Acute Heart Failure Dilated NonischemicFamilial dilated cardiomyopathy Myocardial

Acute Heart Failure • Dilated – – – Nonischemic/Familial dilated cardiomyopathy Myocardial ischemia Valvular disease Peripartum cardiomyopathy Toxin • Non-dilated – – – Myocarditis Myocardial ischemia Valvular disease Infiltrative cardiomyopathy Acute stress cardiomyopathy

Differential Diagnosis • Ischemic heart disease • Valvular heart disease – Endocarditis • Sepsis – immunocompromised • Unmasked cirrhotic cardiomyopathy • Infiltrative cardiomyopathy

Clinical Assessment of Hemodynamic Status Congestion at Rest NO Low Perfusion at Rest N O Y E S YES A B Warm & Dry Warm & Wet (Low Profile) (Complex) L C Cold & Dry Signs/Symptoms of Congestion: : Orthopnea / PND Cough Dyspnea Elevated JVP Hepatomegaly Edema Rales Cold & Wet Possible Evidence of Low Perfusion: Cool extremities/poor pulses Hyponatremia Decreased mental status Hypotension Narrow pulse pressure Renal dysfunction Nohria A et al. , JAMA 2002

Hemodynamic Changes in Cirrhosis • Increased cardiac output 9. 0 ± 3. 0 l/min • Decreased systemic vascular resistance 861 ± 333 dynes-sec-cm-5 • Decreased mean arterial pressure 76 ± 16 mm. Hg • Normal wedge pressure 10 ± 5 Dig Dis Sci 2009; 54: 869

Cirrhotic Cardiomyopathy • “A form of chronic cardiac dysfunction in patients with cirrhosis, characterized by blunted contractile responsiveness to stress, and/or altered diastolic relaxation with electrophysiologic abnormalities in the absence of other known cardiac disease. ” Gut 2008; 57: 268

Differential Diagnosis • • • Ischemic heart disease Valvular heart disease Sepsis Unmasked cirrhotic cardiomyopathy Infiltrative cardiomyopathy

Infiltrative Cardiomyopathy • Amyloidosis • Sarcoidosis • Hemochromatosis

Iron Overload • Hereditary Hemochromatosis – C 282 Y homozygous – C 282 Y/H 63 D compound heterozygote • Secondary – Anemia ± Transfusions – Liver Disease – Dietary iron overload

Porto-systemic Shunting • Decrease in first-pass iron metabolism in the liver • Increased duodenal iron absorption • Although rare, case reports of shunts potentially leading to iron overload states including cardiomyopathy (1960 s)

Diagnosis • Progressive heart failure culminating in cardiogenic shock due to secondary hemochromatosis • Potential contribution from “cirrhotic cardiomyopathy”