OPT 820 Quiz 8 Cardiovascular System PDF

Quiz 8 - Cardiovascular System Peripheral Vascular System and Heart ​ Overview ○​ Vascular disease is responsible for more morbidity/mortality than any other category of human disease ○​ Responsible for 50% deaths ○​ Vascular pathology results in disease...

Quiz 8 - Cardiovascular System Peripheral Vascular System and Heart ​ Overview ○​ Vascular disease is responsible for more morbidity/mortality than any other category of human disease ○​ Responsible for 50% deaths ○​ Vascular pathology results in disease via two principal mechanisms: ​ Narrowing or obstruction of lumen ​ Progressively: atherosclerosis ​ Suddenly: embolism ​ Weakening of vessel walls ​ Causes dilation (pathologic) or rupture ​ ​ Hypertensive Vascular Disease ○​ Hypertension = elevated blood pressure ​ Old thought = systolic over 140, diastolic over 90 ​ Now it’s 130-139/80-89 for hypertension stage 1 ○​ ~25% of Americans have hypertension ​ Increased frequency with black americans with age ○​ Important risk factor for coronary heart disease and stroke ○​ Other problems accelerated by high blood pressure ​ Cardiac hypertrophy, congestive heart failure, aortic dissection, and renal failure ○​ If left untreated ​ 50% die of ischemic heart disease or congestive heart failure ​ 33% die of stroke ○​ Results from genetic predisposition and environmental factors causing increased blood volume and/or peripheral resistance ○​ Hypertensive crisis occurs with systolic pressures over 180 or diastolic pressures over 120 ○​ Hypertensive crisis is further subdivided ​ Hypertensive emergency: elevated BP + end organ damage ​ Hypertensive urgency: elevated BP and no end organ damage ​ End organ = kidneys, eyes, heart, brain = major organs supplied in circulatory system ○​ Vasculature gets very small but VERY important in these areas ○​ Hypertension damages the endothelium, accelerating the formation of atherosclerotic plaques ○​ Degenerative changes in the walls of large and medium sized arteries can lead to aortic dissection and cerebrovascular hemorrhage (hemorrhagic stroke)  ○​ 2 forms of small blood vessel disease are related directly to hypertension, both of which restrict flow through the affected vessels ​ Hyaline arteriolosclerosis ​ Hyperplastic arteriolosclerosis ​ Closely associated with severe hypertension ​ Vessels demonstrate “onionskin” concentric laminations of replicated basement membrane material - CHARACTERISTIC ​ Material encircles the vessel wall and narrows the lumen ​ Affects renal vasculature most commonly ○​ Both affect small arteries and arterioles, resulting in thickening of the vessel walls and narrowing of the lumen ​ ​ Arteriosclerosis ○​ Means hardening of the arteries ​ Generic term for arterial wall thickening and loss of elasticity ○​ 4 patterns ​ Atherosclerosis: most important, due to plaque build up, not same as arteriosclerosis ​ Monckeberg medial calcific sclerosis: calcific deposits in muscular arteries (typically in pts older than 50), usually not clinically significant ​ Fibromuscular intimal hyperplasia: non-atherosclerotic process affecting large muscular arteries, driven by inflammation and causes vascular stenosis ​ Arteriolosclerosis ​ ​ Atherosclerosis ○​ Most common type of vascular disease ○​ Causes most deaths secondary to cardiovascular disease ○​ Involves large and medium sized muscular arteries ○​ Characterized by intimal lesions called atheromas, protruding into the vascular lumina ​ Atheromatous plaques are raised lesions with a soft, yellow core of lipid covered by a firm, white fibrous cap ​ Yellow core is mainly cholesterol OR fat ○​ Medium sized vessels: atheroma may fully or partially occlude the lumen or promote endothelial damage that leads to thrombosis and occlusion ○​ Larger arteries: atheroma may weaken the wall and lead to an aneurysm or break loose and become circulatory emboli ○​ Major consequences: ​ Myocardial infarction (heart attack) ​ Cerebral infarction (stroke) ​ Aortic aneurysms ​ Peripheral vascular disease (gangrene of the legs) ​ ~25% of all deaths in the US ○​ Ubiquitous in developed nations ○​ Mortality rate for ischemic heart disease in the US is among the highest in the world ○​ Immigrants to the US acquire the same predisposition to atherosclerosis as a US born citizen ​ Due to additives in our processed foods ○​ Prevalence and severity of both atherosclerosis and ischemic heart disease are related to several risk factors, both constitutional and acquired ○​ Multiple risk factors have a multiplicative effect ​ Example: 2 risk factors increase risk by 4, 3 risk factors by 7 ○​ Constitutional risk factors (less easily controlled) ​ Increasing age, male gender until after female menopause, genetics ○​ Acquired risk factors (potentially controllable) ​ High cholesterol, hypertension, cigarette smoking, diabetes ○​ Genetics ​ Family history is the most important independent risk factor ​ Gender ​ Male > female ​ Myocardial infarction and other complications of atherosclerosis are uncommon in premenopausal women ​ After menopause, women may eventually have a higher risk when compared to men ○​ Increasing age ​ Accumulation of plaque is a progressive process, don’t usually become evident until they precipitate organ injury ​ Risk rises every decade of life ○​ Hyperlipidemia ​ Sufficient to stimulate development of atherosclerosis even in the absence of other risk factors ​ Major component is low density lipoprotein (LDL - bad cholesterol), delivers cholesterol to peripheral tissues ​ HDL mobilizes cholesterol from developing and existing atheromas and transport it to the liver for excretion in the bile ​ Diets high in saturated fats raise LDL levels, as does smoking and obesity ​ Diets with higher ratios of omega 3 fatty acids are beneficial, as is exercise and moderate consumption of alcohol ○​ Hypertension ​ Major risk factor, increases the risk of ischemic heart disease by 60% ​ Major cause of left ventricular hypertrophy ○​ Cigarette smoking ​ Many years of smoking 1 ppd increases risk by 200% ​ Quitting decreases risk substantially ○​ Diabetes mellitus  ​ Incidence of myocardial infarction is 2x as high in diabetic vs nondiabetic patients ​ 100x risk of atherosclerosis induced gangrene of lower extremities ○​ Additional factors about risk factors ​ 20% of all cardiovascular events occur in the absence of risk factors (do NOT conform to regular risk factors ​ Possible other contributors ​ Elevated c reactive protein, hyperhomocysteinemia, lipoprotein a levels, elevated procoagulants, stress, inactivity ○​ Response to injury hypothesis ​ Atherosclerosis is a chronic inflammatory response of the arterial wall to endothelial injury ​ Central components of the hypothesis ​ Chronic, focal endothelial injury ​ Accumulation of lipoproteins in the vessel wall ​ Monocyte adhesion to the endothelium ○​ Fatty streaks = lipid containing macrophages and smooth muscle cells, not raises and do not alter vascular flow ​ Smooth muscle proliferation ○​ Can convert a fatty streak into an atheroma ○​ The atheromatous plaque (atheroma) = fundamental lesion of atherosclerosis ○​ Principal cause of narrowing artery lumina in adults ○​ Typical plaque: white yellow, protrudes into lumen ○​ Tends to form at points of hemodynamic stress ○​ Size ranges up to several cm (inc pressure of blood) ○​ Coalescence of adjacent lesions occurs (multiple small ones that converge) ○​ As the plaque develops, it decreases luminal size of the artery and adjacent media ​ May result in ischemic changes of tissues and organs supplies by the vessel ○​ Atheroma may undergo one of four complications ​ Calcification (this is in plaque) ​ Fissuring, ulceration or rupture of the plaque may discharge debris into the bloodstream (cholesterol emboli) ​ Superimposed thrombosis (develops in areas of fissures or ulceration) ​ Hemorrhage can cause plaque rupture ○​ Best treatment: prevention ​ Primary interventions: pts who have not had serious complication of an atheroma ​ Know they have atherosclerosis but no heart attack or anything ​ Smoking cessation ​ Hypertension control ​ Weight loss ​ Increase exercise  ​ Lower cholesterol ​ Secondary interventions: pts who have had a previous complication (ex: myocardial infarction) ​ Pharmacologic intervention: lipid lowering drugs, use of antiplatelet medications ​ Surgical interventions ​ ​ Aneurysms and dissections ○​ An aneurysm is a congenital or acquired localized abnormal dilation of a blood vessel or the heart ​ Most develop wherever there is a marked weakness of the wall ​ May occur in any artery or vein, most common and significant in the aorta and intracranial arteries ​ Dilated due to weakness in the wall ○​ True aneurysms: involve all 3 layers of arterial wall ○​ False aneurysms: breach in the vascular wall leading to an extravascular hematoma ​ More of an injury ○​ Aneurysms may be ​ Saccular (spherical in shape) ​ Fusiform (sessile and involving a long segment of the vessel) ○​ Arterial dissection refers to blood spreading between layers of the wall of an artery​ ​ Abdominal Aortic Aneurysm (AAA) ○​ Atheroscloeric aneurysms ○​ Most common type of aortic aneurysm ○​ Most frequent age 50+ ○​ Strong male predominance ○​ Atherosclerosis = primary cause ○​ Dilations occur in areas of severe atherosclerosis that has caused damage to the tunica media of the vessel wall ○​ Most occur in the abdominal aorta below the level of the renal arteries ○​ If untreated ​ Rupture with massive or fatal hemorrhage ​ Impingement upon adjacent structure ​ Occlusion of adjacent vessel ​ Embolism ○​ Risk of rupture correlates to size ​ About 0% 6cm in diameter ○​ Aneurysms >5cm managed aggressively ​ Surgical bypass with prosthetic grafts ​ Allows passageway for blood to go through  ​ Operative mortality for unruptured aneurysms is 5% and increases to 50% for patients undergoing emergency surgery after rupture ​ Monitor for hypertension and refer​ ​ Thoracic Aortic Aneurysm ○​ Associated with hypertension and Marfan syndrome ○​ May give rise to ​ Encroachment on mediastinal structures, breathing difficulties, dysphagia, persistent cough, pain, cardiac disease, rupture ​ ​ Aortic dissection ○​ Arises from intimal tears that allow blood to enter the wall of the aorta ○​ Blood spreads along laminar planes of the media for variable distances ○​ Channel often ruptures outward to cause massive hemorrhage ​ 90% of intimal tears occur within 10 cm of aortic valve ○​ Catastrophic event ○​ Tear is typically 1-5 cm long ○​ Blood enters and spreads along laminar planes ​ Can spread proximally (toward the heart) or distally ○​ May rupture into the pericardial, pleural, or peritoneal cavity or elsewhere ​ Most common cause of death = rupture ○​ In some cases, blood reenters the aortic lumen through a second intimal tear, resulting in a double-barreled aorta ○​ Occurs in two groups ​ Men aged 40-60 with hypertension (90% of cases) ​ Younger patients with systemic or localized abnormalities of connective tissue affecting the aorta (like patients with Marfan syndrome) ○​ In ⅕ cases, the dissection occurs in areas of cystic medial degeneration (cystic medial necrosis)​ ​ Elastin fragmentation with formation of small clefs or cyst like spaces filled with extracellular connective tissue matrix ​ Noted most frequently in patients with hereditary defects of connective tissue such as Marfan syndrome ○​ In most cases, onset of dissection causes excruciating chest pain that will migrate as the dissection advances ○​ Prognosis depends on promptness of diagnosis and level of aorta affected ○​ Dissections restricted to the descending aorta are at lower risk for rupture and often are treated only with antihypertensive medications, 75% survive ○​ Those with more proximal dissections are repaired with a prosthetic graft ​ Treatment is most effective if instituted within minutes to hours after onset of symptoms ​ Overall 10 yr survival of only 40-60%​ ​ Vasculitis  ○​ Nonspecific term: inflammation of vessels ○​ Vessels of any type in virtually any organ can be affected ○​ Most cases are related to immune mediated inflammation or direct invasion by an infection ​ Immune mediated cases may be primary or due to an infection that induced formation of immune complexes or triggered cross reactivity ○​ Associated systemic manifestations such as fever, myalgia, arthralgia and malaise may be present - makes diagnosis more difficult ​ Basic symptoms of an infection, so hard to diagnose ​ Diagnosis is critical since immunosuppressive therapy is appropriate for the immune mediated variants but potentially harmful for infectious vasculitis ​ ​ Giant cell (temporal) arteritis ○​ Giant cell arteritis is the most common form of vasculitis among older adults (50+) ○​ Autoimmune reaction directed toward an antigen on the blood vessel ○​ Predominantly affects larger arteries of the head ​ Temporal, vertebral, and ophthalmic ○​ Symptoms: non-specific fever, fatigue, and weight loss; eventually develop severe pain and headaches ○​ If the superficial temporal artery is involved, it may become nodular and painful to palpation ○​ Most serious complication = ocular involvement ​ ½ patients affected ​ Patients may experience double vision or vision loss ○​ Diagnosis = biopsy temporal artery, granulomatous inflammation w giant cell ​ See TRUE granulomas ○​ Tx = corticosteroids or anti-TNF factor​ ​ Kawasaki disease ○​ Acute illness of infancy and early childhood (most younger than 4) ​ Leading cause of acquired heart disease in children ○​ Unknown cause, but most patients develop it after exposure to one of a variety of infectious agents which triggers an immune reaction ○​ Classic clinical features ​ Fever, bilateral conjunctivitis, erythema and edema of the palms and soles with subsequent desquamation (shed or malt) ○​ Followed by: ​ Isolated cervical lymphadenopathy (severe!) ​ Rash of erythematous plaques (red and flat) or pastures (raised with pus) ○​ Oral mucosa involvement common: diffuse erythematous mucositis (very red mucus membranes)  ○​ Tongue swollen, red with enlargement of the fungiform papillae (strawberry tongue) - also seen in Scarlet fever, not specific for Kawasaki ○​ In ~20% of untreated patients, the coronary arteries become involved ​ Can lead to asymptomatic vasculitis or sudden death ​ Risk reduced with therapy of IV immunoglobulins ○​ In patients without coronary involvement, disease is self limited and responds to NSAIDs​ ​ Granulomatosis with Polyangiitis ○​ Necrotizing vasculitis of unknown etiology ○​ Can be limited to respiratory tract or be widespread (kidneys, eyes, skin, heart) ○​ Oral involvement may lead to the diagnosis ​ Classic lesion is strawberry gingivitis, hyperplastic, erythematous, hemorrhagic lesions of the gingiva with granular surface ○​ Pts will have antiproteinase-3 antineutrophil cytoplasmic antibodies ○​ Clinical features ​ Pneumonitis, chronic sinusitis, mucosal ulcerations of the nasopharynx, renal disease ○​ 80% succumb in a year if left untreated, can be treated successfully with various medications ​ Make an appropriate diagnosis!​ ​ Thromboangiitis obliterans ○​ AKA Buerger disease ○​ Remitting and relapsing inflammatory disorder ○​ Leads to thrombosis of medium and small sized arteries ​ Inflammation spreads to veins and nerves ○​ Occurs in young patients who smoke ○​ Patients may have Raynaud phenomenon ○​ Classic features are claudication, color changes, and temperature changes associated with severe pain ​ Claudication = pain induced by exercise, usually in legs ○​ Overlying ulceration and frank gangrene may occur ○​ Smoking cessation brings dramatic relief if done in early stages, established vascular lesions DO NOT respond ​ ​ Raynaud phenomenon ○​ Results from exaggerated vasoconstriction of digital arteries and arterioles ○​ Involved digits characteristically show red, white, and blue color changes from most proximal to most distal ​ Correlated with proximal vasodilation (red more blood flow), central vasoconstriction (white less blood flow), and distal cyanosis (blue lost oxygen) ○​ Can be primary disease or secondary to many conditions  ○​ Primary: ​ Exaggeration of central and local response to cold or emotion in otherwise healthy individuals ​ No underlying condition, just responds ​ Affects 5% of the population ​ Predilection for young women ○​ Secondary: ​ Vascular insufficiency of the extremities in the context of arterial disease caused by other entities ​ May be the first manifestation of underlying disease ​ Refer for evaluation ​ Underlying disease include systemic lupus erythematosus or scleroderma ​ ​ Venous disorders ○​ 2 most common venous disorders: varicose veins and phlebothrombosis ○​ 90% of venous disease ○​ Can lead to significant clinical citations like embolism or venous stasis with edema​ ​ Varicose veins ○​ Abnormally dilated, tortuous veins produced by prolonged increase in intraluminal pressure and loss of vessel wall support ○​ Risk factors ​ Advancing age: due to loss of tissue tone, atrophy of muscles, and degeneration of vessel walls ​ Standing or sitting for long periods of time can cause elevated venous pressures, which may lead to venous stasis and pedal edema ​ Pregnancy ○​ 20% of adult males and 30% adult females develop lower extremity varicose veins ○​ Most varicose veins are asymptomatic ○​ Those who have complications may experience incompetent valves ​ Lack of passage of blood into veins ​ Leads to venous stasis congestion, edema, and thrombosis ​ Marked stasis and edema also can result in pain, stasis dermatitis, cellulitis, and ulceration ○​ Although varicose veins frequently thrombose, embolization to the lungs is uncommon from superficial veins (in contrast to deep veins) ○​ Varicosities occur in 2 other sites of note: ​ Esophageal varices - associated with portal hypertension of cirrhosis, rupture can be fatal ​ Hemorrhoids - uncomfortable or possible ulceration ​ ​ Phlebothrombosis (thrombophlebitis)  ○​ Interchangeable designations for thrombus formation in legs ○​ Deep leg veins account for more than 90% ○​ Local complaints include pain and edema ○​ Most important aspect: they can give rise to pulmonary emboli leading to morbidity and mortality ○​ Predisposition in patients: experience cardiac failure, pregnancy, obesity, postoperative states, prolonged bed rests, and immobilization ○​ Abdominal cancers (lung, colon, pancreas) who experience phlebothrombosis are at an increased risk as well (Trousseau sign of Malignancy, migratory thrombophlebitis) ​ Medical sign involving episodes of vessel inflammation due to blood clot (thrombophlebitis) which are recurrent or appearing in different locations over time (thrombophlebitis migrans or migratory thrombophlebitis) ​ ​ Lymphedema ○​ Accumulation of interstitial fluid in tissue as a result of lymphatic insufficiency or occlusion ○​ Divided into primary and secondary causes ○​ Patients develop lymphatic dilation and edema distal to the site of obstruction ○​ Persistent edema leads to interstitial fibrosis that may produce a thickening of the skin resembling the texture of an orange peel (peau d orange) ○​ Brawny induration (pathologic hardening and thickening of tissues), infection and chronic skin ulcerations can be noted in chronically affected areas ○​ Specific names for collections in certain cavities ​ Chylous ascites (peritoneal cavity) ​ Chylothorax (chest cavity) ​ Chylopericardium (cardiac activity) ○​ Primary lymphedema can occur as an isolated congenital defect (called simple congenital lymphedema) or as familial Milroy disease (aka heredofamilial congenital lymphedema) ​ Both are caused by faulty development of the lymphatic channels ​ Both are present at birth ​ Legs are most commonly affected site ○​ Secondary lymphedema arises secondary to a number of processes which have the ability to disrupt lymphatic drainage ​ Common causes include blockage, surgical excision, scarring, etc. ​ ​ Tumors of vessels: benign ○​ Vast majority identified in infants at birth ○​ Hemangioma: benign tumor of blood vessel endothelium (can have well formed BV inside of them) ​ Classified as capillary or cavernous, depending upon the size of the proliferating vessels  ○​ Lymphatic malformation (formerly lymphangioma): benign tumor of lymphatics, can also have channels of various size ​ No longer considered true neoplasms ​ ​ Tumors of vessels: malignant ○​ Malignant tumors of blood and lymphatic vessels are called angiosarcoma and lymphangiosarcoma ​ NO SUCH THING AS A HEMANGIOSARCOMA! ○​ Lymphangiosarcoma usually develops after prolonged lymphatic obstruction and lymphedema, most common in the edematous arms of patients treated by radical mastectomy for breast cancer ​ Predisposed to secondary cancer, lack of drainage once breast tissue is removed ​ Has an peau d’orange look ○​ Oral angiosarcoma is not only in the oral cavity, can be seen in the scalp ○​ Kaposi sarcoma is a low grade malignancy of vascular endothelium ​ Most commonly noted with patients with AIDS ​ Assoc with HHV8 ​ Patients have a scattered purplish patches that typically continue to enlarge into raised plaques and ultimately tumor nodules ​ In oral cavity in the US… typically see with AIDS patients The Heart​ ​ Overview ○​ Heart disease is the principal cause of disability and death in all industrialized nations, accounting for 40% of all deaths in the US ○​ Major categories of heart disease ​ Coronary artery disease, hypertensive disease, heart disease related to pulmonary pathosis, valvular heart diseases, primary myocardial diseases, congenital heart disease ○​ Congestive heart disease represents the common endpoint if many different heart disease ​ ​ Congestive heart failure (CHF) ○​ Multisystem derangements that arise when the heart cannot eject the blood delivered to it by venous system ​ Right to lungs to left to systemic ○​ Blood backs up in the venous system with diminished arterial filling, therefore the tissue is not adequately oxygenated because of the reduced blood flow ○​ Underlying causation may be impaired myocardial contractility (myocardial damage) or increased workload placed on the heart (hypertension, pulmonary disease) ​ Not pumping hard enough or pumping too much  ○​ Decreased cardiac output initially triggers a neurohumoral response​ ​ Leads to a release of catecholamines which produces a more forceful contraction and an increase in heart rate ​ Triggers adrenal medulla ​ Heart attempts to further compensate by myocardial hypertrophy which increases cardiac output ​ Eventually mechanisms become ineffective and systemic congestion develops ​ Increase in cardiac musculature requires increased oxygen that cannot be provided and cardiac output falls ​ Congestive heart failure is manifested by changes away from the heart in various organ systems ○​ CHF is divided into left sided and right sided heart failure ​ Failure of one side cannot exist for long without failure of the other ○​ Most patients present with full blown CHF and clinical manifestations of both right and left sided failure ​ ​ Left sided heart failure ○​ Most common causes ​ Ischemic heart disease ​ Systemic hypertension ​ Mitral or aortic valve disease ​ Primary diseases of the myocardium ○​ Left ventricle is usually dilated and manifestations are seen most in lungs, kidney and brain ○​ As the left ventricle fails to keep pace with the venous return from the lungs, hydrostatic pressure in the pulmonary circuit builds ​ Leads to edema and congestion ​ Inc pressure in pulmonary circulation, all pressure ends up in lungs ○​ Major clinical manifestations of left sided heart failure are ​ Dyspnea (breathlessness), orthopnea (dyspnea upon reclining), paroxysmal nocturnal dyspnea (extreme dyspnea that wakes the patient from sleep) ○​ In addition, cough can produce frothy blood tinged sputum ​ Congestion in lungs that leads to inflammation ○​ LSHF also results in reduction of renal perfusion with activation of the sympathetic nervous system and renin-angiotensin system ○​ Aldosterone secretion is increased ​ Result in salt and water retention, and increase in total blood volume ○​ In addition, decreased renal filtration leads to increased nitrogenous products remaining in the blood (prerenal azotemia) ○​ Brain experiences hypoxia causing irritability, restlessness and even stupor and coma (hypoxic encephalopathy)  ​ All of this happens due to overload, but only adds to the overload when it happens​ ​ Right sided heart failure ○​ Most common cause is left sided heart failure ​ Left ventricular failure is associated with pulmonary congestion and elevation in pulmonary arterial pressure ​ Puts an increased burden on the right side of the heart ○​ Pure right sided failure most often occurs with cor pulmonale ​ Right ventricular strain produced by intrinsic disease of lungs or pulmonary vasculature ​ Right ventricle is burdened by increased resistance within the pulmonary circulation, dialtes, and becomes hypertrophic ○​ Isolated right sided heart failure is less common ○​ Occurs in pts with disease of lung parenchyma and/or pulmonary vasculature that result in pulmonary hypertension ​ Cor pulmonale - right heart failure with pulmonary hypertension ○​ Morphologic and clinical effects ​ Produced engorgement of the systemic and portal venous systems ​ Unliked left sided, pulmonary congestion is minimal ○​ ONLY when there is right sided failure too ○​ In right sided failure, pulmonary congestion is minimal, but major consequences are systemic venous congestion and soft tissue edema ​ Sits and static… see leakage in soft tissue ○​ Major organs affected are liver, spleen, kidneys, brain, subcutaneous tissues and portal area of venous drainage ○​ In the liver, there is congestion of the sinusoids of the venous system creating the pattern termed: nutmeg liver (marble pattern) ​ Sinusoids may rupture from engorgement (central hemorrhagic necrosis) ​ With severe hypoxia, the hepatocytes around the central vein can undergo necrosis (centrilobular necrosis) ○​ In the spleen and kidneys, there is marked congestion with enlargement ​ Lack of perfusion in kidneys and brain results in similar clinical findings as those describes under left sided heart failure ​ In addition, peripheral edema develops and can result in ascites (fluid in abdominal cavity (sometimes seen in alcoholics)) or even anasarca (full body swelling) ​ ​ Congenital heart disease ○​ Abnormalities of the heart or great vessels present at birth ○​ Affects 1% of newborns ○​ Range from severe anomalies that cause death prenatally to mild lesions with minimal symptoms ○​ Etiology is almost always unknown (90%), those that are known include ​ Congenital rubella infection (NOT measles, german measles so comp different) ​ Maternal diabetes ​ Chromosomal abnormalities - trisomy 21 ○​ 3 majors groups of CHD ​ Left to right shunt ​ Most common type of congenital cardiac malformation ​ Patients are not initially cyanotic ​ In time, pressure overload of right heart and pulmonary system can lead to reversal of flow, unoxygenated blood is then shunted to the left side of the heart (Eisenmenger syndrome) ​ If left untreated, high volume shunts can produce pulmonary hypertension and cause cyanosis and congestive heart failure ​ Types of left to right shunts ○​ Atrial septal defects ​ Arise from failure of foramen ovale to close ​ Opening in the atrial septum covered by a flap, it acts as a one way valve allowing blood to flow from right to left during intrauterine life ​ At birth, the pressure increases on the left side of the heart, resulting in functional closure of the foramen ovale ​ Defects arise when flap does not overlap properly or fails to permanently fuse with the septum ​ Atrial septal defects usually go unnoticed unless reversal of flow results in cyanosis (less than 10%) ​ NOT born with cyanosis tho ​ Defects less than 1 cm are well tolerated, infective endocarditis is rare - no premed needed ​ Larger defects require repair early in life to prevent pulmonary and cardiac complications (larger than 1 cm) ○​ Ventricular septal defects ​ Most common congenital heart defects at birth ​ Because many small VSDs close spontaneous in childhood, the overall prevalence of VSDs in adults is lower than that of atrial defects ​ Most are noted in associated with other cardiac abnormalities (multiple congenital issues) ​ Large lesions result in difficulties from birth, smaller lesions may not be detected until later  ​ Defects smaller than 0.5 cm tend to close spontaneously or are well tolerated, although these latter patients are at risk for infective endocarditis (unlike patients with ASDs) ​ Larger defects require surgical closure to prevent continuation of a severe left to right shunt often complicated by pulmonary hypertension and congestive heart failure ○​ Patent ductus arteriosus ​ Represents failure of closure of a normal channel that courses between the pulmonary artery and the aorta during fetal development ​ Permits blood to bypass the unoxygenated lungs ​ Normally, vessel closes within the first day or two of life ​ Occasionally, there is no vessel, only a defect in the approximated pulmonary artery and aorta ​ Although no cyanosis is present at birth, early closure is recommended to prevent infective endocarditis and pulmonary cardiac complications ​ AT BIRTH, baby will NOT be cyanotic ​ Right to left shunt (cyanotic shunts) ​ Distinguishable from left to right shunts because cyanosis is present at birth ​ These defects permit the passage of non oxygenated blood into the systemic circulation and also allow emboli arising in peripheral veins to bypass the filtration of the lungs and enter the peripheral circulation (paradoxical embolism) ○​ Septic emboli have resulted in brain abscesses ​ Lack of proper oxygenation results in hypoxemia, hypnea, and cyanosis ​ Severe, long standing cyanosis is associated with clubbing of fingertips (hypertrophic osteoarthropathy) and polycythemia (not polycythemia vera) ○​ UNIQUE CHARACTERISTIC ​ Tetralogy of Fallot (most common) ○​ Most common right to left shunt ○​ Accounts for 5% of all congenital cardiac malformations ○​ Most common cause for cyanotic congenital heart disease ○​ 4 major alterations occur ​ Ventricular septal defect  ​ Dextroposed aorta which overrides the defect (straddles the defect and septum) ​ Obstruction to right ventricular outflow (stenotic pulmonary artery or valve) ​ Right ventricular hypertrophy ○​ Severity varies with degree of obstruction of right ventricular outflow ​ Mild obstruction resembles a VSD with left to right shunt ​ More commonly, severe obstruction leads to cyanosis and right to left shunt with polycythemia and clubbing of fingertips ​ Surgical repair is performed to avoid infective endocarditis, systemic emboli, brain abscesses and cardiopulm problems ​ Transposition of the Great Arteries ○​ Occurs when right ventricle and the pulmonary artery arise from the left ventricle ○​ Incompatibilite with extrauterine life unless some type of shunt (ASD, VSD, or PDA) allows oxygenated blood to reach the aorta ○​ Early surgical correction is mandatory (some are done in utero) ​ Obstruction ​ Usually the result of pulmonic valve stenosis, aortic valve stenosis, or coarctation of the aorta ​ 2 types of obstructive anomalies ○​ Coarctation of the aorta (common) ​ Narrowing or constriction of the aorta ​ Two main types ​ Aorta is constricted proximal to a patent (open) ductus arteriosus ○​ Symptomatic early in life ○​ Presents with cyanosis localized to lower half of the body ○​ Femoral pulse often weaker than those of the upper extremities ○​ Surgical resection and replacement by a prosthetic graft is necessary to prevent death ​ Constriction of the aorta is just distal to the ligamentum arteriosum (closed ductus arteriosus)  ○​ Often asymptomatic and marge unrecognized well into adulthood ○​ Typically hypertension of the upper extremities and lower pressure in the lower extremities ○​ Severity varies with degree of coarctation ○​ Valvular anomalies ​ Anomalies of the aortic or pulmonary valve can create a stenosis or occlusion of the lumen ​ In some cases, no problems arise ​ In others, infective endocarditis or degenerative changes can be seen ○​ A shunt is an abnormal communication between chambers or blood vessels - leads to mix of oxygen and deoxygenated blood ○​ Shunts permit blood flow from left heart to right heart and vice versa ​ ​ Ischemic Heart Disease (IHD) ○​ AKA coronary heart disease ○​ Spectrum of clinical disorders resulting in insufficient blood supply to the heart ○​ Leading cause of death in the US and other industrialized nations ○​ Four clinical syndromes of IHD ​ Angina pectoris (chest pain) ​ Intermittent chest pain caused by transient, reversible myocardial ischemia ○​ Manifest clinically as pressure, heart burn, etc. ​ Three patterns ○​ Typical angina (stable) ​ Occurs in the presence of fixed atherosclerotic narrowing ​ Precipitated by exertion or emotional stress ​ Relieved by rest or nitroglycerin ​ Nitroglycerin decreases venous return, reduces cardiac work, and can increase myocardial blood flow ​ Pain classifically presents as substernal crushing or squeezing pain which may radiate down the left arm or jaw (referred pain) ○​ Prinzmetal angina (variant) ​ Attributed to vasospasm but not invariably superimposed on fixed stenosis ​ Typically arises at rest, pt is NOT stresses ​ May awaken the patient from sleep ​ Best relieved with vasodilators such as nitroglycerin  ○​ Unstable angina (crescendo) ​ Associated with plaque disruption and superimposed thrombosis, distal embolization of the thrombus, and or vasospasm ​ Produces the most intense and prolonged pain and is precipitated by progressively less exertion ​ Presence forewarns of the immediate danger of MI ​ Acute myocardial infarction (MI heart attack) ​ Chronic IHD with congestive heart failure ​ Sudden cardiac death ○​ Syndrome are all late manifestations of coronary atherosclerosis ○​ A lesion obstructing 70% of a vessel lumen generally causes symptomatic ischemia (angina) only during periods of increased demand ○​ A 90% closure can lead to symptomatic ischemia at rest ​ If occlusion enlarges at a slow rate, collateral blood vessels may develop, bypass the occlusion and protect against MI ○​ Prognosis depends on these fixed narrowed areas and dynamic alterations in the morphology of the coronary plaque ​ Includes: ​ Acute plaque changes ○​ Fissuring, hemorrhage into the plaque and overt plaque rupture with embolization of atherosclerotic debris into distal coronary arteries ○​ Changes result in enlargement of the plaque and promote thrombosis at the site ​ Coronary artery thrombosis ○​ Typically arises from a plaque rupture that exposes the subendothelial collagen and initiates platelet aggregation and local coagulation ○​ Subsequent clot may totally block the vessel or produce a nonocclusive mural thrombus (within wall of BV) ○​ Small fragments of the rhombus also can embolize and block distal branches of coronary artery ​ Coronary artery vasospasm ○​ May cause a reduction of cardiac blood flow ○​ Unknown mechanism of action ○​ Most cases, preexisting atherosclerosis is present ○​ Although idiopathic, spasm may be triggered by vasospastic mediators related during platelet aggregation or influenced by other factors such as smoking ​ In approx ⅔ of patients, MIs occur in ruptured plaques with 50% or less occlusion ​ MAJORITY OF MIs ARISE IN PTS WITHOUT WARNING SIGNS  ○​ Plaques contain cholesterol and inflammatory cells with overlying caps of collagen and smooth muscle ​ Plaques with thick caps and fewer inflammatory cells are less prone to rupture ○​ Cholesterol lowering statins may reduce plaque inflammation and therefore reduce prevalence of plaque rupture ○​ Adrenergic stimulation (like when walking or secondary to emotional stress) increase blood pressure and local vasospasm, both of which places stress on vulnerable plaque ​ Lower amt of cholesterol to lower amt of rupture ​ ​ Myocardial Infarction ○​ Necrosis of the heart muscle resulting from ischemia ○​ Acute MI (heart attack) is leading cause of death in industrialized nations ○​ Process may occur at any time at any age ○​ Strong male predominance during women’s reproductive years, then changes with menopause ​ IHD is most common cause of death in elderly women ○​ Risk factors for acute MI are same as atherosclerosis ○​ Most MIs are caused by coronary artery thrombosis in areas of preexisting atherosclerosis ○​ Myocardial necrosis begins within 20-30 mins following coronary artery occlusion ○​ Inner ⅓ of vent wall is termed the subendocardial myocardium ​ Portion of the heart is the last area to receive blood from the coronary vessels and is the least well perfused zone (so start to see damage here first) ○​ Upon coronary occlusion, necrosis begins in this zone as subendocardial infarction ○​ If ischemia continues, the necrosis may involve the full thickness of the ventricular wall and become a transmural myocardial infarction ○​ Transmural necrosis is not inevitable and may be prevented by thrombolytic agents, angioplasty, etc or sufficient flow around the blockage to allow survival of all the myocardium except for the most sensitive inner zone ○​ Necrosis develops over several hours and usually does not reach full size for 3-6 hours ○​ Diagnosis based on 3 sets of data ​ Clinical history ​ Electrocardiogram changes ​ Alterations in serum enzymes ○​ Clinically, the onset usually is sudden with severe, constricting, crushing or burning pain ○​ The pain may be substernal or precordial ○​ Pain often radiates to the neck, mandible, epigastrium, shoulder, or left arm ○​ Pulse generally is rapid and weak and patient is often sweating ○​ Breathlessness may occur due to pulmonary congestion and edema secondary to decreased cardiac output ○​ In contrast to angina pectoris, pain of MI typically lasts from 20 mins to several hours and is not relieved by nitroglycerin or rest ​ STILL admin nitroglycerin ○​ Some patients suffer from less specific symptoms such as burning or substernal or epigastric discomfort resembling heartburn ○​ In about 20% patients, the onset is entirely asymptomatic and the disease is discovered later by routine electrocardiogram ​ Often missed in females ​ Electrocardiogram changes are usually evident from the onset of the attack, but can be missed, changes may be minimal or absent ○​ Alterations in serum enzymes support the diagnosis as ischemically damaged cells leak cytoplasmic enzymes ​ Elevations of serum creatine kinase, particularly the MB isoenzyme is of great specificity as it is specific for myocardium damage ​ CK-MB peaks in 24 hrs and declines within 72 hrs ​ An absence of a change in the levels of CK and CK-MB during the first two days after development of chest pain rules out diagnosis of MI ​ Serum levels of certain contractile proteins, terms troponins, also have proven beneficial ​ Cardiac troponin (cTnI) appears specific for myocardium and remains elevated for 4-7 days after the acute event, allowing diagnosis of an MI long after CK-MB ○​ A number of cardiac complications can arise secondary to MI ○​ Risk of complications and prognosis correlates to the infarct size, site, and thickness of the heart all that is damaged (subendothelial or transmural) ○​ Overall total mortality is about 30% within the first year and thereafter there is a 3-4% year mortality ○​ Several new therapeutic approaches have been utilized ○​ When early medical intervention is available, use of intracoronary thrombolytic medications (clot busters) such as streptokinase or tPA (tissue plasminogen activator) may limit the infarction to subendocardial zone ○​ In patients with unstable angina or who have survived their MI, the stenotic coronary arteries can be bypassed with a portion of leg vein or internal mammary artery (coronary artery bypass grafting, CABG) ​ In time (5-10 yrs), many of these bypasses redevelop stenosis, especially those utilizing veins ○​ Transluminal angioplasty removes (rotating blade), destroys (laser) or compresses (balloon) stenosing atherosclerotic plaques though many of these stenotic areas redevelop  ○​ Stents have been placed at the time of angioplasty in an attempt to prevent redevelopment of the stenosis ​ ​ Arrhythmias ○​ Heart contains a conduction system that regulates the rate and rhythm of cardiac contraction ○​ Arrhythmia refers to abnormalities in myocardial conduction and can be sustained or sporadic ○​ Arrhythmias can manifest as ​ Fast heart rate (tachycardia) ​ Slow heart rate (bradycardia) ​ Irregular rhythm without functional ventricular contraction ​ Irregular rhythm without functional contraction (ventricular fibrillation) ​ No contraction (asystole) ○​ Loss of cardiac output due to sustained arrhythmia can produce produce ​ Lightheadedness ​ Loss of consciousness (syncope) ​ Sudden cardiac death ○​ Ischemia injury is the most common cause of rhythm abnormalities ​ ​ Sudden cardiac death ○​ Defined as unexpected death from cardiac causes within 24 hours of onset ○​ Majority are related to ischemic heart disease ○​ A lethal arrhythmia almost always is the cause of death ○​ Sudden cardiac death is the initial manifestation of ischemia heart disease in about 50% of patients ○​ Patients vulnerable to sudden cardiac death often receive implantation of an automatic cardioverter-defibrillator (not the same as a pacemaker) that sense the electrically counteracts episodes of fibrillation​ ​ Hypertensive heart disease ○​ Systemic hypertensive heart disease (left sided) ​ The combination of hypertension and associated left ventricular hypertrophy ​ With hypertrophy, the heart often is able to maintain adequate output for decades ​ As the wall thickens ○​ Oxygen demand continuously increases ○​ Myocardium stiffens: less flexibility and decreased pump efficiency ​ Hypertensive heart disease is usually asymptomatic and picked up secondary to detection of ventricular hypertrophy upon radiographs or echocardiograms  ​ Symptoms, if present, include headaches, dizziness, nosebleed and or occasionally postural unsteadiness ​ These changes predispose the heart to arrhythmias, MI or congestive heart failure ​ Patients with appropriate therapy can live a normal lifespan ​ Effective control is obtained through the use of antihypertensives ​ Without intervention, worsening ischemic disease, progressive renal damage or stroke can happen ​ Heart failure in the setting of hypertension is associated with a poor prognosis, only 50% patients surviving an additional 5 years ○​ Pulmonary hypertensive heart disease (right sided) (cor pulmonale) ​ Right ventricular enlargement secondary to pulmonary hypertension ​ HIGH BP leads to INC right ventricular enlargement ​ Results from primary disorders of the lung parenchyma or pulmonary vasculature (emphysema, interstitial pulmonary fibrosis, or primary pulmonary hypertension) ​ Acute cor pulmonale ​ Right ventricular dilation following the heart strain typically associated with massive pulmonary embolization (blood clot that dislodges) ○​ Process may be rapidly fatal depending on the size and location of the blockage ​ Chronic cor pulmonale ​ Right ventricular hypertrophy secondary to prolonged pressure overload seen in association with any one of a number of disorders that have the ability to create pulmonary hypertension ○​ Chronic onset allows sufficient time for development of compensatory right ventricular hypertrophy ○​ Hypertrophy may increase the thickness of the wall to the point that the right ventricle may be as thick as the left ○​ In most pts, signs and symptoms of congestive heart develop with lethal ventricular arrhythmias ultimately occurring in some ​ ​ Rheumatic fever and rheumatic heart disease ○​ Rheumatic fever ​ An acute, immunologically mediated, multisystem inflammatory disease that follows a group A B-hemolytic streptococcal pharyngitis (“Strep throat” ​ It occurs in 3% of patients with the strepto infection, most often in children ​ Group A release toxins -> scarlet fever -> rheumatic fever ​ Process arises 2-3 weeks after the infection, at which time pharyngeal cultures for strepto are negative  ​ Ab directed against specific strains cross react with self Ags in the heart, joints, skin and other tissues ​ Joints: nonspecific arthritis that primarily involves large joints (soreness) ​ Skin: scattered subcutaneous nodules and erythematous, maculopapular eruptions exhibiting central clearing upon enlargement ​ Heart: myocarditis ○​ Acute rheumatic heart disease ​ Characterized by discrete inflammatory foci within a variety of tissues ​ Myocardial inflammatory lesions are called Aschoff bodies ​ Pathognomonic for rheumatic fever ​ Comprised of lymphocytes, plasma cells, and macrophages called Anitschkow cells ​ Acute rheumatic fever affects all three layers of the heart (pancarditis) ​ Valve involvement causes inflammatory vegetations known as verrucae ○​ NOT HPV related, due to inflam ​ These acute cardiac changes may resolve or lead to chronic rheumatic heart disease ​ After the initial attack, patients are predisposed to future attacks of acute rheumatic fever with each subsequent streptococcal infection ​ Carditis worsens with each recurrence and the damage is cumulative ○​ Chronic rheumatic heart disease ​ Arises secondary to fibrocalcific thickenings of the affected valves ​ Results in stenosis or improper closure, causing regurgitation ​ Changes usually appear after at least 10 yrs ​ Presentation varies according to which valves are involved, but in time, cardiac decompensation often necessitates valvular replacement ​ ​ Infective endocarditis ○​ Colonization of the heart valves or mural endocardium by microorganisms leading to formation of thrombotic masses of organisms ​ Termed infective vegetations ○​ Virtually any bacterial species as well as fungi and other agents can be responsible ○​ 2 degrees of severity ​ Acute ​ Arises secondary to highly virulent organisms capable of infecting normal hearts ​ S. aureus = leading cause  ​ Process has as stormy onset with rapidly developing fever, chills, weakness, and lassitude (lack of energy) ​ Heart murmur often present secondary to vegetations ​ Without appropriate ab therapy tx, death can occur within days ​ Subacute ​ Arises secondary to organisms of low virulence (like a hemolytic strepto) which colonize sites of previous cardiac abnormality ​ S. viridans is one of the most commonly involved organisms ​ Clinical presentation is more subtle with mild fever, fatigue, weight loss ​ Murmurs are not as frequent, most patients survive with appropriate ab therapy ○​ Less severe overall​ ​ Primary myocardial diseases ○​ Cardiomyopathy (primary, noninflammatory, unknown etiology) ​ Dilated - hypocontraction ​ Markedly dilated hypocontracting hearts with impaired contractile function ​ Usually concurrent ventricular hypertrophy ​ Up to 50% hereditary, some assoc with variety of insults, some are idiopathic ​ Fundamental defect is ineffective contraction ​ Process presents as a slowly developing congestive heart failure that ultimately becomes refractory to therapy ​ 50% of patients die within 2 years and only 25% surviving longer than 5 years ​ Cardiac transplantation is lifesaving, dilated cardiomyopathy is the most common diagnosis of patients referred for cardiac transplantation ​ Hypertrophic - hypercontraction ​ Heavy, muscular hyper contracting heart characterized by hyperplasia of left ventricle and interventricular septum ​ Ventricular wall is abnormally thick and diastolic filling is impaired ​ Ejection of blood from the ventricle is forceful but ineffective ​ Ventricular outflow often is blocked by an abnormal anterior motion of the mitral valve leaflet during systole ​ Process is familial in the majority of patients ​ Most frequently presents following ○​ Dysnpea upon exertion (blood backs up in the lungs) ○​ Angina (poor perfusion often without coronary artery disease) ○​ Attacks of syncope (poor CNS perfusion)  ​ Ventricular arrhythmias leading to sudden death may occur ​ Hypertrophic cardiomyopathy is one of the most common causes of unexplained deaths in young athletes ​ Restrictive - decrease in flexibility (thick) ​ Decrease in ventricular compliance (flex), resulting in impaired ventricular filling during diastole ​ Least common type ​ Occurs secondary to infiltration of the muscle of the myocardium by other tissue that restricts ventricular compliance and filling ​ Ventricle is stiff and inelastic, can be filled only with great effort ​ Affected patients present with fatigue, exertional dyspnea, and chest pain ​ If infiltrative process approaches the conduction system, arrhythmias develop ​ With time, myocardial contractility usually declines, leading to congestive heart failure ○​ Myocarditis (inflammatory) ​ Inflammatory process usually resulting in injury of the heart muscle (myocardium) ​ May be caused by viruses, bacteria or parasites ​ Most arise from systemic enterovirus (Coxsackie) virus infection (HFM disease) ​ Clinical manifestations vary from asymptomatic to severe congestive heart failure ​ Arrhythmias can develop and account for most examples of sudden cardiac death in patients with primary myocarditis ​ Most cases are self limited, though some patients progress to chronic congestive heart failure months to years after the initial infection ​ ​ Pericardial disease ○​ Pericarditis: inflammation of the pericardium ​ Usually secondary to myocardial or endocardial disease (MI, cardiac surgery, mediastinal radiation) or lesions in other sites extending to the pericardium ​ Primary pericarditis is uncommon, but when it occurs, it is usually of viral origin ​ May cause immediate hemodynamic complications if significant fluid collects in the pericardial space (pericardial effusion) ​ May cases resolve without sequelae, while others progress to a chronic fibrosing process ​ Most serious consequence is development of adhesions which vary from delicate fibrous bands (attaching the heart to the pericardial sac) to complete encasement of the heart by dense fibrous connective tissue (constrictive pericarditis)  ​ So heart cannot contract ​ Results in great strain upon cardiac function ○​ Pericardial effusions: fluid accumulation of the pericardial sac ​ Adds compressive stress on the heart, making it harder to contract ​ Nature of the effusion varies with the cause ​ Major types ​ Serous (most often with CHF or hypoalbuminemia) ○​ Leakage of fluid components ​ Serosanguineous (blunt chest trauma, malignancy) ​ Chylous (mediastinal lymphatic obstruction) ○​ Creation of inflammatory products in lymph ​ Pericardial effusions usually are asymptomatic with large volumes of fluid being accommodate in chronic cases ​ Rapidly developing effusions may cause cardiac tamponade ​ Compression of the heart by accumulation of fluid in pericardial sac, often leading to heart failure ​ When pure blood fills the pericardial space, it is termed hemopericardium ​ Often from penetrating cardiac trauma, ruptured aneurysm or myocardial infarction ​ Typically not classified as a type of pericardial effusion ​ In such cases, escaping blood rapidly fills the pericardial space and leads to cardiac tamponade and death ​ ​ Cardiac tumors: Primary ○​ True primary neoplasms of the heart are extremely rare ​ Most are myxomas or rhabdomyomas (benign) ​ NOT true primary neoplasm, tumor of cardiac muscle ​ ​ Cardiac tumors: Secondary ○​ Secondary tumors are more common, but still rare (5% of patients with disseminated cancer) ​ These reach the pericardium through direct extension or metastatic spread ​ The most frequent tumors which directly invade the lymphomas bronchogenic carcinoma and carcinoma of the esophagus ​ Most common metastatic cancers are carcinomas of the lung or breast, melanoma or hematopoietic malignancies (lymphoma, leukemia)