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.

Background: The exact mechanisms underlying the pathogenesis of myocarditis are not always understood, but there is emerging evidence to suggest that genetic factors may play a significant role. Case summary: Herein, we present six cases in which clinical, biochemical, and cardiovascular magnetic resonance data were consistent with myocarditis, and genetic testing subsequently revealed pathogenic filamin C (FLNC) mutations. Three patients presented with ventricular arrhythmias, two with severe biventricular dysfunction, and two suffered sudden cardiac arrest. Three received an implantable cardioverter defibrillator, and one underwent heart transplantation. Cascade testing was useful in identifying other relatives with FLNC mutation. We also present relevant histology results of myocardial specimens showing the presence of lymphocytic infiltration and inflammation, further supporting the potential association between FLNC mutations and a myocarditis phenotype. Discussion: Genetic testing of affected individuals for FLNC mutations and cascade screening in the setting of acute myocarditis may be considered in selected clinical context, such as in acute myocarditis accompanied by severe left ventricular systolic dysfunction, biventricular failure, significant ventricular arrhythmias, or right ventricular involvement.

The 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. We 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. Macitentan 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.

Background: Rotational atherectomy has become increasingly utilised over the past decade. Although a relatively safe procedure in appropriately trained physicians’ hands, there are a number of recognised complications. Case summary: We describe the case of a 64-year-old female who presented with chest pain and was diagnosed with non-ST-segment elevation acute coronary syndrome. A transthoracic echocardiogram (TTE) showed normal biventricular function and no valve disease. Invasive coronary angiogram was performed which revealed a severely calcified ostial right coronary artery (RCA) disease which was felt to be the culprit of the presentation. Balloon dilatation was unsuccessful, therefore, rotational atherectomy with an Amplatz left 0.75 guide and a 1.5 mm rota-burr was utilised and improved calcium burden. This was complicated by ostial dissection, treated with stenting. A TTE following the procedure revealed moderate aortic regurgitation (AR). The patient was discharged as she remained asymptomatic. An outpatient transoesophageal echocardiogram performed eight months later showed evidence of severe eccentric AR. Cardiac magnetic resonance imaging confirmed severe AR with left ventricular dilatation. Repeat angiogram 10 months after index procedure revealed in-stent restenosis, and the patient was accepted by heart multidisciplinary team for aortic valve replacement and grafting of RCA. Discussion: As the field of rotational atherectomy continues to expand, we propose that novel complications such as reported in this case may become recognised. Finally, we stress the importance of multi-modality imaging in the investigation and timely planning of interventions in the management of these patients.

Hypereosinophilic syndrome (HES) is a rare disorder characterised by eosinophilic infiltration of tissues. Myocardial infiltration occurs in 50%–60% of HES and leads to a condition called Loeffler’s endocarditis. This can lead to endomyocardial injury with resultant superimposed thrombus formation and embolic stroke. We describe the case of a 57-year-old female patient presenting with neurological symptoms who was found to have multiple embolic strokes on a background of long-standing eosinophilia. Following a series of investigations, including transthoracic and transoesophageal echocardiographies and cardiovascular MRI, she was confirmed to have Loeffler’s endocarditis with left ventricular thrombus. She was treated successfully with steroids and anticoagulation. We describe the pathophysiology of HES and Loeffler’s endocarditis and stress the crucial role of multimodality cardiac imaging in establishing its diagnosis and treatment monitoring.