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Care of the Pediatric Patient with Cardiovascular Problems Elizabeth Allen RN, MSN • Upon completion the learner will • Define the 4 classifications of congenital heart disease • Compare and contrast examples of each among the classifications • Recall symptoms and treatments of CHF in pediatric patients • State the most common pediatric cardiac dysrhythmia • Differentiate between congenital and acquired heart disease • Identify hematologic disorders affecting the pediatric population Learning Objectives Fetal Circulation • Video of Fetal Circulation • http://youtu.be/uwswhoKfkmM • Video of Heart Embryology • http://youtu.be/5DIUk9IXUaI Fetal Circulation • Apex • 4th ICS left of the MCL • Apical pulse • Auscultate • Murmurs • S3 is normal in children • Pulses • Infant • Brachial, Femoral • > 1 year • Radial, Carotid • Stroke Volume-Cardiac Output • Immature fluid balance compensatory mechanisms Pediatric Anatomic Differences • • • • • Develop in the first 8 weeks of gestation One of the most common birth defects A leading cause of infant mortality in the first year of life 85% survivability Resource: • http://www.nhlbi.nih.gov/health/health-topics/topics/chd/ Congenital Heart Defects • • • • Increased Pulmonary Blood Flow Decreased Pulmonary Blood Flow Obstructive Systemic Blood Flow Mixed Defects Classification of Congenital Heart Defects Classification Types of Defects Increased Pulmonary Blood Flow PDA, Atrial Septal Defect, Ventricular Septal Defect, Atrioventricular Canal Decreased Pulmonary Blood Flow Pulmonary Stenosis, Tetrology of Fallot, Pulmonary or Tricuspid Atresia, Transposition of Great Arteries Obstructive Systemic Circulation Coarctation of Aorta, Aortic Stenosis, Hypoplastic Left Ventricle, Mitral Stenosis, Interrupted Aortic Arch Mixed Defects Transposition of Great Arteries, Total Anomalous Pulmonary Venous Return, Truncus Arteriosus, Double Outlet Right Ventricle Classification • Diagnostic or Interventional • Similar process to adults • Use femoral veins/arteries exclusively • Generally do not leave sheaths in place post cath • Pedal pulse and bleeding assessments frequent and very important! Cardiac Catherization • Communication that allows blood flow from L side (Systemic Circulation) to R side (Pulmonary Circulation) • Pressures on L side of heart greater than R side • Corrective surgery performed early in infancy to prevent irreversible pulmonary vascular disease (pulmonary hypertension) Increased Pulmonary Blood Flow • Maintain PDA with Prostaglandin E • Pharmaceutical PDA closure with Indomethacin • Cath Lab closure of PDA • Surgical closure of PDA- PDA ligation • Patent Ductus Arteriosus (PDA) Increased Pulmonary Blood Flow Increased Pulmonary Blood Flow • Increased Blood Flow to Pulmonary Circulation • Pathophysiology • Decreased cardiac output • Increased pulmonary blood flow • Leads to sympathetic nervous response • Increased heart rate • Increased vasoconstriction • Renin-angiotension release (holding Na and H20) • Resource: • http://www.cincinnatichildrens.org/health/c/chf/ Congestive Heart Failure • Symptoms • Infant • Tires easily, sweats with feeds • Loses weight, FTT • Tachypnea, grunting, retractions, Crackles • Liver palpable below R costal margin • Tachycardia, 3rd heart sound, fatigue • Symptoms • Older Child • Fatigue, exercise intolerance • Crackles, tachypnea • Abdominal pain • Skin mottling, pallor Congestive Heart Failure • Symptoms: • Respiratory- tachypnea, grunting, retractions, hypoxia, crackles • Abdominal- liver palpable below R costal margin, retractions • Cardiac- diaphoresis, tachycardia, third heart sound, edema, JVD, cardiomegaly, fatigue • Failure to Thrive Congestive Heart Failure Treatment • Pharmacological: • Digoxin, diuretics, Beta blocker, ACE inhibitor • Oxygen • High calorie tube feeding • Monitor weight- FTT and fluid retention • Monitor urine output- 1 cc/kg/hr. • Therapies to promote development • Rest and avoid excessive crying Congestive Heart Failure • Defects restrict flow of blood from R side of heart to the lungs • Usually results in hypoxemia and cyanosis • Cyanosis or hypoxia that does NOT respond as expected to oxygen • Blood flow obstruction to lungs PLUS an ASD or VSD will cause a R to L shunt and unoxygenated blood in systemic circulation Decreased Pulmonary Blood Flow • Tetralogy of Fallot (TOF) • Tetralogy =4 • 4 Defects • • • • Pulmonary Stenosis VSD Overriding Aorta R Ventricular Hypertrophy Decreased Pulmonary Blood Flow • Clinical Manifestations • Hypoxia after PDA closure • • • • Polycythemia Clubbing Systolic Murmur Poor growth • May have signs of CHF Tetralogy of Fallot • Pre Surgical Treatment- Avoid or Reduce Hypercyanotic Episodes! • • • • • • Monitor O2 sats, HR, respiratory effort Oxygen Avoid excessive crying, agitation Knee to chest in infant, Treat pain High calorie feeds- may be NGT Therapies to promote development • Surgical Correction • BT Shunt palliative in infancy to increased pulmonary blood flow • Complete surgical correction of VSD and Pulmonary Artery Tetralogy of Fallot Figure 21–6 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. • Often appear similar to those of Decreased Pulmonary Blood Flow • Dependent on Mixed Oxygenated and Un-oxygenated blood for survival Mixed Defects • Clinical Manifestations: • • • • • • • Chronic hypoxemia Fatigue Clubbing Exertional dyspnea Increased metabolic demand Developmental delays Possible CHF Mixed Defects • Obstruct Blood Flow from heart to systemic circulation • Results in elevated L ventricular pressure and LOW cardiac output • Symptoms manifest in infants as PDA closes Obstructive Defects • Some children have no symptoms • Reduced flow to body post coarct • • • • • • • Heart Failure Lower Extremity cyanosis BP difference- lower in legs vs. arms Radial and brachial pulses bounding Renal failure and necrotizing enterocolitis in infants Poor feeding, Failure to Thrive CHF symptoms including dyspnea (back up into lungs) • Coarctation of the Aorta Obstructive Defects • Coarctation of the Aorta • Treatment • Infant with severe obstruction or L ventricular dysfunctionPGE infusion • Balloon catherization • Surgical Correction • Often just follow up post correction, no other problems Obstructive Defects • Supraventricular Tachycardia • Most common dysrhythmia in children • Electrophysiology disturbanceoverriding SA Node • Supra- meaning the conduction wave originates in the atria Dysrhythmias • Kawasaki Disease • Rheumatic Fever Acquired Cardiac Disease • • • • • • Kawasaki Disease Leading cause acquired heart disease in US 80% of cases <5 years, 50% < 2 years Affects Asians, Pacific Islanders more often Unknown cause- thought to be infectious, not contagious Acute fevers, systemic vascular inflammation • Inflammation of small and midsize arteries, including coronary arteries • May cause aneurysms Acquired Cardiac Disease • Kawasaki Disease • 3 Stages- Symptoms • Acute: irritability, high fever, red eyes and throat, rash, feet and hand swelling • Subacute: cracked lips, desquamation of fingers and toes, joint pain, thrombocytosis and cardiac disease • Convalescent: normal, maybe lingering inflammation Acquired Cardiac Disease • Kawasaki Disease • Treatment • Fever- antipyretic • IV Immunglobulin (IVIG)- 1 dose • Monitor: cardiac, CBC, bleeding • Aspirin • When to stop the aspirin? Acquired Cardiac Disease • Rheumatic Fever • Inflammation of connective tissue from immune response to some Group A beta-hemolytic streptococci • May cause long term disease of heart valves, brain, skin and joints • 1-3 weeks post strep infection Acquired Cardiac Disease • Rheumatic Fever Symptoms • Fever • Joint pain/swelling • Murmur (carditis) or chest pain • Rash (not on face & hands • Subcutaneous nodules over tendons, bony prominences • Rheumatic Fever Treatment • Antibiotics • Aspirin- antipyretic, joint pain • Corticosteroids as needed • Monitor cardiac for CHF symptoms Acquired Cardiac Disease • Cardiomyopathy • • • • • Disorder affects chamber size, wall thickness and contractility Most common Dilated Cardiomyopathy Cause: genetic, acquired, secondary to myocardial disorders Usually presents in CHF Treatment: • • • • • • • Digoxin ACE Inhibitor Diuretics Antiarrhythmic Anticoagulants Beta blocker Heart transplant Acquired Cardiac Disease • Iron Deficiency Anemia • Sickle Cell Anemia • Hemophilia Hematological Disorders • Iron Deficiency Anemia • Who has it? • < 2years old/adolescent females. • Pathophysiology • Secondary to blood loss, malabsorption, or poor nutritional intake • Symptoms • Labs • Treatment • Dietary, supplements Hematological Disorders • Sickle Cell Anemia • Red blood cells “Sickle” under stress • Resource • http://www.nhlbi.nih.gov/health/health-topics/topics/sca/ • Pathophysiology • Genetic • Autosomal recessive disorder • African Americans, some Mediterranean decent Partial or complete replacement of the amino acid glutamic acid by the amino acid valine Hematological Disorders • Sickle Cell Symptoms • Pain • Peripheral clotting, ischemia, obstruction • Sickle Cell Treatment • Treat Pain!! • Treat underlying cause (ex. dehydration, infection) • Hydration • Oxygen • Priapism over 4 hours is a medical emergency • Splenectomy common Hematological Disorders • Hemophilia • • • • • • Inherited, sex-linked: almost always affects boys Mild-to-severe bleeding after injury Internal bleeding, bruising, joint bleeds Ineffective Clotting Cascade- missing coagulation Factors 85% have Hemophilia A Also Hemophilia B and von Willenbrand’s disease Hematological Disorders • Hemophilia Treatment • Monitor for bleeding- especially joint bleeding • Provide Factor VIII (Hemophilia A) • Recombinant Factor VIII- not plasma derived • Cryoprecipitate in emergencies if Factor not immediately available Hematological Disorders