Sindrome di Wolff-Parkinson-White
Table of Contents
Definizione
Può essere definitasindrome di Wolff-Parkinson-Whitel‘attivazione precoce di una parte dei ventricoli, accompagnata da accessi di tachicardia sopraventricolare parossistica.
Anatomia
Durante le fasi iniziali dello sviluppo del cuore,le camere cardiache non sono separate dal punto di vista elettrico, al contrario, esse sonostrettamente interconnessesenza soluzione di continuo. Quando si costituisce il tessuto fibroso che forma la struttura del trigono centrale e i due anelli atrioventricolari,scompaionopian pianotutte le fibre muscolari di connessione tra gli atri e i ventricoli,residuando solo il sistema di conduzione specializzato nella giunzione AV. Talora, però, alcuni di questi fasci muscolari di connessione atrioventricolare non scompaiono, costituendo una potenzialevia accessoriadi conduzione AV. Queste vie possono essere localizzate ovunque nel solco atrioventricolare, eccetto che nella sede in cui la valvola mitrale è in stretta continuità con l’aorta. Intorno al 50%-60% esse sono localizzate a livello della parete libera del ventricolo sinistro. I fasci anomali atrioventricolari di conduzione (denominati anche fasci di Kent) sono costituiti da fibre NA+- dipendenti la cui capacità di conduzione è del tipo” tutto o nulla”. Nella maggior parte dei casi, in un individuo è presente una sola via accessoria. In un piccolo numero di soggetti, tuttavia, si possono avere due o più vie anomale di conduzione (vie accessorie multiple). Una via anomala atrioventricolare presente in un paziente può essere manifesta, ossia conduce in modo precoce l’impulso dagli atri ai ventricoli rispetto al nodo AV. Questa pre-eccitazione può essere chiaramente evidenziata all’ ECG, oppure occulta. In questo caso, pur essendo anatomicamente presente, il fascio anomalo non conduce mai l’ impulso dagli atri ai ventricoli, per cui all’ECG non viene notata alcuna anomalia. Una via anomala presente in un paziente può a volte rendersi manifesta e a volte può essere occulta (pre-eccitazione intermittente).
Sintomi
La sindrome di Wolff-Parkinson-White può restare clinicamente muta, essere scoperta fortuitamente durante un esame elettrocardiografico o manifestarsi con accessi di tachicardia sopraventricolare parossistica e spesso con accessi difibrillazione atrialea risposta ventricolare e molto rapida (oltre 200 al minuto).
Diagnosi
La diagnosi di Wolff-Parkinson-White è clinica edelettrocardiografica.
Prognosi
La prognosi dei pazienti con sindrome di WPW è generalmente buona. Per un piccolo gruppo di pazienti, tuttavia, esiste il rischio di sviluppare aritmie che possono causaresincopeemorte improvvisa, un evento particolarmente drammatico in questi casi perché colpisce soggetti giovani, o anche solo adolescenti. Essa, inoltre, solo eccezionalmente costituisce la prima manifestazione sintomatica della malattia, mentre di solito si verifica in pazienti sintomatici per tachiaritmie, che quindi devono essere ben valutati per escludere la presenza di un rischio significativo di morte improvvisa.
Terapia
Il paziente con pre-eccitazione di WPW asintomatica non necessita abitualmente di alcun tipo di trattamento. In caso di episodi di fibrillazione atriale con elevata frequenza ventricolare in un paziente con WPW il trattamento immediato è rappresentato dallacardioversione elettricao, se questa per qualche motivo non è possibile, dall’uso di farmaci della classe IC o III che hanno la capacità di aumentare il periodo refrattario della via anomala, riducendo i rischi secondari all’elevata frequenza di attivazione ventricolare attraverso di essa. Il trattamento definitivo delle aritmie della sindrome di Wolff-Parkinson-White è l’ablazionedella via accessoria.
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