Razlikovanje sprememb v EKG pri športniku od električnih bolezni srca. mag. U. Mazić Pediatrična klinika Ljubljana UKC Ljubljana
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1 Razlikovanje sprememb v EKG pri športniku od električnih bolezni srca mag. U. Mazić Pediatrična klinika Ljubljana UKC Ljubljana
2 Nenadna smrt pri mladih
3 Kardio-vaskularni vzroki za nenadna srčno smrt Circulation.2012; 126:
4 Preventivni pregled športnika Anamneza Klinični pregled EKG.
5 EKG Športnik Pogoste in s treningom povezane spremembe Sinusna bradikardija AV blok I stopnje Inkompletni RBBB zgodnja repolarizacija Izolirani QRS voltažni kriteriji za HLV Redke in s treningom nepovezane EKG spremembe Patološki Q zobec Inverzija T valov depresija ST-segmenta Obremenitev levega preddvora Leva os /levi sprednji hemiblok Desna os/ levi zadnji hemiblok Obremenitev desnega prekata prekatna preekscitacija Kompletni LBBB ali RBBB Dolg ali kratek QT interval Brugadi podobna zgodnja repolarizacija
6 Sinusna bradikardija / aritmija SINUSNA BRADIKARDIJA Normalno za športnika Utrip <60/min junkcijski ritem enostavna AV disociacija sinusna aritmija 15-70% variabilnostjo BOLEZEN SINUSNEGA VOZLA neustrezna sinusna bradikardija oz. nezmožnost ustreznega porasta srčne frekvence ob fiziološkem stresu Pomisli ob utrip <30/min izgine med obremenitvijo
7 Sinusna bradikardija / aritmija
8 OPOZORILNI ZNAKI ZA PATOLOŠKO BRADIKARDIJO Podatki o zdravilih, ki vplivajo na prevodni sistem Družinska anamneza o sinkopah in SCD Podatki o boleznih srca Ponavljajoče se sinkope, vrtoglavice in/ali krči Sinkope med telesnim naporom
9 Obravnava Sinusna bradikardija / aritmija Normalno - Znižanje utripa je pričakovano in glede na stopnjo treninga ali tip športa - Odsotnost simptomov: vrtoglavica sinkopa - Pulz se poviša med obremenitvijo in simpatično aktivacijo, z ohranjeno maksimalno srčno frekvenco - Bradikardija se zmanjša z zmanjšanjem intenzivnosti ali prekinitvijo treningov.
10 Bolezen sinusnega vozla Srčne napake Pridobljena: poškodbe (kirurgija), vnetja (miokarditis) ali zdravil (antiaritmiki) prirojene bolezni ionskih kanalčkov
11 AV blok I stopnje v 35%, AV blok II Mobitz 1 v 10% AV blok I stopnje
12 Obravnava: AV blok NEDOLŽEN ČE - asimtomatski - izgine ob hiperventilaciji ali obremenitvi AV blok II st. Mobitz 2 in AV blok 3 stopnje zmeraj zahtevajo nadaljnjo obravnavo
13 AV blok I st. IZJEMA: neuromuskularna obolenja - miopatije
14 Inkompletni RBBB QRS <120 ms pojavlja se pri 35% - 50% športnikov pogostejši pri vztrajnostnih športih in moških upočasnjeni prevod v RV je posledica povečane votline RV /povečane mase miokarda in NE motenj v prevodnem sistemu
15 Obravnava : Inkompletni RBBB Normalen - negativna družinska /osebna anamneza - normalen klinični pregled POMISLI NA ASD: pogosta najdba pri bolnikih z ASD sec. (klinični pregled!!!, HRV v EKG) ARVC/D, če pojavlja skupaj z inverzijo T ( pod V2 do V3 in V4) ali ob prisotnosti VES z LBBB morfologijo in če QRS >110) redko potrebno ločiti od atipičnega Brugada sindroma (medikamentozni test)
16 Mejni Brugada EKG vs RBBB Tipični RBBB R v V1 and V2 je tipično povezan z recipročnim S v D1 in V6 desni prekordialni odvodi ne kažejo znakov elevacije ST segmenta.
17 Zgodnja repolarizacija (ER) prisotna pri 1-2% zdrave populacije prisotna pri 50-80% športnikov
18 Zgodnja repolarizacija EKG elevacija QRS ST spoja (J točka) za najmanj 0.1 mv, pogosto združena z nazobčanim terminalnim delom QRS najpogosteje v prekordialnih odvodih, z najvišjo ST elevacijo v V3 V4. redkeje dobimo max. ST elevacijo lateralno (V5, V6, L1 in avl), inferiorno (L2, L3 in avf) ali anteriorno (V2 V3).
19 Najpogostejši vzorci zgodnje repolarizacije pri športnikih Corrado D et al. Br J Sports Med 2009;43:
20 Obravnava: zgodnja repolarizacija fiziološka in benigna EKG oblika pri športnikih Ne potrebuje nadaljnje klinične obravnave če je anamneza in klinični pregled normalen POZOR povečana prevalenca EKG oblike ER v inferiornih in/ali lateralnih odvodih najdemo pri bolnikih z IVF Pri športnikih z sinkopo ali srčnim zastojem, ki ostane nepojasnjen, prisotnost ER v inferiornih in/ali lateralnih odvodih s prominentnim upočasnjenim prevajanjem (slurring) je sumljiva za J wave sidrome (Brugada, ERS, IVF) Redko je potrebno farmakološko testiranje (blokatorji Na-kanalčkov), EFŠ ali slikovna diagnostika
21 ER pri temnopoltih atletih ARVC/D
22 EKG razlika med Brugada sindromom in ER pri športnikih
23 Zgodnja repolarizacija vs. J waves sindromi Tip 1 : športniki - ER samo v lateralnih prekordialnih odvodih - tipična za zdrave mlade moške atlete - predstavlja nizko tveganja za aritmični dogodek Tip 2: - ER v inferiornih ali infero-lateralnih odvodih - zmerno tveganje za SCD Tip 3: IVF - ER prisoten globalno v inferiornih, lateralnih in desnih prekordialnih - visoko tveganje za SCD in električne nevihte
24
25 Inverzija T valov ni povezana s športom najdemo pri 2-4% športnikov in splošne populacije juvenilni vzorec valov T: negativni T valovi v V1-V3 lahko vztraja (v V3 do 16. leta starosti) Opozorilo Inverzija T vala 2 mm in v 2 zaporednih odvodih je pri športnikih nespecifičen toda opozorilni znak na potencialne bolezni, ki zvišujejo tveganje za SCD Seattle kriteriji: >1 mm v 2 ali več odvodih V2 V6, II in avf, ali I in avl (izključeno III, avr in V1)
26 Obravnava: inverzija T valov 16 letna atletinja BENIGNA- JUVENILNA OBLIKA inverzije T valov T val v V1-V3 novorojenec lahko še pozitivne po 1 tednu so negativni do 8 leta Pri najstnikih postopno pride do pozitivizacije T valov in sicer najprej v V3, nato V2 in na koncu še v V1 vidno : V3 pozitiven inverzija T <2mm
27 Obravnava: inverzija T valov 25 letni športnik: inverzija T V1-V3 inverzija T > 2mm 18 letni bolnik QRS >110mm epsilon val inverzija T valov sum na ARVC
28 Obravnava: inverzija T valov Inverzija T-valov 2 mm in v 2 zaporednih odvodih pri mladih športnikih lahko predstavlja začetno obliko kardiomiopatij preden jih lahko zaznamo s slikovnimi metodami (ECHO, MR, CT) Normalen morfološki izvid srca ob inverziji T valov ne izključuje bolezni srčne mišice (od starosti odvisna ekspresija) in lahko prav tako povzroči SCD, inverzija T vala pod V1, ki se pojavlja po puberteti, lahko kaže na prirojeno srčno napako, ki povečuje volumsko ali tlačno obremenitev RV (ASD, PS, PAH), ARVC/D ali redkeje dedne bolezni Na/K ionskih kanalčkov inverzija T valov pod V1 se pri po-pubertetnih športnikih pojavlja redko (1.4%), vendar zmeraj zahteva posebno obravnavo zaradi suma na ARVC/D
29 Intraventrikularne motnje prevajanja LBBB, RBBB in bifascikularni bloki (RBBB z levim sprednjim hemiblok ali levim zadnjim hemiblokom) in QRS > 120 (seattle >140) pri športniku zahtevajo nadaljnjo obravnavo zaradi izključitve genetskih progresivnih bolezni prevodnega sistema srca (PCCD)
30 Intraventrikularne motnje prevajanja QRS > 120 msec posteriorni fascikularni blok RBBB RBBB + sprednji levi hemiblok LBB
31 Intraventrikularne motnje prevajanja - PCCD LBBB ni nikoli normalen pri mladem prevajanje je upočasnjeno in s časom se razvijejo bloki prevajanja lahko se pojavijo aritmije - bradikardia in asistolija, in/ali tahikardije Pri nekaterih bolnikih je PCCD (progressive cardiac conduction defect) povezana z mutacijo Na- kanalčkov (Na: SCN5A, SCN1B) in TRPM4 in se lahko obnaša podobno kot Brugada Sy Druga imena za PCCD: Familial Lenègre disease Familial Lev disease Familial Lev-Lenègre disease Familial PCCD Familial progressive heart block Hereditary bundle branch defect Progressive familial heart block
32 Ne-specifične intraventrikularne motnje prevajanja QRS >110 ms in brez kriterijev za LBBB ali RBBB upočasnjeno prevajanje je običajno v miokardu prekatov in ne v prevodnem sistemu Običajno normalno in benigno pri dobro treniranih moških atletih (povečana mas miokarda) Obravnava ob sumu na bolezen miokarda (anamneza, klinični pregled, EKG) potrebujejo nadaljnjo obravnavo
33 Ne-specifične intraventrikularne motnje prevajanja QRS complex >110 (115) ms v V1 V3, epsilon wave (terminalna grba po koncu QRS) in/ali upočasnjen dvig S vala(črna puščica), ARVC/D
34 Undiagnosed Underlying Problems Električne bolezni srca Dedni aritmični sindromi: Sindrom dolge QT dobe (LQTS) 35% Sindrom Brugada (BrS) 32% Kateholaminergična polimorfna ventrikularna tahikardija (CPVT) Sidrom zgodnje repolarizacije (ERS) 6-9% Idiopatska ventrikularna fibrilacija (IVF) Sidrom kratke QT dobe (SQTS) 2% Druge električne bolezni srca Bradikardne motnje srčnega ritma prekatne in nadprekatne aritmije
35 Glavni geni vključeni v dedne električne bolezni srca LQTS, Long QT Syndrome; SQTS, Short QT Syndrome; AF, Atrial Fibrillation; SSS, Sick Sinus Syndrome; PCCD, Progressive Conduction Cardiac Disease; DCM, Dilated cardiomyopathy; HCM, Hypertrophic cardiomyopathy; SIDS, Sudden Infant Death Syndrome; PFHB1B, Progressive Familial Heart Block type 1B.
36 Undiagnosed Underlying Problems Sindrom dolge QT dobe (LQTS) prevalenca vsaj 1: 2000 genetsko dokazljivega bolezen je posledica motenega delovanja predvsem K in Na ionskih kanalčkov Značilna je podaljšana doba QTc - >460 ms otroci - >470ms za moške - >480 ms za ženske - 10% do 20% splošne populacije ima QTc > 440ms. - QTc > 500ms je zmeraj abnormalen - podaljšan. Pri sindrom dolge QT dobe najdemo še notched T val, bradikardije, alterantne T valove, VT TdP
37 Undiagnosed Underlying Problems Long QT Syndrome
38 Undiagnosed Underlying Problems Brugada Syndrome 16 genov EKG abnormalnosti v vsaj enem od odvodov V1-V3 povečano tveganje za VT in SCD pri otrocih navadno ni incidentov povišana telesna temperatura!
39 2. Primer - CPVT Med naporom nastopi tahikardija, vrtoglavica, slabo počutje Podoben napad pred 2 meseci, ki spontano izzveni po 2h EKG ob sprejemu v EIT: VT s širokimi QRS : ATP neučinkovit, konverzija v sinus po infuziji amiodarona
40 Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)
41 Undiagnosed Underlying Problems Kateholaminergična polimorfna ventrikularna tahikardija (CPVT) znani 4 različni geni : RYR2 (50-55%), CASQ2 (2-5%), TRDN, in CALM1(<1%) Motnja v delovanju Ca kanalčkov Najpogostejša je mutacija v Ryanodine receptorju Sinkope in prekatne tahikardije se pojavljajo ob obremenitvah Običajno prva manifestacija že v otroštvu
42 Zdravljenje: CPVT Beta blokator ICD - Dvigaš odmerek dokler motnja ritma ni izzivna - Redne kontrole in preverjanje učinkovitosti zdravljenja 6-12/ mes - Sekundarna prevencija - Primarna: VT ob max. odmerku beta blokerja na obremenitvi Asimptomatski bolniki/nosilci mutacije - Beta bloker (tudi med nosečnostjo) Druge oblike zdravljenja - Ablacija simpatičnih ganglijev - Flecainide (Flecainide + betabloker pri nonresponderju)
43 Primer 16 letni A.B.: na pregled zaradi ocene sposobnosti ukvarjanja s športom Anamneza: brat umrl med igranjem nogometa Teta umrla v starosti 3 mesecev BREZ TEŽAV IN SIMPTOMOV Klinični pregled: normalen Preiskave: EKG HOLTER EKG? ECHO? CIKLO? MR?
44 Undiagnosed Underlying Problems Short QT Syndrome 6 genov: KCNH2, KCNQ1, KCNJ2, CACNA1C, CACNA2D1 in CACNB2. običajno autosomal dominant motnja K kanalčkov kratek QT interval na EKG SQTS1 KCNH2 IKr SQTS2 KCNQ1 IKs SQTS3 KCNJ2 IK1 SQTS4 CACNA1C ICa SQTS5 CACNB2b ICa SQTS6 CACNA2D1 ICa IKr, rectifier K+ current rapid component; IKs, rectifier K+ current slow component; IK1, inward rectifier K+ current; ICa, Ca2+ current.
45 KRITERIJI ZA DIAGNOZO Razred I: QTc 330 msec Razred IIa: QTc 360 msec če je prisoten eden ali več od naslednjih: - Patogena mutacija - Družinska anamneza SQTS - SCD v družini v starosti 40 let - Preživel VT/VF brez srčne bolezni HRS/EHRA/APHRS Expert consensus Statement on the management of Inherited Arrhythmias
46 ZDRAVLJENJE HRS/EHRA/APHRS Expert consensus Statement on the management of Inherited Arrhythmias. ICD pri simptomatskih bolnikih z SQTS, ki : - So preživeli srčni zastoj in/ali - Imajo dokumentirano obstojno VT z/brez sinkope ICD je lahko koristen pri asimptomaskih bolnikih z SQTS in SCD v družini Quinidin je lahko koristen pri asimptomatskih bolnikih z SQTS in SCD v družini Pri SQTS razen pri SQTS1, je sotalol lahko koristen pri asimptomatskih bolnikih z SQTS in SCD v družini
47 Zaključek Za preprečevanje SCD pri športnikih s preventivnimi EKG-ji moramo opraviti pridobiti natančno anamnezo in opraviti natančen klinični pregled razumeti osnove EKG in poznati standarde, ki nam pomagajo ločiti športno srce od bolezni srca
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