Advertisement

Comparison of and Frequency of Morality, Left Ventricular Assist Device Implantation, Ventricular Arrhythmias, and Heart Transplantation in Patients With Familial Versus Nonfamilial Idiopathic Dilated Cardiomyopathy

      We postulated that familial idiopathic dilated cardiomyopathy (F-IDC) is associated with a worse prognosis than nonfamilial IDC (nonF-IDC). Patients with F-IDC had either a strong family history and/or proved genetic mutations. We studied long-term prognosis (mean follow-up: 6.1 ± 4.1 years) of 162 patients with IDC (age: 55.5 ± 17.9 years, men: 57.8%, 50% F-IDC) with an implantable cardioverter-defibrillator or cardiac resynchronization therapy. The primary end point was a composite of death, left ventricular (LV) assist device implant, or heart transplantation. The secondary end point was a ventricular arrhythmia event. There was no significant difference in the prevalence of diabetes, hypertension, New York Heart Association class, medical therapy, and years of follow-up between the F-IDC and nonF-IDC groups. Patients with F-IDC were younger than patients with nonF-IDC (49.1 ± 17.0 years vs 61.6 ± 16.5 years, p <0.001). Mean LV ejection fraction was significantly lower in F-IDC group than in the nonF-IDC group (26 ± 12% vs 31 ± 12%, p = 0.022). The primary end point was achieved in 54 patients in F-IDC group (66.7%) versus 19 in the nonF-IDC group (23.5%) (p <0.001). The Kaplan–Meier survival estimates for the composite end point and for ventricular arrhythmia were significantly lower in the F-IDC versus nonF-IDC (log-rank p ≤0.001 and 0.04, respectively). F-IDC was the only multivariable predictor of the primary composite end point (hazard ratio 3.419 [95% confidence interval 1.845 to 6.334], p <0.001). The likelihood of LV remodeling manifested by LV ejection fraction improvement (≥10%) was significantly lower in F-IDC than nonF-IDC (27.1% vs 44.8%, p = 0.042). In conclusion, F-IDC is a predictor of mortality, need for LV assist device, or heart transplantation. F-IDC is associated with significantly lower event-free survival for primary end point and ventricular arrhythmia than nonF-IDC. F-IDC has significantly lower likelihood of LV reverse remodeling than nonF-IDC.

      Abbreviations:

      F-IDC (Familial idiopathic dilated cardiomyopathy), LVAD (Left ventricular assist device), NonF-IDC (Nonfamilial idiopathic dilated cardiomyopathy)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to American Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Taylor DO
        • Edwards LB
        • Boucek MM
        • Trulock EP
        • Aurora P
        • Christie J
        • Dobbels F
        • Rahmel AO
        • Keck BM
        • Hertz MI
        Registry of the International Society for Heart and Lung Transplantation: twenty-fourth official adult heart transplant report–2007.
        J Heart Lung Transplant. 2007; 26: 769-781
        • Zegkos T
        • Panagiotidis T
        • Parcharidou D
        • Efthimiadis G.
        Emerging concepts in arrhythmogenic dilated cardiomyopathy.
        Heart Fail Rev. 2021; 26: 1219-1229
        • Corsello A.
        An implantable cardioverter-defibrillator but not amiodarone reduced risk for death in congestive heart failure.
        ACP J Club. 2005; 143: 6
        • Bansch D
        • Antz M
        • Boczor S
        • Volkmer M
        • Tebbenjohanns J
        • Seidl K
        • Block M
        • Gietzen F
        • Berger J
        • Kuck KH.
        Primary prevention of sudden cardiac death in idiopathic dilated cardiomyopathy: the Cardiomyopathy Trial (CAT).
        Circulation. 2002; 105: 1453-1458
        • Stavrakis S
        • Asad Z
        • Reynolds D.
        Implantable cardioverter defibrillators for primary prevention of mortality in patients with nonischemic cardiomyopathy: a meta-analysis of randomized controlled trials.
        J Cardiovasc Electrophysiol. 2017; 28: 659-665
        • Køber L
        • Thune JJ
        • Nielsen JC
        • Haarbo J
        • Videbæk L
        • Korup E
        • Jensen G
        • Hildebrandt P
        • Steffensen FH
        • Bruun NE
        • Eiskjær H
        • Brandes A
        • Thøgersen AM
        • Gustafsson F
        • Egstrup K
        • Videbæk R
        • Hassager C
        • Svendsen JH
        • Høfsten DE
        • Torp-Pedersen C
        • Pehrson S
        • Investigators DANISH
        Defibrillator implantation in patients with nonischemic systolic heart failure.
        N Engl J Med. 2016; 375: 1221-1230
        • Bardy GH
        • Lee KL
        • Mark DB
        • Poole JE
        • Packer DL
        • Boineau R
        • Domanski M
        • Troutman C
        • Anderson J
        • Johnson G
        • McNulty SE
        • Clapp-Channing N
        • Davidson-Ray LD
        • Fraulo ES
        • Fishbein DP
        • Luceri RM
        • Ip JH
        Sudden Cardiac Death in Heart Failure Trial (SCD-HeFT) Investigators. Amiodarone or an implantable cardioverter-defibrillator for congestive heart failure.
        N Engl J Med. 2005; 352: 225-237
        • Epstein AE
        • DiMarco JP
        • Ellenbogen KA
        • Estes 3rd, NA
        • Freedman RA
        • Gettes LS
        • Gillinov AM
        • Gregoratos G
        • Hammill SC
        • Hayes DL
        • Hlatky MA
        • Newby LK
        • Page RL
        • Schoenfeld MH
        • Silka MJ
        • Stevenson LW
        • Sweeney MO
        • Smith Jr, SC
        • Jacobs AK
        • Adams CD
        • Anderson JL
        • Buller CE
        • Creager MA
        • Ettinger SM
        • Faxon DP
        • Halperin JL
        • Hiratzka LF
        • Hunt SA
        • Krumholz HM
        • Kushner FG
        • Lytle BW
        • Nishimura RA
        • Ornato JP
        • Page RL
        • Riegel B
        • Tarkington LG
        • Yancy CW
        American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices), American Association for Thoracic Surgery, Society of Thoracic Surgeons. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons.
        J Am Coll Cardiol. 2008; 51: e1-e62
        • Fonarow GC
        • Feliciano Z
        • Boyle NG
        • Knight L
        • Woo MA
        • Moriguchi JD
        • Laks H
        • Wiener I.
        Improved survival in patients with nonischemic advanced heart failure and syncope treated with an implantable cardioverter-defibrillator.
        Am J Cardiol. 2000; 85: 981-985
        • Halliday BP
        • Cleland JGF
        • Goldberger JJ
        • Prasad SK.
        Personalizing risk stratification for sudden death in dilated cardiomyopathy: the past, present, and future.
        Circulation. 2017; 136: 215-231
        • Towbin JA
        • McKenna WJ
        • Abrams DJ
        • Ackerman MJ
        • Calkins H
        • Darrieux FCC
        • Daubert JP
        • de Chillou C
        • DePasquale EC
        • Desai MY
        • Estes 3rd, NAM
        • Hua W
        • Indik JH
        • Ingles J
        • James CA
        • John RM
        • Judge DP
        • Keegan R
        • Krahn AD
        • Link MS
        • Marcus FI
        • McLeod CJ
        • Mestroni L
        • Priori SG
        • Saffitz JE
        • Sanatani S
        • Shimizu W
        • van Tintelen JP
        • Wilde AAM
        • Zareba W.
        2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: executive summary.
        Heart Rhythm. 2019; 16: e373-e407
        • Verdonschot JAJ
        • Hazebroek MR
        • Krapels IPC
        • Henkens MTHM
        • Raafs A
        • Wang P
        • Merken JJ
        • Claes GRF
        • Vanhoutte EK
        • van den Wijngaard A
        • Heymans SRB
        • Brunner HG.
        Implications of genetic testing in dilated cardiomyopathy.
        Circ Genom Precis Med. 2020; 13: 476-487
        • Peters S
        • Kumar S
        • Elliott P
        • Kalman JM
        • Fatkin D.
        Arrhythmic genotypes in familial dilated cardiomyopathy: implications for genetic testing and clinical management.
        Heart Lung Circ. 2019; 28: 31-38
        • Merlo M
        • Gentile P
        • Artico J
        • Cannatà A
        • Paldino A
        • De Angelis G
        • Barbati G
        • Alonge M
        • Gigli M
        • Pinamonti B
        • Ramani F
        • Zecchin M
        • Pirozzi F
        • Stolfo D
        • Sinagra G.
        Arrhythmic risk stratification in patients with dilated cardiomyopathy and intermediate left ventricular dysfunction.
        J Cardiovasc Med (Hagerstown). 2019; 20: 343-350
        • Bozkurt B
        • Colvin M
        • Cook J
        • Cooper LT
        • Deswal A
        • Fonarow GC
        • Francis GS
        • Lenihan D
        • Lewis EF
        • McNamara DM
        • Pahl E
        • Vasan RS
        • Ramasubbu K
        • Rasmusson K
        • Towbin JA
        • Yancy C
        American Heart Association Committee on Heart Failure and Transplantation of the Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Epidemiology and Prevention; and Council on Quality of Care and Outcomes Research. Current diagnostic and treatment strategies for specific dilated cardiomyopathies: a scientific statement From the American Heart Association.
        Circulation. 2016; 134: e579-e646
        • Pinto YM
        • Elliott PM
        • Arbustini E
        • Adler Y
        • Anastasakis A
        • Böhm M
        • Duboc D
        • Gimeno J
        • de Groote P
        • Imazio M
        • Heymans S
        • Klingel K
        • Komajda M
        • Limongelli G
        • Linhart A
        • Mogensen J
        • Moon J
        • Pieper PG
        • Seferovic PM
        • Schueler S
        • Zamorano JL
        • Caforio AL
        • Charron P.
        Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice: a position statement of the ESC working group on myocardial and pericardial diseases.
        Eur Heart J. 2016; 37: 1850-1858
        • Orphanou N
        • Papatheodorou E
        • Anastasakis A.
        Dilated cardiomyopathy in the era of precision medicine: latest concepts and developments.
        Heart Fail Rev. 2022; 27: 1173-1191
        • Hershberger RE
        • Siegfried JD.
        Update 2011: clinical and genetic issues in familial dilated cardiomyopathy.
        J Am Coll Cardiol. 2011; 57: 1641-1649
        • McNally EM
        • Mestroni L.
        Dilated cardiomyopathy: genetic determinants and mechanisms.
        Circ Res. 2017; 121: 731-748
        • Moretti M
        • Merlo M
        • Barbati G
        • Di Lenarda A
        • Brun F
        • Pinamonti B
        • Gregori D
        • Mestroni L
        • Sinagra G.
        Prognostic impact of familial screening in dilated cardiomyopathy.
        Eur J Heart Fail. 2010; 12: 922-927
        • Cannatà A
        • De Angelis G
        • Boscutti A
        • Normand C
        • Artico J
        • Gentile P
        • Zecchin M
        • Heymans S
        • Merlo M
        • Sinagra G.
        Arrhythmic risk stratification in non-ischaemic dilated cardiomyopathy beyond ejection fraction.
        Heart. 2020; 106: 656-664
        • Escobar-Lopez L
        • Ochoa JP
        • Mirelis JG
        • Espinosa MÁ
        • Navarro M
        • Gallego-Delgado M
        • Barriales-Villa R
        • Robles-Mezcua A
        • Basurte-Elorz MT
        • Gutiérrez García-Moreno L
        • Climent V
        • Jiménez-Jaimez J
        • Mogollón-Jiménez MV
        • Lopez J
        • Peña-Peña ML
        • García-Álvarez A
        • Brion M
        • Ripoll-Vera T
        • Palomino-Doza J
        • Tirón C
        • Idiazabal U
        • Brögger MN
        • García-Hernández S
        • Restrepo-Córdoba MA
        • Gonzalez-Lopez E
        • Méndez I
        • Sabater M
        • Villacorta E
        • Larrañaga-Moreira JM
        • Abecia A
        • Fernández AI
        • García-Pinilla JM
        • Rodríguez-Palomares JF
        • Gimeno-Blanes JR
        • Bayes-Genis A
        • Lara-Pezzi E
        • Domínguez F
        • Garcia-Pavia P
        Association of genetic variants With outcomes in patients With nonischemic dilated cardiomyopathy.
        J Am Coll Cardiol. 2021; 78: 1682-1699
        • Khayata M
        • Al-Kindi SG
        • Oliveira GH.
        Contemporary characteristics and outcomes of adults with familial dilated cardiomyopathy listed for heart transplantation.
        World J Cardiol. 2019; 11: 38-46