1. CARDIOVASCULAR SYSTEM: CONGENITAL ABNORMALITIES JAMIE DAVIS, RN, BSN, CEN IN LIEU OF MSNED

2. Pulmonary veins from lungs Right Atrium Left Atrium Left Ventricle Right Ventricle Inferior vena cava Superior vena cava Tricuspid valve Pulmonary valve Pulmonary veins from lungs Mitral valve Aortic valve Aorta Pulmonary artery to lungs Ventricular septum Pulmonary artery to lungs

3. Ductus Venosus Foramen ovale Ductus arteriosis Ductus venosus : oxygenated blood coming from MAMA. After birth turns into ligamentum teres. Foramen ovale: closes after the cord is cut; pressure LA = forced closure of foramen ovale Ductus arteriosis: turns into ligementum arteriosus; closes from increased oxygenation *transition from fetal to pulmonary circulation occurs within a few hours after birth.

4. CARDIAC ASSESSMENT GUIDELINES  HISTORY:  Prenatal and birth history  Family history  Medications  Family history  Feeding patterns  Weight gain  Development  Exercise tolerance  Edema  Respiratory problems  HISTORY:  Prenatal and birth history  Family history  Medications  Family history  Feeding patterns  Weight gain  Development  Exercise tolerance  Edema  Respiratory problems ASSESSMENT: Pulse Rate, rhythm, quality; compare pulse sites BP – is it expected Respirations Color Chest Inspect: heaving; palpate of pulsations or vibrations Heart auscultation S1/S2, murmurs, loud or muffled sounds, extra sounds Fluid status Activity and behavior Activity intolerance, tire with feeding, diaphoresis, behavior changes General Growth pattern

5. CONGENITAL HEART DISEASE (CHD)  > 35 defects known  Unknown etiology in most cases; linked to genetics or environmental factors  Often associated with chromosomal abnormalities (ID @ 18-20 weeks gestation via echo)  1 in 123-170 live births  Bad news: Major cause of death in 1 st year of life  Good news: 85% of newborns are expected to survive to adulthood.  4 types of CHD  Acyanotic  Defects that cause Increased pulmonary blood flow  Obstructive defects  Cyanotic  Decreased pulmonary blood flow  Mixed defects

6. REVIEW OF CONGESTIVE HEART FAILURE (CHF)  Commonly, in children, CHF is secondary to CHD  Results in increased volume or pressure load on ventricles and lead to hypertrophy.  Can see R or L sided failure, eventually both sides will fail S/S: diaphoresis, poor perfusion, mottling, pallor, crackles, tachypnea, tachycardia, dyspnea, retractions, edema, cardiomegaly, tire with feeding, etc. TX: digoxin to improve contractility; ACE inhibitors to reduce afterload; Diuretics to decrease fluid volume; and O2 to decrease cardiac demand RN: sit pt up to decrease workload on heart, encourage rest, decrease stimuli, control room temp, monitor lung sounds and FVE, I &O

7. ACYANOTIC CHD LEFT TO RIGHT SHUNT Types: Increased pulmonary blood flow  Atrial Septal Defect (ASD)  Ventricular Septal Defect (VSD)  Patent Ductus Arteriosus (PDA) Obstruction to systemic flow  Coarctation of the Aorta (COA)  Aortic Stenosis (AS) Non-cyanotic defects:  They don’t get blue  May have trouble gaining wt  Can have CHF and pulmonary overcirculation; sometimes (to make it more confusing) they can be asymptomatic  May have exercise or exertion difficulties

8. an opening in the atrial septum permitting free communication of blood between the atria Often not see until one year of age Blood flow is pushed into RA as well as LV. Depending on the size of the defect: my be asymptomatic or have CHF s/s + murmur and atrial arrhythmias TX: TX: surgical patch closure of large defects if it does not spontaneously close by 4 yrs of age; closer occurs with occlude via heart cath

9. An abnormal opening in the ventricular septum, which allows free communication between the R & L ventricles Mod to severe show no s/s until 6-8 wks of age Blood is returning to the right ventricle instead of though the aorta, causing increase volume  increase workload  R CHF Symptoms of CHF, pulmonary edema, + murmur TX: can close spontaneously, but if a lg hole exist a PA banding, complete repair with patch

10. Persistence of the normal fetal vessel that joins the PA to the Aorta Normally closes in the 1 st wk of life. Flow to aorta  to body, but some returns through PDA back to L side of heart; can flood lungs increase workload to L side of heart Symptoms of CHF or asymptomatic, pulmonary edema, + murmur with widen pulse pressure & bounding pulses TX: Indomethacin or surgically ligated via thoracotomy

11. Obstructive defect Narrowing of the aortic arch – think a rubber band is around the aorta making the blood flow harder to pass Leads to LVH, increase workload to L side of heart Causes increase pressure to proximally to head and UE, and decrease pressure distally to body and LE  increased BP and bounding pulses in arms and weak or absent femoral pulses, cool LE TX: balloon aortoplasty or resection with anastomosis of aorta

12. CYANOTIC CHD RIGHT TO LEFT SHUNT BF GO TO SYSTEMIC CIRCULATION W/O BEING OXYGENATED Types: Decreased pulmonary blood flow  Tetrology of Fallot (TOF) Mixed defects  Transposition of the Great Vessels (TGV)  Hypoplastic Left Heart Syndrome (HLHS ) Non-cyanotic defects:  They get blue, really really blue when they cry or exert themselves  Can grow okay…sometimes  If not repaired, at great risk for:  Endocarditis  Strokes  Immune problems

13. Includes 4 defects: VSD, RVH (right ventricular hypertrophy), pulmonary stenosis, and overriding aorta Pulmonary stenosis – decreases BF to lung and L side of heart Tet spells S/S: hypoxic and cyanotic, very LOUD murmurs, Tet spells Older kids: clubbing, and squatting on playground, boot shaped heart on CXR TX: palliative sx – BT shunt that provided blood flow to PA from subclavian artery; complete repair – 1 st yr, closure of VSD & resection of stenosis

14. TET SPELLS TETRALOGY OF FALLOT HYPERCYANOTIC SPELLS OR PAROXYSMAL HYPOXEMIA SPELLS OCCURS WITH OBSTRUCTIVE DEFECTS TO PULMONARY FLOW; INCREASE ON O2 DEMANDS THAT THE BODY CANT MEET SPASMS DECREASE PULMONARY BF AND INCREASE R TO L SHUNTING COMMONLY SEEN WITH FEEDINGS, CRYING, OR DEFECATING EASILY FATIGUE, “AIR HUNGRY,” DIAPHORESIS, POLYCYTHEMIA, LOC CAN RESULT IN POOR G&D OR POOR WT GAIN TX: OXYGEN, SEDATIVES, KNEE-TO-CHEST POSITION

15. Mixed defect Pulmonary artery and aorta switch places; two separate circulations are coming out of the heart No communication btw pulmonary and circulatory system Septal defect MUST BE PRESENT to allow saturation & unsaturated blood to mix (PDA/PFO). Infant dependent for survival S/S: CHF and cyanosis; CXR – egg heart on a string TX: prostaglandin E will allow mixing of blood to maintain sats of 75%; balloon atrial septostomy with heart cath; arterial switch procedure at 1 st week

16. Mixed defect Underdeveloped Left side of heart: sm LV & aortic atresia BF from LA thru PFO to RA  RV  PA thru PDA to aorta PFO & PDA allow mixing of saturated & desaturated blood S/S: CHF and cyanosis TX: Prostin; fatal w/o surgery; can have heart transplants Surgeries: three stages 1.Norwood procedure (1 wk) 2.Bidirectional glenn shunt (3-8 mo) 3.Modified Fontan procedure (18 mo – 3 yrs)

17. NURSING CARE FOR CHILDREN WITH CHD  Supportive care:  Support family, assess level of understanding, supply info, act as family advocate  Encourage family and patient bonding. Parents tend to detach due to procedures and state of illness  CHD is chronic illness – think neurodevelopmental issues  Education family:  Teach s/s of complications associated with CHD  Medication and nutritional status  No strenuous activities or competitive sports  Educate child:  4-6 yr: knows where heart is and that it “thumps;” draws heart like valentine  9-10 yr: realize that heart is not shaped like valentine and is vital for life  11yr & >: conceptual knowledge. Understand heart is a pumping system and death occurs when it stops.

18. NURSING CARE FOR SURGICAL INTERVENTION  Education:  Teach about surgical environment & equipment used  Prepare for what they will see: lines, drains, CT, et  Proved information about home care post op  no live vaccines 6 mo post op if received blood, reg vaccines are okay  Pre/Post op:  Monitor for arrhythmias  Monitor arterial and venous pressure (MAP/CVP)  Maintain airway & monitor ABC’s  I & O and FVE/FVD  Monitor for complications i.e. CHF, hypoxia, decreased CO, tamponade, pneumothorax, pleural effusion, cerebral edema, or infection  **R to L shunting allows air in venous system to go directly to the brain resulting in air embolism. This means NO AIR LINES

19. ACQUIRED HEART DISEASES INFECTIVE ENDOCARDITIS INFECTIVE ENDOCARDITIS – INFECTION OF THE ENDOCARDIAL, CAN BE R/T SX INTERVENTION S/S: FEVER, FATIGUE, JOINT & MUSCLE ACHES, WT. LOSS, H/A, DIAPHORESIS, DX WITH BC TX: IV ABX LIKE VANC, CIPRO, ROCEPHIN, ETC; SX FOR HEART VALVE REPLACEMENT IF NECESSARY RHEUMATIC FEVER RHEUMATIC FEVER – INFLAMMATORY DISORDER OF THE CONNECTIVE TISSUE R/T GROUP A BETA- HEMOLYTIC STREP S/S: OCCURS 1-3 WK AFTER UNTX STREP; DEVELOPMENT OF NEW MURMUR; CP R/T PERICARDIAL INFLAMMATION; 2 OR MORE LG JOINTS BECOME INFLAMED, NONPRURITIC RASH TO TRUNK (NEVER ON FACE AND HANDS) TX: ABX LIKE PCN, SULFA, ERYTHROMYCIN; ANTIINFLAMMATORIES

20. ACQUIRED HEART DISEASES CONT. KAWASAKI DISEASE KAWASAKI DISEASE– ACUTE FEBRILE, SYSTEMIC VASCULAR INFLAMMATORY DISORDER THAT EFFECTS THE ARTERIES S/S: 1-2 WKS - FEVER, RED THROAT, SWOLLEN HANDS & FEET, MACULOPAPULAR/ERYTHEMA RASH ON TRUNK AND PERINEAL AREA, ENLARGED LYMPH NODES, HEPATIC DYSFUNCTION 2-4WKS – CRACKED LIPS, DESQUAMATION OF SKIN ON TIPS OF FINGERS AND TOES, JOINT PAIN, CARDIAC DISEASE & THROMBOCYTOSIS 6-8 WKS – CHILD WILL APPEAR NORMAL, BUT HAVE LINGERING S/S OF INFLAMMATION TX: IV INFUSION OF IVIG, ASA FOR FEVER AND INFLAMMATION, MONITOR FOR CARDIAC DISEASE X SEVERAL MONTHS

21. References American Heart Association. (2015). Congenital heart defects. Retrieved from http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/Congenital-Heart- Defects_UCM_001090_SubHomePage.jsp http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/Congenital-Heart- Defects_UCM_001090_SubHomePage.jsp Ball, J., Bindler, R., & Cowen, K. (2015). Principles of pediatric nursing: Caring for children. (6 th ed.). Hoboken, NJ: Pearson Meyers, M. Lecture notes: Cardiovascular dysfunctions in children.