Pediatric Nursing Unit Seven Child with Cardiovascular Disorders

Introduction: Cardiovascular disorders in children are commonly caused by congenital anomalies. These anomalies result in hemodynamic changes in circulation, which eventually affect not only the heart and blood vessels but other organs as well.

Introduction: There are two main types of heart diseases in childhood: Congenital heart diseases and malformation of great vessels. Inflammatory heart diseases. There are many factors associated with development of congenital heart defects. They include genetic and environmental factors such as: Maternal infections during pregnancy. Maternal use of drugs. Complications associated with pregnancy and the birthing process.

Focused Health History: Family history of defects / early cardiac disease / siblings with defects. Maternal history of stillborns or miscarriages. Congenital anomalies / genetic anomalies / fetal alcohol syndrome / Down Syndrome and Turner Syndrome. Maternal exposure to rubella. Heart murmur. Tires while eating. Low weight for height.

Focused Health History: Sweats while eating (diaphoretic). Cyanosis, worsens with feeding or activity level. Irritable weak cry. In the older child additional symptoms may include: Chest pain. Decreased activity level. Syncope. Slight of build.

Focused Physical Assessment: General appearance. Integumentary system. Face, nose, and oral cavity. Thorax and lung. Cardiovascular system.

Focused Physical Assessment: Heart Sounds: The atrioventricular valves: (separate atria from ventricle) Tricuspid valve is located between right atrium and right ventricle. Mitral (bicuspid) valve is located between left atrium and left ventricle. Closing of atrioventricular valves cause S1.

Focused Physical Assessment: Heart Sounds: The semilunar valves: (separate the ventricles fropm the vascular system) The pulmonary valve: separates the right ventricle from the trunk of pulmonary arteries. The aortic valve: separates the left ventricle from the aorta. Closing of the semilunar valves causes S2.

Focused Physical Assessment: Heart Sounds: The time between S1 and S2 is ventricular systole, and the time between S2 and S1 is ventricular diastole. Heart Murmurs: These sounds are produced by blood passing through a defective valve, great vessel, or other heart structure. Murmurs are classified by: intensity, location, radiation, timing, and quality.

Focused Physical Assessment: Pulses: Weaker pulses or lower blood pressure in the lower extremities may indicate coarctation of the aorta (COA). Bounding pulses can indicate a patent ductus arteriosus (PDA) or aortic insufficiency. Vital Signs: Heart rate: tachycardia in the absence of fever, crying, or stress may indicate cardiac pathology. Tachypnea, even with rest, chest retractions indicate respiratory distress, possibly resulting from congestive heart failure

Focused Physical Assessment: Knee-chest Position: Child with a cyanotic heart defect squats (assumes a knee-chest position) to relieve cyanotic spells. Sometimes called “tet” spells.

Focused Physical Assessment: First Breath: Pulmonary alveoli open up. Pressure in pulmonary tissues decreases. Blood from the right heart rushes to fill the alveolar capillaries. Pressure in right side of heart decreases. Pressure in left side of heart increases. Pressure increases in aorta.

Diagnostic Test: Chest x-ray: Electrocardiogram ECG or EKG: Define silhouette of the heart. Heart size, shape, pulmonary markings, and cardiomegaly. Electrocardiogram ECG or EKG: Define electrical activity of the heart.

Diagnostic Test:

Diagnostic Test: Echo-cardiogram: Visualize anatomic structures.

Diagnostic Test: Cardiac Catheterization: An invasive test to diagnose or treat cardiac defects through: Visualizes heart and vessels. Measures oxygen saturation of chambers. Measures intra-cardiac pressures. Determines muscle function and pumping action of the heart.

Diagnostic Test: Cardiac Catheterization: Toxicity to Dye: Watch for signs of toxicity to the dye used during the procedure: Increased temperature. Urticaria. Wheezing. Edema. Dyspnea. Headache.

Diagnostic Test: Cardiac Catheterization: Pre-cardiac Catheterization: Assess vital signs with blood pressure. Hemoglobin and hematocrit. Pedal pulses. NPO. Hold digoxin. IV if child is polycythemic.

Diagnostic Test: Cardiac Catheterization: Post-cardiac Catheterization: Vital sign, with apical pulse, and blood pressure q 15 minutes for first hour. Apical pulse for 1 minute to check for bradycardia or dysrhythmias. Assess pulses below the cath site. Record quality and symmetry of pulses. Assess temperature and color of affected extremity. Check dressing for bleeding or hematoma formation.  

Diagnostic Test: Cardiac Catheterization: Home Care Instructions: Keep dressing in place for 24 hours. Keep site dry and clean. Observe site for redness, swelling, drainage, or bleeding. Check temperature. Avoid strenuous exercise. Acetaminophen for pain. Keep follow-up appointment. Pre-procedure medications as ordered.

Left to Right Shunt: Pressures on the left side of the heart are normally higher than the pressures in the right side of the heart. If there is an abnormal opening in the septum between the right and left sides, blood flows from left to the right.

Left to Right Shunt: Clinical Manifestations: The infant is not cyanotic. Tachycardia due to pushing increased blood volume. Cardiomegaly due to increased workload of the heart. Dyspnea and pulmonary edema due to the lungs receiving blood under high pressure from the right ventricle. Increased number of respiratory infections due to blood pooling in the the lungs promoting bacterial growth.

Right to Left Shunts: Occurs when pressure in the right side of the heart is greater than the left side of the heart. Resistance of the lungs in abnormally high. Pulmonary artery is restricted. Deoxygenated blood from the right side shunts to the left side. Hole in septum + obstructive lesion = Deoxygenated blood from the right side of the heart shunts to the left side of the heart and out into the body.

Left to Right Shunt: Clinical Manifestations: The infant is not cyanotic. Tachycardia due to pushing increased blood volume. Cardiomegaly due to increased workload of the heart. Dyspnea and pulmonary edema due to the lungs receiving blood under high pressure from the right ventricle. Increased number of respiratory infections due to blood pooling in the the lungs promoting bacterial growth.

Left to Right Shunt: Clinical Manifestations: Hypoxemia = the result of decreased tissue oxygenation. Polycythemia = increased red blood cell production due to the body’s attempt to compensate for the hypoxemia. Increase viscosity of the blood = heart has to pump harder.

Left to Right Shunt: Potential Complications: Thrombus formation due to sluggish circulation. Brain abscess or stroke due to the un-oxygenated blood bypassing the filtering system of the lungs.

Heart Failure: Major manifestation of cardiac disease. Under 1 year of age due to congenital anomaly. Over 1 year with no congenital anomaly may be due to acquired heart disease.

Heart Failure: Clinical Manifestations of HF: Systemic Venous Congestion: Weight gain, hepatomegaly, edema, jugular vein distension. Pulmonary Venous Congestion: Tachypnea, dyspnea, cough, wheezes: Compensatory Response: Tachycardia, cardiomegaly, diaphoretic, fatigue, failure to grow.

Heart Failure: Digoxin Therapy: Digoxin increases the force of the myocardial contraction. Take an apical pulse with a stethoscope for 1 full minute before every dose of digoxin. If bradycardia is detected. < 100 beats / min for infant and toddler. < 80 beats in the older child. < 60 beats in the adolescent.

Heart Failure: Digoxin Therapy: Signs of Digoxin Toxicity: Bradycardia. Arrhythmia. Nausea, vomiting, anorexia. Dizziness, headache. Weakness and fatigue.

Heart Failure: Interventions: Fluid restriction. Diuretics – Lasix (potassium wasting) or Aldactone (potassium sparing). Bed rest. Oxygen. Small frequent feedings – soft nipple with supplemental NG for adequate calorie intake. Pulse oximeter. Sedatives if needed.

Heart Failure: Feeding: Small frequent feedings. Soft nipple to easy energy needed to suck. 24 calorie formula for added calories. NG feed if not taking in adequate calories to gain weight

Patent Ductus Arteriosus: (PDA) The ductus arteriosus is a connection between the pulmonary artery (low pressure) and aorta (high pressure). The ductus arteriosus is necessary to the fetal circulation and failure to close after birth causes oxygenated blood from the aorta to be pumped back into the pulmonary artery where the blood is under low pressure.

Patent Ductus Arteriosus: (PDA) Ductus normally closes within hours of birth. Incidence 10%. One of the most common benign defects. High risk for pulmonary hypertension. Diagnosis by: Chest x-ray – enlarged heart and dilated pulmonary artery. Echo-cardiogram – show the opening between pulmonary artery and aorta.

Patent Ductus Arteriosus: (PDA) Treatment: Indomethocin: Given po – constricts the muscle in the wall of the PDA and promotes closure. Cardiac Catheterization: Coil is placed in the open duct and acts like a plug. Closed heart surgery: Small incision made between ribs on left hand side and PDA is ligated or tied and cut.

Atrial Septal Defect: (ASD) This cardiac abnormality is characterized by an abnormal opening between the right and left atria, which allows communication and shunting of blood. Blood in left atrium flows into right atrium.

Atrial Septal Defect: (ASD) Children with this cardiac abnormality may be asymptomatic. 10% of defects. Pulmonary hypertension. Reduced blood volume in systemic circulation. If left untreated may lead to pulmonary hypertension, congestive heart failure or stroke as an adult.

Atrial Septal Defect: (ASD) Diagnosis: Heart murmur: May be heard in the pulmonary valve area because the heart is forcing an unusually large amount of blood through a normal sized valve. Echocardiogram: Is the primary method used to diagnose the defect – it can show the hole and its size and any enlargement of the right atrium and ventricle in response to the extra work they are doing.

Atrial Septal Defect: (ASD) Treatment: Surgical closure of the atrial septal defect. After closure in childhood the heart size will return to normal over a period of four to six months. No restrictions to physical activity post closure.

Ventricular Septal Defect: VSD Ventricular septal defect is a condition wherein there is a communication between the right and left ventricles, Opening in the ventricular septum. 30% of defects. Left-to-right shunt. Right ventricular hypertrophy. Deficient systemic blood flow.

Ventricular Septal Defect: VSD Small holes generally are asymptomatic. Medium to moderate holes will cause problems when the pressure in the right side of the heart decreases and blood will start to flow to the path of least resistance (from the left ventricle through the VSD to the right ventricle and into the lungs). This will generally lead to CHF.

Ventricular Septal Defect: VSD Diagnosis: Heart murmur: Clinical pearl a louder murmur may indicate a smaller hole due to the force that is needed for the blood to get through the hole. Electrocardiogram: To see if there is a strain on the heart. Chest x-ray: Size of heart. Echocardiogram: Shows size of the hole and size of heart chambers.

Ventricular Septal Defect: VSD Treatment: CHF: diuretics of help get rid of extra fluid in the lungs. Digoxin if additional force needed to squeeze the heart. FTT or failure to grow may need higher calorie concentration. Will need prophylactic antibiotics before dental procedures if defect is not repaired.

Ventricular Septal Defect: VSD Treatment: Surgical Repair: Over a period of years the vessels in the lungs will develop thicker walls – the pressure in the lungs will increase and pulmonary vascular disease. If pressure in the lungs becomes too high the un-oxygenated blood with cross over to the left side of the heart and un-oxygenated blood with enter the circulatory system. If the large VSD is repaired these changes will not occur.

Coarctation of Aorta: (COA) Congenital narrowing of the descending aorta. 7 % of defects. 80% have aortic-valve anomalies. Difference in BP in arms and legs (severe obstruction)

Coarctation of Aorta: (COA) Diagnosis: In 50% the narrowing is not severe enough to cause symptoms in the first days of life. When the PDA closes, a higher resistance develops and heart failure can develop. Pulses in the groin and leg will be diminished. Echocardiogram will show the defect in the aorta.

Coarctation of Aorta: (COA) Treatment: Prostaglandin may given to keep the PDA open to reduce the pressure changes. The most common repair is resection of the narrowed area with re-anastomosis of the two ends. Surgical complications – kidney damage due to clamping off of blood flow during surgery. High blood pressure post surgery – may need to be on antihypertensives. Antibiotic prophylactic need due to possible aortic valve abnormalities.

Tetralogy of Fallot (TOF): 6% of defects. Most common cardiac malformation responsible for cyanosis in a child over 1 year. Four Components: VSD. Pulmonary stenosis: Narrowing of pulmonary valve. Overriding of the aorta: Aortic valve is enlarged and appears to arise from both the left and right ventricles instead of the left ventricle. Hypertrophy of right ventricle: Thickening of the muscular walls because of the right ventricle pumping at high pressure.

Tetralogy of Fallot (TOF): Clinical Manifestations: Dependent on degree of right ventricular outflow obstruction. Right-to-left shunt. Clubbing of digits> “tet” spells - treated by flexing knees forward and upward> Severe irritability due to low oxygen levels.

Tetralogy of Fallot (TOF): Diagnosis: Cyanosis, Oxygen will have little effect on the cyanosis. Loud heart murmur. Echocardiogram: Demonstrates the four defects characteristic of tetralogy.

Tetralogy of Fallot (TOF): Treatment: If oxygen levels are extremely low prostaglandins may be administered IV to keep the PDA open. Complete repair is done when the infant is about 6 months of age. Correction includes: Closure of the VSD with dacron patch. The narrowed pulmonary valve is enlarged. Coronary arteries will be repaired. Hypertrophy of right heart should remodel within a few months when pressure in right side is reduced.

Tetralogy of Fallot (TOF): Long Term Outcomes: Leaky pulmonary valve that can lead to pulmonary insufficiency. Arrhythmias after surgery. Heart block – occasionally a pacemaker is necessary. Periodic echocardiogram and exercise stress test or Holter evaluation.

Aortic Stenosis: 6% of defects. Aortic stenosis is a narrowing of the aortic valve or the aortic outflow tract. Normally, the aortic valve has three cusps, but in aortic stenosis there may be only two.

Aortic Stenosis: 30% incidence of sudden death. Causes obstruction to blood flow between the left ventricle and aorta. Most common form is obstruction of the valve itself. When the aortic valve does not open properly the left ventricle must work harder to eject blood into the aorta. Left ventricular muscle becomes hypertrophied.

Aortic Stenosis: Diagnosis: Heart murmur or AS is a turbulent noise caused by ejection of blood through the obstructed valve. Electrocardiogram is usually normal. Echocardiogram will show the obstruction and rule out other heart anomalies. Exercise stress test – provides information on impact of the stenosis on heart function.

Aortic Stenosis: Treatment: Cardiac catheterization: Balloon dilation of the narrowed valve. Surgical valvotomy: If the closed procedure does not work – often done when patient is older when severe calcium deposits further obstruct the valve. Recurrent valve obstruction is a complication and if valve replacement is done too early the child may outgrow the valve. Antibiotic prophylaxis especially if valve replacement.

Pulmonary stenosis: Pulmonary stenosis is a narrowing of the pulmonary outflow tract. Because of the narrowing, the right ventricle becomes hypertrophy and eventually it causes right atrium hypertrophy.

Pulmonary stenosis: Clinical manifestation: Presence of murmur, the child may be cyanotic, and may progress to exhibit the symptoms of left ventricular failure.

Hypoplastic Left Heart: One of the most complex defects seen in the newborn and the most challenging of all the congenital defects. All the structures on the left side of the heart are severely underdeveloped. Mitral and aortic valves are either completely closed or are very small – left ventricle is tiny – aorta is small and often only a few millimeters in diameter

Hypoplastic Left Heart: Life threatening shock develops when the ductus arteriosis closes. Low oxygen saturations – will not increase with oxygen administration. Pulses will be weak in all extremities. Plan to deliver infant in a hospital capable of providing the aggressive treatment needed.

Hypoplastic Left Heart: Treatment: Three staged procedure to reconfigure the cardiovascular system: Norwood – right ventricle becomes the systemic ventricle pumping blood to the body. Glenn done at 3-6 months. Fontan done at 2 -3 years of age. Long Term Complications: Easily tiring when participating in sports or other exercises. Formation of blood clots – heparin or Coumadin use. Heart arrhythmias – pace maker. Cardiac failure.

Transposition of the great vessels: In this anomaly, the aorta arises from the right ventricle instead of the left ventricle, and the pulmonary artery from the left ventricle. Unless surgical treatment can be given, death usually takes place within a few weeks.

Bacterial Endocarditis: Infection of endocardial surface of the heart. History of CHD, Kawasaki Disease, Rheumatic Fever, or prosthetic valves are more susceptible to infection. Prophylactic antibiotics with dental care, throat, intestinal, urinary or vaginal infections or surgery.

Kawasaki Disease: Clinical Manifestations: Acute-self limiting disease. Generalized vasculitis. Peak incidence 6 months to 2 years. More common in males and Japanese. Clinical Manifestations: High fever. Conjunctivitis – no drainage. Strawberry tongue. Edema of hands and feed. Reddening of palms and soles. Lymph node swelling. Edema – Hands and Feet. Peeling Finger Tips.

Kawasaki Disease:

Kawasaki Disease: Blood Values: Interventions: Elevated WBC. Elevated ESR. Elevated platelets. Interventions: Intravenous gamma globulin. High dose of ASA while in hospital. Low dose ASA upon discharge. Base-line echocardiogram to assess coronary artery status