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cardiomiopatie e miocarditi

The role of a gender effect (that means differences in clinical manifestations, access to therapies and response to treatments according to gender) in cardiomyopathies remains a matter of debate. Although recent studies have evaluated the differences in the clinical features and prognosis between the two sexes, many issues remain to be elucidated. At present, the only sex-specific condition that affects females is peripartum cardiomyopathy. Recent evidence suggests a pathogenetic role of a prolactin derivative, and ongoing clinical trials are investigating the possibility of targeted therapies using prolactin secretion inhibitors, such as bromocriptine and carbegoline. Although women were considered so far only carriers of X-linked diseases (Anderson-Fabry disease, Danon disease, Hunter syndrome and dystrophinopathies), clinical experience showed a wide spectrum of clinical manifestations in females due to random X chromosome inactivation. Conversely, in mitochondrial diseases (with matrilineal inheritance), cardiomyopathies may occur in the context of clinical multisystemic involvement without significant gender-related differences. Autosomal inherited cardiomyopathies also show different phenotypes and prognostic impact according to gender. The hypothesis of a premenopausal protective role of female hormones towards myocardial involvement has been raised by recent data on transtiretin-related amyloidosis and hypertrophic cardiomyopathy. Preexisting cardiomyopathies may affect pregnancy, labor and delivery in women, since all these conditions are associated with important hemodynamic changes. Women with low-risk hypertrophic cardiomyopathy (asymptomatic and without left ventricular outflow tract gradient) usually can tolerate pregnancy. Conversely, women who are symptomatic before pregnancy or have severe hypertrophy with important outflow tract gradient are at higher risk and should be referred to a tertiary center to be evaluated on a case by case basis. Pregnancy in women with dilated cardiomyopathy and significant left ventricular systolic dysfunction represents a high-risk condition. In addition, information on the clinical course and potential complications in pregnant women with arrhythmogenic right ventricular cardiomyopathy or restrictive cardiomyopathy is limited to individual reports.

I pazienti affetti da cirrosi epatica possono sviluppare una cardiopatia, alla quale si è dato il nome di “cardiomiopatia cirrotica”. Essa consiste in una disfunzione cardiaca cronica caratterizzata da: 1) incremento della gittata cardiaca; 2) disfunzione diastolica correlata ad un alterato rilascio diastolico a riposo; 3) ridotta contrattilità miocardica sotto stimolo; 4) anormalità elettrofisiologiche, in assenza di malattia cardiaca organica accertata. La cardiomiopatia cirrotica è, di solito, subclinica e può essere riconosciuta solo attraverso il riscontro di alterazioni eco- e/o elettrocardiografiche. Tuttavia, essa può slatentizzarsi in occasione di eventi stressanti, quali emorragie digestive, infezioni batteriche, chirurgia maggiore, applicazione di shunt porto-cavale intraepatico transgiugulare [TIPS], od a seguito della somministrazione di farmaci vasocostrittori, situazioni che possono indurre un brusco incremento del lavoro cardiaco. Anche se non frequentemente, dopo trapianto di fegato od applicazione di TIPS è stata descritta l’insorgenza di insufficienza cardiaca conclamata, che rappresenta una causa di decesso in questi contesti. Infine, la presenza di cardiomiopatia cirrotica sembra avere un valore prognostico ai fini della sopravvivenza, che se questo aspetto non è stato ancora compiutamente definito.