Clinical Medicine and Surgery I · Exam 5 — Clin Med Pro Guide

★ Virchow triad (venous stasis, hypercoagulable state, endothelial trauma) and the Wells criteria for deep vein thrombosis (outpatient and emergency department only; low or moderate score: D-dimer first; high score of 3 or more: skip the D-dimer and order the ultrasound) were the two items she stressed hardest; she said to know the risk factors best because the Wells items come straight from them; pulmonary embolism is the biggest complication.

Name of Condition

Deep vein thrombosis (DVT), a form of venous thromboembolism (VTE) Slides 46, 63

Definition

Clot formation in the deep veins, usually in the legs. Venous thromboembolism (VTE) is the umbrella term for deep vein thrombosis (DVT) or pulmonary embolism (PE, a clot that has traveled to the lungs) Slides 46, 63

Etiology (cause)

★ Virchow triad: three big overarching categories that cause a clot: ★ venous stasis (alterations in blood flow), ★ hypercoagulable state (inherited or acquired) and ★ endothelial trauma (vascular endothelial injury). Upper-extremity DVT is most commonly due to pacemakers, internal cardiac defibrillators or indwelling central venous catheters Slides 47, 49

Epidemiology (who)

Lower-extremity DVT is about 10 times more common than upper-extremity DVT, which makes up only 5% to 10% of all DVTs (incidence rising annually). Pulmonary embolism (PE) occurs in up to 6% of upper-extremity DVTs versus 15% to 30% of lower-extremity DVTs. Lower-extremity DVT usually begins in the calf and moves proximally to the popliteal, femoral and iliac veins. Most common proximal veins: femoral and popliteal (thigh); most common distal veins: peroneal (calf); during pregnancy: pelvic veins. Risk is higher over age 60 and slightly higher in males. Contested on the slides: about 50% of patients with a DVT have an occult (unrecognized) PE and about 30% of patients with a PE have a DVT; the second figure looks low against the published literature, so both are the slide's figures and not settled facts Slides 47, 48, 50

Risk Factors

★ Virchow triad, as the slide lists them. ★ Venous stasis: immobilization (long flights, prolonged sitting, postoperative inactivity; the slide gives about a 20-fold increased risk), venous insufficiency, heart failure. ★ Hypercoagulable states: inherited (factor V Leiden, which she called the most common inherited state; prothrombin gene mutation; protein C or S deficiency; antithrombin deficiency) or acquired (cancer; oral contraceptive pill, especially with tobacco use or obesity; postmenopausal estrogen replacement; pregnancy). ★ Endothelial trauma: trauma and burns; surgery (especially knee or hip replacement, or hip fracture); intravenous (IV) drug use (lower-extremity injection); smoking; hypertension. Other risk factors: age over 60; overweight or obesity; heart failure; inflammatory bowel disease (Crohn disease, ulcerative colitis); personal or family history of DVT or PE (a prior DVT or PE means 30 times higher risk of recurrence); slightly higher risk in males; chemotherapy; chronic kidney disease; chronic obstructive pulmonary disease; air travel; estrogen-containing contraceptives; first 6 to 12 weeks postpartum; sedentary lifestyle Slides 49, 50

Pathology

A thrombus forms in a deep vein, usually of the leg, when the Virchow triad is met (stasis, hypercoagulability, endothelial injury). A lower-extremity DVT starts in the calf and propagates upstream to the popliteal, femoral and iliac veins. Part of the thrombus can break off and travel to the lungs, causing a pulmonary embolism (PE) Slides 46, 47, 72

Clinical Manifestation

Upper-extremity DVT often presents only with limb swelling and arm discomfort. Suspect a lower-extremity DVT when the leg has swelling (97% sensitive), pain (86% sensitive), warmth (72% sensitive), erythema, or a cramp or 'charley horse' in the lower calf that persists and worsens over the next few days. A positive Homan sign (calf pain on dorsiflexion of the foot) may be present but is neither sensitive nor specific. Symptoms are confined to the calf in an isolated distal DVT; a proximal DVT may involve just the calf or the whole leg Slide 51

Diagnosis

★ Wells criteria for DVT (risk stratification): used only in the outpatient and emergency department setting, not for inpatients (less useful in hospitalized patients). Items, +1 each unless stated: active cancer (treatment or palliation within 6 months); bedridden recently for more than 3 days or major surgery within 12 weeks; calf swelling more than 3 cm compared with the other leg (measured 10 cm below the tibial tuberosity); collateral (nonvaricose) superficial veins present; entire leg swollen; localized tenderness along the deep venous system; pitting edema confined to the symptomatic leg; paralysis, paresis or recent plaster (cast) immobilization of the lower extremity; previously documented DVT; and an alternative diagnosis at least as likely as DVT scores minus 2. Long travel is a risk factor but is not a Wells item. ★ Score of zero or less: low likelihood (DVT prevalence about 5%), get a D-dimer first; 1 to 2: moderate likelihood (pretest probability about 17%), needs a D-dimer; 3 or more: high likelihood (DVT likely), skip the D-dimer and order the ultrasound. Work-up pathway: low risk: D-dimer negative stops the work-up, D-dimer positive goes to ultrasound of the thigh (negative ends it); high risk: ultrasound of the thigh (a D-dimer, if taken, does not change this, because a negative result still goes to ultrasound); if that ultrasound is negative, either ultrasound of the lower leg, phlebography, or repeat thigh ultrasound in 1 week. Slide wording differs: the slide 55 note says 'less than 0' for the low category while its table says 'zero or less' (the table, which matches the standard rule, is used here), and the second table on that slide leaves out the 'previously documented DVT' item that the first table (the most widely validated set, from 2003) lists at +1. ★ D-dimer: elevation implies increased fibrinolysis; sensitive but not specific, so a positive result means clotting or fibrinolysis somewhere, not necessarily a DVT; it is useful as a rule-out test for DVT and PE. Contrast ascending venography is the 'gold standard' and definitive, but it is invasive and has been replaced by venous duplex ultrasound of the involved extremity. A diagnostic (positive) ultrasound ends the work-up and treatment starts; a nondiagnostic one leads to magnetic resonance imaging or phlebography (invasive x-ray with injected dye). Slide 58 lists electrocardiogram (ECG) findings (sinus tachycardia; S wave in lead I, Q wave in lead III, inverted T wave in lead III) under DVT diagnosis, but these are pulmonary embolism findings, not a test for DVT, and are not part of the DVT work-up (the electrocardiogram comes next week). Differential: ruptured popliteal (Baker) cyst (severe sudden calf discomfort); cellulitis (leg pain with erythema, possibly fever and chills); post-thrombotic (postphlebitic) syndrome (symptomatic chronic venous insufficiency following a DVT, 6 to 24 months later); superficial thrombophlebitis; lymphedema (chronic extremity edema, usually with a history of pelvic surgery, malignancy or radiation therapy); calf muscle pull or tear (inciting injury in the history, possible bruising at the ankle); drug-induced edema (for example dihydropyridine calcium channel blockers such as amlodipine; often bilateral, usually no signs of inflammation) Slides 52, 53, 54, 55, 56, 57, 58, 59

Treatment/Therapy

★ Anticoagulation is the mainstay of treatment; depending on the situation, patients may need thrombolysis and/or lifelong anticoagulation. Three strategies: (1) the classic but waning one, starting with heparin and bridging to warfarin; (2) parenteral therapy switched after 5 days to dabigatran (a direct thrombin inhibitor) or edoxaban (an anti-Xa agent); (3) oral monotherapy with a loading dose then a maintenance dose (rivaroxaban or apixaban, both anti-Xa agents). Options: low molecular weight heparin (LMWH), unfractionated heparin (UFH), fondaparinux (indirect factor Xa inhibitor), rivaroxaban and apixaban (direct factor Xa inhibitors), dabigatran (direct thrombin inhibitor). Contraindications: active bleeding, acute intracranial hemorrhage, major trauma, severe bleeding disorders. Duration: the initial course for a first VTE is a minimum of 3 months for all patients; VTE provoked by a major transient (reversible) risk factor (surgery under general anesthesia over 30 minutes, hospitalization or bed rest for 3 days or more, major trauma or fracture): 3 months; provoked by a minor transient risk factor (estrogen therapy, pregnancy, minor surgery, prolonged travel, minor leg injury): 3 to 6 months; unprovoked first VTE (no identifiable risk factor): indefinite. Outpatient therapy suits patients who are hemodynamically stable, at low bleeding risk, without renal insufficiency and with good support and reliable follow-up; it is not appropriate for iliofemoral (proximal) DVT, concurrent symptomatic PE, high bleeding risk, or other comorbidities that warrant inpatient care. Outpatient options: rivaroxaban; apixaban; LMWH (enoxaparin) or fondaparinux for 5 days, then dabigatran or edoxaban; LMWH or fondaparinux for 5 days overlapping with warfarin until the international normalized ratio (INR) is over 2. The choice rests on clinician experience, bleeding risk, comorbidities, preference, cost and convenience. Chronic renal disease with creatinine clearance below 30 mL/min: hospitalize to start; UFH is given as an intravenous (IV) loading bolus then a continuous infusion, adjusted by the activated partial thromboplastin time (aPTT), together with warfarin overlapping at least 5 days and until the INR is over 2 for at least 24 hours. The most serious adverse effect is hemorrhage; reversal agents for life-threatening or intracranial bleeding: protamine sulfate for UFH or LMWH (the slide's list; it fully reverses UFH but only partly reverses LMWH, a point the slide does not make), idarucizumab for dabigatran, andexanet alfa for apixaban and rivaroxaban (off-label for edoxaban as well), and for warfarin four-factor prothrombin complex concentrate, fresh frozen plasma or IV vitamin K, or oral vitamin K. An inferior vena cava filter (keeps the clot from traveling to the lungs) is used only for an acute proximal lower-extremity DVT with active bleeding, or when anticoagulation is otherwise contraindicated. Thrombolysis with tissue plasminogen activator (tPA) is helpful in a large proximal DVT, or a PE in a hemodynamically stable patient; thrombectomy is another option. Prevention: intermittent pneumatic compression (sequential compression devices), especially if the patient must be immobilized after surgery or during a long hospital stay Slides 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71

Mortality ★

★ Pulmonary embolism is the biggest complication: part of the thrombus can break off and travel to the lungs, so it must not be missed. Without adequate treatment, the cumulative rate of fatal PE was estimated at under 1% in patients with symptomatic DVT without PE, 3% in patients with symptomatic non-massive PE and about 9% in patients with symptomatic massive PE. Post-thrombotic (postphlebitic) syndrome, symptomatic chronic venous insufficiency, can follow 6 to 24 months after a DVT Slides 52, 60, 72

★ She stressed telling phlebitis (vein wall inflammation only) apart from thrombophlebitis (with a clot) because the treatments differ; anticoagulation is added only when there is a big concern, otherwise nonsteroidal anti-inflammatory drugs; recheck within 7 to 10 days.

Name of Condition

Superficial phlebitis and superficial thrombophlebitis Slides 73, 74

Definition

★ Superficial phlebitis: inflammation of the wall of a vein in the absence of thrombus. Superficial thrombophlebitis: phlebitis with thrombus Slide 74

Etiology (cause)

Inflammation of a superficial vein, with or without a clot. Lower-extremity superficial thrombophlebitis is most commonly due to varicose veins; upper-extremity superficial thrombophlebitis is most commonly due to recent intravenous (IV) catheter use (a peripherally inserted central catheter, or PICC line, is the most common) Slide 75

Epidemiology (who)

Not covered in the lecture

Risk Factors

Varicose veins (the most common cause in the lower extremity); sedentary lifestyle or prolonged inactivity (for example bedridden); local trauma (including a procedure involving superficial veins); recent intravenous (IV) catheter use (PICC line most common; the most common cause in the upper extremity); pregnancy or estrogen therapy; malignancy or a hypercoagulable state; history of venous thrombosis of the lower-extremity superficial veins in the absence of varicose veins Slide 75

Pathology

Phlebitis is inflammation of the vein wall alone; thrombophlebitis is the same inflammation with a clot in the vein. On ultrasound the vein is noncompressible with clot and vein-wall thickening. It rarely embolizes Slides 74, 77, 78

Clinical Manifestation

★ Tenderness, pain, induration and erythema along the course of a superficial vein. An indurated 'palpable cord' along a palpable superficial vein, with erythema and warmth of the overlying skin. High fever, fluctuance and/or purulent drainage suggest infection within the vein (suppurative or septic thrombophlebitis) Slide 76

Diagnosis

Based on history and physical examination; confirmed by venous duplex ultrasound (the gold standard), which shows a noncompressible superficial vein with clot and thickening of the vein wall. ★ Wall thickening alone is phlebitis; thickening with a clot is thrombophlebitis Slide 77

Treatment/Therapy

★ Typical treatment: warm compresses, compression, elevation of the affected extremity, and nonsteroidal anti-inflammatory drugs (NSAIDs, for example ibuprofen or diclofenac) to relieve pain and inflammation. ★ If there is extensive clot burden or an intermediate or higher risk of thrombosis, anticoagulation may be initiated (unfractionated heparin, enoxaparin (a low molecular weight heparin), or fondaparinux). ★ Repeat the physical examination within 7 to 10 days to assess for resolution versus progression; if signs or symptoms persist or worsen, repeat the duplex ultrasound Slides 79, 80

Mortality ★

Rarely causes serious complications and rarely embolizes Slide 78

★ She stressed that purulent drainage, high fever, and redness spreading beyond the vein's course (not just along it) signal septic thrombophlebitis, usually with a history of an intravenous line or catheter.

Name of Condition

Septic (suppurative) thrombophlebitis Slide 76

Definition

Infection within a vein that has a clot, arising from superficial thrombophlebitis; the suppurative form of thrombophlebitis Slide 76

Etiology (cause)

Infection within the vein, in the setting of venous cannulation (venipuncture or catheterization) Slide 76

Epidemiology (who)

Uncommon in the absence of a history of venous cannulation; the deck gives no other population data Slide 76

Risk Factors

History of venous cannulation (venipuncture, catheterization) Slide 76

Pathology

Not covered in the lecture

Clinical Manifestation

★ High fever, fluctuance and/or purulent drainage suggest infection within the vein. ★ Suspect it when erythema extends significantly beyond the margin of the vein Slide 76

Diagnosis

Not covered in the lecture

Treatment/Therapy

Not covered in the lecture

Mortality ★

Not covered in the lecture

★ She stressed that the lower-extremity fistulas are the iatrogenic ones (from groin access for catheter procedures), that a large fistula can slow the heart rate when compressed (do not hold it), and that acquired fistulas are treated surgically.

Name of Condition

Arteriovenous (AV) fistula of the extremity, acquired (traumatic or iatrogenic) Slide 82

Definition

An abnormal connection between an artery and a vein (they can exist almost anywhere in the body, depending on the cause); acquired ones arise from trauma or from a medical procedure (iatrogenic) Slide 82

Etiology (cause)

Iatrogenic: use of the groin (femoral vessels, historically and preferentially) as the site for percutaneous arterial and venous access, for example for endovascular procedures. Lower-extremity arteriovenous (AV) fistulas are most commonly iatrogenic for this reason. Trauma is named in the objectives, but the deck gives no traumatic mechanism Slides 82, 84

Epidemiology (who)

★ Lower-extremity fistulas are most commonly iatrogenic, due to groin access for endovascular procedures; iatrogenic fistulas most commonly occur in the lower extremity and affect the femoral vessels Slides 82, 84

Risk Factors

Percutaneous arterial and venous access through the groin (femoral vessels) Slide 84

Pathology

The fistula bypasses the capillary bed (blood passes directly from artery to vein). A fistula can in turn lead to chronic venous insufficiency, and a large one to heart failure and ischemia Slides 82, 85, 11

Clinical Manifestation

After a procedure, evaluate the puncture site with a complete lower-extremity vascular examination and compare the pulses with those before the procedure to ensure there is no change. Watch for infection, chronic venous insufficiency, heart failure and evidence of ischemia. ★ Compression of a large AV fistula can cause reflex slowing of the heart rate (Nicoladoni-Branham sign) Slides 84, 85

Diagnosis

Diagnosis is by physical examination. Duplex ultrasound confirms the diagnosis in patients with a suspected iatrogenic AV fistula. Computed tomography angiography (CTA) and angiography can confirm the diagnosis and give the location and size of the fistula Slides 84, 85

Treatment/Therapy

★ Acquired AV fistula: surgical management, either removing the fistula or decreasing its size. By contrast, a congenital AV fistula is difficult to treat because the communications can be numerous; it is treated conservatively with elastic support hose, and embolization can sometimes remove the fistula Slide 86

Mortality ★

Not covered in the lecture

★ She stressed that upper-extremity fistulas are the ones created for vascular access, and that a healthy fistula has a thrill you can feel and a soft bruit you can hear; a fistula with no bruit is a problem (needs referral).

Name of Condition

Arteriovenous (AV) fistula of the extremity, created for long-term hemodialysis vascular access Slides 81, 83

Definition

An abnormal connection between an artery and a vein, created deliberately to give long-term vascular access for hemodialysis Slide 83

Etiology (cause)

Surgically created for hemodialysis vascular access. ★ Upper-extremity arteriovenous (AV) fistulas are the ones most commonly created for hemodialysis vascular access Slides 82, 83

Epidemiology (who)

Upper arm or forearm are common sites and are generally preferred over the lower extremity Slide 83

Risk Factors

Not covered in the lecture

Pathology

The fistula bypasses the capillary bed (blood passes directly from artery to vein) Slide 82

Clinical Manifestation

★ A healthy AV fistula collapses completely with arm elevation and is characterized by a diffuse thrill and a soft bruit, both with systolic and diastolic components Slide 83

Diagnosis

Diagnosis is by physical examination. Watch for infection, chronic venous insufficiency, heart failure and evidence of ischemia. Compression of a large AV fistula can cause reflex slowing of the heart rate (Nicoladoni-Branham sign). Computed tomography angiography (CTA) and angiography can confirm the diagnosis and give the location and size of the fistula Slide 85

Treatment/Therapy

Not covered in the lecture

Mortality ★

Not covered in the lecture

★ She stressed that a prior deep vein thrombosis is the most common etiology, that hemosiderin brown staining separates venous from arterial disease, that an ankle-brachial index is ordered to rule out peripheral arterial disease before compression (not to diagnose), and that leg elevation must be above the heart.

Name of Condition

Chronic venous insufficiency (CVI), also called post-thrombotic syndrome Slides 3, 12

Definition

Signs of advanced venous disease associated with superficial and/or deep venous reflux and/or obstruction: edema, skin changes (hyperpigmentation, dermatitis, lipodermatosclerosis, which is skin and fat tissue scarring in the lower legs) and venous ulceration. A severe manifestation of venous hypertension and the far end of chronic venous disease, which ranges from telangiectasias to reticular veins to varicose veins to CVI. Classified as primary (intrinsic structural or functional abnormality of the vein wall or valves, leading to valvular reflux) or secondary (obstruction and/or valvular incompetence from a prior deep vein thrombosis (DVT)). The slide treats 'post-thrombotic syndrome' as another name; strictly it is the secondary, post-DVT form Slides 3, 5, 7, 9, 10

Etiology (cause)

Venous hypertension, from inadequate muscle pump function, incompetent venous valves (reflux), venous thrombosis, or other (nonthrombotic) venous obstruction. ★ Prior deep vein thrombosis (DVT) is the etiology she stressed; the slide calls it 'one of the most common' (primary superficial venous reflux also matters), and 25% of patients have no known DVT history, though there may be leg trauma or surgery. Another common cause: progressive superficial venous reflux. Other causes: congenital or neoplastic obstruction of pelvic veins, or a congenital or acquired arteriovenous (AV) fistula Slides 5, 10, 11

Epidemiology (who)

Advancing age and female sex; 25% of patients have no known history of DVT. The deck gives no prevalence figure Slides 8, 11

Risk Factors

Advancing age; female; family history of venous disease; certain hereditary conditions; ligamentous laxity (for example flat feet); increased body mass index (BMI); smoking; lower-extremity trauma; prior venous thrombosis; higher parity; high estrogen states Slide 8

Pathology

Vein valve leaflets fail because they are thickened and scarred (post-thrombotic syndrome) or sit in a dilated vein and are functionally inadequate, so the valves are incompetent. Proximal venous obstruction from chronic thrombus or scarring then complicates the problem. Valves cannot stop venous blood returning toward the foot, so the leg develops venous hypertension, with increased force on subcutaneous veins and tissues of the lower leg (edema). Superficial veins expand and their valves do not close; the deep venous system becomes incompetent because of the expanded superficial veins; and the superficial veins cannot empty into the deep system during walking, because blood is no longer pumped effectively from the lower leg. A healthy valve keeps blood moving in one direction; a diseased valve lets it move in both directions, elevating venous pressure Slides 4, 12, 13

Clinical Manifestation

May be asymptomatic or symptomatic. Symptoms: pruritus of the lower-extremity skin, often starting around the medial malleolus; leg heaviness, aching, pain, throbbing, hyperesthesia or burning, or swelling, worse when standing or with prolonged sitting with the feet dependent, and relieved by limb elevation and walking. ★ Progressive pitting edema of the leg (usually the lower leg) is the primary presenting symptom, usually with dull discomfort worsened by standing. Pruritic dermatitis; over time the skin of the ankle becomes shiny and taut from swelling. ★ Brownish pigmentation from hemosiderin deposits (brown or blue-gray discoloration; venous stasis dermatitis, an eczematous rash with erythema, scaling, weeping, erosions and crusting). With long-term disease, lipodermatosclerosis (firm, indurated areas with skin tacked down to the subcutaneous tissue, commonly at the medial ankle, which can form a constrictive band around the extremity). Ulcers: usually above the ankle on the medial or anterior aspect; painful; clean base with fibrinous exudate and possibly serous drainage; they heal with a thin scar that breaks down easily with minor trauma or increased leg edema. Cellulitis with venous insufficiency shows blanching erythema, with or without pain, and is difficult to diagnose Slides 6, 7, 14, 15, 21, 23

Diagnosis

History and physical examination often identify chronic venous insufficiency as the likely source of skin changes or leg ulcers, but they are insufficient to establish a venous cause and reliably rule out other causes of a leg ulcer. ★ Venous duplex ultrasound is needed to assess venous patency (is there a DVT?) and valvular competence (is there reflux?); it is the first test to confirm the diagnosis, determine the cause, rule out DVT, and determine the level of disease and the anatomy affected. ★ Arterial pulse examination plus an ankle-brachial index (ABI) should be done in any patient with a lower-extremity wound, and in older patients, because compression socks are contraindicated in peripheral artery disease (PAD); she stressed the ABI is ordered to rule out PAD before treating, not to make the diagnosis. Contested on the slides: the slide notes give an ABI of 0.90 or less as PAD but also an ABI below 0.7 as the compression cutoff; these conflict with each other and with other sources, so no compression cutoff is asserted here (the safe rule is to rule out PAD first). Cross-sectional venography with computed tomography (CT) or magnetic resonance imaging (MRI) helps evaluate the deep venous system that ultrasound cannot properly detect. Catheter-based (invasive) venography is the gold standard but is rarely needed to make a diagnosis; it is done before an invasive intervention. Punch biopsy can help rule out other causes if unsure. Differential: heart failure, chronic kidney disease or decompensated liver disease (bilateral lower-leg edema); medications (calcium channel blockers, nonsteroidal anti-inflammatory drugs (NSAIDs), thiazolidinediones); lymphedema (unilateral swelling, no varicosities, pitting edema, brawny discoloration from hemosiderin staining is rare); lipedema (bilateral, symmetric edema just above the ankles, in women); ulcers from diabetic neuropathy, arterial insufficiency (painful, usually but not always on the lateral ankle, with weak pulses), autoimmune disease, sickle cell anemia, or erythema induratum (bilateral, on the posterior lower leg) Slides 26, 27, 28, 29

Treatment/Therapy

Initial nonoperative management is recommended: skin care, leg elevation, exercise and compression therapy. ★ Leg elevation: elevate the feet above heart level; it improves cutaneous microcirculation and reduces edema. Exercise (daily walking and ankle flexion; avoid long periods of sitting and standing) increases calf muscle strength. Skin care: cleansing and emollients to maintain the skin barrier, prevent dryness and fissuring, and decrease scratching. ★ Compression therapy (hosiery or bandages) is the standard treatment: it improves oxygen transport to the skin and subcutaneous tissues, decreases edema, reduces inflammation and compresses dilated veins; stockings must be graduated (pressure greatest at the ankle and less up the leg), which speeds blood flow and prevents venous pooling. Some patients cannot tolerate compression or have contraindications; ★ peripheral artery disease must be ruled out before compression is started. Stasis dermatitis: barrier preparations (for example zinc oxide creams); erythema, inflammation, pruritus and vesicle formation may need a mid-potency topical corticosteroid. Cellulitis: oral antibiotics (cephalexin or clindamycin). Ulcer care: ulcers will not heal unless edema is controlled and compression is applied; refer to wound care management (complete healing can take 4 to 6 months; if not fully healed, work up for underlying infection or other causes). No oral medication has yet been proven useful for venous disease; flavonoids (used to increase venous tone) and aspirin (may accelerate healing of venous ulcers) are mentioned. Surgery aims to occlude or remove the affected vessels, restore proper blood flow and decrease venous hypertension: valvuloplasty is occasionally successful but the incidence of postoperative DVT is high; venous bypass is successful in select patients. ★ Refer to a vascular specialist for arterial insufficiency, nonhealing ulcers, ulcer recurrence, persistent stasis dermatitis, suspected contact dermatitis, diagnostic uncertainty, or significant saphenous reflux. Education: edema recurs if compression socks are not worn consistently, so control it long-term to prevent recurrent ulcerations Slides 30, 31, 32, 33, 34, 35, 36, 37

Mortality ★

No mortality figure is given. Ulcers heal with a thin scar that breaks down easily, complete healing can take 4 to 6 months, and edema recurs (with recurrent ulceration) unless compression is worn consistently Slides 15, 35, 37

★ She stressed that family history is the most common predisposing factor, and that a dull, achy heaviness or fatigue from standing is the most common symptom.

Name of Condition

Varicose veins Slide 38

Definition

Dilated, tortuous subcutaneous veins (3 millimeters or more in size; smaller dilated veins are telangiectasias, under 1 millimeter, and reticular veins, 1 to 3 millimeters), resulting from a combination of venous reflux and venous hypertension Slide 38

Etiology (cause)

A combination of venous reflux and venous hypertension. Superficial veins are commonly involved, usually the great saphenous vein (most commonly) and the short saphenous veins Slides 5, 38

Epidemiology (who)

Women after pregnancy are more likely to develop varicose veins. The deck gives no prevalence figure Slide 39

Risk Factors

★ The most common predisposing factor is family history. Periods of high venous pressure from prolonged standing or heavy lifting; women after pregnancy. Also the risk factors of chronic venous disease: advancing age, female, certain hereditary conditions, ligamentous laxity (for example flat feet), increased body mass index (BMI), smoking, lower-extremity trauma, prior venous thrombosis, higher parity, high estrogen states Slides 8, 39

Pathology

Venous reflux and venous hypertension: diseased valves let blood move in both directions, elevating venous pressure, which triggers venous dilation and, later, skin changes or ulceration. Long-standing varicose veins can progress to chronic venous insufficiency Slides 4, 5, 38, 40

Clinical Manifestation

Can be asymptomatic or symptomatic. ★ If symptomatic, a dull, achy heaviness or feeling of fatigue brought on by periods of standing is the most common symptom. Itching can occur above the ankle or over the varicose veins. Dilated, tortuous veins of the thigh and calf are visible and palpable when the patient is standing; they may be tender to palpation Slide 40

Diagnosis

Diagnosis is clinical, by physical examination of the leg in a dependent position. Lower-extremity Doppler (venous duplex) ultrasound confirms the diagnosis and classifies the anatomic location and extent of venous reflux Slide 41

Treatment/Therapy

Isolated varicose veins (no signs or symptoms of edema, skin changes or ulceration): management is often initially conservative and identical to chronic venous insufficiency: leg elevation, exercise and compression therapy, remembering to rule out arterial disease before starting compression. A trial of conservative therapy with stockings is generally done for weeks to months before venous ablation therapy is offered; the goal of venous intervention is improvement in symptoms and appearance. Sclerotherapy: injecting a sclerosing agent to fibrose and obliterate the veins. Surgical reflux treatment: vein stripping, or laser or radiofrequency catheter Slides 42, 43, 44

Mortality ★

No mortality figure is given. Varicose veins often recur after treatment, and long-standing ones can progress to chronic venous insufficiency Slides 40, 43

Name of Condition

Peripheral arterial disease (PAD), also called peripheral arterial occlusive disease (PAOD) Slide 8

Definition

Lack of blood flow to the musculature relative to its metabolism, producing pain in the affected muscle groups; pathophysiology similar to coronary artery disease Slide 10

Etiology (cause)

Atherosclerotic narrowing of the lower-extremity arteries at three segmental sites: aortoiliac, femoral-popliteal and tibial Slides 8, 12

Epidemiology (who)

Over 60 years old without other risk factors, or 50 years old with risk factors; males more than females; 50% of patients with peripheral arterial disease also have coronary artery disease Slides 10, 11

Risk Factors

Age; male sex; family history (PAD, cardiovascular disease or stroke); cigarette smoking (three times the risk of nonsmokers); diabetes mellitus; dyslipidemia; hypertension; obesity; elevated homocysteine (an amino acid associated with earlier-onset atherosclerosis); sedentary lifestyle Slide 11

Pathology

Decreased blood flow to the extremities, then decreased tissue perfusion, then decreased oxygen and nutrients to the musculature, then tissue damage and pain. Symptom severity depends on the degree of narrowing, the number of arteries affected and the patient's activity level Slide 9

Clinical Manifestation

Often asymptomatic, so ask about functional capacity and decline in activity. Intermittent claudication (Latin for limping): ache, cramp, numbness or fatigue in muscles with activity, relieved by rest (usually 10 minutes); analogous to angina; severity depends on degree of stenosis, collateral circulation and vigor of exercise. Pain site maps to level: buttock and hip = aortoiliac (30%, picture only); thigh = common femoral; upper two-thirds of calf = superficial femoral (60%, picture only; calf claudication is the most common); lower third of calf = popliteal; foot = tibial and peroneal. Leriche syndrome triad: claudication, absent or diminished femoral pulses, erectile dysfunction. Exam: dry, thin, shiny, brittle skin; loss of hair and toenails; brittle nails; muscle atrophy; poor wound healing; dependent rubor (deep red when the leg hangs down); cool limb (a temperature demarcation roughly marks the occlusion level); diminished or absent pulses below the stenosis; capillary refill over 2 seconds; bruits over the abdominal aorta, iliac, femoral and popliteal arteries; xanthelasma; midline sternotomy scar from a prior coronary artery bypass Slides 13, 14, 15, 16, 17, 19, 20, 27, 28, 29, 32, 33, 34, 35

Diagnosis

History (walking distance, how long the patient rests, lifestyle change). Buerger test: elevate one leg to 45 degrees for 1 minute (pallor; cyanosis in severe cases), then dangle the legs over the bed (dependent rubor); in darker skin use temperature, texture and capillary refill (speaker notes). ABI (ankle-brachial index) is the simple bedside, important initial test: normal over 0.9 to 1.3; claudication 0.4 to 0.9; rest pain and tissue loss 0 to 0.4; over 1.3 is abnormal (calcified, noncompressible arteries, for example in diabetes). The ankle-brachial index = each leg's higher ankle pressure over the higher arm pressure; the lower leg value is the overall index (picture only). Pulse volume recordings (plethysmography) show site and severity (picture-only slide). Duplex ultrasound is the mainstay initial imaging. CTA (computed tomography angiography) is contraindicated with contrast allergy or decreased renal function; magnetic resonance angiography is the alternative with contrast allergy but is contraindicated with pacemakers or intracranial aneurysm clips. Conventional angiography is the gold standard: invasive, most accurate; computed tomography angiography, magnetic resonance angiography and conventional angiography mainly guide a planned intervention. Labs: ECG (electrocardiogram), CBC (complete blood count) with differential, lipid profile, CMP (comprehensive metabolic panel) with fasting glucose, renal function, hemoglobin A1c Slides 18, 30, 31, 42, 43, 45, 46, 47, 48, 49

Treatment/Therapy

Risk-factor reduction: smoking cessation; blood pressure goal below 140/90 mmHg (below 130/80 mmHg with diabetes or renal failure); diabetes control; exercise therapy (refer to a supervised exercise rehabilitation program, which beats home-based; 30 to 45 minutes three times a week); weight control. Antiplatelets: aspirin, clopidogrel, cilostazol (antiplatelet and vasodilator) lower cardiovascular risk from myocardial infarction and stroke and improve walking distance. Statins (simvastatin, pravastatin): LDL (low-density lipoprotein) target below 100 mg/dL, below 70 mg/dL if high risk. Pentoxifylline (a xanthine derivative). Folic acid with vitamins B12 and B6 for hyperhomocysteinemia. Revascularization when claudication causes severe functional disability despite exercise or drugs: endovascular PTA (percutaneous transluminal angioplasty) with or without stenting, atherectomy; bypass graft (saphenous vein or synthetic); endarterectomy; amputation Slides 50, 51, 52, 53, 55, 57, 58

Mortality ★

Not covered in the lecture

Name of Condition

Critical limb ischemia: ischemic rest pain and skin ulceration, the most advanced end of peripheral arterial disease Slides 21, 24

Definition

Not covered in the lecture

Etiology (cause)

Advanced peripheral arterial disease: ulcers fail to heal because the blood supply cannot meet the increased demands of healing tissue Slide 24

Epidemiology (who)

Not covered in the lecture

Risk Factors

Not covered in the lecture

Pathology

Elevating the leg removes gravity's help to arterial flow, so the pain worsens; dependency uses gravity to improve perfusion Slides 21, 23

Clinical Manifestation

Constant throbbing, burning or severe shooting rest pain with cool feet, localized to the forefoot and toes, worse on elevation or when reclining, not readily controlled by analgesics (even narcotics). Relieved by hanging the leg over the edge of the bed or by walking around (a paradox, in contrast to claudication). Wakes the patient at night; may sleep in a recliner. Ulcers: painful, often start as minor trauma, at the tips of toes and between digits and over pressure points (metatarsal heads); dry, punched out, minimal bleeding; often surrounded by black necrotic tissue. Dry gangrene: hard, dry, usually distal toes, clear demarcation, common in peripheral arterial disease. Wet gangrene is a surgical emergency: moist, edema, blistering, bacterial infection with risk of sepsis, often spore-forming clostridial species (gas gangrene) Slides 21, 22, 23, 24, 25, 37, 38, 39

Diagnosis

ABI (ankle-brachial index) 0 to 0.4 (rest pain, tissue loss); history questions about night pain, recliner use, response to pain medication and nonhealing toe wounds Slides 22, 25, 42

Treatment/Therapy

Bypass graft is principally used to treat severe critical leg ischemia; amputation is a surgical option Slides 57, 58

Mortality ★

Not covered in the lecture

Name of Condition

Acute limb ischemia; acute peripheral arterial occlusion Slides 59, 66

Definition

Sudden occlusion of a previously patent artery, with abrupt onset of severe pain and absent pulses in the involved extremity. A vascular emergency Slide 59

Etiology (cause)

Thrombosis locally at a previously patent stenotic artery; or embolus, of which thromboemboli are most common (a dislodged intravascular thrombus travels distally and occludes a smaller artery). The heart is the embolic source in 80% to 90% of cases (atrial fibrillation, left ventricular thrombus after a myocardial infarction). Atheroemboli are less likely to cause acute limb ischemia Slide 60

Epidemiology (who)

Not covered in the lecture

Risk Factors

Not covered in the lecture

Pathology

A dislodged thrombus lodges in and occludes a smaller distal artery; light-touch sensory fibers are highly susceptible to ischemia Slides 60, 61

Clinical Manifestation

Sudden onset; may or may not have a history of claudication. Six P's: pain, pallor, pulselessness, paresthesia, paralysis, poikilothermia (difficulty regulating body temperature). Cold, cyanotic extremity; absent pulses distal to the occlusion; sensory deficit over the dorsum of the foot is often the earliest neurologic sign Slides 61, 34

Diagnosis

Categories (Rutherford): I viable (no sensory loss or weakness, Doppler signals audible); IIA marginally threatened (salvageable if promptly treated; minimal toe pain or none, no sensory loss, no weakness); IIB immediately threatened (salvageable with immediate revascularization; sensory loss beyond the toes with rest pain, mild to moderate weakness, arterial Doppler usually inaudible, venous audible); III irreversible (profound sensory loss and paralysis, both Doppler signals inaudible). IIA arterial Doppler is often inaudible with the venous signal audible (the slide 65 table and the Society for Vascular Surgery standards; slide 62's 'often audible' is not accurate). Categories I and IIA have time for imaging (for example computed tomography angiography); IIB should be evaluated and treated in a surgical suite; III generally needs no immediate imaging. Testing depends on category: magnetic resonance angiography, computed tomography angiography or catheter-based arteriography Slides 62, 63, 64, 65, 66

Treatment/Therapy

Immediate vascular surgery consult; anticoagulation with intravenous heparin; intra-arterial catheter-directed thrombolysis with alteplase if under 14 days' duration; thromboembolectomy (catheter or surgical); arterial bypass; amputation. Irreversibly ischemic limbs need major amputation regardless of therapy Slides 66, 67, 64

Mortality ★

Not covered in the lecture

Name of Condition

Acute peripheral arterial occlusion: atheroembolism Slide 68

Definition

Multiple small deposits of fibrin, platelets and cholesterol debris embolizing from proximal atherosclerotic lesions Slide 68

Etiology (cause)

Proximal atherosclerotic plaques; an abdominal aortic aneurysm can also produce embolic blue toe syndrome Slides 7, 68, 89

Epidemiology (who)

Not covered in the lecture

Risk Factors

Not covered in the lecture

Pathology

Debris lodges in small vessels of muscle and skin and may not occlude large vessels, so distal pulses usually remain palpable Slide 68

Clinical Manifestation

Acute pain and tenderness at the site of embolization; digital occlusion causes ischemia and blue toe syndrome, and digital necrosis and gangrene may develop; localized tenderness, pallor and livedo reticularis at the sites of emboli; distal pulses usually palpable Slide 68

Diagnosis

Not covered in the lecture

Treatment/Therapy

Not covered in the lecture

Mortality ★

Not covered in the lecture

Name of Condition

Carotid artery disease (atherosclerotic disease of the extracranial carotid artery) Slides 69, 70, 71

Definition

Atherosclerotic plaque in the carotid artery causing stenosis, embolization and thrombosis Slide 70

Etiology (cause)

Atherosclerosis, which has a predilection for the extracranial carotid artery (the bifurcation and bulb, a low-shear region in the figure) Slide 70

Epidemiology (who)

Responsible for 80% of new noncardioembolic strokes; asymptomatic patients with significant stenosis outnumber symptomatic patients 4 to 1 Slides 71, 72

Risk Factors

Smoking (nearly doubles stroke risk); hypertension, diabetes and hyperlipidemia are managed as comorbidities (no formal risk-factor list) Slide 76

Pathology

As internal carotid stenosis increases, flow becomes more turbulent and the risk of atheroembolization rises; strokes are most often caused by plaque rupture rather than occlusion with thrombosis Slide 71

Clinical Manifestation

Asymptomatic: narrowing in one or both carotids with no stroke or TIA (transient ischemic attack) in the last six months. Symptomatic: focal neurologic symptoms, TIA (focal loss of neurologic function lasting less than 24 hours), stroke. Focal symptoms (speaker notes): sudden intense headache, paralysis, numbness or tingling, vision changes, amaurosis fugax (transient monocular blindness, vision fading like a curtain descending), drowsiness. Exam: inspect neck pulses; with the breath held, auscultate one carotid at a time with the diaphragm then the bell for a bruit; palpate one side at a time Slides 72, 73

Diagnosis

Duplex ultrasonography: most widely used initial screening tool and used for serial monitoring. CTA (computed tomography angiography) or MRA (magnetic resonance angiography) image the cervical and intracranial carotid and vertebral circulation and the brain. Digital subtraction angiography is the gold standard: invasive, not used for screening, used to guide intervention. The USPSTF (United States Preventive Services Task Force) recommended against screening asymptomatic adults in 2014 and reaffirmed it in 2021 (speaker notes) Slides 74, 76

Treatment/Therapy

Medical management for symptomatic and asymptomatic patients with low-grade stenosis below 50%: antiplatelets (aspirin, clopidogrel), antihypertensives, glycemic control, statins, smoking cessation, healthy diet, exercise, weight reduction. Surgery for symptomatic stenosis over 70% or asymptomatic stenosis over 80% with life expectancy over 5 years: carotid artery angioplasty with stenting (usually transfemoral; less invasive, for poor surgical candidates) or carotid endarterectomy (removes plaque through an incision; gold standard for high-grade stenosis; complications stroke or nerve damage) Slides 75, 76, 77, 78

Mortality ★

Not covered in the lecture

Name of Condition

Aortic aneurysm; abdominal aortic aneurysm (AAA) Slides 80, 82

Definition

A pathologic dilation of a segment of a blood vessel: localized dilation greater than 50% of normal diameter. The infrarenal aorta averages 2 cm; over 3 cm is aneurysmal. True aneurysms involve all three wall layers: saccular (part of the circumference, an outpouching) or fusiform (the entire circumference, a diffuse dilation) Slides 82, 83, 84

Etiology (cause)

Cause unclear; failure of elastin and collagen in the media; genetic predisposition (Marfan syndrome, FBN1 gene; Ehlers-Danlos syndrome, COL3A1 gene); atherosclerosis may weaken the wall mechanically. Aneurysm etiologies: Marfan syndrome, syphilis (destroys the vasa vasorum), vasculitis (Takayasu arteritis, giant cell arteritis), trauma, congenital Slides 80, 85

Epidemiology (who)

Men more than women (2 to 1 under 80 years; 1 to 1 over 80 years); Caucasian men have the highest incidence; uncommon in African American, Asian and Hispanic populations. The abdominal aorta is the most common site; 90% are infrarenal, usually involving the bifurcation and common iliac arteries Slides 80, 81, 82

Risk Factors

Smoking, current or past (strongest risk factor); male sex; advancing age; Caucasian race; atherosclerosis; hypertension; family history of abdominal aortic aneurysm; other large-artery aneurysms (iliac, femoral, popliteal). Protective: female sex (though women's aneurysms rupture more often), non-Caucasian race, diabetes Slides 86, 87, 88

Pathology

Medial elastin and collagen failure. Rupture is a sudden escape of blood into the retroperitoneum; rare until the diameter exceeds 5 cm; risk rises with diameter over 5.5 cm, expansion over 0.5 cm in six months, continued smoking and hypertension Slides 80, 90, 99

Clinical Manifestation

Asymptomatic: often an incidental exam finding. Symptomatic unruptured: pain with expansion (constant or intermittent) in the abdomen (hypogastrium), back or flank, may radiate to the low back; isolated groin pain (femoral nerve pressure); early satiety; embolic blue toe syndrome. Ruptured classic presentation (50% of patients): severe abdominal or back pain, pulsatile abdominal mass (virtually diagnostic), hypotension; often misdiagnosed as renal colic, diverticulitis, gastrointestinal hemorrhage or ischemic bowel. Exam: palpate and estimate aortic size; abdominal bruit is nonspecific; peripheral pulses; Grey Turner sign (flank ecchymosis) with retroperitoneal hemorrhage Slides 89, 90, 91

Diagnosis

Abdominal X-ray shows a calcified outline in 75%. Abdominal ultrasound is the study of choice for initial screening and for serial size. CT (computed tomography) better defines size, visceral-artery involvement and extent; MRI (magnetic resonance imaging) is comparable without dye or radiation (stable patients with dye allergy). CTA (computed tomography angiography) is the best diagnostic and pre-intervention planning study. Labs: ECG (electrocardiogram), CBC (complete blood count), CMP (comprehensive metabolic panel), type and crossmatch. Differential: gastritis, appendicitis, urinary tract infection, diverticulitis, pancreatitis, gallstones, bowel obstruction, myocardial infarction, peptic ulcer disease. Surveillance: 3.0 to 3.9 cm, ultrasound every 3 years; 4.0 to 5.4 cm, ultrasound or computed tomography every 6 to 12 months Slides 93, 94, 95, 96, 97, 98

Treatment/Therapy

Medical: manage modifiable risk factors, smoking cessation, aggressive blood pressure control, moderate exercise. Vascular specialist referral at 4.5 cm or larger, and urgently for aneurysmal pain at any size. Repair: elective at 5.0 cm or larger in women and over 5.5 cm in men, or with rapid growth (0.5 cm in six months) (American College of Cardiology/American Heart Association); consider vascular surgery referral at 5.0 cm or larger. Methods, chosen by age and comorbidities: EVAR (endovascular aneurysm repair, a modular graft placed through the iliac or femoral arteries) or open repair (tube or bifurcated prosthetic graft). Symptomatic unruptured: assess hemodynamic stability, large-bore intravenous catheters, pain control, blood pressure control (beta blockers), repair unless contraindicated. Ruptured: hemodynamically unstable, large-bore intravenous catheters, type and crossmatch, surgical emergency Slides 92, 95, 100, 101, 102

Mortality ★

15,000 deaths per year due to abdominal aortic aneurysm in the United States Slide 80

Name of Condition

Thoracic aortic aneurysm: ascending, arch or descending Slides 82, 104

Definition

Same aneurysm definition (localized dilation greater than 50% of normal diameter); normal thoracic diameter varies with location, body habitus, sex and age Slides 82, 83

Etiology (cause)

Not covered in the lecture

Epidemiology (who)

History of hypertension is common Slide 104

Risk Factors

Not covered in the lecture

Pathology

Symptoms come from location and compression of neighboring structures Slide 104

Clinical Manifestation

Most are asymptomatic, found incidentally on chest X-ray. Ascending: aortic regurgitation, heart failure. Arch: hoarseness (recurrent laryngeal nerve). Descending: dyspnea, stridor, wheezing, cough, dysphagia from compression of the trachea or esophagus Slide 104

Diagnosis

Chest X-ray: widened mediastinal shadow; displacement or compression of the trachea or left mainstem bronchus. Labs: CBC (complete blood count), CMP (comprehensive metabolic panel), type and crossmatch. CTA (computed tomography angiography) is most widely used and shows the entire thoracic and abdominal aorta; MRA (magnetic resonance angiography) shows location, extent and size but is time consuming; TEE (transesophageal echocardiography) evaluates the aortic valve and root. Surveillance: below 4.5 cm, computed tomography or magnetic resonance imaging every 3 to 5 years; 4.5 to 5.4 cm, every 6 to 12 months. Chest or back pain with a known or suspected thoracic aneurysm needs urgent imaging Slides 105, 106, 107, 108, 109

Treatment/Therapy

Smoking cessation; beta blockers; aggressive blood pressure control. Repair all symptomatic thoracic aneurysms. Asymptomatic repair depends on diameter, location and expansion: ascending over 5.5 cm, descending over 6.0 cm, growth over 0.5 cm per year. Ascending often needs open repair with aortic root replacement; descending can be open or endovascular Slides 108, 109, 110

Mortality ★

Not covered in the lecture

Name of Condition

Pseudoaneurysm (false aneurysm) Slide 112

Definition

A communication between the arterial lumen and overlying connective tissue after arterial rupture; a blood-filled cavity forms outside the vessel wall and seals the leak as it thromboses Slide 112

Etiology (cause)

Laceration of the artery by an access needle Slide 112

Epidemiology (who)

Common site: femoral artery after cardiac catheterization Slide 112

Risk Factors

Not covered in the lecture

Pathology

Blood outside the vessel wall contained by connective tissue and hematoma. Picture-only discriminators: pseudoaneurysm has a thin wall, irregular outline and surrounding hematoma; a true aneurysm has a thick wall and smooth outline Slides 112, 113

Clinical Manifestation

Pain from increased pressure (swelling) or nerve compression Slide 113

Diagnosis

Not covered in the lecture

Treatment/Therapy

Watch and wait if under 2 cm (may spontaneously thrombose and regress); ultrasound-guided thrombin injection to seal the leak Slide 113

Mortality ★

Not covered in the lecture

Name of Condition

Aortic dissection Slide 114

Definition

Forms from an intimal tear, typically contained by the media; the wall layers separate, creating a false lumen within the media where blood can pool Slide 114

Etiology (cause)

Most common predisposing factor is degeneration of medial collagen and elastin; Marfan syndrome is the classic medial degeneration Slide 115

Epidemiology (who)

Age 60s to 70s (except Marfan syndrome); males more than females; history of hypertension in most cases Slide 115

Risk Factors

Hypertension; Marfan syndrome; medial degeneration Slide 115

Pathology

Intimal tear, then blood dissects into the media and forms a false channel, which may clot Slides 114, 115

Clinical Manifestation

Sudden, severe, tearing or ripping chest pain; location depends on the site and may change as the dissection propagates distally; syncope; hypertension or hypotension; aortic regurgitation from dilatation of the aortic root Slide 116

Diagnosis

ECG (electrocardiogram) may be normal; CT (computed tomography) of the chest and abdomen delineates extent; MRI (magnetic resonance imaging) is good but slower; TEE (transesophageal echocardiography) Slide 117

Treatment/Therapy

Stop progression by reducing blood pressure: systolic 100 to 120 mmHg with a beta blocker (esmolol) or vasodilator (nitroprusside); pain management; surgery (grafting and replacing the diseased aorta) Slide 118

Mortality ★

Acute untreated aortic dissection has a high mortality rate (no figure given) Slide 118

★ Carter flagged (Zoom recording times): family history is extremely important (51:26); alcohol is one of the few causes that can recover (47:50); the echocardiogram is the definitive test (58:27); defibrillator when the ejection fraction stays below 35% on optimized therapy (1:02:33); 50% mortality at 5 years once symptomatic (1:06:44).

Name of Condition

Dilated cardiomyopathy (DCM) Slides 16, 18

Definition

The most common type of cardiomyopathy. Myocardial dysfunction (inability to contract effectively) in the absence of severe occlusive coronary artery disease or pressure or volume overload (for example hypertension, valvular heart disease); dilation and impaired contraction of one or both ventricles Slides 17, 18

Etiology (cause)

Idiopathic (common; genetic variants likely). Familial dilated cardiomyopathy (common, up to 35% of cases; autosomal dominant most common; research focuses on the Lamin A/C gene). Infectious myocarditis: viral (coxsackievirus, parvovirus B19, influenza, HIV (human immunodeficiency virus)) or parasitic (Trypanosoma cruzi, Chagas disease). Chemotherapeutic agents. ★ Long-term excess alcohol (function can recover if intake is reduced or stopped). Thiamine (vitamin B1) deficiency. Systemic lupus erythematosus, rheumatoid arthritis. Peripartum cardiomyopathy (rare; last trimester of pregnancy to 6 months postpartum). Viral myocarditis accounts for 20% of dilated cardiomyopathy cases Slides 21, 22, 23, 24, 48

Epidemiology (who)

10,000 deaths and 46,000 hospitalizations a year in the United States. Most present between 20 and 60 years (can occur in children and older adults). Males more than females. African Americans have a high rate Slide 20

Risk Factors

Past history of hypertension, angina, coronary artery disease, anemia, thyroid dysfunction, breast cancer, prior heart failure or myocardial injury, chemotherapy, arrhythmias, thromboembolic complications. Social: tobacco, alcohol, illicit drugs. ★ Family history of cardiomyopathy or sudden death Slides 25, 28

Pathology

Dilation and impaired contraction; increased total cardiac mass with little or no wall hypertrophy; adverse remodeling produces a globular heart; systolic dysfunction. The distorted valve apparatus causes mitral (earlier) and tricuspid regurgitation; rising end-systolic volume causes further dilation; risk of arrhythmias and thromboembolism. Contested on the slides: 'little or no cardiac wall hypertrophy' (slide 18) versus an ECG (electrocardiogram) that 'may show left ventricular hypertrophy' (slide 37) Slides 18, 19, 37

Clinical Manifestation

Heart failure symptoms: progressive dyspnea on exertion, fatigue, atrial or ventricular arrhythmias, orthopnea, PND (paroxysmal nocturnal dyspnea), peripheral edema with increasing weight and abdominal girth. Exam: left heart failure before right; tachypnea, tachycardia, hypertension or hypotension, irregular pulse, narrow pulse pressure; crackles, pleural effusions, cyanosis; laterally displaced PMI (point of maximal impulse), mitral and tricuspid regurgitation murmurs, S3 gallop, JVD (jugular venous distention), cool extremities, edema; congestive hepatomegaly, hepatojugular reflux (neck-vein distension with firm pressure over the liver), ascites Slides 26, 27, 29, 30, 31, 32, 33, 34

Diagnosis

Labs: CBC (complete blood count) for anemia, CMP (comprehensive metabolic panel), liver function, thyroid function. ECG (electrocardiogram) usually abnormal; a normal electrocardiogram almost rules dilated cardiomyopathy out (slide 37), although Carter said on the recording that it does not rule it out (57:56): unlikely, not excluded; may show atrial fibrillation, premature ventricular contractions, left bundle branch block. ★ Echocardiogram: dilated thin walls, ejection fraction below 40%, all four chambers may dilate, hypokinesis, segmental wall-motion abnormalities, tricuspid or mitral regurgitation, thrombus. Chest X-ray: cardiomegaly, effusions, pulmonary edema, Kerley B lines (1 to 2 cm horizontal lines meeting the pleura at right angles). BNP (B-type natriuretic peptide) elevated; below 100 pg/mL excludes heart failure. Cardiac MRI (magnetic resonance imaging) with gadolinium for inflammatory, ischemic or infiltrative causes. Biopsy is not routine and rarely useful: only for rapidly deteriorating function or systemic disease (hemochromatosis, sarcoidosis, amyloidosis). Differential: acute coronary syndrome, valvular disease, acute pericarditis, tamponade, hyperthyroidism, myocarditis Slides 35, 36, 37, 38, 39, 40, 41

Treatment/Therapy

Same as chronic heart failure; all patients get a beta blocker plus an ACE (angiotensin-converting enzyme) inhibitor (speaker notes). Drug classes: angiotensin-converting enzyme inhibitors, beta blockers, ARBs (angiotensin receptor blockers), aldosterone receptor blockers (spironolactone), sacubitril/valsartan, nitrates, diuretics (loop diuretics the mainstay), digoxin (second line), antiarrhythmics, anticoagulants (only for atrial fibrillation, artificial valves or mural thrombus). Devices: biventricular pacemaker (moderately severe left ventricular dysfunction); ★ ICD (implantable cardioverter-defibrillator) if ejection fraction is below 35% after maximum medical therapy; biventricular implantable cardioverter-defibrillator. LVAD (left ventricular assist device) as a bridge to transplant or recovery, or destination therapy. Cardiac transplantation. Education: medication regimen and follow-up; remove the offending source (alcohol, nutritional deficiency, cardiotoxins); sodium below 2 g/day; genetic counseling with a family history Slides 42, 43, 44, 45

Mortality ★

Poor overall: ★ 50% mortality at 5 years once symptoms emerge (progressive disease and sudden cardiac death from arrhythmia); prognosis depends on response to therapy. 10,000 deaths a year in the United States. Idiopathic dilated cardiomyopathy is the primary indication for cardiac transplantation. Angiotensin-converting enzyme inhibitors, beta blockers and angiotensin receptor blockers reduce mortality; digoxin does not change long-term survival (speaker notes). Patients may live over 5 years with a left ventricular assist device Slides 20, 42, 44, 45

★ Carter flagged (Zoom recording times): biopsy is the gold standard even though it is rarely done, after other causes are excluded (1:26:09); magnetic resonance imaging is the best test for the extent of inflammation (1:25:53); one third go on to dilated cardiomyopathy (1:21:57); viral myocarditis is about 20% of dilated cardiomyopathy (1:19:05).

Name of Condition

Acute myocarditis Slides 46, 47

Definition

Inflammatory disease of the myocardium, with a wide range of presentations from subtle to devastating Slide 47

Etiology (cause)

Viral infection is presumed most frequent in developed countries: parvovirus B19, human herpesvirus 6, enteroviruses (Coxsackie group B), adenoviruses, hepatitis C, cytomegalovirus, HIV (human immunodeficiency virus), COVID-19 (coronavirus disease 2019). Rheumatic fever (beta-hemolytic streptococcus; affects the heart in 50% to 90%). Bacterial: diphtheria, brucellosis, clostridial infection, Legionnaires disease, meningococcus, Mycoplasma pneumonia. Parasitic: Trypanosoma cruzi (Chagas disease). Rickettsial: Q fever, Rocky Mountain spotted fever. Spirochetal: leptospirosis, Lyme disease. Fungal: Candida. Medications: penicillin, thiazide diuretics, tetracycline, methyldopa. Toxins: lithium, arsenic, lead, copper. Autoimmune: systemic lupus erythematosus, rheumatoid arthritis, myasthenia gravis Slides 47, 48, 49

Epidemiology (who)

1.5 million cases worldwide in 2020; no race predilection; incidence similar in males and females, but young males are particularly susceptible; usually an otherwise healthy person Slides 47, 50

Risk Factors

Recent (1 to 2 week) flulike illness in viral myocarditis; no underlying cardiac dysfunction or low cardiac risk Slide 51

Pathology

Inflammation of the myocardium; troponin reflects cardiac myonecrosis Slides 47, 54

Clinical Manifestation

Acute decompensated heart failure in a patient with no underlying cardiac dysfunction or low cardiac risk. Viral prodrome: fever, myalgia, fatigue, respiratory symptoms, gastroenteritis before the heart failure symptoms. Heart failure symptoms: exercise intolerance, shortness of breath, fluid retention, chest pain. Palpitations, syncope or sudden cardiac death from ventricular arrhythmias or atrioventricular block. Exam: cardiogenic shock or frank heart failure; tachycardia, hypotension, raised JVP (jugular venous pressure), S3 gallop, crackles, peripheral edema Slides 51, 52

Diagnosis

CBC (complete blood count) shows leukocytosis; ESR (erythrocyte sedimentation rate) may be elevated; troponin (myonecrosis); ECG (electrocardiogram) sinus tachycardia or arrhythmias; echocardiogram; chest X-ray nonspecific, may show cardiomegaly; cardiac angiography to rule out coronary ischemia; ★ gadolinium-enhanced MRI (magnetic resonance imaging) shows the extent of inflammation. ★ Endomyocardial biopsy is the gold standard, once other causes of heart failure are excluded; recommended for acute deterioration of unknown origin not responding to treatment. Differential: tamponade, cardiogenic shock, cocaine-related cardiomyopathy, ischemic heart disease, valvular disease, coronary atherosclerosis, dilated, hypertrophic, peripartum and restrictive cardiomyopathy Slides 53, 54, 55

Treatment/Therapy

A high degree of suspicion is required. Treatment is directed at the clinical scenario; supportive care is the mainstay; may need aggressive hemodynamic stabilization. As needed: vasodilators, ACE (angiotensin-converting enzyme) inhibitors, diuretics, antiarrhythmics, anticoagulation Slide 56

Mortality ★

Can cause rapidly progressive (often fatal) heart failure and arrhythmia; sudden cardiac death possible. Most patients with mild symptoms recover completely; ★ one third later develop dilated cardiomyopathy Slides 47, 50, 51

★ Carter flagged (Zoom recording times): systolic anterior motion is the telltale sign of obstruction (1:31:24); syncope, especially in a young athlete, needs a work-up (1:33:35); echocardiogram is the test of choice (1:38:54); avoid preload reducers and positive inotropes (1:43:49); his last answer: no strenuous exercise until the obstructive form is worked up (2:10:39). Slides differ on diuretics, amiodarone and the exercise advice (see the study guide).

Name of Condition

Hypertrophic cardiomyopathy (HCM); the obstructive form is hypertrophic obstructive cardiomyopathy (HOCM) Slides 57, 60

Definition

Left ventricular hypertrophy in the absence of causative hemodynamic factors (hypertension, aortic valve disease, systemic infiltrative or storage disease); often asymmetric, any region of the left ventricle, most often the interventricular septum. Two types: without obstruction and with obstruction Slides 58, 60

Etiology (cause)

Mostly inherited, autosomal dominant (one mutated copy from an affected parent is sufficient); at least 1,500 mutations; genes encoding sarcomere proteins Slide 59

Epidemiology (who)

1 in 500 of the general population; males more than females; the leading cause of sudden death in preadolescent and adolescent children Slides 58, 59

Risk Factors

Family history (disease detected in family members prompts evaluation); extreme exertion is linked to sudden death Slides 64, 66

Pathology

Without obstruction: diastolic dysfunction; hypertrophy resists filling (reduced compliance) and raises left ventricular end-diastolic pressure. With obstruction: septal hypertrophy narrows the left ventricular subaortic outflow tract; ★ systolic anterior motion of the mitral valve (high-velocity flow pulls the anterior leaflet into the outflow tract) causes mid-systolic obstruction. Angina from outflow obstruction and the higher oxygen demand of hypertrophied muscle Slides 61, 62, 66

Clinical Manifestation

May be asymptomatic (found through family screening, a murmur or an abnormal ECG (electrocardiogram)). Dyspnea is the common presenting symptom; palpitations (atrial premature contractions, atrial fibrillation, flutter, supraventricular and ventricular tachycardia); ★ syncope (very common; low output on exertion or arrhythmia) and presyncope on standing relieved by lying down both mark high sudden-death risk and need urgent work-up; angina without coronary artery disease; sudden cardiac death (over 80% ventricular fibrillation). Exam: blood pressure and heart rate usually normal; pulsus bisferiens (a double systolic peak); forceful, sustained apical impulse, maybe laterally displaced; S4. Murmurs: outflow obstruction, a crescendo-decrescendo systolic murmur at the left sternal border in the 3rd or 4th intercostal space radiating to the suprasternal notch but not the carotids; mitral regurgitation, holosystolic at the apex radiating to the axilla. Maneuvers: more blood flow in the heart softens the murmur, less flow makes it louder; squatting, passive leg raising and handgrip decrease it; Valsalva, standing and amyl nitrite increase it; respiration has no effect Slides 64, 65, 66, 67, 68, 69

Diagnosis

No specific labs; CBC (complete blood count), fasting glucose, renal and liver function, BNP (B-type natriuretic peptide) and thyroid function look for exacerbating factors; chest X-ray may be normal. ★ Echocardiography is the typical test of choice: diagnosis confirmed by unexplained left ventricular wall thickness of 1.5 cm or more; asymmetric, classically septal hypertrophy; small cavity; ejection fraction usually normal (over 75% late); mitral regurgitation; systolic anterior motion; left atrial enlargement. Electrocardiogram: left ventricular hypertrophy, atrial enlargement, conduction abnormalities, prominent Q waves in inferior (II, III, aVF) and lateral (I, aVL, V4 to V6) leads, left axis deviation. Cardiac MRI (magnetic resonance imaging) when echo is questionable; exercise echocardiography. Ambulatory electrocardiogram for 24 to 48 hours for arrhythmia and sudden-death risk. Catheterization is not needed for diagnosis (only before pacemaker or surgery). Genetic testing is available but does not influence treatment. Differential: aortic stenosis, mitral regurgitation, hypertensive heart disease, athletic heart, restrictive cardiomyopathy Slides 70, 71, 72, 73, 74, 75

Treatment/Therapy

Outflow obstruction: a beta blocker (for example metoprolol) is the initial medication; nondihydropyridine calcium channel blockers (verapamil, diltiazem). Sudden-death prevention: amiodarone (slide 78: proven to reduce sudden cardiac death; the study guide notes it is an adjunct and the defibrillator is the proven device), disopyramide. ★ Avoid, if possible, preload or afterload reducers (nitrates; diuretics only with caution) and positive inotropes (digoxin, epinephrine, norepinephrine). Atrial fibrillation: anticoagulation, amiodarone, radiofrequency ablation. ICD (implantable cardioverter-defibrillator). Surgical myectomy (mitral regurgitation may resolve). Alcohol septal ablation: ethanol infused down the first septal branch of the left anterior descending artery causes a therapeutic infarct, and the septum takes months to thin. ★ Education: mild to moderate noncompetitive recreational activity, maintain hydration, avoid excess alcohol. Transplant if unresponsive to therapy Slides 76, 77, 78, 79, 81, 82

Mortality ★

High incidence of sudden death; sudden cardiac death is the leading cause of mortality in hypertrophic cardiomyopathy, highest in preadolescents and adolescents with extreme exertion, over 80% with ventricular fibrillation. Clinical course varies; heart failure symptoms gradually progress. Alcohol septal ablation and myectomy have comparable 30-day and long-term mortality (picture) Slides 58, 66, 81, 82

★ Carter flagged (Zoom recording times): amyloidosis is the most common cause in the United States (1:50:28); Kussmaul sign (1:55:08); constrictive pericarditis must be differentiated (1:56:11); the echocardiogram can look normal, so step to magnetic resonance imaging (1:58:21); the poorest prognosis of all (1:59:32).

Name of Condition

Restrictive cardiomyopathy (RCM) Slides 83, 84

Definition

Impaired diastolic filling (diastolic dysfunction) with reasonably preserved left ventricular chamber size; wall thickness normal or slightly increased Slide 84

Etiology (cause)

Idiopathic (no cause found). ★ Infiltrative: amyloidosis (most common in the United States). Storage: hemochromatosis (iron deposits). Genetic (less common than in other cardiomyopathies). Inflammatory: cardiac sarcoidosis (granulomas). Treatment-related: mediastinal radiation, chemotherapy (inflammation, then fibrosis, then restriction) Slides 86, 87

Epidemiology (who)

★ Rare; the least common cardiomyopathy Slide 84

Risk Factors

Prior radiation therapy, chemotherapy or systemic disease Slide 88

Pathology

Stiff ventricular walls impair diastolic filling, reducing cardiac output from reduced left ventricular filling volume; ejection fraction preserved until late; systolic function usually normal; bilateral atrial enlargement with common atrial fibrillation; right heart failure often more prominent. Fibrosis of the sinoatrial and atrioventricular nodes can cause heart block Slides 85, 99

Clinical Manifestation

Often presents late with pronounced cardiopulmonary symptoms. Right-sided heart failure: abdominal pain (hepatomegaly), bilateral pedal edema, ascites; progressive exercise intolerance and dyspnea, orthopnea, PND (paroxysmal nocturnal dyspnea), palpitations from atrial fibrillation (atrial thrombus risk), fatigue, syncope. Exam: more comfortable sitting; right failure may dominate; elevated JVP (jugular venous pressure); S1 and S2 normal but may be distant; secondary mitral or tricuspid regurgitation; S4 if no regurgitation; peripheral edema; ★ Kussmaul sign (paradoxical rise in jugular venous pressure with inspiration, also common in constrictive pericarditis); rales; congestive hepatomegaly, hepatojugular reflux, ascites Slides 88, 89, 90, 91, 92, 93

Diagnosis

ECG (electrocardiogram): low QRS voltage in amyloidosis; atrial fibrillation and bundle branch blocks common. Chest X-ray: cardiomegaly from atrial enlargement, congestion, effusions. Echocardiogram: preserved left ventricular ejection fraction (may fall late), marked atrial enlargement, near-normal systolic function and cavity, normal or thick walls, abnormal diastolic function; echo alone is not definitive against constrictive pericarditis, and cardiac MRI (magnetic resonance imaging) differentiates (pericardial thickening means pericarditis). Cardiac magnetic resonance imaging also when no cause is found; catheterization for ventricular pressure tracings; endomyocardial biopsy when imaging and genetic testing are negative or tissue would change management. Differential: ★ constrictive pericarditis (must be differentiated), acute pericarditis, tamponade, valvular disease, hypertrophic cardiomyopathy Slides 94, 95, 96, 97

Treatment/Therapy

Treat the underlying disorder. Diuretics for pulmonary and systemic congestion, with caution (they lower the preload that stiff ventricles depend on). Beta blockers and the calcium channel blockers verapamil and diltiazem to lengthen filling time, improve relaxation and reduce sympathetic drive. Anticoagulation for atrial thrombi. Permanent pacemaker for conduction disease. Cardiac transplantation Slides 98, 99

Mortality ★

★ The poorest prognosis of all types of heart muscle disease Slide 99

★ Carter flagged (Zoom recording times): 90% are postmenopausal women (2:01:12); it mimics a heart attack, so rule that out and admit to cardiology (2:03:05, 2:03:16); most recover completely (2:03:39).

Name of Condition

Takotsubo (stress) cardiomyopathy, also called broken heart syndrome (Takotsubo is Japanese for octopus trap) Slide 100

Definition

Transient regional systolic dysfunction, principally of the left ventricle; mimics acute coronary syndrome but without angiographic coronary artery disease or acute plaque rupture Slide 101

Etiology (cause)

Unknown Slide 101

Epidemiology (who)

★ 90% of cases are postmenopausal women Slide 101

Risk Factors

A significant emotional or physical stressor: death of a loved one, bad financial news, natural disasters, a newly diagnosed illness, surgery, withdrawal from illicit drugs, near drowning. Patients tend to have low or no cardiac risk factors Slides 101, 102

Pathology

Transient regional left ventricular systolic dysfunction: hypokinesis or akinesis of the midsegment and apical segment of the left ventricle Slides 101, 103

Clinical Manifestation

Chest pain and dyspnea are most common; palpitations, nausea, vomiting, syncope. Exam nonspecific; may look like acute coronary syndrome or acute heart failure: anxious, diaphoretic, tachydysrhythmias or bradydysrhythmias Slide 102

Diagnosis

Troponin I and T usually elevated; BNP (B-type natriuretic peptide) frequently elevated; ECG (electrocardiogram) with ST elevation and T wave inversion; transthoracic echocardiogram with mid and apical wall-motion abnormality; diagnosis confirmed by cardiac catheterization, most often showing normal coronary arteries Slide 103

Treatment/Therapy

★ Follow acute coronary syndrome protocols (no reliable early differences); admit to cardiology; empiric supportive care: beta blockers helpful, anticoagulation if left ventricular thrombus, diuretics if heart failure Slide 104

Mortality ★

No mortality figure given; ★ most patients recover completely Slide 104