CHAPTER XXVI

DISEASES OF THE AORTA AND GREAT ARTERIES

A) DISEASES OF THE AORTA

Etiology of aortic diseases may be primarily divided into congenital and acquired pathological
processes. However, the congenital anomalies express themselves in childhood, and the diagnosis
usually belongs to pediatric survey. By far, the most common etiology in adulthood is the
atherosclerotic involvement, responsible sometimes for dramatic life-threatening situations.

Atherosclerosis Inflammatory disorders

Hypertension Degenerative changes
Infection Trauma

Table XXVI-1: Etiology of acquired aortic diseases.

Classification:
1. Aortic aneurysms.
2. Aortic dissection aneurysm.
3. Aortic occlusive diseases:
 Atherosclerosis:
 Leriche’s syndrome.
 Martorell’s syndrome.
 Stenosis / thrombosis of the left subclavian artery.
 Chronic obstruction of the carotid bifurcation.
 Non atherosclerotic occlusive diseases (stenosing aortitis):
 Giant Cell Arteritis.
 Takayasu’s Disease.
4. Aortitis (nonstenosing diseases)
 Infectious: Syphilitic aortitis, Infectious aortitis, Rheumatic fever.
 Noninfectious: Rheumatoid arthritis, Ankylosing spondylitis, Psoriatic
arthritis, Entheropathic arthropaties, Reiter`s syndrome.
5. Aortic trauma.
6. Anulo-aortic ectazia.

TAKAYASHU – ONISHI DISEASE

(“Pulseless disease”; “Idiopathic medial aortopathy and arteriopathy”)

Incidence:
 The disease is most prevalent in young women (aged 10-30 years).
 Females / males = 8/1.

Pathology: panaortitis, characterized by:

 Adventitial thickening.
 Destroyed media by elastic tissue necrosis.
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  Proliferation and fibrosis of the intima (intimal hyperplasia).

Topographic classification: 4 types

I. Aortic and its branches involvement (8%).
II. Descending aorta (thoracic and abdominal aorta) is involved (atypical aortic coarctation).
III. I + II type
IV. I / II / III, type, involving pulmonary artery (45%).

Clinical features: the evolution passes through 2 stages:

1) Onset: early acute inflammatory process; it can last weeks to months.
Clinical and laboratory findings:
 Night sweats.
 Weight loss, fatigue, anorexia, malaise
 Arthralgias / myalgias
 Low – grade fever.
 ESR 
2) Chronic stage (occlusive phase) is characterized by complete arterial stenosis involving the origin
of vessels (+/- thrombosis). The early acute phase often go unrecognized, with the diagnosis first
being suspected when symptoms and signs of the occlusive phase develop:
 I, III – circulatory insufficiency of the upper limbs, cerebrovascular insufficiency
 II, III – systemic hypertension, mesenteric insufficiency.
 IV – right cardiac failure.
a) Neurological manifestations:
- Cranial and cervical pains:
 “Intermittent claudication of the mandibular muscles”.
 Headache.
- Dizziness, syncope.
b) Ophthalmologic disorders:
- “Visual claudication”, occurring on effort and in orthostatism (transitory
amaurosis).
- Progressive reduction of the visual acuity.
c) Psychical and intellectual disturbances: the intellectual abilities are decreased.
d) Cardiovascular disturbances:
- Diminished / absent arterial pulse on the head and superior limbs regions
compared to the inferior limbs.
- Blood pressure:
 Decreased in the superior limbs
 Normal or increased in the inferior limbs = “Inverse Coarctation”
 Systemic hypertension occurs as a result of acquired aortic coarctation or
involvement of the renal arteries by the arteritis.
- Carotid stenosis murmurs.
- Palpable arterial anastomosis in the thoracic and shoulder regions.
- Cardiac failure produced by systemic or pulmonary hypertension (type IV).
- Aortic regurgitation due to ascending aorta dilatation.
- Aortic or arterial aneurysms (20%)

Clinical positive diagnosis: bilateral palpation and auscultation of the aortic arch branches.

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Paraclinical diagnosis:
1) Laboratory findings:
-  ESR
-  Fibrinogen
-  CRP
-   globulins
-   2 globulins
- hypochromic anemia, leucocytosis, thrombocitosis
-  C 3, C4
2) Angiography (aorta, pulmonary artery): Segmental narrowing of a variable length of
descending or abdominal aorta or narrowing of the origin of major branches of the aorta
occurs as a result of thickening of the aorta or arterial wall.
3) Oscillography: comparative analysis on the superior and inferior limbs.
4) Funduscopic examination: peripapillar arteriovenous anastomosis; optic atrophy without
papilledema.

Evolution: slow progression; the overall 5 years survival rate is 83%. The death may be caused by:
 Cardiac failure
 Cerebrovascular accidents.

Differential diagnosis:

1) Giant cell arteritis (involving temporal artery). Characteristics:

 It usually affects patients > 50 years
 Pain may occur spontaneously or during palpation the temporal or occipital area.
 Renal artery is not involved.

2) Syphilitic aortitis:
 It usually occurs in males > 40 years.
 Argyll – Robertson’s neurological sign is present.
 RBW.

3) Aortitis associated with rheumatic fever:

 Usually the abdominal aorta is involved.

4) Rheumatoid arthritis involves only the ascending aorta.

5) Atherosclerotic aortic disease:

 It usuallyoccurs in patients > 50 years with risk factors.
 Arteriography reveals long stenosis between normal segments.

6) Thromboangiitis obliterans:
 Occurs frequently in smokers.
 Involves peripheral, small arteries.
 May develop toward distal extremities necrosis.

7) Congenital coarctation of aorta.

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 AORTIC ANEURYSMS

Definition: Aortic aneurysm is an abnormal, permanent, and localized dilatation of the aorta. It may
be fusiform or saccular.
Etiology:
1) Congenital aortic aneurysms.
2) Acquired aortic aneurysms:
 Atherosclerosis – systemic hypertension: fusiform aneurysms, usually occur in
males > 60 years, involving descending aorta and the distal segment of aortic arch.
 Syphilis: ascending (proximal) aorta is usually involved. The aneurysms usually
occur > 20 years after the primary infection. Syphilis causes saccular aortic
aneurysms.
 Infectious diseases: bacterial grafting on surgical aortotomy sites.
 Trauma
 Cystic medial necrosis:
- Ascending aorta is involved.
- Aneurysms are fusiform.

Topographic Classification:
1) Thoracic aortic aneurisms.
2) Abdominal aortic aneurysms.

THORACIC AORTIC ANEURYSMS

Etiology:
1) Acquired aneurysms (see above).
2) Congenital aneurysms are rare:
 Marfan’s syndrome.
 Aortic coarctation.
 Aneurysms of the sinuses of Valsava (and coarctation of aorta).

Clinical Features:
1) Often asymptomatic.
2) Chest pain:
 Diffuse, permanent pain, accentuated in systole and on exercise, radiating to the
back, frequently angina – like.
 Acute pain suggests rupture and represents a medical emergency!
3) Compression of adjacent organs:
 Superior vena cava: “mantle edema”, cyanosis of the face.
 Trachea and bronchi: cough, dyspnea.
 Esophagus: dysphagia.
 Recurrent laryngeal nerve: bitonal voice, hoarseness.
 Pulmonary artery: extrinsic stenosis
4) Systemic embolism.
Rupture of the aneurysm may be preceded (weeks before) by pain, unexplained anxiety,
hemoptysis, and can be followed by a fatal bleeding (it may rupture into pleura, trachea,
mediastinum, esophagus). More often, acute rupture occurs without any prior warning.

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Signs:
1) Double systolic expansion areas:
 Precordial
 Supraclavicular
2) Aortic systolic murmur.
3) Asymmetric pulse and blood pressure (due to compression of an aortic branch)

Paraclinical findings:
1) Chest X-ray (posteroanterior and lateral views, barium X-rays) may show:
 Direct signs: regular, homogenous opacity, making in any incidence a common
mass with the aorta. Radioscopy may reveal systolic expansion.
 Indirect signs: compression and deviation of trachea and esophagus; vertebral
“stamp”
2) Aortography is useful in revealing the extent of the aneurysm (Seldinger technique)
3) Echography.
4) CT is the gold standard for the detection of an aneurysm.

Differential diagnosis:

1) “Externalized” aneurysms: structures coming into contact, or deforming sternum.

 Sternum sarcoma
 Thyroid neoplasia on “plunging” goiter (intrathoracic).
 Lymphosarcoma.

2) Radiological visualized aneurysms: Mediastinal tumors:

 Ascending aorta:
- Dermoid cysts: dense, well circumscribed opacities with calcified outline.
- Bronchogenic cysts: changeable opacities, depending on respiration and
position.

 Aortic arch:
- “Plunging” goiter +/- neoplasia: inferior pointed opacity, diagnosed by I 131
scintigraphy
- Thymus tumors: polycyclical outline (neoplasia)
- Mediastinal adenopathy: polycyclical outline

*Boinet’s sign: Downwards drive of the larynx with left sided torsion.

 Descending aorta:
- Neurinomas.
- Esophageal neoplasia, esophageal diverticula, megaesophagus
- Hilar type of the lung carcinoma, adenopathy.
- Mediastinal hydatid cyst.

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 ABDOMINAL AORTIC ANEURYSMS

Etiology: only acquired!

Clinical features: often asymptomatic.

1) Palpable, pulsatile abdominal mass.
2) Pain:
- epigastric pain radiating to the back
- lower back pain
- lumbar pain radiating to the scrotum
N. B.! Acute pain occurs during rupture of the aneurysm!
3) Other systemic atherosclerotic signs.

Paraclinical investigations:
1) Abdominal radiography (anteroposterior and lateral views) may reveal:
- the aneurismal mass and its calcified outline (25%)
- osteolytic vertebral lesions.
2) Aortography exactly reveals the aneurysm and it is commonly used for patients evaluating
before surgical treatment.
3) Echography.
4) CT

Complications:
1) Rupture.
- Diagnosis: hypotension, lumbar pain, pulsatile abdominal mass.
- Rupture into the retroperitoneal space is manifested by flank hematomas.
- Rupture into duodenum produces massive gastrointestinal hemorrhage
(hematemesis, melena).
- Rupture into the peritoneum is characterized by peritoneal irritation, shock,
anemia, and peritoneal effusion.
2) Emboli into the lower limbs arterial tree.
3) Leriche’s syndrome.
4) Septic grafting: fever, abdominal pain, and leukocytosis.
5) Right cardiac failure: arteriovenous fistula due to rupture into the inferior vena cava.

Differential diagnosis:
1) Abdominal tumors: the tumors situated in front of the aorta are pulsatile!
Tumors:
- Pancreatic, gastric, colon tumors.
- Adenopathy of any cause.

2) Hyperkinetic syndrome: pulsatile abdominal aorta, without pain or palpable abdominal mass.

*In the event of rupture, differential diagnosis include:

3) Acute surgical abdominal pathology: acute hemorrhagic pancreatitis, mesenteric infarction,
ulcer perforation, and acute peritonitis of any cause.

4) Gastrointestinal bleeding.
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 AORTIC DISSECTION ANEURYSM

Definition: Aortic dissection is represented by a circumferential or, (less frequently), transverse tear
of the aortic intima, resulting a subintimal hematoma and a false channel within the media.

Incidence:
- Men are more affected than women (males / females ratio ~ 2-3 / 1)
- The peak incidence is at age 60 – 70.

DeBakey’s classification:
1) Type I: dissection involves proximal ascending aorta, aortic arch, and descending aorta for a
variable distance (primary tear is in ascending aorta).
2) Type II: dissection is limited to ascending aorta.
3) Type III: dissection involves descending aorta, distally extending to a variable distance (primary
tear distal to subclavian artery origin).

STANFORD STANFORD
type A type B

Fig. XXVI-1: DeBakey`s classification of aortic dissection comparing with Stanford classification (modified
from DeBakey M.E., Cooley D.A., Surgical management os dissecting aneurysms of aorta, J Thorac Cardiovasc Surg
1965, 49; 130-149).

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 DeBakey classification (proposed in
1965) is by far the most used in medical
practice, but aortic dissection has also other
types of classification: the Stanford
classification (fig. XXVI-1) and the very
Class I Class II new classification in 5 classes, which have
been proposed in 2001 (fig. XXVI-2).

Fig. XXVI-2: The 5-classes classification of

aortic dissection (source: European Heart
Journal, 2001,22,1642-1681).
Class III Class IV Class V

Etiology:
1) Systemic hypertension.
2) Pregnancy (50% from the women with aortic dissection are < 40 years).
3) Valvular aortic stenosis (due to poststenotic dilatation) and bicuspid aortic valve.
4) Congenital diseases of the connective tissue: Marfan’s syndrome, Ehlers-Danlos syndrome.
5) Aortic coarctation (type III).
6) Trauma.

Clinical features:
1) Men > 60 years.
2) Sudden, tearing (“knife-like”) anterior chest pain, radiating to the back, often to the inter-
scapular region. The pain migrates characteristically with propagation of the dissection and is
not aggravated by changes in position or respiration.
3) Sweating, nausea, vomiting, anxiety.
4) Syncope:
- Rupture into pericardium with tamponade.
- Compression of the carotid branches.
5) Left cardiac failure due to acute aortic regurgitation (pulmonary edema, cardiac asthma).

Physical signs:
1) Sudden loss of the peripheral pulse.
2) Shock: cold, sweat extremities. On the onset, blood pressure is normal or increased.
3) Neurological findings: hemiplegia, hemianesthesia (due to carotid obstruction), monoplegia,
paraplegia (spinal artery occlusion), visual disturbances (amaurosis), cerebrovascular
accident.
4) Fever.
5) Hypotension.
6) Collapse.

Paraclinical investigations:
1) Laboratory findings:
 Anemia.
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  Leucocytosis.
  LDH.
  Bilirubin.
2) ECG:
 Left ventricular hypertrophy.
 No evidence of ischemia (it is helpful in distinguishing aortic dissection from
myocardial infarction).
3) Chest X-ray:
 Double outline of the aorta.
 Dilatation of the ascending aorta
 Wide mediastinum.
4) Echocardiography:
 Free mobile flap within the lumen of the ascending aorta.
 Dilatation of the ascending aorta > 42 mm.
 Aortic regurgitation.
6) CT:
 Intimal flap visualization and the extent of the dissection.
 Two lumens (the true and the false lumen).
7) Aortography may be performed to identify:
 The primary tear.
 The double lumen.

Differential Diagnosis
Aortic dissection should always be included in the differential diagnosis of a patient with
unexplained syncope, stroke, congestive heart failure, acute arterial occlusion, or an abnormal aortic
contour on chest roentgenography, even in the absence of chest pain (table XXVI-3).
The management approach to aortic dissection (without rupture) differs especially according to the
location of aneurysm and its progression tendency. The complete topographic diagnosis is always
required (table XXVI-2).

Proximal Distal
1. Initial pain in the anterior chest. 1. Interscapular pain
2. Aortic diastolic murmur. 2. Hypertension
3. Pericardial friction rub. 3. Left pleural effusion
4. Decreased or absent blood pressure or pulses in the right arm.
5. Decreased right carotid artery pulsation.
6. Evidence of myocardial ischemia or infarction on ECG.
7. Marfan’s syndrome.
8. Congenital abnormality of the aortic valve

Table XXVI-2. Clinical clues to proximal versus distal aortic dissection

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 Symptoms Acute aortic Acute myocardial Aortic Aortic Acute Mediastinal
and signs dissection infarction regurgitation aneurysm pericarditis tumor
Chest pain Sudden, tearing Severe, progressive Variable Continuous and Progressive Progressive,
pain (crescendo) Low intensity progressive, due anterior chest persistent pain
Radiating to the Anterior chest pain (rupture of the to compression or pain, aggravated Mediastinal
inter-scapular area Radiating to the sinus of the erosion of by deep compression
and abdomen. arms, neck, back Valsava) adjacent structures inspiration Chest wall
(chest wall) invasion
Dyspnea Absent (only in Usually present Present Rarely present Absent Present (bronchus
aortic dissection (ventricular (ventricular (bronchus compression)
with aortic dysfunction) dysfunction) compression)
regurgitation and
LV failure)
Aortic Present in Absent Present Present in Absent Absent
regurgitation proximal ascending aorta
dissection aneurysm
Sweating Present Present +/- Absent Absent Absent
Hypotension Usually present Usually present +/- Absent Rarely present Absent
(tamponade)
Abnormalities Usually present Absent Absent Possible Pulsus paradoxus Absent
of the pulse (atherosclerotic (tamponade)
(asymmetry) obstruction)
Anemia Present Absent (may be Absent (may be Absent Absent Absent
present in present in
Neurological Usually present Usually absent Absent Absent Absent Possible
manifestations (aortic arch (metastasis)
dissection)

Table XXVI-3: Differential diagnosis of aortic dissection.

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 LERICHE’S SYNDROME

Definition: Chronic occlusive disease of the aorto-iliac arteries.

Etiology: atherosclerosis (associated with thrombosis).It occurs usually in males > 50 years.

Positive diagnosis:
1) Pain (cramp): exercise claudication; typically involves the lower back, buttocks and thighs.
2) Sexual impotence.
Signs:
3) Absence of femoral pulse bilaterally.
4) Murmurs over the inguinal area.
5) Coolness of the lower extremities.
6) Loss of lower limbs pylosity.
7) Atrophy of skin and muscles of the lower limbs.
8) Gangrene (rarely).

Evolution: slowly progressive, chronic, with exacerbations (thrombosis extends to the collateral
vessels).
Complications: thrombosis of the renal artery.

SYPHILITIC AORTITIS

Definition: syphilitic aortitis represents a cardiovascular complication of untreated lues, occurring

10-25 years after initial infection.

Classification of the cardiovascular lesions:

1) Uncomplicated luetic aortitis.
2) Luetic aortic aneurysm.
3) Aortic valvulitis with aortic insufficiency.
4) Coronary ostial stenosis.

Pathology: vasculitis and perivasculitis with lymphocytes and plasmocytes represent the elementary
lesion.
 In I and II stages: small vessels involvement.
 In III stage: aorta, great arteries and heart are involved.
*Syphilitic aortitis usually affects the proximal aorta (vasa vasorum is more affected)
Macroscopy:
- intima – “tree-barking” aspect;
- thickened adventitia;
- media presents retractile scars;
* Syphilitic lesions are often associated with the atheroslerotic ones.
Microscopy:
- the initial lesion is an obliterative endarteritis of the vasa vasorum, involving
adventitia;
- the lesions extend to the media and intima (destruction of collagen and elastic tissue).
* These changes resulting in aortic dilatation, aneurysm formation, and calcifications.
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Clinical features: 1/3 of cases may be asymptomatic, 1/2 develop aortic aneurysms, 1/3 –1/2
develop aortic regurgitation.
1) Aortic regurgitation.
2) Angina due to syphilitic coronaritis.
3) Proximal aortic aneurysm: pain, compression.

Laboratory findings:
1) Positive serologic tests for syphilis:
- VDRL test (30 % false negative).
- Fluorescent treponemal antibody absorbed test (FTA-ABs).
2) Chest X-ray:
- Dilatation and linear calcification of the ascending aorta.
- Dilatation and function of the left ventricle.
3) Echography:
- Aortic dilatation
- Aortic regurgitation
- right ventricle dilatation and function assessment.

B) PERIPHERAL ARTERIAL DISEASES

ACUTE PERIPHERAL ARTERIAL OBSTRUCTION

DEFINITION: Abrupt interruption of the arterial blood flow due to emboli, thrombosis, trauma,
intense arterial spasm, or has an iatrogenic origin.

ETIOLOGY: The most frequent cause is embolism (80%).

I. ARTERIAL EMBOLI:
Causes:
1. Heart diseases: -- the most important source of emboli (>80-90%)
 Rheumatic valvulopathy: mitral + LA thrombosis +/- atrial fibrillation.
 Atrial fibrillation.
 Left intraventricular thrombosis + myocardial infarction, especially in transmural
necrosis of the anterior and apical wall in the first month of evolution (especially for
patients not receiving anticoagulant therapy) and thrombosis inside the ventricular
aneurysm. Arterial embolism may be the first manifestations of the myocardial
infarction.
 Prosthetic valves (inefficient anticoagulant therapy).
 Infective endocarditis vegetations can produce fragmentation and embolism). Candida
produces large emboli.
 Cardiac tumors (atrial myxoma).

2. Extracardiac causes (5-10%):

 Arterial wall-attached thrombi:
 Aortic, iliac artery and femoral artery aneurysms.
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  Ulcerations of the atheromatous plaques.
 Dissecting aneurysm.
 Paradoxical embolism may occur associated with atrial septal defect. Suggestive clues:
acute peripheral arterial ischemia manifestations associated with deep limbs
thrombophlebitis, without other evident causes of arterial emboli.
 Cryptogenic emboli (5-10%): the exact source of arterial emboli cannot be identified
(confused with thrombosis “in situ”.

Frequent peripheral arterial emboli locations:

 The main arteries of the lowers limbs (70-80%).
 Bifurcations of the arteries (bifurcation of the femoral artery – 30-50%) + popliteal artery
= 2 x iliac arteries + aorta (smaller necessary emboli size for occlusion).
 The arteries of the upper limbs are rarely affected.

II. ARTERIAL THROMBOSIS

Predisposing factors:

1. Ateroma:
 Thrombosis on a vessel with an important ATS stenosis (critical stenosis) 
consequently mild, with clinical manifestations of either acute discrete or absent
ischemia (already existing on a collateral circuit).
 Thrombosis on a vessel with minimum ATS stenosis  manifestations of acute
severe ischemia (absence of a collateral circulation).
2. Arterial aneurysms: localized especially on peripheral vascular branches.
3. Non-atherosclerotic vascular lesions:
 Fibromuscular dysplasia.
 Adventitial cystic disease.
 Thromboangitis obliterans.
 Takayashu`s disease.
 Giant cell aortitis – Horton disease.
4. Arterial dissection –thrombi formation inside the dissecting aneurysm.
5. Extrinsic causes:
 Trauma – penetrating or iatrogenic (invasive diagnostic methods).
 External compression (brachial compression syndrome, popliteal compression
syndrome).
1. Hematological and systemic diseases:
 Polycythemia.
 Thrombocitosis.
 Severe disproteinemia.
 Antithrombin III deficit.
 Fibrinolytic system anomalies.
 Platelet aggregation disturbances.
 Hypercoagulation associated diseases:
 Malignant diseases.
 Septicemia.
 Cardiovascular infection with decreased cardiac output.

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Pathophysiology of acute arterial occlusion is multifactorial.
I. Primary factors:
1. Localization of the arterial obstruction and the diameter of the obstructed vessel: bigger
and more proximally located obstruction, the more severe the ischemia.
2. Degree of collateral circulation development (cardinal factor for ischemia development):
Absent in the first hours after an embolic occlusion (if the artery was previously
normal). In thrombotic occlusion the collateral circulation may be well developed
(depends on the chronic atherosclerotic ischemia, which has stimulated the development
of collateral circulation)
* The better is developed the collateral circulation, the more reduced are he signs of ischemia.

3. Thrombus size and thrombosis extension: occlusion of distal ramifications and

especially of collateral vessels, supplementary aggravates ischemia.

II. Secondary factors

1. Thrombus fragmentation and/or lyses: explains the rapid amelioration of ischemia ( after
a few minutes of acute occlusion).
2. Presence of associate venous thrombosis: prolonged acute arterial ischemia the venous
intima may be also ischemic disturbed, resulting a venous thrombosis of ischemic
origin.
3. Arterial spasm: is produced especially in secondary occlusion due to traumatism or
arterial puncture.
4. Hemodynamic condition of the patient: hypotension stimulates the extension of the
ischemia (the peripheral reflex of vasoconstriction determines a decrease of the
collateral flow).

III. Systemic metabolic anomalies:

1. Ischemic neuritis.
2. Ischemic miopathy (~ “crush syndrome”) appears approximately 8 hours after occlusion
leading to myoglobine release in the general circulation, which have dramatic
consequences:
 Acute renal insufficiency.
* Because of the more active metabolism of the nerves and the muscles →ischemia appears earlier
in muscles and nerves (4-6 hours), whilst the teguments resist a longer period of time.
 Hyperkalemia
 Systemic metabolic acidosis
 Myocardial depression
 Nervous system depression
* Hyperkalemia and acidosis can induce:
 Hypotension.
 Arrhythmias.
 Sudden death.
* Complication in the absence of therapy: fever, ischemic gangrene  infection → septicemia
→ death.
* Clinical manifestation can persist after revascularization:
 Cellular edema reduces slowly resulting a progressive amelioration of
ischemia.
 Collateral thrombosis leads to a persistent flow deficit.
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Clinical Features:
The “5 P” of PRATT
1. Pain
2. Pallor
3. Pulseless
4. Paraestesia
5. Paralysis
+
6. Prostration

A. Signs of acute arterial ischemia:

a. Disappearance of arterial pulse. The level of disappearance correlates with the level of
obstruction.
b. Pain:
 Intense and progressive.
 Maximum in the muscles located distal to the occlusion.
 May be hidden by the sensitive disturbances
c. Pallor:
 Initially: marble-like appearance (distal to the occlusion)
 Later: violet-blue/black (gangrene)
*The teguments have a low temperature immediately after the occlusion.
d. Paraestesis (nervous ischemia)- abolition of the tactile sensitivity is sign of severity.
e. Paralysis (nervous and muscular ischemia):
 Ischemic paralysis announces gangrene
 Muscular rigidity and involuntary muscular contraction: signs of severe and
irreversible lesion (revascularization could be lethal).
 Disappearance of perspiration (visceromotor nerves ischemia): sign of severity.

B. Clinical examination of the affected limb:

The point of the arterial pulse disappearance and the cold teguments upper border is found
bellow the zone of the anatomic arterial occlusion (~ with “a joint level”), as is shown in table
XXVI-4.

Temperature modification border Occluded point

Ankle Bifurcation of the popliteal artery
Above he knee (1/3 inf. the thigh) Common femoral artery
2/3 sup. of thigh External iliac artery
Thighs, inferior abdomen Bifurcation of the aorta

Table XXVI-4: Clinical detection of the arterial occlusion site.

C. Clinical examination of the heart and general examination (source of emboli)

 Arrhythmias
 Endocarditis
 Cardiomegaly
 Aortic aneurysm with thrombosis
 Aortic dissection

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Problems of Diagnosis
Differentiation of arterial embolism from thrombotic obstruction is of maximum therapeutical
importance: if emboli  urgent embolectomy, whilst thrombosis needs thrombolytic therapy

Clinical differentiation:
Emboli Thrombosis on ATS
Cardiovascular source of emboli (atrial History of intermittent claudication
fibrillation, mitral valvulopathy, etc.).
Absence of claudication or history of Obstructive arterial lesions on the
chronic arterial obstruction. controlateral limb, without acute ischemia

Arteriography – not necessary if obvious diagnosis: intracardiac thrombosis, aortic aneurysm,

endocardial vegetations.
Emboli Thrombosis
Absence of collateral circulation. Developed collateral circulation.
Evident trajectory between the opacified Trajectory between the opacified lumen of
lumen of the vessel and the emboli. the vessel and the thrombus is not regular;
the contrast substance infiltrates the
proximal zone of the thrombus.
Normal aspect of other vessels Stenotic lesion in other vessels too.

Severity diagnosis

Category Description Capillary Muscle Sensory loss Doppler Doppler venous

return weakness arterial signals signals
Viable Not Intact None None Audible (ankle Audible
immediately pressure > 30
threatened mm Hg)
Threatened Salvageable if Intact, slow Mild, partial Mild, Inaudible Audible
promptly incomplete
treated
Irreversible Major tissue Absent Profound, Profound, Inaudible Inaudible
loss, amputation (marbling) paralysis anesthetic
regardless of
treatment

Table XXVI-5: Clinical categories of acute limb ischemia (Adapted from Rutherford RB, Flanigan DP,
Guptka SK. Suggested standards for reports with lower extremity ischemia. J Vasc Surg 1986; 4:80–94.)

Differential Diagnosis:
a. Acute aortic dissection:
 Sudden loss of peripheral arterial pulse.
 Acute arterial ischemia.
 Diagnosis:
 Sudden violent pain in the anterior thorax or interscapular region radiating to
the lumbar, abdomen, inferior limbs.

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  Perspiration
 Unequal arterial pulse
 Arterial hypotension
 Chest X-ray, echo-transesophagial, echo-abdominal, CT, arteriography –
specific features.

b. Phlegmons cerulea dolens – thrombosis of the deep iliofemural artery

 May have associated arterial ischemia when a massive edema compromises the
arteries.(important edema of thigh and calve, cyanosis from the beginning).
 In acute arterial ischemia edema and phlebitis appears later (hours after pain), and
the teguments modifications are not so severe as in deep iliofemural thrombosis).
c. Neurological diseases – paralysis of neurological origin (but pulse is present)
d. Low cardiac-output conditions:
 Shock
 Hypovolemia.

Prognosis. Outcome.
Without treatment:
 Gangrene
 Acute renal insufficiency
 Septic state→death
Treatment→limb salvation = 85-95%
 Embolectomy with a Fogarty catheter → mortality within the hospital = 15-30%
(myocardial infarct, pulmonary embolism, recurrent arterial emboli)
 Amputation (for patients treated late, preexisting arteriopathy, coronary diseases)
 Post-operative recurrence = 7-12% of case.

CHRONIC OCCLUSIVE ATEROSCLEROTIC ARTERIOPATHY

Definition: Chronic obstructive arterial disease, due to an atherosclerotic lesion, functionally

characterized by a progressive reduction of the arterial lumen, inducing by consequence reduction of
the blood flow and oxygen supply (especially the inferior limbs are frequently affected).

Epidemiology:
 1-1,5% of men under 50 years and >5% of older men.
 it is less frequent in women, but the evolution is more severe.

Pathology. Pathophysiology:

ATEROSCLEROTIC LESION
1. Structure: sclerosis (thick fibrosis) associated with lipidic deposition (atheroma).
2. Localization: proximal segment of the big and medium size arteries:
 Infrarenal abdominal aorta.
 The origin of the primitive iliac arteries and its bifurcations.
 Femoral artery bifurcation.
 Femoral-popliteal junction.
3. Obstruction mechanism:

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  Progressive reducing of the arterial diameter through an increase in size of
atheroma.
 Embolism due to atheromatous plaque

Atherosclerotic plaque progression - 2 phenomena are involved:

 Progressive accumulation of lipids and fibers in the arterial intima (slow process).
 Rupture of the plaque followed by a thrombosis without occlusion, but with
fibrous cicatrisation (rapid process).

Rupture of the plaque:

1. The lipid core, surrounded by fibrotic area loses its stability and leads to fissure formation.
2. Contact between deep structure of plaque and blood flow.
3. Cholesterolic or atheromatous emboli.
4. Parietal hematoma ( through dissection and intrusion of blood in the intimal layer)
5. Initial white thrombus formation, which transforms later into a red or mixed thrombus.
6. Evolution of the thrombus:
a. Partial or total detachment and distal embolization → distal acute arterial
occlusion.
b. Successive deposit of fibrin layers → acute arterial occlusion at the level of the
plaque (associated with parietal hematoma and arterial spasm).
c. Thrombus and hematoma stabilization → the plaque is progressively increasing in
size.

Two natural compensatory phenomena reduce the consequences of the plaque existence on the
circulating flow:
 Vascular remodeling
 Development of the collateral circulation.

Vascular remodeling:
 Progressive dilatation of the healthy arterial wall, leading to a compensatory expansion of
the atherosclerotic plaque.
 Progressive thinning of the media under the atherosclerotic plaque.

Collateral circulation:
 It develops between the pre and post stenotic region of the arterial tree.
 Mechanism: angiogenesis and dilatation of the preexistent anastomoses.

* Claudication occurrence depends on:

a. The severity of the arterial stenosis.
b. The number and the topography of the stenoses.
c. Rapidity of the stenotic process development.
d. The functional capacity of the collateral circulation.

The classic 4 features:

1. Pain.
2. Temperature and color
modification of the
teguments.
3. Weakness until abolition of 285
the arterial pulse.
4. Atrophy.
Diagnosis
A. Chronic arterial ischemia diagnostic evidences
Clinical Aspect:
1. Pain - 4 stages (Leriche-Fontaine classification):
a. Parestesis, short painful episode while waking.
b. Effort claudication.
c. Rest claudication
d. Pain on rest + major trophic disturbances

Intermittent claudication- cardinal feature for chronic atherosclerotic arteriopathy.

 Muscular intense pain – appears on effort and disappears at rest.
 The severity of obstruction is quantified by claudication index (the walking
distance until the pain appears)
Pain on rest:
 Advanced stage of obstruction.
 More intense during the night.
 In stage III holding the leg “hanged at the edge of the bed” relieves it.
 Permanent pain (stage IV) →impossibility to rest without pain → narcotics.

2. Temperature and color modifications of the teguments

 initially: pale, cold;
 later: redish in color, pain on palpation, mild edema.
* The temperature and color indicates the state of the collateral circulation distally to the
obstructed artery.

3. Weakness until abolition of the arterial pulse.

 It may be the only signs in patients with intermittent claudication;
 Arterial auscultation may reveal stenotic murmurs.

4. Atrophy of the lower limbs teguments and muscles

 Initially minor changes: thin, dry teguments (subcutaneous atrophy), reduced
pilosity, thick, friable, fissured nail, muscular atrophy, +/- edematous stasis of the
limbs.
 Latterly major changes:
 Ischemic ulcer:
1. Typically is associated with nocturnal pain (stage IV).
2. Localization: dorsal aspect of the leg or pre-tibial.
3. Circumstances of appearance: spontaneously, or minimal trauma, or after
local infections.
4. Aspect: the base of the ulceration contains granulation tissue. The
teguments around are pale, marble-like, with signs of chronic ischemia.
5. Differential diagnosis:
 Chronic vascular stasis ulcer – localized perimaleolar and
surrounded by dermatitis and chronic venous insufficiency pattern.
 neurotrophic diabetic ulcer: appears in the areas of high pressure
(plantar); not painful.
 Gangrene

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 Supplementary clinical maneuvers
1. Claudication provocation tests:
 Treadmill; standard speed on effort (3,2 km/h) declined 10%
 2 steps Master’s test
 Electro stimulation of the solear muscle.

2. Provocation tests of color and temperature modification:

a. Büger-Ratchow test:
 Lifting of the leg at 60-75% from the dorsal decubitus position and making movements of
the ankle and fingers until the patient feels tired.
 The leg becomes intensely pale + collapse of the veins of the dorsal aspect.
b. Strandness test:
 Standard effort on the treadmill
 Evaluates the kinetics of the ankle arterial pressure recovery after effort (measure the time
of recovery) – correlates with the ischemic severity.
 Positive test: pain appearance and decreased systolic blood pressure at the ankle level.

c. Samuel test - 3 phases:

 Lifting of the extremity at an angle of 45º, which is maintained so 2 minutes. Plantar
pallor appears immediately (compare it with the bilateral and rest aspect).
 Effort phase: the previous position is maintained and the patient is invited to move
repeatedly his ankle (60 motions/min., 90 sec.) Pallor and pain appear
* In normal subjects there are no modification (pallor, pain) in the first 2 phases.
 Declined position: after the effort the patient rests at the margin of the bed, with his leg
hanging over. Observe:
1. Color recovery time.
2. Reactive hyperemia.
3. Venous filling.
1. Color return – normally begins after 1-2 seconds and it is complete after 5-8 seconds.
In chronic occlusive arteriopathy:
 15 s→easy form;
 30 s→ medium form;
 30-60 s→severe form.
2. Reactive hyperemia – normally begins after 4-5 s and reaches it maximum in 8-10 s after
which it decreases. It is delayed in artheriopathy.
3. Venous filling – normally begins in the declined position after 5-7s and amplifies in 8-
12s. Delayed venous filling means poor collateral circulation.

Investigations:
1. Doppler echography + determination of arterial systolic pressure by Doppler
stethoscope (detection of the stenosis site and quantification of its severity)
2. Oscillometry reveals diminished pressure oscillations below stenosis.
3. ARTERIOGRAPHY – reference method for morphological exploration.
4. Intravascular ultrasound

B) Functional quantification of ischemia degree

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 1) LERICHE – FONTAINE Classification (table XXVI-6)
stage I Asymptomatic: obstruction is discovered unexpectedly or paraclinical
stage II Intermittent claudication (effort ischemia)
IIa at > 200m of walking
at < 200m of walking
IIb
stage III Pain at rest (decubitus) – relieved in declined position
stage IV Pain at rest (decubitus) – no relief in declined position + atrophies
2) Rutherford`s clinical categories of chronic limb ischemia (table XXVI-7)
Grade Category Clinical description Objective criteria
0 Asymptomatic Normal treadmill/stress test.
0 Completes treadmill exercise*, ankle pressure
1 Mild claudication after exercise <50 mm Hg but >25 mm Hg less
than brachial.
2 Moderate claudication. Between categories 1 and 3.
I 3 Severe claudication. Cannot complete treadmill exercise and ankle
pressure after exercise <50 mm Hg.
Resting ankle pressure <40 mm Hg, flat or
4 Ischemic rest pain barely pulsatile ankle or metatarsal pulse volume
recording; toe pressure <30 mm Hg.
II Minor tissue loss— Resting ankle pressure <60 mm Hg, ankle or
5 nonhealing ulcer, focal metatarsal pulse volume recording flat or barely
gangrene with diffuse pulsatile; toe pressure <40 mm Hg.
pedal ischemia.
Major tissue loss— Same as category 5
extending above
III 6 transmetatarsal level,
functional foot no longer
salvageable

* Five minutes at 2 mph on a 12% incline .(Adapted from Rutherford RB, Flanigan DP, Guptka SK.
Suggested standards for reports dealing with lower extremity ischemia, 1986).

Positive Diagnosis:
a. Symptoms: intermittent claudication, or pain on rest.
b. Objective signs: pulse is absent in the affected region, modifications of teguments color
and temperature.
c. Other signs of ATS present in other territories to a patient >45 years (coronary, carotid,
cerebral).
d. Provocation tests – positive.
e. Arteriography+/- Doppler

Differential Diagnosis
I. Other arteriopathies (table XXVI-8, modified from Ed. Apetrei)
a) Thromboangiitis obliterans (Buerger`s disease) - inflammatory vasculitis.
 Symptoms onset <40 years.
 Mostly men, heavy smokers.

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  Most affected are small and middle arteries (muscular type) under the level of
humerous and popliteal artery).
 Associated frequently with: Raynaud syndrome, superficial no-varicose
trombophlebitis.
 Absence of :
1. arterial calcifications
2. dyslipidemic syndromes.
3. visceral artery co-affection

Features Thromboangiitis Atherosclerotic Acute arterial

obliterans artheriopathy thrombosis
1. Age of symptoms debut Usually <40 years Usually > 40 Usually <40
years years
2. No varicose superficial Frequent ( 55%) Absent Absent
trombophlebitis
3. Arterial calcification Absent Frequent (85%) Absent

4. Diabetes mellitus Usually absent Present at ~ 20% Usually absent

of the patients
5. Hypercholesterolemia Rare Present in 40% Rare
of patients < 60
years
6. Arteries and aortic murmurs Absent Present in 25% Absent
of cases
7. Ischemic manifestations of the Frequent (~45%) Absent Absent
superior limbs
8. Acute occlusion of the femoral Rare Present in ~ 20 Rare
and iliac artery. % of cases
9. Clinical and/or ECG evidences Rare (<2%) Frequent (at 50% Absent, but >
for myocardial ischemia of cases where 70% have AFb
ATS is debut at and/or LA
the periphery) enlargement
10. Familial antecedents of Rare (~10%) Frequent (~50%) Rare (~12%)
hypertension, DM, obesity.

Table XXVI-8: Differential diagnosis between chronic atherosclerotic arteriopathy,

trombangeitis obliterans and acute arterial thrombosis (modified from Apetrei Ed.).

b. Poliarteritis nodosum:
 Collagen tissue disease with prolonged febrile state and alteration of general status.
 Mostly affects young males.
 Visceral ischemia predominates over peripheral ischemia.
 Associates corticotherapy-sensitive arterial hypertension.
 Muscular biopsy confirms the diagnosis (fibrinoid necrosis)

c. Diabetic arteriopathy:
 Involves medium vessels (e.g. popliteal artery)

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  Tendency toward wet-gangrene

d. Acute arterial thrombosis:

 Clinical context (ischemic heart disease, rheumatic carditis, atrial fibrillation, infective
endocarditis).

e. Takayashu – Onishi disease

II. Functional arteriopathies:

 Raynaud`s disease
 Acrocyanosis

Differential Diagnosis of Claudication:

1. Pain in the thigh produced by the sciatic nerve pathology:
 accentuated by elongation maneuvers
 persistent on rest and during the night
 not associated with peripheral ischemic type modifications.

2. Pain produced by chronic venous insufficiency of the inferior limbs (Vaquez`s venous
claudication)
 appears while waking
 does not calm itself when effort is interrupted, only on lifting the inferior limb in the
decubitus position.
 coexists with: peripheral edema, cyanosis, superficial venous tension
 fever – if trombophlebitis coexists
 absence of arterial pulse disturbances( exception: phlegmatia ceruleea dolens)
3. Muscular pain due to muscular diseases (polimiositis) also associated with muscles atrophy.

Differential Diagnosis of pain on rest

 articular diseases (arthrosis, arthritis)
 muscular disease (associated with muscular atrophy and motor deficit)
 neurotrophyc disease ( sciatica)

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Cardiovasc Surg 1965; 49:130–149.
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