1. Cardiovascular Dysfunctions in Children Congenital Heart Diseases Susan K. Pryor, DNS, RN, CNE

2. Review of Cardiac Anatomy and Physiology

3. Conduction System

4. Hemodynamic of the Normal Heart Hemodynamics –Normally, pressure on R side of heart <L side –Normally, pulmonary circulatory resistance < systemic circulation –Normally, pressure in the pulmonary artery < pressure in aorta –A shunt is abnormal flow from high pressure area to lower pressure area thru an abnormal opening. Often hear a murmur as blood is flowing w/higher pressure.

5. Hemodynamic of the Normal Heart Normally, O2 sat of blood from the IVC & SVC entering R atrium is lowNormally, O2 sat of blood from the IVC & SVC entering R atrium is low Normally, blood returning to heart from pulmonary veins from lungs should be fully saturatedNormally, blood returning to heart from pulmonary veins from lungs should be fully saturated Normally, there is separate circulation of unsaturated & saturated blood.Normally, there is separate circulation of unsaturated & saturated blood. CHD results in altered hemodynamics, flow, & pressureCHD results in altered hemodynamics, flow, & pressure In CHD, the amount of mixed blood that reaches systemic circulation is in varying degrees & leads to hypoxemia & cyanosisIn CHD, the amount of mixed blood that reaches systemic circulation is in varying degrees & leads to hypoxemia & cyanosis

6. Cardiac Development Heart & components begin to develop by 4 weeks and complete by 8 weeks.Heart & components begin to develop by 4 weeks and complete by 8 weeks. Heart begins to beat on 28th day.Heart begins to beat on 28th day. At birth, at the time of the clamping of the umbilical cord and the expansion of the lungs, the hemodynamics of the fetal vascular system undergoes major changes to transition to post-natal circulation

7. Fetal Circulation vs. Pulmonary Circulation

8. Congenital Heart Disease Incidence is 6 per 1000 births per yearIncidence is 6 per 1000 births per year Over 35 defects knownOver 35 defects known A major cause of death in 1st year of lifeA major cause of death in 1st year of life Etiology unknown in most cases, may be a combination of genetic & environmental factors.Etiology unknown in most cases, may be a combination of genetic & environmental factors. Often associated with a chromosomal abnormalityOften associated with a chromosomal abnormality

9. Cardiac Assessment & Diagnostics Hx: Pre-natal and birth hx, family hx, meds, feeding patterns, weight gain, development, exercise intolerance, edema, resp problems.Hx: Pre-natal and birth hx, family hx, meds, feeding patterns, weight gain, development, exercise intolerance, edema, resp problems. Assessment: See Guidelines for Child with Cardiac Condition p. 604Assessment: See Guidelines for Child with Cardiac Condition p. 604 Symptoms may appear at birth or first days/weeksSymptoms may appear at birth or first days/weeks Diagnostics:Diagnostics: –CXR, EKG, Echo, Cardiac Cath, Holter, MRI Clinical Interventions for CHDClinical Interventions for CHD –Palliative/Corrective. Table 21-3, p.606 Cardiac Catheterization Procedures and Surgical ProceduresCardiac Catheterization Procedures and Surgical Procedures

10. CHD Classification Systems Cyanotic or acyanoticCyanotic or acyanotic Primary, secondary, or tertiaryPrimary, secondary, or tertiary Hemodynamic characteristics p. 605Hemodynamic characteristics p. 605 –Increased pulmonary blood flow –Decreased pulmonary blood flow –Obstruction to flow out of the heart –Mixed blood flow Clinical consequences: CHF, cyanosis, or bothClinical consequences: CHF, cyanosis, or both Clinical Therapy: Surgical intervention, Interventional catherization (opening narrowed passages or closing openings i.e. septal occluder p. 606)Clinical Therapy: Surgical intervention, Interventional catherization (opening narrowed passages or closing openings i.e. septal occluder p. 606) Home Care After the Cardiac CatheterizationHome Care After the Cardiac Catheterization

11. CHD Altered Hemodynamics Increased pulmonary blood flowIncreased pulmonary blood flow –CHF Decreased pulmonary blood flowDecreased pulmonary blood flow –Cyanosis Obstruction to blood flow out of ventriclesObstruction to blood flow out of ventricles –L side CHF –R side cyanosis Mixed blood flow (sat & desat blood)Mixed blood flow (sat & desat blood) –Hypoxemia & CHF & Decreased CO –Variable clinical picture

12. CHD Classification AcyanoticAcyanotic –Increased pulmonary blood flow ASD, VSD, PDAASD, VSD, PDA –Obstruction to systemic flow Coarctation of Aorta, Aortic StenosisCoarctation of Aorta, Aortic Stenosis CyanoticCyanotic –Decreased pulmonary blood flow Tetralogy of Fallot, Tricuspid Atresia, Pulmonary StenosisTetralogy of Fallot, Tricuspid Atresia, Pulmonary Stenosis –Mixed defects Transposition of Great Vessels, Total Anomalous Pulmonary Venous Return (TAPVR), Truncus Arteriosis, Hypoplastic Left Heart Syndrome (HLHS)Transposition of Great Vessels, Total Anomalous Pulmonary Venous Return (TAPVR), Truncus Arteriosis, Hypoplastic Left Heart Syndrome (HLHS)

13. Review of Congestive Heart Failure In children, CHF occurs secondary to CHDIn children, CHF occurs secondary to CHD Results in increased volume or pressure load on ventricles (Increased workload) – leads to hypertrophyResults in increased volume or pressure load on ventricles (Increased workload) – leads to hypertrophy R or L sided failureR or L sided failure –R side = Increased CVP & system venous engorgement (hepatomegaly, peripheral edema) –L side = Increased pulmonary pressure & pulmonary edema –Eventually, both sides may fail

14. CHF as a result of CHD S&S CHF in children:S&S CHF in children: –Pulmonary Venous Congestion –Systemic Venous Congestion –Impaired Cardiac Output –High Metabolic Rate

15. Treatment:Treatment: –Digoxin to improve contractility. CHECK DOSE CAREFULLY(2 RNs must ) p. 627 Adm. –ACE inhibitors/Inotropic meds used reduce afterload –Fluid mgmt, diuretics, & oxygen to decrease cardiac demand NCP for CHF pp. 626-627 NCP for CHF pp. 626-627

16. Review of Hypoxemia Hypoxemia:Hypoxemia: –arterial oxygenation (PaO2) that is < normal & can be identified by a decreased O2 sat (SaO2). Hypoxia:Hypoxia: –reduction in tissue oxygenation that results from low SaO2 & PaO2 and results in impaired cellular processes. Cyanosis:Cyanosis: –Seen in decreased SaO2. Results from the presence of deoxygenated Hgb of 5g/dl or more. Both SaO2 & amt of circ Hgb involved. Check mucous membranes, skin, nailbeds. CHD that causes hypoxemia & cyanosis result from desaturated venous blood entering systemic circulation w/o passing through the lungs.CHD that causes hypoxemia & cyanosis result from desaturated venous blood entering systemic circulation w/o passing through the lungs. –2 consequences: polycythemia and clubbing –S&S in children: fatigue w/feeding, poor weight gain, tachypnea, dyspnea, tet spells

17. CHD Defects with Increased Pulmonary Blood Flow - Atrial Septal Defect (ASD) p. 608

18. ©1999-2007, Cincinnati Children's Hospital Medical Center Catheter delivered devices may be used to close selected atrial septal defects avoiding the need for open heart surgery. 1. Secundum ASD 2. Right Atrium 3. Delivery Catheter 4. Amplatzer ASD Occluder 5. Right atrial side deployed 6. Device deployed on septum

19. CHD Defects with Increased Pulmonary Blood Flow - Ventricular Septal Defect (VSD) p. 609

20. Summary Page Ventricular Septal Defect Normal Heart VSD - Patch Repair Ventricular Septal Defect Ventricular septal defect (VSD). Oxygenated blood from the left ventricle shunts via the hole into the right ventricle. This volume load causes enlargement of both ventricles and the pulmonary artery, and exposes the right ventricle and pulmonary arteries to abnormally high pressures. Patch closure of ventricular septal defect. When a large VSD requires surgical closure a patch is typically used. Common materials for patches include Dacron, Gore-tex™, or pericardium. ©1999-2007, Cincinnati Children's Hospital Medical Center

21. CHD Defects with Increased Pulmonary Blood Flow - Patent Ductus Arteriosis (PDA) p.608

22. ©1999-2007, Cincinnati Children's Hospital Medical Center Patent ductus arteriosus shunting blood from the aorta to the pulmonary artery. PDA ligated PDA Ligated Patent Ductus Arteriosis Normal

23. CHD - Obstructive Defects Coarctation of the Aorta (COA) p.620

24. ©1999-2007, Cincinnati Children's Hospital Medical Center 1. Severe narrowing of the aorta in the region of the ductus arteriosus, just beyond the take-off of the vessels to the head and arms. 2. The coarctation segment has been resected and an oblique, end-to-end reconstruction of the aorta is performed.

25. CHD - Obstructive Defects Tetrology of Fallot (TOF) p.614

26. Blalock- Taussig Shunt and Glenn Shunt

27. Tetralogy of Fallot – Tet Spells Also known as hypercyanotic spells or paroxysmal hypoxemia spellsAlso known as hypercyanotic spells or paroxysmal hypoxemia spells Occurs with obstructive defects to pulmonary flowOccurs with obstructive defects to pulmonary flow Spasms Dec pulm flow & Inc R to L shuntingSpasms Dec pulm flow & Inc R to L shunting There is an increase in O2 requirements that the body is unable to meet.There is an increase in O2 requirements that the body is unable to meet. –Seen with feedings, crying, defecation, procedures Easily fatigued, Dyspnea on Exertion, “air hungry”, diaphoresis, LOC, polycythemiaEasily fatigued, Dyspnea on Exertion, “air hungry”, diaphoresis, LOC, polycythemia Frequent episodes can cause cerebral hypoxemia & neuro complications.Frequent episodes can cause cerebral hypoxemia & neuro complications. Risk for CVA (polycythemia) & endocarditisRisk for CVA (polycythemia) & endocarditis Can result in poor G&D & poor weight gainCan result in poor G&D & poor weight gain Treatment:Treatment: –oxygen, MSO4 or sedative, knee-chest position

28. A young child with an uncorrected or partially corrected defect that reduces pulmonary blood flow may squat (assumes a knee–chest position) to reduce systemic blood flow return to the heart.

29. CHD – Mixed Defects - Transposition of the Great Vessels (TGV) – p.621

30. Prostagladin E (Prostin) Keeps the ductus open Increases the patency pt ductus &re- established blood flow Stabilizes child for awhile –Surgery to follow Start @0.1mcg/kg/min and wean to 0.025 mcg/kg/min SE – apnea, fever, flushing –Have intubation equipment at bedside

31. CHD – Mixed Defects – Hypoplastic Left Heart Syndrome (HLHS) – p.622

32. Hypoplastic Left Heart Syndrome (HLHS) Usually fatal w/o surgical intervention (3 days – 1 month)Usually fatal w/o surgical intervention (3 days – 1 month) Surgery in 3 stages:Surgery in 3 stages: –1 st stage Norwood Procedure (1 st week) –2 nd stage Bidirectional Glenn Shunt @ 3- 8 months –3 rd stage Modified Fontan Procedure @ 18 mo-3yrs Option of heart transplant as newbornOption of heart transplant as newborn High mortality rate, palliative care is an optionHigh mortality rate, palliative care is an option

33. Nursing Care of the Child with CHD Supportive careSupportive care –Parents initially react w/shock, anxiety, fear, guilt. They are faced with child’s death. –Support family, assess their level of understanding, supply information as needed, act as pt & family advocate. –Parents may become detached from child due to procedures & being critically ill. Encourage opportunities to bond. –CHD is a chronic illness May have neurodevelopmental sequilaeMay have neurodevelopmental sequilae

34. Nursing Care of the Child with CHD – cont. 2) Education to parents/family Home care after surgery –Heart condition @ their level of understanding. Use pictures, models, or internet sources. Sometimes there are no definite answers. –S&S of complications from CHD & when to call practitioner. –Medications & nutrition –No strenuous activity or competitive sports

35. Nursing Care of the Child with CHD – cont. 3. Educate the child –4-6 yrs: knows where heart is, draws it like a valentine, knows it goes thump- thump –9-10 yrs: realize heart not shaped like valentine and that it makes you live –11 yrs & older: more conceptual knowledge. Sees heart as a pumping system and begins to understand why death occurs when heart stops.

36. Nursing Care of the Child with CHD – cont. 4. Educate for surgery –Teach about the surgical environment & equipment used and where the child will be pre/post op. –Prepare parents for what they will see. –Teach about the incisions & dressings. –Use of heart/lung machine, ETT, chest tubes –D/C planning starts on admit with multidisciplinary team approach – home care

37. Nursing Care of the Child with CHD – cont. 5.Pre/post op nursing care –Immediate post-op nurse:pt ratio 3:1 –Monitor for arrhythmias (common), pacer wires –Maintain thermoregulation, use of heat lamps or warming pads (hypothermia) –Monitor arterial & venous pressures (MAP, CVP) –Maintain airway and monitor ABG’s –Maintain patency & monitor output of chest tubes

38. Nursing Care of the Child with CHD – cont. Pre/post op nursing carePre/post op nursing care –Sedation/analgesia, many meds & drips –Meticulous I&O –Monitor volume status (initial fluid restriction), administer IVF –Monitor for complications: CHF, hypoxia, decreased cardiac output, tamponade, pneumothorax, pleural effusion, cerebral edema, infection

39. Nursing Care of the Child with CHD – cont. *** Shunting from R to L (desat blood to L side of heart) allows air in venous system to go directly to brain, resulting in air embolism. All IV lines, should have filters to prevent air. Check all tubing and syringes. NO AIR IN LINES