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Friday, June 19, 2009

PE a commonly missed fatal disease

CTPA of patient with PE

Pulmonary Embolism

The patient's diagnosis was made based on the CT pulmonary angiogram (CTPA) of the chest, which revealed multiple thrombi extending into the lobar and segmental/ subsegmental branches of the right and left pulmonary arteries. Venous Doppler ultrasonography of both lower extremities was negative for deep vein thrombosis. A CT scan of the abdomen and pelvis with contrast did not reveal any thrombi in the pelvic veins. Venous Doppler ultrasonography of the upper extremities was not performed, as upper extremity thrombi are less common than lower extremity deep venous thrombi.

Pulmonary embolism is a serious and potentially fatal complication of thrombus formation within the deep venous circulation. Pulmonary embolism is the third leading cause of death in the United States, with approximately 650,000 patients developing pulmonary embolism each year. Most cases are not recognized antemortem; up to 80% of cases are diagnosed at autopsy.

The pathophysiology of pulmonary embolus is thought to result from obstructed pulmonary blood flow. Air, amniotic fluid, foreign bodies, parasite eggs, septic emboli, and tumor cells can all embolize in the pulmonary vasculature. The most common embolus is a thrombus, which can form anywhere in the venous system. The most common site for thrombus formation is in the deep veins of the lower extremities. The risk factors for thrombus formation are venous stasis, hypercoagulable state, and vessel wall (endothelial) damage (known as the Virchow triad). Several factors predispose individuals to an increased risk of pulmonary embolism, including an age of over 40 years, obesity, congenital thrombophilia, smoking, cancer, and pregnancy. The risk is also increased by use of oral contraceptives.

Although the presentation of pulmonary embolus can be extremely variable, the typical presentation includes dyspnea, cough, fever, leg pain and swelling, and chest pain. As the thrombus progresses, patients may develop apprehension, diaphoresis, palpitations, nausea, vomiting, chills, and syncope. If patients remain untreated, they may develop syncope, cyanosis, diaphoresis, tachycardia, hypotension, and shock. Less common signs include hemoptysis, atelectasis, wheezing, pleural friction rub, rales, accentuated S2 or S3, tricuspid regurgitation, jugular venous distension, and acute right ventricular strain. Signs of acute right ventricular strain that may be seen on an electrocardiogram include an S-wave in lead I, a Q-wave in lead III, and a T-wave in lead III. Right axis deviation and partial or complete right bundle branch block may also be noted.

The differential diagnosis of pulmonary embolism includes dissecting aortic aneurysm, pneumonia, acute bronchitis, bronchial carcinoma, pericardial or pleural disease, heart failure, costochondritis, pleurisy, pneumothorax, mucus plug, and myocardial ischemia.

Routine laboratory tests have limited value in the diagnosis of pulmonary embolism. Arterial blood gas may reveal hypoxemia, hypocapnia, and respiratory alkalosis with an elevated A-a gradient. A positive D-dimer is not specific for pulmonary embolism, but a negative D-dimer has a 95% negative predictive value; therefore, it is useful in ruling out pulmonary embolism in those with a low pretest probability of disease. Coagulation studies and coagulopathy workup are not useful in the diagnosis of pulmonary embolism, but they may be necessary once the diagnosis has been established to rule out an underlying hypercoagulable condition.
The gold standard for diagnosing pulmonary embolism has been pulmonary angiography; however, CTPA is rapidly gaining in popularity for diagnosing pulmonary embolism, with a sensitivity of 85-94%, and it is likely to become the new gold standard. Spiral CT scanning is less invasive and more available than pulmonary angiography. A ventilation-perfusion scan can be useful in ruling out pulmonary embolism, but it may be less useful in diagnosing pulmonary embolism because many clinical conditions can lead to defects in the perfusion scan as a result of decreased blood flow. A ventilation-perfusion scan is now generally done only when CT angiography is contraindicated. Chest radiography is usually performed, but it is rarely diagnostic and is often completely normal. An ECG is typically obtained to rule out alternative causes of chest pain/dyspnea, but it is also not of much diagnostic value in pulmonary embolism itself. The most common ECG abnormalities in pulmonary embolus include sinus tachycardia and nonspecific T-wave changes. Although the classic S1Q3T3 pattern was noted in hindsight in this patient, especially with return visits, it is not frequently seen.

The treatment for pulmonary embolism falls into 2 categories. The first category includes patients who are hemodynamically stable. For these patients, anticoagulation and prevention of recurrent pulmonary embolism are vital. Anticoagulation is initially obtained with the use of heparin, fondaparinux, or low molecular weight heparin (LMWH). After initial anticoagulation is started, warfarin may be commenced. Bridging with heparin or LMWH for the first 5 days of warfarin therapy, until a therapeutic international normalized ratio (INR; therapeutic range, 2-3) is achieved is necessary. To prevent recurrent pulmonary embolisms, inferior vena cava or other intravenous filters can be placed.
The second category of treatment addresses hemodynamically unstable patients. For these patients, there are 2 major treatment options. The first is direct removal of the clot, through surgical embolectomy or catheter-based extraction by an interventional radiologist. Surgical embolectomy should only be used in patients with contraindications to thrombolytics or failed thrombolytics, or in whom surgery may be the only chance for survival. The second form of treatment is thrombolysis. Although thrombolysis has not been shown to improve mortality, it is often readily available in EDs. Indications for thrombolytic therapy include patients with shock, right heart failure, underlying cardiopulmonary disease, recent pulmonary emboli, or severe pulmonary hypertension. Thrombolytics may cause significant bleeding, including central nervous system bleeding, and they should only be used in appropriate circumstances where the risk/benefit ratio is favorable.

Contraindications of thrombolysis include recent or suspected cerebrovascular accident, intracranial trauma or surgery within the past 2 months, active intracranial disease, major internal bleeding within the past 6 months, uncontrolled hypertension, bleeding diathesis/coagulopathy, recent major surgery within 10 days, recent trauma, infective endocarditis/pericarditis, pregnancy, aortic aneurysm or hemorrhagic retinopathy.

The patient (CT above) was admitted to the intensive care unit and started on LMWH and warfarin. Given the subacute nature of his symptoms and his apparent stability, thrombolytics were not initially administered. The patient's oxygen saturation and symptoms continued to improve during his hospitalization and further laboratory studies, including antinuclear antibody (ANA) , protein C and S, prothrombin mutation, factor V Leiden, lupus anticoagulant, and phosphatidyl antibody, were initiated. He was discharged once his INR was therapeutic at a range of 2-3 and followed up in an anticoagulation clinic to maintain a therapeutic INR. All coagulation panel labs were negative, and he has not had a recurrence of the pulmonary embolism. A CT scan of his chest, abdomen, and pelvis done 1 month after initiation of therapy revealed an approximate 95% decrease in the size of the emboli seen in the left pulmonary artery and an almost 80% decrease in the pulmonary emboli seen in the right pulmonary artery. The embolic source was never identified.

Saturday, June 6, 2009

Takotsubo cardiomyopathy - Stress induced

Watch your stress... you may find yourself suffering from an unusual type of cardiomyopathy (enlargement of the apical aspect of the heart).

(A: Takotsubo Cardiomyopahty scheme with ballooning of the apical aspect of the heart. B: Normal heart cross section)

Takotsubo cardiomyopathy, also known as transient apical ballooning, apical ballooning cardiomyopathy, stress-induced cardiomyopathy, broken-heart-syndrome and simply stress cardiomyopathy, is a type of non-ischemic cardiomyopathy in which there is a sudden temporary weakening of the myocardium (the muscle of the heart). Because this weakening can be triggered by emotional stress, such as the death of a loved one, the condition is also known as broken heart syndrome.

The typical presentation of someone with takotsubo cardiomyopathy is a sudden onset of congestive heart failure or chest pain associated with EKG changes suggestive of an anterior wall heart attack. During the course of evaluation of the patient, a bulging out of the left ventricular apex with a hypercontractile base of the left ventricle is often noted. It is the hallmark bulging out of the apex of the heart with preserved function of the base that earned the syndrome its name "tako tsubo", or octopus trap (bowl) in Japan, where it was first described.

The cause appears to involve high circulating levels of catecholamines (mainly adrenaline/epinephrine). Evaluation of individuals with takotsubo cardiomyopathy typically include a coronary angiogram, which will not reveal any significant blockages that would cause the left ventricular dysfunction. Provided that the individual survives their initial presentation, the left ventricular function improves within 2 months. Takotsubo cardiomyopathy is more commonly seen in post-menopausal women. Often there is a history of a recent severe emotional or physical stress.


Thursday, June 4, 2009

Ankylosing spondylitis

Ankylosing spondylitis is a chronic inflammatory disorder of multiple articular and para-articular structures that principally involves the axial skeleton. It usually affects the sacroiliac joints and the spinal facet joints of the vertebrae. It sometimes involves the appendicular skeleton as well, such as the joints of the greater trochanter, patella, and calcaneum. Other extraspinal manifestations include iritis/uveitis and pulmonary involvement. The basic pathologic lesion of ankylosing spondylitis occurs at the entheses, which are sites at which ligaments, tendons, and joint capsules attach to bone. In the outer layers of the annulus fibrosis of the intervertebral disks, the condition manifests as a formation of new bone. The name of the disease is derived from Greek; "ankylos" means stiffening of a joint, and "spondylos" means vertebra. The disease is classified as a chronic and progressive form of seronegative arthritis. Ankylosing spondylitis affects men 4-10 times more frequently than women, and the symptoms generally appear in those aged 15-35 years. More than 90% of whites with ankylosing spondylitis have the HLA-B27 gene, but 6-8% of those with this gene do not develop the disease.

Symptoms of ankylosing spondylitis include back pain and stiffness, peripheral joint and chest pain, sciatica, anorexia, weight loss, and low-grade fever. The back pain associated with this condition is typically transient at first, but it eventually becomes persistent. It is usually worse in the mornings and resolves with exercise. A typical patient may also complain of waking up with back pain at night. The pain is usually centered over the sacrum, but it may radiate to the groin, buttocks, and down the legs. With time, the back pain usually progresses up the spine and affects the rib cage, resulting in a restriction of chest expansion and diaphragmatic breathing (observed as ballooning of the abdomen during inspiration) as the costovertebral joints become affected. The cervical spine is ankylosed late in the course of the disease, leading to restriction in neck movement and head rotation. Without treatment, the spine eventually becomes completely rigid, with loss of the normal curvatures and movement.

On physical examination, the loss of lateral flexion of the lumbar spine is the earliest objective sign of spinal involvement. The sacroiliitis may be detected by eliciting a tenderness response during percussion over the sacroiliac joints. Objective tests to quantify spinal restriction include touching the toes, the Schober test, and measurement of chest expansion. Additional physical findings include restriction of motion in the peripheral joints and tenderness over the enthuses. The physical exam should also include evaluation for signs of potentially serious cardiovascular and pulmonary complications, such as aortic incompetence secondary to aortitis, conduction defects of the heart, cardiomyopathy, pericarditis, apical fibrosis of the lungs, bronchiectasis, cavitation of the chest, and development of a restrictive ventilatory pattern. Other associated conditions include the development of inflammatory bowel disease, uveitis (in up to 20% of patients), radiculitis secondary to inflamed nerves, and, rarely, amyloidosis.

Specific criteria for the diagnosis of ankylosing spondylitis include the Rome criteria (developed in 1963) and the New York criteria (developed in 1968). Although these criteria have been generally accepted as useful, limitations are recognized and overlaps exist among the clinical and radiologic features of various seronegative spondyloarthropathies. Sacroiliitis is the hallmark of ankylosing spondylitis and is a requisite for the diagnosis under both sets of criteria. Other conditions, such as psoriasis, Reiter disease, enteropathic arthropathy, hyperparathyroidism, and osteitis condensans ilii, may also result in bilateral symmetric sacroiliac joint disease and should be considered in the differential diagnosis. Ankylosing spondylitis may also present with asymmetric sacroiliitis, which may be more characteristic of other conditions, such as psoriasis, Reiter disease, rheumatoid arthritis, and gouty arthritis. Radiographically, diffuse idiopathic skeletal hyperostosis (DISH) has a similar appearance to ankylosing spondylitis; however, DISH typically occurs at a later age and does not involve the sacroiliac joint.

The radiographic changes usually first appear in the sacroiliac joints, followed by the thoracolumbar and lumbosacral spine; this is in line with the natural progression of the disease. The disease then proceeds cephalad up the spine; however, the cervical spine may also be affected without involvement of the thoracic or lumbar spine. Radiographically evident peripheral-joint abnormalities are seen in more than 50% of patients. Abnormalities can also be seen in the symphysis pubis and in the manubriosternal, sternoclavicular, and temporomandibular joints. Spinal findings include osteitis, syndesmophytosis, diskovertebral erosions and destruction (Romanus lesions), and disk calcification. Radiographically, joint involvement appears as joint-space narrowing, periostitis, osseous erosion, and minimal periarticular osteoporosis (less than that seen with rheumatoid arthritis). Sacroiliac joint involvement is usually bilateral and symmetric.

Common laboratory findings are an elevated erythrocyte sedimentation rate (during the acute phase), a positive HLA-B27 histocompatibility antigen, mild leukocytosis, normochromic normocytic anemia (anemia of chronic disease), and negative results for rheumatoid factor.

The general principles of managing chronic arthritis also apply to ankylosing spondylitis. Among the various nonsteroidal anti-inflammatory drugs (NSAIDs) available to treat the disease, indomethacin may be the most effective. The lowest dose that provides pain relief should be used in order to avoid potentially serious complications, such as gastritis, peptic ulcer disease, and renal insufficiency. Sulfasalazine can be useful if peripheral arthritis is substantial, but it may be less effective when spinal and sacroiliac pain are the most prominent symptoms. In the majority of patients, the symptoms persist for life, although in some cases remission does occur.

Physical therapy and exercise can help prevent axial immobility. Specifically, spinal extension and deep-breathing exercises maintain spinal mobility, encourage erect posture, and promote chest expansion. Maintaining an erect posture and sleeping on a firm mattress with a thin pillow can help reduce thoracic kyphosis. Severe hip or spinal involvement may require surgical repair. Antitumor necrosis factor (anti-TNF) agents, such as infliximab and etanercept, are relatively new but often very effective therapeutic agents that may be considered for patients with pain refractory to other interventions.

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