Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy

Background Fabry disease (FD) is a treatable X-linked condition leading to progressive cardiac disease, arrhythmia and premature death. We aimed to increase awareness of the arrhythmogenicity of Fabry cardiomyopathy, by comparing device usage in patients with Fabry cardiomyopathy and sarcomeric HCM. All Fabry patients with an implantable cardioverter defibrillator (ICD) implanted in the UK over a 17 year period were included. A comparator group of HCM patients, with primary prevention ICD implantation, were captured from a regional registry database. Results Indications for ICD in FD varied with 72% implanted for primary prevention based on multiple potential risk factors. In FD and HCM primary prevention devices, arrhythmia occurred more frequently in FD over shorter follow-up (HR 4.2, p < 0.001). VT requiring therapy was more common in FD (HR 4.5, p = 0.002). Immediate shock therapy for sustained VT was also more common (HR 2.5, p < 0.001). There was a greater burden of AF needing anticoagulation and NSVT in FD (AF: HR 6.2, p = 0.004, NSVT: HR 3.1, p < 0.001). Conclusion This study demonstrates arrhythmia burden and ICD usage in FD is high, suggesting that Fabry cardiomyopathy may be more ‘arrhythmogenic’ than previously thought. Existing risk models cannot be mutually applicable and further research is needed to provide clarity in managing Fabry patients with cardiac involvement.

Cardiac Magnetic Resonance Findings and Prognosis in Type 1 Myotonic Dystrophy

Purpose Cardiac involvement is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), but limited information is available about myocardial remodelling and tissue changes. Aim of the study was to investigate cardiac magnetic resonance (CMR) findings and their prognostic significance in DM1. Methods We identified all DM1 patients referred from a neurology unit to our CMR laboratory from 2009 to 2020. Results Thirty-four patients were included (aged 45±12, 62% males). At CMR, 5(15%) had a left ventricular ejection fraction (LVEF)<50% and 4(12%) a right ventricular ejection fraction (RVEF)<50%. Compared to age- and sex-specific reference values, 12(35%) had a decreased end-diastolic volume index (LVEDVi), 7(21%) a decreased mass index (LVMi), and 29(85%) a reduced LVMi/LVEDVi. Nine (26%) showed mid-wall late gadolinium enhancement (LGE; 5±2% of LVM), and 14(41%) fatty infiltration. In a subset of 13(38%) patients, native T1 in the interventricular septum (1,041±53 ms) approached the upper reference limit (1,089 ms) and the extracellular volume was slightly increased (33±2%, reference<30%). Over 2.5(1.5-4.0) years, 2(6%) patients died for infectious and respiratory complications, 5(15%) underwent device implantation; 4/21(19%) with Holter developed repetitive ventricular ectopic beats (VEBs). Lower RV volumes (p=0.043), higher anteroseptal wall thickness (p=0.024) and LV fatty infiltration (p=0.029) were associated with device implantation, LGE mass was associated with VEBs (p=0.003) and death (p<0.001). Conclusion DM1 patients display structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis and fatty infiltration. Such changes, as evaluated by CMR, may anticipate the worsening of electrical disturbances.

Bio-Compatible Sensor for Middle Ear Pressure Monitoring on a Bio-Degradable Substrate

Hypotension in the middle ear can cause serious diseases and hearing disorders. Until now, pressure in the middle ear is measured indirectly by using the impedance of the tympanic membrane (tympanometry). Direct methods are just described in scientific studies and would be harmful in clinical routine. Here, we demonstrate a bio-compatible pressure sensor, which can resolve pressure changes in the range of −7.5 kPa up to +7.5 kPa, and due to its compact design (area of 2 × 4 mm2), can be directly implanted in the human middle ear. Furthermore, the read-out of the pressure sensor can be conveniently done using wireless data communication technologies employing a plate capacitor with an elastic dielectric for pressure monitoring and a planar coil. Thus, our sensor allows for direct pressure measurements in the middle ear, avoiding additional surgeries after device implantation.

Treatment of Unique Bilateral Distal Fusiform Superior Cerebellar Artery Aneurysms with Mini-Flow Diverter Device Implantation: Case Report

Currently, surgical revascularization procedures using intracranial–intracranial (IC-IC) or extracranial–intracranial (EC-IC) bypass and distal clipping or trapping are the valid and rescue treatment modality for extremely rare unilateral distal fusiform superior cerebellar artery (SCA) aneurysms. Yet, in case of bilateral fusiform SCA aneurysms, surgical therapy reaches its limit. Mini-flow diverter devices (FDDs) have only recently become available for treating fusiform aneurysms of such small vessels. We report the unique case of bilateral distal fusiform SCA aneurysms in a 43-year-old man with subarachnoid hemorrhage (Fisher grade IV and World Federation of Neurosurgical Societies [WFNS] grade II) treated with endovascular implantation of bilateral mini-FDDs with excellent outcome and no radiographic signs of infarction. Yet, occlusion of one of the FDDs was found in the follow-up, which again shows the eminent danger of occlusion in case of an implantation of FDDs in such small-caliber vessels, which leaves the discussion about the optimal therapy method open.