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  • 1
    Online Resource
    Online Resource
    Cardiac magnetic resonance diagnosis of Fabry disease leads to incidental diagnosis of Klinefelter syndrome: a case report
    Oxford University Press (OUP) ; 2022
    In:  European Heart Journal - Case Reports Vol. 6, No. 9 ( 2022-09-05)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 6, No. 9 ( 2022-09-05)
    Abstract: Fabry disease is an X-linked lysosomal storage disorder resulting in deficient activity of alpha-galactosidase. Males are general more severely affected however heterozygous females can variably express the disease depending on the degree of random X chromosome inactivation (Lyonization). We present a case where cardiac magnetic resonance diagnosis of late onset Fabry Disease leads to an incidental diagnosis of Klinefelter syndrome. Case summary A 55-year-old male was referred for cardiology assessment after developing atrial fibrillation. Echocardiography demonstrated moderate, concentric LVH (left ventricular hypertrophy). Cardiac magnetic resonance imaging confirmed the presence of concentric increase in LV wall thickness and increased LV (left ventricular) mass. T1 mapping values (Shortened Modified Look-Locker Inversion recovery sequences) were elevated in the basal-mid inferolateral segments and low in the remaining segments. Late gadolinium acquisition showed a pattern suggestive of Fabry disease. Genetic testing of the GLA gene revealed a null variant classified as pathogenic. The variant was found to be heterozygous. This raised the possibility of Klinefelter's syndrome and the diagnosis was confirmed by chromosomal microarray and karyotype. The patient was then referred to Fabry clinic for consideration of enzyme replacement therapy and to the endocrine clinic for testosterone replacement. Discussion The atypical ’cardiac variant‘ Fabry disease should be included in differential diagnosis of left ventricular hypertrophy. In a ‘late onset’ presentation of Fabry Disease, the concomitant presence of Klinefelter syndrome cannot be excluded due to GLA variant present in the heterozygous state.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytac328
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 2948381-5
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  • 2
    Online Resource
    Online Resource
    A case report of profound atrioventricular block in an endurance athlete: how far do you go?
    Oxford University Press (OUP) ; 2022
    In:  European Heart Journal - Case Reports Vol. 6, No. 5 ( 2022-05-04)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 6, No. 5 ( 2022-05-04)
    Abstract: Athletes presenting with 1st-degree atrioventricular block (AVB) on 12-lead electrocardiogram (ECG) may present a diagnostic conundrum, especially when significantly prolonged and associated with higher degrees of block. A pragmatic stepwise approach to the evaluation of these patients is, therefore, crucial. Case summary A 19-year-old waterpolo player was referred for assessment of a 1st-degree heart block and one isolated episode of syncope. All other cardiac investigations were within normal limits except for a 24-h ambulatory ECG which showed Mobitz 1 AVB and episodes of 2:1 block occurring in the context of Wenchebach. An electrophysiological study (EPS) was performed which effectively excluded infranodal conductive tissue disease, confirming physiological intranodal block. Discussion The increase in vagal tone is one of the physiological adaptations to an increased demand in cardiac output in athletes, which explains the presence of 1st-degree AVB in up to 7.5% of athletes. The presence of 2:1 AVB on 24 h ECG raises doubts whether the 1st-degree AVB on resting ECG is pathological or physiological, especially considering this particular patient had suffered an episode of syncope. When this diagnostic uncertainty persists despite non-invasive investigations, including cardiopulmonary exercise testing, invasive EPS may be required to assess the refractoriness of the AV node and at what level within the cardiac conductive system block occurs. The electrophysiological study can effectively rule out infranodal disease by confirming physiological intranodal block using incremental atrial pacing.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytac190
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 2948381-5
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  • 3
    Online Resource
    Online Resource
    Abiotrophia defectiva endocarditis of bicuspid aortic valve: a case report
    Oxford University Press (OUP) ; 2022
    In:  European Heart Journal - Case Reports Vol. 6, No. 10 ( 2022-10-08)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 6, No. 10 ( 2022-10-08)
    Abstract: Patients with a bicuspid aortic valve have increased risk of infective endocarditis, but common organisms are not always the culprit. We describe a case of an otherwise healthy young gentleman with bicuspid aortic valve who experienced Abiotrophia defectiva endocarditis. The aim of this case report is to highlight an uncommon cause of endocarditis associated with significant morbidity and mortality in order to improve the care provided by trainees and clinicians. Case summary A 37-year-old male presented with a 1-month history of fever, weight loss, myalgia, and night sweats. On transoesophageal echocardiography, he was found to have a bicuspid aortic valve with large vegetation and severe aortic insufficiency. Blood cultures were positive for A. defectiva. The endocarditis was successfully treated with surgical aortic valve replacement and 6 weeks of antibiotic therapy. Discussion Bicuspid valves are known to have increased susceptibility to endocarditis. The difficulty of isolating A. defectiva typically leads to delayed diagnosis and significant complications. This case is a reminder to have a high degree of suspicion for organisms which are rare and difficult to isolate because prompt recognition and surgical intervention may improve the outcome of care.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytac394
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 2948381-5
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  • 4
    Online Resource
    Online Resource
    Pitfalls for the non-invasive diagnosis of wild-type transthyretin amyloid cardiomyopathy in a young adult: a case report
    Oxford University Press (OUP) ; 2023
    In:  European Heart Journal - Case Reports Vol. 7, No. 7 ( 2023-07-03)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 7, No. 7 ( 2023-07-03)
    Abstract: Using technetium (Tc)-labelled pyrophosphate (PYP) cardiac scintigraphy, a non-invasive diagnosis of transthyretin amyloid (ATTR) cardiomyopathy can be made without histopathological confirmation. In patients suspected of ATTR cardiomyopathy, however, atypical presentations may necessitate further investigation. Case summary A 30-year-old man with hypertension and end-stage renal disease on peritoneal dialysis presented with progressive exertional dyspnoea. Left ventricular hypertrophy (LVH) with a maximal end-diastolic wall thickness up to 16 mm was detected on echocardiography. Speckle-tracking analysis revealed a reduced longitudinal strain of left ventricle with a relative apical sparing pattern. Although the absence of monoclonal gammopathy, a grade 3 myocardial uptake in 99mTc-PYP cardiac scintigraphy, and negative TTR gene mutation inferred the diagnosis of wild-type ATTR, the relative youth of the patient still raised concerns regarding the diagnosis. Under clinical doubt, he underwent further testing. In non-contrast cardiac magnetic resonance (CMR) with native T1 mapping, the native T1 myocardial value was within the normal range. In endomyocardial biopsy (EMB), there was no evidence of amyloid deposition, negative Congo red staining, and no immunohistochemical evidence of transthyretin expression. These results excluded the diagnosis of ATTR cardiomyopathy and prevented subsequent unnecessary treatments. Discussion When patients with unexplained LVH meet the non-invasive diagnostic criteria for ATTR cardiomyopathy, an EMB should be considered in selected cases. Patients presenting at an atypical age for wild-type ATTR cardiomyopathy, absence of extracardiac symptoms/signs or classic electrocardiogram features for cardiac amyloidosis should be suspected of another diagnosis and require further CMR or EMB to confirm.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytad308
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
    detail.hit.zdb_id: 2948381-5
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  • 5
    Online Resource
    Online Resource
    Multivessel coronary spasm triggered by ganglionated plexi stimulation during atrial fibrillation radiofrequency catheter ablation: a case report
    Oxford University Press (OUP) ; 2023
    In:  European Heart Journal - Case Reports Vol. 7, No. 2 ( 2023-02-03)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 7, No. 2 ( 2023-02-03)
    Abstract: Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia in adults, and it is associated with a high burden of mortality and morbidity worldwide. AF can be managed with rate-control or rhythm-control strategies. The latter is increasingly used to improve symptoms and prognosis in selected patients, especially after the development of catheter ablation. Although this technique is generally considered safe, it is not free from rare but life-threatening procedure-related adverse events. Among these, coronary artery spasm (CAS) is an uncommon but potentially fatal complication that requires immediate diagnosis and treatment. Case summary We report a case of severe multivessel CAS triggered by ganglionated plexi stimulation during pulmonary vein isolation with radiofrequency catheter ablation in a patient with persistent AF, promptly resolved after intracoronary nitrate administration. Discussion Although rare, CAS is a serious complication of AF catheter ablation. Immediate invasive coronary angiography is key for both diagnosis confirmation and treatment of such dangerous condition. As the number of invasive procedures increases, it is important that both interventional and general cardiologists are aware of possible procedure-related adverse events.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytad007
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
    detail.hit.zdb_id: 2948381-5
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  • 6
    Online Resource
    Online Resource
    Preoperative planning using virtual reality for percutaneous transseptal valve-in-valve transcatheter mitral valve replacement: a case report
    Oxford University Press (OUP) ; 2022
    In:  European Heart Journal - Case Reports Vol. 6, No. 10 ( 2022-10-08)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 6, No. 10 ( 2022-10-08)
    Abstract: Virtual reality (VR) technology has been implemented as a pre-procedural planning tool for cardiovascular interventions to enable detailed evaluation of patient anatomy from different vantage points. Here, we employed a VR platform to preoperatively plan for percutaneous valve-in-valve transcatheter mitral replacement (ViV-TMVR) in a prohibitive surgical candidate. Case summary An 85-year-old male with a history of two prior sternotomies for bioprosthetic aortic valve (AV) and mitral valve (MV) 31 mm Medtronic Mosaic bioprosthesis presented with severe mitral regurgitation from a degenerative bioprosthetic MV. The patient was deemed a prohibitive surgical candidate for a third sternotomy and instead was recommended a percutaneous transseptal ViV-TMVR. An electrocardiogram-gated chest computed tomography (CT) provided a neo-left-ventricular outflow tract (neo-LVOT) of 1.89 cm2. This CT was reconstructed to create a 360° VR (360VR) model. A 29 mm SAPIEN three bioprosthetic valve, selected based on the already implanted MV, was placed inside the bioprosthetic MV and analysed in VR at different angles to ensure it would not obstruct the LVOT. The neo-LVOT measured in VR was 3.02 cm2, which would allow for sufficient blood flow without significant obstruction from the new SAPIEN three bioprosthetic valve. The patient tolerated the procedure well. Discussion This case demonstrates the utility of VR as a pre-procedural planning tool for interventional cardiology procedures. Preoperative planning in VR alleviated concerns regarding obstruction of the neo-LVOT and helped confirm safe implantation by clearly showing the three-dimensional spatial relationship between the implants and surrounding patient anatomy.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytac384
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
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  • 7
    Online Resource
    Online Resource
    Nicotinamide Adenine Dinucleotide Phosphate Oxidases in Glucose Homeostasis and Diabetes-Related Endothelial Cell Dysfunction
    MDPI AG ; 2021
    In:  Cells Vol. 10, No. 9 ( 2021-09-04), p. 2315-
    Details
    In: Cells, MDPI AG, Vol. 10, No. 9 ( 2021-09-04), p. 2315-
    Abstract: Oxidative stress within the vascular endothelium, due to excess generation of reactive oxygen species (ROS), is thought to be fundamental to the initiation and progression of the cardiovascular complications of type 2 diabetes mellitus. The term ROS encompasses a variety of chemical species including superoxide anion (O2•−), hydroxyl radical (OH−) and hydrogen peroxide (H2O2). While constitutive generation of low concentrations of ROS are indispensable for normal cellular function, excess O2•− can result in irreversible tissue damage. Excess ROS generation is catalysed by xanthine oxidase, uncoupled nitric oxide synthases, the mitochondrial electron transport chain and the nicotinamide adenine dinucleotide phosphate (NADPH) oxidases. Amongst enzymatic sources of O2•− the Nox2 isoform of NADPH oxidase is thought to be critical to the oxidative stress found in type 2 diabetes mellitus. In contrast, the transcriptionally regulated Nox4 isoform, which generates H2O2, may fulfil a protective role and contribute to normal glucose homeostasis. This review describes the key roles of Nox2 and Nox4, as well as Nox1 and Nox5, in glucose homeostasis, endothelial function and oxidative stress, with a key focus on how they are regulated in health, and dysregulated in type 2 diabetes mellitus.
    Type of Medium: Online Resource
    ISSN: 2073-4409
    URL: Article
    DOI: 10.3390/cells10092315
    Language: English
    Publisher: MDPI AG
    Publication Date: 2021
    detail.hit.zdb_id: 2661518-6
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  • 8
    Online Resource
    Online Resource
    Eosinophilic myocarditis complicated by permanent atrioventricular nodal block: a case report
    Oxford University Press (OUP) ; 2022
    In:  European Heart Journal - Case Reports Vol. 6, No. 2 ( 2022-02-03)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 6, No. 2 ( 2022-02-03)
    Abstract: Eosinophilic myocarditis (EM) is a rare disease with different clinical pictures and disease courses. Little literature is available on the various courses of the disease. Case summary A previously healthy 44-year-old male patient presented with acute heart failure and developed complete atrioventricular (AV) block requiring pacing. Acute heart failure was managed with inotropic support, non-invasive ventilation, and implantation of a permanent AV-sequential pacemaker. Cardiac magnetic resonance imaging was suggestive of myocarditis and endomyocardial biopsy diagnosed EM histologically. Endomyocardial biopsy was essential for definite aetiologic assignment, thus dispelling initial reservations about immunosuppressive therapy. Final treatment strategy consisted of steroids and Azathioprine. Discussion Endomyocardial biopsy is essential to establish diagnosis and targeted treatment in EM, which can rapidly lead to life-threatening conditions. Left ventricular function recovered within 2 weeks in response to immunosuppression and the patient was consistently well during follow-up. Despite the otherwise good response to immunosuppression, complete AV block continued over time.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytac055
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 2948381-5
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  • 9
    Online Resource
    Online Resource
    A long-term follow-up of necrobiotic xanthogranuloma with concomitant large-vessel vasculitis and heart failure with reduced ejection fraction: a case report
    Oxford University Press (OUP) ; 2023
    In:  European Heart Journal - Case Reports Vol. 7, No. 10 ( 2023-10-05)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 7, No. 10 ( 2023-10-05)
    Abstract: Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis and multisystem disorder. Low level of HDL cholesterol associated with a systemic inflammatory profile, which may result from the interaction of monoclonal immunoglobulin and lipoproteins, is a characteristic feature. There is no evidence of NXG-associated large-vessel vasculitis, nor are there any established treatments, although chemotherapy for comorbid multiple myeloma is most often administered. Case summary We describe a case of a 53-year-old male with a first history of heart failure with impaired systolic function. He presented with orbital xanthomas and multiple subcutaneous nodules, and laboratory examination showed elevated levels of C-reactive protein, low HDL, and paraproteinemia. A constellation of these clinical features and pathological findings of skin biopsy led to the diagnosis of NXG. 18F-Fluorodeoxyglucose positron emission tomography (PET)/computed tomography (CT) confirmed increased uptake in the aorta and bilateral common carotid arteries. He began prednisolone treatment with reference to treatment for large-vessel vasculitis. After the treatment, C-reactive protein immediately decreased with markedly increased levels of apolipoprotein A1 (Apo-A1) and HDL. Systolic dysfunction was restored at 6-month follow-up. The patient has not experienced heart failure for 5 years after treatment, and the follow-up PET/CT demonstrated resolution of vascular inflammation. Discussion This is the first report of NXG-associated large-vessel vasculitis. Low-dose prednisolone may benefit for NXG-associated vasculitis and cardiomyopathy. HDL, Apo-A1, and C-reactive protein levels may be useful for monitoring the activity of NXG, and PET/CT was a valuable diagnostic tool for NXG-associated vasculitis.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytad469
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
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  • 10
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    Online Resource
    Electrical storm in hypertrophic cardiomyopathy: Cardiac magnetic resonance and sudden cardiac death risk stratification: a case report
    Oxford University Press (OUP) ; 2022
    In:  European Heart Journal - Case Reports Vol. 7, No. 1 ( 2022-12-27)
    Details
    In: European Heart Journal - Case Reports, Oxford University Press (OUP), Vol. 7, No. 1 ( 2022-12-27)
    Abstract: Risk stratification for sudden cardiac death (SCD) is a key factor in the management of patients with hypertrophic cardiomyopathy (HCM). Cardiac magnetic resonance (CMR) has a unique role in the evaluation of HCM and offers superior diagnostic and prognostic information to assess the indication for a prophylactic implantable cardioverter-defibrillator (ICD). Case summary A 39-year-old patient with non-obstructive HCM with a low ESC HCM Risk-SCD score underwent a CMR revealing a left ventricular apical aneurysm and extensive late gadolinium enhancement; a prophylactic ICD was thus implanted. A month later, the patient was admitted in refractory electrical storm with over 50 appropriate ICD shocks due to sustained ventricular tachycardia. Despite anti-arrhythmic therapy and mechanical ventilation, the evolution was unfavourable with haemodynamic instability; veno-arterial extracorporeal membrane oxygenation was implanted. The patient was submitted to CMR-guided epicardial VT catheter ablation with complications of LV thrombus and severe pericardial effusion. Discussion This case details the complex risk stratification for SCD in patients with HCM, highlighting the important role of CMR in the integrated approach to risk stratification.
    Type of Medium: Online Resource
    ISSN: 2514-2119
    URL: Article
    DOI: 10.1093/ehjcr/ytad010
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
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