Pathology

• compression of teh heart due to rapid accumulation fo fluid in ther pericardial sac
• Prevents chamber from expanding fully; limits ability of heart to pump blood

• inflammation of the pericardial sac lining due to viral (fibrinous), bactrial (purulent), or blood (hemorrhagic causes)
• Pain tends to remain substernal with some referred; worsens upon laying down

• Not enough salt in the ECM
• Hypotonic contraction: ECF volumer decreases, cell swells; decreased total Na in body
• Happen when you lose Na in excess of water; decreased Na intake; inappropriate Na loss
• Treat with IV saline

• Hypotonic expansion: slight increase in ECF, cell swells; normal total Na in body
• Happens when you gain water in excess of salt; psychogenic polydipsia; SIADH; ecstasy
• Treatment is to restrict water intake

• Hypotonic expansion: increase in ECF, cell swells; increased total Na in body
• Happens when TBW increases disproportionately to total Na; renal and heart failure
• Treatment is to restrict water and sodium intake

• Too much salt in the ECM
• Hypertonic contraction: ECF volume decreases and cell shrinks
• Happens when you lose water in excess of sodium; diarrhea; vomiting; diuretics; decreased thirst
• Treat by stabilizing hemodynamics, then replacing water deficit

• Too much salt in the ECM
• Hypertonic contraction: ECF volume decreases and cell shrinks
• Happens when water is lost from ICF and ISF but not vascular; diabetes insipidus
• Treat with hypotonic fluids to replace water deficit

• Too much salt in the ECM
• Hypertonic expansion: ECF volume increases, cell shrinks
• Happens when you gain Na in excess of water; IV-hypertonic saline, excess aldoesterone
• Treat with diuretics and IV-dextrose

• Not an ASD because no septal tissue is missing
• Interatrial shunting cannot occur as long as LA pressure exceeds RA pressure
• An elevation in RA pressure can cause R to L shunting

• Most common heart defect
• Failure of ventricular septation (mostly in membranous portion of septum-most small defects in muscular portions spontaneously close); L to R shunting
• Late cyanosis with shunt reversal

• Failure of atrial septation; most often of secundum type involving fossa ovalis
• may be undetected until adulthood; usually unassociated with other anomalies (septum primum type may be associated with mitral and tricuspid valve anomalies)
• Allows L to R shunting because pressure is higher in the left side; cyanosis results if it switches to R to L
• SOB, easily fatigued, poor growth

• Failure to close after birth; normally closes 10-15 hours after birth and anatomically 2-3 days
• Machine like murmur
• Cyanosis in toes, but not fingers (L-R shunt because of pressure))
• Indomethacin may induce closure

• Most common cyanotic CHD
• VSD, pulm stenosis, overriding aorta (dextraposition of aorta), hypertrophy of RV
• Cyanotic
• Fail to thrive

• Valves are abnormally small, ductus arteriosus remains open
• Asymptomatic unless exertion
• RV hypertrophy

• Constriction of teh aorta, usually just above or below ductus arteriosus
• Hypertension in upper extremities, hypotension in lower extremities; anastomoses develop between subclavian artery and aorta
• Notching of ribs due to dilated intercostal arteries

• Cusps of aortic valve fuse together
• LV hypertrophy

• failure of spiraling of conotruncal ridges (week 6)
• RV pumps to aorta; LV pumps to pulm trunl; 2 systems working in parallel with no connections
• To be compatible with life, must have persistent foramen ovale, VSD, or patent ductus arteriosus
• Cyanosis

• Failure of conotruncal ridges to fuse with one another
• Outflow of entire heart through single vessel
• Cyanosis
• CHF

• Hypercalcemia (shifts Na-activation curve to the right; increases threshold; requires larger depol)
• Hyperkalemia (decreases availability of resting Na-channels)

• Channelopathy
• Decreased ability of SR to control Ca

• Channelopathy
• Reduced inward Na-current

• Channelopathy
• Increased IKR, IKS, and IK1

• Channelopathy
• Problems with Na-K channels

• Channelopathy
• Increased IKR, IKS, IK1
• Impaired gap junctions

• Channelopathy
• Decreased If (Phase 4-time between APs)

• Pulmonary hypertension
• Pulmonary stenosis

• Aortic stenosis
• Chronic systemic hypertension

• Colonization or invasion of the heart valves or mural endocardium by a microbe
• Strep viridians is most common in previously damaged or abnormal heart valves
• S Aureus infects normal or abnormal valves (10-20%)
• Enterococci and HACEK (Haemophilus, Actinobacillus, cardiobacterium, Eikenella, and Kingella) are all common causes
• Prosthetic valve endocarditis is commonly caused by caogulase-negative staphylococci

• formation of vegetations composed to thrombotic debris and organisms
• Acute is typically infection of previously normal heart valve by highly virulent, necrotizing, ulcerative, destructive legions causing organism

• Marantic endocarditis\
• Precipitation of small amount of fibrin and other blood components on valve leafletss (small and sterile)
• Majority of patients have other debilitating diseases
• Vegetations can be colonized by microorganisms leading to IE

• Mitral and tricuspid valvulitis with small, sterile vegetations
• Occasionally encountered with SLE
• Lesions are small single or multiple, sterile, pink vegetations taht often have a warty appearance
• Can be located on underside of AV valves, valvular endocardium, chords, ro mural endocardium of atria and ventricles

• Aortic stenosis: calcification of anatomically normal and congenitally bicuspid aortic valves
• Aortic insufficiency: dilation of the ascending aorta, suually related to hypertension and aging
• Mitral stenosis: rheumatic heart disease
• Mitral insufficiency: myxomatous degeneration

• Acute rheumatic fever is an inflamm disease that follows pharyngeal infection with group A strep (normall children); AI response to strep Ags (Strep0 and DNAase B) results in cross reaction to tissue Ags
• Valve leaflets become deformed by chronic inflammation, fibrosis, and vascular proliferation
• Particularly mitral stenosis; RHD is virtually the only cause of this

• based upon preceding group A strep infection, plus 2 of the following:
• Major (migratory polyarthritis, pancarditis, subcutaneous nodules, erythema marginatum, Sydenham's chorea)
• Minor (fever, arthralgi, increase in acute phase reactants)

• Decrescendo; louder on expiration
• Murmur=diastolic
• Increased aortic pulse pressure
• LV hypertrophy, greater SV

• crescendo-decrescendo; louder on expiration
• murmur=systolic
• decreased aortic pulse pressure; peak LV systolic pressure greater than aortic systolic pressure; paradoxial splitting of S2
• LV hypertrophy

• holosytolic; louder on expiration
• Murmur=systolic
• LV hypertrophy

• Decrescendo-crescendo; louder on expiration
• Murmur=diastolic
• increased LA presure; increased pulmonary venous pressure
• Pulmonary edema; reduced O2 levels in lungs; pulmonary arteriolar constriction; RV hypertrophy

• Decrescendo; louder on inspiration
• diastolic
• increased pulse pressure in pulmonary artery
• RV hypertrophy; greater SV

• Crescendo-decrescendo; louder on inspiration
• Systolic
• decreased pulse pressure in pulmonary artery; peak RV systolic pressure is greater than systolic pressure in pulmonary artery; wide splitting of S2
• RV hypertrophy

• Holosystolic; louder on inspiration
• Systolic
• RV hypertrophy

• decrescendo-crescendo; louder on inspiration
• diastolic
• increased RA pressure; increased systemic vein pressure; increased cap pressure
• Systemic organ edema; reduced O2 levels in systemic organs

Renal disease-->nephron damage-->impaired excretion of sodium and water-->increased blood volume-->increased venous pressure and return-->increased preload-->increased SV-->increased CO-->increased MAP

Atherosclerotic plaques in renal arteries-->impaired renal blood flow-->increased renin secretion-->Ang II leads to vasoconstriction-->increased TPR-->increased MAP

Also, Ang II-->aldosterone-->increased renal sodium reabsorption-->increased blood volume-->increased VR-->increased CO-->increased MAP

• Catecholamine secreting tumor-->increased NE and epi-->arteriolar constriction-->increased TPR-->increased MAP
• EPI and NE also-->venous constriction-->increased VR-->increased SV-->increased CO-->increased MAP
• Epi and NE also-->increase HR-->increase CO-->increase MAP

endothelial cell damage-->decreased NO-->increased vasoconstriction-->increased MAP

Also ET-1-->increased and sustained vasoconstriction-->increased MAP

• Vasculitis
• Acute necrotizing vasculitis of medium-sized muscular arteries
• Fibrinoid necrosis, mixed inflamm cells, thrombosis, infarction of organ
• Presence of P-ANCA
• RX: steroids, anti-inflamms, or immunosuppressives

• Vasculitis
• group of inflammatory lesions affecting small vessels that are thought to be in response to foreign substances
• Localized (w/in skin--includes leukocytoclastic vasculitis, cutaneous vasculitis, and palpable purpura--fibrinoid necrosis, acute inflammation, and extravasation of RBCs) or generalized (systemic hypersensitivity polyarteritis--can be associated with SLE, RA, SS, HSP, malignancy, etc.; arterioles, small arteries; P and C-ANCA, RPGN, and renal failure)

• systemic vasculitis with prominent eosinophilia
• Young people with asthma or allergies
• Necrotizing vasculitis of small and medium sized vessels--granulomas, eosinophilia, and fibrinoid necrosis
• C-ANCA and P-ANCA

• Focal, chronic, granulomatous inflammation fo teh temporal artery
• Most common form of vasculitis
• Cordlike nodule vessels with narrow lumen, granulomatous inflammation with lymphs, plasma cells, macs, eosinophils, necrosis, fragmentation of elastic lamina, and fibrosis of the media and thick intima
• Clinically, benign, self-limiting, muscle aches, rheumatica, visual symptoms, blindness
• Treat with steroids

• Vasculitis
• Systemic necrotizing granulomatous vasculitis of unknown etiology
• involes lungs, kindeys, and upper resp tract
• Parenchymal necrosis, granulomatous inflammation, small arteries and capillaries
• Clinically, hematuria, proteinuria, sinusitis, pneumonitis, pulmonary infiltrate, and C-ANCA

• Vasculitis
• Inflammation of unknown etiology--affects large arteries (aorta and branches)
• Pulseless disease
• Constitutional symptoms, high asymmetrical BP, cardiac symptoms, CHF, syncope, dizziness
• Treat with steroids or surgical reconstruction

• Vasculitis
• acute necrotizing vasculitis of infancy and early childhood
• Fever, rash, conjunctival and oral lesions; lymphadenitis
• Affects coronary arteries; can lead to aneurysms and death
• May be associated with parvovirus B19

• Vasculitis
• Occlusive inflamm disease of medium and small arteries in distal arms and legs
• Cell-mediated immunity, collagen types II and III, acute inflammation, thrombosis, infarcts, gangrene, amputations
• Intermittent caludication, painful ulcers, gangrene

• Risk: age, gender, hyperlipidemia, hypertension, diabetes, CRP (same as for ischemic heart disease)
• Endothelial injury-->adhesion of platelets-->release PDGF-->smooth muscle migrationa nd proliferation
• Foam cells come from macs and smooth muscle cells-->HDL tries to help clear cholesterol from these accumulations
• Smooth muscle cells migrate to intima, proliferate, produce ECM including collagen and proteoglycans
• Atheroma formation leads to aneurysm and rupture, occlusion by thrombosis (dissolution, propagation, organization, canalization, or embolization), or critical stenosis
• Treat with throbolytic drugs, angioplasty or stents, bypass grafts; timing of intervention is essential (<12 hours)