Clinical Spectrum, Therapeutic Options, and Outcome of Advanced @entity712 in XXXX .

@entity1 @entity712 @entity3585 @entity1457 @entity737 @entity299 @entity1121 @entity1002
BACKGROUND: -The clinical course of @entity1 with @entity1121 ( @entity1121 ) and advanced @entity712 ( @entity712 ) subtended by progressive @entity1002 has received limited attention. Our aim was to assess the outcome of @entity712 and impact of treatment options including the @entity737 ( @entity737 ) and @entity299 ( @entity299 ) in @entity1121 @entity1 evaluated at two Italian referral centers over 3 decades. METHODS AND RESULTS: -All-cause mortality and a combined end-point including death, @entity299 or appropriate @entity737 shock was assessed in 71 consecutive @entity1 with @entity712 not related to @entity3585 (7% of the entire @entity1121 cohort) followed for 6.1  6.9 years after development of NYHA class III-IV symptoms. At enrollment, LV ejection fraction was <50% in 55 @entity1 and >50% in 16; all had restrictive LV filling. During follow-up, 35 @entity1 died (49%%; 5-year rate 49%) and 53 met the combined end-point (75%; 5-year rate 62%). Most events occurred in the three years after @entity712 onset (17%/year, compared to only 3%/year subsequently). Appropriate @entity1457 occurred in 11 of 34 implanted @entity1 . Of 37 @entity1 evaluated for @entity299 , 14 were transplanted, 10 listed and 13 excluded; two early post- @entity299 deaths occurred in @entity1 with elevated pulmonary vascular resistance. Eleven of the 14 @entity299 @entity1 were alive at 10  8 years. CONCLUSIONS: -In @entity1121 , advanced @entity712 not associated with @entity3585 portends a severely unfavorable prognosis, particularly in the first three years following onset of symptoms, despite frequently preserved systolic function in about one quarter of the @entity1 . Outcome of @entity299 is favourable but requires early consideration, as the window of opportunity may be short.
