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CV Exam 4 Diseases
Diseases and conditions for CV Exam 4
35
Medical
Professional
10/12/2011

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Term
Stable/Exertional/Typical/Effort Angina
Definition
discomfort is predictable and reproducible at a certain exertion level, relieved by rest/nitroglycerin, transient ischemia/pain, usually due to coronary atherosclerosis, precipitated by heavy meal/exertion/cold/smoking, try to minimize ischemia and improve survival, treat with nitrates/BBs/CCBs/antiplatelets/ranolazine
Term
Variant/Prinzmetal Angina
Definition
chest pain at rest with ST elevation, occurs in young female smokers, circadian rhythm with most attacks in morning hours, triggered by EtOH/vasoconstrictors/ergonovine/cold/HTN, associated with migraines/Raynaud's phenomenon, varies with menstrual cycle/estradiol levels, serious cardia arrhythmias, high risk for SCD, try to decrease vasoconstriction and increase vasodilation, treat with nitrates/CCBs/aspirin, DO NOT USE NON-SELECTIVE BBs
Term
Unstable Angina
Definition
acute coronary syndrome without release of enzyme markers of MI, severe discomfort at rest or with minimal exertion, crescendo pattern, try to releive ischemia and prevent MI/SCD, decrease physical activity, supplement O2, treat with nitroglycerin/morphine/BBs/CCBs/ACEI/Aldosterone Antagonists/antiplatelets/anticoagulants
Term
Myocardial Infarction
Definition
necrosis caused by local ischemia, commonly due to coronary artery thrombosis, necrosis 20-30min after occlusion, subendocardium (inner 1/3) most vulnerable, silent infarcts if diabetic
Term
Complications of an MI
Definition
papillary m. dysfunction, cardiac rupture, acute pericarditis, ventricular aneurysm
Term
Chronic Ischemic Heart Disease/Ischemic Cardiomyopathy
Definition
progressive due to long-term myocardial injury, moderate to severe coronary atherosclerosis, dilated cardiac chambers, myocardial fibrosis with angina/MI/arrhythmias
Term
Sudden Cardiac Death
Definition
due to ischemic heart disease, marked cardiac death, usually due to fatal arrhythmia
Term
Hypertensive Heart Disease
Definition
concentric LVH, increased metabolic requirements of stiff myocardium, enlarged fibers and boxcar-shaped nuclei
Term
Alagille Syndrome
Definition
due to deletion of JAG1 (20p12), peripheral pulmonary stenosis/TOF/left-heart obstruction, liver disease/butterfly vertebrae/renal and ophthalmologic findings
Term
Noonan Syndrome
Definition
due to deletion of PTPN11 (12), pulmonary stenosis/hypertrophy, short stature/webbed neck/facial dysmorphisms/chest deformity, "Male Turner Syndrome"
Term
Holt-Oram Syndrome
Definition
due to TBX5 TF (12q24.1) deletion, ASD/VSD/conduction abnormalities, upper limb radial ray defects
Term
DiGeorge Syndrome/Velocardiofacial Syndrome
Definition
22q11 deletion, due to abnormal migration of neural crest tissue, supracristal VSDS/PTA/TOF, thymic aplasia/hypoparathyroidism/heart defects, palate anomalies/feeding disorders/speech and learning difficulty/renal anomalies
Term
Williams Syndrome
Definition
7q11.23 deletion, elastin arteriopathy with supravalvular AS/PS, hypercalcemia causes renal/skeletal anomalies, social personalities, elfin faces
Term
Rubella (Maternal Infection)
Definition
causes peripheral pulmonary stenosis
Term
Coxsackie (Maternal Infection)
Definition
causes myocarditis
Term
ASD, VSD, PDA
Definition
Left to Right Shunt Defects
Term
AS, PS
Definition
Left/Right obstructive lesions
Term
Eisenmenger Syndrome
Definition
seen in left-to-right shunts, pulmonary resistance surpasses systemic resistance, reversal of flow causes cor pulmonale and heart failure
Term
Left-to-right shunts
Definition
no cyanosis, volume/pressure overloads cause compensation, can progress to Eisenmenger Syndrome
Term
Right-to-left Shunt
Definition
seen in TOF/TGA, mixing of venous blood into systemic circulation, hypoxemia/cyanosis, clubbing of fingers/toes, reactive polycytemia, increased risk of paradoxical embolism
Term
Obstructive Anomalies
Definition
COA, PS, AS, Atresia, narrowing of chambers/valves/vessels, proximal chamber pressure overloads/hypertrophy
Term
Ventricular Septal Defects
Definition
most often with TOF, 30% isolated, membranous (90% of cases, septum doesn't develop), muscular (excess cavitation of muscle tissue, closes spontaneously), small defects cause loud murmurs, large defects equalize pressure and cause soft murmurs, increased risk of infective endocarditis, left heat volume overload/dilation, moderate-large VSDs cause CHF and require surgery <6mo, murmur develops over weeks as flow through pulmonary vessels increases
Term
Atrial Septal Defects
Definition
secundum (90%, in fossa ovalis), primum (adjacent to AV junction), sinus venosus (near SVC), significant ASDs cause left-to-right shunts and increase pulmonary flow, clinically manifest at 30yo or early adolescence, secundums cause a flow murmur/split S2 and must be repaired <4yo, right heart volume overload/dilation, exercise intolerance/arrhythmia, surgerical repair to avoid dilation at 2-5yo (size of vascular catheters limit early intervention)
Term
Atrioventricular Septal Defect
Definition
failure of endocardial cushions of AV canal to fuse, incomplete closure of AV septum and mitral/tricuspid valves, Partial (primum ASD, malformed mitral valve/insufficiency), Complete (large combined septal defect, one central AV valve), 1/3 of cases due to Down Syndrome, can be fixed with surgery but permanent AV valve regurgitation
Term
Patent Ductus Arteriosus
Definition
ductus arteriosus remains open after birth, continuous machine murmur, severe shunting can cause Eisenmonger's Syndrome, differential cyanosis (toes but not fingers, distal to subclavian aa), increased risk of endocarditis/paradoxical embolism, treated medically at birth (O2, PGE1 inhibitors), treated surgically at any age (ligation), flow from high resistance systemic to low resistance pulmonary circulations, left heart volume overload/dilation
Term
Pulmonary Stenosis
Definition
faulty development of spiral ridges of bulbs/truncus arteriosus, increased right heart pressure load/hypertrophy, frequently improve with time, severe cases can cause right ventricualr scarring/arrhythmia, balloon dilation to open vessel, surgical valvectomy if dysplastic (Noonan's Syndrome) causes permanent pulmonary regurgitation
Term
Aortic Stenosis
Definition
valvular/subvalvular/supravalvular, increased left heart pressure/hypertrophy, causes HLHS, commonly have bicuspid aortic valve and calcified AS later in life, LV ischemia due to increased LV pressure and normal coronary pressure, decreased CO, increased risk of SCD, balloon valvulopathy causes permanent aortic regurgitation, eventually must have a valve replacement, Ross procedure, avoid prosthetic valves/anticoagluation in children
Term
HLHS
Definition
hypoplastic LV/aortic arch, endocardial fibroelastosis, PDA
Term
Coarctation of Aorta
Definition
associated with bicuspid aortic valve/berry aneurysms, more common in males than females, upper body increased BP, lower body decreased BP, diagnose with a 4-extremity BP, increased risk of stroke/LVH/claudication, balloon coarctation angioplasty with or without stent placement, resection with end-end anastamosis
Term
Preductile (infantile) Coarctation
Definition
symptomatic early in life, can involve entire arch, right-to-left shunting, cyanosis, often seen in Turner's Syndrome
Term
Postductile (adult) coarctation
Definition
more common, collateral circulation to UE via intercostal aa., rib notching, LE claudication, UE HTN
Term
Tetralogy of Fallot
Definition
most common cyanotic defect, right-to-left shunt, VSD/subpulmonary stenosis/aortic override/RVH, no pulmonary HTN, FTT due to poor peripheral oxygenation, increased risk of emboli/endocarditis/braine abscess, "boot-shaped heart" on X-ray, surgical correction by pulmonary valvectomy/transannular patch/close VSD/pulmonary valve replacement
Term
Transposition of Great Arteries
Definition
RV connected to aorta, LV connected to pulmonary a, incompatible with life without septal defects/PDA, due to non-spiraling of ridges in truncus arteriosus, PGE1 used to maintain ductal patency, balloon atrioseptostomy to open up foramen ovale, "Egg on a string" on Xray, Atrial switching (excellent short-term, long-term RV dysfunction/arrhythmia), Arterial Switching (excellent intermediate-term)
Term
Common Ventricle
Definition
single undivided ventricle with separate atria (cor triloculare), separate or fused mitral/tricuspid valves, some degree of transposition, obligatory mixing of red/blue blood, cyanosis depends on amount of pulmonary flow (better without PS), PGE1 used to maintain ductal patency, HRHS, goals of surgery are adequate flow/decrease heart work/separate red and blue flow, BT/Gleen/Fontan shunts
Term
HRHS
Definition
due to pulmonary atresia
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