High burden of rheumatic heart disease confirmed by echocardiography among Pacific adults living in New Zealand.

BACKGROUND: Despite numerous echocardiographic screening studies of children in high incidence acute rheumatic fever (ARF)/rheumatic heart disease (RHD) communities, little is known about the prevalence of RHD in adults in these populations.We sought to determine the prevalence of RHD in an urban area of South Auckland, New Zealand, where previous studies had shown the prevalence of RHD in children to be around 2%. METHODS: A cross-sectional screening study was conducted between 2014 and 2016. Echocardiography clinics were conducted at an urban Pacific-led primary healthcare clinic in New Zealand. Eligible persons aged 16-40 years were recruited according to a stratified randomised approach. Echocardiograms were performed with a standardised image acquisition protocol and reported by cardiologists. RESULTS: There were 465 individuals who underwent echocardiograms. The overall prevalence of RHD (define and borderline) was 56 per 1000 (95% CI 36 to 78 per 1000). Definite RHD was found in 10 individuals (4 of whom were already under cardiology review at a hospital clinic) with a prevalence of 22 per 1000 (95% CI 9 to 36 per 1000). Non-rheumatic cardiac abnormalities were found in 29 individuals. CONCLUSIONS: There is a high burden of both rheumatic and non-rheumatic cardiac abnormalities in this population. Rates described in New Zealand are as high as lower-middle-income countries in Africa. Addressing knowledge gaps regarding the natural history of RHD detected by echocardiography in adults is a priority issue for the international RHD community.

Echocardiography for latent rheumatic heart disease in first degree relatives of children with acute rheumatic fever: Implications for active case finding in family members.

BACKGROUND: Individuals with Acute Rheumatic Fever (ARF) often report a family history of ARF or Rheumatic Heart Disease (RHD) however the degree of familial susceptibility to RHD is poorly defined. This study aimed to determine RHD prevalence among first degree relatives of ARF patients using echocardiography. METHODS: Children with ARF were recruited from Auckland, New Zealand. Parents and siblings ≥ 4years were offered echocardiography. Echocardiograms were reported according to World Heart Federation 2012 criteria. RHD prevalence in first degree relatives was compared to previously established population rates in the region. FINDINGS: In total, 70 index cases with ARF were recruited. Echocardiography was performed in 94 parents and 132 siblings. There were 3 siblings with definite RHD and 9 with borderline RHD. There were 4 parents with definite RHD. Overall prevalence of RHD (definite and borderline) in siblings was 90/1,000 (95% CI 45-143/1,000) compared to 36/1,000 (95% CI 30-42/1,000) in New Zealand children from high ARF incidence populations (p 0.001). Prevalence of definite RHD in parents was 42/1,000 (95% CI 7-87/1,000) compared to 22/1,000 (95% CI 9-36/1,000) in adults from a high ARF incidence New Zealand population (p 0.249). INTERPRETATION: RHD prevalence in siblings and parents of ARF cases is significantly greater than in comparable background populations. The contribution of hereditary versus environmental risk factors remains uncertain. We recommend targeted echocardiographic case-finding among siblings and parents of ARF/RHD cases in order to detect previously unrecognized latent RHD.

Safe Decompression of the Right Ventricle for PAIVS in Neonates With Coronary Fistulae: Including the Selective Use of Fistula Ligation to Avoid Coronary Steal.

BACKGROUND: There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary. METHODS: All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle. RESULTS: A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days (P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival. CONCLUSION: Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.

Exploring the use of high and low demand simulation for human performance assessment during multiorgan retrieval with the joint scrub practitioner.

Introduction: The National Organ Retrieval Service (NORS) 2015 review recommended a Joint Scrub Practitioner for abdominal and cardiac teams during combined organ retrieval. To evaluate the feasibility of this role, and to understand the functional implications, this study explores the use of simulation and provides a novel and comprehensive approach to assess individual and team performance in simulated multiorgan retrievals. Methods: Two high-fidelity simulations were conducted in an operating theatre with porcine organs, en bloc, placed in a mannequin. For donation after brainstem death (DBD) simulation, an anaesthetic machine provided simulated physiological output. Retrievals following donation after circulatory death (DCD) began with rapid arrival in theatre of the mannequin. Cardiothoracic (lead surgeon) and abdominal (lead and assistant surgeons; joint scrub practitioner, n=9) teams combined for the retrievals. Data collected before, during and after simulations used self-report and expert observers to assess: attitudinal expectations, mental readiness, mental effort, non-technical skills, teamwork, task workload and social validation perceptions. Results: Attitudinal changes regarding feasibility of a joint scrub practitioner for DBD and DCD are displayed in the main body. There were no significant differences in mental readiness prior to simulations nor in mental effort indicated afterwards; however, variance was noted between simulations for individual team members. Non-technical skills were slightly lower in DCD than in DBD. Global ratings of teamwork were significantly (p<0.05) lower in DCD than in DBD. Measures of attitude indicated less support for the proposed joint scrub practitioner role for DCD than for DBD. Discussion: The paper posits that the joint scrub practitioner role in DCD multiorgan retrieval may bring serious and unanticipated challenges. Further work to determine the feasibility of the NORS recommendation is required. Measures of team performance and individual psychological response can inform organ retrieval feasibility considerations nationally and internationally.

The Vanguard Study: Human Performance Evaluation of UK National Organ Retrieval Service Teams Utilizing a Single Scrub Practitioner in Multiorgan Retrieval.

BACKGROUND: The National Organ Retrieval Service (NORS) 2015 review recommended a single scrub practitioner provide support simultaneously to abdominal and cardiothoracic teams in UK multiorgan retrieval. Previously, this model had been used only by the combined abdominal and cardiac team in Scotland. This study reports the impact on performance as part of the Vanguard project, which utilized the single scrub practitioner role with 5 NORS teams, to determine applicability United Kingdom wide. METHODS: Participants comprised members of abdominal (n = 56) and cardiothoracic (n = 54) teams attending UK thoraco-abdominal retrievals. Data were collected by validated psychometric scales to assess individual workload, anxiety, confidence, demands/coping resources, and teamwork. Additional data were collected through open comments and quantitative data describing context and outcome of retrieval. RESULTS: Abdominal and cardiothoracic teams showed different responses when using single (Vanguard) or dual scrub practitioners (Standard). Vanguard configuration was associated with significantly higher anxiety for abdominal but not cardiothoracic teams. Perceived workload increased for abdominal teams during Vanguard but decreased for cardiothoracic teams. Scrub practitioners reported elevated anxiety and decreased confidence in retrievals using Vanguard configuration. CONCLUSIONS: This is the first large study examining human performance during organ retrieval in the United Kingdom. Despite previous regional success, this study showed a significant negative impact of the single scrub practitioner when extrapolated widely to UK teams. As a result of this study, NORS declined to implement the single scrub model. These data support the use of human performance analysis as an essential part of successful development in organ retrieval practice.

Understanding Medical Neglect: When Needed Care Is Delayed or Refused.

Though the most common form of child maltreatment, neglect can prove among the hardest to diagnose, and intervention is equally difficult. In considering neglect of a child's medical needs, a number of factors play important roles. Diagnosis should be motivated foremost by the intent of providing the best ongoing care for the patient, supplying what the child has not been able to receive from the caregiver. Characteristics peculiar to the patient, the parents, the pathologic condition, its possible treatments, and the mutual understanding between the child's caregivers and the treating professionals all help determine why the therapeutic relationship has failed and which interventions will be most effective. Religious and cultural considerations may lead a family to refuse medical treatments, occasionally to the child's detriment. The caregivers' wishes must be taken into account, but legal precedent has affirmed that the patient's welfare remains the paramount concern. Sorting through the opinions and providing clarity can be a challenge. Finally, good medical care can help prevent medical neglect in many cases. Clear communication and empathy remain hallmarks of good medical practice.

Lessons from a pilot for uncontrolled donation after circulatory death in the ED in the UK.

Worldwide there is a shortage of available organs for patients requiring transplants. However, some countries such as France, Italy and Spain have had greater success by allowing donations from patients with unexpected and unrecoverable circulatory arrest who arrive in the ED. Significant advances in the surgical approach to organ recovery from donation after circulatory death (DCD) led to the establishment of a pilot programme for uncontrolled DCD in the ED of the Royal Infirmary of Edinburgh. This paper describes the programme and discusses the lessons learnt.

Inter-rater and intra-rater reliability and agreement of echocardiographic diagnosis of rheumatic heart disease using the World Heart Federation evidence-based criteria.

OBJECTIVE: Different definitions have been used for screening for rheumatic heart disease (RHD). This led to the development of the 2012 evidence-based World Heart Federation (WHF) echocardiographic criteria. The objective of this study is to determine the intra-rater and inter-rater reliability and agreement in differentiating no RHD from mild RHD using the WHF echocardiographic criteria. METHODS: A standard set of 200 echocardiograms was collated from prior population-based surveys and uploaded for blinded web-based reporting. Fifteen international cardiologists reported on and categorised each echocardiogram as no RHD, borderline or definite RHD. Intra-rater and inter-rater reliability was calculated using Cohen's and Fleiss' free-marginal multirater kappa (κ) statistics, respectively. Agreement assessment was expressed as percentages. Subanalyses assessed reproducibility and agreement parameters in detecting individual components of WHF criteria. RESULTS: Sample size from a statistical standpoint was 3000, based on repeated reporting of the 200 studies. The inter-rater and intra-rater reliability of diagnosing definite RHD was substantial with a kappa of 0.65 and 0.69, respectively. The diagnosis of pathological mitral and aortic regurgitation was reliable and almost perfect, kappa of 0.79 and 0.86, respectively. Agreement for morphological changes of RHD was variable ranging from 0.54 to 0.93 κ. CONCLUSIONS: The WHF echocardiographic criteria enable reproducible categorisation of echocardiograms as definite RHD versus no or borderline RHD and hence it would be a suitable tool for screening and monitoring disease progression. The study highlights the strengths and limitations of the WHF echo criteria and provides a platform for future revisions.

The World Heart Federation criteria raise the threshold of diagnosis for mild rheumatic heart disease: Three reviewers are better than one.

BACKGROUND: The World Heart Federation (WHF) criteria, published in 2012, provided an evidence-based guideline for the minimal diagnosis of echocardiographically-detected RHD. Primary aim of the study was to determine whether use of the WHF criteria altered the threshold for the diagnosis of echocardiographically-detected RHD compared with the previous WHO/NIH criteria. A secondary aim was to explore the utility of a three reviewer reporting system compared to a single or two reviewer reporting structure. METHODS: 144 de-identified echocardiograms (RHD, congenital valvar abnormality, physiological valvar regurgitation) were independently reported using the WHF criteria by two reviewers blinded to the previous WHO/NIH diagnosis. If there was discordance between the two reviewers, a third cardiologist independently performed a tie-breaker review. RESULTS: There was a 21% reduction of cases classified as RHD using the WHF criteria compared to the modified WHO/NIH criteria (68 cases compared to 86, p = 0.04). There was a 60% consensus across the different diagnostic categories with 2 reviewers, 89% majority agreement with 3 reviewers. 11% required an open label discussion. There was moderate agreement between 2 reviewers for any RHD, kappa 0.57 (CI 0.44-0.70), with no significant difference in agreement between the different categories. CONCLUSION: The WHF criteria have raised the threshold for the diagnosis of RHD compared to the WHO/NIH criteria. However, inter-reporter variability of the WHF criteria is high. A three reviewer system is likely more accurate than a single or two reporter system for the diagnosis of mild RHD. This has resource implications for echocardiographic screening programmes.

Transcatheter closure of secundum atrial septal defects: has fear of device erosion altered outcomes?

BACKGROUND: Transcatheter device closure has become the established standard of care for suitable atrial septal defects. Device erosion has been a recent focus and has prompted changes in the Instructions for Users documentation released by device companies. We reviewed our entire local experience with atrial septal defect device closure, focussing on the evolution of this procedure in our centre and particularly on complications. METHODS: We carried out a retrospective review of 581 consecutive patients undergoing attempted transcatheter device closure of an atrial septal defect in Auckland from December 1997 to June 2014. We reviewed all complications recorded and compared our outcomes with the current literature. We sought to understand the impact of the evolution in recommendations and clinical practice on patient outcomes in our programme. RESULTS: There were a total of 24 complications (4.1%), including 10 device embolisations (1.7%), nine arrhythmias (1.5%), two significant vascular access-related complications (0.3%), one device erosion (0.2%), one malposed device (0.2%), and one probable wire perforation of the left atrial appendage (0.2%). There was one mortality related to device embolisation. All device embolisations occurred following the change in Instructions for Users after publication of the first device erosion report in 2004. This increase in embolisation rate was statistically significant (p-value 0.015). CONCLUSIONS: In our series, the incidence of device embolisation was higher than that anticipated, with a significant increase following changes to the Instructions for Users. This highlights the need for ongoing data collection on complication incidence and for ongoing review of the impact of changes in clinical practice on complication rates.

Infective endocarditis following Melody valve implantation: comparison with a surgical cohort.

BACKGROUND: Infective endocarditis has been reported post Melody percutaneous pulmonary valve implant; the incidence and risk factors, however, remain poorly defined. We identified four cases of endocarditis from our first 25 Melody implants. Our aim was to examine these cases in the context of postulated risk factors and directly compare endocarditis rates with local surgical valves. METHODS: We conducted a retrospective review of patients post Melody percutaneous pulmonary valve implant in New Zealand (October, 2009-May, 2015) and also reviewed the incidence of endocarditis in New Zealand among patients who have undergone surgical pulmonary valve implants. RESULTS: In total, 25 patients underwent Melody implantation at a median age of 18 years. At a median follow-up of 2.9 years, most were well with low valve gradient (median 27 mmHg) and only mild regurgitation. Two patients presented with life-threatening endocarditis and obstructive vegetations at 14 and 26 months post implant, respectively. Two additional patients presented with subacute endocarditis at 5.5 years post implant. From 2009 to May, 2015, 178 surgical pulmonic bioprostheses, largely Hancock valves and homografts, were used at our institution. At a median follow-up of 2.9 years, four patients (2%) had developed endocarditis in this group compared with 4/25 (16%) in the Melody group (p=0.0089). Three surgical valves have been replaced. CONCLUSIONS: The Melody valve offers a good alternative to surgical conduit replacement in selected patients. Many patients have excellent outcomes in the medium term. Endocarditis, however, can occur and if associated with obstruction can be life threatening. The risk for endocarditis in the Melody group was higher in comparison with that in a contemporaneous surgical pulmonary implant cohort.

Valvular Regurgitation Using Portable Echocardiography in a Healthy Student Population: Implications for Rheumatic Heart Disease Screening.

BACKGROUND: There is increasing use of portable echocardiography as a screening test for rheumatic heart disease (RHD). The prevalence of valvular regurgitation in healthy populations as determined using portable echocardiography has not been well defined. Minimal echocardiographic criteria for RHD have recently been clarified, but the overlap of normal and abnormal valvular regurgitation warrants further study. The aim of this study was to determine the spectrum of echocardiographic findings using portable echocardiography in children from a population with low prevalence of RHD. METHODS: Screening echocardiography was conducted in 396 healthy students aged 10 to 12 years using portable echocardiographic equipment. Echocardiograms were assessed according to 2012 World Heart Federation criteria for RHD. The prevalence of physiologic valvular regurgitation was compared with that found in previous studies of children using large-platform machines. RESULTS: Physiologic mitral regurgitation (MR) was present in 14.9% of subjects (95% CI, 11.7%-18.7%) and pathologic MR in 1.3% (95% CI, 0.6%-2.9%). Two percent (95% CI, 1.0%-3.9%) had physiologic aortic regurgitation, and none had pathologic aortic valve regurgitation. Physiologic tricuspid regurgitation was present in 72.7% of subjects (95% CI, 68.1%-76.9%) and physiologic pulmonary regurgitation in 89.6% (95% CI, 85.7%-91.8%). After cardiology review, no cases of definite RHD were found, but 0.5% of patients (95% CI, 0.1%-1.8%) had pathologic MR meeting World Heart Federation criteria for borderline RHD. Two percent (95% CI, 1.4%-4.6%) of the cohort had minor forms of congenital heart disease. CONCLUSIONS: The spectrum of physiologic cardiac valvular regurgitation in healthy children as determined using portable echocardiography is described and is within the range of previous studies using large-platform echocardiographic equipment. The finding of two children with pathologic-grade MR, likely representing the upper limit of physiologic regurgitation, has implications for echocardiographic screening for RHD in high-prevalence regions.

The conversation: interacting with parents when child abuse is suspected.

This article reviews some of the challenges and pitfalls in communicating with families when abuse is part of the differential diagnosis and offers some suggestions for improving communication with parents and children in these challenging clinical settings.

Add-on clinical effects of simvastatin and ondansetron in patients with schizophrenia stabilized on antipsychotic treatment: pilot study.

OBJECTIVES: There is some evidence that anti-inflammatory treatment may have beneficial effects in schizophrenia and major depression. Statins are cholesterol-lowering agents but have been found to be anti-inflammatory and also decrease C-reactive protein (CRP). Ondansetron is a serotonin (5-HT3) receptor antagonist widely used to prevent nausea and vomiting in patients receiving chemotherapy for cancer. Small studies have suggested that adjunctive ondansetron is efficacious against schizophrenia symptoms. We carried out a feasibility study in schizophrenia patients (within 5 years of first diagnosis) to explore the adjunctive use of simvastatin and ondansetron on positive, negative and general psychopathology. METHODS: This was a 12-week rater-blind placebo-controlled study. A total of 36 patients with DSM-IV diagnosis of schizophrenia were recruited, 12 in each arm. Patients were assessed at baseline and at 12 weeks using Positive and Negative Syndrome Scale (PANSS), Clinical Global Impression (CGI) scale, Global Assessment of Functioning (GAF) and Abnormal Involuntary Movement Scale (AIMS). RESULTS: Both simvastatin and ondansetron provide some evidence of a reduction in symptoms compared with treatment as usual (TAU) on PANSS total score, although this was not statistically significant. In the secondary analyses, no significant differences were seen on CGI, GAF and AIMS. CONCLUSIONS: Anti-inflammatory treatments have been shown to have some beneficial effects in schizophrenia. Both simvastatin and ondansetron provide some evidence of a reduction in symptoms compared with TAU. This study has led to a larger Stanley Medical Research Institute (SMRI)-funded, double-blind, randomized control trial.

Two cases of warfarin-induced tracheobronchial calcification after Fontan surgery.

This study identified tracheobronchial cartilage calcification in children with congenital heart disease. Calcification of the tracheobronchial airways has been found previously in adults receiving warfarin and in children receiving warfarin after mitral valve replacement. A 9-year-old girl who had received a Fontan repair 6 years previously underwent a cardiac computed tomography (CT) scan to evaluate pulmonary artery size. The result was an incidental finding of extensive tracheobronchial cartilage calcification. A retrospective review of all pediatric Fontan patients who had undergone cardiac CT was conducted to search for calcification of the tracheobronchial cartilage. The study investigated ten pediatric Fontan patients who had undergone cardiac CT scanning. Two patients with extensive calcification of the tracheobronchial airways were identified. The index case had hypoplastic left heart syndrome, and the patient had undergone a staged repair with the Fontan at the age of 3 years. A 16-year-old boy with tricuspid atresia had undergone staged repair and Fontan at the age of 3.5 years. These two patients had received continuous warfarin therapy for 6 and 13 years, respectively. Other common causes of airway calcification were excluded from the study. This report describes warfarin-induced tracheobronchial calcification in patients after the Fontan procedure. This finding has possible implications for airway growth and vascular calcification.

Ondansetron and simvastatin added to treatment as usual in patients with schizophrenia: study protocol for a randomized controlled trial.

BACKGROUND: Negative symptoms and cognitive deficits are two partially-related features of schizophrenia which have a major negative impact on social function and objective quality of life. Standard drug treatments have little impact on either. There is some evidence that anti-inflammatory treatment may have beneficial effects in schizophrenia and major depression. Statins are cholesterol-lowering agents that have been found to be anti-inflammatory agents and are also known to decrease C-reactive protein (CRP). Ondansetron is a serotonin (5-HT3) receptor antagonist widely used to prevent nausea and vomiting in patients receiving chemotherapy for cancer. Small studies have suggested that ondansetron is effective as an adjunct drug in improving the symptoms of schizophrenia. METHODS/DESIGN: This is a two center, six-month, double-blind placebo controlled, factorial design study of ondansetron and/or simvastatin added to treatment as usual for patients suffering from schizophrenia, schizoaffective disorder, psychosis not otherwise specified or schizophreniform disorder. This will be a 2 × 2 design, with 54 patients in each cell, giving a total of 216 patients over three years. There will be a screening, a randomization and seven follow-up visits. Full clinical and neurocognitive assessments will be carried out at baseline (randomization), 14 weeks and at 26 weeks, while the positive and negative syndrome scale (PANSS), pill count and side effects checklist will be carried out at every visit. Simvastatin will be started at 20 mg once daily (OD), this will be increased to 40 mg after four weeks. Ondansetron will be administered in an 8 mg dose. DISCUSSION: Anti-inflammatory treatments have been shown to have some beneficial effects in schizophrenia. Both simvastatin and ondansetron provide some evidence of a reduction in symptoms compared to treatment as usual. The aim of this study is to establish the degree of improvement in negative symptoms with the addition of ondansetron and/or simvastatin to treatment as usual. TRIAL REGISTRATION: [corrected] ClinicalTrials.gov NCT01602029.