2Cardiovascular System Heart fully functioning by 8th week of gestationFetal circulation: placenta is the organ of oxygenationDuctus venosusDuctus arteriosusForamen ovaleThe birthing process begins closure of fetal shuntsHeart defects are the most common birth defectLeading cause of birth defect-related deathsThe heart is a vital organ; parents confronted with a child’s cardiac problems are overwhelmed with fear and anxiety.
3Congenital Heart Disease DescriptionA defect in the structure of the heart or in one or more of the large blood vessels that lead to and from the heartMultifactorialResult of genetic-environmental interactionsFour classificationsBased on defect’s effect on blood flowHemodynamics: study of blood circulationShunting: flow of blood through abnormal openingsDefects with increased pulmonary blood flowObstructive defectsDefects with decreased pulmonary blood flowMixed defectsThe rate of incidence of infants born with a congenital heart defect is about one per 125 to 150 deliveries.Genetic factors: History of CHD, chromosomal abnormalitiesEnvironmental factors: Alcoholism, cocaine, rubella, exposure to Coxsackie virus, diabetes mellitus, ingestion of lithium salts, Accutane, advanced maternal age
5Congenital Heart Disease (CHD) Diagnostic toolsNot all testing is necessary for each childLab tests, electrocardiogram, halter monitor, event recorder, chest radiography, echocardiogram, MRI, cardiac catherizationCardiac catherization is an invasive procedureProvides information about anatomy, cardiac pressure, oxygen saturation, cardiac functionSedation is necessaryEntry site kept straight 4-6 hours after procedure
6Congenital Heart Disease (CHD) Signs and symptomsDepend on location and type of heart defectChild may be small for age, condition may be classified as a physiologic failure to thriveExercise intolerance noticed anywhere from infancy to toddler ageClubbing of the fingersFrequent respiratory infections because of pulmonary vascular congestionSquatting position (TET spell)Polycythemia: Body compensates for hypoxemia by increasing number of RBCsWhy do some children with CHD frequently assume a squatting position?
8Congestive Heart Failure (CHF) Early symptomsLack of infant weight gainProgressive symptomsDifficulty feeding/ sucking due to air hungerPeriorbital/facial edemaVein distentionInfant irritability and fatigueHepatomegalySplenomegalyTachycardia/tachypneaDecreased urine outputIncreased work of breathingDiaphoresisMottlingCyanosisPallorCHF is not a disease, but rather symptoms caused by an underlying heart defect.
9Defects with Increased Pulmonary Blood Flow Patent ductus arteriosus (PDA)Passageway connecting pulmonary artery to aorta, avoiding fetal lungsFailure to close causes oxygenated blood to recycle through the lungsOverburdens pulmonary circulationMakes heart work harderOne of the most common cardiac anomaliesSymptomsMachine-like murmur, dyspnea, bounding pulses on exertion, failure to thrive, frequent respiratory infectionsTreatmentIndomethacin/ibuprofen in premature infants to close ductusAmplatzer PDA deviceSurgical repairSymptoms of patent ductus arteriosus may go unnoticed during infancy.PDA occurs twice as frequently in females.Excellent prognosis for corrected PDA.
10Defects with Increased Pulmonary Blood Flow Atrial septal defect (ASD)Abnormal opening between right and left atriaCommon congenital heart anomalySymptomsGenerally asymptomaticCyanosis if blood flow is reversed by heart failureLarge openings may cause failure to thriveTreatmentOpen heart surgeryPercutaneous occluding devicesASD more common in females.Both surgical and nonsurgical procedures have good results.
11Defects with Increased Pulmonary Blood Flow Ventricular septal defect (VSD)Opening between right and left ventricles75% of small VSDs close spontaneously by age 10SymptomsLoud, harsh murmurSystolic tremorModerate/large defects may present CHF symptomsTreatmentPercutaneous transcatheter closureOccluder devices
12Obstructive Defect Coarctation (tightening) of the aorta Symptoms Increased pressure proximal to the defectDecreased pressure distal to the defectHigh blood pressureCHF symptomsTreatmentBalloon angioplastySurgical intervention; anastomosisRisk of developing subacute bacterial endocarditisHigh risk of recurrence for repairs done in infancy.
13Defect with Decreased Pulmonary Blood Flow Tetralogy of FallotThe most common cyanotic heart defectFour defectsStenosis (narrowing of pulmonary artery)Hypertrophy of the right ventricleOver-riding aortaVSDSymptomsCyanosis/hypoxemia episodes with hyperpnea, irritabilityHypoxiaTreatmentOpen heart surgery to correct defectsHypoxia triggered when there is an acute demand for oxygen; i.e., during blood draw, defecation, feeding, crying.How should spells of hypoxia be handled?What is given to stop hyperpnea?
14Mixed Defect Transposition of the great arteries (TGA) Pulmonary artery leaves left ventricleAorta leaves the right ventricleOther defects (septal defects, PDA) must be present to exchange blood which sustains lifeSurvival impossible without surgerySymptomsCHF symptomsAny murmur present is caused by other defects, not TGATreatmentProstaglandin E1 (keeps PDA open)Balloon atrial septotomy (opens septal wall)Corrective surgeryPerformed within the first 2 weeks of lifeProstaglandin E1 and balloon atrial septostomy are used as palliative measures until corrective surgery.Surgery involves switching the arteries.Antibiotic prophylaxis is done for the first 6 months.
15Mixed Defect Hypoplastic left heart syndrome (HLHS) Left side of the heart is underdevelopedHypoplasia of aorta, left ventricle, mitral valveSystemic circulation provided by right side of the heart (Rt ventricle is force of circulation)Ductus arteriosus and foramen ovale must remain patent to survive with HLHSTreatmentPGE1 is administered to maintain a PDASurgery in several stagesNorwood, Glenn, FontanTransplants have been successful, but donor availability, organ rejection, infection, and immunosuppression are complicating factors.
16Congenital Heart Disease Treatment and nursing careNursing goals in the care of the newborn infant can be adapted for all children with heart defectsReduce the work of the heartImprove respirationMaintain proper nutritionPrevent infectionReduce the anxiety of the patientSupport and instruct the parentsWhy is Lanoxin the preferred oral digitalis preparation for children with CHD?The parents of the child need support and understanding over a long period.Why is cerebral thrombosis a concern?
17Congenital Heart Disease Treatment and nursing care (continued)Change child’s position frequently to prevent respiratory complicationsThreat of cerebral thrombosisChest tubes may be used after surgerySystem must be airtightDrainage containers always kept below the level of the chestAvoid unnecessarily disturbing the child; they need to conserve energyCommon medicationsDigoxin (Lanoxin)Dopamine, dobutamine, epinephrineAmrinone, milrinoneACE inhibitorsAngiotensin II receptor blockersDiureticsHow should the nurse measure pulse before medicating with Lanoxin?
18Congenital Heart Disease Treatment and nursing care (continued)Infective endocarditis (IE)High risk for children with complex cyanotic heart diseases or children who have had heart surgeryOrganisms grow on the endocardium or areas of turbulent blood flowSymptomsFever, fatigue, headache, nausea, vomitingDiagnosis and treatmentBlood cultures determine causative organismAntibiotics
19Congenital Heart Disease Home careFamily must understand medication administrationFamily must identify symptoms requiring medical attentionProvide a normal environment within child’s limitsAvoid allowing the child to gain control of the homeLimit settingIntegrate the child into family lifeExplain the possible need for frequent hospitalization to parents and childAvoid rough play for ~6 weeks.All day attendance in school may be too tiring for some conditions.
20Question 12.2Which of the following is NOT one of the four classifications of congenital heart disease?Defects with increased pulmonary blood flowCongestive heart failureDefects with decreased pulmonary blood flowMixed defectsAnswer: B
21Acute Rheumatic Fever Description Signs and symptoms Follows infection with certain strains of Group A beta-hemolytic streptococciSigns and symptomsAbdominal pain, fever, pallor, fatigue, anorexia, unexplained nosebleedsJones criteria aid in diagnosis of rheumatic feverECG is sometimes a useful diagnosticWhen does rheumatic fever typically occur?Rheumatic fever has a tendency to recur, and each attack carries the threat of further damage to the heart.Signs and symptoms of rheumatic fever:Range from mild to severeMay not occur for several weeks after a streptococcal infection
24Acute Rheumatic Fever Treatment and nursing care Elimination of the initial infection is followed by long-term chemo-prophylaxis (prevention of disease by drugs)Intramuscular penicillin G benzathine (Bicillin), given as an intramuscular injection every 28 daysAnti-inflammatory drugs are used to decrease pain and inflammation
25Acute Rheumatic Fever Home care Bed rest during the initial attack is not necessary but is recommended if carditis is presentNurse must verify that parent and child understand activity limitationsParents should provide interesting quiet activitiesLong-term chemo-prophylaxis
26Acute Rheumatic Fever Prevention The nurse is involved in prevention of rheumatic fever in the community by recognizing signs and symptoms of streptococcal infections, doing screening, and referring for treatment