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34 Cards in this Set
- Front
- Back
What are the 2 main causes of congenital heart disease?
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Genetics - familial, chromosomal abnormalities, Familial ASD (chromo 5)
Environment - Congenital rubella infection |
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Left to Righ Shunt
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Cyanosis develops late
ASD, VSD, PDA May cause pulmonary hypertension (if hypertension is permanent, may prevent surgury to repair) |
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Right to Left shunt
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Cyanotic congenital heart disease
Tetralogy of Fallot - transposition of the great arteries. Persistent truncous arteriousus, tricuspid atresia, total anomalous pulmonary venous connection. (IHOP acronym) Paradoxical Emboli Hypertrophic osteoarthropathy (clubbing of fingers and toes) |
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ASD
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Asymptomatic until adulthood
NOT PATENT FORAMEN OVALE Secundum (90%) - deficient oval fossa near septum Primum (5%) - ASD by the AV valves Sinus (5%) - near entrance to SVC (can have bad connections from right pulmonary veins) L to R shunt - pulmonary congestion, lead to neonatal CHF, Murmur, cause hypertrophy of RA and RV Clinical - Surgery |
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VSD
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most common congenital cardiac anomly, often associated with other defects
Membranous/Infundibular/Swiss cheese septum Size of aortic valve orifice Cause L to R shunt - pulmonary hypertension/RV hypertension, can develop into a R to L hypertension Clinical - asymptomatic lesions are not surgically corrected in infancy. Big lesions are corrected at first birthday |
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PDA
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90% isolated
continuous harsh murmur L to R shunt w/o cyanosis Pulmonary vascular disease will eventually develop Should be closed ASAP |
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AVSD
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Failure of superior and inferior endocardial cushions to fuse
Results in AV septal defects and incomplete formation of tricuspid and mitral valves Common in Down Syndrome |
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Tetralogy of Fallot
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R to L Shunt - Most common form of cyanotic congenital heart disease
Features - VSD, RVH, pulmonary stenosis, Overridng aorta, Clinical seriousness depend primarily on severity of subpulmonary stenosis Heart is enlarged and boot shaped, large VSD, possible pulmonary valve atresia/ need patent ductus to survive Pink Tetralogy (L to R shunt) - mild subpulmonic stenosis Classic Tetralogy (R to L shunt) - cyanosis RV failure RARE - due to decompression Can be surgically repaired - worse outcome w/ pulmonary atresia and dilated bronchial arteries are present |
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Transposition of Great Arteries
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Aorta arises from RV and Pulmonary artery arises from LV
Embyological defect - abnormal development of great vessel septa Incompatible with life unless present with a shunt VSD - 35% - stable PDA - 65% - unstable RV hypertrophies/LV atrophies Prognosis - degree of blood mixing/magnitude of hypoxia/RV ability to maintain circulation |
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Truncus Ateriosis
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failure of the aorta and pulmonary artery to split
blood mixes in big vessel accompainied with VSD gives rise to systemic pulmonary and coronary circulation Cyanosis Possible inoperable pulmonary hypertension |
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Coarcation of the Aorta
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twice as common in men
Females with Turner syndrome Infantile form - PDA Manifests in early life - do not survive neonatal period Cyanosis in lower half of body Adult form - ridgelike infolding just distal DA. Children are assymptomatic... Adults have upper extremity hypertension, weak pulses in lower extremity, notching under the ribs Can have murmur throughout systole, may have thrill, LV hypertrophy - cardiomegaly |
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Pulmonary Stenosis and Atresia
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Obstruction of Pulmonary valve
RV Hypertrophy Pulmonary artery may be dilated If is Atretic - PDA is only connection to lungs Stenosis - smaller hole, bigger cyanosis |
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Aortic Stenosis and Atresia
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Valvular aortic stenosis - hypoplastic left heart syndrome due to obstruction of LV outflow
-Must have PDA to allow blood flw to the aorta and coronary arteries Subaortic Stenosis - can be discrete or tunnel - associated with coarcation of the aorta or PDA aortic wall is thickened Williams syndrome may occur (multiorgan systems effected/ hypercalcemia of infancy) Mutation in elastin gene Systolic Murmur/thrill Hypertrophy of LV (unless severe - which is hypoplasia) well tolerated threat of sudden death with exertion |
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What are the general characteristic of dilated cardiomyopathy?
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hypertrophy - dilation - contractile dysfunction
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What are some causes of dilated cardiomyopathy?
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Myocarditis - coxsackie B virus, and other enteroviruses
Alcohol - alcohol/metabolites are toxic to myocardium/ thiamine deficiency/doxirubicin Pregnancy - can occur peripartum Genetic - mitochondrial genes, dystrophin, beta oxidation genes |
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What is the morphology of dilated cardiomyopathy?
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heart is heavy and flabby
all chambers are dilated mural thrombi are common NO Valvular alterations NO coronary artery obstruction |
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What is the clinical presentation of dilated cardiomyopathy?
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slowly progressive CHF
50% die within 2 years Death due to progressive cardiac failure or arrhythmia Embolism from mural thrombus may occur heart transplant recommended |
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Hypertrophic Cardiomyopathy
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Myocardial hypertrophy
abnormal diastolic filling LV outflow obstruction hypercontracting disease Massive hypertrophy without ventricular dilation Asymetrical setum hypertrophy Endocardial thickening Microscopically - excesive hypertrophy, haphazard disarray of bundles of myocytes/fibrosis |
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What is the clinical presentaion of hypertrophic cardiomyopathy? Treatment?
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reduced chamber size and poor compliance with reduced stroke volvume
Impaired diastolic filling of LV massively hypertrophied LV Exertional dyspnea Harsh systolic ejection murmur Angina pain Problems: A fib, infective endocarditis, heart failure, arrhythmias, sudden death (young atheletes) removing septal mass and medications that relax the ventricles |
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Restrictive Cardiomyopathy
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decrease om vemtricular compliance - reults in impaired filling
-Can be confused with restrictive pericarditis, or HCM caused by radiation fibrosis, amyloidosis & sacordosis, space occupying lesion ventricles are normal sizes, but show diffuse fibrosis Conditions: - endomyocardial fibrosis (africa and tropical areas) endocardium and subendocarditis - Loeffler endomyocarditis - eosinophilia or eosinophilic leukemia - surgically remove endomyocardium |
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What are the causes of Myocarditis?
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- viruses (coxsackie viruses A and B/cytomegalovirus/HIV)
-Protozoa (trypanosoma cruzo "chagas") - South America -Helminthic - Trichinosis -Parasites - Toxoplasmosis -Bacteria - Borrella B. (Lyme) and Diptheria -allergic or drugs Chemotherapy - doxorubicin, cyclophasphamide Hyperthyroidism Hypothyroidism |
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What is the Morphology of Myocarditis?
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Heart normal or slightly dilated
Diffuse lesions Ventricular myocardium is flabby Mural thrombi in any chamber interstitial lymphocyte infiltrate and injury hypersensitivity - lymphocytes, macros, eosinophils giant cell myocarditis Chagas disease has trypanosomes near myofibers |
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What is the clinical presentation of myocarditis?
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Spectrum is broad
fatiquw dyspnea, palpitations, precordial pain May Mimic acute MI |
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What are the consequences of pericardial effusion?
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if accumulation is slow and small - pericardial enlargement
Chronic effusion or quick effisiosns - cardiac tamponade |
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Acute Pericarditis
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Serous pericarditis - rheumatic fever, SLE, Scleroderma, Tumors, Uremia.
Neutrophils/lymphs/mast cells (just lymphocytes - chronic cause) Fibrinous - most frequent. Serous and Fibrinous. Can be caused by acute MI, Dressler syndrome, Uremia, Causes a loud pericardial friction rub Purulent - infective organisms. during cardiac surgery (immunosupression is a factor. Pus may cause constrictive pericarditis. Clinically similar to fibrous, but with infection signs Hemorrhagic - blood mixed with fibrous or suppurative effusion. From bacterial infections, diathesis, or surgery. May cause tamponade Caseous - from tuberculosis (maybe fungal). Rare, but most common form of disabling fibrocalcific chronic pericarditis |
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Adhesive Mediastinopericarditis
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Adhesive Mediastinopericarditis - follows pericarditis, sac is obliterated, heart adhered to surrounding structures. Clinically, systolic retraction of ribcage, pulsus paradoxus, cardiac hypertrophy and dilation
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Constrictive Pericarditis
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Encased in fibrous sac, limited expansion, restricted CO.
No Hypertrophy or dilation Heart is quiet treat with pericardectomy |
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Rheumatoid Heart Disease
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fibrous pericarditis
inflamatory granulomatous nodules, below pericardial surface can effect valves, but has no adhesion of valves |
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Myxoma
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single growth, benign - in Atria (fossa ovalis)
Can obstruct AV valve, or have wrecking ball effect Lipidic cells covered by endothelium, resembling poor glands Ball-valve obstruction, embolism, fever-syndrome identified by echocardiograph surgery is curative Can be familial - AD, multiple mysomas, endocrine overactivity |
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Rhabdomyoma
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primary heart tumor in children
obstruction of valvular orifice Spider cells - glycogen laden vacuoles, myofibrils Harmatoma |
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Cardiac effects of Non cardiac Neoplasms
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pericardial effusion
myocardial mets superior vena cava syndrome renal cell carcinoma - block blood return Radiation to treat tumors can also damage heart |
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What are the indications for cardiac transplant
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Dilated cardiomyopathy
Ischemic Heart disease |
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What factors contribute to success for heart transplant?
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candidate selection
immunosupression early diagnosis of allograft rejection |
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What are the major complications of heart transplant?
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rejection - interstritial lymphocyte inflamation
arteriosclerosis - MI CHF, Sudden death Infection Malignancies - Lymphoma due to EBV and immunosupression |