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End-Stage Diastolic and Systolic Heart Failure: Evaluation and Timing of Heart Transplantation

  • Eric D. Popjes
  • Anjali Tiku Owens
  • Mariell Jessup
Chapter

Abstract

End stage hypertrophic cardiomyopathy occurs in an estimated 3–15 % of patients and can present as either systolic or diastolic dysfunction. Risk factors for developing end stage disease include a family history of end stage disease, younger age at initial diagnosis, increased wall thickness and persistent arrhythmia. The classic form of adverse remodeling includes left ventricular cavity dilation with regression of hypertrophy and decrease in ejection fraction. Standard medical therapy for systolic heart failure and consideration of prophylactic defibrillator is indicated when LVEF is less than 50 %. Heart transplant is a viable option for patients with end stage hypertrophic cardiomyopathy, including those with systolic heart failure, diastolic heart failure or refractory arrhythmia. Strategies used to bridge patients to transplant include continuous inotropic infusion, left ventricular assist device, intra-aortic balloon pump, and in rare cases extracorporeal membrane oxygenation. Survival after heart transplant for hypertrophic cardiomyopathy is equal to or better than survival for patients who have other types of cardiomyopathies.

Key Words

Congestive heart failure End-stage HCM LV systolic dysfunction LV Diastolic dysfunction Restrictive physiology Left ventricular assist device (LVAD) Heart transplantation 

References

  1. 1.
    Biagini E, Coccolo F, Ferlito M, Perugini E, Rocchi G, Bacchi-Reggiani L, et al. Dilated-hypokinetic evolution of hypertrophic cardiomyopathy: prevalence, incidence, risk factors, and prognostic implications in pediatric and adult patients. J Am Coll Cardiol. 2005;46(8):1543–50.PubMedCrossRefGoogle Scholar
  2. 2.
    Harris KM, Spirito P, Maron MS, Zenovich AG, Formisano F, Lesser JR, et al. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation. 2006;114(3):216–25.PubMedCrossRefGoogle Scholar
  3. 3.
    Spirito P, Maron BJ, Bonow RO, Epstein SE. Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol. 1987;60(1):123–9.PubMedCrossRefGoogle Scholar
  4. 4.
    Thaman R, Gimeno JR, Murphy RT, Kubo T, Sachdev B, Mogensen J, et al. Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy. Heart. 2005;91(7):920–5.PubMedCentralPubMedCrossRefGoogle Scholar
  5. 5.
    Hamada T, Kubo T, Kitaoka H, Hirota T, Hoshikawa E, Hayato K, et al. Clinical features of the dilated phase of hypertrophic cardiomyopathy in comparison with those of dilated cardiomyopathy. Clin Cardiol. 2010;33(7):E24–8.PubMedCrossRefGoogle Scholar
  6. 6.
    Olivotto I, Maron BJ, Appelbaum E, Harrigan CJ, Salton C, Gibson CM, et al. Spectrum and clinical significance of systolic function and myocardial fibrosis assessed by cardiovascular magnetic resonance in hypertrophic cardiomyopathy. Am J Cardiol. 2010;106(2):261–7.PubMedCrossRefGoogle Scholar
  7. 7.
    Maron MS. Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Cardiovasc Magn Reson. 2012;14:13.PubMedCentralPubMedCrossRefGoogle Scholar
  8. 8.
    Mancini DM, Eisen H, Kussmaul W, Mull R, Edmunds LH, Wilson JR. Value of peak exercise oxygen consumption for optimal timing of cardiac transplantation in ambulatory patients with heart failure. Circulation. 1991;83(3):778–86.PubMedCrossRefGoogle Scholar
  9. 9.
    Yancy CW, Jessup M, Bozkurt B, Butler J, Casey Jr DE, Drazner MH, et al. 2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2013;62(16):e147–239.PubMedCrossRefGoogle Scholar
  10. 10.
    Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, et al. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2011;58(25):e212–60.PubMedCrossRefGoogle Scholar
  11. 11.
    Rogers DPS, Marazia S, Chow AW, Lambiase PD, Lowe MD, Frenneaux M, et al. Effect of biventricular pacing on symptoms and cardiac remodelling in patients with end-stage hypertrophic cardiomyopathy. Eur J Heart Fail. 2008;10(5):507–13.PubMedCrossRefGoogle Scholar
  12. 12.
    Ashrafian H, Mason MJ, Mitchell AG. Regression of dilated-hypokinetic hypertrophic cardiomyopathy by biventricular cardiac pacing. Europace. 2007;9(1):50–4.PubMedCrossRefGoogle Scholar
  13. 13.
    Rose EA, Gelijns AC, Moskowitz AJ, Heitjan DF, Stevenson LW, Dembitsky W, et al. Long-term use of a left ventricular assist device for end-stage heart failure. N Engl J Med. 2001;345(20):1435–43.PubMedCrossRefGoogle Scholar
  14. 14.
    Slaughter MS, Pagani FD, McGee EC, Birks EJ, Cotts WG, Gregoric I, et al. HeartWare ventricular assist system for bridge to transplant: combined results of the bridge to transplant and continued access protocol trial. J Heart Lung Transplant. 2013;32(7):675–83.PubMedCrossRefGoogle Scholar
  15. 15.
    Miller LW, Pagani FD, Russell SD, John R, Boyle AJ, Aaronson KD, et al. Use of a continuous-flow device in patients awaiting heart transplantation. N Engl J Med. 2007;357(9):885–96.PubMedCrossRefGoogle Scholar
  16. 16.
    Cho YH, Deo SV, Topilsky Y, Grogan MA, Park SJ. Left ventricular assist device implantation in a patient who had previously undergone apical myectomy for hypertrophic cardiomyopathy. J Card Surg. 2012;27(2):266–8.PubMedCrossRefGoogle Scholar
  17. 17.
    Wynne E, Bergin JD, Ailawadi G, Kern JA, Kennedy JLW. Use of a left ventricular assist device in hypertrophic cardiomyopathy. J Card Surg. 2011;26(6):663–5.PubMedCrossRefGoogle Scholar
  18. 18.
    Muthiah K, Phan J, Robson D, Macdonald PS, Keogh AM, Kotlyar E, et al. Centrifugal continuous-flow left ventricular assist device in patients with hypertrophic cardiomyopathy: a case series. ASAIO J. 2013;59(2):183–7.PubMedCrossRefGoogle Scholar
  19. 19.
    Topilsky Y, Pereira NL, Shah DK, Boilson B, Schirger JA, Kushwaha SS, et al. Left ventricular assist device therapy in patients with restrictive and hypertrophic cardiomyopathy. Circ Heart Fail. 2011;4(3):266–75.PubMedCrossRefGoogle Scholar
  20. 20.
    Maron MS, Kalsmith B, Udelson JE, Wenjun L, DeNofrio D. Survival after cardiac transplantation in patients with hypertrophic cardiomyopathy. Circ Heart Fail. 2010;3:574–9.PubMedCrossRefGoogle Scholar
  21. 21.
    Coutu M, Perrault LP, White M, Pelletier GB, Racine N, Poirier NC, et al. Cardiac transplantation for hypertrophic cardiomyopathy: a valid therapeutic option. J Heart Lung Transplant. 2004;23(4):413–7.PubMedCrossRefGoogle Scholar
  22. 22.
    Lund LH, Edwards LB, Kucheryavaya AY, Dipchand AI, Benden C, Christie JD, et al. The Registry of the International Society for Heart and Lung Transplantation: Thirtieth Official Adult Heart Transplant Report—2013; focus theme: age. J Heart Lung Transplant. 2013;32(10):951–64.PubMedCrossRefGoogle Scholar
  23. 23.
    Biagini E, Spirito P, Leone O, Picchio FM, Coccolo F, Ragni L, et al. Heart transplantation in hypertrophic cardiomyopathy. Am J Cardiol. 2008;101(3):387–92.PubMedCrossRefGoogle Scholar
  24. 24.
    Huang XS, Chen LW, Pan YC, Lin F, Wang QM, Huang ZY, et al. Outcome post orthotopic heart transplantation for patients with end-stage hypertrophic cardiomyopathy. Zhonghua Xin Xue Guan Bing Za Zhi. 2011;39(2):114–7.PubMedGoogle Scholar
  25. 25.
    Kato TS, Takayama H, Yoshizawa S, Marboe C, Schulze PC, Farr M, et al. Cardiac transplantation in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2012;110(4):568–74.PubMedCrossRefGoogle Scholar
  26. 26.
    Waller Md TA, Hiser Md WL, Capehart Md JE, Roberts Md WC. Comparison of clinical and morphologic cardiac findings in patients having cardiac transplantation for ischemic cardiomyopathy, idiopathic dilated cardiomyopathy, and dilated hypertrophic cardiomyopathy. Am J Cardiol. 1998;81(7):884–94.CrossRefGoogle Scholar
  27. 27.
    Lipshultz SE, Orav EJ, Wilkinson JD, Towbin JA, Messere JE, Lowe AM, et al. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet. 2013;382(9908):1889–97.PubMedCrossRefGoogle Scholar
  28. 28.
    Ragni L, Biagini E, Picchio FM, Prandstraller D, Leone O, Berardini A, et al. Heart transplantation in infants with idiopathic hypertrophic cardiomyopathy. Pediatr Transplant. 2009;13(5):650–3.PubMedCrossRefGoogle Scholar
  29. 29.
    Gajarski R, Naftel DC, Pahl E, Alejos J, Pearce FB, Kirklin JK, et al. Outcomes of pediatric patients with hypertrophic cardiomyopathy listed for transplant. J Heart Lung Transplant. 2009;28(12):1329–34.PubMedCrossRefGoogle Scholar
  30. 30.
    Singh TP, Almond CS, Piercey G, Gauvreau K. Current outcomes in US children with cardiomyopathy listed for heart transplantation. Circ Heart Fail. 2012;5(5):594–601.PubMedCrossRefGoogle Scholar
  31. 31.
    Prandstraller D, Leone O, Biagini E, Picchio FM, Rapezzi C. Prenatal echographic recognition of hypertrophic cardiomyopathy leading to heart transplantation in the newborn. Eur Heart J. 2008;29(7):845.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  • Eric D. Popjes
    • 1
  • Anjali Tiku Owens
    • 2
    • 3
  • Mariell Jessup
    • 3
  1. 1.Heart and Vascular InstitutePenn State Hershey Medical CenterHersheyUSA
  2. 2.Hospital of the University of PennsylvaniaPhiladelphiaUSA
  3. 3.Cardiovascular Medicine Division, Department of MedicineHospital of the University of Pennsylvania, University of Pennsylvania Perelman School of MedicinePhiladelphiaUSA

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