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decreased pulmonary bloodflow, HLHS, BE

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What are the two major groups of cardiac disorders?   Congenital heart disease which are anatomic abnormalities present at birth that result in abnormal cardiac function. Acquired heart disease occurs after birth.  
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What is in the Assessement?   Accurate history. Include details about the mother’s pregnancy and birth history.  
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What is in the Physical Assessment?   Inspect nutritional state. Color. Chest deformities. Unusual pulses. Respiratory status. Clubbing of fingers.  
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What is focused on the Physical Assessment?   Palpate and percuss chest, abdomen and peripheral pulses. Note rate, murmurs and additional heart sounds. Heart rate and rhythm and note the character of the heart sounds.  
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What is used for Diagnostic Evaluation of Cardiopathology in Ped's?   ECG: most commonly used. Echocardiography: non-invasive and painless. Cardiac catheterization: most invasive.  
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What is a complete assessment during preprocedural care for dx's such as catheterization, incisions, etc?   Height and weight. Baseline vital signs with pulse oximetry. Assess pedal pulses. Prepare the child and family for procedure. Provide sedation medications as ordered. Child must be NPO. Make sure the child has IV access.  
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What is observing for complications during postprocedural care for dx's such as catheterization, incisions, etc?   Assess pedal pulses. Assess temperature and color of extremity. Vital signs per protocol. Observe dressing. Fluid intake. Hypoglycemia. If bleeding, apply direct continuous pressure 1 inch above the site. Bedrest for 4 to 6 hours  
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What is a cardiac catheterization?   A radiopaque catheter that is passed through the femoral artery directly into the heart and large vessels.  
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Congenital Heart Disease   low oxygen levels in the blood cause the lips, fingers, and toes to look blue cyanotic.  
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Congenital Heart Disease Pathophysiology   Heart defect or open pathway that produces signs and symptoms indicating anatomical heart defect.  
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Congenital Heart Disease May be caused by what genetic or maternal environmental factors such as Chromosomal abnormalities (Down syndrome, trisomy21, 13 & 18, Turner syndrome), and what seven others? :   Intrauterine rubella exposure. Maternal alcoholism. Diabetes mellitus. Advance maternal age. Maternal drug ingestion. Exposure to environmental toxins and infections. Sibling or parent has history of congenital heart disease..  
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What is timing of CHD Patho?   Occurs in 8 out of 1000 births, and 50% show signs and symptoms before first year of life; may not be manifested until later in life. Principle cause of death during the first year of life.  
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Invasive Tests for CHD   Arterial Blood Gas (ABG). Fluoroscopy. Angiography. Cardiac catheterization.  
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Noninvasive tests   Cardiac magnetic resonance imaging (MRI). Echocardiogram  
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Congenital Heart Disease Clinical Manifestations   Cyanosis. Pallor. Cardiomegaly. Pericardial rubs. Murmurs. Additional Heart Sounds (S3 or S4). Discrepancies between apical and radial pulses. Tachypnea.  
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Congenital Heart Disease Clinical Manifestations Con’t   Dyspnea. Grunting. Digital clubbing. Hepatomegaly. Splenomegaly. Discrepancies between upper and lower extremity blood pressures. Crackles and wheezing  
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Median sternotomy with cardiopulmonary bypass   Direct closure with sutures only or synthetic patch sewn over the opening  
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Pulmonary artery banding   Palliative for symptomatic infants Through a thoracotomy, a strip of woven prosthetic material is passed around the pulmonary artery to constrict it. Decrease pulmonary volume & pressure  
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Prophylactic antibiotics.   To prevent bacterial endocarditis  
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Treatment Prognosis   Depends on location of defect and presence of other defects Operative mortalitiy 5-20% Excellent prognosis after surgery  
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Congenital Heart Disease Nursing Care   Observation. Assist with diagnosis. Take a careful history;  
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What is included in HX for Congenital Heart Disease?   Poor weight gain or sudden increase in weight. Poor feeding habits. Frequent respiratory infections. Unusual posturing. Exercise intolerance. Would rather sit than crawl or walk. Needs frequent rest after limited play periods.  
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Congenital Heart Disease Nursing Care Con’t   Prepare child and family for and assist with: Cardiac catheterizations. EKG. X-rays. Lab studies. Give medications. Maintain nutrition. Prevent infection.  
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What is good education for the family about disorder and methods of treatment:   Assess family's understanding of dx. Reinforce physician's explanation of disorder and treatments. Provide written instructions regarding medication schedules and treatment protocols. Encourage the family to verbalize questions, fears, and concerns.  
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Help parents and child adjust to disorder by what considerations?   Accept initial shock and disbelief, allow for period of grief. Repeat information in simple terms as often as necessary. Foster parent-child attachment, especially with newborns. Introduce parents to other families who have similarly diagnosed childrn  
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Help parents cope with effects of the disorder:   During dyspneic cyanotic spell ("blue spell"). Minimize crying by anticipating needs. Teach family to recognize signs of complications,Design activities that promote normal growth and development. Provide for adequate rest.  
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what are the signs of complications   Heart Failure. Digoxin toxicity. Vomiting. Bradycardia.Dysrhythmias. Increased respiratory effort. Hypoxemia. Cerebral thrombosis. Cardiovascular collapse.  
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What is pulmonary artery banding?   Through a thoracotomy, a strip of woven prosthetic material is passed around the pulmonary artery to constrict it. reduces the vol/press of pulmn blood flow, thus reliev sympns, of CHF preventing PVD  
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Why would you want to minimize crying?   : Because it can be too tiring for the child and may cause cyanosis due to exhaustion.  
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Atrial Septal Defect (ASD   Definition: abnormal openings between the atria, allowing blood from the higher pressure left atrium to flow into the lower pressure right atrium.  
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ASD Three Types:   Ostium primum (ASD 1).  Ostium secundum (ASD 2). Sinus venosus defect.  
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Ostium primum   Opening at lower end of septum. May be associated with mitral valve abnormalities  
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Ostium secundum   opening near center of septum.  
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Sinus venosus defect   Opening near junction of superior vena cava and right atrium. May be associated with partial anomalous pulmonary venous connection.  
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ASD Pathophysiology   At birth, pressure in left atrium exceeds that in right atrium, causing blood to flow from left to right. Oxygenated blood is forced from left atrium (high pressure) to right atrium (low pressure) which recirculates through the lungs.  
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Manifestation   May be asymptomatic. Systolic murmur heard over left intercostal space. Pulmonary congestion  
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Atrial Septal Defect (ASD) Treatment surgical   Surgical dacron patch closure of moderate to large defects. Median sternotomy (open repair) with cardiopulonary bypass is usually performed before school age. ASD 1 may require mitral valve repair or replacement  
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Nonsurgical Atrial Septal Defect (ASD) Treatment   Nonsurgical, ASD 2 may also be closed using devices during cardiac catheterization (outpatient). At some centers, still in clinical trials.  
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Atrial Septal Defect (ASD) Prognosis   Prognosis: excellent, < 1% mortality. Continued follow-up necessary.  
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Ventricular Septal Defect (VSD)   Definition: an abnormal opening in the intraventricular septum; may vary from a small pinhole to complete absence of the septum.  
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(VSD Pathophysiology   Pathophysiology: blood flows from higher-pressure left ventricle (oxygenated blood) to lower pressure right ventricle (unoxygenated blood).  
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Ventricular Septal Defect (VSD) Manifestations   Usually asymptomatic at birth, but signs of heart failure eventually manifest. Loud harsh systolic murmur with palpable thrill. Poor feeding. Cyanosis (late sign- due to reversal of shunt). Pulmonary congestion.  
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Ventricular Septal Defect (VSD) Treatment   Approximately 20-60% of all VSDs close spontaneously during the first year of life. Palliative: pulmonary artery banding in symptomatic infants to equalize shunting until old enough for surgery.  
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Ventricular Septal Defect (VSD) Surgical Treatment   Median sternotomy with cardiopulmonary bypass. Moderate to large defects are repaired with Dacron patch placed over opening  
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(VSD) Nonsurgical Treatment   May be closed using devices during cardiac catheterization. At some centers, still in clinical trials.  
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VSD Prognosis   Prognosis: excellent, < 5% mortality. Continued follow-up necessary  
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Patent Ductus Arteriosus   Definition: failure of fetal ductus arteriosus to completely close within first few weeks after birth  
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What Increases workload on left side of heart due to increased pulmonary blood flow?   Blood from aorta (high pressure) is forced into pulmonary artery (lower pressure) to be reoxygenated in lungs and returned to left atrium and ventricle.  
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what condition may also be a lifesaver for another condition?   PDA may be lifesaving in neonates with cyanotic heart disease and provide the only source of pulmonary blood flow in these infants. (decrease pulmonary flow = cyanotic heart disease)  
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Patent Ductus Arteriosus (PDA) Manifestations   May be asymptomatic in infancy. Continuous "machinelike" murmur, left second intercostal space. Full and bounding pulses due to "runoff" of aortic blood flow into pulmonary artery. Dyspnea with age. wide range b/w Sys/Dias bp, hypoxia  
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(PDA) medical Treatment   Medical Management: indomethacin (Indocin) closes patent ductus in newborns and premature infants.  
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PDA Surgical Mgm   Left thoracotomy: duct tied off (ligated) or divided. Visual-Assisted Thoracoscopic Surgery (VATS): three small incisions on left side of chest; then a thorascope and instruments are used to place clip on ductus arteriosus.  
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Which defect may be necessary to sustain life in neonates with a cyanotic heart defect?   Answer: Patent ductus arteriosus.  
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Coarctation of the Aorta (COA)   constriction or narrowing of aortic arch, or descending aorta  
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COA Pathophysiology   Increased pressure proximal to defect. Decreased pressure distal to defect.  
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Coarctation of the Aorta (COA) Manifestations   Blood pressure in arms will be 20mmHg higher than in legs.   Bounding pulses in upper extremities. Signs of heart failure. Leg cramping on exertion in older children. Epistaxis.  
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Coarctation of the Aorta (COA) Surgical Treatment   Anastomosis. Graft replacement of narrowed section of aorta. Closed heart surgery is performed because structures are outside of heart. Aorta will grow but graft will not.  
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(COA) Treatment Nonsurgical   Ballon angioplasty. If restenosis occurs after surgery for coarctation, a balloon can relieve the obstruction.  
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(COA) Nursing Care Observe post operatively for   Hypertension. Abdominal pain associated with nausea and vomiting. Leukocytosis. Gastrointestinal bleeding or obstruction.  
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(COA) Nursing Care   Administer medications per orders. Perform nasogastric tube decompression. Prognosis: < 5% mortality with isolated COA.  
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What is the treatment for coarctation of the aorta?   Surgical repair.  
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Tetralogy of Fallot (TOF)   Ventricular septal defect. Pulmonic stenosis. Overriding (dextraposition- to the right) aorta. Right ventricular hypertrophy.  
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TOF VSD Patho   an abnormal opening in the intraventricular septum.  Vary in size; left to right shunt  
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Stenosis   Stenosis of the pulmonary artery decreases blood flow to the lungs.  
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Dextraposition   Dextraposition of the aorta.   Detro mean right – aorta displaced to right blood enters from both ventricles  
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Obstruction   Obstruction of flow to the pulmonary artery. Cyanosis increase blood flow to aorta right ventricle hypertrophy  
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Tetralogy of Fallot (TOF) Manifestations   Clubbing of fingers and toes, poor growth. Cyanosis increases with age. Feeding problems. Growth retardation. Frequent respiratory infections. Dyspnea on exertion. Polycythemia. Paroxysmal hyper cyanotic episodes, or "tet" 1st 2 yrs  
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Tetralogy of Fallot (TOF) surgical Treatment   Surgical: Blalock-Taussig procedure. Corrective surgery for all defects performed on older children with good results.  
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Medical   IV prostaglandin E1 therapy.  
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How many defects are there in tetralogy of fallot?   4  
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Hypoplastic Left Heart Syndrome (HLHS)   underdevelopment of the left side of the heart, resulting in an absent or nonfunctional ventricle and hypoplasia of the ascending aorta.  
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(HLHS) Pathophysiology:   Most blood from left atrium flows across a patent foramen ovale into right atrium where it mixes with desaturated blood. Blood flows to right ventricle and into pulmonary artery. Descending aorta receives mixed blood from patent ductus arteriosus  
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HLHS Manifestations:   Manifestations: Symptomatic in the first week of life with cyanosis and CHF. Fatal in the first months of life without interventions  
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HLHS Treatment:   Treatment: Neonates require stabilization with mechanical ventilation and inotropic support preoperatively.  
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(HLHS) Treatment Surgical   Norwood procedure: anastomosis of the main pulmonary artery (to aorta to create new aorta). Modified Fontan procedure: systemic venous return is directed to the lungs without a ventricular pump thru surgical conx b/w the right atrium/pulmn artry  
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(HLHS) Treatment   Bidirectional Glenn shunt: performed at 6-9 months of age to relieve cyanosis. Superior vena cava to side of right pulmonary artery; blood flow to both lungs. Transplant  
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HLHS Prognosis   Prognosis: initial survival of infant depends on a patent foramen ovale and ductus arteriosus to provide a pathway for oxygenated blood to the general body system.  
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What two defects are necessary for survival with hypoplastic left heart syndrome?   Patent foramen ovale and patent ductus arteriosus.  
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Congestive Heart Failure   the inability of the heart to pump an adequate amount of blood to the systemic circulation at normal filling pressures to meet the metabolic demands of the body.  
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Two Categories   Right-sided failure. Left-sided failure. Signs and symptoms are not indicative of which category of defect the child has.  
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Three Groups:   Impaired Myocardium Functioning. Pulmonary Congestion. Systemic Venous Congestion  
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Impaired Myocardial Functioning   Tachycardia. Sweating. Decreased urine output. Fatigue, weakness and restlessness. Anorexia. Decreased peripheral pulses. Decreased blood pressure. Gallop rhythm. Cardiomegaly  
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Pulmonary Congestion   Tachypnea and dyspnea. Retractions and nasal flaring. Exercise intolerance. Orthopnea. Cough, hoarseness. Cyanosis. Wheezing. Grunting.  
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Systemic Venous Congestion   Weight gain. Hepatomegaly. Peripheral edema. Ascites. Neck vein distension.  
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Congestive Heart Failure Diagnosis:   Based on symptoms. Chest x-ray. EKG  
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Therapeutic Management Goals   Improve cardiac function. Remove accumulated fluid and sodium. Decrease cardiac demands. Improve tissue oxygenation.  
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To Improve Cardiac Function how does the provider increase contractility and decrease afterload?   Admin of digitalis glycosides. Digoxin is given PO or IV in divided doses over 24 hours to produce optimal effects. Digoxin (Lanoxin): slow and strengthen hb;ACE inhib Captopril (Capoten): tid Enalapril (Vasotec): bid  
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Remove Accumulated Fluid and Sodium Decrease preload   Diuretics: furosemide (Lasix). chlorothiazide (Diuril). spironolactone (Aldactone). Fluid restrictions.  
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Decrease Cardiac Demands   Limit physical activity. Preserving body temperature. Treating infections. Reduce the effort of breathing. Prescribed medications to sedate an irritable child.  
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Improve Tissue Oxygenation and Decrease Oxygen Consumption   Use supplemental oxygen as ordered. An oxyhood is preferred with young infants whereas a nasal cannula or face tent may be used with older infants and children.   Cool humidification is necessary to counteract the drying effects of the oxygen.  
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Congestive Heart Failure Nursing Considerations   Position for optimal ventilation. Administer oxygen. IV access. Cardiac monitor and pulse oximetry. Assist in measures to improve cardiac function. Decrease cardiac demands.  
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Congestive Heart Failure Nursing Considerations   Reduce respiratory distress. Protect child from infections. Maintain nutritional status. Assist in measures to promote fluid loss. Support child and family.  
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Congestive Heart Failure Nursing Diagnoses   Decreased cardiac output. Ineffective breathing pattern. Fluid volume excess. Activity intolerance. Risk for infection. Altered family processes.  
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Decreased urine output can be a sign of what?   Decreased cardiac output.  
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Bacterial Endocarditis   infection of the valves and inner lining of the heart.  
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Causes   Most common: streptococcus viridans. Staphylococcus aureus. Gram-negative bacteria. Fungi: candida albicans.  
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Organisms enter the bloodstream   Via mouth after dental work. UTI. Heart from cardiac surgery. Directly via bloodstream (long term indwelling catheter).  
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Lesions can break off and invade other tissue.   Spleen Kidney CNS  
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Bacterial Endocarditis Diagnostic Evaluation   EKG can visualize. Definitive diagnosis based on identification of bacteria in blood. clinical Manifestations  
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Based on Clinical Manifestations:   Unexplained fever. Anorexia, malaise and weight loss. Splinter hemorrhages under nails. Petechiae. CHF. Cardiac dysrhythmias. New murmur or change in existing.  
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Bacterial Endocarditis Therapeutic Management:   High dose antibiotics (2-8 wks IV). Blood cultures. Preventative measures. Prophylactic antibiotics 1 wk prior  
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Nursing Considerations:   Need for prophylactic antibiotic therapy. Signs and symptoms of infection. Signs and symptoms of complications.  
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Once a patient has been diagnosed with bacterial endocarditis, what must be done in order to prevent recurrence prior to invasive procedures?   Preventative measures include prophylactic antibiotic therapy 1 week prior to procedures.  
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Rheumatic Fever   an inflammatory disease that occurs after infection with group A beta hemolytic streptococcal pharyngitis. It involves the joints, skin, brain, serous surfaces, and heart.  
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Etiology:   Most significant sequelae is rheumatic heart disease. a strong relationship between upper respiratory infection with group A beta hemolytic streptococci and development of RF.  
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Rheumatic Fever Diagnostic Evaluation   Diagnosis is based upon the presence of two major manifestations or one major and two minor manifestations Children tested for streptococcal antibodies  
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Major   Tachycardia, chest pain, cardiomegaly (carditis) Swollen, painful joints (polyarthritis) Speech disturbance/sudden irregular movement of extremities  
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Minor   Arthralgia Fever  
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Eradicate Group A beta hemolytic streptococcal infection.   Penicillin (PCN) is the drug of choice. Erythromycin (if allergic to PCN).  
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other treatment goal?   Penicillin (PCN) is the drug of choice. Erythromycin (if allergic to PCN).Treat other symptoms: salicylates to control inflammation, fever and pain. Prevent recurrences of the disease  
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How is recurrences of the disease prevented?   Start prophylactic treatment after the acute therapy (monthly intramuscular injections of penicillin G, two doses of oral penicillin daily, or one daily dose of sulfadiazine. Antibiotic prophylaxis for dental work.  
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Rheumatic Fever Nursing Care   During home care, interventions should be focused on rest and adequate nutrition. Educate children on preventing spread of throat infections. Patients with chorea need to be protected and reassured.  
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what are nursing care objectives for rheumatic fever?   Encourage compliance. Facilitate recovery. Provide support. Prevent disease.  
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What infection is responsible for triggering rheumatic fever?   Group A beta hemolytic streptococci.  
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Hyperlipidemia   excessive lipids; hypercholesterolemia refers to excessive cholesterol in the blood. Both are believed to play a role in producing atherosclerosis which eventually can lead to coronary heart disease.  
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Why should children be screened for HLD   Preventative cardiology focuses on screening and management of lipids during childhood. Goal is to identify those at risk and intervene.  
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Hyperlipidemia Treatment   Tx is primarily dietary. AAP guidelines recommend restricting intake of cholesterol and fat. If child does not respond to diet changes, drug therapy may be needed  
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What drug therapy?   Cholestyramine (Questran). Colestipol (Colestid). These two drugs are bile acid-resins or sequestrants which act by binding bile acids in the intestines.  
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Side effects of meds include:   Constipation. Abdominal pain. GI bloating. Flatulence. Nausea.  
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Hyperlipidemia Nursing Considerations   Screening, education and support  
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Education parents and child   About cholesterol, HDLs and LDLs and triglycerides. Behavioral risk factors. Medications. Dietary changes. Keeping all appointments.  
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True or False: Treatment of high cholesterol is primarily through changing the diet.   True. Medications are only used if there is no response from changing the diet.   
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Cardiomyopathy   abnormality in myocardium where cardiac contraction is impaired.  
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Though the etilogy is unknown what primary factors are related to cardiomyopathy?   Familial or genetic causes. Infection. Deficiency states. Metabolic abnormalities. Collagen vascular disease.  
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Secondary factors   Anthracycline toxicity. Hemochromatosis. Duchenne muscular dystropy. Kawasaki disease. Collagen diseases. Thyroid dysfunction.  
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Cardiomyopathy Divided into 3 categories   Dilated cardiomyopathy. Hypertrophic cardiomyopathy. Restrictive cardiomyopathy  
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Dilated Cardiomyopathy   Where ventricles are dilated and contractility is greatly decreased. Most common category with children.  
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Dilated Cardiomyopathy s/s   Tachycardia. Dyspnea. Hepatosplenomegaly. Fatigue. Poor growth.  
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Hypertrophic Cardiomyopathy   Characterized by an increase in heart muscle mass without an increase in cavity size.  
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Hypertrophic Cardiomyopathy symptoms   Chest pain. Dysrhythmias. Syncope. Chest x-rays show enlarged heart. ECG shows ST-T changes.  
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Restrictive Cardiomyopathy   Describes a restriction to ventricular filling caused by endocardial disease. Symptoms are those common with CHF.  
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Cardiomyopathy Therapeutic Management   Treatment is directed towards correcting the underlying problem or managing the symptoms. Dysrhythmias are monitored and treated with anticoagulants. Heart transplantation may be needed for children with worsening symptoms despite medical therapy.  
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Medications are given such as   Digoxin. Diuretics. Beta Blocker: propranolol (Inderal). Calcium-Channel blocker: verapamil (Calan).  
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Cardiomyopathy Nursing Considerations   Restrict activity to decrease demand on the heart. Treat as you would treat a child with CHF. Prepare the patient and family for postoperative period.  
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: Name the three categories of cardiomyopathy.   Dilated, hypertrophic and restrictive cardiomyopathy.   
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Definition: consistent elevation of blood pressure   Systemic Hypertension  
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Two Major Categories   Essential. Secondary.  
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Etiology of Systemic Hypertension:   Most occurs secondary to structural abnormalities or underlying pathologic processes. Most common cause is renal disease, followed by congenital, vascular and endocrine disorders.  
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Systemic Hypertension Diagnostic Evaluation, clinical manifestations   Clinical manifestations depending on age: Infants and young children: Irritability. Head-banging or head-rubbing. May wake up screaming at night.  
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Adolescents and oler children effects as dx eval:   Adolescents and older children: Frequent headaches. Dizziness. Changes in vision.  
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htn defined by percentile   Significant hypertension is a BP persistently between the 95th and 99th percentiles. Severe hypertension is a BP persistently at or above the 99th percentile.  
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Labs include:   Urinalysis. Renal function panel. Lipid profile. CBC. Electrolytes.  
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Systemic Hypertension Therapeutic Management, treating underlying cause, pharmacological, and nonpharmacological, what are pharm interventions?   Beta blockers. ACE inhibitors. Diuretics.  
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Non-pharmacologic interventions:   Nutrition counseling. Weight reduction. Weight control. Exercise program. Counsel adolescents on effects of drug, alcohol and tobacco use.  
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What is the pt educaiton?   How to take BP at home When to contact provider. Medications. Follow-up. Compliance. Referrals.  
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Why is BP cuff size important in getting accurate readings in children?   Cuffs that are too small will give a falsely high reading while cuffs that are too large will give a falsely low reading.  
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Kawasaki Disease   an acute systemic vasculitis of unknown cause  
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Kawasaki Disease Pathophysiology:   Initially extensive inflammation. Arterioles Venules Capillaries Progresses to formation of coronary artery aneurysms.  
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Kawasaki Disease Clinical Manifestations (at least 5 of the following:   Sustained fever. Bilateral conjunctivitis. Changes in oral mucosa, Changes in the extremity, Polymorphous rash. Enlarged non-tender cervical lymph nodes.  
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what are examples of oral mucosa changes?   Fissured lips. Strawberry tongue. Inflammation of the mouth and pharyngeal airways.  
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what are examples of changes in extremity?   Peripheral edema. Erythema and desquamation of palms and soles.  
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kawasaki disease Treatment   IV gamma globulin. Salicylate therapy. Warfarin (Coumadin) therapy  
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Nursing Care   Initial phase-monitor cardiac status. Assess for signs of CHF. Symptomatic relief such as using cool cloths, unscented lotions and soft, loose clothing. Educate parents on suspending all immunizations. Reinforce teaching on recognition of problems  
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What can a nurse do to minimize skin discomforts for a child with Kawasaki Disease?   Provide cool cloths, unscented lotions and soft, loose clothing.  
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