title: Clinical Spectrum, Therapeutic Options, and Outcome of Advanced Heart Failure in Hypertrophic Cardiomyopathy.
abstract: BACKGROUND
  The clinical course of patients with hypertrophic cardiomyopathy and advanced heart failure (HF) subtended by progressive left ventricular dysfunction has received limited attention. Our aim was to assess the outcome of HF and impact of treatment options including the implantable cardioverter-defibrillator and heart transplantation (HT) in patients with hypertrophic cardiomyopathy evaluated at 2 Italian referral centers >3 decades.
  
  
  RESULTS
  All-cause mortality and a combined end point including death, HT, or appropriate implantable cardioverter-defibrillator shock were assessed in 71 consecutive patients with HF not related to outflow obstruction (7% of the entire hypertrophic cardiomyopathy cohort) followed up for 6.1±6.9 years after development of New York Heart Association class III to IV symptoms. At enrollment, left ventricular ejection fraction was <50% in 55 patients and >50% in 16; all had restrictive left ventricular filling. During follow-up, 35 patients died (49%%; 5-year rate, 49%) and 53 met the combined end point (75%; 5-year rate, 62%). Most events occurred in the 3 years after HF onset (17% per year compared with only 3% per year subsequently). Appropriate implantable cardioverter-defibrillator shocks occurred in 11 of 34 implanted patients. Of 37 patients evaluated for HT, 14 were transplanted, 10 listed, and 13 excluded; 2 early post-HT deaths occurred in patients with elevated pulmonary vascular resistance. Eleven of the 14 HT patients were alive at 10±8 years.
  
  
  CONCLUSIONS
  In hypertrophic cardiomyopathy, advanced HF not associated with outflow obstruction portends a severely unfavorable prognosis, particularly in the first 3 years after onset of symptoms, despite frequently preserved systolic function in about one quarter of the patients. Outcome of HT is favorable but requires early consideration, as the window of opportunity may be short.
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.). pasqualucci.daniele@gmail.com.
authors.forename: Daniele
authors.initials: D
authors.lastname: Pasqualucci
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Alessandra
authors.initials: A
authors.lastname: Fornaro
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Gabriele
authors.initials: G
authors.lastname: Castelli
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Alessandra
authors.initials: A
authors.lastname: Rossi
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Anna
authors.initials: A
authors.lastname: Arretini
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Chiara
authors.initials: C
authors.lastname: Chiriatti
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Mattia
authors.initials: M
authors.lastname: Targetti
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Francesca
authors.initials: F
authors.lastname: Girolami
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Marco
authors.initials: M
authors.lastname: Corda
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Pierpaolo
authors.initials: P
authors.lastname: Orrù
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Gildo
authors.initials: G
authors.lastname: Matta
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Stefàno
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Franco
authors.initials: F
authors.lastname: Cecchi
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Maurizio
authors.initials: M
authors.lastname: Porcu
authors.affiliation: From the Departments of Cardiology (D.P., M.C., P.O., M.P.) and Radiology (G.M.), Brotzu Hospital, Cagliari, Italy; Referral Center for Cardiomyopathies (A.F., G.C., A.R., A.A., C.C., M.T., I.O.), Genetic Diagnostics Unit (F.G.), and Cardiac Surgery (P.S.), Careggi University Hospital, Florence, Italy; and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy (F.C.).
authors.forename: Iacopo
authors.initials: I
authors.lastname: Olivotto
mesh_terms: D000328:Adult; D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D015331:Cohort Studies; D017147:Defibrillators, Implantable; D005260:Female; D006333:Heart Failure / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D016027:Heart Transplantation; D006801:Humans; D007558:Italy; D008297:Male; D008875:Middle Aged; D013318:Stroke Volume; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000150:complications* / Q000175:diagnosis / Q000401:mortality
keywords: cardiomyopathy, hypertrophic; defibrillators, implantable; heart failure; heart transplantation; ventricular dysfunction, left