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Heart Failure

Heart Failure. John Lynn Jefferies, MD, MPH, FAAP, FACC Director, Cardiomyopathy, Advanced Heart Failure, and Ventricular Assist Device Programs Co-Director, Cardiovascular Genetics Associate Professor, Pediatric Cardiology and Adult Cardiovascular Diseases The Heart Institute

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Heart Failure

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  1. Heart Failure John Lynn Jefferies, MD, MPH, FAAP, FACC Director, Cardiomyopathy, Advanced Heart Failure, and Ventricular Assist Device Programs Co-Director, Cardiovascular Genetics Associate Professor, Pediatric Cardiology and Adult Cardiovascular Diseases The Heart Institute Cincinnati Children’s Hospital

  2. “Heart failure is a complex clinical syndrome that can result from any structural or functional cardiac disorder that impairs the ability of the ventricle to fill with or eject blood.” Heart Failure Defined Hunt SA et al. Circulation. 2001;104:2996

  3. Heart Failure: Why the Concern? • One in 8 deaths in the US has HF listed on the death certificate • Cost to the US for HF treatment was 39.2 billion dollars in 2010 • To deliver more specialized care in this area, most adult programs have developed HF programs

  4. Heart Failure: Why the Concern? • More than 5 million people in the US with the diagnosis of HF • Over 600,000 new diagnoses each year • Underestimates the pediatric and ACHD population • Many insurers are recognizing the importance of specialized care in their reimbursement

  5. Heart Failure • Heart Failure is a common problem world-wide • Historically thought of as volume overload • Current thought that this is a clinical syndrome • Should never be thought about as a “stand alone” diagnosis • Causes can be numerous • May have multiple etiologies in any patient

  6. Symptoms • Can be quite variable based on age of patient and phenotype • Swelling • Dyspnea on exertion • Palpitations • Weight gain • Syncope • Most common symptom in pediatric population – None • Failure to thrive, not meeting milestones

  7. Jefferies and Towbin. Lancet 2010;375:752-62.

  8. The Heart Failure Syndrome Fall in LV Performance Myocardial Injury Activation of RAAS and SNS (endothelin, AVP, cytokines) ANP BNP Peripheral Vasoconstriction Sodium/Water Retention Myocardial Toxicity Change in Gene Expression Remodeling and Progressive Worsening of LV Function HF Symptoms Morbidity and Mortality Shah M et al. Rev Cardiovasc Med. 2001;2(suppl 2):S2

  9. The Heart Failure Syndrome E Braunwald. NEJM 2008;358:2148-59.

  10. The Heart Failure Syndrome Bowles et al. Herz 2000;28:169-175.

  11. Final Common Pathways ACTC MYH7 TNNC1 TNNT2 TPM1 LMNA LDB3 PLN SCN5A Impaired force transmission Impaired force generation Changes in nuclear structure and function Changes in stretch sensor machinery Calcium regulation Sodium regulation DILATED CARDIOMYOPATHY

  12. Ronco et al. J Am Coll Cardiol 2008;52:1527-39.

  13. Ronco et al. J Am Coll Cardiol 2008;52:1527-39.

  14. Cardiomyopathies • Hypertrophic Cardiomyopathy (HCM) • Dilated Cardiomyopathy (DCM) • Restrictive Cardiomyopathy (RCM) • Left Ventricular Noncompaction (LVNC) • Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

  15. Systolic Heart Failure • Depressed ventricular function • Classically describing the left ventricle • Increasingly described in the right ventricle or in both ventricles • Noninvasive imaging typical mode of diagnosis • Echocardiography, CT, MRI, Nuclear, Invasive Angiography • In adults, typical cause is ischemic • In children, infectious or genetic more common

  16. Heart Failure With Preserved Ejection Fraction (HFpEF) • Formerly known as diastolic heart failure • 50% of adult heart failure cases • Unknown prevalence in children • Outcome similar to those patients with HF and systolic dysfunction • Increasing in incidence compared to HFrEF • Treatment options not as robust • Continuum of myocardial dysfunction?

  17. Mechanism of HFpEF Borlaug and Paulus. Eur Heart J 2011;32:670-679.

  18. Right Ventricular Heart Failure Haddad et al. J Cardiac Fail 2011;17:533-539.

  19. Renal Function in Chronic Heart Failure Waldum et al. J Cardiac Fail 2010;16:374-380.

  20. Renal Function in Chronic Heart Failure Waldum et al. J Cardiac Fail 2010;16:374-380.

  21. Cardiorenal Syndrome in Pediatrics Price et al. Pediatr Crit Care Med 2008;9:279-84.

  22. The Big Picture of Heart Failure Setaguchi et al. Am Heart J 2007;154:260-6

  23. Increasing Need • Difficult to assess the prevalence of cardiomyopathy accurately • ~5 million Americans suffer from symptomatic heart failure • Estimated 50 million Americans meet criteria for Stage A or B • We expect this underestimates the at risk populations • Underuse of appropriate screening for at risk populations

  24. Conclusions • Heart failure is a complex clinical syndrome • Proper management dependent on recognition of appropriate components of care • Not just diuresis and inotropes • Understanding etiology often more challenging in pediatric populations • Collaborative approach with multiple disciplines including Nephrology leads to best outcome

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