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356 CHAPTER 8: The Chest: Chest Wall, Pulmonary, and Cardiovascular Systems; The Breasts
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FIG. 8-45 Contribution of Myocardial Hypertrophy to the Area of Cardiac Dullness. Without chamber
dilatation, concentric cardiac muscle hypertrophy to twice its normal thickness and weight does not increase the area of
cardiac dullness more than the width of the percussing finger. Therefore, after pericardial effusion is excluded, increased area
of dullness must be attributed to dilatation.
Ventricular Hypertrophy: Pulmonic stenosis, ASD, pulmonary hypertension,
hypertrophic cardiomyopathy.
Left ventricular congestive heart failure (CHF).
of impaired LV contractility (systolic dysfunction) and/or decreased LV diastolic compliance (diastolic dysfunction). Decreased LV contractility leads to
increased left ventricular end-diastolic pressure and volume, dilating the LV
to maintain stroke volume at the expense of left atrial hypertension and pulmonary vein distention. The ventricular ejection fraction is preserved when
congestion is principally caused by decreased diastolic compliance, i.e., diastolic dysfunction. Decreased cardiac output with renal hypoperfusion leads
to salt and water retention, weight gain, and edema. Symptoms and signs
are attributable to decreased cardiac output and volume expansion with pulmonary and peripheral vascular congestion.
are pulmonary congestion with dyspnea, orthopnea, nocturia, and cough.
Crackles are heard in the lung bases. When venous capacitance is exceeded,
congestion progresses to right ventricular failure with elevated CVP engorging the jugular veins. Even before the increase in CVP, a hepatojugular reux
sign can be demonstrated. Frequently an S3 develops. Arterial pressure
response to the Valsalva maneuver correlates inversely with the left ventricular lling pressure: absence of the normal systolic pressure “overshoot”
following release of the Valsalva, and failure of the BP to drop during the
breath hold, suggest increased LV lling pressure. With biventricular failure,
the liver can become large, tender, and painful. Chronic congestive hepatomegaly may produce capsular and parenchymal brosis without tenderness.
Edema accumulates as right-sided or bilateral hydrothorax, ascites, and pitting edema of the ankles, legs, genitals, and abdomen. The lips, ears, and
nail beds may be cyanotic. Impaired cerebral circulation may result in confusion and periodic breathing. The most common causes of CHF are chronic
ischemic heart disease followed by dilated cardiomyopathy (pregnancy and
postpartum, alcohol, hemochromatosis, idiopathic, secondary to viral infections), especially in younger patients. Diastolic heart failure is common in
longstanding hypertension with LVH and hypertrophic cardiomyopathies.
Restrictive cardiomyopathy results from amyloidosis, hemochromatosis, sarcoidosis, and other inltrative diseases. DDX: Portal hypertension similarly,
CHF is the functional result
Early symptoms of LV failure
Chest, Cardiovascular, and Respiratory Syndromes 357
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but without orthopnea or elevated jugular venous pressure. Metastatic carcinoma can produce ascites and chest effusions resembling cardiac failure.
CHF is staged according to the American College of Cardiology/
American Heart Association staging system:
Stage A: Patients at substantial risk for heart failure but without struc-
tural heart disease or symptoms of heart failure.
Stage B: Patients with structural heart disease but without signs or symp-
toms of heart failure.
Stage C: Patients with structural heart disease with prior or current symp-
toms of heart disease.
Stage D: Patients with refractory heart failure (symptoms at rest despite
maximal medical therapy) requiring specialized intervention.
Once a patient is assigned to a heart failure stage, he/she cannot be reclassied
to a lower stage, e.g., reverting from Stage C to Stage B. This contrasts with the New
York Heart Association and Canadian Cardiovascular Society classications where
patients move from class to class as symptoms change.
Right ventricular failure and cor pulmonale.
from myocardial damage and RV volume and/or pressure overload. Right
and left ventricular function are interdependent, so primary LV failure is
often accompanied by some degree of RV failure.
the liver enlarges, and above 25 cm ascites, edema, and orthopnea appear.
Venous pressure is always high with RV failure, but it falls before other signs
of failure resolve. The most frequent cause of right ventricular failure is advanced ischemic heart disease. Cor pulmonale is right ventricular failure due
to pulmonary hypertension from primary pulmonary disease, either hypoxia
induced vasoconstriction or obliteration of the pulmonary vascular bed.
CLINICAL OCCURRENCE: Impaired Myocardial Function: Ischemic heart
disease (especially right ventricular infarction), hypertrophic and dilated cardiomyopathies, endomyocardial brosis (e.g., drugs, carcinoid syndrome),
restrictive myocardial disease (e.g., amyloidosis); Volume Overload: TR, ASD
with left-to-right shunt, pulmonic insufciency; Pressure Overload: Primary
pulmonary hypertension, secondary pulmonary hypertension—cor pulmonale (hypoxia caused by emphysema, cystic brosis, interstitial pulmonary
diseases, pneumoconioses, hypersensitivity pneumonitis, pulmonary brosis, etc.), acute pulmonary embolus, chronic thromboembolic pulmonary
hypertension, pulmonic stenosis, Eisenmenger complex, mitral stenosis, pulmonary venoocclusive disease.
Restrictive cardiomyopathy. Decreased diastolic compliance impedes ventric-
ular lling leading to elevated ventricular end-diastolic pressure and reduced
cardiac output. Systolic function is preserved. Patients present with dyspnea
exacerbated by exertion and intermittent pulmonary congestion. Signs of right
ventricular failure with edema, elevated CVP, and ascites may predominate.
The heart is not enlarged. An S4 gallops is common. Causes are ventricular
diastolic dysfunction, inltrative heart disease (amyloid, sarcoid, hemochromatosis), and endomyocardial broelastosis. DDX: This must be distinguished
from hypertrophic cardiomyopathy and constrictive pericarditis.
Right ventricular failure results
When the CVP >22 cm,
Hypertrophic cardiomyopathy. Inherited defects of myocardial contrac-
tile proteins lead to progressive hypertrophy, disorganized myocardial
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architecture, impaired diastolic relaxation, ventricular conduction abnormalities, and dysrhythmias. Asymmetric septal hypertrophy can produce dynamic obstruction to LV outow during early systole. A family history of sudden
death is common. Patients present with dyspnea, angina, and presyncope.
Carotid upstrokes are brisk and may show a bisferiens pattern. An S4 is common. A characteristic murmur augmenting with Valsalva is heard with outow obstruction (see Hypertrophic Obstructive Cardiomyopathy, page 327).
Echocardiography is diagnostic.
Infective endocarditis.
tions harboring the organism. Low virulence organisms, e.g., viridans streptococci, and HACEK organisms, have subacute presentations. High virulence
organisms, e.g., Staphylococcus aureus, and fungi, rapidly destroy valves
and so present acutely, with or without systemic emboli. Subacute Bacterial
Endocarditis (SBE): Patients present with subacute or chronic fever, weight
loss, arthralgia, myalgias, and signs of immune complex disease. Acute
Endocarditis: Patients, often with a history of injection drug use, have fever
and rigors. Peripheral emboli with organ infarction and metastatic infection
are common. Systemic illness, especially with a new insufciency murmur,
should always trigger an endocarditis evaluation. The clinical diagnosis of
infective endocarditis relies on the presence of positive blood cultures, echocardiographic ndings consistent with endocarditis as well as associated
ndings such as peripheral embolic disease, vascular embolic disease, and
immunologic phenomena such as glomerulonephritis, Osler’s nodes and
Roth’s spots.
Nonbacterial thrombotic endocarditis (NBTE). Nonbacterial thrombotic
endocarditis is the result of endocardial inammation with sterile vegetations.
Multiple large emboli suggest marantic endocarditis associated with occult
neoplasms, most often a mucin-secreting adenocarcinoma. Libman–Sacks
lesions (small vegetations composed of brin, lymphocytes, neutrophils and
histiocytes) occur on the valves and endocardium of patients with SLE and
antiphospholipid syndrome. Patients may be asymptomatic, have peripheral
emboli, or present with progressive valve stenosis or regurgitation.
Valvular Heart Disease: These clinical conditions are discussed with their
physical ndings under the sections Cardiovascular Signs, page 313 and
Auscultation of Heart Murmurs, page 282.
Heart valve infection leads to brin-platelet vegeta-
Congenital Heart Disease: Many patients with congenital heart disease sur-
vive into well into adult life. All clinicians should be familiar with the common syndromes. ASDs and small VSDs may be asymptomatic for decades,
eluding diagnosis well into adult life.
Atrial septal defect. See page 329.
Ventricular septal defect. See page 329.
Persistent ductus arteriosus. See page 333.
Coarctation of the aorta. See pages 329 and 368.
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Eisenmenger syndrome. An intra- or extra-cardiac left-to-right shunt con-
necting the pulmonary and arterial circul ations creates chronic volume and/
or pressure overload of the right ventricle and pulmonary arteries leading to
severe pulmonary hypertension and, if left untreated, reversal of ow into
a right-to-left shunt with peripheral hypoxemia and cyanosis. The RV and
PA respond with hypertrophy and bromuscular hyperplasia, respectively.
Suspect Eisenmenger physiology when valve signs are coupled with cyanosis, decreased oxygen saturation not relieved with 100% oxygen, right-to-left
shunt, clubbing of the ngers and/or toes, and polycythemia.
Tetralogy of fallot. The tetralogy is the combination of a VSD, obstruction to
right ventricular outow (usually infundibular pulmonic stenosis), an overriding aorta, and right ventricular hypertrophy (Fig. 8-41D, page 326).
Ebstein anomaly. The congenitally abnormal tricuspid valve, with small thin
cusps and a portion originating below the AV ring, produces an atrialized
portion of the right ventricle. Tricuspid insufciency is not prominent; dysrhythmias are common.
Sudden cardiac death—cardiac arrest. Cardiac arrest demands immedi-
ate treatment for any chance of survival. For 25% of patients, sudden
cardiac death is the rst symptom of severe CAD. Less common causes
are hypertrophic cardiomyopathy (especially in young male athletes),
coronary artery emboli, right ventricular dysplasia, Brugada syndrome,
long-QT syndrome, and anomalous coronary artery anatomy. Public
education seeks to teach all adults in basic cardiopulmonary resuscita-
tion. Increasingly, automated debrillators are present in public places
to reverse fatal ventricular arrhythmias. All health care personnel should
be trained in basic cardiopulmonary resuscitation (BCLS). Nurses and
physicians should be trained in advanced cardiac life support (ACLS).
Mediastinal Tumors: Mediastinal masses rarely cause chest pain. Most attract
attention by compressing normal structures or are found incidentally on chest
X-ray. Signs suggesting a mediastinal tumor are dyspnea (retrosternal goiter),
hoarseness and brassy cough (recurrent laryngeal nerve compression), Horner
syndrome (superior cervical ganglion injury, Chapter 14, page 665), edema
and cyanosis of the arms and neck (SVC obstruction), and chylous pleural effusion. Lymph nodes are enlarged in Hodgkin disease, non-Hodgkin
lymphoma, carcinoma, germ cell tumors, or tuberculosis. Other masses are
thymomas and teratomas (dermoid cyst). Dermoid cysts forming a tracheal
stula may produce the rare symptom of trichoptysis, coughing up of hair.
Pericardial Syndromes
Acute pericarditis. See page 352.
Postpericardiotomy syndrome. See page 353.
Pericardial effusion. Fluid accumulates within the pericardial sac because
of infection, inammation, malignancy, or transudation. Slowly accumulating uid distends the pericardium compressing surrounding lung. Rapidly
accumulating uid is more likely to compress the heart chambers producing
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tamponade. Patients are asymptomatic or symptoms reect the etiology (e.g.,
pain and fever with pericarditis; weakness, nausea and anorexia with uremia)
or the hemodynamic consequences (e.g., shortness of breath and fatigue with
tamponade). Signs include absent precordial impulse, decreased intensity
and/or mufing of heart sounds, and low-voltage ECG. A rub may be present. Large effusions can cause dullness at the left scapular tip because of left
lower lobe atelectasis (Ewart sign).
CLINICAL OCCURRENCE: Congenital: Familial Mediterranean fever, familial
pericarditis; Endocrine: Hypothyroidism; Degenerative/Idiopathic: Sarcoidosis;
Infectious: Bacterial, viral, tuberculosis, fungal, Whipple Disease; Inammatory/
Immune: Post-pericardiotomy, rheumatic fever, SLE, RA, ankylosing spondyli-
tis, scleroderma, granulomatosis with polyangiitis, drug reactions; Mechanical/
Traumatic: Trauma, post-irradiation; Metabolic/Toxic: Drug-induced, uremia;
Neoplastic: Metastatic carcinoma, especially lung and breast, lymphoma;
Vascular: MI, aortic dissection with rupture into the pericardium, chyloperi-
cardium, vasculitis.
Constrictive pericarditis. Progressive pericardial brosis restricts diastolic
lling decreasing cardiac output. Symptoms are shortness of breath and
fatigue. Signs are those of right heart failure: jugular venous distention,
edema, ascites, and hepatic congestion. The initial pericardial injury is caused
by pericarditis (viral, tuberculous, neoplasm), cardiac surgery, mediastinal
irradiation, or uremia.
myopathies and right ventricular failure.
DDX: It must be distinguished from restrictive cardio-
Pericardial tamponade.
compresses the heart impairing diastolic atrial and ventricular lling. Cardiac
output falls and CVP rises. Patient are short of breath and fatigued and may
rapidly progress to hypotension and circulatory collapse. The key physical
signs are an elevated CVP with clear lung elds, no stigmata of chronic right
ventricular failure, and a drop in systolic blood pressure during inspiration
of >10 mm Hg (see pulsus paradoxus, page 335 and Fig. 8-42, page 327).
Pulsus paradoxus may not be found in the presence of aortic insufciency,
left or right ventricular hypertrophy, or pulmonary hypertension. The heart
size is usually normal by exam and chest X-ray. Suspected tamponade should
be urgently evaluated by echocardiography.
ponade are acute PE, right ventricular infarction, constrictive pericarditis,
and restrictive cardiomyopathy.
Disorders of the Arterial and Venous Circulations.
Vasculitis. Large vessel vasculitides are of unknown etiology. Vasculitis of
medium-sized arteries may be associated with infection (polyarteritis nodosa
with hepatitis B and C) or specic immunologic markers (granulomatosis
with polyangiitis with c-ANCA). Small-vessel vasculitides are associated
with immune complex deposition in the vessel walls. In some cases, the
association with a specic infection is strong (e.g., mixed cryoglobulinemia
and chronic hepatitis C infection); in others there is a strong association
with serologic markers (e.g., microscopic polyangiitis and p-ANCA). Each
involves vessel wall inammation leading to vascular obstruction and endorgan damage. The current working classication for vasculitis syndromes
Rapid or massive pericardial uid accumulation
DDX: Easily confused with tam-
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TABLE 8-3Systemic Vasculitis Syndromes.
Size of the Vessel Specific Diseases
Large arteries GCA
Takayasu arteritis
Primary central nervous system vasculitis
a
Medium arteries
Small vessels Leukocytoclastic vasculitis (e.g., Henoch–Schonlein purpura,
Pseudovasculitis
a
May involve small vessels as well.
b
May involve vessels of any size.
From Coblyn JS, McCluskey RT. Case 3-2003: case records of the Massachusetts General Hospital. A 36-yearold man with renal failure, hypertension, and neurologic abnormalities. N Engl J Med. 2003;348:333–342.
Polyarteritis nodosa
Kawasaki disease
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome)
Granulomatosis with polyangiitis (Wegener granulomatosis)
cryoglobulinemia, infections, drugs)
Microscopic polyangiitis
Urticarial vasculitis
Systemic rheumatic syndromes
b
Antiphospholipid syndrome
Embolic phenomena (atrial myxomas, cholesterol, NBTE/Libman–Sacks
endocarditis)
Bacterial endocarditis
Endovascular lymphoma
has proven helpful for selecting appropriate treatment. Vasculitides are classied by the size of the involved vessel, Table 8-3. The clinical manifestations
depend upon the size and distribution of the vessels involved. Symptoms and
signs are due to local ischemia and systemic inammation. Skin involvement
manifests as palpable purpura with or without skin infarction. Involvement
of arteries to other organs gives signs specic to the organ affected, e.g., renal
failure, transient ischemic attacks, stroke, and pneumonitis. The pattern of
involvement suggests the size of the vessel and the specic vasculitis syndrome [Kathiresan S, Kelsey PB, Steere AC, et al. Case 14-2005: a 38-year-old
man with fever and blurred vision. N Engl J Med. 2005;352:2003–2012].
Giant cell arteritis (GCA, temporal arteritis). The cause is unknown.
Histology shows segmental patchy medial necrosis in the temporal
artery, with diffuse mononuclear inltration and giant cells throughout
the vessel wall. Thromboses are frequent. GCA affects major branches of
the proximal aorta, especially the external carotid. Patients over age 50
present with fever and weight loss and may have headache, jaw claudication, and/or scalp tenderness. Visual symptoms forebode irreversible
visual loss from retinal and ophthalmic artery occlusion. The headache is
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severe, persistent, and throbbing. Polymyalgia rheumatica (see Chapter 13,
page 608) may antedate other manifestations or occur simultaneously.
Systemic symptoms (fever, profound weakness, weight loss, malaise,
and prostration) are common and may be the only manifestations of disease. Physical signs are few, but scalp tenderness and tortuous, tender,
or nodular temporal arteries may be present. The overlying skin is often
red and swollen. Vision is often impaired and ophthalmoplegia may be
temporary or permanent. The retina may be normal or show evidence
of retinal ischemia. Prompt diagnosis and treatment can prevent loss of
vision. The typical ophthalmic nding is anterior ischemic optic neuropathy (AION, Chapter 7, page 207). The disk appears pale and swollen because the posterior ciliary arteries supplying the nerve head are
obstructed. Aortic aneurysms (thoracic and abdominal) and dissection
occur with increased frequency [Kelly NP, Gerhard-Herman M, Desai
AS, et al. An unusual cause of leg pain. N Engl J Med. 2017;377;2267-72].
Takayasu aortitis.
producing markedly reduced ow and thrombosis in the involved vessels.
Patients are usually young women presenting with arterial ischemia symptoms. Absent pulses in arm or neck vessels (pulseless disease), carotid sinus
sensitivity with head movement inducing syncope, and ocular disorders,
such as cataract and retinal defects, form a characteristic triad.
Polyarteritis nodosa (PAN). Approximately a third of patients have chron-
ic hepatitis B infection and HbSAg-HbSAb immune complexes have been
found in the vessel walls. Causes of the other cases are not known. Transmural inammation and necrosis of small and intermediate muscular arteries
sometimes extends to adjacent veins and arterioles. Involvement is segmental with a predilection for arterial bifurcations and branch points. Vascular
occlusion leads to tissue ischemia and necrosis. Aneurysms up to 1 cm in
diameter are frequent.
pain in viscera and muscles. Skin lesions are common, especially on the legs,
and subcutaneous nodules may be palpable along the course of vessels or
nerves. Frequently involved organs are the kidneys (renal failure, hypertension), gastrointestinal tract (visceral infarction), heart (MI, pericarditis), liver
(acute to chronic hepatitis), peripheral nerves (mononeuritis multiplex), skin
(subcutaneous nodules over supercial vessels, palpable purpura, livedo reticularis), joints, and muscles (myalgias, arthralgias, arthritis). The lungs are
rarely involved [Case records of the Massachusetts General Hospital. Case
3-2003. A 36-year-old man with renal failure, hypertension, and neurologic
abnormalities. N Engl J Med. 2003;348:333–342].
Granulomatosis with polyangiitis (Wegener).
lomatous inammation of small arteries and veins is strongly associated with
neutrophil anticytoplasmic antibodies to proteinase-3 (c-ANCA).
present with fever, malaise and signs of upper airway disease (sinusitis,
obstructive, and/or destructive symptoms and signs), pulmonary involvement, and/or progressive renal failure. Other organ systems, including the
skin, may be involved. Disease may be limited to the upper airway. It is most
common in the fourth and fth decades. Facial pain and epistaxis are caused
by erosion of the nose (resulting in saddle deformity), sinuses, palate, or
There is inammation of the aorta and its major branches
Symptoms include fever, weight loss, malaise, and
The cause is unknown. Granu-
Patients
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nasopharynx. The lungs may have inltrates, nodules, and cavitations. The
renal lesion is a rapidly progressive glomerulonephritis.
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome).
The cause is unknown. Eosinophilia is associated with nodular lung inltrates and asthma.
association with eosinophilia and persistent slowly evolving abnormalities
on the chest radiographs. Systemic symptoms and signs mimic polyarteritis
nodosa. It may appear during tapering of corticosteroid treatment or introduction of leukotriene inhibitor treatment of asthma [Wolf M, Rose H, Smith
RN. Case 28–2005: a 42-year-old man with weight loss, weakness, and a rash.
N Engl J Med. 2005;353:1148–1157].
Microscopic polyangiitis. The cause is unknown and immune complexes and
complement are not present in the vessel walls and granulomas do not form
(pauci-immune vasculitis). There is a high prevalence of anticytoplasmic antibodies to myeloperoxidase (p-ANCA). Patients are systemically ill with fever,
malaise, dyspnea, nonproductive cough, arthralgias, and myalgias. Acute
rapidly progressive glomerulonephritis is common and pulmonary hemorrhage can be fatal. Except for the absence of airway involvement, it clinically
is indistinguishable from granulomatosis with polyangiitis.
Leukocytoclastic vasculitis. Immune complexes lodge in the walls of termi-
nal arterioles and venules inciting an inammatory response that occludes
vessels producing local tissue infarction. This common vasculitis most fre-
quently affects the skin, particularly on the lower legs. The physical nding
is purpura which is often palpable. The lesions come in crops that clear over
days without scarring. Systemic symptoms (fever, malaise) and involvement
of visceral organs including the kidneys (glomerulonephritis), lungs, gut, and
rarely the heart and central nervous system, can occur. Precipitating events
include infection, drugs, malignancies, and primary inammatory disorders.
Several distinct syndromes are identied, but overlap is frequent.
vasculitis must be distinguished from other causes of vasculopathy including
disseminated fungal (e.g., histoplasmosis), viral (e.g., Rocky Mountain spotted fever) and bacterial infections (e.g., meningococcemia, gonococcemia),
atheroembolism, scurvy, and thrombocytopenia.
Patients present with signs and symptoms of asthma in
DDX: This
Serum sickness. This hypersensitivity vasculitis is caused by antibodies
against a widely disseminated exogenous antigen, most often penicillin. Deposition of antigen–antibody complexes in the subendothelial space elicits
local inammation.
mation at the site of subcutaneous or intramuscular injection. The urticaria
spreads, and large areas of skin may become edematous. An erythematous
rash is often present. Myalgias and arthralgias may be severe; nausea and
vomiting may occur. Generalized lymphadenopathy is frequent.
Secondary vasculitis. This is a leukocytoclastic vasculitis occurring in association with another primary disease or condition inciting immune complex
formation. Common causes are infections (endocarditis, HIV, EBV, etc.), primary inammatory diseases (e.g., SLE, RA, Sjögren syndrome and polymyositis-dermatomyositis), serum sickness, and drugs (see below).
Headache and pruritus are accompanied by wheal for-
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Idiopathic cutaneous vasculitis. This is an immune complex vasculitis limited to the skin and without an identiable inciting event or exposure. It may
be recurrent, and a burning sensation may precede the crops of purpura.
Other skin signs that may accompany the purpura include urticaria, bullae,
and erythematous macules. The lesions may itch.
Drug-induced vasculitis. Many drugs, especially the penicillins and sulfonamides, are associated with immune complex-mediated, typically cutaneous and/or urticarial, vasculitis. A p-ANCA positive small vessel vasculitis
clinically identical to microscopic polyangiitis has been described with use of
propylthiouracil, levamisole (adulterant in cocaine) and hydralazine. Druginduced TTP-HUS (Chapter 6, page 152) may be confused with vasculitis.
Henoch–Schönlein purpura (anaphylactoid purpura).
infection. There is IgA deposition and inammation in the small vessels of the
skin, gastrointestinal tract, and kidneys. Patients, usually children or young
adults, present with palpable purpura on the abdomen, buttocks and lower
extremities, abdominal pain, fever, and heme-positive stools. Proteinuria and
hematuria indicate glomerulitis. Nausea, vomiting, arthralgias, and myalgias
are common. Urticaria may be present. The condition resolves spontaneously
within a few days.
Behçet syndrome. This vasculitis of unknown cause is characterized by the
triad of relapsing iridocyclitis, oral aphthous ulcerations, and genital ulcers.
Most cases occur in Greece, Cyprus, Turkey, the Middle East, and Japan, but
increasing numbers are reported in the United States. There is a high incidence of erythema nodosum and arthritis. A characteristic sign is the formation of sterile pustules at skin puncture sites, pathergy. Many patients have
thrombophlebitis, neurologic disorders, or intestinal involvement. The disease is chronic with relapses and remissions.
Arteriovenous stula: acquired. Communication between an artery and adja-
cent vein may be created surgically to facilitate venous access for hemodialysis or be caused by a stab or gunshot wound, diagnostic catheterization, or
erosion from neoplasm or infectious arteritis. Hemorrhage after the inciting
trauma is profuse but easily controlled. A thrill and bruit may develop some
hours later. After the wound has healed, signs of chronic circulatory disturbance develop. Although stulas may occur in any body part, signs are most
evident when an extremity is involved (Fig. 8-46). Dilated veins and stasis dermatitis indicate venous congestion. Arterial hypoperfusion can produce distal
gangrene. If extremity injury occurs before the epiphyses have closed, hypertrophy of the arm or leg can occur. A thrill and bruit are present throughout
the cardiac cycle, with systolic accentuation. The skin temperature increases
distal to the stula. Paradoxically, these signs assure that an arteriovenous
shunt established surgically to facilitate venous access remains patent. When
the shunt is large, the dilated supercial veins become tense, venous pressure
approaches arterial diastolic pressure, venous ow velocity increases, the RV
dilates, arterial diastolic pressure falls, and cardiac failure may result. External
compression, temporarily closing the stula, produces a sharp slowing of the
heart rate, called the Branham bradycardiac sign. Signs of abdominal or thoracic
shunts are bruits and changes in venous and arterial pressure.
Onset often follows an
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FIG. 8-46 Signs of Arteriovenous Fistula. A. A fistula between the popliteal artery and vein is shown
behind t he knee joint. B. The superficial veins are greatly dilated from blood under arterial pressure; they are tense
to the touch and sometimes pulsatile. Distal to the fistula, the skin is warm f rom the arter ial blood in the veins, cyanotic,
and pigmented from hemostasis. Distal gangrene may occur. At the site of the fistula a thrill and bruit continuous
throughout the cardiac cycle, with systolic accentuation, may be felt. With a large-flow fistula, the arterial pulse pressure is
greater than normal. C. Closure of the fistula by digital compression slows the heart rate (Branham bradycardiac
sign) and augments both systemic systolic and diastolic arterial pressures.
Congenital arteriovenous stula. Cutaneous birthmarks are found in onehalf of cases, so arteriovenous stula should be considered when port-wine
spots, blue-red cavernous hemangiomas, or diffuse hemangiomas are present. Frequently, congenital stulas are quite small, so the signs associated
with acquired lesions are not evident: thrills and bruits may be absent and
the bradycardiac sign of Branham is less pronounced. The affected limb may
be hypertrophied, and it may exhibit increased sweating and hypertrichosis.
There is no history of trauma.
Circulatory Disorders in the Head, Neck, and Trunk: The large arteries and
veins in the head, neck, and trunk are less accessible to inspection and palpation than those in the extremities (Figs. 8-47 and 8-48A). Vascular disorders in
these regions frequently must be inferred from combinations of physical signs.
Carotid artery disease. The carotid arteries supply blood to the head and
brain. Vascular symptoms and signs in the extracranial tissues supplied by
the external carotid artery are unusual. The exception is frequent involvement by giant cell (temporal) arteritis producing jaw claudication and scalp
tenderness. The internal carotid supplies the brain and eye and is frequently
involved with atherosclerotic occlusive disease and atheroembolic events, e.g.,
amaurosis fugax and TIA. The most common sites for atherosclerotic obstruction are the carotid bifurcation, the carotid siphon, and the middle cerebral
artery. Disease of the carotid bifurcation is frequently accompanied by a bruit
audible in the neck. Carotid bruits with ipsilateral cerebral symptoms carry
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