Contemporary management of patients with atrial fibrillation in the Netherlands and Belgium: a report from the EORP-AF long-term general registry.

BACKGROUND: Contemporary data regarding the characteristics, treatment and outcomes of patients with atrial fibrillation (AF) are needed. We aimed to assess these data and guideline adherence in the EURObservational Research Programme on Atrial Fibrillation (EORP-AF) long-term general registry. METHODS: We analysed 967 patients from the EORP-AF long-term general registry included in the Netherlands and Belgium from 2013 to 2016. Baseline and 1­year follow-up data were gathered. RESULTS: At baseline, 887 patients (92%) received anticoagulant treatment. In 88 (10%) of these patients, no indication for chronic anticoagulant treatment was present. A rhythm intervention was performed or planned in 52 of these patients, meaning that the remaining 36 (41%) were anticoagulated without indication. Forty patients were not anticoagulated, even though they had an indication for chronic anticoagulation. Additionally, 63 of the 371 patients (17%) treated with a non-vitamin K antagonist oral anticoagulant (NOAC) were incorrectly dosed. In total, 50 patients (5%) were overtreated and 89 patients (9%) were undertreated. However, the occurrence of major adverse cardiac and cerebrovascular events (MACCE) was still low with 4.2% (37 patients). CONCLUSIONS: Overtreatment and undertreatment with anticoagulants are still observable in 14% of this contemporary, West-European AF population. Still, MACCE occurred in only 4% of the patients after 1 year of follow-up.

Technical Feasibility and Design of a Shape Memory Alloy Support Device to Increase Ejection Fraction in Patients with Heart Failure.

PURPOSE: Heart failure is increasingly prevalent in the elderly. Treatment of patients with heart failure aims at improving their clinical condition, quality of life, prevent hospital (re)admissions and reduce mortality. Unfortunately, only a select group of heart failure patients with reduced ejection fraction are eligible for Cardiac Resynchronization Therapy where 30-40% remain non-responders and need left ventricular support. The aim of this study is to investigate if a shape memory alloy (SMA) is able to increase the ejection fraction of a mono-chamber static heart model by 5%. METHODS: A pediatric ventilation balloon was used as a heart model (mono-chamber). Flexinol®, a SMA, was placed around the heart model in multiple configurations and activated using pulse width modulation techniques to determine influence of diameter and configuration on volume displacement. Furthermore, pressure within the heart model was measured with a custom-made pressure sensor. RESULTS: SMA with a diameter of 0.38 mm, placed in a spiral shape and activated with a duty cycle of 80% and a frequency of 50/min gave the highest ejection fraction increase of 3.5%. CONCLUSIONS: This study demonstrated the feasibility of volume displacement in a static heart model by activation of SMA-wires. Configuration, duty cycle, frequency, pulse intervals and diameter were identified as important factors affecting the activation of SMA-wires on volume displacement. Future research should include the use of parallel SMA-wires, prototype testing in dynamic or ex vivo bench models.

Coronary artery-bronchial artery fistulas: report of two Dutch cases with a review of the literature.

BACKGROUND: Coronary bronchial artery fistulas (CBFs) are rare anomalies, which may be isolated or associated with other disorders. MATERIALS AND METHODS: Two adult patients with CBFs are described and a PubMed search was performed using the keywords "coronary bronchial artery fistulas" in the period from 2008 to 2013. RESULTS: Twenty-seven reviewed subjects resulting in a total of 31 fistulas were collected. Asymptomatic presentation was reported in 5 subjects (19 %), chest pain (n = 17) was frequently present followed by haemoptysis (n = 7) and dyspnoea (n = 5). Concomitant disorders were bronchiectasis (44 %), diabetes (33 %) and hypertension (28 %). Multimodality and single-modality diagnostic strategies were applied in 56 % and 44 %, respectively. The origin of the CBFs was the left circumflex artery in 61 %, the right coronary artery in 36 % and the left anterior descending artery in 3 %. Management was conservative (22 %), surgical ligation (11 %), percutaneous transcatheter embolisation (30 %), awaiting lung transplantation (7 %) or not reported (30 %). CONCLUSIONS: CBFs may remain clinically silent, or present with chest pain or haemoptysis. CBFs are commonly associated with bronchiectasis and usually require a multimodality approach to be diagnosed. Several treatment strategies are available. This report presents two adult cases with CBFs and a review of the literature.

Diagnostic and therapeutic approach of congenital solitary coronary artery fistulas in adults: Dutch case series and review of literature.

BACKGROUND: Coronary artery fistulas (CAFs) are infrequent anomalies, coincidentally detected during coronary angiography (CAG). AIM: To elucidate the currently used diagnostic imaging modalities and applied therapeutic approaches. MATERIALS AND METHODS: Five Dutch patients were found to have CAFs. A total of 170 reviewed subjects were subdivided into two comparable groups of 85 each, treated with either percutaneous 'therapeutic' embolisation (PTE group) or surgical ligation (SL group). RESULTS: In our series, the fistulas were visualised with several diagnostic imaging tests using echocardiography, multidetector computed tomography, and CAG. Four fistulas were unilateral and one was bilateral; five originated from the left and one originated from the right coronary artery. Among the reviewed subjects, high success rates were found in both treatment groups (SL: 97% and PTE: 93%). Associated congenital or acquired cardiovascular disorders were frequently present in the SL group (23%). Bilateral fistulas were present in 11% of the SL group versus 1% of the PTE group. The fistula was ligated surgically in one and abolished percutaneously in another. Medical treatment including metoprolol was conducted in two, and watchful waiting follow-up was performed in one. CONCLUSIONS: Several diagnostic imaging techniques are available for assessment of the anatomical and functional characteristics of CAFs.

Gender-related differences in octogenarians with congenital coronary artery fistula: a report of two cases and a review.

AIM: To highlight gender-related differences in octogenarians with a congenital coronary artery fistula (CAF). MATERIALS AND METHODS: We present two elderly female patients with a congenital fistula, a septuagenarian and a nonagenarian, and review the world literature between 1954-2010. RESULTS: The septuagenarian patient presented with easy fatigability and the nonagenarian patient with acute myocardial infarction contralaterally to the fistula. Coronary angiography (CAG) demonstrated a coronary-pulmonary artery fistula (CPF). The nonagenarian patient underwent percutaneous coronary intervention of the right coronary artery. CAG revealed a CPF associated with a huge multiple aneurysmal formation. Data from 57 mainly symptomatic patients with a mean age of 75.3 years (range 70-87 years) were collected. The cohort was subdivided into female (mean age 84.3 years) and male (mean age 75.2 years) subgroups and compared with each other. Multi-origin (bilateral and multilateral) was prevalent in females, 40% versus 12% in males. Aneurysmal formation was found in females and males in 40% and 18%, respectively. Ethnicity was 65% Caucasian and 35% Asian. Multi-origin fistulas were prevalent in the Asian (45%) compared with the Caucasian (11%) subset. CONCLUSIONS: A septuagenarian and a nonagenarian female patient with congenital CAF are presented. On reviewing the literature, important differences were found between elderly females and males with congenital CAF.

Clinical and diagnostic features of partially anomalous pulmonary venous connection in an adult female patient: a case report and review of the literature.

A 40-year-old woman presented with dyspnoea, chest pain and fatigue. Her medical history was unremarkable. An early systolic ejection murmur was heard in the 3D left inter-costal space. Chest X-ray revealed normal cardiothoracic ratio with an anomalous vessel adjacent to the left pulmonary hilum. Echocardiography and exercise tolerance test were normal. Right heart catheterisation revealed normal pulmonary pressures with normal cardiac output. CT scan and MRI of the thorax were diagnostic for an aberrant pulmonary venous connection between the left lower lobe pulmonary vein and the left brachiocephalic vein without atrial septal defect. She was treated conservatively and remained well.

TWENTE Study: The Real-World Endeavor Resolute Versus Xience V Drug-Eluting Stent Study in Twente: study design, rationale and objectives.

Background. New-generation drug-eluting stents (DES) may solve several problems encountered with first-generation DES, but there is a lack of prospective head-to-head comparisons between new-generation DES. In addition, the outcome of regulatory trials may not perfectly reflect the outcome in 'real world' patients.Objectives. To compare the efficacy and safety of two new-generation DES in a 'real world' patient population.Methods. A prospective, randomised, single-blinded clinical trial to evaluate clinical outcome after Endeavor Resolute vs. Xience V stent implantation. The primary endpoint is target vessel failure at one-year follow-up. In addition, the study comprises a two-year and an open-label five-year follow-up. (Neth Heart J 2010;18:360-4.).

Pulmonary hypertension: the role of the electrocardiogram.

A 54-year-old female was referred to our centre for further evaluation of recently established severe pulmonary hypertension. Six months prior to presentation to the cardiologist of the referring centre, the patient had first experienced exertional dyspnoea. At the time of presentation to the referring cardiologist, the patient's ECG showed signs of an increased right heart load. Interestingly, this patient had undergone a thorough cardiac evaluation in the referring centre seven years before when she presented with severe hyperthyroidism. At that time there were no symptoms or signs of pulmonary hypertension on ECG, echocardiography, or at heart catheterisation. Thorough evaluation in cooperation with the referring centre demonstrated that this patient was suffering from idiopathic pulmonary arterial hypertension, a rare form of pulmonary hypertension. We conclude this report with a discussion on the potential use of the ECG for the diagnosis of increased right heart load. (Neth Heart J 2008;16:250-4.).

[Guideline 'Precautionary measures for contrast media containing iodine']. / Richtlijn 'Voorzorgsmaatregelen bij jodiumhoudende contrastmiddelen'.

Annually, 0.5-1 million injections of contrast media containing iodine are administered in the Netherlands. Almost all contrast media nowadays are low-osmolar and nonionic. Nevertheless, the development ofcontrast-induced nephropathy is still a relevant clinical problem. Through an initiative by the Radiological Society of the Netherlands and with aid of the Dutch Institute for Healthcare Improvement (CBO), a guideline was conceived for the intravascular use of iodine-containing contrast media, based on recent scientific literature. The guideline defines the risk factors for contrast-induced nephropathy. One of the major risk factors is an impaired renal function. It is important to measure the glomerular filtration rate (GFR) in patients with a possible impaired kidney function, preferably by using the 'Modification of diet in renal disease' (MDRD)-study formula. The key measures for avoidance of contrast nephropathy are: limiting the amount of contrast agent used and to assure good hydration, by infusion of sodium chloride 0.9% 12-16 ml/kg body weight, both prior to and after contrast infusion. If time is limited, intravenous administration of sodium bicarbonate is an option. The guideline recommends discontinuation of metformin use from the day of contrast injection, if the GFR < 60 ml/min/1.73 m2, and to restart metformin 2 days following contrast infusion providing the GFR has not significantly deteriorated. Only in the case of previous moderate or severe adverse reactions to contrast media, prophylaxis with corticosteroids and antihistamines is recommended. Iodine allergy or an atopic condition is not a contraindication for the use of iodine-containing contrast media, and no prophylaxis is required. No specific measures are indicated in case of hyperthyroidism, acute pancreatitis, or phaeochromocytoma. Injection of contrast media is not contraindicated in case of pregnancy or lactation.

Down syndrome associated with hypothyroidism and chronic pericardial effusion: echocardiographic follow-up.

We present a 39-year-old male patient with Down syndrome who was evaluated for fatigue, palpitations and bouts of cyanosis. Physical examination showed features of trisomy-21(Down syndrome), with a slow pulse rate, distant cardiac sounds and absent apex beat. He had normal jugular venous pressure without pulsus paradoxus. The ECG showed QRS microvoltage and flattened P and T segments. The 48-hour ambulatory ECG depicted normal sinus rhythm with intermittent short PR interval without tachyarrhythmias. The chest Xray revealed cardiomegaly without pulmonary venous congestion. Although serial transthoracic echocardiographic examination demonstrated pericardial effusion with features of tamponade, there were no overt signs of clinical cardiac tamponade. Biochemically, the serum thyroxine of 3 pmol/l (normal 10 to 25) and thyroid-stimulating hormone of 160 mU/l (normal 0.20 to 4.20)) were compatible with hypothyroidism. The patient was treated with L-thyroxine sodium daily, which was gradually increased to 0.125 mg daily. Within a few months he lost weight and became more alert; furthermore, the symptoms of hypothyroidism and the pericardial effusion resolved. It can be concluded that Down syndrome may be associated with hypothyroidism and pericardial effusion. These were alleviated following hormone replacement. Regular evaluation of thyroid function tests is important in Down syndrome. (Neth Heart J 2007;15:67-70.).

Feasibility of cardiovascular magnetic resonance of angiographically diagnosed congenital solitary coronary artery fistulas in adults.

OBJECTIVE: To evaluate the use of cardiovascular magnetic resonance (CMR) to visualize angiographically-detected congenital coronary artery fistulas in adults. METHODS: CMR techniques were used to study 13 patients, recruited from the Dutch Registry, with previously angiographically diagnosed fistulas. RESULTS: Coronary fistulas were detected in 10 of 13 (77%) patients by CMR and, retrospectively, in two (92%) more. In 93% of these, it was possible to determine the origin and the outflow site of the fistulas. Cardiovascular magnetic resonance allowed demonstration of dilatation of the fistula-related coronary artery in all cases. Tortuosity of fistulas was detected in all visualized patients. Uni-or bilaterality of fistulas as seen on CAG was proven on CMR in all patients. Flow measurement could be performed in 8 patients. A fairly good correlation (r = 0.72) was found between angiographic (mean 6.2 mm, range 1-16) and cardiovascular magnetic resonance (mean 6.3 mm, range 3-15) measured fistulous diameters. CONCLUSIONS: Cardiovascular magnetic resonance of congenital fistulas with clinical significant shunting is feasible and can provide additional physiological data complementary to the findings of conventional coronary angiography.

Dutch survey of congenital coronary artery fistulas in adults: coronary artery-left ventricular multiple micro-fistulas multi-center observational survey in the Netherlands.

BACKGROUND: Congenital coronary artery-left ventricular multiple micro-fistulas (CA-LVMMFs) in adults are rare anomalies. They may cause angina pectoris and myocardial infarction in association with normal coronary arteries. METHODS AND RESULTS: From the medical databases of a Dutch Survey of coronary artery fistulas in adult cardiology population (30,829 patients), we identified 20 patients with CA-LVMMFs out of 71 fistula-subjects between 1996 and 2003. Clinical files and individual coronary angiograms were reviewed and analysed. There were 13 females and 7 males with a mean age of 67.3 years (range 49-82). The main presenting symptoms were angina pectoris and dyspnea in 70% of the patients. The ECG showed pathologic changes in 75%. Exercise tolerance test and 201-thallium stress scintigraphy were positive for myocardial ischemia in 29% and 50%, respectively of the tested patients. In the absence of significant atherosclerotic coronary artery disease, ipsilateral to the fistulas, myocardial infarction was documented in 15% of the patients. Chest X-ray revealed cardiomegaly in 38% of the patients. Congestive heart failure was documented in 10% of the patients. Uni-, bi- and multilateral fistulas were present in 50%, 45% and 5%, respectively. The origin was the LCA in 71% and the RCA in 29% of the fistulas. The majority (97%) originated from the mid or distal segments of the coronary vessels. Among those patients, the coronary arterial tree had single, dual, and triple vessel disease in 25%, 15% and 5%, respectively. Angiographic anatomy precludes surgical intervention; they were all followed by conservative medical management. CONCLUSIONS: Coronary artery-left ventricular multiple micro-fistulas are found more often in female patients. Furthermore, they originated from the distal segment of the coronary arteries. Coronary artery-left ventricular multiple micro-fistulas, in the presence of normal coronary arterial tree, may often lead to angina pectoris and coronary insufficiency.

Dutch survey of coronary artery fistulas in adults: congenital solitary fistulas.

AIMS: Congenital coronary artery fistulas are frequently identified in adult and pediatric populations and they have been associated with various clinical and morphological features. The purpose of this study was to define the clinical and coronary angiographic morphological characteristics of adult patients with congenital solitary CAFs in the Dutch Registry. METHODS AND RESULTS: Fifty-one patients with angiographically documented CAFs were reviewed for clinical evaluation, used non-invasive and invasive diagnostic tools and treatment modalities. Unilateral CAFs were predominant (80%) and 84% of the patients were symptomatic. The most common presenting symptom was angina pectoris (57%). Angina pectoris was present in a quarter of the patients in the absence of coronary artery disease (CAD). Significant CAD was present in 49% of the patients. Twenty-nine percent of the CAFs showed aneurysmal formation, underlying their potential hazard of rupture. Myocardial infarction occurred in 18% of the patients. In 27% of CAFs multiplicity of the origin was found and nearly all fistulas were tortuous (97%). Treatment modalities were conservative medical in 70%, percutaneous transluminal embolisation in 5% and surgical ligation in 25% of the cases. Multiple micro-fistulas from the coronary arteries to the left ventricle were excluded from the study. CONCLUSION: In this national survey series, congenital solitary CAFs can be presented with typical angina pectoris in the absence of obstructive CAD. Fistula-related coronary artery was infrequently involved in the development of ipsilateral myocardial infarction. Tortuousity and multiplicity of the CAFs may determine, for the individual patient, the choice of the currently available treatment modalities.

Dutch survey of congenital coronary artery fistulas in adults.

AIMS: This Dutch survey focused on the clinical presentation, noninvasive and invasive diagnostic methods, and treatment modalities of adult patients with congenital coronary artery fistulas (CAFs). METHODS: Between 1996 and 2003, the initiative was taken to start a registry on congenital CAFs in adults. In total 71 patients from a diagnostic coronary angiographic population of 30,829 at 28 hospitals were collected from previously developed case report forms. Patient demographic data, clinical presentation, noninvasive and invasive techniques and treatment options were retrospectively collected and analysed. RESULTS: Out of 71 patients with angiographically proven CAFs, 51 (72%) had 63 congenital solitary fistulas and 20 (28%) had 31 congenital coronary-ventricular multiple microfistulas. Patients with pseudofistulas were excluded from the registry. Coronary angiograms were independently re-analysed for morphology and specific fistula details. The majority (72%) of the fistulas were unilateral, 24% were bilateral and only 4% were multilateral. The morphological characteristics of these 94 fistulas were as follows: the origin was multiple in 47% and single in 53%; the termination was multiple in 52% and single in 48%; and the pathway of the fistulous vessels was tortuous/multiple in 66%, tortuous/single in 28%, straight/multiple in 3% and straight/single in 3%. Percutaneous transluminal embolisation (PTE) was performed in two (3%) patients; surgical ligation was undertaken in 13 (18%) patients. The overwhelming majority of the patients (56; 79%) were treated with conservative medical management. The total mortality was 6% (4/71) at a mean follow-up period of approximately five years. Cardiac mortality accounted for 4% (3/71); in all three patients, death could possibly be attributed to the presence of the fistula. CONCLUSION: Registry of congenital coronary artery fistulas in adults in the Netherlands is feasible. In spite of restrictions imposed by the Dutch Privacy Law, it was possible to include 71 adult patients with congenital coronary artery fistulas who were eligible for thorough evaluation.

Pacing for conduction disturbances in Steinert's disease: a new indication for biventricular ICD?

Cardiac involvement in classical Steinert muscular dystrophy (dystrophia myotonica, MD1) is characterised by atrial arrhythmias, AV conduction disturbance, ventricular arrhythmias and heart failure. In MD1 patients complaints of fatigue and reduced exercise tolerance are well explained by the muscular weakness, but the same symptoms can be attributed to arrhythmia, atrioventricular block and heart failure. As cardiac pathology is often encountered in MD1 patients, an ECG, echocardiogram and Holter registrations should be performed on a routine basis. We report on two patients with MD1 who developed Mobitz II block as initial presentation of cardiac disease.

Possible complications of subclavian crush syndrome.

A 70-year-old woman with symptomatic Mobitz type II atrioventricular block underwent implantation of a dual-chamber pacemaker 11 years ago. The leads were inserted through a percutaneous puncture of the right subclavian vein, using standard techniques. Both leads were passive fixation leads. Due to battery failure and end of life criteria, the pulse generator (PG) had been routinely replaced six years previously. Predischarge pacemaker control revealed normal pacing, sensing thresholds and impedance for both leads. Because of a syncopal attack subsequent to lead fractures, most likely secondary to right subclavian crush syndrome (SCS) of both leads, she underwent a double lead re-implantation one year after PG replacement by access via left subclavian vein puncture. After a symptom-free period of few years she was re-analysed because of palpitations, dizziness, angina pectoris and tiredness. Pulmonary embolisation and myocardial perfusion defects were detected utilising scintigraphic techniques. Chest X-ray revealed the crushed atrial lead dislocated from the right subclavian region and lodged into the right ventricle towards the inferior septum. Because she was symptomatic, a retrieval technique was applied and the crushed atrial lead was pulled back from the right ventricle and securely fixed to its former position. On maintenance medical treatment, she remains well.

Neurological symptoms and cerebrovascular accidents: manifestations of left-sided cardiac tumours in adults.

We present two adult patients with a left-sided cardiac tumour in whom the diagnosis was established by transthoracic and transoesophageal echocardiography. They both presented with a cerebrovascular accident. Cardiac surgery for tumour excision was offered but refused by one and successfully performed in the other. In one of the patients, right femoro-crural bypass was undertaken because of arterial insufficiency. The patient who refused surgical intervention died secondary to severe septic shock. In the other patient serial transthoracic and transoesophageal echocardiography showed no tumour recurrence at four years of follow-up post tumour extirpation.

Percutaneous transvenous retrieval of intracardiac port-a-cath catheter fragment: a case report.

A female patient, 36 years of age, with a metastasised left breast cancer received several courses of chemotherapy for aggressive local tumour growth and multiple metastatic activity. In the current patient, surgical ablation of the left breast was carried out. Also loco-regional radio-therapy was conducted. To facilitate the administration of chemotherapy courses and prevent thrombophlebitis a vascular access port (port-a-cath) was surgically inserted via the right subclavian vein. After a few successful administrations of chemotherapeutic drugs the vascular port stopped functioning. It was demonstrated that a detached catheter fragment had dislodged into the right ventricle. Successful percutaneous, transvenous removal of the entrapped catheter fragment by the Gooseneck retrieval loop snare from the right ventricle was performed via the right femoral vein access. The procedure was uncomplicated and the patient tolerated the procedure well.