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Cardiac Disorders in Peds Two major groups of disorders: Congenital Aka “born with” Most structural defects Acquired Develop later in life Bacterial endocarditis Rheumatic fever Kawasaki disease Systemic HTN Incidence & Causes 5 to 8 in 1000 live births Cause unknown Multiple factors Genetics/family history Environment Toxins Viruses Maternal chronic illness (diabetes, seizure meds) Chromosomal abnormalities Down syndrome DiGeorge syndrome Noonan or William syndrome Trisomy 13 or 18 Older Classifications of CHD Acyanotic “pink” NO unoxygenated blood goes to the periphery Cyanotic “blue” Unoxygenated blood is shunted to the periphery May be pink Newer Classifications of CHD Hemodynamic characteristics Increased pulmonary blood flow Too much to lungs; “pink”; pulmonary edema Decreased pulmonary blood flow Too little to lungs; “blue”; cyanotic Obstruction of blood flow out of the heart Can’t get to lungs or body Mixed blood flow Most common Comparison of CHD Classification Systems— p.1276 10th ed. Hockenberry Background info/Hemodynamics Review fetal to neonatal circulation (also pp. 1342-1343 Hockenberry, 9th ed.; pp. 1252-1253, 10th ed.) Blood flows from area of high pressure to one of low pressure (Fig. 34-7 p. 1351 Hockenberry, 9th ed.; Fig. 29-7 p. 1262, 10th ed.) The greater the pressure gradient, the greater the rate of flow. The greater the resistance, the lower the rate of flow In the NORMAL HEART, pressures on the R side are less than the L side, and the resistance in the pulmonary circulation is less than that in the systemic circulation. Fetal circulation Fetal to Neonatal Circulation p.1262 10th ed. The proposed pulse-oximetry monitoring protocol based on results from the right hand (RH) and either foot (F). Kemper A R et al. Pediatrics 2011;128:e1259-e1267 ©2011 by American Academy of Pediatrics Tests of cardiac function Prenatal ultrasound Chest x-ray Electrocardiogram (ECG) Echocardiogram Cardiac catheterization Stress test (dobutamine or exercise) Cardiac MRI Cardiac Catheterization Invasive routine diagnostic procedure Benefits Better visualization Actual pressures, sats, hemodynamic values Risks: Hemorrhage Fever N/V loss of a pulse transient dysrhythmias Nursing interventions for Cardiac Catheterization (pp.1348-9 , 9th ed., pp. 1258-60, 10th ed. Hockenberry) Pre-procedure: Complete a thorough hx & physical exam Check for allergies to iodine and shellfish age appropriate teaching & preparation Don’t forget the parents NPO 4-6 hrs before procedure; sedation~ IV or po Monitor VS, SaO2, Hgb, Hct, coags, BMP Mark pedal pulses—before procedure to ensure correct palpation afterwards. Determination the amount of sedation based on the child’s age, condition & type of procedure Cardiac Catheterization Post-procedure: √ for bleeding at site of insertion of catheter in groin √ pulses esp. distal to site of insertion, temp & color of extremities, VS q 15 Remember the 5 P’s (pain, pallor, pulse, paresthesia, paralysis) OR CMTS—circulation, mobility, temperature, sensation √ heart rate for one full minute, for signs of dysrhythmias or bradycardia Prevent bleeding by keeping leg immobilized for 4-8 hrs I & O, especially O. Fluids may be offered po starting with clear liquids. √ Labs; infants are at risk for hypoglycemia—monitor blood glucose as child may need IV with dextrose Encourage the child to void to promote excretion of contrast medium. Cardiac Catheterization (cont’d) Potential cardiac catheterization complications: Nausea &/or vomiting Low-grade fever Loss of pulse in catheterized extremity Transient dysrhythmias Acute hemorrhage from entry site apply direct continuous pressure at 2.5cm above the catherter entry site to localize pressure over the location of the vessel puncture. Keep child flat and notify the physician Prepare for possible administration of additional fluids prn Cath lab Congestive heart failure (Fig. 34-8 p. 1353, 9th ed. ; fig. 29-8 p. 1263, 10th ed. Hockeberry) Symptoms of CHF Increased work of breathing Tachycardia Decreased pulses Decreased urinary output Poor weight gain Diaphoresis with activity Hepatomegaly Cold, cool extremities, especially with stress or activity JVD? Decreased BP is LATE sign Defects with Increased Pulmonary Blood Flow Abnormal connection PDA, ASD, VSD between two sides of heart Symptoms leads to Increased work of Increased blood volume on breathing right side of heart Increased pulmonary Rales/rhonchi blood flow and/or wheezing Decreased systemic blood Failure to thrive flow Patent Ductus Arteriosus Ductus doesn’t close Common in preemies “machinery” murmur audio Treatment Indomethacin Cath lab Ligation Atrial Septal Defect Hole between two atria of heart Usually asymptomatic If not treated, increased risk of atrial dysrhythmia or stroke Usually close on own Ventricular Septal Defect Hole between two ventricles of heart Symptoms related to size & location of VSD and amount of pulmonary blood flow Fix by patching with Goretex Atrioventricular Canal ASD, VSD, and affected mitral & tricuspid valves Associated with Down syndrome Symptoms related to size of holes, degree of valvular involvement, & size of ventricles Often accompanied with pulmonary hypertension Nursing Management AVOID OXYGEN!!!!!!!! Especially pre-op Diuretics—furosemide, chlorothiazide, spironolactone Monitor VS, I & 0, daily wt. Encourage rest periods to conserve energy Monitor labs: Hgb, Hct, electrolytes Closely monitor feedings May need higher calorie feeds Obstructive Defects Coarctation of the aorta, aortic stenosis, pulmonic stenosis Symptoms dependent upon area of obstruction Coarctation of Aorta Narrowed aorta leads to decreased systemic blood flow May not present until early childhood Bounding upper extremity pulses, weak to absent lower extremity pulses HYPERTENSION!!!!!!! Post-op Coarctation Care Neuro checks Urine output Blood pressure PAIN!!!!!!! Aortic stenosis Obstructs blood flow to body Leads to left ventricle hypertrophy Asymptomatic often Chest pain with exercise Sometimes see sudden death Repair with ballooning, repair, or replacement of valve Pulmonary Stenosis and Catheter Placement Leads to right ventricular hypertrophy which may lead to reopening of the foramen ovale. If severe, my lead to congestive heart failure. Defects with Decreased Pulmonary Blood Flow and Mixed Defects May or may not be cyanotic (usually are) Tetralogy of Fallot Transposition of Great Arteries Truncus Arteriosus Hypoplastic Left Heart Syndrome (HLHS) LOTS of other defects that are uncommon, book discusses them Effects of Hypoxemia Main clinical manifestations: Cyanosis Polycythemia Thicker blood Clubbing Clotting abnormalities Delayed growth and development – can be associated with any heart defect Hypoxemia Management Prostaglandin E1 given if cyanosis shown as newborn Assess for and treat tet spells Surgery Corrective or palliative—often staged Prevent dehydration AVOID OXYGEN!!!!! Tetralogy Of Fallot Hypercyanotic “tet spells” Acutely cyanotic ↓ pulm. blood flow & ↑ right to left shunting Prompt tx to prevent brain damage &/or death Calm infant/child Place in knee chest position Toddler will get in “squatting” position to compensate for hypoxia Give oxygen Morphine/fentanyl/versed given Knee-Chest Position Tet Repair Complicated Dependent on how big RV is, how stenotic pulmonic valve is, and how big the VSD is Either fly or die Palliative shunt: modified Blalock-Taussig shunt (p.1364, Table 34-4, 9th ed. Fig. 29-11, p.1274, 10th ed.) Complete repair— operative mortality <3%! Transposition of the Great Arteries NOT GOOD! Cath lab initially Prostaglandins Surgery at 6-7 days old— arterial switch of pulmonary artery and aorta, but also coronary arteries are switched and re-anastomosed. Long term prognosis very good Hypoplastic Left Heart Syndrome VERY VERY VERY BAD!! Mortality rates range from 10-30% (2002-04) Can not correct easily 3 staged surgeries: Norwood, Mod Blalock Taussig, & Glenn procedure vs. transplant Long-term data not in yet, will probably need transplant Management of Children with Mixed Defects Medications Digoxin—KNOW!! pp.1354- 1358, 9th ed. P.1269-70, 10th ed.— good info on meds Improves contractility of heart Review dig toxicity—pulse rates in infants & children Diuretics—furosemide Watch for what ?? Ace-inhibitors (angiotensin converting inhibitors—the PRIL’s) Reduce afterload on the heart make heart pump more efficiently. Beta-blockers—cause decreased heart rate, BP * vasodilatation Decrease cardiac workload Meds-as stated Decrease stimulation Cluster care Maintain neutral thermal environment Sedation for irritable child Remove accumulated fluid & sodium Closely monitor I&O Restrict fluid in acute phase Weigh daily if stable Continued management of CHF Nutrition Smaller, more frequent feeds High calorie formula Decrease respiratory effort Rest Avoid colds, RSV Position with HOB Avoid crying and distress Family support/education Keep them present, holding, rocking, AMAP Improve tissue oxygenation Meds assist with this by increasing efficiency of the heart Oxygen may be added with appropriate order, especially if there is pulmonary edema, or lower respiratory infection. Post-operative Care Neurological checks PAIN!!!!!!!!!!!!!!!!!!! Cardiac monitoring Heart rate Move all extremities Back to baseline Respiratory care Blood pressure Deep breathing Intracardiac pressures IS Chest tube care Quantity & quality of output Urine output Minimum 1 ml/kg/hour Rest & activity Up next day Ambulate GI distress Avoid vomiting Care of the Family and Child with Congenital Heart Disease Help family adjust to the disorder May be grieving loss of normal child Educate family Help family cope with effects of the disorder Prepare child and family for surgery Remember developmental level of child Pain, scars, IS, activity Refer to support group with families who have already been through the experience TOUCH is the IL Assoc. This link opens a broad site, then click on IL. Support group with lots of links for families and persons with CHD Website: From Cincinnati Children’s Hospital Kawasaki Disease Multisystem disorder involving vasculitis & may progress to coronary arteries causing aneurysm formation Leading cause of acquired heart dz in US Etiology still unknown 3 phases: acute subacute convalescent Criteria for KD (must meet 5 out of 6) Box 34-10, p. 1388, Hockenberry (9th ed); Box 29-9, p. 1299 (10th ed.) fever > 5 days conjunctival infection without exudate oral changes: erythema, “strawberry tongue, fissured lips extremities changes: peripheral edema, erythema of palms and soles, peeling of hands & feet erythematous rash cervical lymphadenopathy Other manifestations Symptoms of inflammation C reactive protein level ESR Cardiac symptoms L ventricular function as seen on Echocardiogram Children do NOT generally have sx of CHF Other lab changes Anemia Leukocytosis with ‘L shift’ Kawasaki continued Tx best within first 7- 10 days. : ASA 80-100mg/kg/day initially. This is one dx that requires use of high doses of aspirin even in children. Dose is decreased to 3-5 mg/kg/day once afebrile 48-72 hrs. IVIG 2 g/kg over 8-12 hr Here is a website with some good information on the diagnosis and management of this disease: http://www.kdfoundation.org/ From the American Heart Association p. 1300 10th ed. Newburger, J. W. et al. Circulation 2004;110:2747-2771 Education of parents Teach parents common signs of Aspirin toxicity while on high doses of ASA Tinnitus Headache Dizziness Confusion Teach parents to report recurrence of fever Teach parents CPR Inform parents that final cardiac sequelae may not be known for some time. QUESTIONS