STUDY GUIDE on Cardiovascular Dysfunction in Children Three important structures in the feto-placental circulation: Ductus venosus supply the

he fetal liver Foramen ovale between the 2 atria Ductus arteriosus between the pulmonary artery and aorta Cardiovascular Disorders in Children Congenital Heart Diseases Defects with Increased Pulmonary Blood Flow 1. Atrial Septal Defect (ASD) - abnormal opening between the atria S/Sx: - Harsh systolic murmur - Dyspnea on mild exertion - Echocardiography enlarged right side of the heart and pulmonary circulation Therapeutic Management - ASD 2 closure with a device (Amplatzer septal occluder) - Surgical (between 1 & 3 years of age) - pericardial patch or Dacron patch 2. Ventricular Septal Defect (VSD) abnormal opening between the ventricles S/Sx Surgical treatment - easy fatigue - Cardiac catheterization - slowing of growth pattern - Cardiopulmonary bypass - suture or placement of Dacron patch - ECG right ventricular and pulmonary artery hypertrophy 3. Atrioventricular Canal Defect (AVC) - aka Endocardial cushion defect o Incomplete fusion of endocardial cushion or in the septum of the heart at the junction of the atria and the ventricles S/Sx: Surgical Treatment - Moderate to severe CHF - Patch closure and reconstruction of AV valve tissue - Characteristic murmur - Post operative care - observe for jaundice - Cyanosis that increases with crying 4. Patent Ductus Arteriosus (PDA) - failure of the ductus arteriosus to close within the first weeks of life S/Sx - Machinery-like murmur - Low diastolic pressure - Widened pulse pressure - ECG normal - Bounding pulses Medical Management - INDOMETHACIN administration prostaglandin inhibitor o Watch out for: bleeding, reduced glomerular filtration, diminished GI and cerebral blood flow Surgical Management - Insertion of Dacron-coated stainless steel coils - Ductal ligation thru left thoracotomy or vidoe-assisted thoracoscopic surgery Defects with Obstruction to Blood Flow 1. Pulmonic Stenosis - narrowing at the entrance to the pulmonary artery S/Sx - ECG right ventricular hypertrophy - Cyanosis - Chest X-Ray Cardiomegaly Nonsurgical Management

Page |2 - Balloon angioplasty - treatment of choice Surgical Management - pulmonary valvotomy

2. Aortic Stenosis - narrowing or stricture of the aortic valve S/Sx - Faint pulse, hypotension, tachycardia and inability to suck for long periods - Pain similar to angina when child is active - Dizziness when standing for long periods - ECG left ventricular hypertrophy Nonsurgical treatment - Stabilization with beta blocker or calcium channel blocker before procedure - Balloon valvuloplasty treatment of choice Surgical treatment - aortic valvotomy 3. Coarctation of the aorta - localized narrowing near the insertion of the ductus arteriosus S/Sx: - Bounding pulses in the arms - Weak or absent femoral pulses - Cool lower extremities Surgical Management - end-to-end anastomosis (treatment of choice for children younger than 6 months) - Digoxin and diuretics before surgery Defects with decreased Pulmonary Blood Flow 1. Tetralogy of Fallot consists of 4 defects The classic form includes four defects: - Ventricular septal defect (VSD) - Pulmonic stenosis - Overriding of the aorta - Right ventricular hypertrophy

S/Sx: - Blue spells or tet spells - Anoxic spells crying or feeding - Polycythemia - Squatting or a knee-chest position when resting

Therapeutic Management - Blalock-Taussig procedure temporary procedure (creation of ductus arteriosus) (-) palpable pulse in the right arm after the procedure - Brock procedure - full repair - Management for hypoxic episodes Oxygen administration, knee-chest position, morphine sulfate 2. Tricuspid Atresia - tricuspid valve fails to develop (completely closed) S/Sx - Chronic hypoxemia with clubbing - Cyanosis - Tachycardia Therapeutic Management - Continuous infusion of prostaglandin - Dyspnea - Fontan procedure (Glenn shunt baffle) Defects with mixed Blood flow 1. Transposition of the Great arteries the aorta rises from the right ventricle instead of the left, and the pulmonary artery arises from the left ventricle instead of the right S/Sx - Cyanotic at birth - Cardiomegaly a few weeks after birth Therapeutic Management - Prostaglandin administration - Rashkind procedure (balloon atrial septostomy) to create artificial ASD

Surgical correction - arterial switch procedure

2. Total Anomalous Pulmonary Venous Return Therapeutic Management - Continuous PGE Iv infusion - Surgical management reimplanting the pulmonary veins into the left atrium 3. Truncus Arteriosus Therapeutic Management - Restructuring the common trunk to create separate vessels - Use of homografts - One or more conduit replacement will be needed in childhood 4. Hypoplastic Left Heart Syndrome - underdevelopment of the left side of the heart, resulting in a hypoplastic left ventricle and aortic atresia S/Sx - Mild to moderate cyanosis - Signs of congestive heart failure Therapeutic Management - PGE infusion ensure adequate systemic blood flow Surgical treatment - Heart transplantation Nursing Care of the child in cardiac surgery CARDIAC SURGERY - Correction of Congenital defects through surgery - Surgery of great vessels in the immediate area surrounding the heart - Open-heart surgery relatively blood-free operative site o Heart-lung machine - Closed-heart surgery o Ligation of a PDA or COA Preoperative Nursing Care - Determine childs level of understanding - Correct misunderstanding - Orient child to OR, RR, ICU and equipment - Practice post-op methods - Include parents in teaching sessions - Establish pre-op baseline data for vital signs, activity/sleep patterns, I&O Nursing Care of the child with Congenital Heart Disease - Help the family manage the illness at home - Prepare for invasive procedures - Provide post operative care - Observe V/S and arterial and venous pressure

Post operative Nursing Care - Prevent injury or complications - Promote gas exchange - Monitor I&O - Provide nutrition as ordered - Provide alternative means of communication if mech vent is used - Allow activity as tolerated Maintain respiratory status Monitor fluids Plan for progressive activity Observe for complications

Acquired Cardiovascular Disorders 1. Congestive Heart Failure (CHF) - inability of the heart to pump an adequate amount of blood to the systemic circulation - May be a clinical consequence of CHD - Tachycardia first sign of CHF

Signs and symptoms of CHF Signs of Left-sided heart failure - Orthopnea - Rales - Bloody sputum on coughing (broken lung capillaries) - Cyanotic

Signs of Right-sided heart failure - Hepatomegaly - Anorexia - Dilated neck veins - Lower extremity edema (late sign)

NURSING MANAGEMENT - Reduce cardiac workload by reaching energy requirements - Remove and prevent further accumulation of fluid - Improve tissue oxygenation - Administer digitalis as ordered - Oxygen administration 2. Bacterial Endocarditis - also called Infective Endocarditis or Subacute bacterial endocarditis o Inflammation and infection of the endocardium or valves of the heart Causative agents: - Streptococcus viridans most common - Staphylococcus aureaus - Fungi Candida albicans Signs and Symptoms of Bacterial Endocarditis Low-grade, intermittent fever Pale Malaise Myalgia, arthralgia

Headache, diaphoresis Weight loss Significant murmurs Splenomegaly

Other signs that result from embolus formation - Splinter hemorrhages under the nails - Osler nodes - Janeway spots - Petechiae on the oral mucous membrane Diagnostic Evaluation of BE - Duke criteria guideline for the Diagnosis of Infective Endocarditis - ECG changes - Echocardiography Therapeutic Management of BE - High-dose antibiotic administration - Antifungal agents administration - Surgical intervention PREVENTION - Administration of prophylactic antibiotic therapy - 1 hour before procedures Nursing Care for Bacterial Endocarditis - Advise the familys regular dentist of existing cardiac problems in the child - Maintain highest level of oral health

Laboratory studies Anemia Leukocytosis Hematuria or proteinuria

Stress to parents that any unexplained fever, weight loss, or change in behavior must be brought to practitioners attention - Observation during antibiotic administration 3. Rheumatic Fever - occurs after pharyngitis caused by a beta-hemolytic streptococci o A self-limited illness that involve the joints, skin, brain, serous surfaces and heart o Cardiac valve damage - complication of RF Signs and Symptoms of RF Aschoff bodies Carditis involving muscles of the heart may lead to permanent damage Polyarthritis Erythema marginatum

Subcutaneous nodules Chorea (St. Vitus dance or Sydenham chorea) Arthralgia Fever

Diagnostic Evaluation for RF - Antistreptolysin O (ASLO) titer measure concentration of antibodies formed in the blood against streptolysin O o Normal 0 to 120 Todd units o Over 333 Todd units recent strep infection Therapeutic Management for RF - Reduce cardiac workload - Antibiotic administration - Penicillin or other macrolides and cephalosporins

Salicylates inflammation Digitalis Diuretics

4. Kawasaki Disease - also called Mucocutaneous Lymph Node Syndrome o Vasculitis principal finding formation of aneurysm and myocardial infarction Signs and Symptoms of KD (Acute phase) Abrupt onset of high fever Reddened conjunctivae of the eyes without discharge Red, cracked lips strawberry tongue

Cervical lymph nodes are enlarged Rash perineal area Edema on hands and feet Erythema on palms and soles with peeling

Therapeutic Management for KD - High-dose IVIG - Salicylate therapy - Anticoagulatory therapy (warfarin administration) aneurysms Nursing Care for KD - Monitor cardiac status - Monitor temperature - Record I and O and daily weight - Extra fluid; Monitor IV fluid

Application of cool cloths and unscented lotions Mouth care, lubricating ointment on lips Clear liquids and soft foods Place on quiet environment

Cardiac catheterization - A procedure in which a small radiopaque catheter is passed through a major vein in the arm, leg or neck into the heart to secure blood samples or inject dye, helps to evaluate cardiac function - Ambulatory or 1-day surgery using conscious sedation Nursing Responsibilities

Check for recent chest radiograph, ECG , electrolyte levels and blood Record pedal pulses Measure height and weight Do not draw blood specimens from projected site before the procedure Keep child NPO for 2 to 4 hours beforehand Provide anticipatory guidance o It will not hurt o Momentary speeding of the heart o Lights turned off during the procedure o Dye may have a stinging sensation

Nursing Responsibilities - Assess pressure dressing on the site - Keep child flat on bed for 2 to 3 hours - Assess and monitor vital signs - BP is 10-15% lower - Watch out for adverse reactions o Apnea o Sternal retractions o Dyspnea