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Vascular Disease

Angiopathy

Peripheral vascular disease is a common disorder of both large and small vessels that affects millions of people. Atherosclerosis develops in the vessels resulting in narrowing and hence limiting blood flow. The disease can lead to a loss of limb or life.


Presentation

  • In most people PVD is a slow progressive disease that presents with a history of claudication, extremity ulcers that do not heal, or rest pain.
  • Patients with acute presentation usually present with a cold leg. The acute form of PVD is more likely to be seen in a patient with atrial fibrillation, recent MI or valvular heart disease.
  • In the majority of people the sole presentation of PVD is intermittent claudication. In patients with aorto-iliac disease, this will manifest as pain in the buttock or thigh area while walking for a certain distance. In people with more distal disease the pain may occur in the calf. Predictably the symptoms in both cases will subside with the rest. These individuals usually do not complain of leg pain while standing, sitting or sleeping.
    Some patients with PVD will present with rest pain, which is more serious. The pain signals inadequate perfusion to the tissues. The ischemic pain is often aggravated with heart disease, low cardiac output, hypotension and leg elevation. The condition is often partially relieved by placing the leg in a dependent position so that the effects of gravity perfuse the leg.
  • Many patients with PVD will also complain of erectile dysfunction. Presence of erectile dysfunction is felt to be an indicator for existence of both PVD and ischemic heart disease. Risk factors for erectile dysfunction include sedentary lifestyle, diabetes, obesity, mental stress and atherosclerotic disease.
    Presence of intermittent claudication, decreased or absent femoral pulses and impotence is known as Leriche syndrome. This syndrome is due to narrowing of the distal aorta or iliac arteries and signifies chronic PVD.
  • Patient’s medication list may also be indicative of PVD. Use of aspirin and cilostazol are commonly used to treat patients with PVD.

Physical
Physical exam of a patient with PVD depends on whether the condition is acute or chronic. In a patient with an acutely cold leg the 5Ps include presence of:

  • If the patient has an acutely cold limb, there may be mottling or livedo reticularis, cyanosis and the leg will feel cold to touch.
  • Presence of paresthesia and paralysis indicate that there is impending limb threatening ischemia and urgent revascularization is necessary. No time should be wasted and a vascular consult be called immediately.
  • Auscultation may reveal heart murmurs, palpate pulses in the carotid, abdomen, groin and behind the knee. Listen for bruits which may suggest turbulence and stenosis.
  • Ask the patient to walk or exercise and palpate pulses again. Exercise normally causes decrease in pulses but they do recover within a few minutes. In patients with PVD, recovery of pulses is prolonged.
  • Perform the Allen test to determine if the patient has evidence of restricted blood flow to the hand.
  • Examine skin- which may have a shiny appearance and absence of hair. The skin may be fragile, with brittle nails. Ulcers may be present on the heel, sole or tips of the toes. Non-healing ulcers may be a clue for PVD.
  • The involved extremity may have atrophy of the muscle.
  • Measure the Ankle brachial index (ABI) using a Doppler machine over the posterior tibial artery and brachial artery. The patient must be supine when the test is done. Normal ABI is slightly more than 1, but if it is less than 0.5, it indicates severe PVD.
  • Degree of pallor can be assessed subjectively at bed rest and with leg elevation.
Atrial Septal Defect
  • Atrial septal defects are usually asymptomatic, and are closed surgically or by a catheter implanted device in preschool age children. Rarely, they may cause symptoms in infancy, and management at this age is debated.[ncbi.nlm.nih.gov]
  • Based on our observations, the clinical course of children with common atrium may differ from patients with a large atrial septal defect.[ncbi.nlm.nih.gov]
  • Abstract OBJECTIVE: Surgical indication was determined by lung biopsy in 91 patients with secundum atrial septal defect (ASD) and severe pulmonary hypertension 70 mm Hg of pulmonary arterial peak pressure and/or pulmonary vascular resistance of 8 U/m([ncbi.nlm.nih.gov]
Wound Infection
  • The primary outcome was wound complication, defined as any wound infection, lymphocele, hematoma, dehiscence, pseudoaneurysm, or necrosis.[ncbi.nlm.nih.gov]
Chest Pain
  • Blood flow to the heart can slow down or stop, and this can cause chest pain (angina), shortness of breath, heart attack, or other symptoms. This is known as coronary artery disease.[hackensackumc.org]
  • Symptoms of Pulmonary Hypertension Progressive fatigue Shortness of breath Chest pain or pressure during activity (angina) Dizzy spells that may occur during activity or exercise Fainting Ankle or leg swelling (edema) Increased heart rate (tachycardia[brighamandwomens.org]
  • A person with cardiovascular disease, for instance, might suffer chest pains while an individual with PAD can experience pain or numbness in the legs.[thevascularexperts.com]
  • When symptoms do happen, they may include Chest pain, especially when you breathe in Shortness of breath Coughing up blood Dizziness Fainting Phlebitis There are two forms of phlebitis.[texasheart.org]
  • For example: Blockage in coronary arteries can cause chest pain ( angina ) or a heart attack. If it's in the carotid arteries that supply your brain, it can lead to a stroke or mini stroke, which is called a transient ischemic attack or TIA.[webmd.com]
Hemianopsia
  • A 48-year-old female presented to an emergency room with symptoms of episodic hemianopsia, dysphasia, and facial numbness.[ncbi.nlm.nih.gov]
Cutaneous Manifestation
  • Abstract In the contemporary era of medical diagnosis via sophisticated radiographic imaging and/or comprehensive serological testing, a focused physical examination remains paramount in recognizing the cutaneous manifestations of chronic vascular disease[ncbi.nlm.nih.gov]
Facial Numbness
  • A 48-year-old female presented to an emergency room with symptoms of episodic hemianopsia, dysphasia, and facial numbness.[ncbi.nlm.nih.gov]
Neglect
  • Unlike many other disorders, PVD is often neglected and not recognized. In addition, there is gross misuse and abuse of unproven and unrecognized therapies.[symptoma.com]

Workup

Laboratory Studies
Blood work required in the work up of a patient with PVD includes:

  • CBC
  • Electrolytes
  • Renal function to assess kidney function
  • Lipid profile
  • Coagulation factors
  • ECG to look for arrhythmias, MI
  • CRP, homocysteine and interleukin 6

Imaging Studies

  • The role of plain x-rays is limited.
  • The first study of choice is Doppler ultrasound, which can assess flow and site of occlusion. The technique can be used to assess both the lower and upper extremity and the neck. The quality of the signal may reveal a clue to the type of obstruction but is often unable to differentiate between embolic and thrombotic disease.
  • MRI is not very useful for the extremities but does provide high detail for the large vessels like the aorta. MRI is also not available on an emergency basis and is also expensive.
  • CT angiography is now widely used to assess patients with PVD. Contrast is still required but the images are much superior to an MRI. All patients should have renal function assessed prior to use of contrast.
  • The gold standard for investigation of patients with PVD is the angiogram. It requires the use of dye and allows to localize the atherosclerosis and assess number of vessels involved. The angiogram can help determine whether the cause of the extremity is due to embolic or atherosclerotic disease Angiography is necessary when endovascular stenting is planned. The downside to angiography is the use of dye and need for bed rest for at least 6 hours after the procedure.

Other Tests
Transcutaneous oximetry is also used to assess degree of perfusion in PVD. The technique is being used in outpatient clinics to assess wound healing before and after surgery.

Nephrolithiasis
  • OBJECTIVE: Vascular calcifications are associated with nephrolithiasis. Although studies have demonstrated correlations with vascular disease and calcium stones in kidney stone formers (KSF), an etiologic link has remained elusive.[ncbi.nlm.nih.gov]

Treatment

The treatment of PVD is multidisciplinary and divided into acute and chronic management [5][6].

Acute ichemia

When a patient presents with an acutely cold leg, the treatment is emergency revascularization.  When a patient presents with a cold leg to the ED, attention should first be directed to the ABCs. Two large bore IVs should be inserted and routine blood work is necessary. Because some of the patients may be on blood thinners, a coagulation profile is necessary. If the patient has an acutely cold leg, heparin should be started immediately. The heparin must be administered intravenously and the PTT should be maintained at 1.5-2 times normal. This should be followed by an ultrasound duplex test if there are no pulses palpable. The vascular surgeon must be notified immediately for further care. Most patients require some type of pain medication.

  • In some cases where the patient is not a candidate for surgery, a thrombolytic agent may be used; the type of thrombolytic agent depends on physician preference and experience. Sometimes the radiologist can place a catheter just above the site of occlusion and direct thrombolytic therapy can be administered. The biggest contraindication to thrombolytic therapy is active internal bleeding, recent surgery or trauma.
  • In most cases, surgeons perform an embolectomy under local anesthesia. The procedure requires removal of the blood clot via a small groin incision with a special balloon as instrument.
  • Once the patient is stable, the cause of the emboli is worked up. This may mean obtaining an ECHO and ruling out atrial fibrillation.

Chronic management

Once an individual has been diagnosed with PVD, the treatment involves treating the risk factors and causes [7]:

  • Smoking cessation is highly recommended. Smoking cessation can lead to improvement in leg symptoms, decrease risk of extremity amputation and improve patency of grafts after revascularization. Smoking cessation also reduces risk of cardiovascular events. Thus the patient should be actively encouraged to stop smoking by referral to a smoking cessation program, and use of nicotine replacement products.
  • Managing Diabetes Mellitus: Blood sugars must be aggressively controlled as such an approach has been shown to lower risk of cardiac events and microvascular complications.
  • Treating hyperlipidemia: since high levels of lipids can worsen atherosclerosis, treatment of dyslipidemia is recommended. The aim should be to attain a target LDL level of less than 100 mg/dl and a TG level of less than 150mg/day. Statins should be started as they have been shown to improve pain free ambulation. Niacin or fibrates may be an option for patients with elevated levels of triglycerides.
  • Hypertension Treatment: Hypertension must be controlled in patients with PVD. The drugs of choice for treatment of hypertension include the ACE inhibitors, thiazide diuretics, beta blockers and calcium channel blockers. Both ACE inhibitors and beta blockers have been shown to lower the incidence of new coronary events in patients with PVD. However, when starting ACE inhibitors in patients with PVD, renal function must be closely monitored.
  • Antiplatelet Treatment: There is good evidence showing that antiplatelet agents can lower the risk of an MI, stroke or vascular death in patients with PVD. Current guidelines recommend use of aspirin or Clopidogrel in patients with PVD. Ticlopidine can also be used but because of its adverse effects like thrombocytopenia and neutropenia, it is not used as much. There is no good evidence to suggest that dual antiplatelet therapy is better than a single agent. The treatment is lifelong [8].
  • Exercise: All patients with PVD should be encouraged to walk as this has shown to improve collateral circulation and relieve leg symptoms.
  • Drug therapy: The reversible phosphodiesterase inhibitor, cilostazol, is the only approved medication for PVD patients. It has been shown to promote vasodilatation and inhibit platelet aggregation. The drug has been found to be superior to pentoxifylline for treatment of claudication. Common adverse effects of cilostazol include diarrhea, gastric upset and headache. The drug is contraindicated in patients with heart failure.
  • Pentoxifylline is a methylxanthine derivative that has been shown to reduce blood viscosity and has anti-inflammatory effects; it has only a small benefit in patients with intermittent claudication.
  • Endovascular Interventions: For isolated lesions of the arteries, endovascular stenting and angioplasty is now an option. Percutaneous angioplasty carries a much lower morbidity than open surgery. Stents appear to remain patent for longer periods when used in larger vessels like the iliac arteries. For smaller vessels like the tibial artery, the patency rates with stents are not great. Restenosis is a common problem when stents are used in vessels below the knee. Today endovascular procedure is a great option for patients who are not candidate for bypass surgery.
  • Surgery: Surgery is recommended when there is risk of tissue loss, non-healing wounds and the lifestyle is restricted. Surgery is always attempted to preserve the ischemic limb. There are many types of procedures used treat PVD. The type of procedure depends on the location of lesion, type of lesion and patient morbidity. Some vascular procedures can be performed under regional anesthesia [9][10].
  • For those with critical artery narrowing, the surgeon may perform a bypass or an endarterectomy. For above knee bypass, the surgeon may use a vein or prosthetic graft. For vascular disease below the knee, saphenous vein is usually used as a bypass conduit. Bypass for atherosclerotic disease does carry a mortality rate of 1-4%. Patients need to be thoroughly worked up to ensure that they can withstand the stress of surgery. MI is a common complication in the postoperative period.

Prognosis

The prognosis of patients with PVD is guarded. Because the disease is progressive, eventually most patients develop symptoms. PVD is also associated with a high risk of myocardial ischemia and stroke because the same pathology is present in these disorders. For individuals with mild disease, there may be no symptoms but for those with severe disease, the individual may develop impotence, intermittent claudication, non healing ulcers, decreased exercise endurance, limitation in lifestyle and rest pain. Those with severe leg ischemia may end up with above or below knee amputations. Even with surgical therapy, complications are common and patency rates of grafts are not overly impressive. If the individual with PVD does not change his/her lifestyle, then the morbidity and mortality rates are high [4].

Etiology

The cause of PVD is multifactorial and includes the following:

Epidemiology

PVD is quite common not only in North America but across the globe. In the USA alone, it is estimated that there are nearly 10 million people with PVD. The disorder is often seen after the 5th decade of life and carries a high morbidity. With the aging population, it is estimated that a number of affected people with PVD are also going to significantly increase. PVD occurs with equal frequency in men and women but the outcomes in diabetic women tend to be worse. The primary reason for this are the small sized blood vessels in females which can be readily obstructed by atherosclerotic plaques.
Among races, PVD is slightly more common in African Americans compared to Caucasians and Hispanics. The ethnic susceptibility is likely related to higher prevalence of hypertension, diabetes and obesity in this population. Unlike many other disorders, PVD is often neglected and not recognized. In addition, there is gross misuse and abuse of unproven and unrecognized therapies. Several studies indicate that in primary practice, PVD is often not diagnosed or recognized despite presence of risk factors. This often leads to progression of disease, which ultimately results in extremity amputation. Even when the disorder is recognized, it is not thoroughly worked up and the treatment is often poorly managed.

Sex distribution
Age distribution

Pathophysiology

PVD occurs primarily because of atherosclerosis. The process starts with deposition of cholesterol in the intimal layers of the vessel. Over time, this develops into a cholesterol plaque covered with a fibrous layer. As the size of the plaque grows it can obstruct blood flow distally. Sometimes, the atherosclerotic plaque can rupture and promote thrombosis or generate emboli which occlude blood vessels distally. Atherosclerosis can affects all types of vessels but is most common at points where blood vessels bifurcate. Along these areas there is turbulence and stasis, which promotes shear stress and injury. Atherosclerotic disease is usually segmental in distribution but does vary from patient to patient. When the progression of atherosclerosis is slow, the small and medium sized blood vessels get occluded and numerous collateral vessels develop.

The symptoms of PVD occur when the blood vessel is occluded by thrombus, emboli or acute trauma that compromises tissue perfusion. Overall, atherosclerosis affects the blood vessels in the lower extremities more frequently than those in the upper extremities. Risk factors that predispose patients to formation of thrombosis include

Sudden occlusion of a blood vessel is usually due to emboli, which in most cases are from the heart. Other sources of emboli include foreign bodies, tumor or atheromatous fragments from other blood vessels (eg abdominal aorta). The most common sites where atherosclerosis occurs and have a high tendency to promote thrombosis include:

  • Femoral arteries
  • Iliac arteries
  • Aorta
  • Popliteal artery
  • Carotid artery

The presentation of a patient with atherosclerosis depends on the nature of the occlusion, development of collateral circulation and presence of symptoms. In general, emboli tend to have high morbidity as the patient has not had time to develop collateral circulation. In either case, both emboli and thrombosis results in decreased tissue perfusion to the distal vessel.

Prevention

Individuals who have moderate to severe PVD can be managed as outpatients but need close follow up and monitoring. All patients with PVD should be warned about the harmful effects of smoking and types of physical activities that lead to injury. The individual should be told to avoid cold environments but when going out and should dress warm. They should be educated that many cold and cough medications can act as vasoconstrictors and limit blood supply to the extremity.

Use of illicit drugs like cocaine should be avoided as it may lead to increased arterial tone. In addition, beta-blockers may exacerbate the symptoms of claudication.

After discharge the patient must be started on antiplatelet agents to prevent worsening of PVD and decrease risk of cardiovascular events. In addition, the patient must be told to change lifestyle, discontinue smoking and exercise regularly.

Statins are now prescribed to lower cholesterol and triglycerides preliminary evidence indicates that statins may slow the progression of atherosclerosis.

Summary

Peripheral vascular disease (PVD) is a very common medical disorder, which has the potential to lead to loss of limb or even life, if not properly treated. PVD, when moderate to severe, can lead to decrease in tissue perfusion, and this in turn can lead to pain, non-healing ulcers and decreased ability to walk. While in most cases PVD is due to progressive atherosclerosis, in other cases, it may be due to emboli. Millions of people have varying degrees of PVD, but the disorder can suddenly become acute and the patient will present to the ED with a cold extremity. Immediate treatment is required when treating acute limb occlusion as it can result in very high morbidity and mortality [1] [2] [3].

Patient Information

PVD is common in many people who smoke, have diabetes or hyperlipidemia. The disorder results in narrowing of the blood vessels, which decreases blood supply to the tissues. Almost any organ can be affected in PVD but the most common are the kidneys, legs, and brain. Risk factors for PVD include smoking, diabetes, hypertension, hyperlipidemia and an unhealthy diet. The disorder results in cholesterol deposition over the blood vessels, which gradually leads to narrowing or obstruction of blood flow. The patient may present with pain in the buttock area while walking or in severe cases may present with nonhealing ulcers in the leg, blush and cold legs and rest pain. The diagnosis is made by ultrasound and invasive studies like the CT scan. The treatment depends on the degree and severity of the disease. In patients who suddenly develop a cold leg, immediate surgery is required to remove the blood clot. In those with chronic disease, one should avoid smoking, control blood sugars, ensure better control of blood pressure, exercise and eat a healthy diet. Drugs like aspirin may be useful in some patients.

References

Article

1. Benitez E, Sumpio BJ, Chin J, Sumpio BE. Contemporary assessment of foot perfusion in patients with critical limb ischemia. Semin Vasc Surg. 2014 Mar;27(1):3-15.

2. Patel MR, Conte MS, Cutlip DE, et al. Evaluation and treatment of patients with lower extremity peripheral artery disease: consensus definitions from Peripheral Academic Research Consortium (PARC). J Am Coll Cardiol. 2015 Mar 10;65(9):931-41.

3. Society for Vascular Surgery Lower Extremity Guidelines Writing Group, Conte MS, Pomposelli FB, Clair DG, Geraghty PJ, McKinsey JF, Mills JL, Moneta GL, Murad MH, Powell RJ, Reed AB, Schanzer A, Sidawy AN; Society for Vascular Surgery. Society for Vascular Surgery practice guidelines for atherosclerotic occlusive disease of the lower extremities: management of asymptomatic disease and claudication. J Vasc Surg. 2015 Mar;61(3 Suppl):2S-41S.

4. Alahdab F, Wang AT, Elraiyah TA, et al. A systematic review for the screening for peripheral arterial disease in asymptomatic patients. J Vasc Surg. 2015 Mar;61(3 Suppl):42S-53S.

5. Malgor RD, Alalahdab F, Elraiyah TA, et al. A systematic review of treatment of intermittent claudication in the lower extremities. J Vasc Surg. 2015 Mar;61(3 Suppl):54S-73S.

6. Gulati A, Botnaru I, Garcia LA. Critical limb ischemia and its treatments: a review. J Cardiovasc Surg (Torino). 2015 Apr 14.

7. Tu C, Das S, Baker AB, Zoldan J, Suggs LJ. Nanoscale Strategies: Treatment for Peripheral Vascular Disease and Critical Limb Ischemia. ACS Nano. 2015 Apr 10.

8. Bedenis R, Lethaby A, Maxwell H, Acosta S, Prins MH. Antiplatelet agents for preventing thrombosis after peripheral arterial bypass surgery. Cochrane Database Syst Rev. 2015 Feb 19;2:CD000535.

9. Menard MT, Farber A. The BEST-CLI trial: a multidisciplinary effort to assess whether surgical or endovascular therapy is better for patients with critical limb ischemia. Semin Vasc Surg. 2014 Mar;27(1):82-84.

10. Andrews KL, Houdek MT, Kiemele LJ. Wound management of chronic diabetic foot ulcers: from the basics to regenerative medicine. Prosthet Orthot Int. 2015 Feb;39(1):29-39.

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Last updated: 2019-07-11 21:43