caso 1

Summary: A 72-year-old man complains of several weeks of worsening exertional

dyspnea. He has experienced angina-like chest pressure with strenuous exertion and
near-syncope while climbing a flight of stairs, and now he has symptoms of heart failure
(HF) such as orthopnea and paroxysmal nocturnal dyspnea. Heart failure is also
suggested by physical signs of volume overload (pedal edema, elevated jugular venous
pressure, and crackles suggesting pulmonary edema). The cause of his heart failure may
be aortic valvular stenosis, given the late systolic murmur radiating to his carotid, the
paradoxical splitting of his second heart sound, and the diminished carotid upstrokes.
􀀀 Most likely diagnosis: Congestive heart failure (CHF), possibly as a result of
aortic stenosis.
􀀀 Diagnostic test: Echocardiogram to assess the aortic valve area as well as the
left ventricular systolic function

Considerations
This is an elderly patient with symptoms and signs of aortic stenosis. The valvular
disorder has progressed from previous angina and presyncopal symptoms to heart
failure, reflecting worsening severity of the stenosis and worsening prognosis for
survival. This patient should undergo urgent evaluation of his aortic valve surface
area and coronary artery status to assess the need for valve replacement.

Table 4–1 • NYHA FUNCTIONAL CLASSIFICATION

Class I: No limitation during ordinary physical activity
Class II: Slight limitation of physical activity. Develops fatigue or dyspnea with moderate
exertion
Class III: Marked limitation of physical activity. Even light activity produces symptoms
Class IV: Symptoms at rest. Any activity causes worsening
Table 4–2 • SELECTED CAUSES OF CONGESTIVE HEART FAILURE
Myocardial injury, or other chemotherapeutic agents
• Adriamycin
• Alcohol use
• Cocaine
• Ischemic cardiomyopathy (atherosclerotic coronary artery disease)
• Rheumatic fever
• Viral myocarditis
Chronic pressure overload
• Aortic stenosis
• Hypertension
Chronic volume overload
• Mitral regurgitation
Infiltrative diseases
• Amyloidosis
• Hemochromatosis
Chronic tachyarrhythmia or bradyarrhythmia

The three major treatment goals for patients with chronic heart failure are relief
of symptoms, preventing disease progression, and a reduction in mortality risk. The
heart failure symptoms, which are mainly caused by low cardiac output and fluid
overload, usually are relieved with dietary sodium restriction and loop diuretics.
Because heart failure has such a substantial mortality, however, measures in
an attempt to halt or reverse disease progression are necessary. Reversible causes
should be aggressively sought and treated. Use of angiotensin-converting enzyme
(ACE) inhibitors or angiotensin receptor blockers (ARBs) and some beta-blockers,
such as carvedilol (CAR), metoprolol, or bisoprolol, have been shown to reduce mortality
in patients with impaired systolic function and moderate to severe symptoms.
In patients who cannot tolerate ACE inhibition (or in black patients in whom ACE
inhibitors appear to confer less benefit), the use of hydralazine with nitrates has been
shown to decrease mortality. Aldosterone antagonists such as spironolactone may be
added to patients with NYHA class III or IV heart failure with persistent symptoms,
but patients should be monitored for hyperkalemia. Digoxin can be added to these
regimens for persistent symptoms, but it provides no survival benefit.

Some devices may also be useful in reducing symptoms and mortality in patients
with heart failure. Patients with depressed ejection fraction and advanced symptoms
often have a widened QRS >120 ms, indicating dyssynchronous ventricular
contraction. Placement of a biventricular pacemaker, called cardiac resynchronization
therapy (CRT), to stimulate both ventricles to contract simultaneously can
improve symptoms and reduce mortality. Since patients with class II-III H F and
depressed EF <35% have elevated risk of sudden cardiac death due to ventricular
arrhythmias, placement of an implantable cardiac defibrillator (ICD) should be
considered.
In patients with acute decompensated heart failure, the initial treatment goals are
to stabilize the patient’s hemodynamic derangements and to identify and treat reversible
factors that may have precipitated the decompensation, such as arrhythmias or
myocardial ischemia. Regarding hemodynamics, if patients appear to have elevated
LV filling pressures, they often require intravenous vasodilators such as nitroglycerin
infusion, and patients with decreased cardiac output may require inotropes such
as dobutamine, and if hypotensive, they may require vasoconstrictors such as
dopamine.

Aortic Stenosis
The history and physical findings presented in the scenario suggest that this
patient’s heart failure may be a result of aortic stenosis. This is the most common
symptomatic valvular abnormality in adults. The large majority of cases occur in
men. The causes of the valvular stenosis vary depending on the typical age of presentation:
stenosis in patients younger than 30 years usually is caused by a congenital
bicuspid valve; in patients 30 to 70 years old, it usually is caused by congenital
stenosis or acquired rheumatic heart disease; and in patients older than 70 years, it
usually is caused by degenerative calcific stenosis.
Typical physical findings include a narrow pulse pressure, a harsh late-peaking
systolic murmur heard best at the right second intercostal space with radiation
to the carotid arteries, and a delayed slow-rising carotid upstroke (pulsus parvus
et tardus). The electrocardiography (ECG) often shows left ventricular hypertrophy.

Doppler echocardiography reveals a thickened abnormal valve and can define severity
as assessed by the aortic valve area and by estimating the transvalvular pressure
gradient. As the valve orifice narrows, the pressure gradient increases in an attempt
to maintain cardiac output. Severe aortic stenosis is defined as a valve area less than
1 cm2 (normal 3-4 cm2) and mean pressure gradient more than 40 mm Hg.
Symptoms of aortic stenosis develop as a consequence of the resulting left
ventricular hypertrophy as well as the diminished cardiac output caused by the
flow-limiting valvular stenosis. The first symptom typically is angina pectoris,
that is, retrosternal chest pain precipitated by exercise and relieved by rest. As
the stenosis worsens and cardiac output falls, patients may experience syncopal
episodes, typically precipitated by exertion. Finally, because of the low cardiac
output and high diastolic filling pressures, patients develop clinically apparent
heart failure as described earlier. The prognosis for patients worsens as
symptoms develop, with mean survival with angina, syncope, or heart failure of
5 years, 3 years, and 2 years, respectively.
Patients with severe stenosis who are symptomatic should be considered for aortic
valve replacement. Preoperative cardiac catheterization is routinely performed
to provide definitive assessment of aortic valve area and the pressure gradient, as
well as to assess the coronary arteries for significant stenosis. In patients who are
not good candidates for valve replacement, the stenotic valve can be enlarged using
balloon valvuloplasty, but this will provide only temporary relief of symptoms, as
there is a high rate of restenosis. Percutaneous transcatheter aortic valve replacement
(TAVR) is a new technique that has been developed for patients who are
assessed as having unacceptably high surgical risk, and catheter-based aortic valves
have now been approved for use in Europe and the United States.

caso 2
Summary: This 26-year-old woman, with a history of rheumatic fever during adolescence,
is now in the second trimester of pregnancy and presents with acute onset
of palpitations. She is found to have atrial fibrillation (AF) with a rapid ventricular
response. She has a diastolic rumble suggestive of mitral stenosis, which is the likely
cause of her atrial fibrillation as a result of left atrial enlargement. Because of the
increased blood volume associated with pregnancy and the onset of tachycardia
and loss of atrial contraction, the atrial fibrillation has caused her to develop pulmonary
edema.
􀀀 Most likely diagnosis: Atrial fibrillation caused by mitral stenosis.
􀀀 Next step: Cardiac rate control with intravenous beta-blockers.

CLINICAL APPROACH
AF is the most common arrhythmia for which patients seek treatment; it occurs in
acute, paroxysmal, and chronic forms. During AF, disordered atrial depolarization,
often at rates exceeding 300 to 400 bpm, produces an irregular ventricular response,
depending on the number of impulses that are conducted through the atrioventricular
(AV) node. The ECG is characterized by absence of discrete P waves and an
irregularly irregular ventricular contraction. The incidence of AF increases with age,
affecting 5% to 10% of patients older than 75 years. Although many patients can
maintain a normal activity level and remain essentially asymptomatic with chronic
AF, there are several causes of morbidity from this arrhythmia: it may trigger a
rapid ventricular rate leading to myocardial ischemia or exacerbation of heart failure
in patients with heart disease, and thrombus formation in the noncontractile
atria can lead to systemic embolization (AF is a common cause of stroke).
Anything that causes atrial dilation or excessive sympathetic tone can lead to AF,
but the two most common causes of AF are hypertension and coronary atherosclerosis.
The common causes of AF are listed in Table 8– 1.

Acute AF with rapid ventricular response must be addressed quickly. The four
major goals are: (1) hemodynamic stabilization, (2) rate control, (3) anticoagulation,
and (4) possible conversion to sinus rhythm. If a patient is hemodynamically
unstable (hypotensive, angina pectoris, pulmonary edema), urgent direct current
(DC) cardioversion is indicated. If the patient is hemodynamically stable, ventricular
rate control can generally be achieved with intravenous beta-blockers, calcium
channel blockers, or digoxin, which slow conduction through the AV node. Once
the ventricular rate has been controlled, consideration can be given to reversing
the underlying causes (eg, thyrotoxicosis, use of adrenergic stimulants, or worsening
heart failure) so that patients can undergo cardioversion to sinus rhythm. This
may occur spontaneously or after correction of underlying abnormalities, or it may
require pharmacologic or electrical cardioversion. If the duration of AF exceeds
48 hours, the risk of intra-atrial thrombus formation increases.
Rate control alone (ie, the use of agents to maintain a slow ventricular response
rate) is often effective in managing the symptoms of atrial fibrillation, and it has
been shown to be at least as effective as rhythm control for long-term outcomes.
If patients are unstable or persistently symptomatic, however, they may require
efforts to terminate the atrial fibrillation, and restore sinus rhythm. The most
effective method of terminating AF is electrical cardioversion. After cardioversion,
the return of coordinated atrial contraction in the presence of an atrial thrombus
may result in clot embolization, leading to a cerebral infarction or other distant
ischemic event. Therefore, after 24 to 48 hours of AF, patients should receive
3 to 4 weeks of warfarin therapy prior to and after cardioversion to reduce the
risk of thromboembolic phenomena. Alternatively, low-risk patients can undergo
transesophageal echocardiography to exclude the presence of an atrial appendage
thrombus prior to cardioversion. Postcardioversion anticoagulation is still required
for 4 weeks, because even though the rhythm returns to sinus, the atria do not
contract normally for some time. Pharmacologic antiarrhythmic agents, such as
propafenone, sotalol, and amiodarone may be used to try to maintain sinus rhythm.
Many patients with AF cannot be cardioverted and be expected to remain
in sinus rhythm. Two important prognostic factors are left atrial dilation (atrial
diameter >4.5 cm predicts failure of cardioversion) and duration of AF. The longer
the patient is in fibrillation, the more likely the patient is to stay there (“atrial
fibrillation begets atrial fibrillation”) as a consequence of electrical remodeling
of the heart. In patients with chronic AF, the management goals are rate control,
using drugs to reduce AV nodal conduction (such as digitalis or beta-blockers) as
described earlier, and anticoagulation.
Patients with chronic AF who are not anticoagulated have a 1% to 5% per year
incidence of clinically evident embolization such as stroke. Risk-assessment tools
such as the CHA2DS2-VASc score (CHF, Hypertension, Age ≥ 75, Diabetes mellitus,
Stroke/ transient ischemic attack/ thromboembolism, Vascular disease [prior
myocardial infarction, peripheral arterial disease, or aortic plaque], Age 65-74,
Sex category [female gender]) can be used to estimate stroke risk and need for
anticoagulation. CHA2DS2-VASc score correlates with increasing event rate with
increasing score. For chronic AF caused by valvular disease such as mitral stenosis,
the annual risk of stroke is substantially higher. AF that develops in patients
younger than 60 years without evidence of structural heart disease, hypertension,
or other factors for stroke is termed lone AF, and the risk of stroke is very low, so
anticoagulation with warfarin is not used. Instead, aspirin may be used.
Anticoagulation reduces the risk of stroke in patients with chronic AF by two-thirds.
New oral anticoagulants such as dabigatran and rivaroxaban have been developed for
use in atrial fibrillation, but the oral vitamin K antagonist warfarin remains the most
widely used medication for this purpose. Warfarin does not produce a predictable
dose-related response; therefore, the level of anticoagulation needs to be monitored
by regular laboratory testing using the international normalized ratio (INR). In AF
not caused by valvular disease, the goal INR is 2 to 3.
The major complication of warfarin therapy is bleeding as a consequence of
excessive anticoagulation. The risk of bleeding increases as the INR increases. If
the INR is markedly elevated (eg, INR 6-9) but there is no apparent bleeding, the
values will return to normal over several days if the warfarin is held. For higher
levels of INR (such as > 9) but without bleeding, vitamin K can be administered.
If clinically significant bleeding is present, warfarin toxicity can be rapidly reversed
with administration of vitamin K and fresh frozen plasma to replace clotting factors
and provide intravascular volume replacement.

RHEUMATIC HEART DISEASE

In the case presented in the scenario, the cause of this patient’s AF appears to
be mitral stenosis. Because she has a history of acute rheumatic fever, her mitral
stenosis almost certainly is a result of rheumatic heart disease. Rheumatic heart

disease is a late sequela of acute rheumatic fever, usually becoming symptomatic

many years after the original attack. Valvular thickening, fibrosis, and calcifications
lead to valvular stenosis. The mitral valve is most frequently involved. The aortic
valve may also develop stenosis, but usually in combination with the mitral valve.
The right side of the heart is rarely involved.
Most cases of mitral stenosis in adults are secondary to rheumatic heart disease,
especially in the developing world. Congenital mitral stenosis is also commonly
seen. The physical signs of mitral stenosis are a loud S1 and an opening snap following
S2. The S2-OS (mitral valve opening snap) interval narrows as the severity
of the stenosis increases. There is a low-pitched diastolic rumble after the opening
snap, heard best at the apex with the bell of the stethoscope. Because of the stenotic
valve, pressure in the left atrium is increased, leading to left atrial dilation and, ultimately,
to pulmonary hypertension. Pulmonary hypertension can cause hemoptysis
and signs of right-sided heart failure such as peripheral edema. When AF develops,
the rapid ventricular response produces pulmonary congestion as a consequence
of shortened diastolic filling time. Rate control with intravenous beta-blockers or
calcium channel blockers is essential to relief of pulmonary symptoms. In this case,
the mitral stenosis likely became symptomatic due to the patient’s pregnancy, with
increased blood volume and increased cardiac output of up to 30% to 50%.
WOLFF-PARKINSON-WHITE SYNDROME
Another cause of AF is the Wolff-Parkinson-White (WPW) syndrome. In patients
with this condition, AF may be life threatening. In addition to the AV node,
patients with WPW have an accessory pathway that provides an alternate route
for electrical communication between the atria and ventricles, leading to preexcitation,
that is, early ventricular depolarization that begins prior to normal AV
nodal conduction. A portion of ventricular activation occurs over the accessory
pathway, with the remaining occurring normally through the His-Purkinje system.
This preexcitation is recognized on the ECG as a delta wave, or early upslurring
of the R wave, which both widens the QRS complex and shortens the PR
interval, which represents the normal AV nodal conduction time (Figure 8– 2).
Some patients with the ECG abnormalities of WPW syndrome are asymptomatic;
others have recurrent tachyarrhythmias. Most of the tachycardia is caused by
paroxysmal supraventricular tachycardia; one-third of patients will have AF. AF
with conduction to the ventricles over an accessory pathway is a special case for
two reasons. First, when conducted through the accessory pathway, the widened
QRS may look like ventricular tachycardia, except that it will have the irregular
RR interval of AF. Second, because the AV conduction is occurring through the
accessory pathway rather than through the AV node, the ventricular rate may
be very rapid, and the usual AV nodal– blocking drugs given for ventricular rate
control will not affect the accessory pathway. In fact, beta-blockers, verapamil, and
other AV nodal– blocking agents can, paradoxically, increase the ventricular rate
and should be avoided in WPW patients with AF. If hemodynamically unstable,
DC cardioversion should be performed. If hemodynamically stable, the agent
of choice is procainamide or amiodarone, to slow conduction and convert the
rhythm to sinus.
Electrocardiogram revealing the delta wave of Wolff-Parkinson-White syndrome.
(Reproduced, with permission, from Stead LG, Stead SM, Kaufman MS. First Aid for the
Medicine
Clerkship, 2nd ed. New York: McGraw-Hill, 2006:44.)

• CAUSES OF ATRIAL FIBRILLATION

.Structural heart disease (hypertension, mitral valve disease)
.Ischemic heart disease
.Pericarditis or pericardial injury (postsurgical)
.Pulmonary disease (especially pulmonary embolism)
.Hyperthyroidism
.Stress or increased sympathetic tone (acute illness, pheochromocytoma)
.Alcohol consumption (holiday heart syndrome, alcoholic cardiomyopathy)
.Sick sinus syndrome (tachy-brady syndrome)

caso 3

Summary: A 28-year-old man complains of shaking chills and fever. He also has
a productive cough. He denies intravenous drug use. He has a temperature of
102.5°F, heart rate of 109 bpm, and a new holosystolic murmur at the left lower
sternal border, which increases with inspiration. He has linear streaks of induration
on both forearms, and chest radiograph shows multiple ill-defined nodules.
􀀀 Most likely diagnosis: Infective endocarditis involving the tricuspid valve, with
probable septic pulmonary emboli.
􀀀 Next step: Obtain serial blood cultures and institute empiric broad-spectrum
antibiotics.

Considerations
Although this patient denied IV drug use, his track marks on the forearms are
very suspicious for intravenous drug abuse. He has fever, a new heart murmur very
typical of tricuspid regurgitation, and a chest radiograph suggestive of multiple
septic pulmonary emboli. Serial blood cultures, ideally obtained before antibiotics
are started, are essential to establish the diagnosis of infective endocarditis. The
rapidity with which antibiotics are started depends on the clinical presentation of
the patient: a septic, critically ill patient needs antibiotics immediately; a patient
with a subacute presentation can wait many hours while cultures are obtained.

CLINICAL APPROACH
The clinical presentation depends on the which valves are involved (left-sided vs
right-sided), as well as the virulence of the organism. Highly virulent species, such as
Staphylococcus aureus, produce acute infection, and less virulent organisms, such as the
viridans group of streptococci, tend to produce a more subacute illness, which may
evolve over weeks. Fever is present in 95% of all cases. For acute endocarditis, patients
often present with high fever, acute valvular regurgitation, and embolic phenomena
(eg, to the extremities or to the brain, causing stroke). Subacute endocarditis is more
often associated with constitutional symptoms such as anorexia, weight loss, night
sweats, and findings attributable to immune complex deposition and vasculitis;
these include petechiae, splenomegaly, glomerulonephritis, Osler nodes, Janeway
lesions, and Roth spots. These classic peripheral lesions, although frequently discussed,
are actually seen in only 20% to 25% of cases. Splinter hemorrhages under the nails
may also be seen, but this finding is very nonspecific.
Right-sided endocarditis usually involves the tricuspid valve, causing pulmonary
emboli, rather than involving the systemic circulation. Accordingly, patients
develop pleuritic chest pain, purulent sputum, or hemoptysis, and radiographs may
show multiple peripheral nodular lesions, often with cavitation. The murmur of
tricuspid regurgitation may not be present, especially early in the illness.
In all cases of endocarditis, the critical finding is bacteremia, which usually is
sustained. The initiating event is a transient bacteremia, which may be a result of
mucosal injury, as in dental extraction, or a complication of the use of intravascular
catheters. Bacteria are then able to seed valvular endothelium. Previously damaged,
abnormal, or prosthetic valves form vegetations, which are composed of platelets
and fibrin, and are relatively avascular sites where bacteria may grow protected
from immune attack.
Serial blood cultures are the most important step in the diagnosis of endocarditis.
Acutely ill patients should have three blood cultures obtained over a 2- to 3-hour
period prior to initiating antibiotics. In subacute disease, three blood cultures over
a 24-hour period maximize the diagnostic yield. Of course, if patients are critically
ill or hemodynamically unstable, no delay in initiating therapy is appropriate, and
cultures are obtained on presentation, even while broad-spectrum antibiotics are
administered. Usually it is not difficult to isolate the infecting organism, because
the hallmark of infective endocarditis is sustained bacteremia; thus, all blood cultures
often are positive for the microorganism. Table 12– 1 lists typical organisms,
frequency of infection, and associated conditions.