Congenital Disorders 1.1

• Alteration in circulation of blood that occurs during fetal development

  Congenital Disorders
• where there is no mixing of unoxygenate and oxygenated blood

  acrocyanotically
• where unoxygenated blood mixes with oxygenated blood whether cyanosis occurs or not.

   cyanotically
• 8 – 10 per 1000 live births
• PREDISPOSING / PRECIPITATING FACTORS

  ❑ Maternal rubella and other viruses durng pregnancy

  ❑ Poor maternal nutrition

  ❑ Maternal alcoholism

  ❑ Maternal age over 40

  ❑ Maternal insulin-dependent diabetes

  ❑ Neonatal hypoxia / RDS

  ❑ Parents-siblings with heart disease

  ❑ Born with congenital anomalies

  ❑ Chromosomal defects like Down’s syndrome
• Ventricular Septal Defect (VSD)

  ❑ Acyanotic

  ❑Small or moderate openings may be asymptomatic

  ❑ Large defects

  ▪ Loud, harsh murmur best heard left sternal border radiating throughout precordium

  ▪ Right ventricular hypertrophy

  ▪ Cardiac enlargement

  ❑ Abnormal opening between right and left ventricles

  ❑ Left to right shunting due to incomplete closure of septum; pulmonary vascular resistance
• Patent Ductus Arteriousus (PDA)

  ❑ Acyanotic

  ❑ Machinery-like murmur best heard upper-left sternal border during systole and most diastole

  ❑ Thrill

  ❑ Widened pulse pressure

  ❑ History fatigue, weak cry, breathlessness, feeding difficulties

  ❑ Increased number respiratory infections

  ❑ Communication between pulmonary artery and aorta due to failure of ductus arteriosus to close after birth

  ❑ Left to right shunting; increased pulmonary vascular resistance
• Coarctation of the Aorta (CA)

  ❑ Acyanotic

  ❑ Episodes of sudden epistaxis

  ❑ Full, bounding pulses upper extremities

  ❑ Headaches

  ❑ Leg fatigue

  ❑ Elevated pressure proximal to narrowed portion of lumen of aorta

  ❑ Weak or absent pulses in lower extremities

  ❑ Systolic murmurs

  ❑ Narrowing of aortic lumen

  ❑ Left ventricle must generate higher than normal pressure to eject adequate stroke volume; reduces systolic pressure distal to coarctation
• Pulmonic Stenosis (PS)

  ❑ Acyanotic

  ❑ Systolic murmur best heard over second left intercostal space

  ❑ Thrill

  ❑ Split S2 (a finding upon auscultation of the S2 heart sound, caused when the closure of the aortic valve and the closure of the pulmonary valve are not synchronized during inspiration)

  ❑ Dyspnea if severe

  ❑ Faigue, if severe

  ❑ Obstruction of flow from right ventricles to lungs

  ❑ Increased right side of heart; right sided hypertrophy
• Aortic Stenosis (AS)

  ❑ Acyanotic

  ❑ Systolic murmur throughout precordium

  ❑ Thrill

  ❑ Fatigue / exercise intolerance

  ❑ Epigastric / anginal pain

  ❑ Dyspnea

  ❑ Obstructing flow from left ventricle to aorta

  ❑ Resistance to blood flow in left ventricle; left- sided hypertrophy; increased oxygen demands; pulmonary vascular congestion
• Transposition of Great Vessels (TGV)

  ❑ Cyanotic

  ❑ Tachypnea

  ❑ Arterial pulses full and bounding

  ❑ Murmur present only if VSD/PDA present

  ❑ Reversal of anatomic positions of aorta and pulmonary artery; aorta originates from right ventricle to pulmonary artery from left ventricle

  ❑ Incompatible with extrauterine life; venous blood enters right atrium to right ventricle to aorta and systemic circulation without oxygenation; oxygenated blood enters left atrium and returns to right atrium without supplying oxygen to blood.
• Tetralogy of Fallot (TF)

  ❑ Cyanosis appears during the first year of life

  ❑ Clubbing of fingers in older infants

  ❑ Hypoxic spells

  ❑ Squatting position following any form of exercise

  ❑ Small for age

  ❑ Harsh systolic murmur best heard at middle to upper left sternal border

  ❑ Thrill

  ❑ Four anomalies present

  ▪ Pulmonic Stenosis

  ▪ VSD

  ▪ Aorta overriding VSD

  ▪ Right Ventricular Hypertrophy

  ❑ Right to left shunting impending flow to lungs; right sided hyperthrophy, unoxygenated blood to systemic circulation to VSD overriding aorta
• DIAGNOSTICS TEST 1. Based on findings of history and physical 2. Chest X-ray 3. EKG 4. Echocardiogram 5. Angiogram 6. CBC / blood gases 7. Cardiac catheterization
• Nursing Responsibilities Pre-test

  ▪ Explain procedure to parent/child by using visual aids and doll therapy

  ▪ Schedule EKC, CBC, chest x-ray prior to test

  ▪ NPO for 6 hours prior to test

  ▪ Sedate as needed

  ▪ Accompany chid to test, if possible
• Nursing responsibilities post-test

  ▪ Check vital signs every 15 minutes until stable, then every 2-4 hours for 24 to 48 hours

  ▪ Report tachycardia /bradycardia to doctor immediately

  ▪ Check circulation in involved extremity ; temperature, color, capillary refill

  ▪ Assess operative side for bleeding

  ▪ Monitor I and O

  ▪ Pressure dressing at operative site

  ▪ Keep child relatively quiet
• COMPLICATION: congestive heart failure
• TREATMENT: Dependent on exact cardiac disorder, surgical correction often treatment of choice.
• Nursing Assessment

  1. Obtain a thorough nursing history

  2. Discuss the care plan with the health care team

  3. Measure and records height and weight, and plot on a growth chart

  4. Record vital signs and oxygen saturation

  5. Assess and record skin color, mucous membranes, extremities

  6. Assess for clubbing

  7. Assess chest wall

  8. Assess respiratory pattern

  9. Assess heart sounds

  10. Assess fluid status

  11. Assess and record the child's level of activity
• Measure vital signs


  • At a time when the infant/child is quiet
  • Choose appropriate-size blood pressure cuff
  • Check four extremity BP x 1
• Assess respiratory pattern

  • Before disturbing the child, stand back and count the respiratory rate
  • Loosen or remove clothing to directly observe chest movement
  • Assess for signs of respiratory distress: increased respiratory rate, grunting, retractions, nasal flaring
  • Auscultate for crackles, wheezing, congestion, stridor
• Assess heart sounds

  • Determine rate (bradycardia, tachycardia, or normal for age) and rhythm (regular or irregular)
• Assess fluid status


  • Daily weights
  • Strict intake and output (number of weight diapers; urine output)
• Assess the child's level of activity

  • Observe the infant while feeding
  • Observe the child at play
  • Assess and record findings relevant to the child's developmental age: age-appropriate behavior, cognitive skills, and gross and fine motor skills
• Nursing Diagnoses

  ❑ Impaired Gas Exchange related to altered pulmonary blood flow or pulmonary congestion.

  ❑ Decreased Cardiac Output related to decreased myocardial function.

  ❑ Imbalanced Nutrition: Less Than Body Requirements related to excessive energy demands required by increased cardiac workload.

  ❑ Risk for Infection related to chronic illness.

  ❑ Risk for Infection related to chronic illness.

  ❑ Fear and Anxiety related to life-threatening illness.
• Relieving Respiratory Distress

  1. Position the child in a reclining, semi-upright position.

  2. Suction oral and nasal secretions as needed.

  3. Identify target oxygen saturations and administer oxygen as prescribed.

  4. Administer prescribed medications and document response to medications (improved, no change, worsening respiratory status).

  5. May need to change oral feedings to nasogastric feeding because of increased risk of aspiration with respiratory distress.
• Improving Cardiac Output

  1. Organize nursing care and medication schedule to provide periods of uninterrupted rest

  2. Provide play or educational activities that can be done in bed with minimal exertion

  3. Maintain normothermia

  4. Administer diuretics(furosemide, spironolactone [Aldactone]) as prescribed

  5. Administer digoxin as prescribed

  6. Administer afterload-reducing medication (captoproil[capoten], enalapril[Vasotec] as prescribed
• Diuretics


  • Give the medication at the same time each day. For children do not give a dose right before bedtime
  • Monitor the effectiveness of the dose: measure and record urine output
• Digoxin


  • Check heart rate for 1min. Withhold the dose and notify the phydicisn for bradycardia (heart rate less than 90 beats/min
  • Lead II rhythmn strip may be ordered for PR interval monitoring. Prolonged PR interval indicates first-degree of heart block (dose of digoxin may be withheld)
  • Give medication at the same time each day. For infants and children, digoxin is usually divided and given twice per day
  • Monitor serum electrolytes. Increases incidence of digoxin toxicity associated with hypokalemia
• Afterload-reducing medication


  • When initiating medication for the first time: Check BP immediately before and 1hr after dose
  • Monitor for signs of hypotension: syncope (temporary loss of conciousness), light-headedness, faint pulses
  • Withhold medication and notify the physician according to ordered parameters
• Improving Oxygenation and Activity Tolerance

  1. Place pulse oximeter probe (continous monitoring or measure with v/s) on finger, earlobe, or toe.

  2. Administer oxygen as needed.

  3. Titrate amount of oxygen to reach target oxygen saturations.

  4. Administer response to oxygen therapy: increase in baseline oxygen saturations, improved work of breathing, and change in patient comfort.

  5. Explain to the child how oxygen will help. If possible, give the child the choice for mask oxygen or nasal cannula oxygen.
• Providing Adequate Nutrition for the infant

  1. Small, frequent feedings

  2. Fortified formula or breastmilk (up to 30 cal/oz)

  3. Limit oral feeding time to 15-20 mins

  4. Supplement oral feeds w/ nasogastric feedings as needed to provide weight gain (ie, continous nasogastric feedings at night w/ ad-lib by mouth feeds during the day)
• Providing Adequate Nutrition for the child

  1. Small, frequent meals

  2. High-calorie, nutritional supplements

  3. Determine child's likes and dislikes and plan meals accordingly

  4. Allow the parents to bring the child's favorite foods to the hospital
• Providing Adequate Nutrition

  1. For the infant:

  2. For the child:

  3. Report feeding intolerance: nausea, vomiting, diarrhea.

  4. Document daily weight (same time of day, same scale, same clothing).

  5. Record accurate intake and output; assess for fluid retention.

  6. Fluid restriction not usually needed for children; manage excess fluid with diuretics.
• Preventing Infection

  1. Maintain routine childhood immunization schedule

  2. Administer yearly influenza vaccine

  3. Administer RSV immunization for children younger than age 2 with complex CHD and those at risk for CHF or pulmonary hypertension

  4. Prevent exposure to communicable diseases

  5. Good hand washing

  6. Report fevers

  7. Report signs of URI: runny nose, cough, increase in nasal secretions

  8. Report signs of GI illness; diarrhea, abdominal pain, irritability
• Exception in RSV - respiratory syncitial virus (Sygnasis) and influenza
 


  Immunizations should not be given for 6 wks after cardiovascular surgery
• Reducing Fear and Anxiety

  1. Educate the patient and family.

  2. Provide the family with contact numbers: how to schedule a follow-up visit; hoe to reach a cardiologist during the work week, evenings, weekends, and holidays.
• Measures to maintain the child's health

  • Complete immunization
  • Adequate diet and rest
  • Prevention and control of infections
  • Regular medical and dental checkups
  • The child should be protected against infective endocarditis when undergoing certain dental procedures
  • Regular cardiac checkups
• Teach the family about the effect and its treatment

  1. Provide patients and families with written and verbal information regarding about CHD and medical and surgical treatment options

  2. Signs and symptoms of CHF

  3. Signs of hypercyanotic spells associated with cyanotic defects and need to place child in knee-chest position

  4. Need to prevent dehydration, which increases risk of thrombotic complications

  5. Emergency precautions related to hypercyanotic spells, pulmonary edema, cardiac arrest (if appropriate)

  6. Special home care equipment, monitors, oxygen 
• Encourage the parents and other people (teachers,peers) to treat the child in as normal a manner as possible


  • Avoid overprotection and overindulgence
  • Avoid rejections
  • Promote growth and development with modifications
  • Facilitate performance f the usual developmental tasks within the limits of the chid's physiologic state
  • Prevent adults from projecting their fears and anxiety onto the child
• Family Education and Health Maintenance

  1. Instruct the family in necessary measures to maintain the child’s health:
  2. Teach the family about the effect and its treatment.
  3. Encourage the parents and other people (teachers, peers) to treat the child in as normal a manner as possible.
  4. Initiate a community health nursing referral if indicated.
  5. Stress the need for follow-up care.
  6. Encourage the attendance in support groups for patient and families.