1、HEART,THE HEART,Normal Pathology Heart Failure: L, R Heart Disease Congenital: LR shunts, RL shunts, Obstrustive Ischemic: Angina, Infarction, Chronic Ischemia, Sudden Death Hypertensive: Left sided, Right sided Valvular: AS, MVP, Rheumatic, Infective, Non-Infective, Carcinoid, Artificial Valves Car
2、diomyopathy: Dilated, Hypertrophic, Restrictive, Myocarditis, Other Pericardium: Effusions, Pericarditis Tumors: Primary, Effects of Other Primaries Transplants,NORMAL Features,6000 L/day 250-300 grams 40% of all deaths (2x cancer) Wall thickness pressure (i.e., a wall is only as thick as it has to
3、be) LV=1.5 cm RV= 0.5 cm Atria =.2 cm Systole/Diastole Starlings Law,TERMS,CARDIOMEGALY DILATATION, any chamber, or all HYPERTROPHY, and chamber, or all,STRIATIONSNUCLEUSDISCSSARCOLEMMASARC. RETIC.MITOCHONDRIAENDOTHELIUMFIBROBLASTSGLYCOGENA.N.P.,S.A. NodeAV NodeBundle of HIS L. Bundle, R. Bundle,Ant
4、erior Lateral Posterior Septal,VALVES,AV: TRICUSPID MITRAL SEMILUNAR: PULMONIC AORTIC,CARDIAC AGING,CARDIAC AGING,BROWN ATROPHY, HEART,LIPOFUCSIN,Pathologic Pump Possibilities,Primary myocardial failure (MYOPATHY) Obstruction to flow (VALVE) Regurgitant flow (VALVE) Conduction disorders (CONDUCTION
5、SYSTEM) Failure to contain blood (WALL INTEGRITY),CHF,DEFINITION TRIAD 1) TACHYCARDIA 2) DYSPNEA 3) EDEMA FAILURE of Frank Starling mechanism HUMORAL FACTORS Catecholamines (nor-epinephrine) ReninAngiotensionAldosterone Atrial Natriuretic Polypeptide (ANP) HYPERTROPHY and DILATATION,HYPERTROPHY,PRES
6、SURE OVERLOAD (CONCENTRIC) VOLUME OVERLOAD (CHF)LVH, RVH, atrial, etc.2X normal weight ischemia 3X normal weight HTN 3X normal weightMYOPATHY, aortic regurgitation,CHF: Autopsy Findings,Cardiomegaly Chamber Dilatation Hypertrophy of myocardial fibers, BOXCAR nuclei,Left Sided Failure,Low output vs.
7、congestion Lungs pulmonary congestion and edema heart failure cells Kidneys pre-renal azotemia salt and fluid retention renin-aldosterone activation natriuretic peptides Brain: Irritability, decreased attention, stuporcoma,Left Heart Failure Symptoms,Dyspnea on exertion at rest Orthopnea redistribut
8、ion of peripheral edema fluid graded by number of pillows needed Paroxysmal Nocturnal Dyspnea (PND),LEFT Heart Failure,Dyspnea Orthopnea PND (Paroxysmal Nocturnal Dyspnea) Blood tinged sputum Cyanosis Elevated pulmonary “WEDGE” pressure (PCWP),Right Sided Heart Failure,Etiology left heart failure co
9、r pulmonale Symptoms and signs Liver and spleen passive congestion (nutmeg liver) congestive spleenomegaly ascites Kidneys Pleura/Pericardium pleural and pericardial effusions transudates Peripheral tissues,RIGHT Heart Failure,FATIGUE “Dependent” edema JVD Hepatomegaly (congestion) ASCITES, PLEURAL
10、EFFUSION GI Cyanosis Increased peripheral venous pressure (CVP),HEART DISEASE,CONGENITAL (CHD) ISCHEMIC (IHD) HYPERTENSIVE (HHD) VALVULAR (VHD) MYOPATHIC (MHD),CONGENITAL HEART DEFECTS,Faulty embryogenesis (week 3-8) Usually MONO-morphic (i.e., SINGLE lesion) (ASD, VSD, hypo-RV, hypo-LV) May not be
11、evident until adult life (Coarctation, ASD) Overall incidence 1% of USA births INCREASED simple early detection via non invasive methods, e.g., US, MRI, CT, etc.,GENETICS,Gene abnormalities in only 10% of CHD Trisomies 21, 13, 15, 18, XO Mutations of genes which encode for transcription factorsTBX5A
12、SD,VSD NKX2.5ASD Region of chromosome 22 important in heart development, 22q11.2 deletionconotruncus, branchial arch, face,ENVIRONMENT,RUBELLA TERATOGENS,CHD,LR SHUNTS: all “Ds” in their names NO cyanosis Pulmonary hypertension SIGNIFICANT pulmonary hypertension is IRREVERSIBLE RL SHUNTS: all “Ts” i
13、n their names CYANOSIS (i,.e., “blue” babies) VENOUS EMBOLI become SYSTEMIC OBSTRUCTIONS,LR,ASD VSD ASVD PDA,NON CYANOTICIRREVERSIBLE PULMONARY HYPERTENSION IS THE MOST FEARED CONSEQUENCE,ASD,NOT patent foramen ovale Usually asymptomatic until adulthood SECUNDUM (90%): Defective fossa ovalis PRIMUM
14、(5%): Next to AV valves, mitral cleft SINUS VENOSUS (5%): Next to SVC with anomalous pulmonary veins draining to SVC or RA,VSD,By far, most common CHD defect Only 30% are isolated Often with TETRALOGY of FALLOT 90% involve the membranous septum If muscular septum is involved, likely to have multiple
15、 holes SMALL ones often close spontaneously LARGE ones progress to pulmonary hypertension,PDA,90% isolated HARSH, machinery-like murmur LR, possibly RL as pulmonary hypertension approaches systemic pressure Closing the defect may be life saving Keeping it open may be life saving (Prostaglandin E). W
16、hy?,AVSD,Associated with defective, inadequate AV valves Can be partial, or COMPLETE (ALL 4 CHAMBERS FREELY COMMUNICATE),RL,Tetralogy of Fallot Transposition of great arteries Truncus arteriosus Total anomalous pulmonary venous connection Tricuspid atresia,RL SHUNTS,TETRALOGY of FALLOT most COMMON 1
17、) VSD, large 2) OBSTRUCTION to RV flow 3) Aorta OVERRIDES the VSD 4) RVH SURVIVAL DEPENDS on SEVERITY of SUBPULMONIC STENOSIS Can be a “PINK” tetrology if pulmonic obstruction is small, but the greater the obstruction, the greater is the RL shunt,TGA (TRANSPOSITION of GREAT ARTERIES),NEEDS a SHUNT f
18、or survival PDA or PFO (65%), “unstable” shunt VSD (35%), “stable” shunt RVLV in thickness Fatal in first few months Surgical “switching”,TRUNCUS ARTERIOSIS,TRICUSPID ATRESIA,Hypoplastic RV Needs a shunt, ASD, VSD, or PDA High mortality,Total Anomalous Pulmonary Venous Connection (TAPVC),PULMONARY V
19、EINS do NOT go into LA, but into L. innominate v. or coronary sinus Needs a PFO or a VSD HYPOPLASTIC LA,OBSTRUCTIVE CHD,COARCTATION of aorta Pulmonary stenosis/atresia Aortic stenosis/atresia,COARCTATION of AORTA,MF But XOs frequently have it INFANTILE FORM (proximal to PDA) (SERIOUS) ADULT FORM (CL
20、OSED DUCTUS) Bicuspid aortic valve 50% of the time,PULMONIC STENOSIS/ATRESIA,If 100% atretic, hypoplastic RV with ASD Clinical severity stenosis severity,AORTIC STENOSIS/ATRESIA,VALVULAR If severe, hypoplastic LVfatal SUB-valvular (subaortic) Aortic wall THICK BELOW cusps SUPRA-valvular Aortic wall
21、THICK ABOVE cusps in ascending aorta,HEART DISEASE,CONGENITAL (CHD) ISCHEMIC (IHD) HYPERTENSIVE (HHD) VALVULAR (VHD) MYOPATHIC (MHD),SYNDROMES of IHD,Angina Pectoris: Stable, Unstable Myocardial Infarction (MI, AMI) Chronic IHD CHF (CIHD) Sudden Cardiac Death (SCD)“Acute” Coronary Syndromes: UNSTABL
22、E ANGINA AMI SCD (Sudden Cardiac Death),IHD RISK,Number of plaques Distribution of plaques Size, structure of plaques,ACUTE CORONARY SYNDROMES,“The acute coronary syndromes are frequently initiated by an unpredictable and abrupt conversion of a stable atherosclerotic plaque to an unstable and potent
23、ially life-threatening atherothrombotic lesion through superficial erosion, ulceration, fissuring, rupture, or deep hemorrhage, usually with superimposed thrombosis.”,EPIDEMIOLOGY, million die of IHD yearly in USA 1 million in 1963. Why? Prevention of control controllable risk factors Earlier, bette
24、r diagnostic methods PTCA, CABG, arrythmia control90% of IHD patients have ATHEROSCLEROSIS (no surprise here),ACUTE CORONARY SYNDROME FACTORS,ACUTE PLAQUE CHANGE * Inflammation Thrombus Vasoconstriction,* MOST IMPORTANT,ACUTE PLAQUE CHANGE,Rupture/Refissuring Erosion/Ulceration, exposing ECM Acute H
25、emorrhage,NB: Plaques do NOT have to be severely stenotic to cause acute changes, i.e., 50% of AMI results from thromboses of plaques showing LESS THAN 50% stenosis,INFLAMMATION,Endothelial cells release CAMs, selectins T-cells release TNF, IL-6, IFN-gamma to stimulate and activate endothelial cells
26、 and macrophages CRP predicts the probability of damage in angina patients,THROMBUS,Total occlusion Partial Embolization,VASOCONSTRICTION,Circulating adrenergic agonists Platelet release products Endothelially released factors, such as endothelin,ANGINA PECTORIS,Paroxysmal (sudden) Recurrent 15 sec.
27、15 min. Reduced perfusion, but NO infarction THREE TYPES STABLE: relieved by rest or nitro PRINZMETAL: SPASM is main feature, responds to nitro, S-T elevation UNSTABLE (crescendo, PRE-infarction, Q-wave angina): perhaps some thrombosis, perhaps some non transmural necrosis, perhaps some embolization
28、, but DISRUPTION of PLAQUE is universally agreed upon,MYOCARDIAL INFARCTION,Transmural vs. Subendocardial (inner 1/3) DUH! EXACT SAME risk factors as atherosclerosis Most are TRANSMURAL, and MOST are caused by coronary artery occlusion In the 10% of transmural MIs NOT associated with atherosclerosis
29、: Vasospasm Emboli UNexplained,MYOCARDIAL RESPONSE,PROGRESSION OF NECROSIS,TIMING of Gross and Microscopic Findings,1 day, 3-4 days, 7 days, weeks, months,RE-PERFUSION,Thrombolysis PTCA CABGReperfusion CANNOT restore necrotic or dead fibers, only reversibly injured onesREPERFUSION “INJURY” Free radi
30、cals Interleukins,AMI DIAGNOSIS,SYMPTOMS EKG DIAPHORESIS (10% of MIs are “SILENT” with Q-waves) CKMB gold standard enzyme Troponin-I, Troponin-T better CRP predicts risk of AMI in angina patients,COMPLICATIONS,Wall motion abnormalities Arrhythmias Rupture (4-5 days) Pericarditis RV infarction Infarc
31、t extension Mural thrombus Ventricular aneurysm Papillary muscle dysfunction (regurgitation) CHF,CIHD, aka, ischemic “cardiomyopathy”,Progress to CHF often with no pathologic or clinical evidence of localized infarction Extensive atherosclerosis No infarct H&D present,SUDDEN CARDIAC DEATH,350,000 in
32、 USA yearly from atherosclerosis NON-atherosclerotic sudden cardiac death includes: Congenital coronary artery disease Aortic stenosis MVP Myocarditis Cardiomyopathy (sudden death in young athletes) Pulmonary hypertension Conduction defects HTN, hypertrophy of UNKNOWN etiology,AUTOPSY findings in SC
33、D,75% narrowing of 1-3 vessels Healed infarcts 40%“ARRHYTHMIA” is often a very convenient conclusion when no anatomic findings are present, i.e., “wastebasket” diagnosis,HEART DISEASE,CONGENITAL (CHD) ISCHEMIC (IHD) HYPERTENSIVE (HHD) VALVULAR (VHD) MYOPATHIC (MHD),HHD (Left),DEFINITION: Hypertrophi
34、c adaptive response of the heart, which can progress: Myocardial dysfunction Cardiac dilatation CHF Sudden death,NEEDED for DIAGNOSIS:,LVH (LV2.0 and/or Heart500 gm.) HTN (140/90),PREVALENCE:,WHAT % of USA people have hypertension?,PREVALENCE:,WHAT % of USA people have hypertension?Answer: 25%,HISTO
35、PATHOLOGY,INCREASED FIBER (MYOCYTE) THICKNESS INCREASED nuclear size with increased “blockiness” (boxcar nucleus),CLINICAL,EKG,Summary of LVH Criteria 1) R-I + S-III 25 mm 2) S-V1 + R-V5 35 mm 3) ST-Ts in left leads 4) R-L 11 mm 5) LAE + other criteria Positive Criteria: 1=possible 2=probable 3=defi
36、nite,ATRIAL FIBRILLATION Why?* CHF, cardiac dilatation, pulmonary venous congestion and dilatation,COURSE:,NORMAL longevity, death from other causes Progressive IHD Progressive renal damage, hemorrhagic CVA (Which arteries?) CHF,HHD (Right) = COR PULMONALE,ACUTE: Massive PE CHRONIC: COPD, CRPD, Pulm
37、onary artery disease, chest wall motion impairment,Diseases of the Pulmonary Parenchyma Chronic obstructive pulmonary disease Diffuse pulmonary interstitial fibrosis Pneumoconioses Cystic fibrosis BronchiectasisDiseases of the Pulmonary Vessels Recurrent pulmonary thromboembolism Primary pulmonary h
38、ypertension Extensive pulmonary arteritis (e.g., Wegener granulomatosis) Drug-, toxin-, or radiation-induced vascular obstruction Extensive pulmonary tumor microembolism,Disorders Affecting Chest Movement Kyphoscoliosis Marked obesity (pickwickian syndrome) Neuromuscular diseasesDisorders Inducing P
39、ulmonary Arterial Constriction Metabolic acidosis Hypoxemia Chronic altitude sickness Obstruction to major airways Idiopathic alveolar hypoventilation,HEART DISEASE,CONGENITAL (CHD) ISCHEMIC (IHD) HYPERTENSIVE (HHD) VALVULAR (VHD) MYOPATHIC (MHD),ValvularHD,Opening problems: Stenosis Closing problem
40、s: Regurgitation or Incompetence,70% of all VHD,AS Calcification of a deformed valve “Senile” calcific AS Rheum, Heart Dis. MS Rheumatic Heart Disease,AORTIC STENOSIS2X gradient pressureLVH, ischemiaCardiac decompensation, angina, CHF50% die in 5 years if angina present50% die in 2 years if CHF pres
41、ent,MITRAL ANNULAR CALCIFICATION,Calcification of the mitral “skeleton” Usually NO dysfunction Regurgitation or Stenosis possible FM,REGURGITATIONS,AR Rheumatic Infectious Aortic dilatations Syphilis Rheumatoid Arthritis Marfan MR MVP Infectious Fen-Phen Papillary muscles, chordae tendinae Calcifica
42、tion of mitral ring (annulus),Mitral Valve Prolapse (MVP),MYXOMATOUS degeneration of the mitral valve Associated with connective tissue disorders “Floppy” valve 3% incidence, FM Easily seen on echocardiogram,MVP: CLINICAL FEATURES,Usually asymptomatic Mid-systolic “click” Holosystolic murmur if regu
43、rg. present Occasional chest pain, dyspnea 97% NO untoward effects 3% Infective endocarditis, mitral insufficiency, arrythmias, sudden death,RHEUMATIC Heart Disease,Follows a group A strep infection, a few weeks later DECREASE in “developed” countries PANCARDITIS,ACUTE: -Inflammation -Aschoff bodies
44、 -Anitschkow cells -Pancarditis -Vegetations on chordae tendinae at leaflet junction,CHRONIC: THICKENED VALVES COMMISURAL FUSION THICK, SHORT, CHORDAE TENDINAE,CLINICAL FEATURES,Migratory Polyarthritis Myocarditis Subcutaneous nodules Erythema marginatum Sydenham chorea,INFECTIOUS ENDOCARDITIS,Micro
45、bes Usually strep viridans Often Staph aureus in IVD users Enterococci HAEK (normal oral flora) Hemophilus influenzae Actinobacillus Cardiobacterium Eikenella Kingella Fungi, rickettsiae, chlamydia,INFECTIOUS ENDOCARDITIS,Acute: 50% mortality (course=days) SUB-acute: LOW mortality (course=weeks),VEG
46、ETATIONS,INFECTIVE 5mm NON-Infective 5mm,DIAGNOSIS=MMm, Mmmm, mmmmm,MAJOR Positive blood culture(s) indicating characteristic organism or persistence of unusual organism Echocardiographic findings, including valve-related or implant-related mass or abscess, or partial separation of artificial valve
47、New valvular regurgitationminor Predisposing heart lesion or intravenous drug use Fever Vascular lesions, including arterial petechiae, subungual/splinter hemorrhages, emboli, septic infarcts, mycotic aneurysm, intracranial hemorrhage, Janeway lesions Immunologic phenomena, including glomerulonephri
48、tis, Osler nodes, Roth spots, rheumatoid factor Microbiologic evidence, including single culture showing uncharacteristic organism Echocardiographic findings consistent with but not diagnostic of endocarditis, including new valvular regurgitation, pericarditis,NON-infective VEGETATIONS,5 mm PE Trous
49、seau syndrome (migratory thrombophlebitis with malignancies) s/p Swan-Ganz Libman-Saks with SLE (both sides of valve),Carcinoid Syndrome,Episodic skin flushing Cramps Nausea & Vomiting Diarrhea serotonin, 5HIAA in urine FIBROUS INTIMAL THICKENING RV, Tricuspid valve, Pulmonic valve (all RIGHT side) Similar to what Fen-Phen does on the LEFT side,ARTIFICIAL VALVES,Mechanical Xenografts (porcine)60% have complications within 10 years,HEART DISEASE,CONGENITAL (CHD) ISCHEMIC (IHD) HYPERTENSIVE (HHD) VALVULAR (VHD)MYOPATHIC (MHD)PERICARDIAL DISEASE,
