Congenital Heart Defects

Ventricular Septal defect

• Can be small or large 

• Assessment Data: 

  • HF 

  • Feeding and Failure to Thrive 

• Additional Diagnostics

  • Cath, ECHO, Chest X Ray, 12 Lead, Blood Gas, 

• Cardiac Cath: Why? 

  • Evaluate the extent of flow being pumped to the pulmonary circulation and to evaluate hemodynamic pressures/stability 

• Nursing interventions:

  • Informed Consent 

  • NPO 

  • VS + Pre Op and Post Op Assessment 

  • Check Pedal Pulses and Mark with a Marker 

  • Allergies

  • Labs: Renal, CBC, CMP 

• Same considerations post cath for adult (i.e. BP frequently, lie flat, Tele, Immobilization, Assessments, etc) 

  • Child will need to keep extremities straight for 4-6 hours with no movement and will be positioned on their back 

  • Pulse and CMST checks above and below cath sites 

  • I&Os; contrast can have a diuretic effect → think about hydration 

  • Monitor tele for arrhythmias 

  • Assessments, VS 

• Client Education:

  • Keep site dry for 3 days 

  • Temperature and Elevated Temp call 

  • Rest for 3 days 

Mitral Regurgitation

• Doesn’t close properly causing backflow 

• The mitral valve separates the left atrium and left ventricle (think tri before you buy (bicuspid = mitral)

• Oxygenated blood is returned to the left atrium from the lungs and pumped into the left ventricle where the oxygenated blood is then pumped into systemic circulation 

• Surgical Planning: 

  • Repair: Balloon Valvuloplasty Most Common 

  • Replace: valve replacement - requires open heart surgery 

    • Mechanical Valve: last 20-30 years, increased risk for thromboembolism; require lifelong anticoagulants, clicking noise drives patients crazy 

    • Biologic Valves: made of pig/cow, last 8-20 years, no long-term anticoagulant unless there is a history of atrial fibrillation (3 months anticoagulated)

  • All valve replacements will require prophylaxis antibiotics 

• Post-Valve Replacement Interventions: 

  • Assessments: Respiratory, Circulatory, Neuro 

  • Intensive care for 24-72 hours 

  • Transfer to telemetry stepdown unit 

• Client Teaching: 

  • Coumadin (Warfarin) requires ongoing blood test ptINR

  • Desired INR when on warfarin 2.0-3.0 

  • Biological Valves require only 3 months of anticoagulants 

  • Prophylactic Antibiotics treatment before and after the following: 

    • Dental procedures, GI surgeries, Cesarean deliveries, Surgeries for implanting a device, such as a pacemaker or defibrillator

Patent Ductus Arteriosus (PDA)

• Second most common congenital heart defects 

• Initially a PDA may produce no clinical effects, but with age may precipitate pulmonary vascular disease, typically causing symptoms by age 40 

• Right ventricular pressure increases in an attempt to overcome the increase in pulmonary pressure. The prolonged increase iN RV pressure creates right ventricular hypertrophy 

• Risk factors: 

  • Non-modifiable: 

    • Genetics, Age, Gender 

  • Modifiable: 

    • German measles during pregnancy 

    • Uncontrolled diabetes 

    • Drug/Alcohol Use 

    • Presence of other congenital heart defects 

• In a child with a large left to right shunt, oxygen will decrease pulmonary vascular resistance while increasing the systemic vascular resistance, which leads to increased left to right shunting. Monitor the child carefully and use oxygen only as prescribed 

  • Over oxygenation can lead to blindness due to increase pressure 

• Physical Exam: 

  • Tachycardia

  • Tachypnea

  • Bounding Pulses 

  • Widened Pulse Pressures (Systolic and diastolic pressures far apart) 

  • Diastolic BP low

  • Harsh, Continuous, Machine Like Murmur 

    • Loudest under the left clavicle at the first and second intercostal spaces 

• Fixes: 

  • Pharmacological Management: 

    • Indomethacin 

      • NSAID

      • Given in 3 doses, 12 hours apart 

  • Closed by coil embolization or device via cardiac catheterization 

  • May also be surgically ligated 

Cyanotic congenital heart defects

** usually start with T

• Decreased Pulmonary Flow: 

  • Tetralogy of Fallot: 4 defects 

    • Pulmonary Stenosis, VSD, Overriding Aorta, Right Ventricular Hypertrophy 

  • Tricuspid Atresia: 3 tricuspid valve 

• Mixed Disorders: 

  • Truncus Arteriosus: Vessels join to make 1

  • Hypoplastic Left heart Syndrome 

  • Transposition of the Great VesselsL 2 major vessels switched  

• Nursing Priority: 

  • Infants and children less than 2 years with CHD should receive a monthly RSV vaccination during RSV season 

• Nursing Assessments: 

  • Cyanosis, Cooling, Clamminess of skin 

  • RR and work of breathing 

  • SpO2 via pulse oximeter 

  • Retractions, SOB, noisy breathing 

  • Changes in skin color with Positional Changes (fetal position/Knee bending) 

  • Loud, Harsh Murmur on auscultation of the heart (TOF)

  • Adventitious sounds on auscultation of the lungs 

  • Clubbing of fingers 

  • Irritability 

  • Labs for polycythemia (when hemoglobin and hematocrit are raised to try to compensate)

  • Prepare infant and family for ECHO, EKG, and cardiac cath 

• Nursing Assessment continued: 

  • Note color changes with activity, crying and feeding 

    • Hypercyanosis develops suddenly → increased cyanosis, Hypoxia, dyspnea, agitation 

    • Usually 60-80% at baseline 

    • 4mL/kg/hr is okay for IV fluids (dextrose or saline) 

  • As the child gets older they may compensate for hypercyanotic spells 

    • Specific postures such as bending the knees, assuming the fetal position, squatting 

    • These positions improve pulmonary flow by increasing systemic vascular resistance 

**Tet spell = hypercyanotic 

Acyanotic Congential defects

• Obstructive Disorders: 

  • Coarctation of the aorta 

  • Aortic Stenosis 

  • Pulmonary Stenosis 

• Increased Pulmonary Blood flow (Left to right shunts) 

  • Patent ductus arteriosus (PDA) 

  • Atrial Septal Defect (ASD) 

  • Ventricular Septal Defect (VSD)

Tetralogy of Fallot

TOF Interventions: 

• Avoiding activity in extreme weather 

• Providing small feeding volumes → high calorie no more than 20 min

• The need for prophylaxis antibiotics for dental care 

• Oxygen 100% in the face mask 

• Report any temperature elevation greater than 100.4F 

• For tet spells position fetally 

• Good hydration → bc of polycythemia causing thick blood

• Gavage feeding: promotes less metabolic demand for feeding 

• RSV Vaccinations

TOF Fixes: 

• 4-12 months for corrective surgical fixes 

• Initially palliation with systemic to pulmonary anastomosis: 

  • Blalock-Taussig Shunt: an end to side anastomosis of the subclavian artery and the pulmonary artery 

    • Avoid BP measurements and venipunctures in the affected arm, pulse will not be palpable in that arm because of the use of the subclavian artery for the shunt 

    • Monitor for ventricular arrhythmias following a corrective repair 

  • Waterston Shunt: an anastomosis of the descending aorta and the pulmonary artery 

• Definitive correction involves patch closure of the VSD and repair of the pulmonary valve and the right ventricular outflow tract