Cardiovascular system V. Žampachová I. PAÚ Cardiovascular diseases nMajor cause of morbidity and mortality (1/3 of deaths) nLeading risk factors: ↑ serum cholesterol level, smoking, hypertension (CV disease itself), obesity/metabolic syndrome, sedentary lifestyle Common clinical signs nGeneral nWeakness nFatigue nWeight change (↑ due to edema) nPoor exercise tolerance nCyanosis Common clinical signs nMusculoskeletal nMuscular fatigue / pain nChest, shoulder, neck, jaw, arm pain / discomfort nPeripheral edema nIntermittent claudication (leg pain / cramps / discomfort) Common clinical signs nDizziness (abnormal blood pressure, cardiac arrythmia) nHeadache (arteritis, hypertension) nLoss of vision (retinopathy, transient ischemic attack) nChest pain (myocardial ischemia/infarction, pulmonary embolism, aortic dissection) nPalpitations (ischemic heart disease, valvular disease) Common clinical signs nCough (left ventricular failure, antihypertensive drugs – ACE inhibitors) nAcute dyspnea (acute left ventricle failure, pulmonary embolism) nChronic dyspnea (congestive cardiac failure, aortic valve disorder, congenital heart disease) nSwollen ankles (congestive cardiac failure, venous insufficiency) n Implication for the therapist nEvaluation of possible cardiac signs nAssessment of possible risks of adverse cardiac event nEvaluation of type/degree of organ impairment, level of disability, functional limitations nIndividual exercise program (mode, duration, intensity, frequency) commonly necessary Implication for the therapist nExercise nPrimary and/or secondary prevention of cardiovascular diseases nIncrease of CV functional capacity, ↓ of myocardial oxygen demand nAdjunctive therapy for lipid management (endurance exercise) Indications for discontinuing/modifying exercise nSymptoms nNew-onset or easily provoked anginal chest pain n↑ episodes, intenstity, duration of angina (unstable angina) nDiscomfort in the upper body nFainting, dizziness nSudden severe dyspnea nSevere fatigue nNausea, vomiting nBack pain during exercise Indications for discontinuing/modyfying exercise nClinical signs nPallor, peripheral cyanosis; cold + moist skin nConfusion nResting heart rate ˃130/min or ≤40/min nArrythmias (irregular heartbeats, palpitation) nBlood pressure (BP) abnormalities: fall in systolic BP during increasing workload; rise of systolic BP ˃250 mm Hg and/or diastolic ˃115 mm Hg nInability to converse during activity nSigns of CNS involvment (confusion, delirium, stroke, …) nRecent myocardial infarction (within 48 hours) nAcute infection or fever ˃37,8°C Morphology npericardial sac – cca 30ml clear yellowish fluid n n heart size – approx. the person‘s closed fist nmale = 300 – 350 g, n - hypertrophy > 400g n n myocardium: n è RV 3 – 4 mm n è LV 12 – 15 mm n n foramen ovale n - closed x opened è paradoxical embolia è srdce6 Heart failure n heart unable to pump blood at a rate sufficient for metabolic demands of the tissues n n systolic dysfunction - ↓ myocardial contractile function (ischemic injury, pressure or volume overload – valvular disease, hypertension, cardiomyopathy) n diastolic dysfunction - inability to dilatate sufficiently (massive LV hypertrophy, myofibrosis, amyloidosis) n n cardial and/or extracardial pathologic changes Cardial changes n ndisproportion between heart function and peripheral vascular resistance n n differencies due to rapidity of development: n – sudden → acute dilatation n n – chronic → adaptation → → → n myocardial hypertrophy ( nutritional demands) +/- ventricular dilatation (enhanced contractility), + activation of neurohumoral systems (norepinephrin, renin-angiotensin system, atrial natriuretic peptide) n if not treated → progression into heart failure n Extracardial changes nvenous congestion (filled vessels)– blood stays ahead of the heart, e.g. liver (→ hepar moschatum) n ninduration (firmer consistency) – decreased oxygen + nutrients → loss of functional cells + fibroproduction (liver, spleen, kidney) n noedema – congestion + outflow of fluid from capillaries visible /palpable in soft tissues n ncyanosis (bluish discoloration) – increased level of deoxygenated hemoglobin visible on acral parts Chronic venous congestion (nutmeg liver - hepar moschatum) n Ischemic heart disease (IHD) ngroup of pathophysiologically related syndromes resulting from myocardial ischemia (hypoxia or anoxia, ↓ nutrients, ↓ removal of metabolites) nimbalance between the demand and supply by coronary arteries. n important factor – coronary AS n n forms: nangina pectoris nmyocardial infarction (MI) nchronic IHD with heart failure nsudden cardiac death Pathogenesis of IHD nAS of coronary aa. ncommonly at a. branching nfixed obstruction by plaque (fibrous, atheromatic) nacute plaque change (rupture, erosion, haemorrhage, thrombosis) n75% stenosis – ischemia during ↑ workload – stable angina pectoris n90% stenosis –ischemia even at rest – ustable angina – preinfarction nnon-atherosclerotic n – coronary emboli – endocarditis, atrial fibrillation, mural thr., paradoxical e. n – coronary vasospasm n – aortic dissection n – coronary vasculitis n – congenital coronary aa. defects n –hematologic disorders, amyloidosis, shock, etc. n n n Angina pectoris (AP) n transient myocardial ischemia → chest pain !!! - n1. stable (typical) n – due to increased workload, duration ≤ 15 min, relieved by rest or nitroglycerin n – no myocardial necrosis n –subendocardial LV myocardium n n2. unstable n – increasing frequency / duration of pain attack, even at rest n – plaque disruption + mural thrombosis, possible vasospasm n – preinfarction angina n n3. variant (Prinzmetal) angina n – mostly unrelated to physical activity, coronary vasospasm - vasodilatative therapy n n n Myocardial infarction n ischaemic coagulative necrosis n n causes: n usually coronary thrombosis n complicated atheromatic plaque n event. embolism n spasm n inflammation n rarely systemic causes. n ngross n evolution; first signs (red, softer) after 12 hrs n 2-3 days established infarction (yellowish, haemorrhagic rim) n weeks – formation of firm white fibrotic scar n n Myocardial infarction + coronary thrombosis Image011 Myocardial infarction IM _myoc-fresh-ihf-322a 1 subendocardial coagulative necrosis 2 hyperemic rim 3 normal myocardium 4 epicardium 2 Myocardial infarction 1 1 2 4 3 Myocardial infarction ntransmural (QIM, STEMI) - + ST elevation on ECG n – ≥ ¾ of wall thickness, breadth >25 mm n – complete coronary artery obstruction n emergency angioplasty/stenting n n non-transmural (subendocardial, Non-STEMI) n – internal ¼ to ½ of LV wall n – collateral blood flow, incomplete obstruction, shorter ischemia MI complications nsudden death (arrythmia) ncardiogenic shock (contractile dysfunction) npericarditis epistenocardiaca n → sero-fibrinous inflammation nmural thrombosis n → embolism into systemic circulation (→ brain, kidney, intestine, spleen infarction) nventricular aneurysm n → acute – risk of rupture, trhrombosis; chronic – LV insufficiency ncardiac rupture n → free wall, septum, : tamponade / acute heart failure npapillary muscle rupture n → valvular incompetence → acute heart failure _heart-infarct+thrombus MI – mural thrombosis _heart-ruptured-wall-inf Mi – rupture _cardiac-tamponade-2-66e _cardiac-tamponade-1-66e 1 lung 2 pericardial sac 3 blood coagulum 4 thoracic wall 3 1 2 1 1 4 4 MI – rupture, tamponade CV143 1 aneurysm w. thrombosis 2 RV 3 LV 4 mitral valve 4 1 2 3 MI – LV aneurysm Chronic ischemic heart disease (IHD) nangina pectoris or MI in anamnesis n nprogressive heart failure due to ischemic myocardial damage è LV failure è congestive RV failure n nheart hypertrophy + dilatattion, myofibrosis and/or post-MI scars n nmultiple coronary arteries with significant AS stenosis n nimminent risk of MI, sudden cardiac death due to arrythmia, heart failure Disperse myofibrosis of the heart nRepeated multiple microinfarcts („unstable angina pectoris“) nRepair by scarring nDisperse scars – small whitish foci in myocardium n Sudden cardiac death n= unexpected death from cardiac causes, without preexisting symptoms or within 1 hr of the onset of symptoms n nmost commonly due to lethal arrythmia (ventricular fibrillation, asystole) n nsudden collapse without signs of acute MI n nother causes: ndissecting/ruptured aortic aneurysm npulmonaty thrombembolism nmassive intracerebral haemorrhage nheritable conditions incl. anatomic, electrical – channelopathies n n n n Myocarditis nmyocardial inflammatory damage without ischemia nrapidly (days) progressive heart insufficiency n ngross: n cardiac dilatation, flabby, mottled myocardium n n etiology: n viruses, ricketsia, chlamydia, bacteria (diphtheria, sepsis), fungi, protozoa (toxoplasmosis), helminths (trichinosis) n immune-mediated (drug hypersesitivity, postviral, rheumatic fever, rejection) npost-tachycardia nionising radiation nunknown (giant-cell myocarditis, …) Cardiomyopathies n= heart disease due to myocardial abnormality, with heart dysfunction ndiagnosis after exclusion of IHD, valvular disease, congenital d. or hypertension nPossible cause of sudden death in younger people n nheterogenous group of disorders: ndilated (DCM) most common n– dilatation + hypertrophy,¯ LV contraction, possible mural thrombosis; 20–50% genetic (AD); alcoholic, peripartum, myocarditis... n nrestrictive cardiomyopathy: diastolic dysfunction, ¯ of compliance - ¯ filling, myocardial stiffness nhypertrophic (HCM) n – massive LV hypertrophy, 100% genetic, diastolic dysfunction n n specific CM n– Duchenne muscle dystrophy, toxic (drugs), endocrine d., metabolic d. (hemochromatosis, amyloidosis, glycogenosis,…) n Arrythmias nDisturbance of heart rate and/or rhytm nPathologic changes in cardiac conductive system nVentricular or atrial nTachycardia (↑ heart rate) or bradycardia (↓ heart rate) nDifferent patterns (ECG) nDifferent clinical significance – benign respiratory sinus arrythmia x ventricular fibrillation (fatal without resuscitation) Valvular heart disease ncongenital defects nendocarditis (rheumatic – immune-mediated, infective, thrombotic non-infective, in SLE) ndegenerative changes (mucoid, calcification, fibrosis in ischemic heart disease) ndilatation of the ventricles (relative incompetence) n Infective endocarditis n ncommonly by highly virulent microorganisms nStrep. pyogenenes, Strep. pneumoniae, Staph. aureus, … ev. fungi nsubacute IE – less virulent microorganisms nviridans streptococci npredisposition: ndeformed valve, bioprosthesis, stomatologic, surgical procedures, postcatethrization, i.v. drug addicts ntooth brushing, chewing (oral flora common source) in immunodeficient patient n nbacteremia - endocardial damage by bacteria - trombosis = infective vegetation n n _heart-endocarditis-bacterial-valve-destruction 1 vegetation 2 endocardium 3 papillary muscle 4 myocardium Infective endocarditis – valve destruction 1 2 3 4 Rheumatic fever, rheumatic heart disease nacute non-purulent, immune-mediated systemic poststreptococcal inflammation (cross-reactive antibodies) n nacute stage: PANCARDITIS nacute endokarditis, commonly recurrent n nchronic stage: nvalvular calcification - stenosis + incompetence n n Congenital cardiovascular disease nApprox. in 1% of newborns nUsual cause of heart failure in children nVariable types : nPathological shunts – open communication between spaces which should be closed (septa) nCongenital stenoses nComplex congenital defect – combination of multiple malformations n n Ventricular septal defect Ventricular septal defect 1) n Pericardial effusion n - transudate in congestive heart failure or hypoproteinemia, slow accumulation (up to 500ml – pericardial dilatation) n n n hemopericardium n – wall rupture in MI or aortic root dissection → fatal cardiac tamponade n n n diastolic filling restriction • Pericardial pathology Pericardial pathology n Inflammatory exudate in pericarditis: n n non-infectious n – pericarditis epistenocardiaca (post-MI) uremic, post-operative, n n infectious n – hematogenous, direct spread, lymphogenous; variable agents n n nHealing: may be complicated. Fibrinolysis x organisation by granulation tisssue → adhesions, dystrophic calcification. n n n n Acute fibrinous pericarditis Peric Atherosclerosis nMULTIFACTORIAL COMPLEX DISEASE n– unknown exact cause, combination of chronic inflamation, fibrosis, lipid deposition n Atherosclerosis ndisease of large and medium-sizes arteries with lipid deposition into intima nactive inflammatory process n nendogenous risk factors, mostly noninfluenceables: nage, MxF (estrogen), familiar factors (f. hypercholesterolemia), hereditary homocysteinemia n nexogenous risk factors: nhyperlipidemia (LDL) ←← hypothyreoidism, nephrotic sy; nhypertension, diabetes mellitus, life style smoking (nicotine, CO), sedentary life, food + obesity; ↑CRP n Atherosclerosis nEndothelial injury n - mechanic (↑BP, turbulence) n - endotoxins, immune complexes, exogennous toxins (cig. smoke), ↑ cholesterol n nLipoprotein insudation (LDL) – oxidation in intima n nInflammation n - blood monocytes (→foam cells), T-cells, platelets, smooth muscle cells n nRepair - proliferation of myointimal cells n - synthesis of collagen, elastin, proteoglycans → fibrotic plaque, + lipid accumulation - atheromatous plaque n n stable plaque under repeated inflammation turns into unstable plaque – fibrous cap + endothelium rupture - thrombus n 3 - patogeneze aterosklerózy.emf 3 - patogeneze aterosklerózy.emf 3 - patogeneze aterosklerózy.emf 3 - patogeneze aterosklerózy.emf 3 - patogeneze aterosklerózy.emf Atherosclerosis - pathogenesis Atherosclerosis stages/changes nfatty streak - reversible nfibrotic plaque - irreversible natheromatous plaque - irreversible ncomplicated atheromatous plaque (ulceration, calcification, thrombosis) Atherosclerosis – fatty streak CV016 _aorta-fatty-streaks-1-330c _aorta-heavy-athero-330d Atherosclerosis – plaque ulceration, mural thrombosis Atherosclerosis nSEQUELS: arterial occlusion in situ n chronic (→ hypoxia, atrophy) n acute (→ ischemia, infarction, encephalomalatia) n embolism (thrombus, plaque material) n weakening of arterial wall (aneurysm), risk of rupture n bleeding (from plaque, fissured wall) n calcification (hypertensive factor) n n C:\Documents and Settings\Patol\Dokumenty\Haškovcová\patologie myokardu\c-1.jpg 1 abdominální aorta 2 trombóza a. mesenterica 3 truncus coeliacus 1 Atherosclerosis – complications thrombosis/thrombembolia 2 3 GI031 4 trombóza koronární a. 4 C:\Documents and Settings\Patol\Dokumenty\Haškovcová\patologie myokardu\d-1.jpg Aneurysm n localized, blood-filled balloon-like bulge in the wall of a blood vessel. n the circle of Willis in the brain, thoracic and abdominal aortic aneurysm n n atherosclerotic aneurysm x syphilitic n n etiology: n hereditary defects in the structure, atherosclerosis, inflammation, disease process, accidents … n n false aneurysm n serpentine aneurysm, arteriovenous aneurysm n n n _aorta-abd-aneurysm-330e _aneurysm-circ-willis-331f 3 a. cerebri anterior 4 a. cerebri media 5 a. cerebri posterior 6 a. basilaris 7 aneurysm 1 abdominal aorta 2 aneurysm Atherosclerosis – complications– aneurysm 1 2 3 4 5 6 7 ntear in aortic intima - intramural bleeding through media, false lumen, possible „double-barreled“ aorta ntypical in ascending aorta, 1–8 cm above aortic valve nante– and retrograde spread to the aortic root ncommon thrombosis in false lumen nrisk of external rupture (→ hemopericardium), progression at the aortic branches (→ variable organ ischemia), heart failure n npredisposition – hypertension, Marfan sy, cystic medial necrosis, … Aortic dissection Dissecting aneurysm Image036 Serpentine aneurysm - a. lienalis Image039 Arteriosclerosis nin muscular arteries (middle sized) nsmooth muscle hypertrophy nintimal fibrosis ncollagenisation of elastic membrane nhyalinisation n nage and/or hypertension related changes n→ nephrosclerosis (→ shrinkage of kidneys, decreased function), cerebral ischemia, … n Renal arteriosclerosis - nephrosclerosis Image027 Renal arteriosclerosis Image028 Systemic hypertension nIncrease in total peripheral vascular resistance n nPrimary (essential) hypertension (heritable basis, acquired risk factors – sympathetic overactivity incl. stress, high salt intake, …) n nSecondary hypertension (renal, endocrine hyperfunction, aortic coarctation, drug induced) Systemic hypertension nBenign hypertension – gradual (years – decades) progression of organ damage n nMalignant (accelerated) hypertension – severe, often acute damage nRenal (→ renal insufficiency) nHeart (→ cardiac failure) nBrain (→ stroke, usually brain haemorrhage) nRetina (→ blurred vision, blindness) Systemic hypertension and heart n90–95% essential , major risk factor for AS, ischemic heart disease nAdequate control (life style changes, medication) necessary n nwork overload → LV adaptation to peripheral resistance = cor hypertonicum (concentric LV hypertrophy) → limited compensatory mechanisms → cor hypertonicum decompensatum (dilatation of hypertrophic LV) n n→ heart insufficiency (+relative coronary incompetence) Cor hypertonicum Image031 Cor hypertonicum Image032 LV hypertrophy LV hypertrophy Cor hypertonicum - evolution hypertrofie myokardu LKS Orthostatic hypotension nPostural hypotension – drop in systolic (20 mm Hg) or systolic + diastolic (10 mm Hg) blood pressure with concomitant pulse increase (15 beats/min) on standing from a supine or sitting position nAcute or chronic nCommon in older adults nSyncope, fall, organ ischemia (MI, brain transient ischemic attack) nAutonomic nervous dysfunction nVariable other causes (blood volume depletion, prolonged imobility, malnutrition, alcoholism, antihypertensive drugs) Vasculitis nVessel wall inflammation n nClassification according cause: infectious x non-infectious (commonly immune-mediated, antibodies in the blood ANCA+/ANCA-) n nAffected organs : all organs with vessels nType (size) of vessel involved: Large-vessel n Medium-vessel n Small-vessel nORL: - repeated respiratory tract inflammation n - exudate rich in plasma cells + eosinophils n nKidney: - glomerulonephritis n nLung: - variable presentation of lung diseases + hemoptysis n nSkin: - ulceration, necrosis, petechiae-purpura n nGIT: - ischemic ulcerations (sharply demarcated, without HP, minimal inflammation) n nChronic debilitating disease – clinical signs of tumor!! n Possible clinical signs of systemic vasculitis • fever, nausea, myalgia, arthralgia • skin purpura • signs of nephritis • abdominal pain n n n ngeneral malaise (~ severe influenza, long duration, resistant to usual therapy) n sinusoid course (relapse --- remission --- relapse--) n Patient presentation Thrombosis nMain cause of local blood flow disorders n nintravital intravascular pathological blood coagulation n ntrombocytic agregation, fibrinogen transformation → fibrin, thrombus formation Thrombosis nEndothelial injury most important (trauma, AS, microorganisms, toxins, inflammation) – coagulation factors activation nStagnation: turbulent non-laminar blood flow, adhesion, common in veins nCoagulation disorders: ↑ coagulability or ↓ fibrinolysis na) inborn defects: F V (Leiden) genetic mutation, … b) acquired: oral contraceptives, disseminated tumors, DIC n Thrombosis Image046 Thrombosis nX haematoma – intravital extravascular blood clot nX cruor – postmortal intravascular blood clot n n nGross: nmural (usually heart, arteries) nobturating (veins) Venous thrombosis Image048 Image047 Fate of thrombi Thrombosis nFate of thrombi nPropagation – growth in the direction of blood flow nOrganisation – fixation to the vessel wall, reparation through fibroproductive inflammation, retraction, recanalisation ndissolution - resolution nembolism Thrombus organisation nreactive changes in the thrombus, growth of young immature fibrotic (granulation tissue), later collagen production n nThrombus retraction, resorption by granulation tissue, recanalisation, surface covered by endothelium Disseminated intravascular coagulation nDIC nAcquired coagulopathy, 40% mortality nWidespread endothelial damage or release of tissue thromboplastin (part of cell membranes) into circulation in: nmajor tissue trauma nobstetric complication: protracted labor, placental abruption (amniotic fluid embolism) ninfection (menigococcal sepsis) nneoplasms, liver disease, etc. n n n n n DIC n1. phase: diffuse activation of coagulation in microcirculation (brain, lungs, liver, kidney, heart) – ischaemia, organ failure n n2. phase: coagulation factors consumption, activation of fibrinolysis → hemorrhagic diathesis n hemolytic anemia Deep vein thrombosis (DVT) and pulmonary embolism (PE) nOcclusion of a vein by a thrombus with secondary inflammatory reaction in the wall of the vein (thrombophlebitis) nRisk of thrombus detachment, lung thrombembolism nVenous thrombembolism – VTE, anticoagulation therapy necessary nSignificant health problem nDue to: nImmobility (venous stasis) nTrauma (venous damage) nLifestyle – smoking, DM, obesity, hormonal status (oral contraceptives, …) nHypercoagulation incl. genetic factors n Varicose veins nAbnormal dilatation of veins + valve inkompetence + risk of superfitial thrombosis nWomen ˃ men nUsually lower extremities nInherited trait + high venous pressure (prolonged standing, sitting; hormonal changes, obesity, heart failure) nPreventive measures + exercise Cardiovascular tumors nCardiac tumors: rare, mostly benign nHemangioma: benign vascular (endothelial) tumor, any localisation possible, common on skin, mucosa nRed-blue focus nSize mm – 15 cm nHemangiosarcoma: malignant vascular tumor, any localisation possible, rare, very aggressive, fatal nred to purple patches n raised plaques n nodules n n Kaposi sarcoma special type of angiosarcoma, in immunodeficiency (HIV) - 11 - Kaposi makro Image028 Kaposi sarcoma