What is the most likely diagnosis?A 66-year-old woman presents to your office complaining of shortness of breath and bilateral leg edema that have been worsening for 3 months. She emphatically tells you, “I get out of breath when I do housework and I can’t even walk to the corner.” She has also noticed difficulty sleeping secondary to a dry cough that wakes her up at night and further exacerbation of her shortness of breath while lying flat. This has forced her to use three pillows for a good night’s sleep. She denies any chest pain, wheezing, or febrile illness. She has no past illnesses and takes no medications. She’s never smoked and drinks socially. On examination, her blood pressure is 187/90 mm Hg, her pulse is 97 beats/min, her respiratory rate is 16 breaths/min, her temperature is 98°F (36.6°C), and her oxygen saturation is 93% on room air by pulse oximetry. She has a pronounced jugular vein. Cardiac examination reveals a pansystolic murmur. Examination of her lung bases produces dullness bilaterally. You find 2+ pitting edema of both ankles. An electrocardiogram (ECG) shows a normal sinus rhythm and a chest x-ray demonstrates mild cardiomegaly with bilateral pleural effusions. You decide she needs further workup, so you call the hospital where you have admitting privileges and arrange for a telemetry bed.
What is the most likely diagnosis?
What is the next diagnostic step?
What is the initial step in therapy?
Summary: A 66-year-old woman presents to your office with worsening shortness of breath, bilateral leg edema, and three-pillow orthopnea. She is not known to be hypertensive, but her blood pressure (BP) is 187/90 mm Hg and she is only saturating 93% on room air. Her examination reveals jugular venous distension (JVD), a cardiac murmur, and decreased breath sounds at both lung bases. On a chest x-ray, you find bilateral pleural effusions and decide to admit her for further workup and management.
• Most likely diagnosis: New-onset congestive heart failure (CHF)
• Next diagnostic step: Serial cardiac enzymes and ECGs; blood work to include a CBC, electrolytes, and renal function; echocardiogram
• Initial therapy: Telemetry monitoring, IV diuretics, and oxygen
1. Know how to clinically recognize congestive heart failure (CHF).
2. Understand the classification of CHF.
3. Understand the mechanism of action of the drugs used in the treatment of acute and chronic CHF.
4. Understand the underlying pathophysiology that occurs in CHF and the rationale for treatment options.
5. Be familiar with the outpatient management of CHF and the importance of patient education.
This 66-year-old woman presented with congestive heart failure. Her most immediate problem is oxygenation and volume overload on her weakened heart. The first priority is optimizing oxygen exchange by administering oxygen via nasal cannula, dilating pulmonary vasculature, and decreasing cardiac preload and afterload. Most cases of CHF are caused by either coronary artery disease or hypertension, so it is imperative to admit these patients for serial cardiac enzymes and further assessment of heart function. The overloading of fluid in the lungs is a common cause of anxiety and distress in patients with acute CHF because of the continuous struggle to oxygenate adequately. This anxiety activates sympathetic pathways and mounts catecholamine-induced responses, which produce further worsening of acute heart failure by causing tachycardia and increasing peripheral vascular resistance, leading to greater stress on the heart and worsening of symptoms. These triggers can, in part, be suppressed by the use of an agent such as morphine sulfate, which acts as both an anxiolytic and a vasodilator. Furosemide (Lasix) is the diuretic of choice, not only for its diuretic effect but also for its immediate vasodilatory action on bronchial vasculature. Admitting these patients to the hospital allows for closer maintenance of homeostasis in their fluid balances and evaluation of any underlying condition that may have precipitated the CHF. Other medications, including angiotensin-converting enzyme (ACE) inhibitors and β-blockers, help to control heart failure symptoms by decreasing preload and afterload, and reducing cardiac remodeling.
CONGESTIVE HEART FAILURE: Imbalance in pump function where the heart fails to maintain the circulation of blood adequately
FRAMINGHAM HEART STUDY: Large, prospective cohort study of the epidemiologic factors associated with cardiovascular diseases
CHF is divided into two main categories: systolic and diastolic dysfunction. Systolic dysfunction exists when there is a dilated left ventricle with impaired contractility. Diastolic dysfunction occurs in a normal or intact left ventricle that has an impaired ability to relax, fill, and eject blood. Table 27–1 lists the findings frequently associated with CHF.
Table 27–1 • ETIOLOGIES OF HEART FAILURE
Dyspnea on exertion is the most sensitive symptom for the diagnosis of CHF, but its specificity is much lower. Other symptoms, which are common but less sensitive for the diagnosis, include dyspnea at rest, anxiety, orthopnea, paroxysmal nocturnal dyspnea, and cough productive of pink, frothy sputum. Nonspecific symptoms sometimes reported are weakness, lightheadedness, abdominal pain, malaise, wheezing, and nausea. Patients may have a medical history of hypertension, coronary artery disease, or other heart diseases (eg, cardiomyopathy, valvular disease). Histories of cigarette smoking and alcohol abuse may also be found.
The symptoms and signs that occur are unique and characteristic of the alterations to the normal physiologic function. Symptoms of right-sided heart failure include venous congestion, nausea/vomiting, distension/bloating, constipation, abdominal pain, and decreased appetite. Common signs of right-sided heart failure are fluid retention, weight gain, peripheral edema, JVD, hepatojugular reflux, hepatic ascites, and splenomegaly.
Left-sided heart failure manifests with pulmonary congestion, resulting in the symptoms of dyspnea on exertion, paroxysmal nocturnal dyspnea, orthopnea, wheezing, tachypnea, and cough. The signs of pulmonary congestion are bilateral pulmonary rales, S3 gallop rhythm, Cheyne-Stokes respiration, pleural effusion, and pulmonary edema. Pulmonary edema is often the first manifestation of congestive heart failure, but it can also be caused by a variety of noncardiac conditions (Table 27–2).
Table 27–2 • NONCARDIAC CAUSES OF PULMONARY EDEMA
Signs common to both left- and right-heart failure are tachycardia, cardiomegaly, cyanosis, oliguria, nocturia, and peripheral edema. Symptoms common to both include weakness, fatigue, confusion (delirium), decreased mental status, insomnia, decreased exercise tolerance, headache, stupor, coma, paroxysmal nocturnal dyspnea, and declining functional status.
After the Framingham Heart Study was reviewed, criteria were devised to help diagnose CHF by both signs and symptoms. Two major criteria or one major and two minor criteria can lead to a presumptive diagnosis of CHF. The major signs are paroxysmal nocturnal dyspnea, JVD, rales, cardiomegaly, pulmonary edema, S3 gallop, central venous pressure greater than 16 cm H2O, circulation time of 25 seconds, hepatojugular reflex, and weight loss of 4.5 kg over 5 days of treatment. Minor criteria include bilateral ankle edema, nocturnal cough, dyspnea on exertion, hepatomegaly, pleural effusions, decreased vital capacity by one-third of maximum, and tachycardia.
Of the general population between the ages of 50 and 59 years, 1% to 2% will have CHF, but that number increases to 6% to 10% in persons older than age 65. Approximately 30% to 40% of patients with CHF are hospitalized every year; it is the leading diagnosis-related group (DRG) among hospitalized patients older than age 65 years. The 5-year mortality rate remained essentially unchanged from 1971 to 1991, at 60% in men and 45% in women. Data from the Framingham Heart Study shows a median survival of 3.2 years for men and 5.4 years for women with CHF. The most common cause of death is progressive heart failure, but sudden death may account for up to 45% of all deaths. African Americans are 1.5 times more likely to die of CHF than are white people. Nevertheless, African-American patients appear to have similar or lower inhospital mortality rates than white patients. The prevalence is greater in men than in women for patients ages 40 to 75 years; after the age of 75, however, there is no difference.
After an initial assessment of the “ABCs”—airway, breathing, and circulation—patients presenting with dyspnea suggestive of heart failure should be evaluated with a history, physical examination, and focused testing. The testing should be designed to confirm CHF (or lead to an alternate diagnosis), identify a cause, and assess the severity of the disease. These initial tests should include blood tests, radiographic studies, electrocardiography, and echocardiography.
Initial blood tests should generally include a complete blood count (CBC), serum electrolytes, renal function tests, hepatic function tests, and cardiac enzymes. A high white blood cell count can help to identify the presence of an underlying infection, a common triggering event of CHF. Anemia is another common trigger of CHF. In an anemic patient, the oxygen-carrying ability of the blood is reduced. The cardiac output must increase to compensate for this. If the anemia is mild, or if the heart is normal, this compensation may occur without producing symptoms; if the anemia is severe or if there is underlying cardiac abnormality (from previous ischemia, hypertension, valvular abnormality, etc), heart failure may occur.
Electrolyte abnormalities are common in the presence of CHF. Neurohumoral responses to a failing heart result in water and sodium retention and potassium excretion. Severe heart failure can result in a dilutional hyponatremia. Medications used by patients with chronic heart disease (diuretics, ACE inhibitors, others) also can lead to electrolyte abnormalities. Increased vascular congestion can lead to passive congestion of the liver, resulting in increases in serum transaminases. Severe CHF can lead to jaundice as a consequence of impaired hepatic function caused by congestion. Serial measurement of cardiac enzymes is necessary to evaluate for the presence of acute myocardial infarction as the inciting event.
One of the neurohumoral responses to the presence of a failing ventricle is release of brain natriuretic peptide (BNP). The “Breathing Not Properly” study, published in 2004, showed that elevated levels of BNP and its prohormone (pro-BNP) can be used to assist in the diagnosis of CHF as a cause of acute dyspnea. Elevated levels of BNP and pro-BNP are a sensitive and specific marker for the diagnosis of CHF. In a dyspneic patient, a level of BNP less than 100 pg/mL suggests that the symptoms are unlikely to be caused by CHF; a BNP level greater than 500 pg/mL is consistent with the diagnosis of CHF.
ECG findings in CHF are variable. An ECG is useful to evaluate for evidence of acute ischemia or arrhythmia as cause of the CHF and can also reveal the presence of ventricular hypertrophy, often seen in chronic hypertension. Chest x-ray can also show cardiomegaly and cardiac chamber enlargement. Typically, the cardiothoracic ratio is greater than 50%. One of the earliest chest x-ray findings in CHF is cephalization of the pulmonary vasculature. As the failure progresses, interstitial pulmonary edema can be seen as perihilar infiltrates, often in a butterfly pattern. Pleural effusions can also be found. Effusions are usually bilateral but, if unilateral, are more often seen on the right hemithorax than the left.
Echocardiography is the gold-standard diagnostic modality in the presence of CHF. It may help to identify regional or global wall motion abnormalities, cardiomyopathy, ventricular or septal hypertrophy, and cardiac ejection fraction. It also can find cardiac tamponade, pericardial constriction, and pulmonary embolus. Echocardiography also is useful in identifying valvular stenosis or regurgitation, either of which can lead to heart failure. These findings aid in the determination of whether the heart failure is a systolic or diastolic dysfunction, an important distinction in the decision of appropriate treatment.
CHF severity is characterized by the symptoms a patient has and the degree that the symptoms limit a patient’s lifestyle. There are several classification systems in use; two of the most widely used are the New York Heart Association (NYHA) and the American Heart Association (AHA) classifications. Table 27–3 summarizes these systems. The classification of CHF is important in determining the appropriate treatment and prognosis for the patient.
Table 27–3 • CLASSIFICATION OF SEVERITY OF CONGESTIVE HEART FAILURE
In all cases of acute CHF, the initial management imperative is the “ABCs”—airway, breathing, and circulation. Supplemental oxygen, initially 100% via non-rebreather face mask, should be administered. If necessary, ventilation can be assisted with continuous positive airway pressure (CPAP), bilevel positive airway pressure (BiPAP), or mechanical ventilation. Cardiac and continuous pulse oximetry monitors should be placed and IV access obtained.
When acute pulmonary edema caused by CHF is diagnosed, the next step in management is the administration of a loop diuretic. Furosemide is generally the treatment of choice, both for its potent diuretic effect and for its rapid bronchial vasculature vasodilation. Nitrates, particularly nitroglycerin when given IV, reduce myocardial oxygen demand by reducing preload and afterload. Nitroglycerin also can rapidly reduce blood pressure and is the treatment of choice in a patient who has CHF and whose blood pressure is elevated. It should be used with caution or avoided in a hypotensive patient. IV morphine sulphate can be an effective adjunct to therapy. Along with its analgesic and anxiolytic properties, morphine is a venodilator (primary effect) and arterial dilator, resulting in a reduction in preload and an increase in cardiac output.
Most patients who present to the emergency department (ED) with symptomatic CHF will require admission to a telemetry unit for treatment and monitoring. Discharge criteria from the ED includes the gradual onset of symptoms, rapid resolution of symptoms with treatment, oxygen saturation of >90% on room air, and exclusion of an acute coronary syndrome as the cause of the CHF.
Patient education is an important aspect of the care for all patients with CHF. All patients should be advised about the importance of dietary sodium and fluid restriction. A normal American diet contains 6 to 10 g sodium chloride a day; initial restriction in patients with CHF should be 2 to 4 g/d. Stricter restrictions may be necessary in those with more severe disease. Overweight and obese patients should be counseled on appropriate caloric restrictions and encouraged to exercise to reduce weight. The importance of strict management of blood pressure and modification of other cardiac risk factors should be emphasized as well.
ACE inhibitors should be considered first-line therapy in patients with CHF and reduced left ventricular function. ACE inhibitors reduce preload, afterload, improve cardiac output, and inhibit tissue renin-angiotensin systems. The result of this is an improvement in symptoms and a reduction in mortality. ACE inhibitors can also delay the development of symptomatic CHF in asymptomatic patients with a reduced cardiac ejection fraction. Angiotensin receptor blockers (ARBs) can be used in place of an ACE inhibitor in a patient who does not tolerate an ACE inhibitor because of side effects (eg, cough).
ACE inhibitors are contraindicated in pregnancy, hypotension, hyperkalemia, and bilateral renal artery stenosis, and should be used with caution in patients with renal insufficiency.
For many years, the teaching was to avoid the use of β-blockers in the setting of CHF. However, more recent data support the use of β-blockers for both systolic and diastolic heart failure. The administration of β-blockers, especially in high doses, in the setting of acute CHF, can worsen symptoms; consequently, initial doses should be low and titrated up over several weeks. β-Blockers can reduce the sympathetic tone and the cardiac muscle remodeling associated with chronic heart failure. β-Blockers reduce mortality in patients with an ejection fraction of less than 35% and are primarily indicated in patients with NYHA Class II or III heart failure, or in patients with coronary artery disease.
Diuretics should be used to reduce fluid overload in both the acute and chronic settings. Loop diuretics (furosemide, bumetanide, torsemide, ethacrynic acid) can be used in all stages of CHF and are useful in pulmonary edema and refractory heart failure. Thiazide diuretics (hydrochlorothiazide, chlorthalidone, others) are used in mild heart failure and may be used in combination with other diuretics in more severe CHF. Diuretic doses can be adjusted based on daily weight measurements by the patient.
The aldosterone antagonist spironolactone reduces mortality in advanced heart failure. It also functions as a diuretic and should be considered in NYHA Class III and IV heart failure. Patients on this medication must be closely monitored for the development of hyperkalemia, which can become profound and lead to arrhythmia.
Calcium channel blockers, in general, are contraindicated in systolic heart failure, because they increase mortality. The exception to this is the dihydropyri-dine calcium channel blocker amlodipine (Norvasc), which does not increase or decrease mortality. Nondihydropyridine calcium channel blockers (diltiazem, vera-pamil) are useful in heart failure caused by diastolic dysfunction, as they promote increased cardiac output by lowering heart rate, which allows for more ventricular filling time.
Approximately one-third of patients with NYHA Class III or IV heart failure and reduced ejection fraction have ECG evidence of abnormal ventricular conduction (ie, prolonged QRS duration). These patients can be helped by promoting synchronous contraction of both the right and left ventricles using a biventricular pacemaker. This process, also known as cardiac resynchronization therapy, has been shown to reduce mortality and hospitalization in patients with symptomatic CHF in spite of maximal medical therapy.
27.1 A 57-year-old man who has known New York Heart Association Class II Heart Failure presents to clinic after noting to become dyspneic with significant exertion. On physical examination, his BP is 140/86 mm Hg, pulse 86 beats/min, and respiratory rate 20 breaths/min. A 2/6 pansystolic murmur is best heard at the right sternal border. There is no JVD, but 1+ pretibial and pedal edema are noted. He currently takes an ACE inhibitor and aspirin. Which one of the following additional medications have been shown to improve longevity in this situation?
A. Warfarin (Coumadin)
B. Digitalis
C. β-Blocker
D. Nondihydropyridine calcium channel blocker
E. Amiodarone (Cordarone)
27.2 A 52-year-old man with a long-standing history of marginally controlled hypertension presents with gradually increasing shortness of breath and reduced exercise tolerance with pain in his calves that causes him to stop walking after one block. His medications include enalapril and metoprolol. His physical examination reveals a blood pressure of 140/90 mm Hg, a respiratory rate of 22 breaths/min, heart rate of 88 beats/min, bibasilar rales, and trace pitting edema. Posterial tibial and dorsalis pedis pulses are 1+. Which of the following diagnostic tests is most appropriate in the further evaluation of this patient?
A. Cardiac MRI
B. 12-lead ECG
C. Spiral CT
D. Two-dimensional echocardiography with Doppler
E. Posteroanterior and lateral chest radiographs
27.3 A 64-year-old man is noted to have congestive heart failure because of coronary artery disease. Over the past 2 days, he has developed progressive dyspnea and orthopnea. On examination, he is found to be in moderate respiratory distress, has JVD, and rales on pulmonary examination. He is diagnosed with pulmonary edema. Which of the following agents is most appropriate at this time?
A. Hydrochlorothiazide
B. Furosemide
C. Carvedilol
D. Spironolactone
E. Digitalis
27.4 A 70-year-old African-American man with New York Heart Association Class III heart failure sees you for follow-up. He has shortness of breath with minimal exertion. The patient is adherent to his medication regimen. His current medications include lisinopril 40 mg twice daily, carvedilol 25 mg twice daily, and furosemide 80 mg daily. His blood pressure is 100/60 mm Hg, and his pulse rate is 70 beats/min and regular. Physical examination findings include a few scattered bibasilar rales, an S3 gallop, and no peripheral edema. An ECG reveals a left bundle branch block and echocardiography reveals an ejection fraction of 25%. Which of the following is the best next step for this patient?
A. Increase the furosemide dosage to 80 mg twice daily.
B. Refer for coronary angiography.
C. Increase the lisinopril dosage to 80 mg twice daily.
D. Increase the carvedilol dosage to 50 mg twice daily.
E. Refer for cardiac resynchronization therapy.
27.1 C. β-Blockers are recommended to reduce mortality in symptomatic patients with heart failure. Because polypharmacy can reduce compliance, the role that digoxin plays in heart failure management is unclear. Calcium channel blockers should be used with caution in patients with heart failure because they can cause peripheral vasodilation, decreased heart rate, decreased cardiac contractility, and decreased cardiac conduction.
27.2 D. The most useful diagnostic tool for evaluating patients with heart failure is two-dimensional echocardiography with Doppler to assess left ventricular ejection fraction (LVEF), left ventricular size, ventricular compliance, wall thickness, and valve function. It should be performed during the initial evaluation. Chest radiography and 12-lead electrocardiography should be performed in all patients presenting with heart failure, but should not be used as the primary basis for determining which abnormalities are responsible for the heart failure.
27.3 B. Furosemide, a loop diuretic, is a first-line agent in CHF exacerbation with pulmonary edema. The other medications listed may be used in the management of CHF, but are not indicated in an acute exacerbation.
27.4 E. This patient is already receiving maximal medical therapy. The 2002 joint guidelines of the American College of Cardiology, the American Heart Association (AHA), and the North American Society of Pacing and Electrophysiology endorse the use of cardiac resynchronization therapy (CRT) in patients with medically refractory, symptomatic, NYHA Class III or IV disease with a QRS interval of at least 130 ms, a left ventricular end-diastolic diameter of at least 55 mm, and a left ventricular ejection fraction (LVEF) less than or equal to 30%. These guidelines were refined by an April 2005 AHA Science Advisory, which stated that optimal candidates for CRT have a dilated cardiomyopathy on an ischemic or nonischemic basis, an LVEF less than or equal to 35%, a QRS complex greater than or equal to 120 ms with sinus rhythm, and are NYHA functional Class III or IV despite maximal medical treatment for heart failure.
The initial hour in the management of a patient with either new-onset CHF or an acute exacerbation is crucial to their outcome.
Simple measures, such as decreasing cardiac preload by sitting the patient up with their legs on the ground and their arms by their side, maintaining an airway and giving oxygen, and giving sublingual nitroglycerin, can alleviate CHF immediately.
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