00 Cardiovascular Disorders Lange Outline Usmle Step 2

Front 1

Symptoms
The relationship of symptoms to effort, and relief by cessation of
activity, is characteristic. Asymptomatic at rest.

Diagnosis
Typical symptoms, especially in presence of one or more risk factors
(see section I.D). Exam often normal. Electrocardiogram (ECG) can
be normal in absence of prior myocardial infarction. Cardiac stress
testing (exercise or pharmacologic), with or without adjunctive nu-
clear imaging or echocardiography. In selected cases, coronary an-
giography. Cardiac enzymes (creatine kinase [CK], troponin) will
show laboratory evidence of severe ischemia/myocardial infarction
(MI) (see following sections)

Back 1

Stable Angina Pectoris

Description
Disagreeable chest discomfort, commonly substernal and often de-
scribed as heaviness or squeezing. Can be felt anywhere from epigas-
trium to jaw, arm(s), neck, or back. Dyspnea may accompany chest
discomfort or occur alone. Provoked by exertion or emotional up-
set, relieved within minutes by rest.

Pathology
Increase in myocardial demand for oxygen and nutrient substrate
that exceeds available supply. Coronary blood supply restricted by
arterial narrowing due to atherosclerotic plaque. In some cases, ex-
cessive myocardial demand (e.g., left ventricular hypertrophy due to
aortic stenosis, thyrotoxicosis) can outpace blood supply through
normal coronary arteries, or anemia can impair oxygen delivery.
Stable Angina Pectoris
• Central chest discomfort, can be felt in back, arm(s), jaw, upper abdomen
• Predictable with physical activity or emotional upset, relief with rest
• Cardiac stress test usually abnormal, cardiac catheterization shows CAD
• Medical therapy with nitrates, β-blockers, calcium channel blockers, aspirin, risk factor
modification (long term)
• Revascularization with coronary artery bypass graft (CABG) or PCI

Hint 1

Treatment Steps
1. Nitrates, β-blockers, aspirin, empiric anticoagulation may be used
(heparin, low-molecular-weight heparin).
2. Reduction of cardiovascular risk factors.
3. Treatment of MI if present (see section I.C).
4. Revascularization if indicated (see section I.C).

Front 2

Symptoms
Discomfort identical to stable angina pectoris, except usually more
severe, and occurs under conditions of rest or minimal activity. Un-
stable angina may occur de novo or in patient with known stable
angina pectoris. Commonly occurs at night or in early morning and
may wake patient. May progress to acute myocardial infarction

Diagnosis
Typical symptoms, especially in presence of one or more risk factors.
Exam may be normal. The ECG may show ischemic ST-T abnormali-
ties, especially if recorded during an attack. Exercise stress testing
contraindicated until patieent is asymptomatic and stable. Coronary
angiography in selected cases.

Back 2

Unstable Angina Pectoris

Description
Disagreeable chest discomfort, commonly substernal and often de-
scribed as heaviness or squeezing. Can be felt anywhere from the
epigastrium to pharynx, arm(s), neck, or back. Occurs unpre-
dictably at rest or in a sharply and abruptly worsening pattern com-
pared to previous stable angina. Dyspnea may accompany chest dis-
comfort or occur alone. Duration of unstable angina attacks
generally 20 minutes or less (an attack lasting hours suggests myocardial infarction).

Pathology
Decrease in supply of coronary blood flow and oxygen to a level be-
low that required for baseline metabolic needs of myocardium.
Coronary blood flow is interrupted by fibrin and platelet plug devel-
oping on a fissured or ruptured atherosclerotic plaque. Coronary
vasospasm plays role in some patients.
Unstable Angina Pectoris
• Central chest discomfort, can be felt in back, arm(s), jaw, upper abdomen
• Unpredictable at rest or abruptly worsening pattern of angina, prolonged duration (20 minutes
or more)
• Cardiac catheterization shows CAD, often with ruptured plaque and/or thrombus
• ECG often abnormal (ST depression or T inversion) but no myocardial necrosis by serum
cardiac markers
• Medical therapy with nitrates, β-blockers, aspirin, heparin (unfractionated or low molecular
weight), glycoprotein IIbIIIa platelet receptor blockers, risk factor modification (long term)
• Revascularization with CABG or PCI

Hint 2

Treatment Steps
1. Nitrates, β-blockers, aspirin, empiric anticoagulation may be used
(heparin, low-molecular-weight heparin).
2. Reduction of cardiovascular risk factors.
3. Treatment of MI if present (see section I.C).
4. Revascularization if indicated (see section I.C).

Front 3

Symptoms
Prolonged chest discomfort, most commonly substernal, but may be felt in arm(s), jaw, back, pharynx, epigastrium. Dyspnea, diaphoresis common. Syncope may occur. Pain is sometimes “atypical,” particularly in females and diabetics (see Clinical Pearl).

Diagnosis
Typical history, especially in patient with one or more risk factors for coronary artery disease. Exam may disclose bradycardia, tachycardia, hypotension, hypertension, cardiac gallop(s), cardiac murmur, congestive heart failure (CHF).
The ECG is usually suggestive or diagnostic (ST elevation) of acute myocardial ischemia. Serial serum cardiac marker testing (creatine phosphokinase [CPK] with myocardial
isoenzyme, troponin I or T) confirmatory. Look for evolution of infarction pattern on serial ECGs.

Back 3

Acute Myocardial Infarction (AMI)

Description
Clinical syndrome of ischemic myocardial necrosis. Amount of
necrosis variable depending on quantity of myocardium affected by ischemia, duration of ischemia, collateral blood supply, and treatment administered within the first few hours.

Pathology
Caused by an abrupt decrease in coronary arterial blood flow or, less commonly, by a severe and prolonged increase in myocardial oxygen need that cannot be met by available coronary arterial flow. Abrupt decrease in coronary artery flow usually due to thrombus formation on fissured or ruptured atherosclerotic plaque or hemorrhage into atherosclerotic plaque. Coronary vasoconstriction (spasm), coronary
artery embolus, or spontaneous artery dissection rare causes. Tachycardia or sustained severe hypertension can cause prolonged increase in myocardial oxygen demand sufficient to cause myocardial necrosis. Irreversible necrosis can develop within 1–6 (variable) hours after onset of persistent ischemia.
Acute Myocardial Infarction
• Central chest discomfort, can be felt in back, arm(s), jaw, upper abdomen
• Onset without warning, duration prolonged (often hours)
• ECG usually abnormal, often diagnostic (ST elevation) or suggestive (ST depression)
• Myocardial necrosis by serum cardiac markers
• Medical treatment with aspirin, heparin (unfractionated or low molecular weight), glycoprotein IIbIIIa platelet receptor blockers, β-blockers, nitrates, risk factor modification (long term)
• Urgent reperfusion with thrombolytic drug or PCI
• Cardiac catheterization shows CAD with ruptured plaque and/or thrombus
• Possible complications: mitral regurgitation, ventricular septal defect, cardiac rupture, left ventricular aneurysm

Hint 3

Treatment Steps
1. Aspirin, nitrates, heparin (unfractionated or low molecular weight), glycoprotein IIbIIIa platelet receptor blockers, β-blockers, angiotensin-converting enzyme (ACE) inhibitors (see Cram Facts).
2. Urgent myocardial reperfusion using thrombolytic therapy (alteplase [t-PA], reteplase [r-PA], streptokinase, anistreplase [AP-SAC], or emergency percutaneous transluminal intervention [PCI]) (Fig. 1–1).
3. Treatment of arrhythmia as required.
4. Cardiac catheterization followed by cardiac surgery or PCI may be required for complications of recurrent angina or infarction, mitral regurgitation (acquired) ventricular septal defect, cardial rupture, cardiogenic shock, ventricular aneurysm.
BLOOD THINNERS USED IN AMI
HEPARIN
• Given as continuous intravenous infusion
• Advantage is it can be turned off and reversed quickly
• Disadvantage is it can be difficult to regulate, requiring frequent monitoring of the partial thromboplastin time (PTT)
LOW-MOLECULAR-WEIGHT HEPARIN (LMWH)
• Enoxaparin (Lovenox), dalteparin (Fragmin)
• Given subcutaneously once or twice a day
• No routine monitoring needed (unless patient is morbidly obese or in renal failure)
• Disadvantage is the inability to reverse the drug acutely
GLYCOPROTEIN IIb/IIIa INHIBITORS
• Eptifibatide (Integrilin)
• Acts to inhibit platelet aggregation
• Given intravenously with heparin or LMWH in acute coronary
syndromes

Front 4

CARDIOVASCULAR CAUSES
OF DYSPNEA
differential diagnosis

Back 4

CARDIOVASCULAR CAUSES
OF DYSPNEA
• Cardiomyopathy (dilated, hypertrophic, or restrictive)
• Myocarditis
• Ischemic heart disease (stable or unstable angina, acute MI, acute pulmonary edema)
• Valvular heart disease (especially aortic stenosis,
aortic regurgitation, mitral stenosis, mitral regurgitation)
• Pulmonary embolism
• Primary pulmonary hypertension
• Pericardial effusion/tamponade
• Congenital heart disease (depending on form & severity)
• Arrhythmia

Front 5

• Dyspnea, orthopnea, fatigue, swelling, palpitations.
• Pulmonary rales, edema, cardiac gallop(s), ascites, cardiac enlargement

Back 5

Low-Output Heart Failure

Description
Inability of the heart to deliver adequate blood to meet the meta-
bolic needs of the body in the resting state or with day-to-day activi-
ties, or to do so only at the expense of elevated filling pressure
(Frank–Starling relationship). Most common form of heart failure.

Pathology
Myocardial dysfunction can affect either systolic (contractile) or di-
astolic (relaxation) function. Often, systolic and diastolic dysfunc-
tion coexist, although in some cases (e.g., hypertrophic cardiomy-
opathy) diastolic dysfunction can dominate. Systemic vascular
resistance (SVR) usually increased.
Left ventricle (LV) often affected alone early on, but in severe
cases, both right ventricle (RV) and LV usually involved. Most common cause of RV failure is LV failure.
Systemic hypertension and coronary artery disease are common
causes of CHF. Other etiologies are numerous and include
rheumatic heart disease, valvular diseases, and myocardial diseases.
Low-Output CHF
• Inability of heart to deliver blood adequate for metabolic needs without elevation of filling
pressure. Depressed ejection fraction on echocardiogram.
• Systolic (depression of ventricular contractility) and diastolic dysfunction usually coexist;
diastolic dysfunction alone less common.
• Multiple etiologies, including systemic hypertension, CAD, valvular disease, primary myocardial
disease, rheumatic heart disease, congenital heart disease.
• Echocardiogram to assess cardiac function, valves.
• Neurohormonal pathophysiology (renin–angiotensin and sympathetic nervous systems).

Hint 5

Diagnosis
• History of characteristic symptoms, especially in patient with
known heart disease.
• Workup includes chest x-ray, ECG, Doppler echocardiography, nu-
clear and cardiac imaging. New lab test is BNP (see Clinical Pearl).

Treatment Steps
1. Salt restriction, diuretic as needed for fluid overload.
2. Angiotensin-converting enzyme inhibitors (ACEIs), digitalis for
patients with impaired LV function.
3. Angiotensin receptor blockers (ARBs) or hydralazine/nitrates for
patients intolerant of ACEIs.
4. β-blockers (carvedilol, metoprolol, bisoprolol) for patients with
impaired LV function.
5. Treat arrhythmias as required.
6. Cardiac surgery for selected patients with severe valve dysfunction
• Salt restriction, diuretic, ACE inhibitor, digitalis, β-blocker, treat underlying cause.

Front 6

• Dyspnea, orthopnea, fatigue, swelling, palpitations.
• Pulmonary rales, edema, cardiac gallop(s), ascites, cardiac enlargement
may have bounding pulses and hyperdynamic circula-
tion with warm and flushed extremeties.

Back 6

High-Output Heart Failure

Description
Inability of the heart to pump adequate blood in face of a condition
that requires an increased cardiac output to meet metabolic needs of
the body.

Pathology
Arteriovenous shunting (peripheral circulation admixture) and per-
sistent increased cardiac output (relative to low-output CHF) leads
to salt retention, increased blood volume, and the CHF syndrome.
SVR is normal or reduced (see Cram Facts)
AUSES OF HIGH-OUTPUT
HEART FAILURE
• Severe anemia
• Thyrotoxicosis
• Acute beriberi
• Paget’s disease
• Large arteriovenous
fistula(e)
High-Output CHF
• Inability of heart to deliver blood adequate for increased metabolic needs without elevation of
filling pressure.
• Much less common than low-output CHF.
• Systolic and/or diastolic dysfunction.
• Etiologies include severe anemia, Paget’s disease, beriberi, thyrotoxicosis, large arteriovenous
fistula(e)

Hint 6

Treatment Steps
1. Salt restriction, diuretic.
2. Treat underlying cause.
• ECG with RVH, echocardiogram with RV enlargement and signs of pulmonary hypertension.
• Salt restriction, diuretic, treat underlying cause.

Front 7

Symptoms
Severe dyspnea and orthopnea, often of abrupt onset, with di-
aphoresis. See Table 1–1.

Back 7

Acute Pulmonary Edema

Description
Abrupt increase in pulmonary capillary pressure and vascular vol-
ume of lungs, causing impaired gas exchange and constituting a
medical emergency. Most commonly occurs on cardiogenic basis,
but can occur as a result of noncardiac disease.

Pathology
Cardiogenic pulmonary edema caused by elevation of pulmonary
venous pressure (often precipitous)

Hint 7

Treatment Steps
1. Upright position of head and thorax, supplemental oxygen.
2. Intravenous loop diuretic, nitrates, morphine sulfate.
3. Mechanical ventilation in severe cases.
4. Diagnose and correct contributing factors (see Clinical Pearl).
When acute pulmonary
edema occurs, the cause
of the decompensation
should be sought.
Causes include:
• Acute MI/ischemia
• Dietary indiscretion (high-
sodium meal(s)
• Acute valvular
dysfunction (i.e., rupture
of mitral valve chordae
leading to acute mitral
regurgitation)
• Arrhythmia
• Severe anemia

Front 8

Symptoms
Initial symptoms (fatigue, dyspnea) may be attributed to the pul-
monary condition. Once RV failure is more advanced, passive he-
patic congestion and lower extremity edema can occur. Exam find-
ins include RV gallop or heave, jugular venous distention (JVD),
ascites, and edema.

Back 8

or Pulmonale

Description
Altered structure and function of the right ventricle due to primary pulmonary disease.

Pathology
Lung disease can affect the heart through structural changes of pul-
monary vasculature and/or severe hypoxia.
Severe hypoxia can lead to moderate or severe pulmonary hyper-
tension by causing pulmonary vasoconstriction.
Obliteration of pulmonary vasculature sufficient to cause
significant pulmonary hypertension can occur in pulmonary
embolism, primary pulmonary hypertension, CREST syndrome (cal-
cinosis, Raynaud’s phenomenon, esophageal dysfunction, sclero-
dactyly, telangiectasia).

Hint 8

Diagnosis
Clinical setting. The ECG may show right atrial (RA) and right ven-
tricular hypertrophy (RVH). Echocardiography (RV enlargement/
hypertrophy, tricuspid regurgitation). Arterial blood gases and pul-
monary function testing. Some patients may need right heart
catheterization to confirm diagnosis and to assess response of pulmonary vasculature to vasodilators.

Treatment Steps
Treatment of CHF (see section II.A) and treatment of the underlying condition.

Front 9

An early (before the
next expected sinus node depolarization) beat originating from an
atrial focus outside of the sinus node (Fig. 1–2). Frequent PACs may
portend atrial tachycardia, atrial fibrillation, or atrial flutter

Back 9

Premature Atrial Contraction (PAC)

Front 10

An early beat
originating from the atrioventricular junction (AV node).

Back 10

Premature Junctional Contraction (PJC)

Front 11

A reg-
ular tachycardia (usually 150–250/min) of atrial or atrioventricular
junction origin. Most common mechanism is reentry within the atri-
oventricular junction, but may be due to automatic atrial focus or to
reentry involving atria and ventricles utilizing a conduction tract by-
passing to atrioventricular junction.

Back 11

Paroxysmal Supraventricular Tachycardia (PSVT)

Front 12

A regular tachycardia (usually < 130 per min) due to increased rate of automaticity of the atrioventricular junction. Most commonly due to digitalis toxicity, acute myocardial infarction, or myocarditis.

Back 12

Nonparoxysmal Junctional Tachycardia

Front 13

An irregular tachycardia
(usually 120–200/min) due to rapid atrial depolarizations with vary-
ing nonsinus P wave morphologies (at least three different con-
tours). Usually associated with severe and decompensated lung dis-
ease chronic obstructive pulmonary disease [COPD]).

Back 13

Multifocal Atrial Tachycardia (MAT)

Front 14

A grossly irregular supraventricular rhythm
(may be slow but usually rapid in untreated state) with coarse or fine
atrial fibrillation waves and no identifiable P waves (Fig. 1–3) (see
Clinical Pearl)

Back 14

Atrial Fibrillation
• Patients have an
increased risk of stroke
and are therefore given
anticoagulation with
Coumadin unless
contraindications exist.
• No consensus on which
is a primary goal of atrial
fibrillation: rate control of
atrial fibrillation versus
conversion to normal
sinus rhythm.

Front 15

A supraventricular rhythm with identifiable (“saw-
tooth” ECG baseline) continuous atrial activity with atrial rate of
250–350 per minute (Fig. 1–4). Ventricular rate depends on degree
of block at atrioventricular junction. Ventricular rate of 150 per
minute (2:1 AV block) is common

Back 15

Atrial Flutter

Front 16

—An early beat
originating from any portion of either ventricle (Fig. 1–5). Found in
a variety of diseases or in apparently normal individuals, clinical sig-
nificance depends on underlying cardiac diagnosis

Back 16

Premature Ventricular Contraction (PVC)

Front 17

Consists of three or more consecutive ventricular beats originating from any portion of either ventricle.
Usually paroxysmal and of abrupt onset and termination. May be sustained (≥ 30 beats), nonsustained (< 30 beats), monomorphic (each beat with similar or identical QRS morphology), or polymorphic (QRS morphology varies beat to beat).

Back 17

Ventricular Tachycardia (VT)
Can cause syncope, CHF, angina, or lead to ventricular fibrillation; P waves may or may not be identifiable.

Front 18

A grossly chaotic ventricular tach-
yarrhythmia (ventricular rate > 250 per min or uncountable) with-
out identifiable P waves.

Back 18

Ventricular Fibrillation (VF)

Hint 18

Rapidly fatal if not treated immediately by
direct current countershock (defibrillation). Ventricular flutter, a
ventricular tachyarrhythmia of 150–250 per minute without identifi-
able ST segments or T waves, has same clinical significance as VF.

Front 19

Automatic implantable cardiac defibrillators (AICDs) are becoming
more common. Patients often have an EPS (electrophysiology
study). Whether their arrhythmias (usually ventricular tachycardia
or fibrillation) are inducible, along with their baseline ejection fraction, will determine whether they are candidates for an AICD.

Back 19

Automatic Implantable Cardiac Defibrillator

Front 20

Failure of impulse formation in the sinus node.
Asystole will result unless an escape rhythm (ectopic atrial, junc-
tional, ventricular) develops

Back 20

Sinus Arrest

Front 21

An abnormally slow rate (< 50/min) due to
a slowing of the rate of depolarization of the sinus node. May be
found in healthy individuals, but may be associated with vasovagal re-
action, MI, drug therapy, bradytachycardia syndrome, or aging. Usu-
ally asymptomatic, but can cause episodic weakness or syncope

Back 21

Sinus Bradycardia

Front 22

Prolongation of the PR interval > 0.20
seconds in adults due to slowing of conduction in the AV junction.
May or may not be associated with bradycardia

Back 22

First-Degree AV Block

Front 23

Intermittent failure of the cardiac impulse originating in the atria to reach the ventricles, is of two types:
Mobitz Type I—Caused by block at the atrioventricular junction, is most common type of second-degree heart block.
Mobitz Type II—Caused by block at site distal to the AV junction.Less common than type I, but often a precursor to complete in-franodal AV block (see Fig. 1–6)

Back 23

Second-Degree AV Block

Front 24

Due to complete block of cardiac impulse at the AV junction, His bundle, or both right and left bundle branches. Atrial and ventricular activities are independent. The resultant escape QRS complex is generally regular and will be normal
(narrow, originating from the AV junction) or wide (idioventricular, originating from the ventricles), depending on the level of block. This bradyarrhythmia can cause weakness, angina, CHF, or syncope

Back 24

Third-Degree (Complete) AV Block

Front 25

• Symptoms: Dyspnea (at rest or with exertion), orthopnea, fatigue
• Signs: Pulmonary congestion (rales), cardiomegaly, left ventricular heave and/or gallop

Back 25

LEFT-SIDED HEART FAILURE

Front 26

• Symptoms: “Swelling,” fatigue
• Signs: JVD, cardiomegaly, RV heave or gallop, ascites, hepatosplenomegaly, lower extremity edema

Back 26

RIGHT-SIDED HEART FAILURE

Front 27

Symptoms of heart failure

Diagnosis
Clinical scenario. Chest x-ray with cardiomegaly, pulmonary conges-
tion. Echocardiography shows cardiac dilatation with reduced ven-
tricular ejection fraction. Laboratory studies may confirm specific
etiologies.

Back 27

Congestive (Dilated) Cardiomyopathy

Description
Congestive (dilated) cardiomyopathy is characterized by dilated and
poorly contractile ventricles. Atria are often dilated as well. Right
and left ventricles are often affected together; however, the right or
left ventricle may be affected predominantly, depending on the specific cause or stage of the disease process

Pathology
Decreased ventricular contractility (systolic dysfunction) results in
reduced cardiac output and/or pulmonary congestion (CHF). Basis
for systolic dysfunction depends on etiology and, in some cases, is
unknown. Diastolic dysfunction often coexists. Etiologies are numerous (see Cram Facts).
ETIOLOGIES OF DILATED
CARDIOMYOPATHY
1. Ischemic CAD,
prior MI
2. Systemic hypertension
3. Idiopathic (cause
unknown)
4. Viral (including
coxsackie, human
immunodeficiency
virus [HIV])
5. Familial (increasing
evidence for genetic
basis in many cases)
6. Postpartum/
peripartum
7. Alcoholic (toxic)
8. Chagas’ heart disease
(trypanosomiasis)
9. Radiation therapy
10. Beriberi (thiamine
deficiency)
11. Drug induced
(anthracyclines)
12. Cocaine abuse

Hint 27

Treatment Steps
1. Specific etiology of the cardiomyopathy should be determined to
guide treatment and prognosis. Cardiac catheterization is usually
indicated initially to rule out ischemic heart disease.
2. Treatment of CHF (see section II.A).

Front 28

Symptoms
Dyspnea, chest pain, near-syncope, or syncope, which can be caused
by arrhythmia (ventricular tachycardia or atrial fibrillation) or LV
outflow tract obstruction.
On exam, systolic murmur at lower left sternal border, bifid
carotid pulse (in cases of LV outflow obstruction), S4. May have
murmur of mitral regurgitation.

Back 28

Hypertrophic Obstructive
Cardiomyopathy (HOCM)

Description
A form of cardiomyopathy characterized by a hypertrophic and
nondilated left ventricle in the absence of a systemic or coexisting
cardiac condition capable of producing left ventricular hypertrophy
(LVH). Often disproportionate hypertrophy of the ventricular sep-
tum, which may produce variable amounts of obstruction in the left
ventricular outflow tract. Right ventricle can be hypertrophied as well. May be familial or sporadic

Pathology
Clear evidence for genetically determined abnormalities of several con-
tactile proteins in the cardiac myocyte. Diastolic dysfunction causes
pulmonary congestion, restriction to adequate left ventricular filling.
Dynamic obstruction to left ventricular outflow common, but does not always occur. Systolic dysfunction can occur in advanced cases.
HYPERTROPHIC
CARDIOMYOPATHY
• Important to diagnose
due to an increased risk
of sudden death,
particularly in young
people.
• The murmur of HOCM
decreases with handgrip,
increases with standing

Hint 28

Diagnosis
Patient history, family history, and exam. ECG shows LVH. Echocardiography usually diagnostic.

Treatment Steps
1. Avoid dehydration.
2. Strenuous activity prohibited.
3. β-Blockers.
4. Calcium channel blockers.
5. Surgical myectomy of hypertrophied ventricular septum in severely
obstructed patients when medical therapy inadequate.
6. Permanent atrioventricular pacing beneficial in some refractory
cases.
7. Alcohol ablation of a portion of the ventricular septum by PCI techniques.
8. Screening of family members.

Front 29

Symptoms
Fatigue. Edema.
On exam, neck vein distention, edema, ascites. May mimic constrictive pericarditis.

Diagnosis
Echocardiography may show endomyocardial fibroelastosis and/or
restricive filling pattern. Cardiac catheterization. Endometrial biopsy
sometimes used.

Back 29

Restrictive Cardiomyopathy

Description
A form of cardiomyopathy characterized by noncompliant, poorly
distensible ventricle(s) on basis of infiltrative process. Ventricular
systolic function often normal, and heart may not be enlarged. Least
common form of cardiomyopathy

Pathology
Any infiltrative process of myocardium that results in interstitial fi-
brosis or thickening of heart wall (not myocyte hypertrophy).
• Amyloidosis
• Sarcoidosis
• Endomyocardial fibroelastosis (may occur with or without idio-
pathic hypereosinophilic syndrome)
• Glycogen storage disease

Hint 29

Treatment Steps
1. Diuretic, salt restriction.
2. Treat underlying disease.

Front 30

Symptoms
Often preceded by upper respiratory tract infection (viral). Many
cases mild and self-limited, may even be asymptomatic. In more se-
vere cases, fever, dyspnea, edema, chest pain.
On exam, tachycardia, pulmonary congestion, edema, gallops,
cardiomegaly.

Back 30

Myocarditis

Description
Inflammation of the myocardium with inflammatory cellular infiltrate and varying degrees of myocyte necrosis. Myocarditis may be
difficult or impossible to distinguish clinically from cardiomyopathy
(see preceding sections). Some forms of cardiomyopathy may evolve
from myocarditis.

Pathology
Viral—Very common; especially coxsackie B, but also influenza,
varicella, echo virus, infectious mononucleosis, coxsackie A, measles,
HIV-2 with the acquired immune deficiency syndrome (AIDS), and others. Bacterial fungal, rickettsial causes rare.

Hint 30

Diagnosis
History, exam. Chest x-ray with cardiomegaly, CHF. Elevated sedi-
mentation rate. Viral cultures and titers useful in some cases. Blood
cultures (bacterial). Right ventricular endomyocardial biopsy may have a role.

Treatment Steps
1. Treat congestive heart failure.
2. Treat underlying condition.
3. Immunosuppresive therapy in selected cases.

Front 31

Symptoms
Chest pain, often sharp and knifelike, central or left precordial, can radiate to shoulders or back. Often positional and with pleuritic component; however, may be asymptomatic. May evolve into chronic form.

Diagnosis
History. On exam, hallmark is three-component pericardial friction rub, but only one or two components may be audible, or rub may not be present.
Often leukocytosis, elevated sedimentation rate.
Acute and convalescent viral titers.
ECG with diffuse ST-T abnormality, typically with diffuse elevation of ST segment.
Echocardiography may show pericardial effusion. Important to establish etiologic diagnosis if possible.

Back 31

Acute Pericarditis

Description
Inflammatory process involving pericardium, by spread either from myoepicardium or from adjacent structure.
- PERICarditis:
Pulsus paradoxus
ECG changes
Rub
Increased JVP
Chest pain [worse on inspiration, better when lean forward]

CAUSES OF PERICARDITIS
• Infectious––most commonly coxsackie B virus, other viral,
bacterial, fungal, parasitic, or tuberculosis.
• Dressler syndrome (post cardiotomy and post myocardial infarct) likely autoimmune in nature.
• Malignancy.
• Idiopathic.
• Trauma.
• Radiation therapy.
• Uremia.
• Systemic lupus erythematosus.
• Rheumatic fever.
• Rheumatoid arthritis.
• Scleroderma.
- PERICARDITIS
P - Post Traumatic
E - Endocrine: Hypothyroid
R - Renal Failure
I - Infection: TB, Viral, Fungal, AIDS, Bacterial
C - Collagen Vascular Disease (SLE, RA)
A - Aneurysm
R - Rheumatic Fever- Radiation
D - Drugs: Hydralazine, Minoxidil, Procainamide
I - Infarction - AMI
TI - Tumor Invasion
S - Syphilis, Scleroderma, Serum Sickness
- PR DIP, ST UP:
Post-pericardiectomy
Rheumatic fever
Drugs (eg isoniazid, hydralazine, procainalmide)
Infection (eg TB, coxsackie, strep)
PE
SLE/Scleroderma
Tumours/ Thyroid disease/TB
Uraemia
Post MI (includes Dressler's)
- CARDIAC RIND:
Collagen vascular disease
Aortic aneurysm
Radiation
Drugs (such as hydralazine)
Infections
Acute renal failure
Cardiac infarction
Rheumatic fever
Injury
Neoplasms
Dressler's syndrome

Hint 31

Treatment Steps
1. Symptomatic and supportive.
2. Nonsteroidal anti-inflammatory drugs (NSAIDs) or aspirin.
Steroids occasionally required.
3. Treat underlying disease.

Front 32

Symptoms
May be asymptomatic or have chest pain of pericarditis. Cardiac tam-
ponade with hemodynamic collapse can occur

Back 32

Pericardial Effusion

Description
Accumulation of serous or serosanguinous fluid in pericardial space.
Can be acute or chronic. Rapidity of fluid collection the major determinant of hemodynamic effect.

Pathology
Most common causes are viral, idiopathic, uremic, rheumatoid syn-
dromes, thyroid disease, and malignant pericarditis; however, any
cause of pericarditis can cause pericardial effusion, and effusion may be the presenting sign of pericardial disease.

Hint 32

Diagnosis
History, exam. May have pericardial friction rub or dullness at left
lung base posteriorly due to compression of lung by pericardial sac
(Ewart’s sign). Chest x-ray with “cardiomegaly.” The ECG may show
pattern of pericarditis, also electrical alternans; echocardiography
documents presence and size of effusion and may show signs of car-
diac tamponade, if present. Pericardiocentesis and/or pericardial biopsy helpful in establishing etiology

Treatment Steps
1. Treat underlying disorder.
2. Pericardial biopsy or pericardiocentesis may be required.
3. Treat pericarditis symptoms (see above), if present.

Front 33

Symptoms
Acutely ill. Dyspnea and hemodynamic collapse. If tamponade develops slowly, can mimic CHF.

Diagnosis
History, exam. Neck vein distention in setting of hypotension and
pulsus paradoxicus. Heart sounds may be muffled, and pericardial friction rub may be heard.

Back 33

Cardiac Tamponade
(by Pericardial Effusion)

Description
Marked reduction of cardiac output due to interference with normal
cardiac filling by the compressive effect of pericardial effusion. Rate
of accumulation of pericardial fluid important. Rapid accumulation of
fluid can cause cardiac tamponade by relatively small volumes of pericardial fluid (as little as a few hundred milliliters)

Pathology
Can occur with pericardial effusion of any cause, but most com-
monly associated with trauma, cardiac surgery, malignancy. Occa-
sionally, cardiac tamponade is due to pericardial effusion from viral or idiopathic pericarditis or following radiation therapy.

Hint 33

Treatment Steps
1. Pericardiocentesis or surgical pericardiotomy without delay can
be lifesaving.
2. Treat underlying disease after relief of cardiac tamponade.

Front 34

Symptoms
Edema, ascites, hepatic congestion, fatigue.
Exam shows signs of systemic venous congestion, especially neck
vein distention. May have Kussmaul’s sign and/or pericardial knock.

Back 34

Constrictive Pericarditis

Description
Chronic or subacute process of thickening of pericardium resulting in cardiac encasement.

Pathology
Almost any cause of acute pericarditis can lead to pericardial con-
striction, but most commonly due to radiation therapy, virus, idio-
pathic process, collagen vascular disease, uremia, or malignancy.
Thickened noncompliant pericardial sac causes marked limita-
tion to cardiac filling during ventricular diastole, resulting in systemic venous congestion and limitation of cardiac output reserve.

Hint 34

Diagnosis
History, exam. The ECG is nonspecific, but often shows low QRS
voltage. Chest x-ray often with cardiomegaly, may show pericardial
calcification. Echocardiography may show thickened pericardium.
Cardiac catheterization confirms diagnosis by demonstration of
characteristic hemodynamics (“dip and plateau” contour of ventricular diastolic pressure).

Treatment Steps
1. Diuretic, salt restriction.
2. Definitive therapy requires surgical pericardiectomy.
3. Treat underlying disease.

Front 35

• Symptoms of angina, dyspnea, syncope
• Harsh, mid- to late-peaking systolic murmur at base of heart radiating to neck; S4 gallop; LV
heave; aortic regurgitation may coexist (see below)
• ECG with LVH, left atrial abnormality

Back 35

Aortic Stenosis (AS)
• Etiologies include degenerative process (calcific AS of the elderly), congenital malformation
(e.g., bicuspid), and rheumatic heart disease (RHD)

Hint 35

• Confirm by Doppler echocardiography, cardiac catheterization
• Bacterial endocarditis prophylaxis
• Aortic valve replacement for symptomatic patients

Front 36

• Symptoms of dyspnea, fatigue (angina, syncope rare)
• High-pitched, decrescendo diastolic murmur loudest at left sternal border or base, also heard at
LV apex; S3 common; LV enlargement
• ECG with LVH, often left atrial abnormality

Back 36

Aortic Regurgitation (AR)
• Etiologies multiple, including congenital malformation, RHD, aortic root abnormality (e.g., Marfan
syndrome, dissection), trauma, aortitis, endocarditis

Hint 36

• Confirm by Doppler echocardiography, cardiac catheterization
• Bacterial endocarditis prophylaxis
• Aortic valve replacement in selected cases

Front 37

• Symptoms of dyspnea, fatigue, or RV failure
• Low-pitched (rumble) diastolic murmur at LV apex; opening “snap” early in diastole; loud S1;
MR (see below) may coexist
• ECG frequently with left atrial abnormality or atrial fibrillation, may have RVH

Back 37

Mitral Stenosis (MS)
• Usually due to rheumatic heart disease

Hint 37

• Confirm with Doppler echocardiography, cardiac catheterization
• Bacterial endocarditis prophylaxis
• High risk of systemic thromboembolism, risk reduced with warfarin anticoagulation
• Mitral valve replacement/repair or percutaneous balloon valvuloplasty in selected cases

Front 38

• Symptoms of dyspnea, fatigue
• High-pitched holosystolic (or crescendo systolic in case of MV prolapse) murmur at LV apex or left sternal border; S3 (chronic) or S4 (acute); LV enlargement (chronic)
• ECG with left atrial abnormality, LVH, may show atrial fibrillation

Back 38

Mitral Regurgitation (MR)
• Etiologies multiple, including degenerative process (myxomatous change), ischemia, RHD,
endocarditis, connective tissue disease (e.g., Marfan syndrome)
• Chronic or (less common) acute forms, depending on etiology

Hint 38

• Confirm with Doppler echocardiography, cardiac catheterization
• Bacterial endocarditis prophylaxis
• Mitral valve replacement/repair in selected cases

Front 39

Symptoms
Fever, malaise, polyarthritis, chorea, rash. Dyspnea from acute heart
failure caused by valvular regurgitation and/or myocarditis. Acute
pericarditis can occur. Many cases resolve without clinical sequelae.
Most common in children 5–15 years of age, although can occur in any age group.

Back 39

Acute Rheumatic Fever

Description
Inflammatory disease affecting skin, joints, heart, subcutaneous tis-
sue, and central nervous system that develops following group A
streptococcal pharyngitis. Common cause of valvular heart disease,
but incidence declining in developed countries. Valvular sequelae
include stenosis and/or regurgitation of aortic and/or mitral valves, less commonly affects right-sided heart valves.
1. Major Criteria
• Carditis—most common and most reliable sign
• Erythema marginatum
• Polyarthritis
• Chorea (Sydenham’s)
• Subcutaneous nodules
2. Minor Criteria
• Fever
• Arthralgia
• Prior documented ARF or preexisting evidence of rheumatic
heart disease
• Prolonged PR interval on ECG
• Elevated sedimentation rate or increased C-reactive protein

Pathology
Invasive infection with group A streptococcus probably elicits an an-
tibody response with cross-reactivity with host tissues (autoimmune
mechanism).

Hint 39

Prevention
Prevention of initial attack of ARF by adequate and prompt antibi-
otic therapy for streptococcal pharyngitis.
Prevention of recurrent attack of ARF by long-term prophylaxis
against recurrent group A streptococcal infection.

Diagnosis
History, exam. Many of the signs, symptoms, and laboratory findings
of acute rheumatic fever (ARF) are nonspecific, so the modified
Jones criteria are often required for diagnosis: two major criteria, or
one major and two minor criteria, and evidence (usually serologic) of preceding streptococcal infection.

Treatment Steps
1. Antibiotic treatment (but treatment of established attack does not
prevent subsequent heart involvement).
2. Bed rest, salicylates.
3. Sedatives for chorea.
4. Corticosteroids for moderate or severe carditis.
5. Diuretics, salt restriction if CHF. Cardiac surgery rarely required for severe valvular regurgitation during acute phase of illness

Front 40

Symptoms
Dyspnea, often precipitated by atrial fibrillation. Fatigue. Left atrial
thrombi can cause systemic embolization. In advanced cases, right
heart failure with edema, hepatic congestion.
On exam, loud S1, opening snap in early diastole, low-pitched di-
astolic murmur (“rumble”) at LV apex. May have pulmonary conges-
tion. If RV failure, edema and neck vein distention

Back 40

Mitral Stenosis (MS)

Description
Narrowing of the mitral valve orifice following inflammation of ante-
rior and posterior mitral leaflets and variable degree of fusion and
shortening of chordae tendineae. Causes obstruction to LV inflow
from the left atrium. May coexist with mitral regurgitation.

Pathology
Almost always a sequel of rheumatic fever (years to decades after
acute attack). Rarely, congenital or due to mitral annular calcifica-
tion. Atrial myxoma can mimic hemodynamic abnormality of MS.
Increase in left atrial pressure causes pulmonary congestion, left
atrial enlargement, atrial arrhythmias.

Hint 40

Diagnosis
History, exam. Acute pulmonary edema with new onset AF may be
presenting symptom of previously clinically silent mitral stenosis (MS).
With left atrial enlargement or AF, ECG may have RVH. Chest x-ray
with left atrial enlargement, pulmonary venous hypertension. Doppler
echocardiography extremely helpful. Cardiac catheterization.

Treatment Steps
1. Diuretic, salt restriction.
2. Control of atrial fibrillation, if present.
3. Anticoagulation to lower risk of thromboembolism.
4. Bacterial endocarditis prophylaxis.
5. Mitral valve surgery or percutaneous valvulotomy in advanced cases.

Front 41

Symptoms
Will vary markedly from asymptomatic to severely compromised due to CHF, depending on degree and acuteness of MR, left ventricular function, and associated arrhythmias. Systemic embolization may occur in setting of atrial fibrillation.
On exam, holosystolic or crescendo (mitral valve prolapse
[MVP]) systolic murmur, loudest at LV apex. May have S3 (chronic MR) or S4 (acute MR). Pulmonary congestion in severe cases.

Back 41

Mitral Regurgitation (MR)

Description
Reflux of blood from the left ventricle into the left atrium during ventricular systole through the mitral valve orifice.
Severe regurgitation causes left ventricular volume overload and pulmonary congestion.
May be acute or chronic, and may coexist with mitral stenosis.

Pathology
Mechanism of MR can involve dysfunction of any portion of the mitral valve apparatus: annulus, leaflets, chordae tendineae, papillary muscles, or adjacent left ventricular wall. Pathologic processes with potential to affect one or more valve components are multiple and include rheumatic fever, endocarditis, AMI, degenerative (MVP syndrome [Fig. 1–7]), Marfan’s syndrome, congenital lesions, trauma, mitral annular dilatation, collagen vascular disease syndromes, or calcification.

Hint 41

Diagnosis
History, exam. Chest x-ray often shows cardiomegaly, and may show CHF. The ECG often shows LVH, left atrial abnormality. Doppler echocardiography very useful to assess mechanism and severity of MR. Cardiac catheterization.

Treatment Steps
1. Salt restriction, diuretic for CHF.
2. Vasodilators (ACE inhibitors, hydralazine, nitrates, sodium nitroprusside), especially in treatment of acute MR with CHF and/or low cardiac output.
3. Treat arrhythmias as required.
4. Bacterial endocarditis prophylaxis.
5. Mitral valve surgery (replacement/repair) for selected cases.

Front 42

Symptoms
Angina pectoris, CHF, syncope.
On exam, crescendo–decrescendo mid- or late-peaking harsh systolic murmur in aortic area radiating to the neck. Carotid upstroke diminished and delayed. The S2 is absent or diminished.

Back 42

Aortic Stenosis (AS)

Description
Obstruction to blood flow from the left ventricle into the aorta at the level of the aortic valve. May coexist with aortic regurgitation.

Pathology
Failure of the aortic valve orifice to open fully during ventricular systole may be caused by marked thickening and calcification of the aortic valve leaflets (as in degenerative calcific aortic stenosis [AS]), commissural fusion from inflammation (as in AS after rheumatic fever), or may be congenital in origin (as in AS developing on a bicuspid aortic valve [see Fig. 1–8]).

Hint 42

Diagnosis
History, exam. ECG with LVH. Chest x-ray with cardiomegaly, CHF in some cases. Doppler echocardiography very helpful. Cardiac catheterization.

Treatment Steps
1. Surgical aortic valve replacement for symptomatic AS.
2. Bacterial endocarditis prophylaxis.
3. Diuretic, salt restriction for CHF.

Front 43

Symptoms
Dyspnea. Fatigue. Palpitations. Syncope and angina uncommon.
CHF in severe cases. Asymptomatic in mild cases.
On exam, decrescendo diastolic murmur loudest at left sternal
border or aortic area, cardiomegaly, S3 gallop. In severe, acute aortic regurgitation, murmur may be inaudible.

Back 43

Aortic Regurgitation (AR)

Description
Reflux of blood from the aorta into the left ventricle during ventric-
ular diastole due to incompetent aortic valve. Can take acute or
chronic form. May coexist with aortic stenosis.

Pathology
Loss of aortic valve competence can be caused by developmental ab-
normality, destruction of aortic leaflets, or by loss of support of aor-
tic leaflets due to aortic root disease. Etiologic factors include endo-
carditis, congenital (including bicuspid aortic valve), trauma,
inflammation (rheumatic fever), dissection of the ascending aortic,
aneurysm of the ascending aorta, aortitis (ankylosing spondylitis, syphilis, Reiter’s syndrome).

Hint 43

Diagnosis
History, exam. The ECG with LVH. Chest x-ray with cardiomegaly,
possible CHF; aortic root dilatation often found. Doppler echocardiography. Cardiac catheterization.

Treatment Steps
1. Salt restriction, diuretics for CHF.
2. Vasodilators.
3. Bacterial endocarditis prophylaxis.
4. Surgical aortic valve replacement in selected cases.

Front 44

Symptoms
Pulselessness, pain, pallor, paresthesias, paralysis, and poikilother-
mia.

Back 44

Arterial Embolism

Description
Heart is the source of arterial emboli in 90% (see Cram Facts). Left
atrial myxoma (histology of its embolus is diagnostic). Symptoms are
acute in onset and occur at bifurcations (femoral bifurcation most
common).
CARDIAC SOURCES
OF EMBOLI
• Left ventricular thrombus
(post-MI patients)
• Left atrium clot (atrial
fibrillation)
• Valvular disease
(prosthetic valve,
endocarditis, rheumatic
heart disease) with or
without right-to-left
cardiac shunt
• Left atrial myxoma
(primary cardiac tumor,
no risk factors)

Hint 44

Diagnosis
Physical examination, duplex scan, arteriography.

Treatment Steps
1. Heparinization.
2. Consider thrombolytic therapy (tPA).
3. Embolectomy (does not treat the embolus but prevents further
emboli or propagation of thrombosis) can restore flow to tissues.

Front 45

Symptoms
Fever. Malaise. Anemia, weight loss, emboli, renal failure, metastatic abscess, arthritis, CHF.
On exam, elevated temperature, evidence of systemic (left heart involvement) or pulmonary (right heart involvement) embolization, cardiac murmurs (any regurgitant lesion), signs of CHF

Back 45

Endocarditis

Description
Infection of endocardium, usually valvular but may involve mural endocardium or prosthetic heart valve. Subacute and acute forms, depending on organism involved and clinical setting. Abnormal heart valves and congenital heart lesions most often affected, but may occur in the normal heart.

Pathology
Most common causative organism of subacute bacterial endocarditis is Streptococcus viridans. Most common cause of acute bacterial endocarditis is Staphylococcus aureus. Other causes include Staphylococcus epidermidis, gram-negative bacteria, fungi, pneumococcus, gonococcus, and (rarely) spirochetes and rickettsiae. Mitral valve most commonly involved, followed by aortic and tricuspid. Pulmonic valve rarely affected by endocarditis.
HACEK ORGANISMS
These are slow-growing gram-negative bacilli and
are responsible for about 3% of all cases of
endocarditis.
• Haemophilus parainfluenza
• Haemophilus paraphrophilus
• Actinobacillus actinomycetemcomitans
• Cardiobacterium hominis
• Eikenella corrodens
• Kingella kingae

Hint 45

Diagnosis
History, exam. High index of suspicion if preexisting cardiac lesions or illicit intravenous drug use. Blood cultures. Doppler echocardiography. Elevated sedimentation rate, rheumatoid factor.

Treatment Steps
1. Early treatment (even while blood culture results pending) with appropriate bacteriocidal or fungicidal antibiotics.
2. Cardiac surgery with replacement of affected valve in selected cases. For CHF due to cardiac valve dysfunction, recurrent emboli on treatment, myocardial abscess.

Front 46

Symptoms
Claudication, rest pain, tissue loss. Leriche syndrome: buttock clau-
dication, impotence, diminished femoral pulses, bruit, thrill, eleva-
tion pallor, dependent rubor.

Back 46

Aortoiliac Occlusive Disease

Description
Arteriosclerosis involves arteries singly or in combination. Slow progression of occlusive disease allows for collateral formation.
ATHEROSCLEROSIS RISK
FACTORS
• Cigarette smoking
• Diabetes
• Hypertension
• Hyperlipidemia
• Genetics

Hint 46

Diagnosis
History and physical. Noninvasive tests: ankle–brachial index (Fig.
18–4), pulse volume recording, duplex scan, arteriography.

Treatment Steps
Medical—Control risk factors, control hypertension, lower choles-
terol, stop smoking, cilastozol (Pletal), exercise program.
Surgical—Bypass, endarterectomy, angioplasty with or without
stent (consider patient’s comorbid factors and location and extent of the lesion in deciding for stent or open procedure).

Front 47

Complications of Prosthetic
Heart Valves
Endocarditis—(Early or late after implantation), embolization,
perivalvular leak, mechanical failure, hemorrhagic complications of
anticoagulation.

Back 47

Prosthetic Heart Valve

Description
Artificial device designed to be surgically implanted as a functional
replacement for a cardiac valve that is severely malfunctioning. Valve
repair is sometimes an alternative to replacement

Hint 47

Prosthetic Valve Types
Mechanical Prosthesis—Designs include ball-in-cage (Starr Ed-
wards) and tilting disc (St. Jude, Hall Medtronic, Bjork Shiley) mod-
els, are considered durable, but require chronic anticoagulation.
Bioprosthesis—Designs (porcine, bovine pericardial, homograft)
generally do not require long-term anticoagulation, but valve deterioration a problem especially in younger patients

Front 48

Symptoms
Intact aneurysms are asymptomatic. Expanding or leaking causes back pain and abdominal pain. Pulsatile abdominal mass.

Back 48

Abdominal Aortic Aneurysm

Description
Typically involve infrarenal aorta, but may extend above renals. Most
are fusiform. More degenerative than atherosclerosis. More common in males. (See Fig. 18–5.)
ATHEROSCLEROSIS RISK
FACTORS
• Cigarette smoking
• Diabetes
• Hypertension
• Hyperlipidemia
• Genetics

Hint 48

Diagnosis
Physical examination, calcific outline on plain x-ray, ultrasound, CT scan (Fig. 18–6), arteriography.

Treatment Steps
1. Asymptomatic less than 5 cm: observe.
2. Greater than 5 cm: Elective aneurysmectomy with aortobifemoral
graft or tube graft.
3. Endovascular repair (stent grafts) undergoing clinical trials. En-
dovascular abdominal aortic aneurysm (AAA) repair has more limitations based on anatomic issues.

Front 49

Symptoms
Harsh systolic murmur at left sternal border, radiates to right precordium. Spontaneous closure 30–50% of cases. If shunt small, may be asymptomatic. If shunt large, CHF and potential of pulmonary vascular disease with reversal of shunt and Eisenmenger syndrome

Back 49

Ventricular Septal Defect (VSD)

Description and Pathology
Most common congenital heart lesion, other than congenital abnormality of the aortic valve. Persistent opening in the upper portion of the ventricular septum (membranous septum) with resultant left-to-right shunt at the ventricular level.

Hint 49

Diagnosis
History, exam. The ECG may show LVH. Doppler echocardiography should be diagnostic. Chest x-ray with cardiomegaly and increased pulmonary vasculature if shunt significant. Cardiac catheterization.

Treatment Steps
1. For asymptomatic patient with small VSD, observation.
2. For patient with large VSD and significant shunt flow, surgical repair.
3. Bacterial endocarditis prophylaxis.

Front 50

Symptoms
Claudication, rest pain, tissue loss.

Back 50

Femoropopliteal Occlusive Disease

Description
Arteries involved singly or in combination.

Hint 50

Diagnosis
Physical examination, noninvasive tests, arteriography

Treatment Steps
1. Control risk factors, cilastozol (Pletal), pentoxifylline (Trental),
exercise programs.
2. Surgery for disabling claudication, rest pain, or tissue loss.

Front 51

Symptoms
Often asymptomatic as child or teenager. Dyspnea, fatigue, and atrial arrhythmias develop in early or middle adulthood, sometimes sooner. Right heart failure can occur. In advanced cases, severe pulmonary HTN and Eisenmenger syndrome can develop.
On exam, fixed splitting of second heart sound, systolic ejection murmur at left upper sternal border, and right ventricular enlargement. If CHF has developed, neck vein distention and edema. Mitral regurgitation due to MVP (secundum ASD) or cleft in anterior mitral leaflet (primum ASD).

Back 51

Atrial Septal Defect (ASD)

Description and Pathology
Persistent opening in atrial septum, most commonly in region of fossa ovalis (ASD of secundum septum), but can also occur in lower portion of atrial septum (ASD of primum septum) or in sinus node region of the atrial septum (sinus venosus ASD). Left-to-right shunt at the atrial level. More common in females.

Hint 51

Diagnosis
History, but note that patient may be asymptomatic.
The ECG almost always with incomplete or complete right bundle branch block (RBBB), and in addition usually has left axis deviation in cases of ASD of primum septum.
Chest x-ray with cardiomegaly and increased pulmonary vasculature.
Doppler echocardiography diagnostic in most cases.
Cardiac catheterization.

Treatment Steps
1. Observation for small shunt if asymptomatic.
2. Surgical repair or percutaneous closure for significant shunt.

Front 52

Symptoms
Transient ischemic attacks (amaurosis fugax), stroke, vertebral–basi-
lar insufficiency (ataxia, vertigo, diplopia), diminished pulses, bruits.

Back 52

Cerebrovascular Disease

Description
Lesions of the extracranial cerebral vessels. Decreased cerebral per-
fusion from occlusive lesions, thrombosis, or embolization. Majority due to thromboembolic etiologies.

Hint 52

Diagnosis
Duplex scan, arteriography (Fig. 18–7). Angiogram can be done as
an intervention, but CT or magnetic resonance angiography is possi-
ble without the risks of an invasive procedure.

Treatment Steps
1. Medical therapy. Antiplatelet agents (aspirin, persantine,
ticlopide).
2. Endarterectomy.
3. Carotid balloon angioplasty with stenting undergoing clinical trials.

Front 53

Symptoms
Hallmark is continuous “machinery” murmur at upper left sternal
border. S2 may be paradoxically split. Wide systemic pulse pressure
with abnormally low diastolic pressure. Left ventricular volume overload can lead to CHF.

Back 53

Patent Ductus Arteriosus

Description and Pathology
Arteriovenous fistula between aorta and left pulmonary artery due
to failure of embryonic ductus to close after birth. Left-to-right shunt
increases work on the left heart. Fistulous connection commonly in-
serts near origin of left subclavian artery. More common in births at
high altitude, in births to mothers exposed to rubella in first trimester of pregnancy, in premature babies, and in females.

Hint 53

Diagnosis
History, exam. The ECG with LVH. Chest x-ray with cardiomegaly
and increased pulmonary vasculature. Cardiac catheterization.

Treatment Steps
1. Surgical closure, optimal time 1–2 years of age.
2. Bacterial endocarditis prophylaxis

Front 54

Symptoms
Asymptomatic in 50%, swelling, tenderness, Homan’s sign (calf pain with dorsiflexion of foot). Phlegmasia alba dolens: milk leg. Phlegmasia cerulea dolens: venous gangrene

Back 54

Deep Vein Thrombosis (DVT)

Description
Those at risk: elderly, bedridden, hip replacement and other orthopedic procedures, pelvic and abdominal procedures, trauma, malignancy, and estrogen therapy. Virchow’s triad: stasis, intimal damage, hypercoagulability.

Hint 54

Diagnosis
Duplex scan, venography, impedance plethysmography, fibrinogen scan (best study for calf DVT).

Prophylactic Measures
1. Early ambulation postoperatively.
2. Low-dose subcutaneous heparin or enoxaparin/low-molecular-weight heparin.
3. Elastic compression stocking or intermittent pneumatic compression device.

Treatment Steps
1. Bed rest (for iliofemoral DVT or extensive clot burden).
2. Thrombolytic therapy with iliofemoral DVT.
3. Gradient compressive hose.
4. Inferior vena cava (IVC) filter if failure of anticoagulation or contraindications or complications from anticoagulation.
5. Venous thrombectomy (and fasciotomies for compartment syndrome in cases of severe compromise). Very uncommon.
MEDICATIONS FOR DVT
• Unfractionated heparin (maintain partial thromboplastin time 1.5 × control)
or
• Low-molecular-weight heparin (once or twice daily subcutaneous injection)
and
• Warfarin (Coumadin) orally for long-term treatment

Front 55

Symptoms
Vary greatly from asymptomatic to severe hypertension resulting in headache or CHF, intracranial hemorrhage, lower extremity claudication. Aortic rupture a risk.
On exam, upper extremity hypertension with reduced or absent lower extremity pulses, systolic ejection murmur (may be continuous) anterior chest and upper back. May also have aortic regurgitation due to bicuspid aortic valve, which is associated with coarctation of aorta.

Back 55

Coarctation of Aorta

Description and Pathology
Narrowing of aortic lumen, usually immediately distal to origin of left subclavian artery near insertion of ligamentum arteriosum. Associated with Turner syndrome. A cause of secondary hypertension.
More common in males.

Hint 55

Diagnosis
History, exam. The ECG often with LVH. Chest x-ray may show cardiomegaly, rib notching, dilated aorta, and left subclavian artery.
Conventional contrast aortography or magnetic resonance angiography (MRA). Doppler echocardiography.

Treatment Steps
1. Surgical correction, optimal time 4–8 years of age.
2. Bacterial endocarditis prophylaxis.

Front 56

Symptoms
Dull ache, feeling of leg heaviness relieved by elevation. Dilated
veins along anatomic distribution of greater and lesser saphenous
veins. May be accompanied by signs of chronic deep venous disease.

Back 56

Varicose Veins

Description
Dilated, tortuous veins in the leg.
Primary—Normal deep system.
Secondary—Diseased deep system.

Hint 56

Diagnosis
Clinical examination, Trendelenburg test, duplex scan, Perthes’ test.

Treatment Steps
1. Support hose.
2. Sclerotherapy.
3. Ligation and stripping.

Front 57

Symptoms
Polycythemia, retardation of growth, exercise intolerance. Charac-
teristic “squat maneuver” after exercise.
On exam, prominent right ventricular impulse, systolic ejection
murmur with thrill at left sternal border, single S2, cyanosis, club-
bing.

Back 57

Tetralogy of Fallot

Description and Pathology
Most common cyanotic congenital heart lesion in patients older
than 1 year of age. Consists of:
1. VSD (usually large).
2. Narrowing of right ventricular outflow tract (usually infundibular,
right ventricular airflow tract obstruction).
3. Overriding of aorta above VSD (aka dextroposition of aorta).
4. Right ventricular hypertrophy.
The result of this complex of abnormalities is a right-to-left shunt
at the ventricular level and reduced blood flow to the lungs. About
25% of patients have a right-sided aortic arch.
Squat maneuver”: Patient
squatting increases
systemic resistance,
therefore decreasing right-
to-left shunt.

Hint 57

Diagnosis
History, exam. Chest x-ray with decreased pulmonary vasculature,
boot-shaped heart (“coeur en sabot”). Doppler echocardiography.
The ECG with RVH. Cardiac catheterization confirms diagnosis and
defines level and severity of RV outflow obstruction.

Treatment Steps
1. Surgical corrections, either palliative procedure followed by de-
finitive operation at a later time or initial total surgical correc-
tion.
2. Bacterial endocarditis prophylaxis.
3. Treat arrhythmias (can occur before or after surgical correction)
as required.

Front 58

Description
Inflammation and thrombosis of vein, pain, swelling, warmth.

Back 58

Superficial Thrombophlebitis
Mondor disease is superficial thrombophlebitis of the thoracoepigastric veins of the anterior chest wall or breast. Migratory thrombophlebitis is associated with pancreatic carcinoma.

Hint 58

Treat with hot compresses, leg elevation, analgesics, excision of vein if purulent, systemic anticoagulation if process extends to deep venous system, or ascending superficial thigh.

Front 59

Symptoms
Depends on severity and complexity of lesion. The complete endo-
cardial cushion defect (common AV canal) usually presents in in-
fancy with weight loss, CHF, recurrent pulmonary infections. Incom-
plete defects (e.g., ostium primum ASD) similar in presentation to
more common ostium secundum ASD.

Back 59

Endocardial Cushion Defect

Description and Pathology
Persistent opening in lower atrial and upper ventricular septa associ-
ated with developmental abnormalities of tricuspid and/or mitral
valves, caused by failure of the endocardial cushions to fuse properly
during development. Spectrum of defect ranges from ostium pri-
mum ASD only (usually with some abnormality of mitral valve) to a
complete atrioventricular canal defect with a common ASD/VSD
and a common atrioventricular valve. The complete defect is associ-
ated with Down syndrome and mental retardation.

Hint 59

Diagnosis
History, exam. Doppler echocardiography establishes diagnosis and
extent of defect. The ECG with first-degree AV block, left axis devia-
tion. Chest x-ray with cardiomegaly, increased pulmonary vasculature steps CHF in severe forms.

Treatment Steps
1. Depends on extent and complexity of defect.
2. Surgical repair for “partial” or “incomplete” defect (ASD of pri-
mum septum) often occurs in childhood or young adulthood.
Surgical repair for some patients with complete defect.
3. Bacterial endocarditis prophylaxis.

Front 60

Symptoms
Often asymptomatic. Headache.

Diagnosis
The Seventh Report of the Joint National Committe on Prevention,
Detection, Evaluation, and Treatment of High Blood Pressure
(www.nhlbi.nih.gov) recommends the following classification of
blood pressure (BP) for adults > 17 years:
Blood Pressure Category SBP (mm Hg) DBP (mm Hg)
Normal < 120 ≤ 80
Prehypertension 120–139 80–89
Hypertension Stage I 140–159 90–99
Hypertension Stage II ≥ 160 ≥ 100
SBP = systolic blood pressure, DBP = diastolic blood pressure
Diagnostic workup should include: History and physical (assess-
ing risk factors and comorbidities, revealing identifiable causes of
hypertension, assessing presence of end-organ damage) (see below).
Labs: urinalysis, blood glucose, hematocrit, lipid panel, potassium,
calcium, creatinine. Urinary albumin/creatinine ratio is optional.
ECG.
Identifiable causes of hypertension (see also section VIII.B):
Sleep apnea, drugs/medications, chronic renal disease, primary hy-
peraldosteronism, renovascular disease, Cushing’s syndrome or
chronic steroid therapy, pheochromocytoma, coarcation of aorta,
thyroid or parathyroid disease.

Back 60

Primary (Essential, Idiopathic) Hypertension

Description
Accounts for about 90% of cases of systemic hypertension, usual on-
set 25–55 years of age. Many with family history of hypertension or
atherosclerotic disease. Often found in patients with other cardiovas-
cular risk factors. More common in blacks (20–30% of blacks) and patients with diabetes mellitus
Chronic licorice ingestion
can cause hypertension.

Hint 60

Treatment Steps
1. Lifestyle modifications include weight reduction, DASH diet,
sodium restriction, physical activity (aerobic), and moderation of
alcohol intake.
2. Multiple medications exist, including thiazides, β-blockers, ACE
inhibitors, calcium channel blockers, aldosterone antagonists,
and angiotension receptor blockers. Select the drug(s) most ap-
propriate for the patients (see Table 1–2).

Front 61

Symptoms
Similar to essential hypertension, except may have signs and symptoms related to specific cause of 2nd hypertension

Back 61

Secondary Hypertension

Description
Elevated blood pressure associated with a documented condition known to cause hypertension. Accounts for about 10% of all cases of hypertension. Can occur at any age, but suspect if patient < 25 years or > 60 years of age.
• HYPERALDOSTERONISM
• PHEOCHROMOCYTOMA
• RENAL ARTERY STENOSIS
• CUSHING’S SYNDROME OR DISEASE

Hint 61

Diagnosis
Pay close attention to symptoms and signs of secondary causes. Eliminate medications that can raise blood pressure, including oral contraceptives, illicit drugs, herbs, steroids, over-the-counter cold preparations with phenylephrine or pseudoephedrine, and all NSAIDs.
CAUSES AND TESTS FOR SECONDARY HYPERTENSION
HYPERALDOSTERONISM
• Serum chemistries (hypoklemic metabolic alkalosis)
• PRA ratio (random plasma aldosterone: plasma renin)
• Captopril-suppression test
• Serum aldosterone level
• 24-hour urinary aldosterone excretion
• Salt loading test
PHEOCHROMOCYTOMA
• 24-hour urine collection for creatinine, total catecholamines, vanillylmandelic acid (VMA),and metanephrines
• MRI to visualize adrenal tumors
• Scanning with iodine-131–labeled metaiodobenzylguanidine (MIBG) (reserved for cases when pheochromocytoma confirmed biochemically but CT/MRI fail to visualize a tumor)
RENAL ARTERY STENOSIS
• Renal ultrasound with dopplers
• Radionuclide scanning with captopril
• CT or MR guided angiography
CUSHING’S SYNDROME OR DISEASE
• Overnight dexamethasone suppression test
• 24-hour urine for free cortisol

Treatment
1. Eliminate secondary cause, if possible. Surgical (e.g., surgery or stenting for renal artery stenosis, surgery for pheochromocytoma) possible in many cases.
2. Antihypertensive therapy if specific approach to correct secondary cause impossible or not completely effective.

Front 62

Symptoms
Eyeground hemorrhages. May have focal or nonfocal central nervous system (CNS) signs. If CHF present, pulmonary congestion and cardiac gallops. Cardiac ischemia (angina).

Back 62

Accelerated (Malignant) Hypertension

Description
Abrupt worsening of systemic arterial hypertension with diastolic pressure > 120 mm Hg associated with neurologic dysfunction (severe headache, cerebrovascular accident (CVA), visual disturbances, papilledema, convulsions), acute renal failure (with or without chronic renal insufficiency), or cardiac dysfunction (acute MI, pulmonary edema).

Hint 62

Diagnosis
Diastolic arterial pressure ≥ 120 mm Hg associated with acute and severe manifestations of cardiac, neurologic, or renal dysfunction.
ECG with LVH, may show acute cardiac ischemia.
Chest x-ray (CXR) may show cardiomegaly and/or CHF

Treatment Steps
1. Prompt control of high blood pressure to moderate range with sodium nitroprusside, trimethaphan, nicardipine, hydralazine, labetalol.
2. Adjunctive use of β-blocker and/or diuretic.
3. Switch to oral antihypertensive agents when stable.

Front 63

Symptoms
Signs of poor tissue perfusion (altered mental status, cool extremities, oliguria, metabolic acidosis) associated with systemic arterial hypotension.

Back 63

Systemic Hypotension with Shock

Description
Hypotension is usually defined <90 mm Hg systolic in adults.
Shock is said to exist when hypotension is accompanied by signs and symptoms of poor tissue perfusion. Shock can exist with systemic arterial pressure above 90 mm Hg systolic in some cases.

Pathology
Normal systemic arterial pressure is maintained by an interplay be-
tween cardiac output and SVR. A decline in one factor that cannot
be compensated for by an increase in the other will cause systemic hypotension. Furthermore, at Cardiac Index under approximately
2.0 L/min per square meter of body surface area (BSA), peripheral
perfusion will be inadequate, no matter how high the SVR.
Hypovolemia—Causes systemic hypotension by reducing venous
return to the heart, thereby reducing cardiac output. Hypovolemia
can be absolute (e.g., hemorrhage, dehydration) or can be relative
(e.g., anaphylactoid reaction, sepsis).
Cardiogenic—Cause of systemic hypotension is due to an inade-
quate output of the heart under conditions of adequate venous return
(i.e., hypovolemia not present). Inadequate cardiac output under
these conditions can be due to severe mechanical (valvular or pericar-
dial) or myocardial (e.g., cardiomyopathy, acute MI) dysfunction.
Severe pulmonary artery obstruction (e.g., severe pulmonary em-
bolism, severe pulmonary vasculature disease) can uncommonly be
a cause of hypotension by causing restriction of left heart filling.

Hint 63

Diagnosis
History, exam. Hypotension with symptoms and signs of poor tissue perfusion. History helps to distinguish hypovolemic from cardiogenic etiology. Neck vein distention and pulsus paradoxicus clues to possible cardiac tamponade

Treatment Steps
Hypovolemic Shock
1. Intravenous fluids (crystalloids, blood products if appropriate).
2. Trendelenburg position for optimal venous return to the central circulation.
3. If pressors (e.g., dopamine, norepinephrine, epinephrine) used, only temporary measure until intravascular volume is restored.
4. Treat underlying cause.
Cardiogenic Shock
1. Inotropic agents (dobutamine, dopamine, amrinone). Vasodilator if tolerated.
2. Intra-aortic balloon counterpulsation.
3. Pericardiocentesis for cardiac tamponade.
4. Treat underlying cause.

Front 64

Description
Bluish, dusky discoloration of skin and mucous membranes.

Back 64

Cyanosis

Symptoms
Central cyanosis is apparent on skin and warm mucous membranes. Peripheral cyanosis is apparent on skin in exposed areas, where vasoconstriction may be present, and may not be visible on warm mucous membranes

Pathology
Due to an excess (> 4 g/dL) of reduced hemoglobin in capillary blood.
Central Cyanosis—Caused by abnormally high amount of reduced hemoglobin in arterial blood due to right-to-left intracardiac shunt (usually congenital heart disease) or patent Foramen ovale or to inability of severely diseased lung parenchyma to adequately oxygenate systemic venous blood. Methemoglobinemia.
Peripheral Cyanosis—Caused by excess removal of oxygen from normally saturated blood during abnormally slow flow through capillary bed during shock or cold exposure.

Hint 64

Treatment Steps
Treat underlying condition.

Front 65

ATHEROSCLEROSIS AND LIPOPROTEINS/
HYPERLIPIDEMIA

Description
Although there are a number of different genetic diseases that cause
abnormal lipid profiles, the majority of people have more of an ac-
quired dyslipidemia from a poor diet. The two major concerns from
a cardiovascular risk standpoint are an elevated LDL and a de-
pressed HDL. In general, diet is recommended as a means to correct
the LDL and HDL. Depending on the clinical scenario, however,
drug therapy may also be initiated early (see Table 1–4).
The NCEP (National Cholesterol Education Program) has made
recommendations on specific lipid (LDL) goals for patients in terms
of the number of risk factors or the presence of coronary heart dis-
ease (CHD) they have.
Major cardiovascular risk factors (exclusive of LDL levels):
• Tobacco use.
• Hypertension (140/90 or patients taking blood pressure med-
ications).
• Depressed HDL (< 40 mg/dL).
• Family history of premature CAD/CHD (in a male first-degree
relative < 55 years old or female first-degree relative < 65 years
old).
• Age: Males age ≥ 45 or females age ≥ 55.
• Note that an HDL ≥ 60 mg/dL counts as a negative risk factor.
If present, a risk factor can be subtracted.
Atherosclerotic vascular disease (e.g., peripheral vascular disease,
carotid stenosis, abdominal aortic aneurysm) and diabetes mellitus are regarded as CHD equivalents.

Back 65

• Goal LDL < 130 mg/dL
Low risk (patients with zero to one risk factor):
• Goal LDL < 160 mg/dL

Front 66

s defined by the NCEP (National Cholesterol Education Program), the metabolic syndrome is defined
by three or more of the following:
• Central obesity (measured by waist circumference):
Men ≥ 40 inches, women ≥ 35 inches
• Fasting triglycerides ≥ 150 mg/dL
• HDL cholesterol:
Men < 40 mg/dL, women < 50 mg/dL
• Blood pressure ≥ 130/85 mm Hg
• Fasting glucose ≥ 110 mg/dL

Back 66

METABOLIC SYNDROME
With an increase in obesity, the metabolic syndrome is becoming increasingly common. Patients with
the metabolic syndrome are at increased risk of cardiovascular disease and insulin resistance

Front 67

Symptoms
Claudication of lower (or, less commonly, upper) extremities distal
to the point of occlusion. Pattern of claudication useful in predict-
ing level of occlusion (buttock, thigh, sexual impotence in males [il-
iac]; calf [femoral, popliteal]). Rest pain and ulceration of skin can
occur in advanced cases.
On exam, diminished arterial pulsations, vascular bruits, and
consequence of poor arterial flow (sparse hair, ulceration) may be
seen.

Back 67

Occlusive Arterial Disease

Description
Narrowing or occlusion of large- and medium-sized arteries resulting
in reduction or total disruption of flow of oxygenated blood. Associ-
ated with hypertension, hypercholesterolemia, cigarette smoking,
male sex, diabetes mellitus.

Pathology
Atherosclerosis is most common cause of occlusive peripheral artery
disease; however, inflammation or trauma can also be a cause of arterial
occlusive disease. Embolism or acute thrombosis can result in sudden
total occlusion of an artery and is usually a medical emergency
RISK FACTORS FOR
PERIPHERAL ARTERY
DISEASE
Occlusive vascular
diseases have basically the
same risk factors as
coronary artery disease

Hint 67

Diagnosis
History, exam. Noninvasive evaluation includes measurement of an-
kle and brachial arterial pressures before and after exercise. Angiog-
raphy confirms site of obstruction and evaluates distal arterial
runoff.

Treatment Steps
1. Medical therapy (pentoxyfylline, cilastazol) may improve micro-
circulation and peripheral oxygen delivery.
2. Reconstructive arterial surgery or percutaneous angioplasty/
stenting for disabling symptoms or rest ischemia.
3. Risk factor modification (see above).
4. Avoid vasoconstricting drugs.

Front 68

Symptoms
Fever, malaise, myalgia. Headache, scalp tenderness, jaw claudication, visual symptoms.

Back 68

Giant Cell Arteritis (Temporal Arteritis)

Description
Systemic disorder with involvement of aorta and large branches (particularly carotid). Usually in patients older than 55 years of age.
Temporal arteritis is particularly worrisome because it can result in irreversible vision loss.

Pathology
Segmental granulomatous inflammation of unknown cause, resulting in necrosis of media and occlusion of arterial lumen.

Hint 68

Diagnosis
Clinical scenario. Laboratory findings of elevated sedimentation rate, abnormal liver function tests, and anemia are nonspecific.
Biopsy of involved artery (often temporal artery).

Treatment Steps
Corticosteroids, usually for months

Front 69

Symptoms
Clinical pattern determined by which organ systems involved, but
systemic hypertension with kidney involvement in vast majority.
Acute MI may occur. Other symptoms may reflect involvement of
skin, gastrointestinal (GI) tract, spleen, CNS.

Back 69

Polyarteritis

Description
Segmental vasculitis of small- to medium-sized arteries, causing dysfunction in multiple organ systems. Patients usually middle aged.

Pathology
Small- to medium-sized arteries affected by segmental necrotizing
granulomatous inflammation (varies from media only to full thick-
ness) of unknown cause.

Hint 69

Diagnosis
Clinical picture. Multisystem involvement with biopsy evidence of active arteritis.

Treatment Steps
Corticosteroids. Other therapy as indicated for specific organ dysfunction (e.g., acute MI).

Front 70

Symptoms
Many asymptomatic, especially congenital AV fistulae, which tend to
be smaller than acquired type. Venous and/or arterial insufficiency
in extremity affected. CHF due to high-output state.

Back 70

Arteriovenous Fistula

Description
Abnormal communication between artery and vein.

Pathology
May be acquired as result of trauma (blunt or penetrating) or con-
genital. Sequelae of arteriovenous (AV) fistula can be local (in-
creased venous pressure with swelling of extremity, or arterial insuffi-
ciency distal to the fistula) or systemic (high-output CHF, decreased
diastolic BP), and will depend on size of fistula and site involved

Hint 70

Diagnosis
History, exam. Thrill and bruit over fistula. Prompt decrease in
pulse rate (Nicoladoni Branham sign) when fistula is occluded. Angiography demonstrates location and size of fistula.

Treatment Steps
Surgical excision or intra-arterial embolization in most acquired cases. Congenital forms are often followed conservatively.

Front 71

Symptoms/Diagnosis
Many asymptomatic. May cause pain in legs, superficial skin ulcera-
tion, increased pigmentation

Back 71

Varicose Veins

Description
Dilated and tortuous veins of lower extremities. Most common vascular disease of the lower extremities.

Pathology
Usually a secondary condition as a sequel to deep vein thrombosis
(causing chronic deep venous insufficiency), or resulting from obe-
sity, pregnancy, ascites, right heart failure, prolonged standing.
Less commonly, a primary condition as a result of hereditary
weakness of valves and walls of veins

Hint 71

Treatment
Elastic support. May require sclerotherapy or surgical treatment
(stripping).

Front 72

Symptoms
Pain and erythema in affected area.
May cause fever and swelling. Embolism rare

Back 72

Superficial Thrombophlebitis

Description
Occlusion of a vein by thrombus with variable amount of inflammation of vein wall. Can occur in superficial veins (varicose or nonvaricose) or in deep venous system (iliofemoral, femoropopliteal, calf, axillary–subclavian). Predisposing factors include CHF, obesity, MI, trauma, postoperative state, malignancy, pregnancy, oral contraceptives.

Hint 72

Diagnosis—Exam, history.

Treatment Steps
Superficial Thrombophlebitis
1. Warm moist packs. Elevation of limb.
2. NSAIDs.
3. In persistent or recurrent cases, anticoagulation and possible surgery.

Front 73

Symptoms
Pain, swelling, fever. May
cause increased superficial venous pattern around site of deep
obstruction. Pulmonary embolism is common.

Back 73

Deep Vein Thrombophlebitis (DVT)
Thrombophlebitis

Description
Occlusion of a vein by thrombus with variable amount of inflamma-
tion of vein wall. Can occur in superficial veins (varicose or nonvari-
cose) or in deep venous system (iliofemoral, femoropopliteal, calf, axillary–subclavian). Predisposing factors include CHF, obesity, MI, trauma, postoperative state, malignancy, pregnancy, oral contraceptives.

Hint 73

Diagnosis
Deep Vein Thrombophlebitis—Exam, history (especially regarding predisposing conditions). Impedance plethysmography and Doppler
flow studies very helpful in documenting thrombosis proximal to calf veins. Contrast venography rarely needed.

Treatment Steps
Deep Vein Thrombophlebitis
1. Full-dose anticoagulation with heparin (unfractionated or low
molecular weight) followed by oral warfarin for 3–6 months.
2. If anticoagulation cannot be used, consider inferior vena cava
(IVC) filter device (commonly used Greenfield filter).
3. Prevention with subcutaneous heparin (unfractionated or low
molecular weight), warfarin, or calf compression devices dur-
ing periods of high risk for DVT.

Front 74

Symptoms
Sharp, knifelike chest pain of sudden onset, often felt initially substernally
(ascending aortic dissection) but may then radiate to neck
and interscapular area as dissection progresses distally. Pain of descending
thoracic aorta dissection may be felt only in interscapular
area. Acute aortic regurgitation, acute myocardial infarction, hemopericardium
with cardiac tamponade, paraplegia (occlusion of spinal arteries); CVA can occur as a consequence of destruction by the
plane of dissection.

Back 74

Dissecting Aneurysm of Aorta

Description
Rupture of blood into the media of the aortic wall followed by dissection
in circumferential and longitudinal fashion. Severe clinical
consequences. Usually presents acutely (but may be chronic). Associated
with hypertension, Marfan’s syndrome, pregnancy, coarctation
of the aorta, trauma (usually blunt). Men more commonly affected
than women. Peak incidence 35–70 years of age.
Three anatomic patterns of aortic dissection have been described:
Type I—Dissection begins in proximal aorta and extends distally
for variable distance.
Type II—Dissection limited to aortic arch.
Type III—Dissection begins distal to left subclavian artery and extends
distally for variable distance.

Pathology
Separation of elements of media layer of aortic wall by an intramural
hematoma, which gains access to the media through a tear in aortic
intima. Occasionally, intramural hematoma may develop without intimal
tear. Underlying condition in most cases appears to be cystic
medial necrosis. The intramural hematoma may or may not exit into
the true aortic lumen distally.

Hint 74

Diagnosis
History, exam. May have unequal blood pressure in upper or lower
extremities, acute aortic regurgitation, cardiac tamponade, CHF.
Aortography generally confirms diagnosis, but transesophageal
echocardiography (TEE), computed axial tomography (CT scan),
and magnetic resonance imaging (MRI) also highly accurate in detecting
aortic dissection.

Treatment Steps
1. Severe clinical consequences with grave outcome probable if not
detected and treated promptly.
2. Immediate control of BP, if hypertensive, with fast-acting parenteral
agents (e.g., sodium nitroprusside, trimethaphan, nicardipine).
3. β-Blocker (IV) to reduce shear forces in ascending aorta.
4. Emergency surgical treatment of types I and II.
5. Medical therapy of type III unless complications develop.

Front 75

Symptoms
Depends on location and severity. May be asymptomatic. Dilatation
of aortic root may lead to loss of aortic valve support and aortic regurgitation
and CHF. Compression of adjacent structures can lead to
symptoms of chest pain, hoarseness, dysphagia. With severely dilated
aneurysms, rupture with fatal hemorrhage is a constant threat.

Back 75

Aneurysm of the Thoracic
Aorta (Nondissecting)

Description
Significant dilatation of one or more segments of thoracic aorta.
Most commonly involves ascending aorta from root to origin of innominate
artery. May involve transverse arch segment alone.
Aneurysm of the descending thoracic aorta may extend into the upper
abdominal aorta (thoracoabdominal aortic aneurysm), and is often
associated with significant hypertension.

Pathology
Atherosclerosis is the most common cause. Cystic medial necrosis
may be seen in some aortic aneurysms, especially those of ascending
aorta. Trauma, syphilis, aortitis less common causes. Marfan syndrome.

Hint 75

Diagnosis
History, exam. Dilatation of the aorta will be apparent on chest x-ray.
Conventional contrast or magnetic resonance aortography is required
for anatomic localization of aneurysm if surgery is contemplated.

Treatment Steps
Surgical resection with graft replacement for severe aneurysms, especially
with significant aortic regurgitation (may need aortic valve replacement),
rupture or impending rupture, local compression syndromes.

Front 76

HEART TRANSPLANTATION

Description
Homograft cardiac orthotopic transplantation in carefully selected patients with life-threatening, severely symptomatic, and otherwise inoperable myocardial, valvular, coronary artery, or congenital heart disease. In some cardiac patients with severe pulmonary vascular disease (most commonly congenital heart patients), a heart–lung transplant is required

Back 76

Clinical Aspects of Posttransplantation Care
Infection with a variety of organisms and rejection are major causes of morbidity and mortality after cardiac transplantation, which requires chronic immunosuppressive therapy.
Clinical signs of rejection (CHF, gallop rhythm, low QRS voltage on ECG) are insensitive, and the best method for evaluating acute rejection is right ventricular
endomyocardial biopsy.
Graft coronary atherosclerosis, probably a form of chronic rejection, is a common problem. The transplanted cardiac patient will not experience angina because the transplanted heart is denervated.

Front 77

Symptoms
Loss of consciousness (usually abrupt) or severe lethargy. May be
preceded by chest pain, palpitations, dyspnea. Must be distinguished
from other causes of loss of consciousness or syncope (e.g., neurologic,
hemorrhagic, metabolic, toxic).

Back 77

CARDIOPULMONARY ARREST

Description
Cessation of effective cardiac and/or pulmonary function, resulting
in hemodynamic collapse or severe cerebral dysfunction. Spontaneous
cardiopulmonary arrest most often of cardiac etiology in
adults (usually ventricular fibrillation due to CAD) and of pulmonary
etiology in children.

Hint 77

Diagnosis
Pulselessness (or ineffective very rapid or very slow pulse) in large artery of unresponsive victim, absence of effective spontaneous respiration.

Treatment Steps
Basic Life Support (BLS)
• Open airway.
• Ventilate effectively—breathing.
• Support circulation with external cardiac compression.
Assess effectiveness of BLS with evaluation of pupillary reactivity
and palpation of pulsation in large artery. Continue until advanced cardiac
life support available.
Advanced Cardiac Life Support (ACLS)
• Maintain airway and adequate ventilation with adjunctive
equipment (mask, endotracheal tube).
• Arrhythmia recognition (ECG) and appropriate treatment
(electrical and pharmacologic).
• Intravenous access.
Often most important aspect of ACLS is prompt defibrillation
for ventricular fibrillation (most common cause of cardiopulmonary
arrest in adults). The use of portable automated external defibrillators
(AEDs) has become much more common in schools, public
venues.