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13 The risk of developing HF was six times greater in hypertensive versus

normotensive patients.

Cardiac Contractility

The terms contractility and inotropic state are used synonymously to describe the

myocardium’s ability to develop force and shorten its fibers independent of preload

or afterload. Myocardial contractility is decreased when myocardial fibers are

diminished or poorly functioning as may occur in patients with primary

cardiomyopathy, valvular heart disease, CAD, or after a myocardial infarction (MI).

Defects in contractility play a major role in systolic HF, but are not a factor for

diastolic dysfunction. Drugs such as β-adrenergic blockers or anthracyclines

precipitate HF by decreasing myocardial contractility.

Heart Rate

An increased HR is a reflex mechanism to improve CO as EF declines. The

sympathetic nervous system is the major mediator of this response. The workload and

energy demands of a rapid HR ultimately place strain on the heart and can eventually

worsen HF.

p. 264

p. 265

Pathophysiology

When the heart begins to fail, the body activates several complex compensatory

mechanisms in an attempt to maintain CO and oxygenation. These include increased

sympathetic tone, activation of the renin–angiotensin–aldosterone system (RAAS),

sodium and water retention, and other neurohormonal adaptations, which lead to

cardiac “remodeling” (ventricular dilatation, cardiac hypertrophy, and changes in LV

shape). The consequences of these adaptive mechanisms can create more harm than

good (Fig. 14-2). The relative balance of each of these adaptive processes varies

depending on the type of HF (systolic versus diastolic dysfunction) and from patient

to patient with the same disorder. An understanding of the potential benefits and

adverse consequences of these compensatory mechanisms is essential to

understanding the signs, symptoms, and treatment of HF.

14

SYMPATHETIC (ADRENERGIC) NERVOUS SYSTEM

The body’s normal physiologic response to a decreased CO is generalized activation

of the adrenergic (sympathetic) nervous system as evidenced by increased circulating

levels of norepinephrine (NE) and other catecholamines. The inotropic (increased

contractility) and chronotropic (increased HR) effects of NE initially maintain nearnormal CO and perfusion of vital organs such as the central nervous system (CNS)

and myocardium. Adverse consequences of NE activation include impaired sodium

excretion by the kidneys, restricted ability of the coronary arteries to supply blood to

the ventricle (myocardial ischemia), increased arrhythmias, hypokalemia, and

oxidative stress triggering cell death (apoptosis).

Chronic high levels of catecholamines are harmful to the heart because they

decrease β1

-receptor sensitivity and reduce β1

-receptor density on the surface of

myocardial cells by 60% to 70% in severe HF.

14–20 The normal ratio of β1

- to β2

-

receptors in the heart is ˜80:20. As a response to overstimulation, this balance is

shifted to a ratio of ˜70:30 in the failing myocardium by downregulation of β1

-

subtype receptors. This selective downregulation of β1

-receptors is accompanied by

“uncoupling of the β1

- and β2

-receptor activity,” whereby the number of β2

-receptors

is unchanged but the responsiveness of these receptors can be reduced by 30%.

15

Over time, this leaves the myocyte less responsive to adrenergic stimuli and further

decreases contractile function. β-Blockers cause the upregulation of the

downregulated β1

receptors and, the re-sensitization of the uncoupled β2

-receptors

protecting the heart from the deleterious effects of catecholamines.

21

Alterations in sympathetic adrenergic receptors are partially determined by genetic

phenotype. Among African American patients with HF, a disproportionately high

incidence of polymorphisms for variants of the β1

-receptor that are associated with

increased function is seen. Additionally, the combined presence of a variant of the

β1

-receptor (β1Arg389) and α2C-adrenergic receptor (α2cDel322–325) results in

adrenergic overstimulation and increases the risk of HF. These combined defects are

found less often in whites, perhaps partially explaining a higher incidence of HF in

African Americans. A better understanding of α- and β-receptor phenotypes may

someday lead to improved prevention and treatment of HF.

22

RENAL FUNCTION AND THE RENIN–ANGIOTENSIN–ALDOSTERONE

SYSTEM

The reduced CO in HF leads to the stimulation angiotensin II, which is a potent

vasoconstrictor. Angiotensin II is also a potent stimulator of the sympathetic nervous

system, which increases SVR. Renal vascular resistance is increased, and the

glomerular filtration rate (GFR) is decreased. As the GFR decreases, more sodium

and water are reabsorbed. A diminished effective circulating plasma volume and

angiotensin II also stimulate release of antidiuretic hormone (ADH) from the

pituitary, resulting in the retention of free water in the collecting ducts.

Figure 14-2 Adaptive mechanisms in systolic heart failure. +, beneficial results; –, negative (detrimental) effects;

ADH, antidiuretic hormone; CO, cardiac output; HR, heart rate; H2O, water; Na

+

, sodium; RAAS, renin–

angiotensin–aldosterone system; SNS, sympathetic nervous system; SV, stroke volume.

p. 265

p. 266

The kidney releases renin when renal perfusion pressure is decreased. Renin

converts angiotensinogen into angiotensin I. Angiotensin I is metabolized to

angiotensin II, under the influence of angiotensin-converting enzyme (ACE) (Fig. 14-

2). Angiotensin II has multiple effects favoring sodium and water retention. Its

vasoconstricting effect decreases GFR, and it stimulates the adrenal glands to secrete

aldosterone, which increases sodium reabsorption. Angiotensin II stimulates

increased synthesis and release of vasopressin, thereby increasing free water

retention and stimulation of thirst centers in the CNS. Finally, angiotensin II

stimulates NE release. The net result of decreased renal perfusion is detrimental.

Increased sodium and water retention increase preload, whereas angiotensin II–

induced vasoconstriction increases SVR and afterload.

A chronic excess of aldosterone causes fibrosis in the myocardium, kidneys, and

other organs.

23 Thus, aldosterone promotes remodeling of organs and fibrosis

independent of angiotensin II.

OTHER HORMONAL MEDIATORS

Endothelins

Several other regulatory hormones and cytokines have been identified as playing a

role in the pathogenesis and adaptation to HF. The first of these are the

endothelins.

24,25 Endothelin-1 (ET-1) is the most active. ET-1 is synthesized by

vascular and airway smooth muscle, cardiomyocytes, leukocytes, and macrophages.

Serum concentrations of ET-1 are elevated in HF, pulmonary HTN, MI, ischemia,

and shock, and cause vasoconstriction, potentiation of cardiac remodeling, and

decreased renal blood flow (RBF). Although the effects of ET-1 are detrimental in

HF, its pharmacology is complex and dependent on the relative balance of two

distinct G protein–coupled receptor subtypes referred to as ETA and ETB. As

illustrated in Table 14-2, ET-1 can elicit opposing effects from each receptor, with

the net effect being dependent on the relative density of the two receptors.

Possible future therapeutic agents could be specific inhibitors of one or more of

the enzymes to prevent activation of ET-1. Alternatively, selective inhibitors of ET A

receptors could shift responses toward the favorable aspects of ETB receptor

activation. Bosentan and tezosentan are nonselective dual ETA/ETB antagonists.

Bosentan has U.S. Food and Drug Administration (FDA) approval for the treatment

of pulmonary HTN and is being investigated for use in HF.

8,9

Natriuretic Peptides

Natriuretic peptides (NPs) are a family of peptides containing a common 17–amino

acid ring. A-type natriuretic peptide, previously referred to as atrial natriuretic

peptide or atrial natriuretic factor, is secreted by the atria in response to dilatation.

Similarly, B-type natriuretic peptide (BNP) is produced by the ventricular

myocardium in response to elevations of end-diastolic pressure and volume. Type-C

natriuretic peptide (CNP) is secreted by lung, kidney, and vascular endothelium in

response to shear stress. Collectively, the NPs are considered to be a favorable form

of neurohormonal activation. Among their positive attributes are antagonism of the

renin–angiotensin system, inhibition of sympathetic outflow, and ET-1 antagonism.

The net effect is peripheral and coronary vasodilation decreasing preload and

afterload. As their name implies, they also have diuretic or natriuretic properties,

with improved RBF and glomerular filtration resulting from afferent arteriolar

dilation and possibly efferent arteriolar constriction. Sodium reabsorption is blocked

in the collecting duct due to indirect aldosterone inhibition. NPs also inhibit

vasopressin secretion and block salt appetite and thirst centers in the CNS which

contributes to diuresis. CNP has minimal diuretic properties.

26

The BNP precursor is cleaved to produce the biologically active C-terminal

fragment (BNP) and an inactive N-terminal fragment (NT-proBNP). Plasma level

measurement of either BNP or NT-proBNP can be used as a biologic marker to

differentiate HF-induced dyspnea from other causes of respiratory distress.

27–29 BNP

levels less than 100 pg/mL usually indicate absence of HF, whereas levels greater

than 400 pg/mL are highly indicative of HF. However, the interpretation of BNP

levels between 100 and 400 pg/mL can be challenging because elevated levels are

also associated with renal failure, pulmonary embolism, pulmonary HTN, and

chronic hypoxia.

30 Higher concentrations correlate with the severity of HF. Clinical

resolution of symptoms is often accompanied by a decline in BNP concentration.

Natriuretic hormone analogs or inhibitors of their metabolism have been

investigated for drug therapy of HF. Nesiritide is a recombinantly produced human

BNP approved by the FDA for IV management of acute HF exacerbations in

hospitalized patients.

31,32 Downregulation of NP receptors, however, occurs during

chronic HF, reducing the protective benefit of their actions and possibly limiting their

usefulness as therapeutic entities.

Vasopressin Receptor Antagonists

Volume overload is associated with increased hospitalization. The potent

vasoconstrictor ADH (arginine vasopressin) is inappropriately elevated in HF. Early

studies with tolvaptan (selective vasopressin subtype V2 receptor antagonist)

demonstrated improvement in congestive symptoms of HF and overall hemodynamic

profile but no improvement in long-term outcome.

33

Table 14-2

Biologic Effects of Endothelin-1

Organ System ETA Receptor Effects ETB Receptor Effects Other Effects

Blood vessels Potent vasoconstrictor Vasodilation mediated

through nitric oxide and

prostacyclin release

Collagen deposition

Heart Hypertrophy and

remodeling

↑ HR

+/– inotropic effects

Lungs Bronchoconstriction

Kidney Afferent and efferent

vasoconstriction

Natriuresis and diuresis

Decrease in RBF and

GFR

Neuroendocrine Release of

catecholamines, renin,

aldosterone, and ANH

ANH, atrial natriuretic hormone; ETA, endothelin A; ETB, endothelin B; GFR, glomerular filtration rate; HR, heart

rate; RBF, renal blood flow; +/–, positive/negative.

From Ergul A. Endothelin-1 and endothelin receptor antagonists as potential cardiovascular therapeutic agents.

Pharmacotherapy. 2002;22:54.

p. 266

p. 267

The EVEREST trial studied the efficacy of vasopressin antagonism in HF.

34,35

Tolvaptan was evaluated in 4,133 patients with acute decompensated heart failure

(ADHF) with NYHA class III or IV and LVEF of less than 40%. All patients also

received standard therapy (ACE inhibitors [ACEIs], angiotensin receptor blockers

[ARBs], β-blockers, diuretics, nitrates, and hydralazine). The patients were

randomly assigned within 48 hours of hospitalization to 30 mg/day tolvaptan or

placebo. The trial was a composite of three distinct analyses. The primary end point

for the two identical short-term trials was to assess the change in global clinical

status and body weight at day 7 or the day of discharge. The primary outcome for the

long-term trial was all-cause mortality and cardiovascular (CV) death or HF

hospitalizations. The results of the short-term trial showed modest improvement in

the global clinical score compared with placebo. The main clinical benefit was

change in body weight. The long-term trial failed to show any significant difference

between the study drug and placebo in the primary end points. Common side effects

of tolvaptan were dry mouth and thirst. In a small number of patients, hyponatremia

was corrected. Because long-term mortality benefits are lacking and the cost of the

drug is high, the use of tolvaptan is restricted to patients with hypervolemic

hyponatremia associated with HF despite fluid restriction and diuretic use.

Calcium Sensitizers

Calcium sensitizers represent another class of drugs investigated for the treatment of

ADHF. They exert positive inotropic effects by stabilizing the calcium–troponin C

complex and facilitating actin–myosin cross-bridging without increasing myocardial

consumption of adenosine triphosphate.

36 Levosimendan, the prototype for this drug

class, has a dual mechanism of action to increase myocardial contractibility and

induce vasodilation. Unlike other inotropic agents, it does not affect the intracellular

calcium concentrations and, therefore, has a lower potential for proarrhythmia. The

safety and efficacy of levosimendan in ADHF has been evaluated in placebocontrolled trials and in comparative trials with dobutamine. In patients with

decompensated HF, levosimendan significantly reduced the incidence of worsening

HF and improved hemodynamic indices.

37–39

In addition, mortality was lower in the

levosimendan group. These trials, however, were not powered to show a difference

in mortality as an end point. A subsequent trial comparing levosimendan with

dobutamine in ADHF, designed to confirm the beneficial effects on morbidity and

mortality, did not reduce all-cause mortality, which contrasts with earlier studies.

40

The most common adverse effects associated with levosimendan are headache and

hypotension. Currently, levosimendan is approved in Europe for ADHF.

Inflammatory Cytokines, Interleukins, Tissue Necrosis Factor, Prostacyclin, and

Nitric Oxide

Vascular endothelial cells release pro-inflammatory cytokines, vasodilator and

vasoconstrictor substances, including interleukin cytokines (IL-1β, IL-2, IL-6), tumor

necrosis factor (TNF-α), prostacyclin, and nitric oxide (NO; also known as

endothelium-derived relaxing factor).

41–43 The role of these mediators in the

pathogenesis of HF is unclear. Recent studies have shown that patients with HF have

elevated levels of the pro-inflammatory cytokines IL-1β, IL-6, and TNF-α that

correlate with the severity of disease.

43–45

Initial enthusiasm for use of the TNF-α

receptor antagonist etanercept as a treatment for HF has been abandoned after

disappointing results in trials.

46 At least 47 adverse event reports were made to the

FDA describing new-onset HF or exacerbation of existing HF with etanercept and

infliximab in patients being treated for either Crohn’s disease or rheumatoid

arthritis.

47

Other investigators have tried using either NO or prostacyclin (epoprostenol) as

vasodilators with mixed success.

48–50 There was a trend toward increased death rates

with prostacyclin despite improved hemodynamic status during the Flolan

International Randomized Survival Trial (FIRST).

50

CARDIAC REMODELING

Progression of HF results in cardiac remodeling, characterized by changes in the

shape and mass of the ventricles.

51 The three primary manifestations of cardiac

remodeling are chamber dilatation, LV myocardial hypertrophy, and a resulting

spherical shape of the LV (Fig. 14-3). Cardiac remodeling, which starts before the

appearance of clinical symptoms, contributes to the progression of the disease.

Cardiac Dilatation

Cardiac dilatation results from hypervolemia. End-diastolic volume increases,

myocardial fibers are stretched, and the ventricle(s) become dilated. In the healthy

heart, the end-diastolic volume is 110 to 120 mL. With an EF of 60%, the SV would

be 70 mL, leaving an end-systolic residual volume in the ventricle of 40 to 50 mL.

With HFrEF, the end-diastolic volume increases resulting in an enlarged heart.

Cardiac dilatation is less evident in HFpEF because normal contractility is

maintained and the stiffened LV is resistant to filling and less likely to dilate (Fig.

14-3).

Frank–Starling Curve

The Frank–Starling curve (Fig. 14-4) demonstrates a curvilinear relationship

between LV myocardial muscle fiber “stretch” (wall tension) and myocardial work.

As stretch increases, the volume of blood ejected (SV) with each contraction

increases. In systolic HF, the work capacity for any degree of stretch is diminished.

A simple analogy is drawn using a balloon. The greater amount of air blown into a

balloon, the more it stretches and, if released, the farther it flies around a room. As

the balloon gets old, it loses its elasticity and thus has less recoil. Similarly,

dilatation of the ventricles initially may serve as an effective compensating

mechanism in systolic failure, but becomes inadequate as the elastic limits of the

muscle fibers are reached. HR also increases to maintain CO when SV is low. The

disadvantage of cardiac dilatation is increased myocardial oxygen demand.

Theoretically, as cardiac dilatation progresses beyond a certain point, CO could

decrease (as visualized on the descending limb of the Starling curve), but this is

rarely observed clinically.

Cardiac Hypertrophy

Cardiac hypertrophy represents an absolute increase in myocardial muscle mass and

muscle wall thickness (Fig. 14-3). This is analogous to increased skeletal muscle

mass in response to weightlifting. Cardiac hypertrophy should not be confused with

cardiac dilatation.

FUNCTIONAL LIMITATION CLASSIFICATION AND STAGES OF HEART

FAILURE

New York Heart Association Classification

The NYHA classification scheme identifies four categories of functional disability

associated with HF. Patients in class I are well compensated with no physical

limitations and lack symptoms with ordinary physical activity. In class II, ordinary

physical activity results in mild symptoms and imparts slight limitations on exercise

tolerance. Patients in class III are comfortable only at rest; even less than ordinary

physical activity leads to symptoms. In class IV, symptoms of HF are present at rest

and no physical activity can be undertaken without symptoms. Determination of class

is subjective and will vary among observers.

p. 267

p. 268

Figure 14-3 Cardiac remodeling. Dilated cardiomyopathy (DCM) results in thinning of the left ventricular walls

and a decrease in systolic function; in hypertrophic cardiomyopathy (HCM), there is a marked thickening of the left

ventricular walls leading to diastolic or systolic failure; and in restrictive cardiomyopathy (RCM), the left ventricular

walls may be normal, hypertrophic, or slightly dilated, resulting in a decrease in diastolic compliance. Ao, aorta; LA,

left articular; LV, left ventricular. (Adapted with permission from Topol EJ et al, eds. Textbook of Cardiovascular

Medicine. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006.)

A shortcoming of the NYHA classification scheme is that it does not include

asymptomatic individuals who are at high risk for developing HF and may benefit

from preemptive lifestyle changes and drug therapy. The 2001 ACC/AHA guidelines

introduced a new staging system that can be used in conjunction with the NYHA

classifications.

4 Patients in stage A have HTN, CAD, diabetes mellitus, or other

conditions that, if left untreated, can result in the development of overt HF. HF

symptoms or identifiable abnormalities of the myocardium or heart valves are absent

in stage A. Patients in stage B remain asymptomatic but have structural defects within

the heart (LV hypertrophy, dilatation, low EF, or valvular disease). Patients in stage

C exhibit varying degrees of HF along with structural changes in the heart. Stage D in

the ACA/AHA scheme roughly correlates with NYHA class IV. Patients in this latter

category are frequently hospitalized, and are considered to have end-stage disease.

Figure 14-1 summarizes these two classification schemes and how they overlap. The

most recent guidelines

1 expand the definition of HF and add two subgroups to the

HFpEF category: those with borderline EF (LVEF 41%–49%), and those with

improved EF (patients with previously reduced EF, but now with an LVEF >40%).

Figure 14-4 Representation of Frank–Starling ventricular function curve.

Overview of Treatment Principles

The ACC/AHA guidelines

1,5,6 updated their previous recommendations and expanded

the role of both aldosterone antagonists and cardiac-resynchronization therapy (CRT)

in patients with milder symptoms of HF. A clinical algorithm from the ACC/AHA

guidelines is found in Figure 14-5.

1 The ACC/AHA Task Force recommends that

most patients with HFrEF should routinely receive GDMT including an ACEI or an

ARB, a β-adrenergic blocker, and an aldosterone receptor antagonist. Diuretics are

recommended for patients with congestion. The combination of hydralazine and

nitrate should be considered in in black patients with symptoms despite receiving

other GDMT therapy or in patients unable to tolerate and ACEI or ARB. Digoxin is

potentially beneficial in symptomatic patients with HFrEF already receiving optimal

medical therapy to decrease HF hospitalizations.

The goals of therapy for HF are to abolish disabling symptoms, avoid

complications such as arrhythmias, improve the quality of life, and prolong survival.

Short of a heart transplant, none of the treatment measures are curative.

Nonspecific medical management of HF includes addressing CV risk factors,

correcting underlying disease states (HTN, ischemic heart disease, arrhythmias, lipid

disorders, anemia, or hyperthyroidism), performing moderate physical activity as

tolerated, undergoing immunization with influenza and pneumococcal vaccines, and

discontinuing possible drug-induced causes. The role of NPs, endothelin inhibitors,

vasopressin receptor antagonists, and calcium sensitizers continues to be

investigated. Non-digitalis inotropic agents and TNF-α inhibitors, although

theoretically valuable, have yielded disappointing results and significant

complications, including arrhythmias and increased mortality rates.

p. 268

p. 269

Figure 14-5 Stages in the development of heart failure and recommended therapy by stage. ACEI, angiotensinconverting enzyme inhibitors; ARB, angiotensin receptor blockers; BB, β-blockers; ARNI, angiotensin II receptor

blocker neprilysin inhibitor; CAD, coronary artery disease; CrCl, creatinine clearance; DM, diabetes mellitus; EF,

ejection fraction; HLD, hyperlipidemia; HTN, hypertension; ICD, implantable cardioverter-defibrillator; LVH, left

ventricular hypertrophy.

αNYHA II or III who tolerate ACEI/ARB with adequate BP and no hx of angioedema – replace with ARNI to

further reduce morbidity and mortality, must wait at least 36 hours of the last dose of ACEI

βNYHA II-IV unless est. CrCl < 30 mL/min and serum potassium > 5.0 mEq/L

cNYHA III or IV in black patients

dNYHA II or III, LVEF ≤35%, maximally tolerated dose of beta blocker, sinus rhythm, heart rate 70 bpm or

greater

Treatment of HFpEF is less well defined.

12,52–54 A sodium-restricted diet and

diuretics are indicated for symptomatic relief of SOB or edema. Because these

patients typically present with comorbid conditions (atrial fibrillation [AF], HTN,

diabetes mellitus, and CAD), treatment of comorbidities with available therapies

should aim to decrease CV events and improve survival.

PHYSICAL ACTIVITY

Patients should be encouraged to exercise to maintain physical conditioning.

Treatment goals include prolonging life and improving quality of life. The results of

The Effects of Exercise Training on Health Status in Patients with Chronic Heart

Failure (HF-ACTION)

55

trial showed that a highly structured exercise program in HF

patients did not reduce all-cause mortality or all-cause hospitalization when

compared with patients who were getting the usual care in which exercise was

simply encouraged. However, according to the HF-ACTION substudy, the structured

exercise training program improved the overall Kansas City Cardiomyopathy

Questionnaire (KCCQ) score (a test that includes questions on physical limitations,

symptoms, quality of life, and social limitations). The improvement occurred early on

and was sustained for 3 years. The ACC/AHA HF guidelines

1,5,6

recommend exercise

training as safe and effective for patients with HF who are able to participate in

symptom control.

1,5,6 However, during acute exacerbations, bed rest and restricted

physical activity decrease the metabolic demands, and minimize gravitational forces

contributing to edema. Renal perfusion is increased in the prone position, resulting in

diuresis and mobilization of fluid.

SODIUM-RESTRICTED DIET

Dietary indiscretion (high salt intake) is often cited as the cause of HF exacerbations

and hospital admissions. Several observational studies have demonstrated

associations between lower dietary sodium intake and reduced hospitalizations and

mortality.

56,57

In contrast, several randomized trials imply that dietary sodium

restriction in patients with CV diseases may be deleterious. One of the largest metaanalysis in patients with HFrEF showed that compared with control groups (2,800

mg/day), the sodium-restricted groups (1,800 mg/day) had increased morbidity and

mortality.

58 The trials included in this analysis had several limitations. High doses of

diuretics were used in conjunction with strict fluid restriction, which could have

resulted in intravascular volume depletion and adverse outcomes. Also, many

patients were not on optimal doses of GDMT. There is limited evidence to support

sodium restriction in the inpatient setting. In one trial, sodium restriction greater than

the standard 2,000 mg/day in hospitalized HFrEF patients did not affect clinical

stability or decrease length of stay.

59 For these reasons, further randomized trials are

needed to elucidate the impact of sodium restriction on outcomes in HF patients.

Because high sodium intake leads to HTN, LV hypertrophy, and CV disease, the

ACC/AHA HF guidelines

1,5,6

recommend 1.5 g/daily sodium restriction for patients

with stage A and B HF. In patients with advanced HF (stages C and D), <3 g/daily is

recommended due to lack of sufficient data to support any specific degree of

restriction.

p. 269

p. 270

Table 14-3

Loop Diuretic Dosing

1

Furosemide Bumetanide Torsemide

IV loading doses 40 mg 1 mg 20 mg

Maximum total daily dose 600 mg 10 mg 200 mg

Ceiling dose

Normal renal function 80–160 mg (PO/IV) 1–2 mg (PO/IV) 20–40 mg (PO/IV)

Clcr

: 20–50 mL/minute 160 mg (PO/IV) 2 mg (PO/IV) 40 mg (PO/IV)

ClCr

: <20 mL/minute 200 mg (IV), 400 mg (PO) 8–10 mg (PO/IV) 100 mg (PO/IV)

Bioavailability 10%–100% 80%–90% 80%–100%

Duration of action 6–8 hours 4–6 hours 12–16 hours

ClCr

, creatinine clearance; IV, intravenous; PO, oral.

Even though adherence to dietary sodium restriction is considered “a cornerstone

of HF disease management,” there are many challenges in implementing dietary

sodium restriction. Barriers such as lack of knowledge, interference with

socialization, limited access to appropriate food selections, and increased taste

preference for salty foods all contribute to lack of adherence to low sodium diet.

60,61

Although less than 1 g of sodium chloride (NaCl) is required to meet physiologic

needs, the average U.S. diet contains 10 g. Dietary sodium can be reduced to 2 to 4 g

of NaCl by eliminating cooking salt. This diet is more palatable and leads to better

adherence than a severely salt-restricted diet.

DIURETICS

Only those points salient to the treatment of HF are included in this chapter. Diuretic

use is discussed in Chapter 9, Essential Hypertension and Chapter 27, Fluid and

Electrolyte Disorders.

Diuretics are indicated in HF patients with congestion (pulmonary and/or

peripheral edema). They produce rapid symptomatic relief. Because activation of the

RAAS and sympathetic nervous system contributes to the progression of HF,

diuretics should be combined with an ACEI and a β-blocker unless contraindications

exist.

1

Initially, the goal of diuretic therapy is symptomatic relief of HF by decreasing

excess volume without causing intravascular volume depletion. Once excess volume

is removed, therapy is aimed at maintaining sodium balance and preventing

accumulation of new fluid, while avoiding dehydration. The rate at which volume can

be removed is limited by its rate of mobilization from the interstitial to the

intravascular fluid compartment. If diuresis is too vigorous, intravascular volume

depletion, hypotension, and a paradoxical decrease in CO may result. Diuretic doses

are titrated so that urine output increases and weight decreases, generally by 0.5 to

1.0 kg daily.

1

The effectiveness of diuretics depends on the amount of sodium delivered to their

site of action and the patient’s renal function.

62,63 Proximal tubular reabsorption of

sodium is increased in patients with severe HF when RBF is compromised,

rendering thiazide and potassium-sparing diuretics (which act primarily on the distal

tubule) minimally effective. Thiazides are believed to lose their effectiveness when

creatinine clearance decreases to less than 30 mL/minute. Metolazone is an exception

in that its activity may be preserved in these patients. The loop diuretics (furosemide,

bumetanide, and torsemide) are more potent than thiazides, and retain their

effectiveness in renal insufficiency. Thus, in most patients with HF, loop diuretics

are preferred. In addition to having activity in the ascending limb of the loop of

Henle, furosemide has vasodilating properties that decrease renal vascular

resistance. The usual recommended doses for the loop diuretics are found in Table

14-3.

62

The effectiveness of diuretics depends on active secretion of the drug in the

proximal tubule. Slow absorption (even if bioavailability is high) or protein binding

impairs tubular delivery and compromises diuretic response. Once the drug is in the

tubule and the threshold for diuresis is met, further drug delivery produces no greater

diuresis. Increasing single doses beyond the ceiling dose produces no additional

diuretic response; however, improved diuresis may be obtained by giving the drug

more frequently.

A combination of diuretics (a loop diuretic and thiazide) is used in patients who

are refractory to high-dose loop diuretics.

62,63 Despite their effects on reducing HF

symptoms, loop diuretics do not counteract the underlying cause of HF or modify

mortality rates.

ALDOSTERONE ANTAGONISTS

The aldosterone antagonists eplerenone and spironolactone exert a mild diuretic

effect by competitive binding to the aldosterone receptor site in the distal convoluted

renal tubules. The Randomized Aldactone Evaluation Study (RALES) investigators

found that spironolactone reduced both morbidity and mortality in patients in NYHA

class III and IV.

64 The authors speculated that the beneficial effect of spironolactone

was related to a reduction in aldosterone-induced vascular damage and myocardial

or vascular fibrosis rather than its diuretic effect. Similarly, reduced mortality was

observed in patients with LV dysfunction after a recent MI who were treated with

eplerenone.

65 The ACC/AHA HF guidelines

1,5,6

recommend the addition of an

aldosterone antagonist in all patients with HFrEF who are already on ACEIs (or

ARB) and β-blockers irrespective of the severity of symptoms and in patients

immediately after MI who have LV dysfunction or diabetes.

65 On initiating

aldosterone receptor antagonists, GFR should be>30 mL/minute/1.73 m2 and

potassium levels <5 mEq/dL to avoid the risk of hyperkalemia or renal insufficiency.

ANGIOTENSIN-CONVERTING ENZYME INHIBITORS AND

ANGIOTENSIN RECEPTOR BLOCKERS

Drugs with vasodilating properties are a primary treatment for HF. Arterial dilation

provides symptomatic relief of HF by decreasing arterial impedance (afterload).

Venous dilation decreases LV congestion (preload). The combination of these two

properties provides additive benefits to alleviate the symptoms of HF and increase

exercise tolerance. The first vasodilator drugs to be studied were hydralazine (an

arterial dilator) and nitrates (predominately venous dilators). By combining these

two drugs, significant reductions in HF symptoms can be achieved along with a

reduction in mortality rates. With the advent of ACEIs, the use of hydralazine and

nitrates has been relegated to a secondary role.

ACEIs possess both afterload- and preload-reducing properties and volumereducing potential. They produce similar hemodynamic effects to the hydralazine–

nitrate combination as a single agent, favorably modify cardiac remodeling, and have

a more tolerable

p. 270

p. 271

side effect profile. These advantages led to the recommendations that ACEIs are

the drugs of choice for initial therapy, even in patients with relatively mild LV

systolic dysfunction.

1

The ACC/AHA guidelines

1,5,6 state that ACEIs should be prescribed to all patients

with HFrEF unless they have a contradiction to their use or are unable to tolerate

these drugs. In general, ACEIs are used with β-blockers. ACEIs should be initiated at

low doses, and titrated to target doses if well tolerated. Fluid retention can blunt the

therapeutic effects, and volume depletion can potentiate the adverse effects of ACEIs.

Clinicians should attempt to use doses that have been shown to reduce CV events in

clinical trials, but they should not delay the initiation of β-blockers in patients

because of a failure to reach target ACEI doses.

1

The pharmacologic actions of all the ACEIs are essentially identical, but some of

them have not been studied or received FDA approval for use in HF (Table14-4).

Their value in HFpEF still is unclear. A related class of drugs is the ARBs.

66,67

ARBs offer theoretic advantages compared with ACEIs by being more specific for

angiotensin II blockade (preferentially bind to AT1

receptors) and having a lower

risk of drug-induced cough.

ARBs can be used as alternatives to ACEIs in patients who are intolerant

(angioedema or cough). Triple combination of an ACEI, ARB, and an aldosterone

receptor antagonist is potentially harmful for patients and therefore is listed as a

class III (avoid) recommendation in the ACC/AHA guidelines.

1,5,6 Currently, only

candesartan and valsartan are FDA-approved for the treatment of HF.

β-ADRENERGIC BLOCKING AGENTS

Until the mid-1990s, β-blockers were contraindicated in patients with HFrEF. This

was based on the belief that sympathomimetic agonists and other positive inotropic

drugs were the logical choices to counteract systolic failure and that negative

inotropic drugs would exacerbate HF. A better understanding of the pathophysiology

of HF led to a rethinking of this logic.

15–20,22

In combination with ACEIs, β-blockers

are considered first-line agents in patients with HFrEF. The ACC/AHA guidelines

state that β-blockers should be prescribed to all patients with HFrEF unless they

have a contraindication to their use or are unable to tolerate the treatment. Intolerance

or resistance to other HF therapies should not preclude or delay the initiation of βblocker use in patients with HFrEF.

1 Although some patients can have a temporary

worsening of symptoms, continued use results in improved quality of life, fewer

hospitalizations, and most importantly, a reduction in mortality by approximately

34% when added to other HF therapies. Extended-release metoprolol succinate,

carvedilol, and bisoprolol are FDA approved for use in HFrEF. Metoprolol and

bisoprolol are both partially selective β1

-blockers, and carvedilol is a mixed α1

- and

nonselective β-blocking agent.

DIGITALIS GLYCOSIDES (DIGOXIN)

Digoxin has several pharmacologic actions on the heart. It binds to and inhibits

sodium-potassium (Na

+

/K+

) adenosine triphosphatase (ATPase) in cardiac cells,

decreasing outward transport of sodium and increasing intracellular concentrations of

calcium within the cells. Calcium binding to the sarcoplasmic reticulum causes an

increase in the contractile state of the heart.

At one time, the primary benefit of digoxin in HFrEF was assumed to be an

increase in the force of contraction (positive inotropic effect). We now know that

digoxin has beneficial neurohumoral and autonomic effects caused by reducing

sympathetic tone and stimulating parasympathetic responses at serum concentrations

below those associated with positive inotropism.

68,69

Inhibition of Na

+

/K+ ATPase in

vagal afferent fibers sensitizes cardiac baroreceptors, resulting in reduced

sympathetic outflow from the CNS. Similarly, inhibition of Na

+

/K+ ATPase in renal

cells reduces renal tubular reabsorption of sodium and indirectly suppresses renin

secretion. This has led to the suggestion that the positive benefits of digoxin can be

obtained by using small doses.

Digoxin decreases the conduction velocity and prolongs the refractory period of

the atrioventricular (AV) node. This AV node–blocking effect prolongs the PR

interval and is the basis for use of digoxin in slowing the ventricular response rate in

patients with AF and other supraventricular arrhythmias (see Chapter 15, Cardiac

Arrhythmias).

Several studies have confirmed a clinical benefit for digoxin in reducing HF

symptoms, independent of rhythm status, but there are no data that demonstrate a

beneficial effect on survival. Digoxin is used for symptom management in patients

optimally treated with ACEIs, β-blockers, and aldosterone antagonists. In

symptomatic patients in stage C or stage D, digoxin can reduce HF-related

hospitalizations.

1 Digoxin can also be considered in patients with HF who also have

AF, although β-blockers are more effective than digoxin in controlling the ventricular

response, especially during exercise.

OTHER VASODILATING DRUGS: HYDRALAZINE AND NITRATES

Although ACEIs are drugs of choice, the first vasodilators evaluated in HFrEF were

hydralazine and nitrates. Hydralazine provides symptomatic relief of HF by

decreasing afterload. Nitrates have venous dilating properties that decrease preload.

Used in combination, these two agents have additive benefits in alleviating the

symptoms of HF and increasing exercise tolerance. Importantly, the hydralazine–

isosorbide dinitrate combination was the first treatment regimen to show improved

survival in severe HF compared with placebo

70

(while patients continued their

diuretic or digitalis therapy). The African American Heart Failure Trial (AHeFT)

71

showed that the addition of hydralazine combined with isosorbide dinitrate to

standard HF therapy with an ACEI or a β-blocker improved survival and reduced HF

hospitalizations. Based on the results of AHeFT, the FDA approved the combination

product of hydralazine and isosorbide dinitrate (BiDil) for the treatment of HFrEF as

an adjunct to standard HF therapy in African American patients. The combination of

hydralazine and nitrate is reasonable in patients with current or prior HF symptoms

and reduced LVEF who cannot tolerate ACEIs or ARBs, and is also recommended

for patients self-described as African American with moderate to severe symptoms

on optimal therapy with ACEIs, β-blockers, and diuretics.

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