Parkinson's disease (PD) has been associated with the development of impulse control disorders (ICDs), possibly due to overstimulation of the mesolimbic system by dopaminergic medication. Preliminary reports have suggested that deep brain stimulation (DBS), a neurosurgical procedure offered to patients with treatment-resistant PD, affects ICD in a twofold way. Firstly, DBS allows a decrease in dopaminergic medication and hence causes an improvement in ICDs. Secondly, some studies have proposed that specific ICDs may develop after DBS. This paper addresses the effects of DBS on ICDs in patients with PD. A literature search identified four original studies examining a total of 182 patients for ICDs and nine case reports of 39 patients that underwent DBS and developed ICDs at some point. Data analysis from the original studies did not identify a significant difference in ICDs between patients receiving dopaminergic medication and patients on DBS, whilst the case reports showed that 56% of patients undergoing DBS had poor outcome with regards to ICDs. We discuss these ambivalent findings in the light of proposed pathogenetic mechanisms. Longitudinal, prospective studies with larger number of patients are required in order to fully understand the role of DBS on ICDs in patients with PD.

BackgroundThe Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation.Case presentationWe describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance.ConclusionsMacitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promising effects in patients with pulmonary arterial hypertension. The patient we report has shown considerable improvement in exercise capacity following introduction of this medication and thus we suggest further randomised trials to establish the role of different endothelin-receptor antagonists in the management of the Fontan circulation.

ejection fraction (EF) increased. In patients, LVEDV and LVESV decreased on exercise, with an increase in LVSV and LVEF. RVEDV increased in size with a trend for ESV to be higher than at rest. RVSV and RVEF remained unchanged. Between groups, at rest, LVESV and LVSV were lower in patients with no significant differences in RV function. However, on exercise, patients had a significantly higher RVESV and lower RVSV and RVEF, compared to HCs. Conclusion: Exercise CMR is a sensitive, accurate test which can determine physiological changes in RV and LV function. We have shown that on exercise, when compared to HC, the LV in patients with iPAH decreases in size, the RV increases in size and RV dysfunction becomes apparent which was otherwise not present at rest. This could potentially be valuable in the assessment of response to treatment and prognosis in patients with iPAH. Introduction Carcinoid heart disease (NET-CHD) is a frequent and adverse complication of carcinoid syndrome due to right ventricular (RV) failure. Medical therapy alone has a 2-year survival of approximately 20% and while surgical valve replacement is effective in improving symptoms and may increase survival, peri-operative risk remains approximately 15-20%. Transthoracic echocardiography (TTE) is considered the gold standard for assessment of NET-CHD and data on the role of cardiac magnetic resonance imaging (CMR) are limited despite recognised advantages in assessment of the right heart. The aim of this study was to assess the role of CMR in assessment of NET-CHD. Methods This is a retrospective cohort study of 50 consecutive patients with proven NET referred with elevated NT pro-BNP to the European Centre of Excellence for Neuroendocrine Tumours in Birmingham between [2005][2006][2007][2008][2009][2010][2011][2012][2013][2014][2015]. At referral, all subjects underwent comprehensive left ventricular (LV) and RV assessment with CMR (1.5T Siemens Avanto), including deformation (Tissue Tracking, cvi42 ® Circle Cardiovascular Imaging), and late gadolinium enhancement (LGE). Results In total, 36 patients were diagnosed with NET-CHD and 14 without (CHD-neg). Right sided valve disease was universal in NET-CHD: severe tricupid regurgitation (97%), severe pulmonary regurgitation (86%). On CMR, RV end-diastolic volume (EDV) and end-systolic volume (ESV) were increased (120 ± 30ml/m 2 vs. 67 ± 14 ml/m 2 , p < 0.01; 49 ± 20ml/m 2 vs. 11 ± 3ml/m 2 , p < 0.01) but with no difference in RVEF (60 ± 14% vs. 60 ± 9% p = 0.92). There was early evidence of ventricular-ventricular interaction, with reduction in both LVEDV (53 ± 16ml/m 2 vs. 72 ± 16 ml/ m 2 , p < 0.01) and LVESV (19 ± 10 ml/m 2 vs. 28 ± 16ml/ m 2 , p < 0.05) in NET-CHD, but no difference in LV ejection fraction (67 ± 8% vs. 63 ± 14%, p = 0.3). There was no difference in LV global longitudinal strain (GLS) or circumferential strain (GCS) between groups. RV LGE indicative of endocardial plaques was present in 6/36 (17%) but not observed in CHD-neg. Diffuse LV LGE was present 5 NET-CHD patients. Over f...

Background Patients with prosthetic heart valves (PHV) require long-term follow-up, usually within a physiologist led heart valve surveillance clinic. These clinics are well established providing safe and effective patient care. The disruption of the COVID-19 pandemic on services has increased wait times thus we undertook a service evaluation to better understand the patients currently within the service and PHV related complications. Methods A clinical service evaluation of the heart valve surveillance clinic was undertaken to assess patient demographics, rates of complications and patient outcomes in patients who had undergone a PHV intervention at our institute between 2010 and 2020. Results A total of 294 patients (mean age at time of PHV intervention: 71 ± 12 years, 68.7% male) were included in this service evaluation. Follow-up was 5.9 ± 2.7 years (range: 10 years). 37.1% underwent baseline transthoracic echo (TTE) assessment and 83% underwent annual TTE follow-up. Significant valve related complications were reported in 20 (6.8%) patients. Complications included a change in patient functional status secondary to significant PHV regurgitation (0.3%) or stenosis (0.3%), PHV thrombosis (0.3%) or infective endocarditis (3.7%). Significant valve related complications resulted in ten hospital admission (3.4%), two re-do interventions (0.6%), and four deaths (1.3%). Conclusions This service evaluation highlights the large number of patients requiring ongoing surveillance. Only a small proportion of patients develop significant PHV related complications resulting in a low incidence of re-do interventions and deaths.

IntroductionFabry disease (FD) is an X-linked lysosomal storage disorder caused by enzyme deficiency, leading to glycosphingolipid accumulation. Cardiac accumulation triggers local tissue injury, electrical instability and arrhythmia. Bradyarrhythmia and atrial fibrillation (AF) incidence are reported in up to 16% and 13%, respectively.ObjectiveWe conducted a systematic review evaluating AF burden and bradycardia requiring permanent pacemaker (PPM) implantation and report any predictive risk factors identified.MethodsWe conducted a literature search on studies in adults with FD published from inception to July 2019. Study outcomes included AF or bradycardia requiring therapy. Databases included Embase, Medline, PubMed, Web of Science, CINAHL and Cochrane. The Risk of Bias Agreement tool for Non-Randomised Studies (RoBANS) was utilised to assess bias across key areas.Results11 studies were included, eight providing data on AF incidence or PPM implantation. Weighted estimate of event rates for AF were 12.2% and 10% for PPM. Age was associated with AF (OR 1.05–1.20 per 1-year increase in age) and a risk factor for PPM implantation (composite OR 1.03). Left ventricular hypertrophy (LVH) was associated with AF and PPM implantation.ConclusionEvidence supporting AF and bradycardia requiring pacemaker implantation is limited to single-centre studies. Incidence is variable and choice of diagnostic modality plays a role in detection rate. Predictors for AF (age, LVH and atrial dilatation) and PPM (age, LVH and PR/QRS interval) were identified but strength of association was low. Incidence of AF and PPM implantation in FD are variably reported with arrhythmia burden likely much higher than previously thought.PROSPERO databaseCRD42019132045.