless, this treatment has several limitations and is often contraindicated, particularly in the elderly population in whom the risk of stroke is high. Therefore, occluding the left atrial appendage is a logical approach to prevent thrombus formation and subsequent cardioembolic events in these patients. We present a review of clinical outcomes of patients with atrial fibrillation undergoing left atrial appendage closure and the challenges faced in this field.

Improving access to mental healthcare for displaced Syrians: case studies from Syria, Iraq and Turkey.

The conflict in Syria has led to an unprecedented humanitarian crisis that extends across multiple countries in the area. Mental health services were undeveloped before and now face huge strain and unmet need. The World Health Organization and others have developed a programme to build capacity in the delivery of mental health services in an integrated healthcare package to refugees and displaced people. The tool used for this is the mhGAP Intervention Guide and complementary materials. In this paper we refer to training in Turkey, Iraq and Syria where health professionals were trained to roll out this community-based integrated approach through primary healthcare. We describe field case examples that show the complexity of situations that face refugees, displaced people and those caught in active conflict. Training improved the knowledge and skills for managing mental health disorders in primary healthcare. Further work needs to be done to demonstrate greater access to and utilisation of services, client outcomes and organisational change with this approach.

Indications, techniques and results of percutaneous patent foramen ovale closure.

Patent foramen ovale (PFO) has been shown to be more prevalent in certain disease states, suggesting a potentially causative role in some patients with cryptogenic stroke, migraine headache or a number of other conditions. Percutaneous PFO closure has become a reasonable treatment option in a subset of those patients. Our objective is to review the possible indications of PFO closure. We further elucidate the technical aspects of PFO closure, provide an overview of the available devices and summarize results of PFO closure procedures.

Transitioning mental health & psychosocial support: from short-term emergency to sustainable post-disaster development. Humanitarian Action Summit 2011.

INTRODUCTION: The Working Group (WG) on Mental Health and Psychosocial Support participated in its second Humanitarian Action Summit in 2011. This year, the WG chose to focus on a new goal: reviewing practice related to transitioning mental health and psychosocial support programs from the emergency phase to long-term development. The Working Group's findings draw on a review of relevant literature as well as case examples. OBJECTIVES: The objective of the Working Group was to identify factors that promote or hinder the long term sustainability of emergency mental health and psychosocial interventions in crisis and conflict, and to provide recommendations for transitioning such programs from relief to development. METHODS: The Working Group (WG) conducted a review of relevant literature and collected case examples based on experiences and observations of working group members in implementing mental and psychosocial programming in the field. The WG focused on reviewing literature on mental health and psychosocial programs and interventions that were established in conflict, disaster, protracted crisis settings, or transition from acute phase to development phase. The WG utilized case examples from programs in Lebanon, the Gaza Strip, Sierra Leone, Aceh (Indonesia), Sri Lanka, and New Orleans (United States). RESULTS: The WG identified five key thematic areas that should be addressed in order to successfully transition lasting and effective mental health and psychosocial programs from emergency settings to the development phase. The five areas identified were as follows: Government and Policy, Human Resources and Training, Programming and Services, Research and Monitoring, and Finance. CONCLUSIONS: The group identified several recommendations for each thematic area, which were generated from key lessons learned by working group members through implementing mental health and psychosocial support programs in a variety of settings, some successfully sustained and some that were not.

Mental health and psychosocial support in crisis and conflict: report of the Mental Health Working Group.

INTRODUCTION: The Working Group on Mental Health and Psychosocial Support was convened as part of the 2009 Harvard Humanitarian Action Summit. The Working Group chose to focus on ethical issues in mental health and psychosocial research and programming in humanitarian settings. The Working Group built on previous work and recommendations, such as the Inter-Agency Standing Committee's Guidelines on Mental Health and Psychosocial Support in Emergency Settings. OBJECTIVES: The objective of this working group was to address one of the factors contributing to the deficiency of research and the need to develop the evidence base on mental health and psychosocial support interventions during complex emergencies by proposing ethical research guidelines. Outcomes research is vital for effective program development in emergency settings, but to date, no comprehensive ethical guidelines exist for guiding such research efforts. METHODS: Working Group members conducted literature reviews which included peer-reviewed publications, agency reports, and relevant guidelines on the following topics: general ethical principles in research, cross-cultural issues, research in resource-poor countries, and specific populations such as trauma and torture survivors, refugees, minorities, children and youth, and the mentally ill. Working Group members also shared key points regarding ethical issues encountered in their own research and fieldwork. RESULTS: The group adapted a broad definition of the term "research", which encompasses needs assessments and data gathering, as well as monitoring and evaluation. The guidelines are conceptualized as applying to formal and informal processes of assessment and evaluation in which researchers as well as most service providers engage. The group reached consensus that it would be unethical not to conduct research and evaluate outcomes of mental health and psychosocial interventions in emergency settings, given that there currently is very little good evidence base for such interventions. Overarching themes and issues generated by the group for further study and articulation included: purpose and benefits of research, issues of validity, neutrality, risk, subject selection and participation, confidentiality, consent, and dissemination of results. CONCLUSIONS: The group outlined several key topics and recommendations that address ethical issues in conducting mental health and psychosocial research in humanitarian settings. The group views this set of recommendations as a living document to be further developed and refined based on input from colleagues representing different regions of the globe with an emphasis on input from colleagues from low-resource countries.

Evaluation of the AMPLATZER vascular plug for embolization of peripheral vascular malformations associated with congenital heart disease.

OBJECTIVE: The purpose of this study is to evaluate the recently FDA-approved AMPLATZER Vascular Plug in the embolization of vascular lesions associated with congenital heart disease (CHD). BACKGROUND: Fistulas and arteriovenous malformations have been occluded using various devices. The AMPLATZER Vascular Plug is a self-expandable, cylindrical device, used for embolization in the peripheral vasculature. METHODS: A total of 84 vessels in 52 patients with CHD from 11 centers were occluded with 89 AMPLATZER Vascular Plugs, delivered through a coronary guide catheter in various vascular sites, including collaterals, pulmonary arterio-venous and coronary artery fistulas, transhepatic tracts, central shunts, patent ductus arteriosus (PDA), and excluded hepatic vein. Complete vessel occlusion was demonstrated within 10 min in 94% of patients. RESULTS: There was no device embolization, vascular disruption, or procedure-related complication. One vascular plug implanted in a large type C PDA required surgical removal followed by PDA ligation, after 5 weeks from successful implant because of significant residual flow through the device. CONCLUSIONS: The AMPLATZER Vascular Plug is an effective transcatheter occlusion device in the embolization of a wide variety of vascular lesions associated with CHD. Based on our early experience, caution should be used when considering the Vascular Plug as a closure device for large PDA.

Hybrid pediatric cardiac surgery.

Minimally invasive strategies can be expanded by combining standard surgical and interventional techniques. We performed a longitudinal prospective study of all pediatric patients who have undergone hybrid cardiac surgery at the University of Chicago Children's Hospital. Hybrid cardiac surgery was defined as combined catheter-based and surgical interventions in either one setting or in a planned sequential fashion within 24 hours. Between June 2000 and June 2003, 25 patients were treated with hybrid approaches. Seventeen patients with muscular ventricular septal defects (mVSDs) (mean age, 4 months; range, 2 weeks-4 years) underwent either sequential Amplatzer device closure in the catheterization laboratory followed by surgical completion (group 1A, n = 9) or one-stage intraoperative off-pump device closure (group IB, n = 8) with subsequent repair of any concomitant heart lesions. Eight patients with branch pulmonary artery (PA) stenoses (group 2) underwent intraoperative PA stenting or stent balloon dilatation along with concomitant surgical procedures. All patients survived hospitalization. Complications from the hybrid approach were mostly confined to groups 1A and 2. At a mean follow-up of 18 months, 2 group 1A patients died suddenly several months after discharge. All other patients are doing well. Hybrid pediatric cardiac surgery performed in tandem by surgeons and cardiologists is safe and effective in reducing or eliminating cardiopulmonary bypass. Patients with mVSDs who are small, have poor vascular access, or have concomitant cardiac lesions are currently treated in one setting with the perventricular approach.

Multicenter experience with perventricular device closure of muscular ventricular septal defects.

Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure. Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs.

Pulmonary function studies in Kuwaiti children with sickle cell disease and elevated Hb F.

Conflicting ventilatory defects have been reported in children with sickle cell disease (SCD). In Kuwait, the disease is relatively mild with a low incidence of acute chest syndrome and other complications, presumably due to the Arab-Indian haplotype chromosomal background and elevated Hb F levels. There have been no previous studies of pulmonary function in patients with this haplotype. Pulmonary function test (PFT) was carried out on 28 steady state children with SCD (21 homozygous sickle cell (SS), seven S beta(o) thal) and two group of controls: 17 age- and sex-matched healthy children and 10 children with HbH disease. The charts of the SCD patients were reviewed for frequency of acute chest syndrome and vaso-occlusive crisis. The mean values of forced vital capacity (FVC) (83.2 +/- 11.9 vs. 91.2 +/- 11.7) and vital capacity (VC) (81.5 +/- 11.8 vs. 90.5 +/- 10.9) were significantly lower in the SS patients compared with healthy controls (p < 0.05). Similarly, these values were significantly lower than in those of the HbH group (p < 0.001 for VC and p < 0.01 for FVC). The mean forced expiratory volume in 1 s (FEV1) was lower in SS patients (86.4 +/- 11.5) compared with healthy controls (94.2 +/- 14.2), but the difference was not significant (p = 0.07). Also, the FEV1 was significantly lower in SS patients than in the HbH group (p < 0.001). There was no significant difference in the PFT parameters between SS patients with acute chest syndrome and those without. Although patients with frequent vaso-occlusive crisis had lower PFT parameters, the differences were not significant in comparison to those with infrequent crisis. This study revealed an early restrictive and obstructive pulmonary function pattern in steady state children with SCD. The finding also indicates that the changes of PFT parameters in SS patients could not be attributed to anaemia per se as patients with HbH who also have chronic anaemia did not show similar changes. This observation underscores the early occurrence of pulmonary involvement, even in patients with an otherwise relatively mild SCD.

Intestinal permeability is increased in bronchial asthma.

BACKGROUND: Increased intestinal permeability has been reported in one study of adult asthmatics. AIM: To determine whether children with asthma have altered intestinal permeability. METHODS: Thirty two asthmatic children, and 32 sex and age matched controls were recruited. The dual sugar (lactulose and mannitol) test was used to evaluate intestinal permeability, and the percentage of ingested lactulose (L) and mannitol (M) in the urine, and the L:M ratio were determined. All patients were skin prick tested for common aeroallergens, and specific IgE to some food items was determined. RESULTS: The median value of L in asthmatic children (2.29, IQR 0.91-4.07) was significantly higher than that in controls (0.69, IQR 0.45-1.08), and that of M was almost similar. The ratio L:M was significantly higher in asthmatic children (0.20, IQR 0.11-0.40) than in controls (0.06, IQR 0.04-0.09). Intestinal permeability did not correlate with eczema, inhaled steroids, positive skin prick test to aeroallergens, or severity of asthma. CONCLUSIONS: Intestinal permeability is increased in children with asthma, suggesting that the whole mucosal system may be affected.

Characteristics of asthmatic children in Kuwait.

This article summarizes clinical characteristics and identifies sensitizing allergens in 135 asthmatic children under 13 years of age in Kuwait, a desert environment with scant vegetation and weather conditions least associated with asthma. There were 84 males (M:F 1.65:1). Almost 70% were breast-fed (1-24 months), 59% had eczema, 52% allergic rhinitis, 78% of first-degree relatives had atopy, and 52% of parents were consanguinous. Cough was the presenting symptom in 92% and together with wheezing occurred in 76%. Most (91%) were < or = 5 years of age at diagnosis and 42% < 2 years. Mean duration of symptoms prior to diagnosis was 9.3+/-2 months (1 week-1 year). Viral upper respiratory tract infections, cigarette smoke, and exercise were the commonest triggers of symptoms (79%, 68%, 62%). Fumes of traditional Bokhour (incense) constituted a major indoor hazard. The most common sensitizing allergens were pollens of imported plants, molds, house dust mites, cockroaches, and peanuts. Management showed considerable under-treatment and included alternative medicines. In conclusion, childhood asthma in this desert environment starts at an early age, and is associated with high rate of atopy and high frequency of sensitization to aero- and food allergens. Asthmatic children are disadvantaged by delay in diagnosis, undertreatment, exposure to indoor cigarette smoke, and local traditions.

Hospital management of children with acute asthma exacerbations in Kuwait: adherence to international guidelines.

OBJECTIVES: To evaluate the in-patient management of children with acute exacerbation of bronchial asthma and its adherence to international guidelines. SUBJECTS AND METHODS: Medical records of 100 consecutive admissions for acute exacerbation of asthma to the paediatric wards at Mubarak Al-Kabeer Hospital, Kuwait, from October through December 1999 were retrieved. Data relevant to asthma symptoms, severity, treatment regimens and discharge plan were collected and evaluated. The mean age of patients was 4.3 years, ranging from 2 to 12 years. Admissions totalled 82 males and 18 females, with hospital stays ranging from 1 to 11 days (mean = 2.6 days). RESULTS: There were no fatalities. Reported episodes were severe (17%), moderate (11%) and mild (9%); degree of severity was not documented in 63 cases. Pulse, respiratory rate, colour and use of accessory muscles were not documented in 48, 48, 47 and 63% of the patients, respectively. Arterial blood gas was checked in 16 patients and pulse oximetry in 71. Full blood count, serum urea and electrolytes were obtained in 86 patients and chest radiographs in 76. All patients received nebulized salbutamol. However, some received additional medications: ipratropium bromide (79%), steroids (95%), intravenous aminophylline (5%) and antibiotics (43%). Two children were shifted to an intensive care unit, 1 of whom needed intubation. On discharge, there was no documentation of follow-up arrangements, or prophylactic anti-inflammatory drugs or other medications in 52, 81 and 32 patients, respectively. CONCLUSION: Patient management showed deficits, and documentation was inadequate. Areas of particular concern were assessment of patients, unnecessary investigations and use of antibiotics, and lack of post-discharge planning and prophylaxis with steroids. Adherence to the international guidelines was partial.

Performance of students in the final examination in paediatrics: importance of the "short cases".

AIMS: To determine which component of the final examination in paediatrics at Kuwait University best predicted the final outcome. METHODS: The performance of 356 medical students in the short cases, the long case, multiple choice questions (MCQs), and the essay components of the final examination in paediatrics at Kuwait University was correlated with the final grade, and the mean difference between each component and the final score calculated. RESULTS: The correlation was highest for the short cases, followed by MCQs, the long case, and essays. The mean difference between the final score and that of short cases was not significant, but was highly significant for the other components. CONCLUSION: Results show that performance in the short cases component of the final examination in paediatrics is a better discriminator of competence than that in the long case.

Successful combined orthotopic liver transplant and transcatheter management of atrial septal defect, patent ductus arteriosus, and peripheral pulmonic stenosis in a small infant with Alagille syndrome.

We report on a 4-kg infant with Alagille syndrome and congenital heart disease consisting of atrial septal defect (ASD), patent ductus arteriosus (PDA), and severe peripheral pulmonic stenosis. He underwent successful orthotopic liver transplant along with catheter closure of the ASD and PDA using the Amplatzer device and placement of Palmaz stents in both pulmonary arteries.