PCM SP Final - University of Arizona



PCM Fall Exam

Notes on Asthma, COPD, Cystic Fibrosis, Community-Acquired Pneumonia, Pulmonary Embolism, Mitral Stenosis

Asthma

Essentials of Diagnosis:

• Episodic or chronic symptoms of airflow obstruction: breathlessness, cough, wheezing, and chest tightness.

• Symptoms frequently worse at night or in the early morning.

• Prolonged expiration and diffuse wheezes on physical examination.

• Limitation of airflow on pulmonary function testing or positive bronchoprovocation challenge.

• Complete or partial reversibility of airflow obstruction, either spontaneously or following bronchodilator therapy.

General Considerations:

• Common disease affecting 5% of population.

• Men and women equally affected.

• 470,000 hospital admissions and 5,000 deaths in USA attributable to asthma.

• Hospitalization and death rates highest among blacks aged 15 – 24 years.

• Prevalence, hospitalizations, and fatal asthma have all increased in USA over the past 20 years.

Definitions and Pathogenesis:

• Asthma is a chronic inflammatory disorder of the airways.

• Airway inflammation underlies disease chronicity and contributes to airway hyperresponsiveness, airflow limitation, and to respiratory symptoms.

• Genetic predispostion recognized, related to atopy.

• Both specific and nonspecific precipitants exist.

Clinical Findings:

• Episodic wheezing, difficulty breathing, chest tightness, cough.

• Frequency of symptoms is highly variable.

• Symptoms may occur spontaneously or be precipitated or exacerbated by many different triggers.

• Asthma symptoms are frequently worse at night.

• Nasal mucosal swelling, increased nasal secretions, and nasal polyps are often seen in patients with allergic asthma.

• Eczema, atopic dermatitis, or other manifestations of allergic skin disorders may also be present.

• Hunched shoulders and use of accessory muscles of respiration suggest increased work of breathing.

• Chest examination may be normal between exacerbations in patients with mild asthma.

• Wheezing during normal breathing or prolonged forced expiration correlates well with the presence of airflow obstruction.

• During severe asthma wheezing may disappear, and the only diagnostic clue on auscultation may be globally reduced breath sounds with prolonged expiration.

• The evaluation for asthma should included spirometry (FEV1, FVC, FEV1/FVC) before and after the administration of a short-acting bronchodilator.

Complications:

• Exhaustion, dehydration, airway infection, cor pulmonale, tussive syncope.

• Rare pneumothorax.

• Hypercapnic and hypoxic respiratory failure in severe disease.

Differential Diagnosis:

• Upper airway disorders: vocal cord paralysis, vocal cord dysfunction syndrome, foreign body aspiration, laryngotracheal masses, tracheal narrowing, tracheomalacia, airway edema.

• Lower airway disorders: COPD, bronchiectasis, allergic brochopulmonary mycosis, cystic fibrosis, eosinophilic pneumonia, bronchiolitis obliterans.

• Systemic vasculitides: Churg-Strauss syndrome, others with pulmonary component.

• Psychiatric: conversion disorders, Munchausen syndrome, malingering.

Classification:

• Mild intermittent: Symptoms ≤ 2 times a week with normal function between exacerbations. Nighttime symptoms ≤ 2 times a month.

• Mild persistent: Symptoms > 2 times per week but < 1 time a day. Nighttime symptoms > 2 times a month.

• Moderate persistent: Daily symptoms and/or daily use of inhaled short-acting β2-agonist. Exacerbations affect activity. Nighttime symptoms > 1 time a week.

• Severe persistent: Continual symptoms. Limited physical activity. Frequent exacerbations and nighttime symptoms.

Treatment:

• Corticosteroids: most potent and consistently effective anti-inflammatory agents currently available. Inhaled agents used for long-term control and systemic agents used for prompt control of short-term exacerbations.

• Long-acting bronchodilators

• Short-acting bronchodilators: β2-agonists and others.

Approach to Treatment:

• Principal goals: correction of hypoxemia, reversal of airflow obstruction, reduction of the likelihood of recurrence of obstruction.

• Periodic assessments and ongoing monitoring of asthma are essential to determine if the goals of therapy are being met.

COPD

Essentials of Diagnosis:

• History of cigarette smoking.

• Chronic cough and sputum production (in chronic bronchitis) and dyspnea (in emphysema).

• Rhonchi, decreased intensity of breath sounds, and prolonged expiration on physical examination.

• Airflow limitation on pulmonary function testing.

General Considerations:

• COPD is a disease state characterized by the presence of airflow obstruction due to chronic bronchitis or emphysema; the airflow obstruction is generally progressive, may be accompanied by airway hyperreactivity, and may be partially reversible.

• Most patients with COPD have features of both emphysema and chronic bronchitis.

• Chronic bronchitis: characterized by excessive secretion of bronchial mucus and is manifested by productive cough for 3 months or more in at least 2 consecutive years in the absence of any other disease that might account for this symptom.

• Emphysema: abnormal, permanent enlargement of air spaces distal to the terminal bronchiole, with destruction of their walls and without obvious fibrosis.

• Cigarette smoking is clearly the most important cause of COPD.

Clinical Findings:

• Patients with COPD characteristically present in the fifth or sixth decade of life complaining of excessive cough, sputum production, and shortness of breath.

• Symptoms have often been present for 10 years or more.

• Dyspnea is often noted initially only on heavy exertion, but this exacerbates as the condition progresses.

• Clinical findings may be completely absent early in the course of COPD.

• “Pink puffers” = emphysema predominant.

• “Blue bloaters” = bronchitis predominant.

Differential Diagnosis:

• Clinical, imaging, and laboratory findings should enable the clinician to distinguish COPD from other obstructive pulmonary disorders.

• These include: bronchial asthma, bronchiectasis, cystic fibrosis, bronchopulmonary mycosis, central airflow obstruction.

Complications:

• Stable COPD: acute bronchitis, pneumonia, pulmonary thromboembolism, concomitant left ventricular failure.

• Advanced COPD: pulmonary hypertension, cor pulmonale, chronic respiratory failure.

• Spontaneous pneumothorax and hemoptysis are also possible.

Prevention:

• Elimination of chronic exposure to tobacco smoke.

• Smoking cessation.

Treatment:

• Smoking cessation.

• Oxygen therapy.

• Bronchodilators.

• Antibiotics for treating acute exacerbations.

• Lung transplantation.

Prognosis:

• Poor.

• Median survival time of patients with severe COPD is about 4 years.

• Degree of pulmonary dysfunction at the time the patient is first seen is the most important predictor of survival.

Cystic Fibrosis

Essentials of Diagnosis:

• Chronic or recurrent cough, sputum production, dyspnea, and wheezing.

• Recurrent infections or chronic colonization of the airways with nontypeable Haemophilus influenzae, mucoid and nonmucoid Pseudomonas aeruginosa, Staphylococcus aureus, or Burkholderia cepacia.

• Pancreatic insufficiency, recurrent pancreatitis, distal intestinal obstruction syndrome, chronic hepatic disease, nutritional deficiencies, or male urogenital abnormalities.

• Bronchiectasis and scarring on chest radiographs.

• Airflow obstruction on spirometry.

• Sweat chloride concentration above 60 meq/L on two occasions or mutations in genes known to cause cystic fibrosis.

General Considerations:

• Most common cause of severe chronic lung disease in young adults.

• Most common fatal hereditary disorder of Caucasians in the USA.

• Autosomal recessive disorder affecting about one in 3200 Caucasians; one in 25 is a carrier.

• Caused by abnormalities in CFTR protein that results in altered chloride transport and water flux across the apical surface of epithelial cells.

• Mutation referred to as ΔF508 accounts for 60% of CF cases.

Clinical Findings:

• CF should be suspected in a young adult presenting with a history or chronic lung disease (especially bronchiectasis), pancreatitis, or infertility.

• Cough, sputum production, decrease exercise tolerance, and recurrent hemoptysis are typical complaints.

• Patients also often complain of facial (sinus) pain or pressure and purulent nasal discharge.

• Steatorrhea, diarrhea, and abdominal pain are also common.

• Digital clubbing, increase anteroposterior chest diameter, hyperresonance to percussion, and apical crackles are noted on physical examination.

• Sinus tenderness, purulent nasal secretions, and nasal polyps may also be seen.

• Chloride sweat test used for diagnosis.

Treatment:

• Early recognition and comprehensive multidisciplinary therapy improve symptom control and the chances or survival and amelioration of symptoms.

• Tx includes: clearance and reduction of lower airway secretions, reversal of bronchoconstriction, treatment of respiratory tract infections and airway bacterial burden, pancreatic enzyme replacement, nutritional and psychosocial support.

• Lung transplantation for advanced cystic fibrosis.

Prognosis:

• Median survival age is now 31 years.

• Death occurs from pulmonary complications or as a result of terminal chronic respiratory failure and cor pulmonale.

Community-Acquired Pneumonia

Essentials of Diagnosis:

• Symptoms and signs of an acute lung infection: fever or hypothermia, cough with or without sputum, dyspnea, chest discomfort, sweats or rigors.

• Bronchial breath sound or rales are frequent auscultatory findings.

• Parenchymal infiltrate on chest radiographs.

• Occurs outside of the hospital or less than 48 hours after admission in a patient who is not hospitalized or residing in a long-term facility for more than 14 days before the onset of symptoms.

General Considerations:

• Approximately 2 – 3 million cases diagnosed each year in the USA.

• Most deadly infectious disease and sixth leading cause of death in USA.

• Mortality and morbidity risk factors include: advanced age, alcoholism, comorbid medical conditions, altered mental status, respiratory rate ≥ 30 breaths/min, hypotension, and BUN > 30 mg/dL.

Definitions and Pathogenesis:

• Pulmonary defense mechanisms (cough reflex, mucociliary clearance system, immune responses) normally prevent the development of lower respiratory tract infections following aspiration of oropharyngeal secretions containing bacteria or inhalation of infected aerosols.

• Community-acquired pneumonia occurs when there is a defect in one or more of the normal host defense mechanisms or when the infectious pathogen overwhelms the host.

• Streptococcus pneumoniae is the most common bacterial pathogen (2/3 of bacterial isolates).

• Other common bacterial pathogens include Haemophilus influenzae, Mycoplasma pneumoniae, Chlamydia pneumoniae, Staphylococcus aureus, Neisseria meningitidis, Moraxella catarrhalis, Klebsiella pneumoniae, other gram-negative rods, and legionella species.

• Viral causes of community-acquired pneumonia include influenza virus, respiratory syncytial virus, adenovirus, and parainfluenza virus.

Clinical Findings:

• Acute or subacute onset of fever, cough with or without sputum production, and dyspnea.

• Other common symptoms include rigors, sweats, chills, chest discomfort, pleurisy, fatigue, myalgias, anorexia, headache, and abdominal pain.

• Common physical findings include fever or hypothermia, tachypnea, tachycardia, and mild arterial oxygen desaturation.

• Chest examination is often remarkable for altered breath sounds and rales.

• Dullness to percussion may be present if a parapneumonic pleural effusion is present.

• Differential diagnosis includes upper respiratory tract infections, reactive airway diseases, congestive heart failure, bronchiolitis obliterans organizing pneumonia, lung cancer, pulmonary vasculitis, pulmonary thromboembolic disease, and atelectasis.

• Sputum gram stains are often used to help identify causative organisms.

• Chest radiography may confirm the diagnosis and detect associate lung diseases.

Treatment:

• Antimicrobial therapy should be indicated promptly after the diagnosis of pneumonia is established and appropriate specimens are obtained.

• Decisions regarding hospitalization should be based on prognostic criteria.

• Local resistance pattern data should guide empirical antibiotic therapy.

Prevention:

• Polyvalent pneumococcal vaccine has the potential to prevent or lessen the severity of the majority of pneumococcal infections in immunocompetent patients.

• Influenza vaccine is also an important prevention technique.

Pulmonary Embolism

Essentials of Diagnosis:

• Predisposition to venous thrombosis, usually of the lower extremities.

• Usually one of the following: dyspnea, chest pain, hemoptysis, syncope.

• Tachypnea and a widened alveolar-arterial PO2 difference.

• Characteristic defects on ventilation-perfusion lung scan, spiral CT scan of the chest, or pulmonary angiogram.

General Considerations:

• Common, serious, and potentially fatal complication of thrombus formation within the deep venous circulation.

• Estimated to cause 50,000 deaths each year in the USA and is the third leading cause of death among hospitalized patients.

• Management demands a vigilant systematic approach to diagnosis and an understanding of risk factors so that appropriate preventative therapy can be given.

• Many substances can embolize to the pulmonary circulation, and deep venous thrombi of the calf muscle circulation are the most common.

• Pulmonary embolism and deep venous thrombosis are two manifestations of the same disease.

• Risk factors include: venous stasis, injury to the vessel wall, and hypercoaguability.

Clinical Findings:

• Clinical diagnosis is notoriously difficult for two reasons: (1) the clinical findings depends on both the size of the embolus and the patient’s preexisting cardiopulmonary status; (2) common symptoms and signs of pulmonary emboli are not specific to this disorder.

• The most sensitive findings are dyspnea, pain on inspiration, and tachypnea.

• Other common findings are cough, leg pain, hemoptysis, crackles, tachycardia, and abnormal heart sounds.

• ECG abnormalities are found in 70% of PE patients.

• D-dimer testing is extremely sensitive, such that the absence of D-dimer using ELISA provides strong evidence against PE.

• Chest radiograph and perfusion scan are used together to evaluate PE.

• Spiral CT, MRI, and venous thrombosis studies can also be used.

• Pulmonary angiography remains the reference standard for the diagnosis of PE.

Prevention:

• Prophylactic intervention is extremely important, but remains underutilized.

• Mechanical devices such as compression stockings and pneumatic compression devices are utilized.

• Heparin therapy is also utilized in the hospital setting.

Treatment:

• Anticoagulation with heparin is a form of secondary prevention.

• Thrombolytic therapy for patients with established PE.

Mitral Stenosis

Essentials of Diagnosis:

• Dyspnea, orthopnea, and paroxysmal nocturnal dyspnea.

• Symptoms often precipitated by onset of atrial fibrillation or pregnancy.

• Prominent mitral first sound, opening snap (usually), and apical diastolic crescendo rumble.

• ECG shows left atrial abnormality and, commonly, atrial fibrillation. Echo-Doppler confirms diagnosis and quantifies severity.

General Considerations:

• Nearly all patients with mitral stenosis have underlying rheumatic heart disease, though a history of rheumatic fever is often absent.

Clinical Findings:

• Characteristic finding of mitral stenosis is a localized middiastolic murmur low in pitch whose duration varies with the severity of the stenosis and the heart rate.

• The valve opens in early diastole with an opening snap; the sound is sharp and widely distributed over the chest.

• Echocardiography is the most valuable technique for assessing mitral stenosis.

Treatment and Prognosis:

• Mitral stenosis may be present for a lifetime with few or no symptoms, or it may become severe in a few years.

• In most cases, there is a long asymptomatic phase, followed by subtle limitation of activity.

• Atrial fibrillation often precipitates more severe symptoms, which usually improve with control of the ventricular rate or restoration of sinus rhythm.

• Once atrial fibrillation occurs, the patient should receive warfarin anticoagulation therapy even if sinus rhythm is restored.

• Indications for relieving the stenosis include the following: (1) uncontrollable pulmonary edema; (2) limiting dyspnea and intermittent pulmonary edema; (3) evidence of pulmonary hypertension with right ventricular hypertrophy or hemoptysis; (4) limitation of activity despite ventricular rate control and medical therapy; (5) recurrent systemic emboli despite anticoagulation with moderate or severe stenosis.

• Surgery is the definite therapeutic intervention.

Arteriosclerotic heart disease AKA

Coronary Heart Disease

Genetics: Tendency is inheritable

Incidence/Prevalence in USA: Common. Causes 35% of deaths in men age 35-50. Death rate age 55-64 - 1:100.

Predominant age: Men 50-60, women 60-70, for peak clinical manifestations

Predominant sex: Male > Female

SIGNS AND SYMPTOMS:

• Variable. May remain clinically asymptomatic even in advanced disease states, eg, silent ischemia.

• Clinical manifestations

◊ Substernal chest pain

◊ Exertional dyspnea

◊ Orthopnea

◊ Paroxysmal nocturnal dyspnea

◊ Cardiac arrhythmias

◊ Systolic murmur

◊ Cardiomegaly

◊ Pedal edema

CAUSES:

• Atherosclerosis

• Narrowing of coronary arteries

• Embolism compromising coronary arteries at orifices

• Subintimal atheromas in large and medium vessels

RISK FACTORS:

• Elevated low density lipoprotein (LDL)

• Decreased high density lipoprotein (HDL)

• Elevated triglycerides

• Smoking

• Family history of premature arteriosclerosis

• Obesity

• Hypertension

• Stress

• Sedentary life style

• Increasing age

• Male sex

• Diabetes mellitus

LABORATORY:

• Elevated triglycerides

• Elevated total cholesterol

• Elevated low density lipoproteins

• Decreased high density lipoproteins

• Elevated cholesterol/HDL ratio

IMAGING:

• Angiography - narrowed coronary arteries

• Echocardiography - wall motion abnormalities

• Pharmacologic stress tests (dobutamine, dipyridamole, adenosine) - positive

• Stress thallium test - positive

[pic]TREATMENT

APPROPRIATE HEALTH CARE:

• Outpatient for management of risk factors

• Inpatient for acute ischemic syndromes

GENERAL MEASURES:

• Prevention of further progression of the disease

◊ Smoking cessation

◊ Treatment of hypercholesterolemia (diet, drugs)

◊ Increase high density lipoprotein (diet, exercise)

◊ Control of blood pressure

◊ Diabetes mellitus treated early and adequately

◊ Exercise

◊ Prophylactic aspirin

◊ Stress reduction

◊ Diet changes

◊ Weight loss

◊ Estrogen replacement therapy in postmenopausal women is currently controversial

• Treatment of complications

◊ Covered elsewhere under the individual topics (e.g., angina pectoris, myocardial infarction, heart failure, stroke, peripheral arterial occlusion, etc.)

[pic]MEDICATIONS

DRUG(S) OF CHOICE:

• Aspirin, 160-325 mg/day, unless contraindicated

• Cholesterol-lowering agents

◊ Cholestyramine or colestipol, (bile acid sequestrants) 12-32 gm orally BID-QID

◊ Niacin 2-6 gm daily in divided doses (highly efficacious, but side effects restrict use)

◊ Gemfibrozil 600 mg bid

◊ Probucol 500 mg bid

◊ Colesevelam 3.75-4.375 g/day

◊ Ezetimibe 10 mg/day

◊ HMG-CoA reductase inhibitors (dose varies with product): atorvastatin (Lipitor), fluvastatin (Lescol), lovastatin (Mevacor), pravastatin (Pravachol), simvastatin (Zocor)

PATIENT MONITORING:

Monitor cholesterol, triglyceride levels, other preventive programs (weight loss, smoking cessation)

POSSIBLE COMPLICATIONS:

• Myocardial infarction

• Ventricular fibrillation

• Congestive heart failure

• Angina pectoris

• Sudden cardiac death

EXPECTED COURSE/PROGNOSIS:

Guardedly favorable. Many risk factors can be modified.

[pic]Aortic regurgitation

DESCRIPTION:

Retrograde flow from the aorta into the left ventricle through incompetent aortic cusps. Symptoms include dyspnea, shortness of breath, palpitations, orthopnea. Usual course - acute; chronic.

SYNONYMS:

• aortic valve insufficiency

Murmur: Systolic. Aortic area, radiating to the neck, gets louder then quiter ie. Diamond shaped, medium pitch, harsh quality

Associated signs: Decreased A2, Ejection click, S4, Narrow pulse pressure, slow rising and delzyed pulse.

CAUSES:

• bacterial endocarditis, aortic dissection, ankylosing spondylitis, aortic stenosis, rheumatic fever, giant cell arteritis, syphilis, Marfan syndrome, osteogenesis imperfection, Reiter's syndrome, rheumatoid arthritis, cystic medial necrosis, sinus of Valsalva aneurysm, hypertension, arteriosclerosis, myxomatous degeneration of valve, dissection of aorta, bicuspid aortic valve

TREATMENT:

• aortic valve replacement

Aortic valvular stenosis

An acquired or congenital obstruction to systolic left ventricular outflow across the aortic valve

Incidence/Prevalence in USA:

• Except for mitral regurgitation due to myocardial disease, valvular aortic stenosis is the most common fatal cardiac valve lesion

• Bicuspid aortic valve has a frequency of 400 per 100,000 live births

Predominant age:

• Age < 30 years - predominantly congenital

• Age 30 to 70 years - most commonly congenital or rheumatic

• Age > 70 years - most commonly degenerative calcification of the aortic valve

Predominant sex:

• Congenital bicuspid valves: Male > Female (4:1)

• Congenital unicuspid valves: Male > Female (3:1)

SIGNS AND SYMPTOMS:

• Angina pectoris (most frequent symptom, occurring in 50-70% of patients with severe aortic stenosis)

• Near syncope

• Syncope (often exertional, occurs in 15-30% of patients with severe aortic stenosis)

• Exertional dyspnea

• Orthopnea

• Paroxysmal nocturnal dyspnea

• Palpitations

• Fatigue

• Neurologic events (transient ischemic attack or cerebrovascular accident) due to embolization

• Systolic crescendo-decrescendo murmur, usually best heard at the second right sternal border (may have associated thrill) and may radiate into the carotid arteries

• Ejection (early systolic) click

• Prolonged ejection time

• Delayed, small carotid upstroke

• Delayed/decreased intensity of A2

• Paradoxical splitting of S2

• Left ventricular heave

• A high pitched diastolic blow may be present at the left sternal border (associated aortic regurgitation)

CAUSES:

• Congenital etiologies

◊ Unicuspid valve

◊ Bicuspid valve (not inherently stenotic, but becomes so as a result of 'wear and tear' thickening and calcification; a calcified bicuspid valve is the most common cause of isolated aortic stenosis in adults)

◊ 3 cusped valve with fusion of commissures

◊ Hypoplastic annulus

• Acquired etiologies

◊ Rheumatic (or, rarely, other inflammatory disease)

◊ Degenerative calcific aortic stenosis in the elderly

DIFFERENTIAL DIAGNOSIS:

• Mitral regurgitation, either primary or secondary to underlying coronary artery disease or dilated cardiomyopathy. Mitral regurgitation, however, is usually an apical, high frequency, pansystolic murmur, often radiating to the axilla.

• Hypertrophic obstructive cardiomyopathy. This murmur is also a systolic crescendo-decrescendo murmur, but is best heard at the left sternal border and may radiate into the axilla. However, this murmur characteristically is intensified by moving from squatting to standing position and/or Valsalva maneuver, and lessened by changing from standing to squatting.

• Aortic supravalvular stenosis

• Discrete subaortic stenosis

• 50% incidence of concomitant coronary artery disease

SPECIAL TESTS:

ECG: Left ventricular hypertrophy, often with associated ST segment depression, conduction defects, left atrial enlargement, ventricular arrhythmias

IMAGING:

• Chest x-ray

◊ May be normal in compensated, isolated valvular aortic stenosis

◊ Cardiac hypertrophy early, later cardiomegaly

◊ Post stenotic dilatation of the ascending aorta

◊ Calcification of aortic valve cusps (may require fluoroscopy to visualize)

DIAGNOSTIC PROCEDURES:

• Echocardiography:

◊ Aortic valve morphology, thickening, calcifications

◊ Decreased aortic valve excursion

◊ Planimetry of aortic valve area

◊ Left ventricular hypertrophy

◊ Left ventricular ejection fraction

◊ Chamber dimensions

◊ Presence or absence of wall motion abnormalities suggestive of coronary artery disease

• With Doppler echocardiography:

◊ Transvalvular gradient

◊ Valve area

◊ Diastolic function

◊ Associated aortic regurgitation

• Cardiac catheterization:

◊ Transvalvular gradient

◊ Valve area

◊ Left ventricle ejection fraction

◊ Concomitant coronary artery disease

[pic]TREATMENT

APPROPRIATE HEALTH CARE:

Outpatient except for surgical intervention

GENERAL MEASURES:

• Aortic stenosis is a progressive disease. The asymptomatic patient with non-critical aortic stenosis can be closely followed with appropriate evaluation.

• All patients with valvular aortic stenosis should receive endocarditis prophylaxis, prior to dental work or invasive procedures regardless of age, etiology or severity of the stenosis (as recommended by the American Heart Association in Circulation, 1997; 96: 358-366)

• Patients with a rheumatic etiology should receive (in addition to endocarditis prophylaxis prior to dental work or invasive procedures) rheumatic fever prophylaxis, especially if less than 35 years of age, or continue to be in close contact with young children

SURGICAL MEASURES:

• Prompt aortic valve replacement is clearly indicated in patients with symptomatic severe aortic stenosis

• Balloon angioplasty of stenotic aortic valves may be of benefit in the pediatric patient with congenital disease. Also feasible (although one must expect suboptimal results) in the elderly, debilitated patient who may not tolerate valve replacement.

ACTIVITY:

In known or suspected severe aortic stenosis, vigorous physical activity is contraindicated

PATIENT EDUCATION:

• Educate the patient about the symptoms of symptomatic aortic stenosis and to report these promptly should they occur

• If moderate or severe aortic stenosis is known or suspected, instruct the patient to avoid vigorous physical activity

• Instruct the patient when prophylactic antibiotics are needed for medical or dental procedures

[pic]FOLLOWUP

PATIENT MONITORING:

• Asymptomatic patients without critical aortic stenosis should be followed with a history and physical examination every 3-6 months

• An echocardiogram should be performed every 6-12 months to assess progression

• Advise the patient to immediately report any symptoms referable to the aortic stenosis

PREVENTION/AVOIDANCE:

• Bacterial endocarditis prophylaxis

• Rheumatic fever prophylaxis, where indicated

• Avoidance of vigorous physical activity

POSSIBLE COMPLICATIONS:

• Progressive stenosis

• Sudden death

• Congestive heart failure

• Angina

• Syncope

• Hemolytic anemia

• Infective endocarditis

EXPECTED COURSE/PROGNOSIS:

• Mean life expectancy without intervention in patients with aortic stenosis is 5 years after the onset of exertional chest discomfort, 3 years after the onset of syncope, 2 years after the development of heart failure

• Sudden death occurs in 15 to 20% of patients with symptomatic aortic stenosis

ASSOCIATED CONDITIONS:

• Coronary artery disease is present in 50% of patients with aortic stenosis

• Aortic regurgitation (particularly seen in calcified bicuspid valves and rheumatic disease)

• Mitral valve disease (primarily in rheumatic heart disease)

Aortic valvular stenosis

DESCRIPTION:

An acquired or congenital obstruction to systolic left ventricular outflow across the aortic valve

Incidence/Prevalence in USA:

• Except for mitral regurgitation due to myocardial disease, valvular aortic stenosis is the most common fatal cardiac valve lesion

• Bicuspid aortic valve has a frequency of 400 per 100,000 live births

Predominant age:

• Age < 30 years - predominantly congenital

• Age 30 to 70 years - most commonly congenital or rheumatic

• Age > 70 years - most commonly degenerative calcification of the aortic valve

Predominant sex:

• Congenital bicuspid valves: Male > Female (4:1)

• Congenital unicuspid valves: Male > Female (3:1)

SIGNS AND SYMPTOMS:

• Angina pectoris (most frequent symptom, occurring in 50-70% of patients with severe aortic stenosis)

• Near syncope

• Syncope (often exertional, occurs in 15-30% of patients with severe aortic stenosis)

• Exertional dyspnea

• Orthopnea

• Paroxysmal nocturnal dyspnea

• Palpitations

• Fatigue

• Neurologic events (transient ischemic attack or cerebrovascular accident) due to embolization

• Systolic crescendo-decrescendo murmur, usually best heard at the second right sternal border (may have associated thrill) and may radiate into the carotid arteries

• Ejection (early systolic) click

• Prolonged ejection time

• Delayed, small carotid upstroke

• Delayed/decreased intensity of A2

• Paradoxical splitting of S2

• Left ventricular heave

• A high pitched diastolic blow may be present at the left sternal border (associated aortic regurgitation)

CAUSES:

• Congenital etiologies

◊ Unicuspid valve

◊ Bicuspid valve (not inherently stenotic, but becomes so as a result of 'wear and tear' thickening and calcification; a calcified bicuspid valve is the most common cause of isolated aortic stenosis in adults)

◊ 3 cusped valve with fusion of commissures

◊ Hypoplastic annulus

• Acquired etiologies

◊ Rheumatic (or, rarely, other inflammatory disease)

◊ Degenerative calcific aortic stenosis in the elderly

RISK FACTORS:

Prior rheumatic fever

DIFFERENTIAL DIAGNOSIS:

• Mitral regurgitation, either primary or secondary to underlying coronary artery disease or dilated cardiomyopathy. Mitral regurgitation, however, is usually an apical, high frequency, pansystolic murmur, often radiating to the axilla.

• Hypertrophic obstructive cardiomyopathy. This murmur is also a systolic crescendo-decrescendo murmur, but is best heard at the left sternal border and may radiate into the axilla. However, this murmur characteristically is intensified by moving from squatting to standing position and/or Valsalva maneuver, and lessened by changing from standing to squatting.

• Aortic supravalvular stenosis

• Discrete subaortic stenosis

PATHOLOGICAL FINDINGS:

• Left ventricular hypertrophy

• Myocardial interstitial fibrosis

• Aortic valvular calcification in older patients

• 50% incidence of concomitant coronary artery disease

SPECIAL TESTS:

ECG: Left ventricular hypertrophy, often with associated ST segment depression, conduction defects, left atrial enlargement, ventricular arrhythmias

IMAGING:

• Chest x-ray

◊ May be normal in compensated, isolated valvular aortic stenosis

◊ Cardiac hypertrophy early, later cardiomegaly

◊ Post stenotic dilatation of the ascending aorta

◊ Calcification of aortic valve cusps (may require fluoroscopy to visualize)

DIAGNOSTIC PROCEDURES:

• Echocardiography:

◊ Aortic valve morphology, thickening, calcifications

◊ Decreased aortic valve excursion

◊ Planimetry of aortic valve area

◊ Left ventricular hypertrophy

◊ Left ventricular ejection fraction

◊ Chamber dimensions

◊ Presence or absence of wall motion abnormalities suggestive of coronary artery disease

• With Doppler echocardiography:

◊ Transvalvular gradient

◊ Valve area

◊ Diastolic function

◊ Associated aortic regurgitation

• Cardiac catheterization:

◊ Transvalvular gradient

◊ Valve area

◊ Left ventricle ejection fraction

◊ Concomitant coronary artery disease

TREATMENT

GENERAL MEASURES:

• Aortic stenosis is a progressive disease. The asymptomatic patient with non-critical aortic stenosis can be closely followed with appropriate evaluation.

• All patients with valvular aortic stenosis should receive endocarditis prophylaxis, prior to dental work or invasive procedures regardless of age, etiology or severity of the stenosis (as recommended by the American Heart Association in Circulation, 1997; 96: 358-366)

• Patients with a rheumatic etiology should receive (in addition to endocarditis prophylaxis prior to dental work or invasive procedures) rheumatic fever prophylaxis, especially if less than 35 years of age, or continue to be in close contact with young children

SURGICAL MEASURES:

• Prompt aortic valve replacement is clearly indicated in patients with symptomatic severe aortic stenosis

• Consider aortic valve replacement in asymptomatic patients with critical aortic stenosis (aortic valve area < 1.0 cm2 or gradient > 50 mm Hg [> 6.6 kPa]) particularly if there is left ventricular dysfunction, increasing cardiomegaly, and clinical symptoms

• Surgical valve replacement consists of the removal of the stenotic, native valve and placement of a prosthetic mechanical or tissue valve

• Balloon angioplasty of stenotic aortic valves may be of benefit in the pediatric patient with congenital disease. Also feasible (although one must expect suboptimal results) in the elderly, debilitated patient who may not tolerate valve replacement.

ACTIVITY:

In known or suspected severe aortic stenosis, vigorous physical activity is contraindicated

DIET:

No restrictions except sodium restriction in presence of congestive heart failure

PATIENT EDUCATION:

• Educate the patient about the symptoms of symptomatic aortic stenosis and to report these promptly should they occur

• If moderate or severe aortic stenosis is known or suspected, instruct the patient to avoid vigorous physical activity

• Instruct the patient when prophylactic antibiotics are needed for medical or dental procedures

MEDICATIONS

DRUG(S) OF CHOICE:

• None for treatment. Prophylactic antibiotics when needed.

• The use of vasodilators, nitrates, calcium channel blockers, beta blockers as well as diuretics are potentially hazardous in aortic stenosis and should be used cautiously, if at all

[pic]FOLLOWUP

PATIENT MONITORING:

• Asymptomatic patients without critical aortic stenosis should be followed with a history and physical examination every 3-6 months

• An echocardiogram should be performed every 6-12 months to assess progression

• Advise the patient to immediately report any symptoms referable to the aortic stenosis

PREVENTION/AVOIDANCE:

• Bacterial endocarditis prophylaxis

• Rheumatic fever prophylaxis, where indicated

• Avoidance of vigorous physical activity

POSSIBLE COMPLICATIONS:

• Progressive stenosis

• Sudden death

• Congestive heart failure

• Angina

• Syncope

• Hemolytic anemia

• Infective endocarditis

EXPECTED COURSE/PROGNOSIS:

• Mean life expectancy without intervention in patients with aortic stenosis is 5 years after the onset of exertional chest discomfort, 3 years after the onset of syncope, 2 years after the development of heart failure

• Sudden death occurs in 15 to 20% of patients with symptomatic aortic stenosis

[pic]MISCELLANEOUS

ASSOCIATED CONDITIONS:

• Coronary artery disease is present in 50% of patients with aortic stenosis

• Aortic regurgitation (particularly seen in calcified bicuspid valves and rheumatic disease)

• Mitral valve disease (primarily in rheumatic heart disease)

AGE RELATED FACTORS:

Pediatric: N/A

Geriatric: Increased incidence of degenerative calcific aortic stenosis

Others: N/A

PREGNANCY:

Severe critical aortic stenosis tolerates poorly the hemodynamic changes in pregnancy, labor and delivery. Pregnancy should be avoided with critical aortic stenosis.

SYNONYMS: N/A

ICD-9-CM:

424.90 Endocarditis, valve unspecified, unspecified cause

SEE ALSO:

OTHER NOTES:

As the left ventricle is relatively noncompliant in aortic stenosis, atrial contraction is an important component of diastolic filling. The loss of this component with the onset of atrial fibrillation can cause acute clinical and hemodynamic deterioration.

[pic]

Mitral regurgitation due to papillary muscle dysfunction

DESCRIPTION:

Retrograde blood flow from the left ventricle in the left atrium through an incompetent mitral valve. Characteristics - fatigue, orthopnea, systolic murmur, left ventricular hypertrophy, S3 gallop. Usual course: chronic; progressive; acute (after myocardial infarction).

Systems(s) affected:

Cardiovascular

CAUSES:

• coronary artery disease

• infiltrative diseases

• cardiac tumors

TREATMENT:

• dental endocarditis prophylaxis

• surgical endocarditis prophylaxis

• nitrates

• calcium channel blockers

• diuretics

• digitalis

• inotropic agents

• afterload reducing agents

• mitral valve replacement

Mitral regurgitation due to rheumatic fever

DESCRIPTION:

Retrograde blood flow from the left ventricle into the left atrium through an incompetent mitral valve. Characteristics - history of rheumatic fever, fatigue, dyspnea, holosystolic murmur, left ventricular hypertrophy. Usual course - chronic; progressive disability.

Systems(s) affected:

Cardiovascular

CAUSES:

• autoimmune cross reaction between streptococcal antigens and heart tissue

TREATMENT:

• medical endocarditis prophylaxis

• dental endocarditis prophylaxis

• surgical endocarditis prophylaxis

• afterload reducing agents

• nitrates

• calcium channel blockers

• digitalis

• diuretics

• mitral valve replacement

Angina Pectoris

General Considerations

• Precordial chest pain, usually precipitated by stress or exertion, relieved rapidly by rest or nitrates.

• Electrocardiographic or scintigraphic evidence of ischemia during pain or stress testing.

• Angiographic demonstration of significant obstruction of major coronary vessels

• Usually due to atherosclerotic heart disease

o Congenital abnormalities

o Emboli

o Arteritis

o Dissection

o Severe myocardial hypertrophy

o Severe aortic stenosis or regurgitation

o Increased metabolic demands (i.e. hyperthyroidism, marked anemia, paroxysmal tachycardias with rapid ventricular rates)

• History

o Diagnosis depends principally upon the hx

o Circumstances that precipitate and relieve angina

o Characteristics of the discomfort

▪ Sensation of tightness, squeezing, burning, pressing, chocking, aching, bursting, “gas”, indigestion or an ill-characterized discomfort

o Location and radiation

▪ Behind or slightly to the left of the sternum

▪ Radiates to left shoulder and upper arm, lower jaw, back of neck, high in left back

▪ Location can vary widely in pts

o Durations of attacks

▪ Short duration and subsides completely w/o residual discomfort

▪ Attacks more than 30 minutes are unusual and suggest the development of unstable angina, MI, or an alternative dx

o Effect of nitroglycerin

▪ Dx of angina is strongly supported if sublingual nitroglycerine invariable shortens an attack and if prophylactic nitrates permit greater exertion or prevent angina entirely

o Risk factors – see coronary artery disease

• Signs

o Examination during an attack

▪ Significant elevation in systolic and diastolic BP, although hypotension may occur

▪ Gallop rhythm

▪ Apical systolic murmur

▪ Arrhythmias

• Differential Diagnosis

o Anterior chest wall syndrome – reproducible by local pressure

o Intercostals neuritis (due to herpes zoster, DM, etc)

o Cervical or thoracic spine disease involving the dorsal roots

o Peptic ulcer

o Chronic cholecystitis

o Esophageal spasm

o Functional GI disease

o Reflux esophagitis

o Degenerative & inflammatory lesions of left shoulder

o Thoracic outlet syndromes

o Spontaneous pneumorthorax

o Pneumonia

o Pulmonary embolization

o Thoracic aorta dissection

• Evaluation of pts w/ angina pectoris

o Laboratory

▪ Serum lipid levels

▪ Anemia

▪ Diabetes

o ECG

▪ Normal in 25%

▪ Old MI, nonspecific ST-T changes

o Exercise Electrocardiography

• Scintigraphic Assessment of Ischemia

o Myocardial perfusion scintigraphy

o Radionuclide angiography

o Positron emission tomography

• Echocardiography

• Never Imagine Modalities

o CT

o MRI

• Ambulatory ECG monitoring

• Coronary Angiography

Treatment

• Acute Attack

o Sublingual nitroglycerin – acts in 1-2 minutes

o Nitroglycerin buccal spray

o May be repeated in 3-5 minute intervals

o Pain not responding to 3 doses or lasting more than 20 minutes may represent evolving infarction and pts should seek immediate medical attention

• Prevention of further attacks

o Avoiding of aggravating factors

o Nitroglycerin

o Long-acting nitrates

▪ Isosorbide dintirate

▪ Isosorbide mononitrate

▪ Oral sustained-release nitroglycerin preparations

▪ Nitroglycerin ointment

▪ Nitroglycerin patches

o Beta-blockers

o Calcium channel blocking agents

o Platelet inhibiting agents

o Risk reduction

o Revasculariztion

Prognosis

• In pts with stable sx’s and normal ejection fraction, mortality is 1-2% per year

• Outlook in individual pts is unpredictable, nearly half of deaths are sudden

• Accelerating sx’s have a poorer outlook

• Poor exercise tolerance and extensive ischemia have more severe disease and a poorer prognosis

Acute Myocardial Infarction

General Considerations

• Sudden but not instantaneous development of prolonged (>30 minutes) anterior chest discomfort (sometimes felt as “gas” or pressure) that may produce arrhythmias, hypotension, shock, or cardiac failure

• Rarely painless, masquerading as acute congestive heart failure, syncope, stroke, or shock

• ECG: ST segment elevation or depression, evolving Q waves, symmetric inversion of T waves

• Elevation of cardiac enzymes (CK-MB, troponin, T, or troponin I)

• Appearance of segmental wall motion abnormality by imagine techniques

Clinical Findings

• Symptoms

o Premonitory pain – alteration in the pattern of angina, unusual “indigestion” or pressure or squeezing felt in the chest

o Pain of infarction – more common at rest and in early morning

▪ Nitroglycerin has little effect; even opiates may not relieve the pain

o Associated Symptoms - cold sweat, weakness, apprehensiveness, restless – seeking position of comfort, light-headed, syncope, dyspnea, orthopnea, cough, wheezing, n&v, abdominal bloating

o Painless infarction – in minority of cases, pain is absent or minor and is overshadowed by the immediate complications

o Sudden death & arrhythmias – approximately 20% of pts with AMI will die before reaching the hospital; usually in the first hour and chiefly due to ventricular fibrillation

• Signs

o General – anxiousness, sweating profusely, HR maybe high or low, BP maybe be high (esp. in HTN) or low due to shock, respiratory distress usually indicates heart failure, fever may appear after 12 hours and persist for several days

o Chest – basilar rales, more extensive rales or diffuse wheezing suggests pulmonary edema

o Heart – may be unimpressive or very abnormal, JVD, atrial gallops, ventricular gallops, pericardial friction rubs

o Extremities – edema is usually not present, cyanosis and cold temperature indicate low output. Peripheral pulses should be noted

• Laboratory findings

o Cardiac sensitive markers of myocardial damage

▪ CK-MB

▪ Troponin I & T

• Electrocardiography – normal tracing is rare

o Peaked T waves

o ST segment elevation or depression

o T wave inversion

o New Q waves

• Chest x-ray

o Signs of CHF often lag behind the clinical findings

o Signs of aortic dissection should be sought as a possible alternative dx

• Echocardiography – normal wall motion makes an infarction unlikely

• Scintigraphic studies

Treatment

• Aspirin (clopidogrel in case of allergy)

• Thrombolytic therapy- greatest benefit in first 3 hours

• Acute coronary angiography and stenting

• CCU monitoring

• Low dose oxygen therapy

• Analgesia, morphine, meperidine

• Beta-blockers

• Nitrates

• ACE inhibitors

• Antiarrhythmic prophylaxis

• Calcium channel blockers

• Anticoagulation

Congestive Heart Failure

General Considerations

• Left ventricular failure: exertional dyspnea, cough, fatigue, orthopnea, paroxysmal nocturnal dyspnea, cardiac enlargement, rales, gallop rhythm, pulmonary venous congestion

• Right ventricular failure: elevated venous pressure, hepatomegaly, dependent edema, usually due to left ventricular failure

• Assessment of left ventricular function is a crucial part of dx and tx

Clinical Findings

• Symptoms

o SOB - exertional dysnea, orthopnea, paroxysmal nocturnal dyspnea, rest dyspnea

o Chronic non-productive cough, worse in the recumbent position

o Nocturia

o Fatigue and exercise intolerance

o RUQ pain, passive congestion of liver

o Loss of appetite and nausea due to edema of the gut or impaired GI perfusion and peripheral edema

o Pulmonary edema

o Acute exacerbations

▪ Alternations in therapy (or pt noncompliance)

▪ Excessive salt & fluid intake

▪ Arrhythmias

▪ Excessive activity

▪ Pulmonary emboli

▪ Infection

▪ Propagation of the underlying disease

• Signs

o May appear comfortable at rest

o Dyspneic during conversation

o Vital signs may be normal – tachycardia, hypotension, reduced pulse pressure may be present

o Cold extremities

o Diaphoresis

o JVD

o Rales / crackles

o Hyperthyroidism or hypothyroidism

o Ascites

o Peripheral pitting edema

o Sustained left ventricular impulse – dilation & hypertrophy

• Laboratory findings

o Anemia

o Renal insufficiency

o Electrolytes – hyponatremia

o Hemochromatosis

• ECG & Chest x-ray

o Underlying or second arrythmia

o Size & shape of heart

o Pleural effusions, bilateral or right-sided

• Cardiac catheterization

o Valvular disease

o CAD

Pharmacologic Treatment

• Correction of reversible causes – valvular lesions, myocardial ischemia, arrhythmias (persistant tachycardias), alcohol or durg induced myocardial depression, intracardial shunts, high-output states, calcium channel blockers, antiarrhythmic drugs, NSAIDs, hemochromatosis, sarcoidosis, amyloidosis

• Diet and activity – salt restricted, restriction of activity.

o Stable pts – regular exercise regimen

• Diuretic therapy

• ACE inhibitors, Angiotensin II receptor, spironolactone

• Beta-blockers

• Digitalis glycosides

• Vasodilators

o Nitrates

o Hydalazine

o Alpha-adrenergic blockers

• Positive inotropic agents

• Calcium channel blockers

• Anticoagulation

• Antiarrhythmic therapy

Nonpharmacologic treatment

• Case management and exercise training

• Coronary revascularization

• Cardiac transplantation

Prognosis

• Poor, annual mortality rates ranging from 5% in stable pts to 30-50% in pts with advanced, progressive sx’s

• Poorer prognosis – severe left ventricular dysfunction, prominent symptoms, limitation of exercise capacity, secondary renal insufficiency, hyponatremia, elevated plasma catecholamine levels

Peptic Ulcer Disease

General Considerations

• Hx of nonspecific epigastric pain present in 80-90% of pts with variable relationship to meals

• Ulcer sx’s characterized by rhythmicity and periodicity

• Ten to 20% of pts present with ulcer complications w/o antecedent sx’s

• Of NSAID-induced ulcers, 30-50% are asymptomatic

• Upper endoscopy with antral bx for H. pylori is diagnostic procedure of choice in most pts

• Gastric ulcer bx or documentation of complete healing necessary to exclude gastric malignancy

• Slightly more common in men that women

• Can occur in any age group, more commonly occur between 30-55 (duodenal) 50-75 (gastric)

Etiology

• NSAIDs, H. Pylori, Zollinger-Ellison

Clinical Findings

• Symptoms & Signs

o Epigastric pain – gnawing, dull, aching or hunger-like

o Relief with food or antacids and recurrence 2-4 hours later

o 2/3 of duodenal and 1/3 gastric ulcers cause nocturnal pain that awakens pts

o Nausea & anorexia may occur

o Significant vomiting and weight loss are unusual w/ uncomplicated ulcer disease and suggest gastric outlet obstruction or gastric malignancy

o Mild. Localized epigastric tenderness to deep palpation may be present

o Fecal occult blood testing is positive in 1/3 of pts

o “Coffee grounds” emesis, hematemesis, melena, or hematochezia

• Laboratory findings

o Anemia

o Leukocytosis

o Serum amylase

• Endoscopy

o Procedure of choice for dx

o Bx for H. pylori

o 3-5% of gastric ulcers prove to be malignant

• Imaging

o Barium upper GI series for uncomplicated pts with dyspepsia

o Reevaluation with endoscope after 8-12 weeks of therapy

Differential Dx

• Acute pancreatitis, acute cholecystitis, choledocholithiasis, esophageal rupture, gastric volvulus, ruptured aortic aneurysm

Treatment

• PPI, H2 receptor antagonists, sucralfate, bismuth, misoprostol

• Antibiotics for H. pylori

• Balanced meals at regular intervals

• Moderate EtOH intake is not harmful

• Smoking retards healing and is discouraged

Appendicitis

General Considerations

• Early: periumbilical pain; later, RLQ pain and tenderness

• Anorexia, N&V, obstripation

• Low-grade fever and leukocytosis

• Most common abdominal surgical emergency

• Most commonly between ages of 10-30

• If untreated, gangrene and perforation develop w/in 36h

Clinical Findings

• Symptoms and signs

o Vague colicky periumbilical or epigastric pain

o Pain shift to RLQ, manifested as a steady ache that is worsened by walking or coughing

o Nausea and one or two episodes of vomiting

o Protracted vomiting or vomiting before the pain suggests another dx

o A sense of constipation is typical

o Local tenderness and guarding

o Psoas sign and obturator sign

• Laboratory – moderate leukocytosis (10,000-20,000 WBC)

• Imaging – none necessary with typical appendicitis

o Ultrasound has dx accuracy of over 85% - good for younger women

o Abdominal CT

Differential Diagnosis

• Appendicitis should be considered in the DD of all pts with abdominal pain

• Gastroenteritis (typically with NVD)

• Gynecological disorders

o Salpingitis

o Tubo-ovarian abscess

o Tubal pregnancy

o Ureteral colic

• Pyelonephritis

• Diverticulitis

• Perforated colonic cancer

• Crohn’s ileitis

• Perforated peptic ulcer

• Cholecystitis

• Mesenteric adenitis

• Meckel’s diverticulitis

Treatment

• SURGERY! YEAH!!

• Systemic antibiotics

Prognosis – mortality is extremely low even with perforated appendicits, mortality is only 0.02% though it reaches 15% in the elderly

Irritable Bowel Syndrome

General Considerations

• Chronic functional disorder characterized by abdominal pain, alternations in bowel habits

• Limited evaluation to exclude organic causes of symptoms

• Symptoms usually begin in late teens to early 20s

• Chronic lower abdominal sx’s and bowel complaints that may be continuous or intermittent

• Discomfort or pain

o Relieved with defecation

o Onset associated with a change in frequency of stool

o Onset associated with a change in form of stool

• Abnormal stool frequency, form, passage, feeling of incomplete evaculation, passage of mucus, bloating or a feeling of abdominal distention

• Other complaints: dyspepsia, heartburn, chest pain, fatigue, urologic dysfunction, gynecologic symptoms, anxiety, depression

• Pathogenesis

o Abnormal motility

o Heightened visceral nociception

o Psychosocial abnormalities

Clinical Findings

• Signs & Symptoms

o Late teens to early 20s, sx’s present for at least 3 months

o Abdominal distention

o Abdominal pain relieved by defecation

o More frequent stools with onset of abdominal pain

o Looser stools with onset of pain

o Intermittent, crampy lower abdominal pain

o Relieved by defectaion, worsened by stress, and worse for 1-2h after each meal

o Problems with constipation, diarrhea, alternating constipation & diarrhea

o Physical exam is usually normal – possible abdominal tenderness in lower abd.

o New onset of sx’s in a pt over 50 warrants further examination

• Laboratory findings and special examinations

o CBC, serum albumin, SED rate, stool occult blood

o Thyroid function tests

o Stool O&P

o 24-h stool collection – daily stool in excess of 300g/d is atypical for this dx

o in pts under 40, flexible sigmoidoscopy should be performed

o pts over 40 who have not had a previous evaluation, barium enema or colonoscopy should be considered

• Differential Diagnosis

o Colonic neoplasia

o Inflammatory bowel disease

o Causes of Chronic constipation

o Causes of Chronic diarrhea

o Endometriosis

o Lactase deficiency

o Depression & anxiety

• Treatment

o Reassurance

o Diet

• Food diary

• Lactose intolerance

• Sorbital intolerance

• Trial of high fiber

o Antispasmodic agents

o Antidiarrheal agents

o Anticonstipation agents

o Psychotropic agents

• Depression

• Anxiety

• Prognosis

o Majority learn to cope with their sx’s and lead productive lives

PCM SP Final

Notes on Inflammatory Bowel Disease (Crohn's and UC), Colon Polyps, Viral Hepatitis, Acute Pancreatitis, Ectopic Pregnancy

Inflammatory Bowel Disease (Crohn's and UC)

- IBD includes ulcerative colitis & Crohn’s disease, and although these appear to be distinct entities, same pharmacologic agents are used to treat both (see below)

Crohn’s Disease – a chronic, recurrent disease characterized by patchy transmural inflammation involving any segment of the GI tract from the mouth to the anus

Hallmarks

• Insidious onset

• Intermittent bouts of low-grade fever, diarrhea, RLQ pain

• RLQ mass & tenderness

• Perianal disease with abscess, fistulas

• Xray evidence of ulceration, structuring, or fistulas of SI

History: focus on history of fever, constitutional signs, abdominal pain, # of liquid bowel movements per day, prior surgical resections

PE: focus on patient’s temperature, weight, nutritional status, presence of abdominal tenderness/mass, rectal examination, extraintestinal manifestations

Common Clinical Constellations

• Chronic inflammatory disease – most common presentation, low grade fever, malaise, weight loss, loss of energy; there may be intermittent, nonbloody diarrhea; cramping or steady RLQ or periumbilical pain; PE reveals focal tenderness (usually RLQ); a palpable, tender mass that represents thickened or matted loops of inflamed intestine, RLQ

• Intestinal obstruction – narrow of small bowel as a result of inflammation, spasm, or fibrotic stenosis; postprandial bloating, cramping pains, loud borborygmi (def: A rumbling noise produced by the movement of gas through the intestines)

• Fistulization with or w/o infection – subset of patients develop sinus tracts penetrating through the bowel and forming fistulas to a number of locations; fistulas to the mesentery usually asymptomatic but can manifest as fever, chills, tender abd mass, leukocytosis; fistulas from the colon to SI or stomach can result in bacterial overgrowth with diarrhea, weight loss, malnutrition; fistulas to bladder/vagina produce recurrent infections

• Perianal disease – anal fissures, perianal abscesses, fistulas

• Extraintestinal manifestations – erythema nodosum, pyoderma gangrenosum (def: presence of boggy, purplish ulcers with undermined borders, appearing mostly on the legs), episcleritis (def: inflammation of the sclera of the eye), thromboembolic events, arthritis, oral lesions, increased risk of gallstones

Lab Findings/Labs to Order

• Poor correlation between lab studies and clinical picture

• CBC and serum albumin should be obtained – look for anemia, leukocytosis, hypoalbuminemia

• Erythrocyte sedimentation rate, C-Reactive protein – elevated during active inflammation

• Stool samples – examine for routine pathogens, ova, parasites, and C difficile toxin

Diagnostic Studies

• Initial diagnosis of Crohn’s based upon clinical picture with supporting radiographic findings

• Upper GI series with small bowel follow-through – look for ulceration, strictures, fistulas

• Barium enema or colonoscopy – evaluate colon; typical endoscopic findings include ulcers, strictures, segmental involvement (areas of normal appearing mucoso next to inflamed mucosa, see Robbins)

• Useful serologic tests – antineutrophil cytoplasmic antibodies with perinuclear staining (pANCA) found in 60-70% of pts with Crohn’s, 5-10% with UC…. Antibodies to yeast S cerevisiae (ASCA) found in about the same %

Differential

• Irritable bowel syndrome – normal x-rays, abd pain and diarrhea

• Acute appendicitis – acute fever, RLQ pain

• Intestinal lymphoma – fever, pain, weight loss, xrays may mimic Crohn’s disease

• Undiagnosed AIDS – fever, diarrhea

Treatment

• Well balanced diet; advise pt according to presentation (i.e. fiber supplementation if mainly colonic involvement; no raw fruits/veg, popcorn, nuts if obstructive symptoms; iron/B12 supplements may be needed)

• Symptomatic treatment of diarrhea – cholestyramine 2-4 g or colestipol 5 g, 2 or 3 times daily before meals

• 5-aminosalicyclic acid agents – sulfasalazine, oral mesalamine agents, etc; unclear mechanism of anti-inflam affects

• corticosteroids

• mercaptopurine/azathioprine – use for refractory Crohn’s/UC

•

Intestinal lymphoma – fever, pain, weight loss, xrays may mimic Crohn’s disease

Ulcerative Colitis – chronic, recurrent disease characterized by diffuse mucosal inflammation involving only the colon, typically involves rectum extending proximally

Hallmarks

• Bloody diarrhea

• Lower abdominal cramps and fecal urgency

• Anemia, low serum albumin

• Negative stool cultures

• Sigmoidoscopy is the key to diagnosis

History: ask about stool frequency, presence and amount of rectal bleeding, cramps, abd pain, fecal urgency, and tenesmus (def: A painfully urgent but ineffectual attempt to urinate or defecate)

PE: focus on patient’s volume status (orthostatic BP, pulse), nutritional status; on abd exam look for tenderness & evidence of peritoneal inflammation; red blood may be present on rectal exam

3 Stages of Disease

• Mild to moderate – gradual onset of infrequent diarrhea (>5 movements per day) with intermittent rectal bleeding and mucus; stools may be formed too loose in consistency; fecal urgency and tenesmus; LLQ cramps relived by defecation; no significant abd tenderness; pts with moderate disease have increased symptoms (i.e. abd tenderness, more severe diarrhea, etc)

• Severe – more than six to ten bloody movements per day, severe anemia, hypovolemia, impaired nutrition with hypoalbuminemia; abd pain and tenderness present; look for rapidly worsening symptoms with signs of toxicity (known as Fulminant colitis)

• Extracolonic manifestations – same as Crohn’s

Lab Findings/Labs to Order

• Get hematocrit, Erythrocyte sedimentation rate, serum albumin

• Stool sample – to rule out infectious colitis

Diagnostic Studies

• Sigmoidoscopy – gold standard for establishing diagnosis of UC; mucosal appearance characterized by edema, friability, mucous, erosions

• Plain abd xrays – look for significant colonic dilation

Differential

• Idiopathic ulcerative colitis – initial presentation indistinguishable from other causes of colitis

• Infectious colitis – get stool results to rule out

• CMV colitis – immunocompromised pts

• Ischemic colitis – may involve rectosigmoid in elderly pts with cardiovascular disease

Treatment

• 2 main goals are to terminate the acute, symptomatic attack and to prevent recurrence of attacks

• same options as with Crohn’s disease, treatments depends on severity of UC

• surgical therapy – for patients with severe disease who fail to improve after 7-10 days corticosteroid therapy, or with fulminant disease or toxic megacolon

Colon Polyps – discrete mass lesions that protrude into the lumen of the colon, may be nonfamilial adenomatous polyps or familial adenomatous polyposis

Hallmarks

• most pts are completely asymptomatic

• chronic occult blood loss may lead to iron deficiency

• large polyps may ulcerate, resulting in intermittent hematochezia

Tests

• fecal occult blood testing – detect ................
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