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  1. GENERAL OVERVIEW: Genetic abnormalities and environmental insults often lead to congenital disorders of the newborn. Successful transition to independent extrauterine life may pose a major challenge to infants compromised by anatomical or physiological disorders. Knowledge regarding the implications of the neonate's structural or metabolic problems enables the nurse to identify early signs of health problems and to plan, provide, and evaluate appropriate outcome-directed care to safeguard the status of the infant with a congenital disorder.
  2. DISORDERS AFFECTING FLUID-GAS TRANSPORT: congenital heart disease
    1. Pathophysiology—altered hemodynamics, due to persistent fetal circulation or structural abnormalities.
      1. Acyanotic defects—no mixing of blood in the systemic circulation.
        1. Patent ductus arteriosus.
        2. Atrial septal defect.
        3. Ventriclar septal defect.
        4. Coarctation of the aorta.
      2. Cyanotic defects—unoxygenated blood enters systemic circulation.
        1. Tetralogy of Fallot.
        2. Transposition of the great vessels.
    2. Etiology—unknown. Associated with maternal:
      1. Prenatal viral disease (e.g., rubella, coxsackievirus).
      2. Malnutrition; alcoholism.
      3. Diabetes (poorly controlled).
      4. Ingestion of lithium salts.
    3. Assessment:
      1. Patent ductus arteriosus (see Figure 5-5. Patent Ductus Arteriosus, Ventricular Septal Defect).
        1. Characteristic machine murmur, mid to upper left sternal border (cardiomegaly); persists throughout systole and most of diastole; associated with a "thrill."
        2. Widened pulse pressure.
        3. Bounding pulse, tachycardia, "gallop" rhythm.
      2. Atrial septal defect (see Figure 5-3. Atrial Septal Defect, Sids: What to Tell Families).
        1. Characteristic crescendo-decrescendo systolic ejection murmur.
        2. Fixed S2 splitting.
        3. Dyspnea, fatigue on normal activity.
        4. Medical diagnosis—cardiac catheterization, x-ray.
      3. Ventricular septal defect (see Figure 5-4. Ventricular Septal Defect).
        1. Loud, harsh, pansystolic murmur; heard best at left lower sternal border; radiates throughout precordium. ( Note: may be absent—due to high pulmonary vascular resistance → equalization of interventricular pressure.)
        2. Medical diagnosis—cardiac catheterization, ECG, chest x-ray.
      4. Coarctation of the aorta (see Overview of the Most Common Types of Congenital Heart Disease).
        1. Absent femoral pulse.
        2. Late systolic murmur.
        3. Decreased blood pressure in lower extremities.
        4. Medical diagnosis: x-ray.
      5. Tetralogy of Fallot ("blue" baby) (see Figure 5-6. Tetralogy of Fallot).
        1. Acute hypoxic/cyanotic episodes.
        2. Limp, sleepy, exhausted; hypotonic extended position—postepisode.
        3. Medical diagnosis—cardiac catheterization.
      6. Transposition of the great arteries(see Figure 5-7. Transposition of the Great Arteries).
        1. Cyanotic after crying or feeding.
        2. Progressive tachypnea—attempt to compensate for decreased PaO2, metabolic acidosis.
        3. Heart sounds vary; consistent with defect.
        4. Signs of CHF.
        5. Medical diagnosis—cardiac catheterization, x-ray, ECG.
    4. Analysis/nursing diagnosis:
      1. Fluid volume excess related to persistent fetal circulation, structural abnormalities.
      2. Impaired gas exchange related to abnormal circulation, secondary to pathology.
      3. Altered nutrition, less than body requirements, related to exhaustion, dyspnea.
    5. Nursing care plan/implementation:
      1. Goal: minimize cardiac workload.
        1. Minimize crying—snuggle; pacifier—to meet psychological needs.
        2. Keep clean and dry.
      2. Goal: maintain thermal stability—to reduce body need for oxygen.
      3. Goal: prevent infection.
        1. Strict aseptic technique; standard precautions.
        2. Hand washing.
      4. Goal: parental emotional support.
        1. Encourage verbalization of anxiety, fears, concerns.
        2. Keep informed of status.
      5. Goal: health teaching—explain, discuss:
        1. Diagnostic procedures.
        2. Treatment procedures.
        3. Basic care modalities.
      6. Goal: promote bonding. Encourage parents to participate in infant care, as possible.
      7. Medical-surgical management: surgical intervention/repair of congenital cardiac abnormality.
    6. Evaluation/outcome criteria:
      1. Experiences no respiratory distress in immediate postnatal period.
      2. Completes transfer to high-risk center without incident, if applicable.
      3. Surgical intervention successful, where applicable.
  3. DISORDERS AFFECTING FLUID-GAS TRANSPORT: hemolytic disease of the newborn
    1. Rh incompatibility
      1. Pathophysiology (see Health Promotion and Maintenance).
      2. Etiology (see Rh isoimmunization, Health Promotion and Maintenance).
      3. Assessment:
        1. Prenatal—maternal Rh titers, amniocentesis.
        2. Intrapartum—amniotic fluid color:
          1. Straw-colored: mild disease.
          2. Golden: severe fetal disease.
        3. Direct Coombs' blood test; positive test demonstrates Rh antibodies in fetal blood.
      4. Nursing care plan/implementation—exchange transfusion:
        1. Goal: health teaching.
          1. Explain purpose and process to parents:
            1. Removes anti-Rh antibodies and fetal cells that are coated with antibodies.
            2. Reduces bilirubin levels—indicated when 20 mg/dL in term neonate and 15 mg/dL in preterm.
            3. Corrects anemia—supplies RBCs that will not be destroyed by maternal antibodies.
            4. Rh-negative type O blood elicits no reaction; maximum exchange is 500 mL; duration of exchange: 45 to 60 minutes.
        2. Goal: minimize transfusion hazards.
          1. Warm blood to room temperature, since cold blood may precipitate cardiac arrest.
          2. Use only fresh blood—to reduce possibility of hypocalcemia, tetany, convulsions.
          3. pillImageGive calcium gluconate, as ordered, after each 100 mL of transfusion.
        3. Goal: prepare for transfusion procedure. Ready necessary equipment—monitor, resuscitation equipment, radiant heater, light.
        4. Goal: assist with exchange transfusion.
          1. Continuous monitoring of vital signs; record baseline, and every 15 minutes during procedure.
          2. Record: time, amount of blood withdrawn; time and amount injected; medications given.
          3. Observe for: dyspnea, listlessness, bleeding from transfusion site, cyanosis, cardiovascular irregularity or arrest; coolness of lower extremities.
        5. Goal: posttransfusion care.
          1. Assessment:
            1. Observe for: dyspnea, cyanosis, cardiac arrest or irregularities, jaundice, hypoglycemia; frequent vital signs.
            2. Signs of sepsis—fever, tachycardia, dyspnea, chills, tremors.
          2. Nursing care plan/implementation:
            1. Maintain thermal stability—to reduce physiological stress, possibility of metabolic acidosis.
            2. Give oxygen—to relieve cyanosis.
            3. Keep cord moist—to facilitate repeat transfusion, if necessary.
            4. Maintain nutrition/hydration—feed per schedule.
      5. Evaluation/outcome criteria:
        1. Infant's hemolytic process ceases; bilirubin level drops.
        2. Infant makes successful transition to extrauterine life.
        3. Infant experiences no complications of therapeutic regimen.
        4. Infant shows evidence of bonding.
    2. ABO incompatibility
      1. Pathophysiology—fetal blood carrying antigens A/B enters maternal type O bloodstream → antibody formation → antibodies cross placenta → hemolyze fetal RBCs. Note: less severe than Rh reaction.
      2. Etiology:
        1. Type O mother carries anti-A and anti-B antibodies.
        2. Even first pregnancy is jeopardized if fetal blood enters maternal system.
        3. Reaction possible if fetus is type A, type B, or type AB and mother is type O.
      3. Assessment:
        1. Jaundice within first 24 hours.
        2. Rising bilirubin levels.
        3. Enlarged liver and spleen.
      4. Nursing care plan/implementation: Goal: reduce hazard to newborn.
        1. Prepare for exchange transfusion with O-negative blood.
        2. Phototherapy may be ordered if bilirubin is 10 mg/dL, and anemia is mild or absent.
        3. Close monitoring of status.
        4. Supportive care.
      5. Evaluation/outcome criteria:
        1. Infant responds to medical/nursing regimen.
        2. Infant's assessment findings within normal limits.
    3. Hyperbilirubinemia
      1. Pathophysiology—bilirubin, a breakdown product of hemolyzed RBCs, appears at increased levels; exceeds 13 to 15 mg/dL. Bilirubin is safe when bound with albumin and conjugated by user for body excretion; danger is when unconjugated and deposits in CNS.
        1. WARNING: There is no "safe" serum bilirubin level; kernicterus is a function of the bilirubin level and neonatal age and condition; poor fluid-and-caloric balance subjects the infant (especially the preterm infant) to kernicterus at low serum bilirubin levels.
        2. Kernicterus—high bilirubin levels result in deposition of yellow pigment in basal ganglia of brain → irreversible retardation.
      2. Etiology:
        1. Rh or ABO incompatibility, during first 48 hours.
        2. Resolution of an enclosed hemorrhage (e.g., cephalohematoma).
        3. Infection.
        4. Drug induced—vitamin K injection, maternal ingestion of sulfisoxazole (Gantrisin).
        5. Bile duct blockage.
        6. Albumin-binding capacity is exceeded.
        7. "Breastfeeding jaundice" (e.g., pregnanediol in milk). Breastfeeding is not dangerous and not a cause of physiological jaundice.
        8. Dehydration.
        9. Immature liver (interferes with conjugation).
      3. Assessment:
        1. Jaundice noted after blanching skin to suppress hemoglobin color; noted in sclera or mucosa in dark-skinned neonates; make sure light is adequate; spreads from head down, with increasing severity.
        2. Pallor.
        3. Concentrated, dark urine.
        4. Blood level determination—hemoglobin or indirect bilirubin (unconjugated, unbound bilirubin deposits in CNS).
        5. Kernicterus—similar to intracranial hemorrhage.
          1. Poor feeding or sucking.
          2. Regurgitation, vomiting.
          3. High-pitched cry.
          4. Temperature instability.
          5. Hypertonicity/hypotonicity.
          6. Progressive lethargy; diminished Moro reflex.
          7. Respiratory distress.
          8. Cerebral palsy, mental retardation.
          9. Death.
      4. Analysis/nursing diagnosis:
        1. Fluid volume (RBC) deficit related to hemolysis secondary to blood incompatibility.
        2. High risk for injury (brain damage) related to kernicterus.
        3. Altered thought processes (mental retardation) related to brain damage secondary to kernicterus.
        4. Knowledge deficit (parental) related to infant condition.
      5. Nursing care plan/implementation:
        1. Medical management:
          1. Prenatal—amniocentesis.
          2. Postnatal—exchange transfusion, phototherapy.
        2. Goal: assist bilirubin conjugation through phototherapy.
          1. Cover closed eyelids while under light to protect eyes. (If Biliblanket is used, no need to cover eyes.) Remove eye pads when not under light (feeding, cuddling, during parental visits).
          2. Expose as much skin as possible—to maximize exposure of circulating blood to light. Remove for only brief periods.
          3. infoImageChange position q1h—to maximize exposure of circulating blood to light.
          4. Note: any loose green stools as bile is cleared through gut; watch for skin breakdown on buttocks.
          5. Monitor temperature—to identify hyperthermia. (Not necessary if using Biliblanket.)
          6. pillImagePush fluids (to 25% more than average) between feedings—to counteract dehydration. Breast milk has natural laxative effects that help clear bile.
        3. Goal: health teaching. Explain, discuss phototherapy, bilirubin levels, implications.
        4. Goal: emotional support.
          1. Encourage verbalization of anxiety, fears, concerns.
          2. Encourage contact with infant.
          3. Reassure, as possible.
      6. Evaluation/outcome criteria:
        1. Infant's hemolytic process ceases; bilirubin level drops.
        2. Infant makes successful transition to extrauterine life.
        3. Infant experiences no complications of therapeutic regimen.
        4. Infant shows evidence of effective bonding.