Difetti del setto interventricolare (DIV)
Table of Contents
Definizione
Perdifetti del setto interventricolare(DIV) si intende un difetto di formazione del setto che separa i due ventricoli, con conseguentecomunicazione diretta tra ventricolo destro e ventricolo sinistro. Rappresentano la cardiopatia congenita più frequente (circa 25%) e vengono diagnosticati perlopiù in età pediatrica; è quindi rara la diagnosi di DIV nell’età adulta. Idifetti del setto interventricolarepossono presentarsi come difetto isolato o associato ad altre anomalie congenite. Possono essere distinti in base a tre parametri:la sede, le dimensioni e il numero. La classificazione dei DIV su base anatomica tiene conto che il setto interventricolare è costituito da due porzioni: un’ampia muscolatura e una piccola membranosa. Nell’ambito della porzione muscolare del setto interventricolare sono ulteriormente distinguibili tre componenti:
- Una porzione di afflussoo inlet, che separa la valvola mitrale dalla tricuspide e si porta verso il basso.
- Una porzione trabecolata, che va dall’inserzione delle corde tendine dei lembi valvolari della tricuspide verso il basso fino all’apice, e poi fino alla cresta sopraventricolare.
- Una porzione di efflusso o outlet, detta anche infundibolare, liscia, che si estende dalla cresta sopraventricolare verso la valvola polmonare.
La porzione membranosa del setto interventricolare è invece molto piccola e localizzata appena sotto la valvola aortica e spesso divisa in due parti dal lembo settale della tricuspide. I DIV possono essere quindi classificati a seconda della porzione del setto interessata.E’ definito muscolare quando i suoi margini sono interamente costituiti da miocardio. La maggior parte dei DIV è tuttavia localizzata a livello della porzione membranosa del setto; si distinguono allora DIV di tipo membranoso “puro” se il difetto riguarda esclusivamente il setto membranoso e DIV perimembranosi ( i più frequenti) se il difetto membranoso si estende alle adiacenti porzioni muscolari di inlet, outlet o trabecolare.
Fisiopatologia
In presenza di un DIV, il quadro fisiopatologico che ne consegue è caratterizzato da una shunt sinistro-destro a livello del difetto, iperafflusso polmonare, sovraccarico di volume delle sezioni sinistre del cuore e rischio di ipertensione polmonare. L’entità di questi effetti emodinamici dipende dall’ampiezza del difetto e dalle resistenze anteriolari polmonari.
UnDIV piccolo o emodinamicamente restrittivoè un difetto che determinashunt sinistro-destro di lieve entità, come indicato dal rapporto tra flusso polmonare e flusso siatemico (QP/QS<1,5) accompagnato da un basso rapporto tra pressione sistolica polmonare e aortica. Un DIV restrittivo non provoca alterazioni emodinamiche significative e può anche chiudersi spontaneamente durante l’infanzia e talvolta nell’età adulta.
UnDIV moderatamente restrittivoè caratterizzato da un moderatoshuntsinistro-destro(QP/QS= 1,5-2,2) e un rapporto pressorio sistolico polmonare/aortico < 0,66. Questo difetto determina quindi un sovraccarico di volume delle sezioni sinistre, con conseguenti dilatazione e disfunzone atriale e ventricolare sinistra, e un aumento delle resistenze vascolari polmonari.
UnDIV ampio o non restrittivoè accompagnato da unampioshunt(QP/QS>2,2) eun rapporto pressorio sistolico polmonare/aortico > 0,66. In questo difetto il sovraccarico di volume ventricolare sinistro si manifesta precocemente nell’infanzia, con progressivo sviluppo di ipertensione polmonare causata da iperafflusso polmonare, tipicamente sistolica ancora reversibile.
UnDIV conSindrome di Eisenmengerha un rapporto pressorio sistolico polmonare/aortico pari a 1 e un QP/QS <1 con comparsa di un netto shunt destro-sinistro.
Manifestazioni cliniche
Se il DIV è piccolo, il paziente è generalmente asintomatico; tuttavia questi piccoli difetti rappresentano un rischio continuo diendocarditebatterica. I DIV più ampi determinano un aumento significativo del flusso polmonare, per cui i pazienti lamentano i sintomi della congestione polmonare:
- Facile affaticabilità e dispnea da sforzo, spesso scatenati nell’età adulta dall’insorgenza di fibrillazione atriale.
- Più tardi si può avereedema polmonareacuto.
Nei pazienti con DIV ampio non restrittivo e sindrome di Eisenmenger sono presenti:
- Cianosicentrale e ippocratismo digitale
- L’edema periferico testimoniascompenso cardiaco destro.
Diagnosi
L‘ecocardiogramma 2Dpermette di porre la diagnosi del DIV, rilevando sede, dimensioni e conseguenze emodinamiche del difetto.
Con ilDoppler continuoè possibile misurare il gradiente pressorio tra i due ventricoli.
Con ilcolor- Dropplersi definiscono ulteriormente la presenza e la direzione dello shunt e si può escludere la presenza di eventuali anomalie associate tipo insufficienza aortica o ostruzione al tratto di efflusso ventricolare destro o sinistro.
Ilcateterismo cardiacoconsente di porre la diagnosi in modo molto preciso, di valutare il rapporto QP/QS, quindi l’entità dell’ iperafflusso polmonare e lo stato delle resistenze vascolari polmonari. Questo studio, tuttavia, si rende necessario soltanto quando non appare chiara l’importanza emodinamica di un DIV e devono essere valutate pressioni e resistenze polmonari. In alcuni centri il cateterismo cardiaco interventistico viene effettuato per eseguire la chiusura percutanea del difetto.
Terapia
Lacorrezione di scelta è chirurgica, anche se in alcuni centri viene eseguita la chiusura percutanea del difetto con dispositivo occludente. Le indicazioni all’intervento sono rappresentate dalla presenza di un DIV emodinamicamente significativo. Dopo la correzione, in assenza di DIV residuo, il rischio di endocardite, così come il rischio di insufficienza aortica evolutiva, viene virtualmente eliminato. Al contrario, in presenza di residuo o in caso di mancata indicazione alla chiusura, è sempre raccomandata la profilassi dell’endocardite batterica. La mortalità chirurgica è molto bassa (< 2% ) anche negli adulti e il recupero anatomofunzionale è completo, quando non vi sia shunt residuo e in condizione di normalizzazione delle pressioni polmonari. La sopravvivenza a distanza dei soggetti operati nei primi anni di vita in assenza di shunt e/o ipertensione polmonare residuo è molto buona. essendo sovrapponibile a quella dei soggetti normali.
Ti potrebbero interessare:
- Cardiopatie congenite
- Difetti del setto interatriale
- Sindrome di Eisenmenger
- Tetralogia di Fallot
- Endocardite infettiva
- Edema polmonare
In che modo la Cardiomiopatia Ipertrofica modifica il cuore?
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