CONGENITAL HEART DISORDERS

Arrhythmias Atrial Septal Defect Atrioventricular Canal Defect Cardiomyopathy Cardiopulmonary Arrest Coarctation Of the Aorta Congestive Heart Failure Dyslipidemia Endocarditis Hypertension Hypoplastic Left Heart Syndrome Kawasaki Disease Patent Ductus Arteriosus Persistent Pulmonary Hypertension Pulmonary Stenosis Tetralogy Of Fallot Total Anomalous Pulmonary Venous Return Transposition Of The Great Arteries Transposition Of The Great Arteries Ventricular Septal Defect Triscuspid Atresia Truncus Arteriosus

RESPIRATORY DISORDERS

Acute nasopharyngitis Aspiration Asthma Atelectasis Bronchial obstruction Bronchitis Bronchioectasis Bronchiolitis Bronchopulmonary Dysplasia Choanal Atresia Croup Cystic Fibrosis Epiglottis Epistaxis Influenza Laryngitis Pharyngitis Pneumonia Pneumothorax Respiratory Syncytial Virus Retropharyngeal Abscess Sinusitis Status Asthmaticus Tonsillitis Tracheomalacia Tuberculosis http://www.scribd.com/doc/154276198/Pediatric

I.

Pediatric Cardiovascular

A. General concepts 1. Definition: present at birth - consequences are congestive heart failure and hypoxemia 2. Etiology: usually not known - associated with maternal factors: a. infection b. alcoholism c. age over 40 years d. diabetes mellitus, type one e. genetics, chromosomal changes 3. Findings of congenital heart defects a. child small for age

Congenital Heart Defects

b. physiological failure to thrive c. exercise intolerance d. dyspnea while feeding e. squatting position f. clubbing of fingers g. cyanosis and/or pallor may be associated with poor perfusion h. unusual pulsations 4. Physical consequences of congenital heart defects a. increased workload; pulmonary hypertension; decreased systemic output; cyanotic defects b. can lead to hypoxemia and polycythemia c. concern: formation of thrombus with embolus d. blood flow patterns may be affected B. Acyanotic defect - infant/child is "pink" but child may develop cyanosis 1. Pathology: hole in the heart's internal wall a. blood flows from heart's arterial (left) to venous (right) side or a "left to right shunt", not systemically, but only within heart itself b. size of defect will determine severity of condition 2. Common types:

C. Cyanotic defect: infant/child is usually "blue", but child may appear pink 1. Pathology a. unoxygenated blood mixes with oxygenated, via a "right to left shunt" b. decreases oxygenation to the entire system c. results far more severe than acyanotic d. thrombus formation is always a concern

D. Diagnostics 1. EKG: noninvasive, painless, infants and young children may require mild sedation 2. Cardiac catheterization a. presence of diaper rash may postpone procedure b. preparation depends on level of growth and development c. post procedure child may have difficulty complying with keeping insertion point in correct position E. Nursing care of the child with a congenital heart defect 1. Emotional, physiological, and psychological interventions 2. Assisting the child and family to adjust to special needs 3. Goals of treatment a. child will maintain adequate oxygenation and physiological stability b. family will understand signs and symptoms of the condition, and how to manage each of them c. child will attain milestones of normal growth and development d. when child has surgery, child and family will be prepared, know prognosis, and know how to give home care afterward e. improve cardiac function f. remove accumulated fluid g. decrease cardiac demands h. improve tissue oxygenation 4. Nursing interventions a. recognize CHF FINDINGS OF CONGESTIVE HEART FAILURE

b. monitor height, weight, vital signs, pulses, pulse oximeter, intake and output c. medications: digoxin (Lanoxin), furosemide (Lasix) or chlorothiazide (Diuril), ACE inhibitors: Capoten, Vasotec d. monitor serum potassium level e. recognize and treat pain appropriately: pharmacological and nonpharmacological interventions f. maintain a safe environment g. conserve energy h. maintain proper nutrition, with small, frequent feedings monitor sodium intake, fluid restriction may be required i. support and discuss treatment with parents j. place in proper position - slanting position with head elevated, older babies in infant seats, occasional knee-chest 5. Teaching points a. inform parents about findings of infection b. discuss possible behavior changes the child may exhibit c. counsel parents about high-risk children requiring antibiotic prophylaxis for dental work -occur after birth as a result of infection, autoimmune responses, environmental or familial tendencies F. Hyperlipidemia: excessive lipids 1. Etiology: dietary, heredity 2. Pathophysiology

Acquired Cardiovascular Disorders

a. increased lipids and cholesterol b. causes atherosclerosis, leading to coronary heart disease 3. Diagnosis: lab tests: increased LDL, lipids and cholesterol; decreased HDL 4. Management a. diet: ADA diet in two steps: i. < 30 kcal from fat; < ten from saturated fat; < 300 mg/L cholesterol ii. < 30 kcal from fat, < seven from saturated fat; < 200 mg/L cholesterol b. medications: colestipol (Colestid), niacin (Nicor), cholestyramine (Questran) 5. Nursing interventions a. encourage screening for at risk children with family history b. teach dietary guidelines or refer to dietician G. Bacterial endocarditis: inflammation of the valves of the inner lining of the heart 1. Etiology: possible causative agents a. strept b. staph c. fungi 2. Pathophysiology a. organisms enter blood stream (vascular dissemination) b. form vegetation on endocardium 3. Diagnostics a. EKG changes b. sedimentation rate evaluated c. WBC elevated d. blood cultures 4. Management a. high doses of antibiotics 5. Nursing interventions a. monitor for signs of infection b. provide rest periods 6. Teaching points a. need for long-term IV therapy b. side effects of antibiotics c. high-risk children need prophylactic antibiotic therapy before dental work or other invasive procedures Pediatric Respiratory Respiratory Infections A. General concepts 1. Etiology: bacterial, viral; often influenced by age, season, preexisting disorder, living conditions 2. Findings: increased respiratory and heart rate, fever, nausea/vomiting, nasal discharge and blockage, mucus production, coughing, adventitious lung sounds 3. Nursing care goals a. child will not exhibit findings of respiratory distress, will be able to clear secretions, and remain comfortable with a patent airway b. child will not spread infection to others c. child will ingest adequate fluids, and maintain hydration 4. Management

II.

a. medications: antibiotics, antipyretics b. possible: anti-inflammatory, anti-mucolytics, bronchodilators, oxygen as needed 5. Nursing interventions a. chest physiotherapy b. provide nutrition and encourage fluids - Jello, soup, puddings c. promote rest and comfort d. prevent spread of infection e. ease respiratory efforts - warm mist 6. Teaching points a. handwashing b. avoid contact with affected children B. Respiratory failure: inability to maintain adequate oxygenation 1. Predisposing factors a. obstructive anomalies, aspiration b. infections, tumors, anaphylaxis c. restrictive conditions: respiratory distress, cystic fibrosis, pneumonia, pneumothorax d. paralytic conditions 2. Findings a. restlessness, mood changes b. changes in LOC c. increasing rates of respiration and pulse d. dyspnea 3. Management a. frequent observation and physical exams, with pulse oximeter b. correct hypoxemia, maintain ventilation and deliver oxygen c. monitor for side effects and expected outcomes of therapy C. Airway obstruction and basic life support 1. Cardiac arrest is usually due to prolonged hypoxemia secondary to inadequate ventilation, oxygen or circulation 2. When following guidelines for pediatric life support, consider not just the child's age, but also size. Individual anatomy and development will vary. 3. Airway clearance techniques a. determine conscious versus unconscious child b. for infants and toddlers: back blows and chest thrusts c. for preschool and school-age: modified Heimlich maneuver ("astride") Infant Respiratory Disorders D. Respiratory distress syndrome (RDS): "hyaline membrane disease" 1. Definition: related to development delay in lung maturation 2. Etiology a. premature infants: usually due to surfactant deficiency b. newborns: birth asphyxia, multiple gestations, diabetic mother c. older children: trauma, drowning 3. Pathophysiology a. decrease in amount and/or quality of pulmonary surfactant b. in older children, surfactant may be washed out by drowning or fluid aspiration c. increased alveolar surface tension d. impaired gas exchange

e. increased pulmonary resistance f. hypoperfusion 4. Findings

Findings of Respiratory Distress Syndrome The transition from respiratory-related to systemic related indicates a progressive severity of the disease 1. Respiratory related a. rales b. expiratory grunting c. nasal flaring d. retractions tachypnea, increased effort e. respiratory tachypnea paradoxic "seesaw" f. respirations apnea 2. Systemic related nasal flaring a. flaccid body substernal retractions b. nonresponsive expiratory grunt, possible apnea c. hypotension cyanosis d. shock hypoxia

a. b. c. d. e. f. g. 5. Diagnostics a. physical exam, pulse oximeter b. serum: ABG's, glucose, calcium c. chest radiograph confirmation diffuse pattern over both lung fields that resembles ground glass 6. Management:

a. oxygen therapy (possible mechanical ventilation) and chest physiotherapy to maintain ventilation and oxygenation b. medications: possible medications: surfactant, prophylactic antibiotics, diuretics, inotropes, methyl-xanthines 7. Nursing interventions: a. frequent respiratory assessment. b. maintain acid base balance and tissue perfusion c. electrolyte status d. suction as indicated 8. Teaching points: a. inform parents about equipment b. positioning of infant c. need for meticulous care by nurses due to subtle changes possible in infants oxygenation status E. Bronchopulmonary dysplasia: (BPD), chronic obstructive lung disorder 1. Etiology - at risk: a. infants requiring oxygen and/or lengthy mechanical ventilation b. infants surviving RDS c. lung immaturity 2. Pathophysiology a. mechanical ventilation presses lung tissue b. bronchial epithelium is damaged c. products of inflammation introduced, alveolar walls become thick, fibrotic d. continued mechanical ventilation affects the growth of new cells and paralyzes cilia e. lungs develop cystic areas (sacs) and atelectasis (collapsed alveoli); mucus moves less 3. Findings a. tachycardia and tachypnea b. increased work of breathing c. pallor d. cyanosis with activity e. restlessness 4. Management a. oxygen (possible mechanical ventilation) b. medications: diuretics, bronchodilators, anti-inflammatory agents in gradually decreasing amounts c. diet: increased calorie formulas and hydration d. provide supplemental oxygen at home as needed 5. Nursing interventions: a. provide rest periods b. observe for fluid overload or pulmonary edema c. provide age appropriate toys d. frequent respiratory assessment F. Apnea: cessation of breathing for over 20 seconds 1. Etiologies a. prematurity b. foreign-body aspiration, drowning, or trauma

2.

3. 4. 5.

c. incorrect positioning d. gastroesophageal reflux e. infections f. seizure g. hypoglycemia Pathophysiology: dependent on type of apnea: a. central - absence of respiratory effort and air movement b. obstructive - respiratory effort but no air movement c. mixed - first central, then obstructive Findings a. depend on type (above) b. color changes, hypotonia Diagnostics: laboratory tests, chest x-rays, EEG, ECG, pneumocardiography, upper GI series Management a. home apnea monitoring and basic life support (BLS) training to family b. medications: based on type and underlying condition

Respiratory Conditions G. Upper respiratory tract infections (URI)

1. Etiology: often acute viral nasopharyngitis, or the "common cold" 2. Pathophysiology a. organism invades mucous membranes b. edema, vasodilation and increased mucus production c. usually self-limiting 3. Findings: nasal congestion, sneezing, colored nasal discharge, low grade fever, cough, irritability 4. Management: a. medications: antipyretics, decongestants (oral or nasal), analgesics b. cool mist humidifier c. adequate fluids d. rest e. assess for presence of earache (otitis media), temperature over 38.3 degrees Celsius and refusal to eat - may indicate complications H. Sinusitis

1. Etiology: viral, bacterial, URI, obstructive deformities, cystic fibrosis 2. Findings a. cold that does not improve b. chronic nasal congestion, purulent nasal discharge c. headache

d. tenderness over sinus areas e. halitosis 3. Diagnostics: sinus x-rays, CT 4. Management a. medications 1. antibiotics 2. antipyretics 3. analgesics 4. decongestants 5. antihistamines 5. Nursing care a. monitor respirations b. warm mist (possible) I. Acute pharyngitis 1. Etiology: streptococcus (bacterial); or viral (often accompanies tonsillitis) 2. Findings a. bacterial i. "sicker" with sudden onset ii. very sore throat iii. high fever iv. headache b. viral i. gradual onset ii. fever iii. cough, rhinitis 3. Diagnostics a. throat culture b. rapid strep 4. Management a. medications: penicillin for strep, possible antipyretics and analgesics 5. Nursing interventions a. monitor for complications such as acute rheumatic fever and acute glomerulonephritis b. monitor fluid intake c. moisture compress to neck cold or warm d. cool liquids or ice chips J. Tonsillitis 1. Etiology: bacterial, viral in association with pharyngitis 2. Pathophysiology: infection and inflammation enlarge tonsils; as airway narrows, it hinders swallowing and breathing a. palatine tonsils usually visible during oral exam b. pharyngeal tonsils are also known as the adenoids 3. Findings a. "kissing tonsils" b. sore throat c. halitosis d. mouth breathing; snores at night e. fever

4. Diagnostics: history, physical exam, throat culture 5. Management a. supportive: antibiotics, fluids, rest, antipyretics, analgesics b. surgery: tonsillectomy done after 3 episodes of tonsillitis in one year c. adenoidectomy may be done with tonsillectomy, if adenoiditis is present. 6. Nursing interventions a. assess for frequent swallowing bleeding may be the cause b. assess any vomitus c. place child on side d. medicate for pain as needed e. avoid fluids with red or brown color f. soft foods avoid highly seasoned food 7. Teaching points: teach parents a. child needs quiet activity b. monitor for bleeding c. antipyretics and analgesics no ASA d. soft foods as throat is very sore K. Croup syndromes (including laryngitis, tracheitis, epiglottitis)

Similarities and Differences of Diseases Included in the Croup Syndromes

1. Definition: several airway-blocking infections, common in children

a. signs of croup i. inspiratory stridor ii. harsh/brassy cough, barking cough iii. hoarse voice iv. respiratory distress b. types, by primary area affected: i. subglottal area: acute spasmodic croup, laryngitis, laryngotracheobronchitis (LTB), tracheitis ii. supraglottal area: epiglottitis

2. Etiology a. usually viral b. occasionally bacterial (tracheitis, epiglottitis) c. younger children with "true croup" (spasmodic croup) d. older children with tracheitis and epiglottitis 3. Pathophysiology: mucosa inflamed; edema narrows the airway 4. Findings a. classic: "barky" harsh cough, stridor, hoarseness,fever, purulent secretions, dyspnea if severe b. bacterial: child looks "sicker" c. epiglottitis manifests the four "D's" i. drooling ii. dysphagia (difficulty swallowing) iii. dysphonia (hoarse voice) iv. distressed inspiratory efforts 5. Management a. viral i. cool air/mist; fluids ii. if inpatient, nebulized racemic epinephrine and inhaled steroids

iii. antipyretics b. bacterial: same as above with antibiotics, possible intubation c. epiglottitis is a medical emergency; tracheotomy may be necessary 6. Nursing interventions a. teach parent and child signs of impending airway obstruction b. report increased pulse, respirations, retractions, increased restlessness c. never attempt to directly visualize epiglottis with tongue depressor; it could precipitate laryngospasm Lower Airway Disorders L. Bronchiolitis: acute infection at bronchiolar level 1. Etiology a. viral: RSV (respiratory syncytial virus) most common b. occasionally bacterial 2. Pathophysiology a. virus spreads via direct contact b. enters body via nose or eye c. leads to edema, mucus accumulation and cellular debris which obstruct bronchioles d. can progress to atelectasis 3. Findings - infection is rare in children older than 2 years of age a. usually mild URI b. sneezing, productive cough, low-grade fever, rhinorrhea (nasal discharge), adventitious lung sounds c. otitis media and conjunctivitis may also be present 4. Diagnostics: history, RSV/viral nasal washing, chest x-ray 5. Management depends on severity a. mild: fluids, humidification, rest b. severe: hospitalization, antiviral, IV fluids, possibly bronchodilators, steroids and mechanical ventilation. Maintain contact secretion precautions c. prophylaxis: respiratory syncytial virus immune globulin in high risk infants 6. Nursing interventions a. admit each child to single room or with other RSV infected children b. teach handwashing and use of contact precautions c. assess fluid volume status 7. Teaching points a. check immunization schedule. RSV IVIG may interfere with immune response. No vaccines for 9 months after child receives these M. Pneumonia: inflammation of lung parenchyma

1. Etiology: usually classified by anatomic distribution or pathogen a. most commonly viral b. sometimes bacterial c. sometimes aspiration of foreign substance 2. Pathophysiology a. triggers terminal airways, alveoli infiltrate and cell destruction b. cellular debris falls into lumen c. bacterial agent can reach circulatory system via pulmonary lymphatics d. may occur as a complication of another illness 3. Findings a. can be abrupt or insidious b. adventitious lung sounds, fever, malaise, nonproductive cough, restless, lethargic c. progressing to more severe with retractions, respiratory distress, productive cough 4. Diagnostics a. increased WBC - chest x-Ray reveals consolidation in lungs or presence of fluid 5. Management - depends on type a. viral - oxygen, chest physiotherapy, fluids b. bacterial - antibiotics, oxygen, chest physiotherapy, fluids c. aspiration - supportive therapy, treatment of secondary complication risk d. administer pneumococcal vaccine to at risk individuals 6. Nursing interventions a. frequent assessment of respiratory status b. cool mist tent - change clothing frequently c. cluster nursing care N. Aspiration of foreign body

1. Etiology: child aspirates solids, liquids, vegetative matter into air passages, most common in older infants and children up to three years of age 2. Pathophysiology: most substances become lodged in bronchi, and severity is determined by location, substance aspirated and extent of obstruction 3. Findings: sudden coughing, gagging, wheezing, cyanosis, dyspnea, and stridor 4. Diagnostics: chest x-ray, fluoroscopy, bronchoscopy 5. Management: direct laryngoscopy or bronchoscopy to remove object, then supportive therapy 6. Nursing interventions a. recognize signs of FB aspiration b. administer back blows or Heimlich maneuver as indicated 7. Teaching points a. parents need to know most likely causes to prevent aspiration 1. peanut butter 2. balloons 3. aluminum tabs from soda cans 4. paper clips O. Long-term respiratory dysfunction: asthma/reactive airway disease 1. Definition: chronic inflammatory disorder of the airways, manifested by periods of exacerbations and remissions; has an allergic component 2. Etiology a. genetic predisposition b. triggers are: allergens, infection, stress, exercise, medical conditions, medications. c. types: intrinsic, extrinsic, occupational 3. Pathophysiology

a. trigger leads to an immediate phase reaction (cell activated, with mast cell, eosinophils and histamine released with other mediators of inflammation) b. resulting in bronchoconstriction with additional granulocyte response with more inflammatory presence

c. later phase reaction (additional inflammation and hyperresponsiveness) d. bronchospasm and obstruction cause most symptoms 4. Findings a. classic: hacking cough, wheeze on expirations, dyspnea b. cough may be nonproductive at first, to productive with mucus c. change in LOC, restlessness 5. Diagnostics a. physical exam, history b. pulmonary function tests (PFT) c. bronchodilator treatments, chest x-ray, skin testing, CBC with differential d. allergy testing 6. Management a. goals: normal growth and development, patent airway, good control b. preventive: allergen control and avoidance

c. use of peak flow meter d. medications 1. long term control (preventor) medications: to achieve and maintain control of inflammation; also called controllers 1. corticosteroids - anti-inflammatory 2. cromolyn sodium - nonsteroidal anti-inflammatory 3. nedocromil - anti-allergic and anti-inflammatory 4. long acting beta adregenics (Albuterol) used for acute exacerbations 5. methylxanthines (theophylline) bronchodilator 6. leukotriene modifiers (Zileuton) - mediator of inflammation 2. quick relief (rescue) medications to treat acute symptoms and exacerbations 1. short acting beta adrenergics 2. anticholinergics 3. systemic corticosteroids 7. Acute management a. use of bronchodilators b. steroids (inhaled, IV and/or oral) c. oxygen, IV fluids, possibility of intubation 8. Nursing Interventions a. monitor child for respiratory distress and/or need for nebulizer treatments. b. place semi-high Fowlers position c. stay with child if at all possible - have parent stay during acute phase of illness d. monitor fluid volume status 9. Teaching points a. child and family must comply with medications and treatments b. correct use of metered-dose inhaler (MDI) with a spacer c. risks include overuse of bronchodilators 10. Complications a. exercise-induced bronchospasm acute and reversible, usually stop in 20-30 minutes b. status asthmaticus emergency situation P. Cystic fibrosis 1. Definition: inherited autosomal recessive trait - abnormal mucus secretion and obstruction 2. Etiology a. genetic origin b. basic defect exocrine gland dysfunction 3. Pathophysiology a. mucous glands produce a thick mucoprotein that accumulates instead of a thin freely flowing secretion b. affects respiratory, gastrointestinal and reproductive systems 4. Findings a. gastrointestinal 1. meconium ileus 2. steatorrhea 3. prolapse of rectum 4. failure to grow b. respiratory 1. thick tenacious mucus causes patchy atelectasis cough

5. Diagnostics a. sweat chloride test - iontophoresis - Pilocarpine over 60 mEq/L is diagnostic of CF b. chest x-ray: patchy atelectasis c. stool analysis 6. Management a. CPT b. medications: bronchodilators, antibiotics (Pseudomonas aeruginosa - Cepacia, and staph aureus) c. D-Nase - decreases viscosity of mucus d. enzymes administered with meals and snacks to replace pancreatic enzymes 7. Nursing interventions a. high protein, high caloric well balanced diet, vitamins ADEK should be added b. frequent hospitalizations to treat respiratory infections c. monitor IV fluids

2. barrel-shaped chest 3. clubbing of fingers and toes