ΕΠΙΣΤΗΜΟΝΙΚΑ ΚΙ … ΑΛΛΑ”από τον Ομ. Καθηγητή, Ιωάννη Γουδέβενο, 16.5.2024 – Νέες ACC/AHA οδηγίες για HCM

Συνάδελφοι- Συναδέλφισσες

Ο αείμνηστος ΦΙΚΙΏΡΗΣ  τουλάχιστον  είχε και χιούμορ.  Eίπαμε τα κοινωνικά δίκτυα είναι να γράφει κάθε πικραμένος το κοντό και το μακρύ του. Αναρωτιέμαι οι επιστημονικές ενώσεις(υπέρτασης κυρίως) τι κάνουν. Μόνο συνέδρια ή Μπάστε σκύλοι αλέστε!

Νεκρολογία:  Eφυγε σε ηλικία 76 ετών ό καθηγητής και ιδρυτής της ΚΧ κλινικής στην Αλεξανδρούπολη(πρώτο CABG 2 000) Γιώργος Μπουγιούκας.  Ενας από τους ταγμένους να φυλάγει Θερμοπύλες!

 Για τις  παθήσεις :  αυξημένη Lp(a), αμυλοείδωση(ATTR),  νόσο του Fabry, ΗCM υπάρχει έντονο ενδιαφέρον για το μόνο λόγο ότι (θα)  υπάρχουν  νέα διαθέσιμα φαρμακευτικά σκευάσματα.  Όπως θα  διαπιστώσετε δημιουργούνται νέες επιστημονικές εταιρείες(ακαδημίες), συνέδρια και ξεχωριστά ιατρεία.  Το παράδοξο είναι ότι ενώ πρόκειται αρχικά για σπάνιες παθήσεις  που στη συνέχεια  προκύπτουν τόσες πολλές είναι απίστευτο αλλά εξηγήσιμο. “Το αγώγι ξυπνάει τον αγωγιάτη” είναι κανόνας κάθε αγοράς.

 Θεραπείες(εκτός από την ινσουλίνη, ΧΜΒΗ)  που (θα) χορηγούνται υποδόρια.

Και Lasix ΥΠΟΔΟΡΙΑ

ΔΙΑΒΗΤΗΣ: glucagon-like peptide-1 (GLP-1) agonists (dulaglutide, exenatide, liraglutide, lixisenatide, and semaglutide), a GLP-1 agonist and glucose-dependent insulinotropic polypeptide agonist (tirzepatide. Μια φορά την εβδομάδα.

ΛΙΠΙΔΙΑ: Οι αναστολείς PCSK9, μονοκλωνικά αντισώματα, που χορηγούνται ανά 15 μέρες 30 ή και κάθε εξάμηνο, ανάλογα με το είδος του φαρμάκου. Lerodalcibep 3ης γενιάς PCSK9 inhibitor μια φορά το μήνα. Ιnclisiran, που χρησιμοποιεί τεχνολογία μικρού παρεμβαλλόμενου RNA a small interfering ribonucleic acid (siRNA), κάθε  6 μήνες.

Για αύξηση λειτουργικότητας της HDL: εβδομαδιαία χορήγηση   CSL112 apolipoprotein A-1 (apoA-1).  Για αυξημένα τριγλυκερίδιαχυλομικρά: Olezarsen  is an antisense oligonucleotide targeting messenger RNA for apolipoprotein C-III, which is a genetically validated target for triglyceride lowering  μια φορά το μήνα,  Plozasiran  κάθε 12 εβδομάδες

ΥΠΕΡΤΑΣΗ:  Zilebesiran δυο φορές το χρόνο

JOURNAL CLUB
Ntaios G, Baumgartner H, Doehner W, et al. Embolic Strokes of Undetermined Source: A Clinical Consensus Statement of the ESC Council on Stroke, the European Association of Cardiovascular Imaging and the European Heart Rhythm Association of the ESC. Eur Heart J 2024;Apr 30:[Epub ahead of print]. Ελεύθερη πρόσβαση  O  Γιώργος   Ντάιος πήρε μεταγραφή από Παθολογική ΠΠΓΝ Λάρισας στη Παθολογική ΑΧΕΠΑ.  Η διαδρομή με  ποδοσφαιρικούς όρους ΠΙΕΡΙΚΟΣ (birth place Κατερίνη), ΑΕΛ, ΠΑΟΚ.

 Newer Pharmacologic Treatments in Adults With Type 2 Diabetes: A Clinical Guideline From the American College of Physicians.  Ann Intern Med 2024;Apr 19:[Epub ahead of print].  Ελεύθερη πρόσβαση

ΚΕΥ POINTS  για υπερευαισθησία  στην ασπιρίνη

  1. Aspirin hypersensitivity has an estimated prevalence of 0.6-2.5% in the general population.
  1. A careful medical history reviewing the symptoms associated with a reported aspirin allergy are essential, as correct classification of the reaction is used to determine management and prevent unnecessary aspirin avoidance. Aspirin reactions range from hypersensitivity either pharmacological or immunological, intolerance, and rare reactions.
  2. Cross-reactive or pharmacological reactions can result in aspirin or  NSAID exacerbated respiratory disease  characterized by the respiratory triad of rhinitis, nasal polyps, and asthma as well as cutaneous disease , urticaria or angioedema , or anaphylaxis. Reaction may be triggered by chemically unrelated NSAIDs and is mediated by COX-1 induced downstream overproduction of leukotrienes, which leads to vasoconstriction, bronchoconstriction, and pro-inflammatory effects. Desensitization is recommended for patients experiencing NERD, less effective for those with urticaria, and not recommended for patients with severe anaphylaxis.
  3. Selective or immunological reactions can result in immediate or delayed symptoms including asthma, urticaria, angioedema, anaphylaxis, or maculopapular exanthemas. Reaction may be triggered by NSAIDs in the same chemical group and this immune system response is mediated by  IgE for Type I hypersensitivity reactions or T cells in the delayed Type IV reaction. Desensitization can be performed for asthma, urticaria, or angioedema; however, it is not recommended for severe anaphylaxis or delayed reactions.
  4. Type I hypersensitivity includes single NSAID-induced urticaria, angioedema, and anaphylaxis, which may range from mild to severe and occurs within 1 hour of NSAID administration after a previous exposure. This IgE response is mediated by histamine, which results in vasodilation, bronchial and smooth muscle contraction, glandular secretion, and pruritus within minutes. Single NSAID-induced delayed hypersensitivity reaction  may occur within several hours to days after re-exposure and results in T cell and other immune cell activation resulting in inflammation and/or cell lysis.
  5. Intolerance or pseudo-allergy may be reported as dyspepsia or GI bleeding. The mechanism of this reaction is through COX-1 induced hypoproduction of prostaglandins or may be psychosomatic. Desensitization is not needed, although a PPI or aspirin dose reduction can be considered.
  6. Rare reactions to aspirin include aseptic meningitis, Stevens-Johnson syndrome, and erythema multiforme. These may be selective for specific NSAIDs with a variable and typically idiopathic reaction mechanism. Desensitization is not recommended.
  7. Proposed desensitization protocols vary; however, most involve administering increasing doses of aspirin over a time period (typically hours ranging to days) with or without pretreatment with agents such as antihistamines, antileukotrienes, and corticosteroids. Response is evaluated by measuring vitals, symptoms, and/or spirometry. Symptomatic treatment should be provided if a reaction is observed. Success of various desensitization procedures with protocols ranging from 1 to 5.5 hours has been reported as 87.5% to 100%. Aspirin tolerance is only achieved for the dose reached during the successful desensitization procedure.
  8. For the nonacute setting, patients with CAD and suspected true aspirin hypersensitivity should undergo a desensitization protocol after consult with an allergist/immunologist. For nonemergent percutaneous PCI, aspirin desensitization is recommended prior to coronary angiography. For patients requiring an emergent PCI, angiography and/or PCI should be performed with a P2Y12 inhibitor and/or glycoprotein IIb/IIIa inhibitor with aspirin desensitization started immediately after the procedure.
  9. An interdisciplinary approach promoting collaboration between cardiologists, immunologists/allergists, and other members of the care team is recommended when possible. Counseling and follow-up are critical to ensure uninterrupted continuation of aspirin, as cessation would increase prothrombotic risk and require another desensitization.

 Grimaldi S, Migliorini P, Puxeddu I, Rossini R, De Caterina R.Aspirin Hypersensitivity: A Practical Guide for Cardiologists. Eur Heart J 2024;Apr 26:[Epub ahead of print]

Νέες ACC/AHA οδηγίες για HCM (οι προηγούμενες το 2020 και οι ESC το 2023) JACC, Circulation 2024 epub ahead of print

To «κουφό»

Με μια ματιά- at a glance

1. Shared decision-making is essential to provide the best clinical care. This involves thoughtful dialogue among patients, families, and their care team in which health care professionals present

all available testing and treatment options; discuss the risks, benefits, and applicability of those options to the individual patient; and

ensure the patient expresses their personal preferences

and goals to develop their treatment plan.

2. Although the primary cardiology team can initiate evaluation, treatment, and longitudinal care, referral to multidisciplinary HCM centers with appropriate expertise can be important to optimizing

care for patients with HCM. Challenging treatment decisions—where reasonable alternatives  exist, where the strength of recommendation is weak (eg, any decision relying on a Class of 2b) or is particularly nuanced (eg, interpretation of genetic testing; ICD decision-making), and for HCM-specific invasive procedures—may critically benefit from involving specialized HCM centers.

3. Careful ascertainment of family history, counseling patients with HCM about the potential for genetic transmission of HCM, and options for genetic testing are cornerstones of care.

Screening first-degree family members of patients with HCM, using either genetic testing, serial imaging, or electrocardiographic surveillance as appropriate, can begin at any age and can be influenced by specifics of the patient and family history and family preference. Because screening recommendations for family members hinge on the pathogenicity of any detected variants, the reported pathogenicity should be reconfirmed every 2 to 3 years, and input from specialized HCM centers with genetics expertise may be valuable.

4. Assessing a patient’s risk for SCD is an important component of management. Integrating the presence or absence of established risk markers with tools to estimate individual risk score will facilitate the patient’s ability to participate in decision-making regarding ICD placement. These discussions should incorporate a patient’s personal level of risk tolerance and their specific treatment goals.

5. The risk factors for SCD in children with HCM carry different weights and components than those used in adult patients. Pediatric risk stratification also varies with age and must account for different body sizes. Coupled with the complexity

of placing implantable cardioverter-defibrillators in

young patients with anticipated growth and a higher

risk of device complications, the threshold for

implantable cardioverter-defibrillator implantation

in children often differs from adults. These differences

are best addressed at comprehensive HCM

centers with expertise in caring for children with

HCM. New risk calculators, specific to children and

adolescents, have been validated and can help young

patients and their families contextualize their estimated

risk of sudden cardiac death.

6. Cardiac myosin inhibitors are now available to treat

patients with symptomatic obstructive HCM. This new class of medication inhibits actin-myosin interaction, thus decreasing cardiac contractility and reducing LV outflow tract obstruction.

Mavacamten is the only FDA–approved agent. These agents can be beneficial for patients with obstructive HCM who do not derive adequate symptomatic relief from first-line drug therapy.

7. Invasive septal reduction therapies (surgical septal myectomy and alcohol septal ablation), when performed by experienced HCM teams at dedicated centers, can provide safe and effective symptomatic relief for patients with drug-refractory or severe outflow tract obstruction. Given the data on the significantly improved

outcomes at comprehensive HCM centers, these decisions

represent an optimal opportunity for referral.

8. Patients with HCM and persistent or paroxysmal AF have a sufficiently increased risk of stroke such that oral anticoagulation with DOACs (or alternatively warfarin) should be considered the default treatment option irrespective of the CHA2DS2-VASc score. New tools to stratify risk for incident AF have been developed

and may assist in determining the frequency of screening patients with ambulatory telemetry. Because rapid AF is often poorly tolerated in patients with HCM, maintenance of sinus rhythm and rate control are key treatment goals.

9. Exercise stress testing is particularly helpful in determining overall exercise tolerance and for latent exercise provoked LV outflow tract obstruction. Because children may not describe symptoms readily, routine exercise testing can be particularly important for young patients.

10. Increasingly, data affirm that the beneficial effects of

exercise on general health are extended to patients

with HCM. Healthy recreational exercise (light [<3

METs], moderate [3-6 METs], and vigorous [>6 METs) has not been associated with increased risk of ventricular arrhythmia events in short-term studies. If patients pursue rigorous exercise training for the purpose of performance or competition, it is important to engage in a comprehensive discussion and seek input from expert HCM professionals regarding the potential risks and benefits, to develop an individualized training plan, and to establish a regular schedule for reevaluation .

ΠΕΡΙΣΤΑΤΙΚΟ ΤΗΣ ΕΒΔΟΜΑΔΑΣ JAMA May 7, 2024 Volume 331, Number 17. Tilt test

A 43-year-old woman presented witha1-year history of recurring symptoms of sudden onset of fatigue, palpitations, dyspnea, chest pain, lightheadedness, and nausea that were associated with standing and resolved with sitting.   Η ΑΠ χωρίς μεταβολές στη καθιστική και όρθια θέση και οι σφύξεις 93 και 119/min αντίστοιχα. Ο υπόλοιπος  έλεγχος χωρίς ευρήματα

Στο Tilt table testing, the patient experienced light headedness and nausea when moved from horizontal to the upright position. Results of the tilt table test are shown in the fig

The hemodynamic finding of increased HR without simultaneous hypotension is characteristic of Postural orthostatic tachycardia syndrome (POTS). The syndrome is defined as chronic orthostatic intolerance for at least 3 months with a sustained HR increase of 30/min or more above baseline (40/min for patients aged 8-19 years) that occurs within 10 minutes of standing up or head-up tilt, without simultaneous hypotension. The patient was treated with minorine, 7.5mg 3times daily, pyridostigmine, 60mg 3times daily, and ivabradine, 2.5mgtwice daily. She was instructed to increase salt intake to 5 to 10 g/d, drink fluids up to 2 to 3 L/d, and wear lower extremity compressive garments.

 To επόμενο περιστατικο παρουσιάσθηκε στο  Medicine Case Conference,  MASSACHUSETTS GENERAL HOSPITAL. N Engl J Med 2024;390:1514-22.

 27 χρονών νεαρός με αδυναμία κάτω άκρων, τρόμο στα χέρια, απώλεια βάρους, διάρροιες  και κάλιο 1.8  mmol/l

Διάγνωση  Thyrotoxic periodic paralysis.

Αλγόριθμος για τη διάγνωση της υποκαλιαιμίας

Πως να διαγνώσετε το αντιφωσφολιπιδαιμικό σύνδρομο(APLS)

2023 ACR/EULAR antiphospholipid syndrome classification criteria l. Ann Rheum Dis 2023;82:1258–1270

Aυτό που αρέσει στη Θεσσαλονίκη είναι ο τρόπος που “τα στερνά τιμούν τα πρώτα”.  Ασυνήθιστες συμπεριφορές για ιατρικές και δη ακαδημαικές κοινωνίες. Φάνηκε και στο τελευταίο “Trends”  όπου συναντηθήκαμε  οι “παλιές καραβάνες” (Γαβριηλίδης, Μόχλας, Γιαννόγλου, Κανονίδης, Χατζημιλτιάδης, Μουτούδης, Δρόσος, Γκελερής, Καρβούνης, Καζινάκης κ α).

Απουσίαζε  ο Πατριάρχης Γιώργος  Παρχαρίδης αλλά το γήρας…

Tέλος Όχι η Ματίνα δεν σχολίασε την EUROVISION!

Συναδελφικά Γουδέβενος Γιάννης Γιάννινα

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