Congestive Heart Failure (CHF)

Definitions

  • Heart Failure with Preserved Ejection Fraction (HFpEF): defined as congestive heart failure with EF >50%
  • Heart Failure with Borderline Preserved Ejection Fraction (HFpEF, Borderline): defined as congestive heart failure with EF 41-49%
    • Clinical characteristics are similar to patients with HFpEF
  • Heart Failure with Improved Ejection Fraction (HFpEF, Improved): defined as congestive heart failure with EF >40% in patient with prior HFrEF
    • This represents the subset of HFpEF patient who previously previously had HFrEF: these patients with improvement or recovery in ejection fraction may be clinically distinct from those with persistently preserved or reduced ejection fraction
  • Heart Failure with Reduced Ejection Fraction (HFrEF): defined as congestive heart failure with EF equal to or <40%

Epidemiology

Statistics

  • Lifetime Risk of Developing Congestive Heart Failure for Americans Equal to or >40 y/o: 20%
  • Incidence of Congestive Heart Failure (US): >650k new cases diagnosed annually
    • Incidence increases with age
  • Prevalence of of Clinically Manifest Congestive Heart Failure (US): 5.1 million patients
    • Prevalence continues to increase
  • Prevalence of Asymptomatic Left Ventricular Dysfunction: ranges from 6-21%
    • Prevalence increases with age
    • Left Ventricular Dysfunction Prevention Study: untreated asymptomatic left ventricular dysfunction carries a 10% risk for developing congestive heart failure symptoms and an 8% risk of death or congestive heart failure-related hospitalization annually
  • Annual Costs (Heath Care + Lost Productivity) Related to Congestive Heart Failure (US): $30 Billion

Risk Factors

  • Coronary Artery Disease (CAD) (see Coronary Artery Disease, [[Coronary Artery Disease]]): relative risk = 8.1
    • Most common etiology of systolic congestive heart failure in Western countries
    • Presence of athersclerotic disease (of coronary arteries, cerebral arteries, or peripheral arteries) increases the risk of developing congestive heart failure
  • Cigarette Smoking (see Tobacco, [[Tobacco]]): relative risk = 1.6
  • Diabetes Mellitus (DM) (see Diabetes Mellitus, [[Diabetes Mellitus]]): relative risk = 1.9
    • Diabetes mellitus is a risk factor for congetsive heart failure, independent of age, hypertension, obesity, hyperlipidemia, and coronary artery disease
    • Diabetes mellitus also adversely impacts the outcome of patients with established congestive heart failure
  • Valvular Heart Disease: relative risk = 1.5
  • Hypertension (see Hypertension, [[Hypertension]]): relative risk = 1.4
    • Increases the risk of congestive heart failure at all ages
    • Risk of congestive heart failure increases with the degree of blood pressure elevation, older age, and longer duration of hypertension
    • Long-term treatement of both systolic and diastolic hypertension decreases the risk of congestive heart failure by 50%
  • Obesity (see Obesity, [[Obesity]]): relative risk = 1.3
    • Represents a risk factor for both systolic and diastolic congestive heart failure
  • Metabolic Syndrome (Any 3 of the Following -> Abdominal Adiposity + Hypertrgliglyceridemia + Low Density Lioproteinemia + Hypertension + Fasting Hyperglycemia): treatment of hypertension, hyperlipidemia, and diabetes mellitus decrease the risk of developing congestive heart failure

Relative Prevalence of Etiologies in Patients Who Initially Presented with Unexplained Dilated Cardiomyopathy (2000) [MEDLINE]

  • Idiopathic Dilated Cardiomyopathy (50%)
  • Other (10%)
  • Myocarditis (9%)
  • Ischemic Heart Disease (7%)
  • Infiltrative Disease (5%)
  • Peripartum Cardiomyopathy 4%)
  • Hypertension (4%)
  • Human Immunodeficiency Virus (HIV) (4%)
  • Connective Tissue Disease (3%)
  • Substance Abuse (3%)
  • Doxorubicin (1%)

Etiologic Classification (American Heart Association Classification, 2006) [MEDLINE]

General Comments

  • Definition of Cardiomyopathy: “Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure–related disability.”
  • Result of Cardiomyopathy
    • Diastolic/Systolic Dysfunction
    • Primary Electrical Disease with Propensity for Arrhythmias
  • Difficulties with Classification Schemes
    • Some diseases do not have a uniformly static expression and may evolve (due to remodeling and other factors) from one category to another during their natural clinical course
      • Examples: hypertrophic cardiomyopathy, amyloidosis, and other infiltrative disease may progress from a non-dilated (often hyperdynamic) state with ventricular stiffness to a dilated form with systolic dysfunction
    • Qantitative assessment of ventricular size is a continuum and patients can vary widely in their degree of dilatation (with minimal cavity enlargement early in a disease process): it is often difficult to strictly differentiate dilated and non-dilated forms of cardiomyopathy

Primary Cardiomyopathies (Predominantly Involving the Heart)

Genetic

  • Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D)
  • Conduction System Disease
    • Lenegre Disease
    • Sick Sinus Syndrome
  • Glycogen Storage Diseases
    • Danon
    • PRKAG2
  • Hypertrophic Cardiomyopathy
  • Ion Channelopathies
    • Brugada Syndrome
    • Catecholaminergic Polymorphic Ventricular Tachycardia
    • Idiopathic Ventricular Fibrillation
    • Long-QT Syndrome
    • Short-QT Syndrome
  • Left Ventricular Noncompaction
  • Mitochondrial Myopathies

Mixed (Predominantly Non-Genetic; Familial Disease with a Genetic Oigin has been Reported in a Minority of Cases)

  • Dilated Cardiomyopathy: this is a heterogeneous group of disorders characaterized by ventricular dilation and decreased myocardial contractility in the absence of abnormal loading (valvular heart disease, hypertension)
  • Restrictive Cardiomyopathy (Non-Dilated and Non-Hypertrophied

Acquired

  • Cardiomyopathy in Infants of Insulin-Dependent Diabetic Mothers
  • Myocarditis (Inflammatory Cardiomyopathy) (see Myocarditis, [[Myocarditis]])
    • Epidemiology: accounts for approximately 9% of initially unexplained cardiomyopathy cases
    • Clinical
      • Acute Course: distinct onset with severe hemodynamic compromise
      • Subacute Course: indistinct onset with better tolerated left ventricular dysfunction
  • Peripartum Cardiomyopathy
    • Incidence: occurs in 1:1300-1:4000 liver births
    • Risk Factors: advanced maternal age, multiparity, African descent, and long-term tocolysis
    • Clinical: left ventricular dysfunction occurs in the last trimester or early puerperium
  • Tachycardia-Induced Cardiomyopathy
    • Physiology: may be caused by supraventricular tachycardias, ventricular tachycardia, ventricular pacing at high rates, or frequent premature ventricular contractions (PVC’s)
    • Clinical: degree of left ventricular dysfunction is correlated with the duration of the tachyarrhythmia
    • Treatment: often reversible (but may not be complete in some cases)
  • Stress Cardiomyopathy (Takotsubo Cardiomyopathy)
    • Epidemiology: most often affects post-menopausal women
    • Diagnosis
      • Acute reversible left ventricular dysfunction (triggered by acute emotional or physical stress) in the absence of significant coronary artery disease
      • Echocardiogram with distinctive apical ballooning
    • Clinical
      • Acute Coronary Syndrome with Transiently Elevated Cardiac Enzymes: typical presentation

Secondary Cardiomyopathies

Endomyocardial

  • Endomyocardial Fibrosis
  • Hypereosinophilic Syndrome (Löeffler’s Endocarditis)

Infiltrative (Accumulation of Abnormal Substances in Extracellular Space Between Myocytes)

  • Amyloidosis (see Amyloidosis, [[Amyloidosis]])
    • Epidemiology: cardiac involvement is most common with AL amyloidosis (monoclonal kappa or lambda light chains) and senile TTR amyloidosis (wild-type transthyretin) subtypes
  • Gaucher’s Disease
  • Hunter’s Syndrome
  • Hurler’s Syndrome

Storage (Accumulation of Abnormal Substances Intracellularly Within Myocytes)

  • Fabry’s Disease
  • Hemochromatosis (see Hemochromatosis, [[Hemochromatosis]])
    • Clinical: systolic or diastolic dysfunction
  • Niemann-Pick Disease
  • Ochronosis
  • Oxalosis
  • Pompe’s Disease

Inflammatory

  • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
    • Epidemiology: may affect as many as 25% of patients with systemic sarcoidosis (but is often underdiagnosed)
    • Clinical
      • Asymptomatic Left Ventricular Dysfunction
      • Congestive Heart Failure
      • Atrioventricular Blocks
      • Atrial/Ventricular Arrhythmias
      • Sudden Cardiac Death

Neoplasm

Hematologic Disease

Endocrine

  • Acromegaly (see Acromegaly, [[Acromegaly]])
    • Physiology: myocardial hypertrophy with interstitial fibrosis, lympho-mononuclear cell infiltration, myocyte necrosis, and biventricular concentric hypertrophy
  • Diabetes Mellitus (see Diabetes Mellitus, [[Diabetes Mellitus]])
    • Mortality Rate is Related to Hemoglobin A1C Level: inverted-U relationship with highest mortality rate with extremely low or high hemoglobin A1C levels
  • Growth Hormone Deficiency: growth hormone and insulin-like growth factor 1 and required for cardiac development
  • Hyperparathyroidism (see Hyperparathyroidism, [[Hyperparathyroidism]])
  • Hyperthyroidism (see Hyperthyroidism, [[Hyperthyroidism]])
    • Physiology: most commonly occurs in the setting of persistent sinus tachycardia or atrial fibrillation -> consequently, may be related to the tachycardia alone
  • Hypothyroidism (see Hypothyroidism, [[Hypothyroidism]])
    • Clinical: both systolic and diastolic dysfunction have been reported
      • Myxedema: low cardiac output state usually results from bradycardia, decreased ventricular filling, decreased myocardial contractility and decreased myocardial work
  • Obesity (see Obesity, [[Obesity]])
    • Physiology
      • Excessive adipose tissue results in increasing circulating blood volume with resulting increased cardiac output, increased cardiac work, and hypertension
      • Possible contributions of lipotoxicity-induced cardiac myocyte injury and myocardial lipid accumulation
  • Pheochromocytoma (see Pheochromocytoma, [[Pheochromocytoma]])

Cardiofacial

  • Lentiginosis
  • Noonan Syndrome

Neuromuscular/Neurologic

  • Chronic Progressive External Opthmoplegia (Kearns-Savre)
  • Duchenne-Becker Muscular Dystrophy
  • Emery-Dreifuss Muscular Dystrophy
  • Familial Centronuclear Myopathy
  • Fascioscapulohumeral Dystrophy (Landouzy-Dejerine)
  • Friedrich’s Ataxia
  • Humuloperitoneal Ataxia
  • Juvenile Progressive Spinal Muscular Atrophy (Kugelberg-Welander)
  • Limb-Girdle Muscular Dystrophy
  • Myotonia Atrophica (Steinert)
  • Myotonic Dystrophy
  • Neurofibromatosis
  • Tuberous Sclerosis

Nutritional

  • Carnitine Deficiency (see Carnitine, [[Carnitine]])
    • Physiology: L-carnitine is a necessary cofactor for fatty acid oxidation
    • Clinical: progressive skeletal myopathy and cardiomyopathy
  • Keshan’s Disease
  • Kwashiorkor
  • Niacin Deficiency (Pellagra) (see Niacin, [[Niacin]])
  • Selenium Deficiency (see Selenium, [[Selenium]])
  • Thiamine Deficiency (Beriberi) (see Thiamine, [[Thiamine]])
  • Vitamin C Deficiency (Scurvy) (see Vitamin C, [[Vitamin C]])

Rheumatologic

  • Behcet’s Disease (see Behcet’s Disease, [[Behcets Disease]])
  • Churg-Strauss Syndrome (see Churg-Strauss Syndrome, [[Churg-Strauss Syndrome]])
  • Microscopic Polyangiitis (see Microscopic Polyangiitis, [[Microscopic Polyangiitis]])
  • Polyarteritis Nodosa (PAN) (see Polyarteritis Nodosa, [[Polyarteritis Nodosa]])
  • Polydermatomyositis (see Polydermatomyositis, [[Polydermatomyositis]])
  • Rheumatoid Arthritis (RA) (see Rheumatoid Arthritis, [[Rheumatoid Arthritis]])
    • Epidemiology: development of dilated cardiomyopathy is rare
    • Physiology: microvasculitis and microcirculatory disturbances, resulting in myocarditis
    • Clinical
      • Mycocarditis: myocardial disease can occur in the absence of clinical symptoms or EKG changes
      • Pericarditis
  • Scleroderma (see Scleroderma, [[Scleroderma]])
    • Epidemiology: rare etiology of dilated cadiomyopathy
    • Clinical: subclinical systolic dysfunction is present in the majority of scleroderma patients
  • Systemic Lupus Erythematosus (SLE) (see Systemic Lupus Erythematosus, [[Systemic Lupus Erythematosus]])
  • Wegener’s Granulomatosis (see Wegener’s Granulomatosis, [[Wegeners Granulomatosis]])

Drug/Toxin

  • 5-Fluorouracil (5-FU) (see 5-Fluorouracil, [[5-Fluorouracil]])
  • Amphetamine (see Amphetamine, [[Amphetamine]])
  • Anabolic Steroids (see xxxx, [[xxxx]])
  • Antimony (see Antimony, [[Antimony]])
  • Antracyclines
    • Daunorubicin (Daunomycin, Cerubidine) (see Daunorubicin, [[Daunorubicin]])
      • Physiology: iron-chelating agents that prevent generation of oxygen free radicals (dexrazoxane) are cardioprotective against the development of anthracycline-induced cardiomyopathy
    • Doxorubicin (Adriamycin) (see Doxorubicin, [[Doxorubicin]])
      • Physiology: iron-chelating agents that prevent generation of oxygen free radicals (dexrazoxane) are cardioprotective against the development of anthracycline-induced cardiomyopathy
  • Arsenic (see Arsenic, [[Arsenic]])
  • Carbon Monoxide (see Carbon Monoxide, [[Carbon Monoxide]])
  • Carbon Tetrachloride (see Carbon Tetrachloride, [[Carbon Tetrachloride]])
  • Catecholamines
  • Chloroquine (see Chloroquine, [[Chloroquine]])
  • Clozapine (see Clozapine, [[Clozapine]])
  • Cobalt (see Cobalt, [[Cobalt]])
  • Cocaine (see Cocaine, [[Cocaine]])
    • Epidemiology: long-term cocaine abuse can produce cardiomyopathy (even in the absence of coronary artery disease, myocardial infarction, or vasculitis)
  • Cyclocphosphamide (Cytoxan) (see Cyclophosphamide, [[Cyclophosphamide]])
    • Epidemiology: cardiotoxicity is associated with high-dose administration
  • Emetine (see Emetine, [[Emetine]])
  • Ephedra (see Ephedra, [[Ephedra]])
    • Epidemiology: banned by the FDA
    • Clinical
      • Left Ventricular Systolic Dysfunction/Congestive Heart Failure
      • Sudden Cardiac Death
  • Ethanol (see Ethanol, [[Ethanol]])
    • Epidemiology: one of the most important etiologies of dilated cardiomyopathy
      • Alcoholic cardiomyopathy most commonly occurs in men 30-55 y/o who have been drinking heavily for >10 yrs
      • In contrast, mild-moderate ethanol consumption has been found to be protective against the development of congestive heart failure in poulation studies
  • Interferons
  • Lead (see Lead, [[Lead]])
  • Lithium (see Lithium, [[Lithium]])
  • Mercury (see Mercury, [[Mercury]])
  • Methamphetamine (see Methamphetamine, [[Methamphetamine]])
    • Acute Methamphetamine Intoxication
    • Chronic Methamphetamine Abuse
  • Methylphenidate (Ritalin) (see Methylphenidate, [[Methylphenidate]])
  • Methysergide (see Methysergide, [[Methysergide]])
  • Mitomycin-C (see Mitomycin, [[Mitomycin]])
  • Phenothiazines (se Phenothiazines, [[Phenothiazines]])
    • Aliphatic Compounds
      • Chlorpromazine (Thorazine, Largactil, Megaphen) (see Chlorpromazine, [[Chlorpromazine]])
      • Levomepromazine
      • Methotrimeprazine (Nozinan, Levoprome)
      • Promazine (Sparine)
      • Triflupromazine
    • Piperidines
      • Mesoridazine (Serentil)
      • Thioridazine (Mellaril) (see Thioridazine, [[Thioridazine]])
    • Piperazines
      • Fluphenazine (Prolixin)
      • Perphenazine (Trilafon)
      • Prochloperazine (Compazine) (see Prochlorperazine, [[Prochlorperazine]])
      • Trifluoperazine (Stelazine)
  • Phosphorous
  • Taxol Derivatives
    • Docetaxel (Taxotere) (see Docetaxel, [[Docetaxel]])
    • Paclitaxel (Taxol) (see Paclitaxel, [[Paclitaxel]])
  • Trastuzumab (Herceptin) (see Trastuzumab, [[Trastuzumab]])
    • Epidemiology: risk or cardiotoxicity is increased with concomitant anthracycline administration
    • Physiology: unlike anthracycline-induced cardiotoxicity, risk of cardiomyopathy with trastuzumab does not appear to be related to cumulative dose or result in ultrastructural myocardial changes
    • Treatment: often reversible with discontinuation of trastuzumab
  • Tricyclic Antidepressants
    • XXXX (see xxxx, [[xxxx]])
  • Zidovudine (see Zidovudine, [[Zidovudine]])

Other

  • Hypokalemia (see Hypokalemia, [[Hypokalemia]])
  • Radiation

Other

Coronary Artery Disease (CAD) (see Coronary Artery Disease, [[Coronary Artery Disease]])

  • Epidemiology: most common etiology of systolic congestive heart failure (accounts for 62% of all cases)

Hypertension (see Hypertension, [[Hypertension]])

  • Epidemiology: common etiology of systolic congestive heart failure (accounts for 10% of all cases)

Valvular Heart Disease

  • General Comments: common etiology of systolic congestive heart failure
  • Aortic Insufficiency (AI) (see Aortic Insufficiency, [[Aortic Insufficiency]])
  • Aortic Stenosis (AS) (see Aortic Stenosis, [[Aortic Stenosis]])
  • Mitral Stenosis (see Mitral Stenosis, [[Mitral Stenosis]])

Miscellaneous

  • Ruptured Sinus of Valsalva Aneurysm (see xxxx, [[Sinus of Valsalva Aneurysm]])
  • Metoclopramide (Reglan) (see Metoclopramide, [[Metoclopramide]]): due to increased aldosterone secretion, resulting in fluid retention

Physiology

Left Ventricular Hypertrophy

  • Etiology of Left Ventricular Hypertrophy
    • Coronary Artery Disease (CAD)
    • Diabetes Mellitus (DM)
    • Hypertension
    • Valvular Heart Disease
  • Degree of Left Ventricular Hypertrophy Correlates with the Risk of Heart Falure of Any Etiology: interestingly, there does not appear to be an ability to separate compensatory from pathologic left ventricular hypertrophy
  • Distinguishing Systolic vs Diastolic Congestive Heart Failure
    • Mean LV Mass-Volume Ratio: mean LV mass-volume ratio is increased in diastolic congestive heart failure (2.12), as compared to systolic congestive heart failure (1.22) and controls (1.49) [MEDLINE]

Diagnosis

Electrocardiogram (EKG)

  • xxx

Plasma Brain Natriuretic Peptide (BNP) or N-Terminal pro-B-Type Natriuretic Peptide (NT-pro-BNP) (see Brain Natriuretic Peptide, [[Brain Natriuretic Peptide]])

  • Both are derived from the 108-amino acid precursor peptide (proBNP108) that is generated by cardiac myocytes in response to myocardial stretch (and other triggers)
  • BNP is elevated in both systolic and diastolic congestive heart failure
  • Positive Predictive Value: lower values of BNP exclude the presence of congestive heart failure with a reasonably high positive predictive value
    • However, BNP is less specific, as there are multiple other etiologies which may elevate BNP levels

Echocardiogram (see Echocardiogram, [[Echocardiogram]])

  • xxx

Cardiac Catheterization with Coronary Angiogram

  • xxx

Swan-Ganz Catheterization (see Swan-Ganz Catheter, [[Swan-Ganz Catheter]])

  • xxx

Endomyocardial Biopsy

  • xxx

Exercise Testing

  • Ventilatory Anaerobic Threshold: similar in systolic and diastolic congestive heart failure, both are decreased as compared to controls [MEDLINE]

CXR/Chest CT

  • xxx

  • CXR/Chest CT patterns:

    • Cardiomegaly with usually bilateral effusions: cardiomegaly is almost always present (in absence of cardiomegaly, only 4% of effusions are due to CHF)/ pulmonary vascular congestion (may be seen)
      • Autopsy studies, 88% are bilateral/ 8% are unilateral right-sided/ 4% are unilateral left-sided (Race, 1957)
      • Mean volume on right side was only slightly greater than volume on left side: presence of highly assymmetric effusions suggests an etiology other than CHF

Clinical Manifestations

General Comments

  • Comparison of Clinical Manifestations in Systolic vs Diastolic Congestive Heart Failure: clinical manifestations are very similar for systolic and diastolic congestive heart failure [MEDLINE]
  • New York Heart Association (NYHA) Clinical Classification of Congestive Heart Failure Severity
    • Class I: no limitation of physical activity
      • No symptoms of congestive heart failure (fatigue, dyspnea) occur with ordinary physical activity
    • Class II: slight limitation of physical activity
      • Symptoms of congestive heart failure occur with ordinary activity, but there are no symptoms at rest
    • Class III: marked limitation of physical activity
      • Symptoms of congestive heart failure occur with less than ordinary physical activity, but there are no symptoms at rest
    • Class IV: inability to carry out any physical activity without discomfort
      • Symptoms of congestive heart failure may occur even at rest

Cardiovascular Manifestations

  • Low Cardiac Output State/Cardiogenic Shock (see Cardiogenic Shock, [[Cardiogenic Shock]])
  • Ventricular Thrombus (see Intracardiac Thrombus, [[Intracardiac Thrombus]])
  • Poor Tolerance of Hemodynamic Stresses: in diastolic congestive heart failure
    • Atrial Fibrillation (see Atrial Fibrillation, [[Atrial Fibrillation]]): loss of atrial contraction leads to significantly impaired ventricular filling and decreases stroke volume
    • Hypertension (see Hypertension, [[Hypertension]]): leads to increased left ventricular wall stress, which impairs myocardial relaxation
      • This is particularly true when hypertensive is abrupt and severe
    • Myocardial Ischemia: leads to increased left atrial pressures with consequent increase in pulmonary artery pressure
    • Tachycardia (see Sinus Tachycardia, [[Sinus Tachycardia]]): leads to shortened diastole, which decreases ventricular filling

Gastrointestinal/Hepatic Manifestations

Hematologic Manifestations

Pulmonary Manifestations

Cardiac Asthma (see Obstructive Lung Disease, [[Obstructive Lung Disease]])

  • Physiology: pulmonary venous hypertension
    • Mitral Stenosis: upper lobe vascular distention occurs with relative lower lobe oligemia
  • Diagnosis
    • Pulmonary Function Tests (PFT’s)
      • Mitral Stenosis: decreased FEV1, decreased FVC, and increased RV correlate with the mitral valve gradient
        • Methacholine Challenge: may be positive (likely due to dilation of bronchial vessels induced by methacholine)
    • CXR: left atrial enlargement
      • Double-Shadow Sign: due to enlarged LA
      • Straightened Left Heart Border: due to enlarged LA
    • Echocardiogram: diagnostic of mitral stenosis, aortic stenosis, and left-sided systolic and diastolic dysfunction
  • Clinical
    • Dyspnea
    • Hemoptysis: especially seen in mitral stenosis cases
    • Wheezing

Pleural Effusion (see [[Pleural Effusion-Transudate]] and [[Pleural Effusion-Exudate]])

  • Epidemiology
    • Most common cause of pleural effusion (about 500,000 cases per year in USA)
    • In LV failure, effusions occur 8% of the time (by autopsy studies, 72% had effusions >250 mL)
    • CHF is the most common cause of bilateral pleural effusion
    • 25% of effusions in patients with CHF are due to other etiologies
  • Physiology
    • Mechanism: leakage of fluid mostly from alveolar capillaries (rather than pleural capillaries) -> interstitial edema -> passage of fluid across visceral pleura into pleural space
      • In volume-loading studies in sheep, pleural fluid contains the same protein content as the lymph and interstitial edema fluid in the lung (about 25% of all the edema fluid in the lung passes into the pleural space)
      • Clearance of Fluid from Pleural Space: almost all fluid exits pleural space via parietal lymphatics (lymphatic clearance is decreased in the presence of increased systemic veous pressure)
    • Hemodynamic Correlates of Congestive Heart Failure-Associated Pleural Effusion: presence of pleural effusion by ultrasound correlates more closely with pulmonary venous pressure than with systemic venous pressure or pulmonary artery pressure
      • Effusions occur mainly with left-sided congestive heart failure and are uncommon in patients with isolated right-sided congestive heart failure
  • Diagnosis
    • Need for Thoracentesis: as many as 25% of pleural effusions in patients with congestive heart failure are due to other causes -> therefore, thoracentesis may be required in the setting of fever, unilateral/asymmetric effusion, pleuritic chest pain, or absence of cardiomegaly on chest x-ray
    • Transudate: however, congestive heart failure-associated transudate may convert to an exudate over time (with a long-standing effusion) or with diuresis (even over a couple of days)
      • Appearance: usually straw-colored or yellow (although may be blood-tinged in some cases)
      • LDH Ratio: <0.6
      • Pleural Fluid LDH: <66% of upper limit of normal for serum
      • Total Protein Ratio: <0.5
      • Serum/Pleural Albumin Gradient (SPAG): may be useful in ambiguous cases to confirm that fluid is actually a transudate
        • SPAG >1.2 g/dL: confirms that fluid is a transudate
      • Cell Count/Diiff: may be PMN-predominant in some cases
  • Clinical
    • Decreased Breath Sounds
    • Dullness to Percussion
    • Resting/Exertional Dyspnea: dyspnea may be out of proportion to the size of pleural effusion

Pulmonary Edema (see Pulmonary Edema, [[Pulmonary Edema]])

  • Interstitial Pulmonary Edema
    • Diagnosis
      • CXR Pattern
        • Interstitial Infiltrates
        • Kerley B Lines: thickened interlobular septa (seen at periphery of lung) due to pulmonary lymphatic obstruction (associated with increased LA pressures)
  • Alveolar Pulmonary Edema
    • Diagnosis
      • CXR Pattern: alveolar filling

Pulmonary Hypertension (see Pulmonary Hypertension, [[Pulmonary Hypertension]])

  • Epidemiology
    • Left-sided congestive heart failure is the most frequent cause of pulmonary hypertension
  • Physiology
    • Increased left atrial pressure -> passive backward transmission of the pressure leading to increased pulmonary artery pressure
  • Diagnosis
    • Swan-Ganz Catheter: elevated pulmonary artery pressure with normal pulmonary vascular resistance (PVR)
      • In some patients with left heart disease, the elevation of pulmonary artery pressure is out of proportion to that expected from the elevation of left arterial pressure
      • In patients referred to cardiac transplant clinics, pulmonary hypertension with PVR >3.0 Wood units is reported in 19% to 35% of patients: the elevation of PAP and PVR is due to either the increase of pulmonary artery vasomotor tone and/or pulmonary vascular remodeling
  • Clinical
    • Dyspnea (see Dyspnea, [[Dyspnea]])
    • Signs of Right-Sided Congestive Heart Failure
  • Treatment: the efficacy and safety of pulmonary artery hypertension medications in this population is unclear

Other

Other Manifestations


Treatment

Aldosterone-Receptor Antagonists

  • Agents
    • Eplerenone (Inspra) (see Eplerenone, [[Eplerenone]])
    • Spironolactone (Aldactone) (see Spironolactone, [[Spironolactone]])
  • Indications
    • xxx

Angiotensin Converting Enzyme (ACE) Inhibitors (see Angiotensin Converting Enzyme Inhibitors, [[Angiotensin Converting Enzyme Inhibitors]])

  • Agents
    • xxx
  • Indications
    • xxx

Angiotensin II Receptor Blockers (ARB’s) (see Angiotensin II Receptor Blockers, [[Angiotensin II Receptor Blockers]])

  • Agents
    • xxx
  • Indications
    • xxx

Beta Blockers (see β-Adrenergic Receptor Antagonists, [[β-Adrenergic Receptor Antagonists]])

  • Agents
    • xxx
  • Indications
    • xxx

Digoxin (Lanoxin) (see Digoxin, [[Digoxin]])

  • Indications
    • xxx

Diuretics

  • Agents
    • xxx
  • Indications
    • xxx

Hydralazine + Isosorbide Dinitrate (see Hydralazine, [[Hydralazine]] and Isosorbide, [[Isosorbide]])

  • Indications
    • xxx

Statins (see HMG-CoA Reductase Inhibitors, [[HMG-CoA Reductase Inhibitors]])

  • No Clinical Benefit, in the Absence of Other Indications

BNP-Guided Therapy

  • Systematic Review/Meta-Analysis (2015) [MEDLINE]: BNP-guided therapy may improve the clinical outcomes of congestive heart failure patients, if substantial reduction of BNP can be achieved

Treatment of Congestive Heart Failure-Associated Pleural Effusion

  • Therapeutic Thoracentesis: may be required to relieve dyspnea
  • Treatment of Underlying CHF: usually leads to resolution of pleural effusion within days of diuresis
  • Pleurodesis: may be necessary in few cases with symptomatic persistent or recurrent effusion even after vigorous treatment of CHF
    • Talc Slurry (5g) (see Talc, [[Talc]]) or Doxycycline (5 mg/kg) (see Doxycycline, [[Doxycycline]]): preferred agents
    • Bleomycin (see Bleomycin, [[Bleomycin]]): not recommended (ineffective in a rabbit model)
  • Pleuroperitoneal Shunt: alternative to pleurodesis (successfully used in a few cases)

Prognosis

  • 5-Year Mortality Rate: 50%

References

  • Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med. 2000;342(15):1077 [MEDLINE]
  • Pathophysiological characterization of isolated diastolic heart failure in comparison to systolic heart failure. JAMA. 2002 Nov 6;288(17):2144-50 [MEDLINE]
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