Zespół wolffa-parkinsona-white’a (wpw)
Rokowania, prognozy i postęp choroby
Zespół Wolffa-Parkinsona-White’a (WPW) występuje u 1-3/1000 osób i charakteryzuje się obecnością dodatkowej drogi przewodzenia, co predysponuje do arytmii, w tym migotania przedsionków (AF) i migotania komór (VF). Nagły zgon sercowy (SCD) jest rzadkim, ale poważnym powikłaniem, z częstością od 0,0002 do 0,0015 na pacjento-rok, a w niektórych badaniach do 0,25-0,39% rocznie. Czynniki ryzyka SCD obejmują m.in. płeć męską, wiek <35 lat, krótki okres refrakcji dodatkowej drogi (<240 ms), obecność wielu dróg przewodzenia oraz rodzinny wywiad nagłej śmierci sercowej. Diagnostyka elektrofizjologiczna (EPS) jest kluczowa w ocenie ryzyka, zwłaszcza przy stwierdzeniu wywołanego migotania przedsionków i najkrótszego odstępu RR <250 ms (lub <200 ms podczas wlewu izoproterenolu). Bezobjawowi pacjenci mają generalnie dobrą prognozę, jednak ryzyko arytmii i SCD pozostaje wyższe niż w populacji ogólnej, co uzasadnia profilaktyczne badania i ewentualną ablację.
Prognozy dla pacjentów z zespołem Wolffa-Parkinsona-White’a (WPW)
Zespół Wolffa-Parkinsona-White’a (WPW) jest stosunkowo częstym zaburzeniem rytmu serca, występującym u około 1-3 osób na 1000. Mimo że wiąże się z pewnymi ryzykami, prognozy dla pacjentów są obecnie bardzo dobre, szczególnie po wdrożeniu odpowiedniego leczenia.12 Przeżywalność pacjentów z rozpoznanym i właściwie leczonym zespołem WPW jest doskonała, szczególnie gdy zastosowano leczenie ablacją przezskórną, która może prowadzić do całkowitego wyleczenia.3
Ryzyko nagłego zgonu sercowego
Nagły zgon sercowy (SCD) stanowi najpoważniejsze, choć rzadkie powikłanie zespołu WPW. Badania populacyjne określają częstość nagłych zgonów sercowych u pacjentów z zespołem WPW na poziomie od 0,0002 do 0,0015 na pacjento-rok.4 Inne badania podają wyższą roczną częstość występowania nagłych zgonów sercowych wynoszącą około 0,25-0,39%.5 Częstość występowania SCD w zespole WPW wynosi około 1 na 100 przypadków objawowych w okresie obserwacji do 15 lat.6 Warto podkreślić, że zespół WPW został powiązany z nagłym zgonem sercowym u dzieci i młodych dorosłych.7
Czynniki ryzyka powikłań
Istnieją określone czynniki, które zwiększają ryzyko nagłego zgonu sercowego u pacjentów z zespołem WPW:89
- Płeć męska
- Wiek poniżej 35 lat
- Wywiad migotania przedsionków lub częstoskurczu przedsionkowo-komorowego nawrotnego (AVRT)
- Obecność wielu dodatkowych dróg przewodzenia
- Przegrodowa lokalizacja dodatkowej drogi przewodzenia
- Zdolność do szybkiego przewodzenia zstępującego przez dodatkową drogę
- Krótki okres refrakcji dodatkowej drogi przewodzenia (< 240 ms)
- Rodzinny wywiad przedwczesnej nagłej śmierci sercowej
U pacjentów z zespołem WPW najbardziej podatnych na migotanie komór (VF) występuje w wywiadzie migotanie przedsionków (AF).10 Przyczyną nagłego zgonu sercowego w zespole WPW jest najczęściej szybkie przewodzenie migotania przedsionków do komór przez dodatkową drogę, co skutkuje migotaniem komór.11
Prognozy dla pacjentów bezobjawowych
Pacjenci z bezobjawowym zespołem preekscytacji widocznym tylko w EKG mają generalnie bardzo dobrą prognozę, choć ryzyko nagłego zgonu sercowego jest u nich wciąż wyższe niż w populacji ogólnej.1213 Wiele z tych osób z czasem rozwija objawowe arytmie, którym można zapobiegać dzięki profilaktycznemu badaniu elektrofizjologicznemu (EPS) i ablacji przezskórnej.14
Ryzyko śmiertelnych arytmii pozostaje obecne również u bezobjawowych i objawowych pacjentów w podeszłym wieku z zespołem WPW.15 W tej grupie wiekowej ryzyko migotania przedsionków wzrasta, co zwiększa także ryzyko pierwszego epizodu szybkiego migotania przedsionków.16
Wpływ wieku na rokowanie
Wiek pacjenta ma istotny wpływ na rokowanie w zespole WPW. Badania długoterminowe wykazały, że osoby z większą podatnością na migotanie przedsionków są zwykle młodsze, częściej mają indukowaną arytmię i krótki efektywny okres refrakcji drogi dodatkowej (APERP) wynoszący 250 ms.17
Nagły zgon sercowy lub zdarzenia zagrażające życiu, takie jak migotanie przedsionków lub migotanie komór, są rzadkie u dzieci z zespołem WPW.18 Jednak bez długoterminowych danych obserwacyjnych trudno jest określić, czy podgrupa dzieci z wariantami genetycznymi w genach związanych z migotaniem przedsionków będzie narażona na zwiększone ryzyko migotania przedsionków w wieku dorosłym.19
Badania wskazują na związany z wiekiem wzrost częstości nawrotów napadowego migotania przedsionków po skutecznej ablacji prądem o częstotliwości radiowej dodatkowych dróg. Odsetek nawrotów jest niski u pacjentów poniżej 50 roku życia (12%), ale znacznie wyższy u starszych pacjentów – 35% u osób powyżej 50 roku życia i 55% u pacjentów powyżej 60 roku życia.20
Wpływ leczenia na prognozę
Prognozy dla pacjentów z zespołem WPW znacznie się poprawiły wraz z rozwojem leków przeciwarytmicznych i technik ablacji w ciągu ostatnich 80 lat.21 Dla pacjentów z objawowym zespołem WPW, czynnikami wysokiego ryzyka lub silnymi preferencjami, ablacja przezskórna prądem o częstotliwości radiowej może być leczeniem z wyboru, oferującym wysokie wskaźniki powodzenia przy niskim ryzyku powikłań.22
Skuteczność ablacji
Ablacja jest często metodą definitywnego leczenia zespołu WPW. Po skutecznym zabiegu ablacji ryzyko nagłego zgonu sercowego znacząco się zmniejsza.23 Należy jednak zauważyć, że migotanie przedsionków może nadal występować i być objawowe u niektórych pacjentów po skutecznej ablacji dodatkowej drogi, choć nie niesie wtedy takiego samego ryzyka nagłego zgonu sercowego.24
W długoterminowym badaniu (mediana obserwacji 6,9 roku) porównującym pacjentów z zespołem WPW leczonych ablacją (n = 872) i bez ablacji (n = 1461) z grupą kontrolną (n = 11 175), Bunch i współpracownicy stwierdzili podobnie niskie wskaźniki śmiertelności, ale wyższe ryzyko wystąpienia migotania przedsionków u pacjentów z WPW w porównaniu z grupą kontrolną.25 Ryzyko długoterminowej śmiertelności było wyższe u osób, które nie przeszły ablacji, w porównaniu z grupą leczoną ablacją, podczas gdy ryzyko wystąpienia migotania przedsionków było wyższe w grupie z ablacją. Ablacja nie zmniejszyła więc ryzyka migotania przedsionków.26
Znaczenie badań elektrofizjologicznych
Badanie elektrofizjologiczne (EPS) jest najbardziej wiarygodną metodą ustalenia rokowania w zespole WPW.27 Zespół WPW jest uważany za czynnik ryzyka nagłego zgonu, gdy obserwuje się następujące połączenie: wywołane trwałe migotanie przedsionków oraz najkrótszy odstęp RR między pobudzeniami z preekscytacją krótszy niż 250 ms w stanie kontrolnym u dorosłych lub krótszy niż 200 ms podczas wlewu izoproterenolu.28
Czynniki genetyczne i ich wpływ na prognozę
Ostatnie badania wskazują na znaczącą rolę czynników genetycznych w długoterminowej prognozie pacjentów z zespołem WPW.2930
Związek z migotaniem przedsionków
Liczne badania wykazały, że pomimo ablacji dodatkowej drogi, ryzyko migotania przedsionków pozostaje wysokie u pacjentów z zespołem WPW w porównaniu do populacji ogólnej.31 Badania wskazują na zwiększone obciążenie rzadkimi szkodliwymi wariantami genów związanych z migotaniem przedsionków w zespole WPW, co sugeruje, że czynniki genetyczne determinujące rozwój dodatkowych dróg mogą być powiązane ze zwiększoną podatnością mięśnia przedsionków na migotanie przedsionków u części pacjentów.32
Zgodnie z literaturą, czynniki ryzyka rozwoju migotania przedsionków w zespole WPW obejmują zaawansowany wiek (rozpoznano dwa szczytowe okresy występowania migotania przedsionków, jeden w wieku 30 lat, a drugi w wieku 50 lat), płeć męską i wcześniejsze omdlenia.33
Znaczenie badań genetycznych
Badania genetyczne identyfikują istotne rzadkie warianty de novo i dziedziczone w pediatrycznej kohorcie pacjentów z WPW i dostarczają podstaw do przyszłych prac nad identyfikacją osób wysokiego ryzyka.34 Dane silnie wskazują na rozszerzenie spektrum zespołu Ankyrin B o zespół WPW.35
Potrzebne są dalsze badania, aby powiązać molekularne odkrycia w zespole WPW z długoterminowymi wynikami u pacjentów.36 Zrozumienie tych związków może w przyszłości pomóc w lepszej stratyfikacji ryzyka i spersonalizowanym podejściu do leczenia.
Wnioski końcowe
Prognozy dla pacjentów z zespołem WPW są generalnie korzystne, zwłaszcza po zastosowaniu odpowiedniego leczenia. Ryzyko nagłego zgonu sercowego, choć istnieje, jest stosunkowo niskie i można je dalej zmniejszyć poprzez identyfikację pacjentów wysokiego ryzyka oraz zastosowanie ablacji przezskórnej. Cechy demograficzne, kliniczne oraz elektrofizjologiczne pomagają w stratyfikacji ryzyka.3738
Badania genetyczne mogą w przyszłości odegrać istotną rolę w identyfikacji pacjentów z podwyższonym ryzykiem rozwoju migotania przedsionków i związanych z nim powikłań. Pomimo skutecznej ablacji dodatkowej drogi, u niektórych pacjentów utrzymuje się zwiększone ryzyko migotania przedsionków, co wymaga długoterminowej obserwacji.3940
Zespół WPW może być również obserwowany u osób powyżej 60 roku życia, a w tej grupie wiekowej występuje zwiększone ryzyko nawrotu migotania przedsionków po ablacji, co należy uwzględnić w planowaniu długoterminowej opieki nad takimi pacjentami.41
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Materiały źródłowe
- #1 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Once identified and appropriately treated, WPW syndrome is associated with an excellent prognosis, including the potential for permanent cure through radiofrequency (RF) catheter ablation. […] Asymptomatic patients with only preexcitation on ECG generally have a very good prognosis. Many develop symptomatic arrhythmias over time, which can be prevented with prophylactic EPS and RF catheter ablation. […] Patients with a family history of sudden cardiac death (SCD) or significant symptoms of tachyarrhythmias or cardiac arrest have worse prognoses. However, once definitive therapy is performed, including curative ablation, the prognosis is once again excellent. […] Mortality in WPW syndrome is rare and is related to SCD. The incidence of SCD in WPW syndrome is approximately 1 in 100 symptomatic cases when followed for up to 15 years.
- #2 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Wolff-Parkinson-White (WPW) syndrome is a relatively common arrhythmia affecting ~1-3/1000 individuals. […] Several studies have indicated that despite ablation of an accessory pathway, the risk of AF remains high in patients compared to general population. […] Our findings show an increased burden of rare deleterious variants in genes linked to AF in WPW syndrome, suggesting that genetic factors that determine the development of accessory pathways may be linked to an increased susceptibility of atrial muscle to AF in a subset of patients. […] The risk of sudden cardiac death (SCD) in WPW, albeit small, is pertinent with reported incidence of approximately 0.25-0.39% annually. […] It has been shown that patients with WPW syndrome who are most susceptible to VF have a history of atrial fibrillation (AF).
- #3 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Once identified and appropriately treated, WPW syndrome is associated with an excellent prognosis, including the potential for permanent cure through radiofrequency (RF) catheter ablation. […] Asymptomatic patients with only preexcitation on ECG generally have a very good prognosis. Many develop symptomatic arrhythmias over time, which can be prevented with prophylactic EPS and RF catheter ablation. […] Patients with a family history of sudden cardiac death (SCD) or significant symptoms of tachyarrhythmias or cardiac arrest have worse prognoses. However, once definitive therapy is performed, including curative ablation, the prognosis is once again excellent. […] Mortality in WPW syndrome is rare and is related to SCD. The incidence of SCD in WPW syndrome is approximately 1 in 100 symptomatic cases when followed for up to 15 years.
- #4 Wolff-Parkinson-White Syndrome – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK554437/
WPW pattern is a rare condition, and most patients with preexcitation on ECG will never have symptoms, associated arrhythmias, or the most feared complication of sudden cardiac death. […] Two population studies put the rate of sudden cardiac death between 0.0002 to 0.0015 per patient-years for patients with WPW pattern. Some risk factors place a patient at higher risk for sudden cardiac death, including male gender, age less than 35 years, history of atrial fibrillation or AVRT, multiple accessory pathways, septal location of the accessory pathway, the ability for rapid anterograde conduction of the accessory pathway. […] Despite the low prevalence of WPW pattern or the low incidence of serious complications, it remains a dangerous medical condition. The prognosis for patients with WPW pattern has improved significantly as antiarrhythmic medications, and ablation techniques were developed over the last 80 years. For patients who have WPW syndrome, high-risk factors, or strong preference, radiofrequency catheter ablation can be curative and has high success rates with low rates of complications.
- #5 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Wolff-Parkinson-White (WPW) syndrome is a relatively common arrhythmia affecting ~1-3/1000 individuals. […] Several studies have indicated that despite ablation of an accessory pathway, the risk of AF remains high in patients compared to general population. […] Our findings show an increased burden of rare deleterious variants in genes linked to AF in WPW syndrome, suggesting that genetic factors that determine the development of accessory pathways may be linked to an increased susceptibility of atrial muscle to AF in a subset of patients. […] The risk of sudden cardiac death (SCD) in WPW, albeit small, is pertinent with reported incidence of approximately 0.25-0.39% annually. […] It has been shown that patients with WPW syndrome who are most susceptible to VF have a history of atrial fibrillation (AF).
- #6 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Once identified and appropriately treated, WPW syndrome is associated with an excellent prognosis, including the potential for permanent cure through radiofrequency (RF) catheter ablation. […] Asymptomatic patients with only preexcitation on ECG generally have a very good prognosis. Many develop symptomatic arrhythmias over time, which can be prevented with prophylactic EPS and RF catheter ablation. […] Patients with a family history of sudden cardiac death (SCD) or significant symptoms of tachyarrhythmias or cardiac arrest have worse prognoses. However, once definitive therapy is performed, including curative ablation, the prognosis is once again excellent. […] Mortality in WPW syndrome is rare and is related to SCD. The incidence of SCD in WPW syndrome is approximately 1 in 100 symptomatic cases when followed for up to 15 years.
- #7 WPW syndrome: Rare cause of sudden cardiac death in young people – Symptoms and causes – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/wolff-parkinson-white-syndrome/symptoms-causes/syc-20354626
Wolff-Parkinson-White (WPW) syndrome has been linked to sudden cardiac death in children and young adults. […] The episodes of fast heartbeats seen in Wolff-Parkinson-White syndrome usually aren’t life-threatening. But serious heart problems can occur. Rarely, the syndrome may lead to sudden cardiac death in children and young adults.
- #8 Wolff-Parkinson-White Syndrome – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK554437/
WPW pattern is a rare condition, and most patients with preexcitation on ECG will never have symptoms, associated arrhythmias, or the most feared complication of sudden cardiac death. […] Two population studies put the rate of sudden cardiac death between 0.0002 to 0.0015 per patient-years for patients with WPW pattern. Some risk factors place a patient at higher risk for sudden cardiac death, including male gender, age less than 35 years, history of atrial fibrillation or AVRT, multiple accessory pathways, septal location of the accessory pathway, the ability for rapid anterograde conduction of the accessory pathway. […] Despite the low prevalence of WPW pattern or the low incidence of serious complications, it remains a dangerous medical condition. The prognosis for patients with WPW pattern has improved significantly as antiarrhythmic medications, and ablation techniques were developed over the last 80 years. For patients who have WPW syndrome, high-risk factors, or strong preference, radiofrequency catheter ablation can be curative and has high success rates with low rates of complications.
- #9 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Even in patients with asymptomatic WPW, the risk of SCD is increased above that of the general population. […] Other factors that appear to influence the risk of SCD are the presence of multiple bypass tracts, short AP refractory periods ( 240 ms), AF and atrial flutter, or a family history of premature sudden death. […] The cause of SCD in WPW syndrome is rapid conduction of AF to the ventricles via the AP, resulting in ventricular fibrillation (VF). […] However, a study hypothesized that two mechanisms are involved in the pathogenesis of AF in patients with WPW syndrome: one is related to the AP that predisposes the atria to fibrillation, and the other is independent from the AP and is related to increased atrial vulnerability present in these individuals. […] Notably, AF may still occur and be symptomatic in some patients after successful ablation of the bypass tract, but AF does not then carry the same associated risk of SCD.
- #10 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Wolff-Parkinson-White (WPW) syndrome is a relatively common arrhythmia affecting ~1-3/1000 individuals. […] Several studies have indicated that despite ablation of an accessory pathway, the risk of AF remains high in patients compared to general population. […] Our findings show an increased burden of rare deleterious variants in genes linked to AF in WPW syndrome, suggesting that genetic factors that determine the development of accessory pathways may be linked to an increased susceptibility of atrial muscle to AF in a subset of patients. […] The risk of sudden cardiac death (SCD) in WPW, albeit small, is pertinent with reported incidence of approximately 0.25-0.39% annually. […] It has been shown that patients with WPW syndrome who are most susceptible to VF have a history of atrial fibrillation (AF).
- #11 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Even in patients with asymptomatic WPW, the risk of SCD is increased above that of the general population. […] Other factors that appear to influence the risk of SCD are the presence of multiple bypass tracts, short AP refractory periods ( 240 ms), AF and atrial flutter, or a family history of premature sudden death. […] The cause of SCD in WPW syndrome is rapid conduction of AF to the ventricles via the AP, resulting in ventricular fibrillation (VF). […] However, a study hypothesized that two mechanisms are involved in the pathogenesis of AF in patients with WPW syndrome: one is related to the AP that predisposes the atria to fibrillation, and the other is independent from the AP and is related to increased atrial vulnerability present in these individuals. […] Notably, AF may still occur and be symptomatic in some patients after successful ablation of the bypass tract, but AF does not then carry the same associated risk of SCD.
- #12 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Once identified and appropriately treated, WPW syndrome is associated with an excellent prognosis, including the potential for permanent cure through radiofrequency (RF) catheter ablation. […] Asymptomatic patients with only preexcitation on ECG generally have a very good prognosis. Many develop symptomatic arrhythmias over time, which can be prevented with prophylactic EPS and RF catheter ablation. […] Patients with a family history of sudden cardiac death (SCD) or significant symptoms of tachyarrhythmias or cardiac arrest have worse prognoses. However, once definitive therapy is performed, including curative ablation, the prognosis is once again excellent. […] Mortality in WPW syndrome is rare and is related to SCD. The incidence of SCD in WPW syndrome is approximately 1 in 100 symptomatic cases when followed for up to 15 years.
- #13 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Even in patients with asymptomatic WPW, the risk of SCD is increased above that of the general population. […] Other factors that appear to influence the risk of SCD are the presence of multiple bypass tracts, short AP refractory periods ( 240 ms), AF and atrial flutter, or a family history of premature sudden death. […] The cause of SCD in WPW syndrome is rapid conduction of AF to the ventricles via the AP, resulting in ventricular fibrillation (VF). […] However, a study hypothesized that two mechanisms are involved in the pathogenesis of AF in patients with WPW syndrome: one is related to the AP that predisposes the atria to fibrillation, and the other is independent from the AP and is related to increased atrial vulnerability present in these individuals. […] Notably, AF may still occur and be symptomatic in some patients after successful ablation of the bypass tract, but AF does not then carry the same associated risk of SCD.
- #14 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Once identified and appropriately treated, WPW syndrome is associated with an excellent prognosis, including the potential for permanent cure through radiofrequency (RF) catheter ablation. […] Asymptomatic patients with only preexcitation on ECG generally have a very good prognosis. Many develop symptomatic arrhythmias over time, which can be prevented with prophylactic EPS and RF catheter ablation. […] Patients with a family history of sudden cardiac death (SCD) or significant symptoms of tachyarrhythmias or cardiac arrest have worse prognoses. However, once definitive therapy is performed, including curative ablation, the prognosis is once again excellent. […] Mortality in WPW syndrome is rare and is related to SCD. The incidence of SCD in WPW syndrome is approximately 1 in 100 symptomatic cases when followed for up to 15 years.
- #15 Management of Wolff-Parkinson-White Syndrome in the Elderly | ECR Journalhttps://www.ecrjournal.com/articles/management-wolff-parkinson-white-syndrome-elderly?language_content_entity=en
Wolff-Parkinson-White (WPW) syndrome is associated with atrioventricular re-entrant tachycardia, but a patient showing a pre-excitation syndrome on electrocardiogram (ECG) may remain asymptomatic. […] The risk of sudden death was reported as being relatively high (1.5%) in studies of symptomatic patients and lower in asymptomatic patients. […] However, some studies indicate that a risk of life-threatening arrhythmias remains present in asymptomatic and symptomatic elderly patients with WPW syndrome. […] The risk of atrial fibrillation increases in elderly patients, hence the risk of a first episode of rapid atrial fibrillation. […] The diagnostic value of non-invasive studies is low. […] Electrophysiological study is the most reliable method of establishing the prognosis of WPW syndrome.
- #16 Management of Wolff-Parkinson-White Syndrome in the Elderly | ECR Journalhttps://www.ecrjournal.com/articles/management-wolff-parkinson-white-syndrome-elderly?language_content_entity=en
Wolff-Parkinson-White (WPW) syndrome is associated with atrioventricular re-entrant tachycardia, but a patient showing a pre-excitation syndrome on electrocardiogram (ECG) may remain asymptomatic. […] The risk of sudden death was reported as being relatively high (1.5%) in studies of symptomatic patients and lower in asymptomatic patients. […] However, some studies indicate that a risk of life-threatening arrhythmias remains present in asymptomatic and symptomatic elderly patients with WPW syndrome. […] The risk of atrial fibrillation increases in elderly patients, hence the risk of a first episode of rapid atrial fibrillation. […] The diagnostic value of non-invasive studies is low. […] Electrophysiological study is the most reliable method of establishing the prognosis of WPW syndrome.
- #17 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Indeed, several studies indicate that despite ablation, the risk of AF remains significant in patients with WPW syndrome. […] Long-term follow up studies have shown that individuals with high susceptibility to AF tend to be younger, are more likely to have inducible arrhythmia, and have a short antegrade effective refractory period of accessory pathway (APERP) of 250 ms. […] Our data strongly indicates expanding the spectrum of Ankyrin B syndrome to include WPW syndrome. […] Importantly, our study identified an increased burden of rare deleterious alleles in genes associated with AF in WPW syndrome compared to controls (P =0.0023). […] Sudden cardiac death or adverse events such as life-threatening AF or VF are rare in children with WPW syndrome and were not observed in our pediatric cohort during the length of the study.
- #18 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Indeed, several studies indicate that despite ablation, the risk of AF remains significant in patients with WPW syndrome. […] Long-term follow up studies have shown that individuals with high susceptibility to AF tend to be younger, are more likely to have inducible arrhythmia, and have a short antegrade effective refractory period of accessory pathway (APERP) of 250 ms. […] Our data strongly indicates expanding the spectrum of Ankyrin B syndrome to include WPW syndrome. […] Importantly, our study identified an increased burden of rare deleterious alleles in genes associated with AF in WPW syndrome compared to controls (P =0.0023). […] Sudden cardiac death or adverse events such as life-threatening AF or VF are rare in children with WPW syndrome and were not observed in our pediatric cohort during the length of the study.
- #19 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Without long-term follow up data, it is indeed challenging to determine if the subset of children carrying high deleterious alleles in AF genes would be at an increased risk of AF during adulthood. […] Our study identifies significant rare de novo and inherited genetic variants in the pediatric cohort of WPW and provides a framework for future work for ascertaining high-risk affected individuals. […] Further studies are required to relate molecular findings in WPW syndrome with long-term outcomes in patients.
- #20 Management of Wolff-Parkinson-White Syndrome in the Elderly | ECR Journalhttps://www.ecrjournal.com/articles/management-wolff-parkinson-white-syndrome-elderly?language_content_entity=en
WPW syndrome is considered to represent a risk of sudden death when the following association is observed: sustained atrial fibrillation is induced and the shortest respiratory rate (RR) interval between pre-excited beats is less than 250ms in the control state in adults or less than 200ms during isoproterenol infusion. […] The recurrence rate of paroxysmal atrial fibrillation after successful radiofrequency ablation of accessory pathways shows an age-related increase, being low in patients under 50 years of age (12%) and high in the older patients: 35% in patients over 50 years of age and 55% in patients over 60 years of age. […] In conclusion, WPW syndrome can be observed in subjects over 60 years of age.
- #21 Wolff-Parkinson-White Syndrome – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK554437/
WPW pattern is a rare condition, and most patients with preexcitation on ECG will never have symptoms, associated arrhythmias, or the most feared complication of sudden cardiac death. […] Two population studies put the rate of sudden cardiac death between 0.0002 to 0.0015 per patient-years for patients with WPW pattern. Some risk factors place a patient at higher risk for sudden cardiac death, including male gender, age less than 35 years, history of atrial fibrillation or AVRT, multiple accessory pathways, septal location of the accessory pathway, the ability for rapid anterograde conduction of the accessory pathway. […] Despite the low prevalence of WPW pattern or the low incidence of serious complications, it remains a dangerous medical condition. The prognosis for patients with WPW pattern has improved significantly as antiarrhythmic medications, and ablation techniques were developed over the last 80 years. For patients who have WPW syndrome, high-risk factors, or strong preference, radiofrequency catheter ablation can be curative and has high success rates with low rates of complications.
- #22 Wolff-Parkinson-White Syndrome – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK554437/
WPW pattern is a rare condition, and most patients with preexcitation on ECG will never have symptoms, associated arrhythmias, or the most feared complication of sudden cardiac death. […] Two population studies put the rate of sudden cardiac death between 0.0002 to 0.0015 per patient-years for patients with WPW pattern. Some risk factors place a patient at higher risk for sudden cardiac death, including male gender, age less than 35 years, history of atrial fibrillation or AVRT, multiple accessory pathways, septal location of the accessory pathway, the ability for rapid anterograde conduction of the accessory pathway. […] Despite the low prevalence of WPW pattern or the low incidence of serious complications, it remains a dangerous medical condition. The prognosis for patients with WPW pattern has improved significantly as antiarrhythmic medications, and ablation techniques were developed over the last 80 years. For patients who have WPW syndrome, high-risk factors, or strong preference, radiofrequency catheter ablation can be curative and has high success rates with low rates of complications.
- #23 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Once identified and appropriately treated, WPW syndrome is associated with an excellent prognosis, including the potential for permanent cure through radiofrequency (RF) catheter ablation. […] Asymptomatic patients with only preexcitation on ECG generally have a very good prognosis. Many develop symptomatic arrhythmias over time, which can be prevented with prophylactic EPS and RF catheter ablation. […] Patients with a family history of sudden cardiac death (SCD) or significant symptoms of tachyarrhythmias or cardiac arrest have worse prognoses. However, once definitive therapy is performed, including curative ablation, the prognosis is once again excellent. […] Mortality in WPW syndrome is rare and is related to SCD. The incidence of SCD in WPW syndrome is approximately 1 in 100 symptomatic cases when followed for up to 15 years.
- #24 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Even in patients with asymptomatic WPW, the risk of SCD is increased above that of the general population. […] Other factors that appear to influence the risk of SCD are the presence of multiple bypass tracts, short AP refractory periods ( 240 ms), AF and atrial flutter, or a family history of premature sudden death. […] The cause of SCD in WPW syndrome is rapid conduction of AF to the ventricles via the AP, resulting in ventricular fibrillation (VF). […] However, a study hypothesized that two mechanisms are involved in the pathogenesis of AF in patients with WPW syndrome: one is related to the AP that predisposes the atria to fibrillation, and the other is independent from the AP and is related to increased atrial vulnerability present in these individuals. […] Notably, AF may still occur and be symptomatic in some patients after successful ablation of the bypass tract, but AF does not then carry the same associated risk of SCD.
- #25 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
In a study that evaluated the long-term (median, 6.9 y) natural history of WPW in adult patients treated with (n = 872) and without catheter ablation (n = 1461) compared to a control group (n = 11,175), Bunch et al found similarly low death rates but higher incident AF risk in patients with WPW versus the control group. […] The risk of long-term mortality was higher in those who did not undergo ablation compared to the group treated with ablation, whereas the risk of incident AF was higher in the ablation group. Thus, ablation did not reduce the risk of AF. […] According to the literature, risk factors for the development of AF in the setting of WPW syndrome include advancing age (two peak ages for AF occurrence are recognized, one at 30 years and the other at 50 years), male sex, and prior history of syncope. […] Certain factors increase the likelihood of VF, including rapidly conducting APs and multiple pathways. […] Cases have also been reported in association with esophageal studies, digoxin, and verapamil.
- #26 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
In a study that evaluated the long-term (median, 6.9 y) natural history of WPW in adult patients treated with (n = 872) and without catheter ablation (n = 1461) compared to a control group (n = 11,175), Bunch et al found similarly low death rates but higher incident AF risk in patients with WPW versus the control group. […] The risk of long-term mortality was higher in those who did not undergo ablation compared to the group treated with ablation, whereas the risk of incident AF was higher in the ablation group. Thus, ablation did not reduce the risk of AF. […] According to the literature, risk factors for the development of AF in the setting of WPW syndrome include advancing age (two peak ages for AF occurrence are recognized, one at 30 years and the other at 50 years), male sex, and prior history of syncope. […] Certain factors increase the likelihood of VF, including rapidly conducting APs and multiple pathways. […] Cases have also been reported in association with esophageal studies, digoxin, and verapamil.
- #27 Management of Wolff-Parkinson-White Syndrome in the Elderly | ECR Journalhttps://www.ecrjournal.com/articles/management-wolff-parkinson-white-syndrome-elderly?language_content_entity=en
Wolff-Parkinson-White (WPW) syndrome is associated with atrioventricular re-entrant tachycardia, but a patient showing a pre-excitation syndrome on electrocardiogram (ECG) may remain asymptomatic. […] The risk of sudden death was reported as being relatively high (1.5%) in studies of symptomatic patients and lower in asymptomatic patients. […] However, some studies indicate that a risk of life-threatening arrhythmias remains present in asymptomatic and symptomatic elderly patients with WPW syndrome. […] The risk of atrial fibrillation increases in elderly patients, hence the risk of a first episode of rapid atrial fibrillation. […] The diagnostic value of non-invasive studies is low. […] Electrophysiological study is the most reliable method of establishing the prognosis of WPW syndrome.
- #28 Management of Wolff-Parkinson-White Syndrome in the Elderly | ECR Journalhttps://www.ecrjournal.com/articles/management-wolff-parkinson-white-syndrome-elderly?language_content_entity=en
WPW syndrome is considered to represent a risk of sudden death when the following association is observed: sustained atrial fibrillation is induced and the shortest respiratory rate (RR) interval between pre-excited beats is less than 250ms in the control state in adults or less than 200ms during isoproterenol infusion. […] The recurrence rate of paroxysmal atrial fibrillation after successful radiofrequency ablation of accessory pathways shows an age-related increase, being low in patients under 50 years of age (12%) and high in the older patients: 35% in patients over 50 years of age and 55% in patients over 60 years of age. […] In conclusion, WPW syndrome can be observed in subjects over 60 years of age.
- #29 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Indeed, several studies indicate that despite ablation, the risk of AF remains significant in patients with WPW syndrome. […] Long-term follow up studies have shown that individuals with high susceptibility to AF tend to be younger, are more likely to have inducible arrhythmia, and have a short antegrade effective refractory period of accessory pathway (APERP) of 250 ms. […] Our data strongly indicates expanding the spectrum of Ankyrin B syndrome to include WPW syndrome. […] Importantly, our study identified an increased burden of rare deleterious alleles in genes associated with AF in WPW syndrome compared to controls (P =0.0023). […] Sudden cardiac death or adverse events such as life-threatening AF or VF are rare in children with WPW syndrome and were not observed in our pediatric cohort during the length of the study.
- #30 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Without long-term follow up data, it is indeed challenging to determine if the subset of children carrying high deleterious alleles in AF genes would be at an increased risk of AF during adulthood. […] Our study identifies significant rare de novo and inherited genetic variants in the pediatric cohort of WPW and provides a framework for future work for ascertaining high-risk affected individuals. […] Further studies are required to relate molecular findings in WPW syndrome with long-term outcomes in patients.
- #31 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Wolff-Parkinson-White (WPW) syndrome is a relatively common arrhythmia affecting ~1-3/1000 individuals. […] Several studies have indicated that despite ablation of an accessory pathway, the risk of AF remains high in patients compared to general population. […] Our findings show an increased burden of rare deleterious variants in genes linked to AF in WPW syndrome, suggesting that genetic factors that determine the development of accessory pathways may be linked to an increased susceptibility of atrial muscle to AF in a subset of patients. […] The risk of sudden cardiac death (SCD) in WPW, albeit small, is pertinent with reported incidence of approximately 0.25-0.39% annually. […] It has been shown that patients with WPW syndrome who are most susceptible to VF have a history of atrial fibrillation (AF).
- #32 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Wolff-Parkinson-White (WPW) syndrome is a relatively common arrhythmia affecting ~1-3/1000 individuals. […] Several studies have indicated that despite ablation of an accessory pathway, the risk of AF remains high in patients compared to general population. […] Our findings show an increased burden of rare deleterious variants in genes linked to AF in WPW syndrome, suggesting that genetic factors that determine the development of accessory pathways may be linked to an increased susceptibility of atrial muscle to AF in a subset of patients. […] The risk of sudden cardiac death (SCD) in WPW, albeit small, is pertinent with reported incidence of approximately 0.25-0.39% annually. […] It has been shown that patients with WPW syndrome who are most susceptible to VF have a history of atrial fibrillation (AF).
- #33 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
In a study that evaluated the long-term (median, 6.9 y) natural history of WPW in adult patients treated with (n = 872) and without catheter ablation (n = 1461) compared to a control group (n = 11,175), Bunch et al found similarly low death rates but higher incident AF risk in patients with WPW versus the control group. […] The risk of long-term mortality was higher in those who did not undergo ablation compared to the group treated with ablation, whereas the risk of incident AF was higher in the ablation group. Thus, ablation did not reduce the risk of AF. […] According to the literature, risk factors for the development of AF in the setting of WPW syndrome include advancing age (two peak ages for AF occurrence are recognized, one at 30 years and the other at 50 years), male sex, and prior history of syncope. […] Certain factors increase the likelihood of VF, including rapidly conducting APs and multiple pathways. […] Cases have also been reported in association with esophageal studies, digoxin, and verapamil.
- #34 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Without long-term follow up data, it is indeed challenging to determine if the subset of children carrying high deleterious alleles in AF genes would be at an increased risk of AF during adulthood. […] Our study identifies significant rare de novo and inherited genetic variants in the pediatric cohort of WPW and provides a framework for future work for ascertaining high-risk affected individuals. […] Further studies are required to relate molecular findings in WPW syndrome with long-term outcomes in patients.
- #35 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Indeed, several studies indicate that despite ablation, the risk of AF remains significant in patients with WPW syndrome. […] Long-term follow up studies have shown that individuals with high susceptibility to AF tend to be younger, are more likely to have inducible arrhythmia, and have a short antegrade effective refractory period of accessory pathway (APERP) of 250 ms. […] Our data strongly indicates expanding the spectrum of Ankyrin B syndrome to include WPW syndrome. […] Importantly, our study identified an increased burden of rare deleterious alleles in genes associated with AF in WPW syndrome compared to controls (P =0.0023). […] Sudden cardiac death or adverse events such as life-threatening AF or VF are rare in children with WPW syndrome and were not observed in our pediatric cohort during the length of the study.
- #36 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Without long-term follow up data, it is indeed challenging to determine if the subset of children carrying high deleterious alleles in AF genes would be at an increased risk of AF during adulthood. […] Our study identifies significant rare de novo and inherited genetic variants in the pediatric cohort of WPW and provides a framework for future work for ascertaining high-risk affected individuals. […] Further studies are required to relate molecular findings in WPW syndrome with long-term outcomes in patients.
- #37 Wolff-Parkinson-White Syndrome – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK554437/
WPW pattern is a rare condition, and most patients with preexcitation on ECG will never have symptoms, associated arrhythmias, or the most feared complication of sudden cardiac death. […] Two population studies put the rate of sudden cardiac death between 0.0002 to 0.0015 per patient-years for patients with WPW pattern. Some risk factors place a patient at higher risk for sudden cardiac death, including male gender, age less than 35 years, history of atrial fibrillation or AVRT, multiple accessory pathways, septal location of the accessory pathway, the ability for rapid anterograde conduction of the accessory pathway. […] Despite the low prevalence of WPW pattern or the low incidence of serious complications, it remains a dangerous medical condition. The prognosis for patients with WPW pattern has improved significantly as antiarrhythmic medications, and ablation techniques were developed over the last 80 years. For patients who have WPW syndrome, high-risk factors, or strong preference, radiofrequency catheter ablation can be curative and has high success rates with low rates of complications.
- #38 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
Once identified and appropriately treated, WPW syndrome is associated with an excellent prognosis, including the potential for permanent cure through radiofrequency (RF) catheter ablation. […] Asymptomatic patients with only preexcitation on ECG generally have a very good prognosis. Many develop symptomatic arrhythmias over time, which can be prevented with prophylactic EPS and RF catheter ablation. […] Patients with a family history of sudden cardiac death (SCD) or significant symptoms of tachyarrhythmias or cardiac arrest have worse prognoses. However, once definitive therapy is performed, including curative ablation, the prognosis is once again excellent. […] Mortality in WPW syndrome is rare and is related to SCD. The incidence of SCD in WPW syndrome is approximately 1 in 100 symptomatic cases when followed for up to 15 years.
- #39 Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillationhttps://pmc.ncbi.nlm.nih.gov/articles/PMC7275694/
Indeed, several studies indicate that despite ablation, the risk of AF remains significant in patients with WPW syndrome. […] Long-term follow up studies have shown that individuals with high susceptibility to AF tend to be younger, are more likely to have inducible arrhythmia, and have a short antegrade effective refractory period of accessory pathway (APERP) of 250 ms. […] Our data strongly indicates expanding the spectrum of Ankyrin B syndrome to include WPW syndrome. […] Importantly, our study identified an increased burden of rare deleterious alleles in genes associated with AF in WPW syndrome compared to controls (P =0.0023). […] Sudden cardiac death or adverse events such as life-threatening AF or VF are rare in children with WPW syndrome and were not observed in our pediatric cohort during the length of the study.
- #40 Wolff-Parkinson-White Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/159222-overview
In a study that evaluated the long-term (median, 6.9 y) natural history of WPW in adult patients treated with (n = 872) and without catheter ablation (n = 1461) compared to a control group (n = 11,175), Bunch et al found similarly low death rates but higher incident AF risk in patients with WPW versus the control group. […] The risk of long-term mortality was higher in those who did not undergo ablation compared to the group treated with ablation, whereas the risk of incident AF was higher in the ablation group. Thus, ablation did not reduce the risk of AF. […] According to the literature, risk factors for the development of AF in the setting of WPW syndrome include advancing age (two peak ages for AF occurrence are recognized, one at 30 years and the other at 50 years), male sex, and prior history of syncope. […] Certain factors increase the likelihood of VF, including rapidly conducting APs and multiple pathways. […] Cases have also been reported in association with esophageal studies, digoxin, and verapamil.
- #41 Management of Wolff-Parkinson-White Syndrome in the Elderly | ECR Journalhttps://www.ecrjournal.com/articles/management-wolff-parkinson-white-syndrome-elderly?language_content_entity=en
WPW syndrome is considered to represent a risk of sudden death when the following association is observed: sustained atrial fibrillation is induced and the shortest respiratory rate (RR) interval between pre-excited beats is less than 250ms in the control state in adults or less than 200ms during isoproterenol infusion. […] The recurrence rate of paroxysmal atrial fibrillation after successful radiofrequency ablation of accessory pathways shows an age-related increase, being low in patients under 50 years of age (12%) and high in the older patients: 35% in patients over 50 years of age and 55% in patients over 60 years of age. […] In conclusion, WPW syndrome can be observed in subjects over 60 years of age.