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Congestive Heart Failure and Cardiomyopathy Mark Bromley PGY-1 Case 1 • A 63-year-old male presents with breathlessness x 3 days. ?Approach, ?Hx PMHx: MI 3 years ago • 4-vessel CABG • asymptomatic since surgery with no complaints of CP HPI: Over the last 3 months, the patient notes onset of shortness of breath while unloading groceries, and walking stairs. – 2 weeks ago, he was unable to complete his daily one-mile walk at the high school track. He noted swelling in his feet and ankles. – 4 days ago he woke at 2 am short of breath and had to sleep in his recliner the rest of the night. He has been unable to lay flat in bed at night since then and has slept on 3 pillows. – Yesterday, he became breathless walking from one room to another – He presents today with extreme shortness of breath – He denies chest pain BP 108/52 P 140, irreg. R 30 and labored Temp 99°F Ht: 5'8" Wt: 210. General: Breathless, moderately obese male in acute distress sitting upright complaining "I am going to die. Please help me." Chest: Scattered rhonchi throughout, rales bilateral one third lower bases. Cough is productive and frothy. CVS: Tachycardia and irreg. Grade 3/6 systolic murmur at LSB, S3 gallop noted. JVP to jaw Abd: Liver palpable three centimeters below right costal margin. HJR. Extremities: 4+ pitting edema of lower extremities to the knees. Pulses intact. • • • • • • • Organization of CHF Importance Pathophysiology Diagnosis Etiologies – Decompensation Management Cardiomyopathy Importance • Increasing burden • Aging population Improved survival (Hypertension/CAD) • Nationwide, heart failure affects more than 400 000 Canadians1 • (10% of those >75) • >50 000 new cases are diagnosed /yr1 Bad Disease • Mortality • 1 year – 10-20% • 6 year – 60-80% • ED visits • 20% new diagnosis • 80% repeat visit (decompensation) Definition • CHF – Inability of the heart to maintain adequate vital organ perfusion at normal filling pressures • Decreased exercise capacity • Associated neurohumoral-endocrine changes, initially compensatory but ultimately maladaptive • Pulmonary Edema – A condition associated with increased loss of fluid from the pulmonary capillaries into the pulmonary interstitium and alveoli The heart is a pump that works together with the lungs. It pumps blood in 2 ways 1. It pumps blood from the heart to the lungs to pick up oxygen. The oxygenated blood returns to the heart 2. It then pumps blood out into the circulatory system of blood vessels that carry blood through the body • In HF the pumping action of the heart becomes less and less efficient/powerful • The heart does not pump blood as well as it should • When this happens, blood does not move efficiently through the circulatory system • It starts to back up, increasing the pressure in the blood vessels, forcing fluid from the blood vessels into body tissues. Physiology • ↓ CO = HR X Stroke Volume • SV = preload + contractility - afterload • Preload – initial stretching of the cardiac myocytes prior to contraction • Afterload – the "load" that the heart must eject blood against – Aortic pressure Stroke Volume – Hypertrophy is a mechanism that allows more muscle fibers to share the work • Contractility – ability of a cardiac muscle fiber to contract at a given fiber length • catecholamines (norepi and epi) • sympathetic stimulation • Ca++ Neurohormonal Activation Jackson, G et al. BMJ 2000;320:167-170 Copyright ©2000 BMJ Publishing Group Ltd. [Nor-epi] and Mortality Jackson, G et al. BMJ 2000;320:167-170 Copyright ©2000 BMJ Publishing Group Ltd. Sympathetic Activation Jackson, G et al. BMJ 2000;320:167-170 Copyright ©2000 BMJ Publishing Group Ltd. Remodelling Post MI Jackson, G et al. BMJ 2000;320:167-170 Copyright ©2000 BMJ Publishing Group Ltd. Classification of Heart Failure • Low Output • Cardiac output is low, but demand for blood flow is normal • The heart is unable to meet this demand and fails • Dx: IHD, HTN, dilated cardiomyopathy, valvular and pericardial dz • High Output • High-output heart failure Cardiac output is normal or a little bit high • Demand for blood flow is abnormally high • (hyperthyroidism, anemia, severe infections) • The heart is unable to deliver the increased amount of blood and fails • Dx: hyperthyroidism, anemia, pregnancy, AV fistulas, beriberi, & Paget’s • Rx: ↓volume overload and correct the underlying disorder Classification of Heart Failure • Acute • MI, Acute Valve Dysfunction • largely systolic • sudden reduction in cardiac output often results in systemic hypotension without peripheral edema • Chronic • Cardiomyopathy • arterial pressure tends to be well maintained until very late in the course • there is often accumulation of peripheral edema Classification of Heart Failure • Right Sided (right ventricle) • (pulmonic stenosis or pulmonary hypertension) • When the right side of the heart starts to fail, fluid collects in the feet and lower legs • As the heart failure becomes worse, the legs swell and eventually the abdomen collects fluid (ascites, hepatic congestion) • Weight gain accompanies the fluid retention and is an excellent measure of how much fluid is being retained. • Left Sided (left ventricle) • left ventricle is mechanically overloaded (aortic stenosis) or weakened (post MI) • When the left side of the heart starts to fail, fluid collects in the lungs (Orthopnea/PND) • Breathing becomes more difficult, and the patient may feel short of breath, particularly with activity or lying down • This extra fluid in the lungs makes it more difficult for the airways to expand on inhalation • ↓O2 diffusion Classification of Heart Failure • Systolic – The heart has difficulty contracting and pumping out enough blood • weakness, fatigue and decreased ability to exercise ↓Ejection fraction = (stroke volume)/(end diastolic volume) – A normal ejection fraction is greater than 50% – Systolic heart failure has a ↓EF < 50%. • Diastolic – The heart is unable to fill properly during diastole – ↑ filling pressure – This impedes blood filling into the heart → backup into the lungs → CHF symptoms – ↑ in patients > 75 years; women; HTN – Ejection fraction is normal Classification of Heart Failure Directional Heart Failure • Backwards heart failure – The ventricle is not pumping out all the blood that comes into it. ↑ ventricular filling pressure and systemic or pulmonary edema – In fact, the heart can only meet the needs of the body if the ventricular filling pressure is high • Forward heart failure – The heart is not pumping out enough blood to meet the needs of the body – ↓ blood reaches the kidneys, they conserve salt and water, which contributes to excess fluid retention and edema – Forward failure also decreases the blood flow to various organs, causing weakness and fatigue Diagnosis • • • • History Phsyical EKG CXR History • Prior Heart Dz, Dyspnea, PND, Orthopnea, Fatigue, Cough, Bloating, Angina, ↑Wt/↑Girth, Nocturia • Precipitants – ↑salt, non-compliance, new meds, NSAIDs, palpitations, angina • Comorbidities • (COPD, Renal Dz, DM) PE • • • • ↑HR ↑RR JVP HJR Precordial exam: – Apical impulse • Location, Size, Sustained • (45’ LLD position – exp) – Loud P2 – S3 (ventricular vibration with rapid filling) • Low pitched (bell) 45’ LLD position • Lungs: – Cracks / Wheezes • Peripheral Edema Case 2 58 F with known Idiopathic Dilated Cardiomyopathy •Cardiac Cath (05): N coronaries LVEF 35% •Meds: ACE-I, Diuretics, Digoxin •↑SOBOE x 3weeks •OE: Displaced MPI •Soft S3 •Pre-sacral edema Case 3 70 M – Obese •SOBOE and fatigue x 3 months •No Orthopnea/PND •PMHx: Smoke x 40 pack-years, HTN (poorly controlled) DMII •OE: BP 180/100 •Sustained MPI •Bilateral Rales •Mild/Moderate Pretib Edema X-ray • Cardiomegally – ↑LV or hypertrophied ventricular wall – Cardiothoracic Ratio > 50% • Redistribution (cephalization) – Systolic dys-fxn → ↑filling pressure – Upper lobe vessels > Lower lobe vessels • Blunting of costophrenic Angle • Peribronchial cuffing • Kerly-B lines Peri-Bronchial Cuffing “First, strike for the jugular and let the rest go!” • Oliver W. Holmes Jr. Etiology • Dysrhythmia – Tachy • • • • ↓Diastolic filling time +/- atrial kick ↓CO ↓Coronary perfusion ↑ Myocardial O2 demand – Brady • ↓HR • ↓CO = SV x ↓HR • Infection • ↑ systemic met demands • Pulm infection = ↓O2 • Tachycardia • Anemia • Isovolumic hemodilution • ↑CO meet O2 demands • ↓Coronary O2 delivery • Pregnancy • ↑ demand for CO • Acute Myocarditis • ↓ contactility • Acute valvular dysfunction • 2o to MI • ↑preload or ↑↑afterload • PE • ↓O2 supply • ↑ Pulmonary Hypertension • Pharmacologic • ↓ inotropic effects • ↑ Na / H2O retention Etiology • Decompensation/Causes of exacerbation • FAILURE: Forgot medication25% Arrhythmia/ Anemia 10% Ischemia/ Infarction/ Infection (Pneumonia) 25% Lifestyle: taken too much salt Up-regulation of CO: pregnancy, hyperthyroidism Renal failure Embolism: pulmonary 10% Inappropriate treatment 20% Failure to seek care Case 1 Management • A 63-year-old male presents with breathlessness x 3 days. ?Approach, ?Hx PMHx: MI 3 years ago • 4-vessel CABG • asymptomatic since surgery with no complaints of CP HPI: Over the last 3 months, the patient notes onset of shortness of breath while unloading groceries, and walking stairs. – 2 weeks ago, he was unable to complete his daily one-mile walk at the high school track. He noted swelling in his feet and ankles. – 4 days ago he woke at 2 am short of breath and had to sleep in his recliner the rest of the night. He has been unable to lay flat in bed at night since then and has slept on 3 pillows. – Yesterday, he became breathless walking from one room to another – He presents today with extreme shortness of breath – He denies chest pain Management • Treat the maladaptation • ↑ Vascular resistance • ↑ Sympathetic tone • ↑ Total blood volume • ? LMNOP Management • • • • • • • • • Nitrates Nitrates Nitrates O2 Ventilation Assist Devices ACE I Diuretics Morphine Inotropes Airway/Breathing • Non-rebreather facemask delivering 100% O2 • Once initial therapy has begun, oxygen supplementation can be titrated in order to keep the patient comfortable and arterial oxygen saturation above 90 percent. NIPPV • If respiratory distress and/or hypoxia persist, consider non-invasive PPV – ↓ preload, ↓ afterload, and ↑ left ventricular performance – Meta-analysis of 15 clinical trials: ↓mortality and ↓intubation with NPPV compared to conventional therapy – Patients who fail/do not tolerate/have contraindications to NPPV should be intubated – Positive end-expiratory pressure is often useful for improving oxygenation • • • • • • • Multi-centre RCT 130 pts Cardiogenic Pulmonary Edema Emergency Department Medical therapy+O2 (65 pts) non-invasive pressure support ventilation (65 pts) Primary outcome – need for intubation Loop Diuretics • ↑Na & H2O excretion • Useful in volume overload • Rapid onset – Mild vasodilator • ↑1/2 life in CHF …be careful • Depletion of K + and Mg++ Loop Diuretics • Dosing – not well established • High dose Lasix and low dose Nitro has worse outcomes than low dose Lasix and high dose Nitro Morphine • Controversial • Weak vasodilator / ↓ Resp Drive • ↑ ICU admisssions (OR = 3) Sacchetti • ?Anxiolytic Nitrates • ↓preload @ low dose • ↓afterload @ high dose Nitrates - Route • SL/Spray (0.1 - 0.4mg / 5 min) x3 – 50-100ug/min • IV Drip (5-10ug/min) – titrate to effect – (100-200ug) • Transdermal – Peripherally shut down – Unreliable absorption • Contraindications / Cautions – Viagra – RV MI – Fixed Aortic Lesions Case 4 • 67 M ↑SOB Visibly Distressed 96 28 200/110 75% ORA • PMHx: HTN, DM II • EKG: Nitroprusside • Direct smooth muscle relaxant • Balanced reduction of pre/after load • Continuous pressure monitoring – good – Avoid hypotension Case 5 • 67 M ↑SOB Visibly Distressed 96 28 140/90 75% ORA • PMHx: HTN, DM II • Meds: Inconsistent Natriuretic Peptide Jackson, G et al. BMJ 2000;320:167-170 Copyright ©2000 BMJ Publishing Group Ltd. Nesiritide • • • • • Recombinant BNP ↓ Aldosterone ↓ Endothelin ↑ Na & H2O excretion – no reflex tachy Good for Nitro contraindications VMAC • • • • Randomized, Double Blind 489 inpatients IV Nisiritide vs IV Nitrates vs Placebo PCWP and Dyspnea – Improvement in PCWP – 2mm HG @ 6h – No change in Dyspnea • Problems: • • • • ↓Nitro dosing Prolonged Hypotension Not ED patients Industry sponsored Vosodilator Therapy: Nesiritide • Efficacy Phase – 432 patients – In the efficacy phase, 127 patients underwent hemodynamic monitoring with a pulmonary artery catheter – A six hour infusion of nesiritide (0.015 and 0.03 µg/kg per min) decreased pulmonary capillary wedge pressure – (6 and 10 mmHg versus an ↑ of 2 mmHg for placebo) – Improved the clinical status in a greater number of patients (60% and 67% versus 14%) • Comparative phase – 305 patients – Randomly assigned to nesiritide or standard vasoactive agents for seven days without hemodynamic monitoring – Compared to standard treatment with a single vasoactive agent (dobutamine, milrinone, nitroglycerin, or nitroprusside) – nesiritide produced a similar significant improvement in clinical status and reduction in dyspnea and fatigue that persisted during the entire infusion period. – Asymptomatic, dose-related hypotension was the most common side effect • Death within 30 days tended to occur more often among patients randomized to nesiritide therapy – 35 [7.2%] of 485 vs 15 [4.0%] of 377 patients • RR from meta-analysis: 1.74 – (95% confidence interval [CI], 0.97-3.12; P = .059) • Hazard ratio after adjusting for study, 1.80 – (95% CI, 0.98-3.31; P = .057) Vasodilator Therapy • Nitroglycerin: ↓preload ↓afterload • Nitroprusside: ↓ ↓ afterload • Nesiritide - BNP ACE Inhibitors • Clear longterm benefits 1. 2. 3. 4. • Vasodilate Block Aldosterone Bradykinin System ↓ Remodeling Take longer to work than nitrates • (peak effect 45min) Effect of ACE-I Jackson, G et al. BMJ 2000;320:167-170 Copyright ©2000 BMJ Publishing Group Ltd. Placebo-Controlled, Randomized, Double-Blind Study of Intravenous Enalaprilat Efficacy and Safety in Acute Cardiogenic Pulmonary Edema • The purpose of this study was to evaluate the efficacy and safety of a single IV 2-hour infusion of enalaprilat (1 mg) • placebo-controlled, randomized, double-blind study – 20 CHF patients (NYHA class III or IV) • Compared with placebo, enalaprilat – – – – – – – ↓ pulmonary capillary wedge pressure (-37% versus -10%, P=.001), ↓ diastolic and mean systemic blood pressures (-21% vs 0%, P=.009, and -18% vs -1%, P=.026) ↓ diastolic and mean pulmonary blood pressures (-21% vs -8%, P=.040; -18% vs -9%, P=.046) ↓ brachial and renal resistances (-44% versus -14%, P=.017, and -22% versus -2%, P=.014) ↑ brachial and renal blood flows (+77% versus +8%, P=.036, and +12% versus 0%, P=.043) ↑ arterial oxygen tension (+2% versus -16%, P=.041) ↑ arterial oxygen saturation (+1% versus -2%, P=.045) • Enalaprilat did not affect CO or carotid or hepatosplanchnic hemodynamics • Excluded those already on ACE I BNP • Why do we need another diagnostic test? – Diagnostic uncertainty • How uncertain are we? • How uncertain should we be? – ER docs are rarely wrong when they rate the probability of HF as very high or very low2 – ER docs are uncertain of the diagnosis in the in-betweeners (30%)2 BNP • ↑Ventricular pressure & ↑Stretch – Pro-BNP → Nt-BNP(↑spec) & BNP(↑sens) • • • • • Prospective, Blinded 1586 pts presenting with acute dyspnea ED Study Bedside assay “Gold Standard” = 2 cardiologists Breathing Not Properly Breathing Not Properly • ad Breathing Not Properly • • • • • • • Prospective diagnostic test evaluation 7 centers 1586 patients BNP – blinded Gold Standard – Cardiology Chart Review 97% certainty by ED physician At “80%” EP certainty • Sens: 49% Specificity: 96% • At 100 pg/ml • Sens: 90% Specificity: 73% • adf Breathing Not Properly - Analysis • BNP brings us closer to the “gold standard” • Low Prob -EP • 17% had CHF • 90% would have been corrected by BNP • High Prob -EP • 4% did not have CHF • 80% would have been corrected by BNP • Uncertain -EP • BNP correctly classified 74% • Misclassified 7% BNP • Levels < 100 pg/ml may prompt clinician to focus on alternative diagnosis (COPD) • Reasonable neg pred value • Prognostic Value • ?Variation with Age/Gender/Kidney fxn Prospective, Randomized, Controlled Single blind 452 Pts with dyspnea Diagnostic Strategy (BNP) vrs Standard Assessment Told EP if <100 CHF unlikely, >500 CHF likely, 100-500 indeterminate End Points: Time to D/C & Total Cost Safety: Similar Conclusion: BNP improved evaluation thereby improving and cost – no change in safety time to D/C • BNP strong prognostic indicator in symptomatic and asymptomatic individuals • Death or Cardiac Death • Additive to LVEF Cardiomyopathy • http://www.medmovie.com/mmdatabase /MedMovieRedirect.aspx?ClientID=27 Case • Previously well 10 year old girl presents with 2 weeks of progressive right sided ABD pain. General malaise. Mild fatigue on exertion. • Parents report mild lethargy, pallor and decreased PO intake over same period. Shallow rapid breathing, worse when reclining at night. – – – – – – 37.8oC 123 24 94/54 Pale irritable HEENT: MMM. No adenopathy CVS: regular S1S2, no murmer, PPPx4, no edema CHEST: AE=AE slight decrease at bases ABD: palpable liver edge below umbilicus • EKG: sinus tach, LAD, flattened T-waves, decreased voltages. Post Myocarditis Dilated Cardiomyopathy • Most common CM – 30% idiopathic – Hereditary • X-linked (dystrophin gene) – – – – Booze Heavy Metal Drugs Infectious • Viral • Chagas – – – – – – Post partum Collagen vascular disease Glycogen storage disease Thiamine, PO4, zinc deficiency Amyloidosis Neuromuscular disorders Dilated Cardiomyopathy • Biopsy helpful for etiology • Rx same as other CHF • MDC (Metoprolol in Dilated CM) – 34% reduction in Death • Multicenter Myocarditis Treatment Trial – no benefit of corticosteroids and azathioprine for Rx of biopsy-proven inflammation in dilated CM HCM • Inappropriate hypertrophy w/o stimulus • Usually asymmetric • 4% mortality per year (sudden death) HCM • 50% familial (Dominant Inheritance) • Mechanism – – – – – Abn Ca++ kinetics Abn sympathetic stim Abn Coronaries Subendocardial ischemia Structural abn HCM • 25% 1st relatives of HCM • Usually 3rd decade – not always • M>F HCM - Presentation • • • • • Sudden Death Dysrhythmia CHF Presyncope / Syncope Angina HCM • CHF Sx OE: • ↑JVP – prominent “a” wave • Double impulse pulse • PMI laterally displaced and increased • SEM / HSM • Split S2 HCM • Genetic Studies • ECG • Echo – LV outflow gradient >50mmHg – Diastolic dysfunction – HOCM: septum >1.4:1 ratio to post wall HCM – Approach/Mgmt • • • • • • • • • ABCD Normal CHF / CAD Rx Myomectomy Catheter septal ablation MV replacement ICD B-blockade CCB Anti-arrhythmias Restrictive • Least common CM • Incidence – likely under diagnosed • Poor prognosis Cardiomyopathy • http://www.medmovie.com/mmdatabase /MedMovieRedirect.aspx?ClientID=27 Restrictive • Idiopathic restrictive cardiomyopathy – EndoMyocardial Fibrosis – Loeffler eosinophilic endomyocardial disease • Secondary restrictive cardiomyopathy – – – – – – Radiation Hemochromatosis Amyloidosis Scleroderma Carcinoid heart disease Glycogen storage disease of the heart Diagnosis • Chest x-ray – Absence of cardiomegaly, normal cardiac silhouette – CHF • Electrocardiogram – – – – – LBBB common, RBBB possible Low voltage Nonspecific ST-T changes Various arrhythmias Chamber enlargement • Echocardiography – Normal to symmetrically thickened walls – Rapid early-diastolic filling, slow latediastolic filling – Normal or slightly reduced ventricular volume and systolic function • Cardiac catheterization – Elevated ventricular end-diastolic pressure – Dip and plateau configuration of the diastolic portion of the ventricular pressure pulse – Normal to slightly decreased ejection fraction – Prominent x and y descent • Endomyocardial biopsy – May detect typical eosinophil infiltration in the inflammatory stage – May detect myocardial fibrosis in later-stage cases – Negative findings do not exclude diagnosis • Treat underlying cause if possible • Therapy similar for other causes of CHF • Consider anticoagulation as prone to stasis – thromboembolism • Thanks Shawn for the resources! References 1. 2. Kostuk WJ. Congestive heart failure: what can we offer our patients? CMAJ 2001;165(8):1053-5 Schwam E. B-type natriuretic peptide for diagnosis of heart failure in emergency department patients: a critical appraisal. Acad Emerg Med 2004;11:686-91. Risk of HF after AMI Jackson, G et al. BMJ 2000;320:167-170 Copyright ©2000 BMJ Publishing Group Ltd. • 20 pts Pulmonary Edema • Looked like needed intubation • 2 intubated • Also had COPD • Mean Treatment Duration 2 ½ h