Emergency Card Research Articles

Extensive Expertise in Endocrinology: Adrenal crisis in assisted reproduction and pregnancy.

Appropriate management of adrenal insufficiency in pregnancy is challenging due to the rarity of both primary, secondary and tertiary forms of the disease as well as the lack of evidence-based recommendations to guide clinicians to glucocorticoid and sometimes also mineralocorticoid dosage adjustments. Debut of adrenal insufficiency during pregnancy requires immediate diagnosis as it can lead to adrenal crisis, intrauterine growth restriction and foetal demise. Diagnosis is difficult due to overlap of symptoms of adrenal insufficiency and its crisis with those of pregnancy. Adrenal insufficiency in stable replacement treatment needs careful monitoring during pregnancy to adapt to the physiological changes in the requirement of the adrenal hormones. This is hampered because the diagnostic threshold of most adrenocortical hormones is not applicable during pregnancy. The frequent use of assisted reproduction technology with controlled ovarian hyperstimulation in these patient groups with disease induced low fertility has created an unrecognised risk of adrenal crises due to accelerated oestrogen stimulation with increased risk of even be life-threatening complications for both the woman and foetus. The area needs consensus recommendations between gynaecologists and endocrinologists in tertiary referral centres to alleviate such increased gestational risk. Patient and partner education, use of the EU emergency card for management of adrenal crises can also contribute to better pregnancy outcomes. There is a strong need of more research on e.g. improvement of glucocorticoid replacement as well as crisis management treatment, and biomarkers for treatment optimisation in this field, which suffers from the rare nature of the diseases and poor funding.

Implementation and impact of perioperative guidelines and the steroid emergency card for the management of adrenal insufficiency and prevention of adrenal crisis

Implementation and impact of perioperative guidelines and the steroid emergency card for the management of adrenal insufficiency and prevention of adrenal crisis

National steroid emergency card and delivery of education to specialties within NHS grampian on patients at risk of adrenal insufficiency and prevention of adrenal crisis

National steroid emergency card and delivery of education to specialties within NHS grampian on patients at risk of adrenal insufficiency and prevention of adrenal crisis

Consensus recommendations on organization of care for individuals with Phelan-McDermid syndrome

The manifestations of Phelan-McDermid syndrome (PMS) are complex, warranting expert and multidisciplinary care in all life stages. In the present paper we propose consensus recommendations on the organization of care for individuals with PMS. We indicate that care should consider all life domains, which can be done within the framework of the International Classification of Functioning, Disability and Health (ICF). This framework assesses disability and functioning as the outcome of the individual's interactions with other factors. The different roles within care, such as performed by a centre of expertise, by regional health care providers and by a coordinating physician are addressed. A surveillance scheme and emergency card is provided and disciplines participating in a multidisciplinary team for PMS are described. Additionally, recommendations are provided for transition from paediatric to adult care. This care proposition may also be useful for individuals with other rare genetic neurodevelopmental disorders.

Medical emergency card for Steinert's disease: an unmet need

Myotonic dystrophy type 1 (DM1) or Steinert's disease (ORPHA 273; OMIM #160900) is a rare disorder of genetic origin with muscular manifestations (muscle weakness and myotonia), early-onset cataracts (before 50 years of age) and systemic manifestations (cerebral, endocrine, cardiac, gastrointestinal tract, uterus, skin and immune system). Its clinical expressivity is highly variable and ranges from lethal forms in infancy to mild late-onset disease. Its low frequency prevents emergency medical professionals from becoming familiar with the essential precautions for its treatment. In order to alleviate this lack of information, those affected by DM1 have, in the countries of our environment, a medical emergency card (Tarjeta de Emergencias Medicas, TEM) that the patient should always carry with him/her and give to the physician before receiving emergency care. To define the TEM. To describe the TEM for DM1 already implemented. To list the advantages for patients and professionals of their use. Some of the TEM for DM1 currently in use in France and the United Kingdom are described. The arguments justifying their implantation in our setting are presented in detail. The TEM for DM1 managed by a physician can improve the emergency medical care of patients affected by Steinert's disease.

FACIAL SWELLING, RESPIRATORY FAILURE, FLUCTUATING COMPLEMENT 4 LEVELS, AND RELIGIOUS CONSIDERATION IN A PEDIATRIC PATIENT

<h3>Introduction</h3> Hereditary Angioedema is a rare condition defined by acute, recurring episodes of edema, with the upper airway being particularly dangerous. HAE Type 2 is caused by the dysfunction of the C1-esterase inhibitor with low C4 levels. <h3>Case Description</h3> A 10-year-old female presented with facial and airway swelling unresponsive to steroids, antihistamines, and epinephrine that required intubation. C4 complement was low but then was normal prior to treatment. The patient's religion did not allow for plasma products, but a plasma derived C1 esterase inhibitor product was given emergently with improvement in her swelling. C1 Esterase Inhibitor Function was found to be low. A non-formulary recombinant C1 esterase inhibitor product was arranged for hospital and later home use. At 11 years she was admitted in respiratory failure. She decompensated with failed attempts at intubation and required cricothyrotomy. A recombinant C1 esterase inhibitor product was given. She remained with tracheostomy for 7 days then was de-cannulated. She was discharged with an oral plasma Kallikrein inhibitor, emergency recombinant C1 esterase inhibitor product, and a HAE emergency card. In addition to formulary HAE treatment, a recombinant product was placed in restricted use. A best practice advisory was developed in the EMR to advise physicians of the patient's HAE status. <h3>Discussion</h3> Although HAE Type 2 is well characterized, this is a rare report of a Pediatric patient with HAE requiring intubation on initial presentation and later cricothyrotomy. The challenge was her respiratory failure as a presenting sign of HAE, fluctuating C4 levels, religious consideration, and implementation of an EMR advisory.

Assessing the Knowledge of Sick-Day Rules Among Patients on Long-Term Glucocorticoids and Healthcare Professionals in a UK District General Hospital

Abstract Introduction Adrenal insufficiency can happen because of decreased cortisol production as a result of negative feedback on the hypothalamic–pituitary–adrenal axis, caused by excess exogenous glucocorticoids (GC). The most common cause of adrenal insufficiency is, in fact, abrupt stoppage of exogenous GC. Patients with adrenal insufficiency are at risk of developing life-threatening adrenal crisis if GC is reduced or stopped abruptly, or if GC dose is not increased during periods of increased stress.The adrenal crises should be preventable with education of patients and healthcare professionals (HCPs) about “sick-day” rules, the importance of “steroid cards,” and the utility of parenteral steroids. Methods This study was aimed at assessing the knowledge of sick-day rules in patients on long-term GC as well as HCPs.Patients aged above 18 years on long-term GC presenting to the endocrinology clinic over 6 weeks were included after audit-committee approval. Results We assessed 18 consecutive patients in total, 61% (11/18) of whom said that they have received information about sick-day rules from an HCP. Only 38% (7/18) had access to emergency hydrocortisone-kit.We assessed 36 HCPs. Thirty-six (13/36) percent were aware of steroid emergency card and 50% (18/36) HCPs exhibited awareness regarding steroid dose during acute event and 27% of (10/36) HCPs were aware of accessible hospital intranet resources. Conclusion This study revealed significant lack of awareness of sick-day rules among patients and HCPs alike. Majority of patients failed to carry steroid emergency card or medic alert bracelet and majority of HCPs and patients lacked knowledge of sick-day rules.

P016 Steroid prescribing and risk of adrenal insufficiency: an audit of giant cell arteritis cases

Abstract Background/Aims On 13th August 2020 a joint National Patient Safety Alert (NPSA) was issued regarding the introduction of a new steroid emergency card to support recognition and treatment of adrenal crisis. Providers must ensure patients are assessed regarding their risk of adrenal insufficiency, and where appropriate issued a steroid emergency card. Rheumatology departments frequently prescribe patients long-term courses of steroids as treatment for inflammatory disease, placing these patients at risk of secondary adrenal insufficiency. We identified new giant cell arteritis (GCA) patients as a well-defined patient group at high risk for secondary adrenal insufficiency. As such we decided to audit our steroid prescribing practice in these patients, in light of the NPSA. Methods All new confirmed cases of GCA treated at the Royal United Hospital for Rheumatic Diseases between 1/1/19 and 31/12/20 were identified (n = 90). Standards for audit were developed in accordance with the NPSA advice. In addition to issuing emergency cards we assessed whether patients were informed of the risk of adrenal insufficiency, symptoms suggestive of adrenal insufficiency and sick day rules. A thorough review of all available clinical notes was undertaken for all patients to assess compliance with the above standards. Data collection was completed on 16/8/21. Results No patients were given steroid emergency cards at their first visit; four were given cards during measured follow up. 5/90 patients were given written information about risk of adrenal insufficiency at their first visit; a further six were informed during measured follow up. 1/90 patients were given written information about signs/symptoms of adrenal insufficiency at their first visit; a further six were informed during measured follow. No patients were given written information about sick day rules at their first visit; a further 9 were informed during measured follow up. Qualitative record review suggested that inconsistencies in follow up pathways may have compounded these issues. Conclusion Existing departmental practices to inform GCA patients regarding the risk of secondary adrenal insufficiency, and ensure provision of steroid emergency cards are inadequate. Our audit only measured in house documentation, it is possible that primary care prescribers may also be addressing some of these areas. We have already instigated several measures to address these issues. Our GCA service has been amended as part of a wider service improvement project - frequency of follow has improved and dedicated GCA clinics created. Patients are given standardised written information on steroids and a personalised reduction plan. Education sessions have been provided to medical and nursing teams. Wider trust initiatives including electronic prescribing of emergency cards have been introduced, and their incorporation into our service is ongoing. Re-audit is planned in 6 months (January 2022). Disclosure J. Ellis: None. L. Ibbotson: None. B. Mulhearn: None. S. Skeoch: None.

Emergency card, emergency medication, and information leaflet for the prevention and treatment of adrenal crisis (Addison crisis): an Austrian consensus document

Ein wichtiges Ziel bei der Behandlung der Nebenniereninsuffizienz ist die Prävention der Nebennierenkrise (auch akute Nebenniereninsuffizienz oder Addison-Krise genannt). Um in Österreich eine bessere Implementierung sowie Harmonisierung der Maßnahmen zur Prävention und Therapie der Nebennierenkrise zu erreichen, wurde dieses Konsensusdokument erarbeitet. Folgende Maßnahmen werden grundsätzlich für alle Patient*innen mit Nebenniereninsuffizienz empfohlen und in diesem Manuskript ausführlich erörtert: 1. Versorgung mit einer Notfallkarte („steroid emergency card“) sowie evtl. auch mit einem Armband oder einer Halskette (oder Ähnlichem) mit medizinischem Alarmhinweis „Nebenniereninsuffizienz, benötigt Glukokortikoide“. 2. Versorgung mit einem Hydrocortison-Notfallkit zur Injektion (alternativ auch Suppositorien/Zäpfchen zur Notfallapplikation) sowie ausreichenden oralen Glukokortikoiddosen für Stresssituationen/Erkrankungen. 3. Schulung von Patient*innen und Angehörigen zur Steigerung der Glukokortikoidtherapie in Stresssituationen bzw. bei Erkrankungen („sick day rules“) und zur Selbstinjektion von Hydrocortison. 4. Versorgung mit einer Behandlungsleitlinie (Informationszettel) zur Prävention und Therapie der Nebennierenkrise, welche bei Bedarf auch dem Gesundheitspersonal gezeigt werden soll. 5. Versorgung mit einer Notfall-Telefonnummer des behandelnden endokrinologischen Teams und/oder medizinisch geschulter Betreuungspersonen bzw. Angehöriger. 6. Regelmäßige (vorzugsweise jährliche) Wiederholung der Schulungsmaßnahmen. Dieses Konsensusdokument beinhaltet auch ausführliche Empfehlungen für die perioperative Glukokortikoidtherapie sowie für diverse andere Stresssituationen.

Adrenal glands and diabetes

Adrenal glands and diabetes

A PATIENT WITH CHEST PAIN AND ST-SEGMENT ELEVATION ON A RESTING ECG – A CASE STUDY

The study aimed to discuss selected causes of chest pain in patients with ischemic changes on the resting ECG. The Emergency Medical Service (EMS) team was dispatched to a 49-year-old patient reporting crushing, retrosternal chest pain radiating to the left upper limb. The research material was obtained from the analysis of the patient’s medical records during his stay in the Emergency Department (ED), the Interventional Cardiology Department, as well as from the medical emergency card and the dispatch order of the EMS team. The study used an individual case study method. An urgent qualification for coronary angiography and percutaneous coronary intervention is the priority in providing healthcare service to a patient with the suspected acute coronary syndrome with ST-segment elevation. The ED staff followed the guidelines on management of acute coronary syndrome with ST-segment elevation.

AACE2021-A-1022: Management of Adrenal Insufficiency: Survey of Physicians’ from the Middle East and North Africa

Treatment of adrenal insufficiency (AI) requires correct lifelong use of glucocorticoids with early dose adjustments to cover the increased demand in stress to avoid life-threatening emergencies. We aimed to determine physicians' knowledge on the pathophysiological and clinical aspects of AI in the two regions of North Africa and the Middle East. An electronic questionnaire on physiology, pharmacology, and clinical management of AI of 96 participants. Half of the participants were attending physicians, and 44,.2% practiced adult endocrinology. Knowledge of physiology and pharmacology of GC hormones (e.g., normal, daily cortisol production and half-life of hydrocortisone (HC) was variable. Physicians' patterns in prescribing and monitoring the adequacy of glucocorticoid (GC) replacement therapy revealed that the majority (81.3%) identified HC preparations for use in GC replacement therapy, followed by prednisone/prednisolone (46.9%), longer-acting prednisolone formulations (13.5%), and Dexamethasone (13.5%). The most frequently reported sign for GC under-replacement was “nausea” (84.4%), followed by “weight loss” (81.3%), “headaches” (44.8%) and “sleep disturbances” (36.5%). The most frequently reported for GC over-replacement was “high blood pressure” (91.6%), followed closely by “weight gain” (90.5%) and “increased blood glucose” (86.3%). A few, 6.3% and 3.2% of respondents considered “weight loss” and “low blood pressure” as symptoms of GC over-replacement, respectively. The situations most frequently identified as necessary for adjustments of GC dosages were "acute severe disease" (92.7%) and "fever more than 38°C" (79.2%) during acute severe disease, dose increase 2-5-fold by 77.1% of respondents. Strangely, some respondents reported dose reduction or even omission. The majority (80.9%) identified the need for emergency, standby medications for patients with AI. Additionally, 94.7% reported the need to issue an emergency card, and 80.9% reported the need for relative and friends' education regarding AI. When traveling, packing adequate GC preparations would be recommended by 90.5%, emergency card (89.5%), and emergency medications (70.5%). In the investigated settings, physicians’ knowledge of physiology and pharmacology of glucocorticoids, medical replacement strategies in AI, and prevention of adrenal crisis may be insufficient. There is a need for more education and training on AI in both medical and endocrine forums.

PARPACT: Paramedic Palliative Care Test : Validation of aquestionnaire to assess palliative care knowledge and self-efficacy expectations of paramedics

HintergrundAngesichts der Vielzahl von Palliativpatienten, die vom Rettungsdienst versorgt werden, spielen Aus- und Weiterbildung in palliativmedizinischen Themen eine immer größere Rolle. Zur Verbesserung der Entscheidungsfindung in Rettungssituationen wurde in vielen Städten ein Palliativ- bzw. Notfallausweis eingeführt.Ziel der ArbeitUm den Erfolg von Bildungsmaßnahmen und den Effekt des Palliativ- bzw. Notfallausweises zu überprüfen, wurde ein Fragebogen zur Ermittlung von palliativem Wissen und palliativer Selbstwirksamkeitserwartung im Rettungsdienst entwickelt und validiert.Material und MethodenDie Entwicklung und Inhaltsvalidierung erfolgte mithilfe eines Delphi-Prozesses. Zur Konstruktvalidierung wurde die Faktorenanalyse genutzt. Die Kriteriumsvalidität wurde anhand von 22 speziell in Palliative Care geschulten Pflegekräften überprüft. Die Reliabilität wurde mittels Cronbachs α als Maß der internen Konsistenz ermittelt.Ergebnisse291 von 750 Rettungsdienstmitarbeitern nahmen an der freiwilligen Befragung teil. Nach Abschluss des Delphi-Prozesses bestand Konsens, dass die wichtigen Themen Schmerz, Dyspnoe, Sedierung, Sterbebegleitung, Sterbehilfe und rechtliche Aspekte im Fragebogen abgedeckt sind. Das Ergebnis der Faktorenanalyse sprach für eine 6‑Faktoren-Lösung. Bei der Kriteriumsvalidierung zeigte sich ein signifikanter Unterschied im palliativen Wissen zwischen den Palliative-Care-Pflegekräften (MRang 289,73) und den Rettungsdienstmitarbeitern (MRang 146,97, U = 281.000, r = 0,40, p < 0,001). Cronbachs α lag für die Wissensfragen bei 0,70 und für die Subskala der palliativen Selbstwirksamkeitserwartung bei 0,82.DiskussionMit dem Paramedic Palliative Care Test (PARPACT) liegt ein validiertes Messinstrument zur Überprüfung von Bildungsmaßnahmen im Rettungsdienst vor.

Adrenal crisis and mortality rate in adrenal insufficiency and congenital adrenal hyperplasia.

Primary adrenal insufficiency (PAI) is characterized by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids. Addison's disease (AD) and congenital adrenal hyperplasia (CAH) are the most frequent disorders in adults and children, respectively. Despite the diagnostic advances and the availability of glucocorticoid and mineralocorticoid replacements, adrenal crisis (AC) is still a potentially lethal condition contributing to the increased mortality, not only during the first year of life, but also throughout life. Failure in increasing glucocorticoid doses during acute stress, when greater amounts of glucocorticoids are required, can lead to AC and an increase morbimortality rate of PAI. Considering a mortality rate of 0.5 per 100 patient years, up to 1,500 deaths from AC are expected in Brazil in the coming decade, which represents an alarming situation. The major clinical features are hypotension and volume depletion. Nonspecific symptoms such as fatigue, lack of energy, anorexia, nausea, vomiting, and abdominal pain are common. The main precipitating factors are gastrointestinal diseases, other infectious disease, stressful events (e.g., major pain, surgery, strenuous physical activity, heat, and pregnancy), and withdrawal of glucocorticoid therapy. Suspected AC requires immediate therapeutic action with intravenous (iv) hydrocortisone, fluid infusion, monitoring support, and antibiotics if necessary. AC is best prevented through patient education, precocious identification and by adjusting the glucocorticoid dosage in stressor situations. The emergency card, warning about acute glucocorticoid replacement, has high value in reducing the morbidity and mortality of AC.

ENDOCRINOLOGY IN THE TIME OF COVID-19: Management of adrenal insufficiency.

We provide guidance on prevention of adrenal crisis during the global COVID-19 crisis, a time with frequently restricted access to the usual level of healthcare. Patients with adrenal insufficiency are at an increased risk of infection, which may be complicated by developing an adrenal crisis; however, there is currently no evidence that adrenal insufficiency patients are more likely to develop a severe course of disease. We highlight the need for education (sick day rules, stringent social distancing rules), equipment (sufficient glucocorticoid supplies, steroid emergency self-injection kit) and empowerment (steroid emergency card, COVID-19 guidelines) to prevent adrenal crises. In patients with adrenal insufficiency developing an acute COVID-19 infection, which frequently presents with continuous high fever, we suggest oral stress dose cover with 20 mg hydrocortisone every 6 h. We also comment on suggested dosing for patients who usually take modified release hydrocortisone or prednisolone. In patients with adrenal insufficiency showing clinical deterioration during an acute COVID-19 infection, we advise immediate (self-)injection of 100 mg hydrocortisone intramuscularly, followed by continuous i.v. infusion of 200 mg hydrocortisone per 24 h, or until this can be established, and administration of 50 mg hydrocortisone every 6 h. We also advise on doses for infants and children.

NHS launches steroid emergency card to be carried by patients at risk of adrenal crisis

NHS launches steroid emergency card to be carried by patients at risk of adrenal crisis

Is there a need for an emergency card in hypoparathyroidism?

Patients with hypoparathyroidism are at risk of both hypocalcemic and hypercalcemic crisis. Patients report that health professionals do not always respond adequately in an acute situation. The extent and handling of severe hypo- and hypercalcemia in hypoparathyroidism is unknown. To outline the need for a medical emergency card for primary hypoparathyroidism. Postal survey amongst Norwegian and Swedish patients with chronic hypoparathyroidism of all causes. Altogether 455 invitations were sent (333 from Norway and 122 from Sweden). Three hundred and thirty-six of 455 (74%) patients responded (253 from Norway and 83 from Sweden). The majority were women (79%), and the main cause was postsurgical hypoparathyroidism (66%). Overall 44% and 16% had been hospitalized at least once for hypo- or hypercalcemia, respectively. Eighty-seven per cent felt that an emergency card would be highly needed or useful. Amongst those hospitalized for hypocalcemia, 95% felt a card was needed compared to 90% amongst those hospitalized for hypercalcemia. Five per cent believed that a card would not be useful. The majority answered that an acute card is highly needed or useful. Hospitalization foracute hypocalcemia was more common (44%)than for acute hypercalcemia (16%). As a result of this survey, an emergency card will be distributed in three European countries to test its utility.

Management of adrenal emergencies in educated patients with adrenal insufficiency—A prospective study

To evaluate the management of adrenal emergencies (AE) requiring parenteral glucocorticoid (GC) treatment in patients with chronic adrenal insufficiency (AI). Prospective, multicentre, questionnaire-based study. Participating patients (n=150) with chronic AI were provided with a questionnaire on the management of emergency situations, which had to be completed and sent back in case of an AE. In addition, patients were contacted by phone on a regular basis. Fifty-nine AE in 39 patients were documented. The time interval from contact to arrival of a medical professional was 20minutes (1-240). In total, in 43 AE, patients received parenteral GC by a medical professional. The time interval between showing the emergency card and GC injection by a medical professional was 60minutes (5-360). A total of 26 patients administered GC by self-injection. The time from the beginning of symptoms to GC injection was significantly shorter in case of self-injection (self-injection vs injection by medical professional; 85minutes [20-280] vs 232.5minutes [1-3135]; P<.001). After self-injection, 62% of the patients were treated outpatient, compared to 27% of the patients after exclusive injection by a medical professional (P=.008). To improve the emergency management, most of the patients (84%) indicated a need for an easier way of self-injection. While management of AE by both patients and medical professionals still shows high variability, patients profit from the option of self-injection. Patient care, including education of patients and health-professionals, as well as the way of GC administration, needs further optimization.

Adrenal crisis

Patients with chronic adrenal insufficiency suffer from reduced quality of life and increased mortality. An association between mortality and adrenal crisis is assumed. The frequency of adrenal crisis is about 8/100 patient years. The main causes are infectious disease. Pathophysiology is poorly understood to date. An association with an exaggerated inflammatory response due to a lack of glucocorticoid modulation as well as mineralocorticoid deficiency and diminished adrenomedullary function are discussed. The therapy of adrenal crisis includes prompt parenteral administration of hydrocortisone combined with isotonic saline. To prevent adrenal crisis, patients are equipped with an emergency card and set and educated in glucocorticoid dose adjustment.

Cardiac device implantation in Fabry disease: A retrospective monocentric study.

The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD.Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy. Six patients (4M/2F) required a pacemaker (PM) for sinus node dysfunction (n = 4) or atrioventricular disease (n = 2). One female patient required an internal cardioverter-defibrillator (ICD) to prevent sudden cardiac death because of nonsustained ventricular tachycardia (nSVT). One female patient required PM-ICD for sinus node dysfunction and nSVT. One patient underwent CD implantation before the diagnosis of FD. The annual rate of CD implantation was estimated at 1.90 per 100 person years. On univariate analysis at the end of the follow-up period, the factors associated with ACAs requiring CD implantation were as follows: delayed diagnosis of FD, delayed initiation of enzyme replacement therapy, age at the last follow-up visit, and severe multiorgan phenotype (hypertrophic cardiomyopathy, chronic kidney disease, and/or sensorineural hearing loss). On multivariate analysis, age at diagnosis of FD and age at the last follow-up visit were independently associated with an increased risk of ACAs requiring CD (P < 0.05).Considering the high frequency of ACAs requiring CD implantation and the risk of sudden death in patients with FD, regular monitoring is mandatory, especially in patients with a late diagnosis of FD and/or with a severe phenotype. Regular Holter ECGs, therapeutic education of patients, and deliverance of an emergency card including a phenotype summary are crucial in the care of FD patients.Available guidelines for device therapy and the efficacy of enzyme replacement therapy for arrhythmias or conduction abnormalities are discussed.