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Cardiomiopatia dilatativa

Table of Contents

Introduzione        

•Epidemiologia

 

• Sintomi

 

 •Diagnosi

 

 

 

 

Introduzione

La cardiomiopatia dilatativa (CMPD) viene definita come “Malattia del muscolo cardiaco caratterizzata da dilatazione  ridotta contrattilità del ventricolo sinistro o di entrambi i ventricoli” e rappresenta, assieme alla forma ipertrofica, restrittiva e alla displasia aritmogena del ventricolo destro uno dei quattro sottotipi principali di cardiomiopatia. 

 

Epidemiologia

La prevalenza della CMPD nella popolazione generale è stimata essere circa 1 caso ogni 2.500 abitanti e l’incidenza pari a 4-8 nuovi casi/100.000 individui/ anno. Tuttavia, la sua reale frequenza è certamente superiore, considerando che la maggior parte dei soggetti ancora asintomatici ma già con le “stimmate” della malattia( dilatazione e disfunzione ventricolare sinistra) non vengono identificati sino a che non compaiono i primi sintomi e segni riferibili a scompenso cardiaco o a turbe del ritmo e della conduzione .

Sintomi

 

La CMPD può manifestarsi in pazienti di tutte le età, ma nella maggior parte dei casi l’esordio avviene tra i 20 e i 50 anni. 

La malattia colpisce prevalentemente il sesso maschile, con un rapporto maschi femmine di circa 3:1. Dal punto di vista clinico, la CMPD si manifesta più frequentemente con scompenso cardiaco o aritmie ventricolari o sopraventricolari. In oltre il 50% dei pazienti, la presentazione clinica è rappresentata da un quadro di scompenso cardiaco sinistro; in una minore percentuale di casi, possono essere prevalenti i segni di scompenso destro. Le aritmie sono un’evenienza frequente nel CMPD e non di rado costituiscono le prime manifestazioni cliniche; tuttavia, solo raramente sincope e morte improvvisa rappresentano l’esordio della malattia. Un dolore toracico, in genere da sforzo e talora con le caratteristiche di un angina rappresenta il sintomo principale di esordio della CMPD  nel 10-20% dei casi; in questi pazienti, è stata dimostrata una minore riserva coronarica. Nel 2-4% dei casi, usualmente con avanzato compromissione della funzione ventricolare e marcata cardiomegalia, la manifestazione clinica iniziale è costituita da un episodio embolico sistemico o polmonare. Talvolta, il sospetto di CMPD  viene posto a paziente asintomatico. Si tratta di casi scoperti fortuitamente in occasione di una visita medica (ad esempio, per riscontro di un soffio cardiaco) o di un indagine strumentale (ad es. per il riscontro di blocco di branca sinistra all’elettrocardiogramma o di cardiomegalia alla radiografia del torace), effettuate per altri motivi.

Diagnosi

 

Di fronte ad una presentazione clinica suggestiva di CMPD, è necessario integrare i dati anamnestici e clinici con le opportune indagini strumentali e di laboratorio.

Elettrocardiogramma. 

La tachicardia sinusale è un dato di frequente riscontro nelle ECG standard. Possono essere presenti anche turbe della conduzione atrio-ventricolare ed intra-ventricolare, in particolare il blocco di branca sinistra e anche onde Q di “pseudo-necrosi” in sede anteriore, in associazione con estesa fibrosi di questa regione. Anche le alterazioni della ripolarizzazione sono di frequente riscontro, come pure l’intero spettro delle aritmie sopraventricolari e ventricolari.

Radiogramma toracico 

La cardiomegalia (rapporto cardio toracico >0.5) è di comune riscontro, come pure i segni di redistribuzione a carico del circolo polmonare. Congestione interstiziale di alveolare sono spesso documentabili nelle forme più avanzate.

Ecocardiogramma 

L‘anamnesi, l’esame obiettivo, l’ ECG e la radiografia del torace non sono in grado di fornire elementi specifici che consentano con sicurezza la diagnosi CMPD, la quale richiede la presenza di alcuni criteri evidenziabili solamente con l’esecuzione di un ecocardiogramma. La CMPD è classicamente caratterizzata,

da un punto di vista ecocardiografico, dalla presenza di una dilatazione globale del ventricolo sinistro associata a diffuse alterazioni della cinetica parietale con ridotta funzione di pompa. (frazione di eiezione inferiore <45%. Nei casi in fase avanzata, il veicolo sinistro, oltre ad essere di volume notevolmente aumentato assume una geometria caratterizzata da una morfologia più grossa e quindi meno ellissoidale che di norma.

L’ecocardiogramma è anche in grado di documentare eventuali asincronia nella contrazione inter e intra-ventricolare che possono cntribuire a peggiorarela funzione di pompa cardiaca.

 

Metodiche invasive

La coronarografia rimane un’ indagine di fondamentale importanza per la diagnosi differenziale tra CMPD e cardiopatia ischemica in fase dilatativa ipocinetica. E’ indicata nei pazienti di sesso maschile di età superiore a 35 anni con uno o più fattori di rischio coronarico e/o indicatori clinico strumentali suggestivi di coronaropatia. Il cateterismo cardiaco consente uno studio emodianamico dettagliato con la misurazione delle pressioni di riempimento ventricolare e della portata cardiaca, e mantiene un ruolo importante nella valutazione della gravità e nella stratificazione prohnostica nei pazienti con CMPD .

Familiarità

 

Lo studio in una famiglia di CMPD si basa su un’accurata costruzione dell’albero genealogico  e della storia familiare 

(volta ad individuare il possibile pattern di trasmissione della malattia) è sullo screening clinico strumentale (ECG, ecocardiogramma), di tutti i parenti di primo grado (genitori,

fratelli/sorelle figli) del probando (primo individuo affetto di una famiglia che giunge all’osservazione). La valutazione clinico strumentale andrebbe ripetuta periodicamente non solo nei familiari affetti ma anche in quelli sani per escludere un’evoluzione tardiva della malattia dovuta alla bassa penetranza. La CMPD viene definita familiare due: 1) in presenza di due o più individuiaffetti in una famiglia o 2) in presenza di un parente di primo grado di un paziente con CMPD che abbia avuto una morte improvvisa, documentata ed inaspettata, ad una età inferiore di 35 anni.

 

 

 

 

 

 

 

 

 

 

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