• Atrial Septal Defect
  • An inherited condition where there is nonclosure of the foramen ovale at birth, resulting in congenital heart disease. Usually asymptomatic until the third or fourth decades of life.

    Symptoms include exertional shortness of breath, fatigue and palpitations.

• Ventricular Septal Defect
  • A congenital heart defect where an abnormal opening exists in the ventricular septum. Blood flows from the higher pressure left ventricle (through the defect) into the right ventricle.

• Patent ductus arteriosus (PDA) is a persistence of the fetal connection (ductus arteriosus) between the aorta and pulmonary artery after birth, resulting in a left-to-right shunt. Symptoms may include failure to thrive, poor feeding, tachycardia, and tachypnea. A continuous machine-like murmur in the upper left sternal border is common.

• https://health.google.com/health/ref/Coarctation+of+the+aorta

• "Tet" spells
  • calm the infant
  • Knee-chest position
  • 100% oxygen by mask
  • Morphine SQ or IV

• Four defects
  • Ventricular Septal Defect (VSD)
  • Pulmonic Stenosis
  • Overriding Aorta
  • Right Ventricular Hypertrophy
• http://wwwnemours.org/hospital/de/aidhc/service/cardiac-center/defect/tof/flash.html

• Cyanosis
• Murmur
• Poor growth
• Hypercyanotic or "Tet spells"
• Clubbing of fingers
• Squatting

• Monitor growth pattern
• Observe for CHF
• Cyanosis and Tet spells
• Pre and post op cares

• An imperforated tricuspid valve
  • results in no communication between the right atrium and the right ventricle
• Foramen Ovalve (or ASD) allows blood flow to left side with enlarded mitral valve and left ventricle
• Usually have a hypoplastic or absent right ventricle
  • Often have pulmonic stenosis
• Third most common cyanotic heart defect
• http://www.nemours.org/hospital/de/aidhc/service/cardiac-center/defect/tricuspid-atresia/flash.html

• Central cyanosis
• Tachypnea
• Murmur
• Clubbing
• Polycthemia (too many RBC)
• Poor Growth
• Hypercyanotic spells

• Prostaglandin
  • Maintains the neonate PDA dependent

• Procedure used to increase ASD size (balloon septostomy) in Tricuspid Atresia

• Monitor growth pattern
• Observe for CHF
• Dehydration
• Cyanosis & hypercyanotic spells
• Pre/post op cares

• Pulmonary Artery Leaves the left ventricle and the aorta leaves the right ventricle
  • results in two parellel, separate circuits
• Septal defects or PDA REQUIRED for mixing to occur
• Those with no VSD are dependant on their PDA for life.
• http://www.nemours.org/hospital/di/aidhc/service/cardiac-center/defect/tga/flash.html

• Varying degrees of cyanosis
• CHF
• Cardiomegaly
• Clubbing
• Poor growth
• CXR shows characteristic "egg on it's side" shape

• Continuous prostaglandin drip (PGE1)
• or Rashkind balloon atrial septostomy

• Left side of heart is underdeveloped with small ventricle and aorta
• Ductus arteriousus allows for systemic circulation from the main PA
• Most of pulmonary venous return is shunted to right atrium

http://www.nemours.org/hospital/de/aidhc/service/cardiac-center/defect/hlhs/flash.html

• CHF
• Tachypnea
• Poor feeding
• Cyanosis
  • Severe of PDA closes
    • Low cardiac output
    • Hypoxia
    • Acidosis
    • Shock

• Prostaglandin to maintain PDA
• Surgical
• Heart Transplant

• ASD, VSD, and abnormal AV valves
• 30% are kids w/Down syndrome

• Failure of truncus arteriosus to divide into the aorta and pulmonary artery.

http://www.nemours.org/hospital/de/aidhc/service/cardiac-center/defect/truncus-arteriosus/flash.html

Pulmonary HTN

and

Treatment

• Elevated mean PAP>25 at rest or >30 with exercise
• Initially reversible although becomes irreversibe with prolonged vasoconstriction and vascular obstruction.
• Increased PAP causes vessel wall thickening and severe irreversible vasocontriction and vasular obstruction.  Less blood is pumped to lungs, so child becomes cyanotic.
• Treatment:
  • Surgical intervention if caught early
  • otherwise prevention of CHF

• Fever lasting 1-2 weeks
• Nonpurulent conjuctivitis
• Erythematous/fissured lips
• Strawberry tongue, erythema/edema of palms/soles
• Generalized desquamation
  • which is a rash that peels the skin, usually starts under the nail beds
• Aneurysms form early in 2nd stage of illness

• Elevated:
  • WBC
  • Platelet
  • Sed Rate
  • Increased T4/T8 ratio
• Negative Cultures
  • Throat
  • Urine
  • CSF

• Rest
• Fluids
• Monitor Temp
• Cardiac status
  • observe for MI, CHG
• Administer IV gamma globulin
• Aspirin for fever
• Teach:
  • NO LIVE immunizations for 3 months after IVIG therapy

• Damaged valves can become further damaged with repeated infections
• Streptococcal prophylaxis is lifelong if there is actual valve involvement
• Intramuscular penicillin, administered montly, is the drug of choice
• Alternatives include oral penicillin twice daily or oral sulfadiazine once a day.