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Stenosi mitralica

 

Definizione

 

La stenosi mitralica si verifica quando l’orifizio valvolare mitralico si riduce a un’ area inferiore a 2 cm², con conseguente ostruzione al flusso in entrata al ventricolo sinistro. In condizioni normali, l’orifizio valvolare mitralico ha un’area di 4-6 cm². Una stenosi mitralica si considera lieve in presenza di un’area compresa tra 1,5-2 cm², moderata in presenza di un’area compresa tra 1-1,5 cm² e grave se l’area è <1cm².

 

Eziologia

La causa più frequente della stenosi mitralica pura rimane la malattia reumatica, anche se un’anamnesi positiva per tale malattia è presente solo nel 60% dei casi. Essa è divenuta rara nei paesi industrializzati, dove la stenosi mitralica dell’adulto giovane si osserva soltanto gli ambienti socio-economici più depressi.Una causa degenerativa è rappresentata dalla calcificazione dell’anulus mitralico e delle aree sottostanti (in persone in età avanzata). Le forme congenite o secondarie a una calcificazione massiva dell’anulo mitralico sono eccezionali.

 



 

Fisiopatologia

Quando l’area valvolare è inferiore a 1 cm², la pressione atriale sinistra aumenta considerevolmente determinando, una volta superati i 25 mmHg la comparsa delle manifestazioni cliniche (in genere la dispnea da sforzo è il sintomo precoce più frequente). Tali manifestazioni cliniche possono aggravarsi nelle situazioni in cui aumenti la frequenza cardiaca, in quanto le diastole e il tempo di riempimento ventricolare si accorciano. Per questa ragione, gli episodi di tachicardia sono generalmente mal tollerati. In questi pazienti, la fibrillazione atriale (FA) è particolarmente mal tollerata sia per l’aumento della frequenza cardiaca sia per la perdita del contributo della contrazione atriale al riempimento ventricolare, che in questi pazienti ha un ruolo  più importante rispetto ai soggetti sani. Ulteriori aumenti di pressione a livello dei capillari polmonari, specie se avvengono bruscamente, possono determinare una trasudazione di liquido all’interno degli alveoli e dare origine all’edema polmonare. Un altro fenomeno conseguente all’ipertensione polmonare può essere la rottura di connessioni venose polmonari-bronchiali, responsabili della comparsa di emottisi.

 

Manifestazioni cliniche

La maggior parte dei casi di stenosi mitralica interessa donne nella quarta decade di vita. I primi sintomi si manifestano abitualmente a distanza, anche notevole, di tempo dall’episodio di malattia reumatica. Essi compaiono inizialmente in situazioni che comportano aumento della portata e/o della frequenza cardiaca. Quindi, nella maggior parte dei casi il primo sintomo è la dispnea da sforzo. In diversi casi, tuttavia, i primi sintomi possono verificarsi in altre condizioni di aumentato lavoro cardiaco, quali gravidanza, anemia grave, tireotossicosi, febbre e fibrillazione atriale. L’aumento della pressione nell’ atrio sinistro e nelle vene polmonari causato da queste situazioni determina la comparsa di dispnea. Con la progressione della stenosi, l’entità dello sforzo sufficiente a indurre la dispnea si riduce progressivamente e il paziente comincia a manifestare limitazioni nelle proprie attività quotidiane. Successivamente possono manifestarsi dispnea parossistica notturna​ e ortopnea, legate alla ridistribuzione del pool ematico nel letto polmonare con il decubito supino. Episodi di edema polmonare acuto possono verificarsi in occasione di improvvisi aumenti del flusso transmitralico o di insorgenza di fibrillazione atriale; in alcuni pazienti l’edema polmonare acuto può anche costituire la prima manifestazione clinica della malattia​.

Altri sintomi correlati all’elevata pressione venosa polmonare sono la tosse e, in alcuni casi l’emottisi. In fasi molto avanzate della malattia possono comparire sintomi da attribuire all’atriomegalia sinistra quali la disfonia (da compressione del nervo laringeo ricorrente sinistro) e la disfagia (da compressione dell’esofago​).

Complicanze

Tra le complicanze della stenosi mitralica, la più frequente è la comparsa di fibrillazione atriale, dovuta all’ingrandimento dell’atrio sinistro. La complicanza più temibile è rappresentata dalle embolie sistemiche (cerebrali, renali, spleniche, periferiche), conseguenti alla formazione di trombi nell’ atrio sinistro dilatato o nell’ auricola. L’insorgenza di fibrillazione atriale aumenta in modo sostanziale il rischio di questi eventi embolici.




Esame obiettivo

In presenza di stenosi mitralica grave, si possono osservare cianosi periferica ed eritema malare con colorazione bluastra e violacea. Tali segni sono secondari alla bassa gittata cardiaca e all’aumento delle resistenze periferiche​.

 

Esami strumentali

ECG

Le alterazioni ECG più caratteristiche della stenosi mitralica sono quelle relative all’ingrandimento dell’atrio sinistro, rilevabile nel 90% dei pazienti con una stenosi significativa della valvola. L’ECG inoltre, consente di documentare la comparsa di eventuale aritmie atriali, di cui, la fibrillazione atriale è la più frequente.

Radiologia

Il reperto radiologico più caratteristico è il doppio contorno dell’atrio destro secondario all’ingrandimento dell’atrio sinistro. Possono emergere reperti di congestione polmonare come ridistribuzione vascolare, linee di Kerley, versamento pleurico ecc..  in casi di patologia avanzata emergono segni di dilatazione della cavità di destra e segni radiologici di ipertensione polmonare.

Ecocardiografia

E’ la tecnica diagnostica di elezione. Serve non solo per valutare la morfologia valvolare ma anche per escludere altre cause di ostruzione mitralica, determinare la gravità della stenosi mediante il gradiente pressorio medio transmitralico o il calcolo dell’area valvolare, determinare la presenza concomitante di insufficienza mitralica, le dimensioni dell’atrio sinistro e la presenza di trombi. Permette inoltre di calcolare uno score valvolare che determina se la stenosi può essere trattata con valvuloplastica.

 

Terapia

Medica

Trattamento della tachicardia

  • L’utilizzo di farmaci bradicardizzanti consente di aumentare la durata delle diastole e, il riempimento ventricolare.

Trattamento dell’insufficienza cardiaca

  • Limitazione del consumo di sale e diuretici.

Trattamento della FA

  • Controllo della frequenza cardiaca (beta-bloccanti calcio-antagonisti, digossina).
  • Valutare la cardioversione elettrica o farmacologica per cercare di ripristinare il ritmo sinusale.
  • Valutare la possibilità di una terapia anticoagulante.

 



 

 Procedure interventistiche

Sono indicate nei pazienti con stenosi mitralica di grado almeno moderato area <1,5 cm² e sintomi.

Le opzioni sono due:

Valvuloplastica percutanea con palloncino

  • Si tratta di una tecnica di elezione, se praticabile, in quanto presenta un tasso di mortalità più basso (1% rispetto al 4-5 % della sostituzione valvolare). E’ indicata nei pazienti con valvole mobili, flessibili e poco calcificate.  E’ controindicata in presenza di trombi nell’atrio sinistro o in caso di insufficienza mitralica significativa concomitante. Tra le complicanze si annoverano l’insufficienza mitralica residua e, a lungo termine, restenosi.

Sostituzione valvolare chirurgica

Nelle valvole molto calcificate, con score sfavorevole per valvuloplastica, si interviene con la sostituzione delle valvole con apposite protesi. Viene effettuata in pazienti sintomatici e solo in via eccezionale nei pazienti asintomatici con altre indicazioni interventistiche, dato l’alto rischio chirurgico.

 

 

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