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What is the direction of blood flow through the foramen ovale?
Bypasses lungs by going from right atrium to left atrium
What is the direction of blood flow through the ductus arteriosus?
Bypasses lungs by going from pulmonary artery to aorta
What is the direction of blood flow through the ductus venosus?
Bypasses liver by going from umbilical vein to inferior vena cava
What is heart failure?
Heart is unable to pump blood effectively, causing low tissue perfusion or blood being backed up
What is left-sided HF and what are its signs?
Blood backs up into lungs → tachypnea, dyspnea, crackles, cough, too fatigued to eat
What is right-sided HF and what are its signs?
Blood backs up into systemic circulation → peripheral edema, weight gain, jugular vein distention, hepatomegaly
What are the defects that cause increased pulmonary blood flow?
Atrial septal defect (ASD), ventricular septal defect (VSD), and atrioventricular canal defect (AVCD)
What is left-to-right shunting?
Oxygenated blood from the left side of the heart pumps into the right side of the heart through abnormal opening
What is the pathophysiology behind increased pulmonary blood flow?
Left-to-right shunting increases pulmonary blood flow → increases pulmonary artery resistance and cardiac workload
What are potential complications of increased pulmonary blood flow?
Pulmonary HTN, HF, and Eisenmenger syndrome (reversal shunting to right-to-left)
Why is supplemental oxygen avoided in congenital heart defects with increased pulmonary blood flow?
Oxygen causes pulmonary vasodilation, increasing left-to-right shunting which further increases pulmonary blood flow and worsens congestion
What is an atrial septal defect (ASD)?
Abnormal opening in the septum/wall separating the right and stronger left atria
What are clinical manifestations of a larger atrial septal defect?
SOB, easily fatigued, poor feeding and weight gain; smaller defects are unnoticeable
What is the treatment for atrial septal defect?
Spontaneous closure, typically by 18 months
How do small VSDs and small ASDs present themsleves?
Asymptomatic most times
What is a ventricular septal defect (VSD)?
Abnormal opening between the right and stronger left ventricles, causing increased pulmonary blood flow
What are the clinical manifestations of moderate-to-large VSD?
Right ventricular hypertrophy, harsh holosystolic murmur along left sternal border, signs of HF
What is the treatment for VSDs?
Spontaneous closure by age 2 for small VSDs; surgical repair or catheter-based occlusion devices early to prevent lifelong vascular changes
What is a atrioventricular canal defect (AVCD)?
Abnormal development of the center of the heart, containing a ASD, VSD, and one common AV valve
What heart defect is associated with down syndrome?
Atrioventricular Canal Defect (AVCD)
How is the ductus arteriosus closed after birth?
Placenta is removed and causes prostaglandins E1 and E2 levels to decrease → newborn breathes → lower prostaglandins + increased oxygen causes ductus arteriosus to constrict
What is a patent ductus arteriosus (PDA)?
Failure of the ductus arteriosus to close after birth, maintaining the connection between the pulmonary artery and aorta
What is the pathophysiology for a patent ductus arteriosus (PDA)?
Aortic pressure is higher than the pulmonary artery → some blood flows down to lower pressure in lungs → increases left sided heart workload
What is the treatment for a patent ductus arteriosus (PDA)?
IV ibuprofen and indomethacin which inhibit prostaglandins
What happens if medications are unable to close a patent ductus arteriosus?
Cardiac cath, surgery (thoracotomy), or litigation
What are obstructive disorders?
Defects involving narrowing or blockage of heart valve or blood vessel, restricting blood flow and increasing pressures/workload
What are the types of obstructive disorders?
CoA, aortic stenosis, pulmonic stenosis
What is coarctation of the aorta (CoA)?
Narrowing of the lumen of the aorta, restricting blood flow from the left ventricle to the rest of the body
What is the difference between preductal and postductal CoA?
Preductal narrowing occurs before the ductus arteriosus; postductal narrowing occurs after it
How does CoA affect blood pressure above and below the narrowing?
BP is high above the obstruction and lower below it because less blood reaches the lower body
What are clinical manifestations of the upper body in CoA?
BP 20 mmHg higher in arms than legs, epistaxis, vertigo, headache
What are clinical manifestations of the lower body in CoA?
Weak or absent femoral pulses, cool extremities, delayed cap refill and UOP
What is collateral circulation?
Alternative blood vessels enlarge to help deliver blood around the obstruction
What is a sign of collateral circulation in CoA?
Rib notching
What is the nursing management for CoA?
Monitor and compare perfusion with all 4 lower and upper extremities
What medication is given to infants with CoA?
Prostaglandin E1 to keep ductus arteriosus open, allowing an alternative pathway for blood to reach lower part of body when aorta is obstructed
What medication is given to older children with CoA?
Antihypertensives (beta-blockers, ACE inhibitors)
What are defects that cause decreased pulmonary blood flow?
Tetralogy of Fallot and tricuspid atresia
What are the conditions that make up Tetralogy of Fallot?
VSD, pulmonic stenosis, overriding aorta, and right ventricular hypertrophy (RVH)
What is an overriding aorta?
Aorta positioned over VSD, receiving blood from both ventricles (including poorly oxygenated blood)
What is the pathophysiology for Tetrology of Fallot?
Right-to-left shunt that causes less oxygenated blood to enter systemic circulation, causing hypoxemia and cyanosis
What is a right-to-left shunt?
Deoxygenated blood from the right side of the heart bypasses the lungs and enters the left atrium
What triggers Tat spells in Tetralogy of Fallot?
Anything that decreases oxygen sats → crying, feeding, straining while pooping, dehydration, fever, activity
What are clinical manifestations of Tetralogy of Fallot?
Tat (hypercyanotic) spells, cyanosis, hypoxemia, clubbing
What are Tat spells?
Sudden episodes of severe cyanosis caused by acute decrease in pulmonary blood flow and increased right-to-left shunting
What is the nursing management for Tat spells?
Knee-to-chest or squatting, oxygen, comfort child, medications as prescribed (morphine, -olol)
Why do we place child in knee-to-chest position or squatting during a Tat spell?
It increases systemic vascular resistance (SVR), making it harder for blood to flow from right to left ventricle
What is tricuspid atresia?
Tricuspid valve fails to develop, preventing blood flow from right atrium to right ventricle
How does blood reach the left side in tricuspid atresia?
Through an ASD, PFO, VSD, or PDA to allow blood to reach the lungs
What is the pathophysiology for tricuspid atresia?
Deoxygenated blood crosses to the left side through alternative opening, mixing with oxygenated blood and lowering overall oxygen saturation
What is the treatment for tricuspid atriesa?
Give prostaglandin E1 to maintain ductal patency until surgery
What are mixed disorders?
CHDs where oxygenated and deoxygenated blood mix, resulting blood with lower oxygen saturation that enters systemic circulation
What are some types of mixed disorders?
Transposition of the great arteries (TGA), truncus arteriosus, hypoplastic left heart syndrome
What is transposition of the great arteries (TGA)?
Right ventricle leads to the aorta and the left ventricles lead to the pulmonary arteries, creating a parallel circulation where blood can’t be oxygenated
Why is TGA described as parallel circulation?
Deoxygenated blood returns to the body, while oxygenated blood returns to the lungs
What are clinical manifestations of TGA?
Severe cyanosis at birth that doesn’t improve with 100% oxygen
Why are ASD, VSD, or PDA important in TGA?
It allows oxygenated and deoxygenated blood to mix, so some oxygenated blood can reach systemic circulation
What is the treatment for TGA?
Prostaglandins E1, balloon atrial septostomy, and arterial switch operation
What is truncus arteriosus?
Only one major artery leaves the heart instead of having two separate arteries (aorta and pulmonary artery)
What is the pathophysiology behind truncus arteriosus?
Both ventricles pump into one common artery → mixed blood, pulmonary over circulation, and decreased systemic blood flow
What is acute rheumatic fever (ARF)?
A delayed inflammatory acute complication of group A streptococcal (GAS) pharyngitis, occurring 2-6 weeks after infection
What can repeated episodes of ARF cause?
Permanent heart valve damage, leading to rheumatic heart disease (chronic)
What is sydenham chorea?
Sudden, involuntary jerky movements
What is migratory polyarthritis?
Painful swollen joints where inflammation moves from one joint to another
What are erythema marginatum?
Red rash with central clearing and elevated edges
What are subcutaneous nodules?
Firm, painless nodules over the wrists, elbows, and knees
What is carditis in ARF?
Inflammation of the heart that can cause murmurs, tachycardia, and heart failure
What makes up the JONES clinical manifestations criteria in ARF?
Joint involvement, carditis, subcutaneous nodules, erythema marginatum, and Sydenham chorea
What are the main medications to manage ARF?
Penicillin (IM or oral) and ibuprofen to reduce fever, joint pain, and inflammation
What is the nursing management for carditis in ARF?
Give corticosteroids, IVIG, diuretics, digoxin, and strict bed rest
Why is bedrest important in ARF?
Decreases cardiac workload while heart is inflamed, especially when carditis or HF is present
When can a child with ARF resume ambulation?
When CHF resolves, ESR/CRP decreases, and HR returns to normal
What is given to manage Sydenham chorea?
Phenorbarbital or diazepam to control involuntary movements
Why is heart rate (apical pulse) monitoring important in ARF?
Persistent tachycardia indicates ongoing cardiac inflammation or increased workload; strong indicator of improvement
Why should the heart rate be monitored during sleep in ARF?
HR is lower during sleep, so persistent tachycardia during sleep indicates current heart stress
What should be educated to parents of children wtih ARF?
Enforce bedrest, full abx course, long-term abx prophylaxis will be needed, and report worsening cardiac
What is Kawasaki’s Disease?
An acute systemic vasculitis of small and medium-sized blood vessel, including coronary arteries
Why is Kawasaki disease dangerous to the heart?
Inflammation can cause coronary artery aneurysms, blood clot formation, and ischemia
What occurs during the acute phase of Kawasaki disease?
High fever that lasts 5 days, extreme irritability!!, unresponsive to abx and antipyretics
What occurs during the subacute phase of Kawasaki disease?
Fever resolves, but other manifestations remain; high risk of coronary artery aneurysm
What occurs during the convalescent phase of Kawasaki disease?
All clinical signs have resolved, lab values normal, takes 6-8 weeks onset
What oral mucosa findings occur with Kawasaki disease?
Strawberry tongue, dry cracked lips, redness of oral mucosa
What eye findings occur with Kawasaki disease?
Bilateral red eyes w/ conjunctivitis without purulent drainage
What skin findings occur with Kawasaki disease?
Widespread red rash over trunk and extremities (polymorphous), desquamation/peeling around fingers and toes
What lymphatic and musculoskeletal findings occur with Kawasaki disease?
Unilateral cervical lymphadenopathy, joint tenderness, or arthritis
What are medications given for Kawasaki disease?
High-dose aspirin (80-100 mg/kg/day divided into 4 doses) and single high-dose IVIG infusion
What is the nursing management for Kawasaki disease?
Provide a quiet environment!!!, offer ice chips or cold wash cloth, and oral hygiene w/ vaseline
What are signs of aspirin toxicity?
Headache, confusion, dizziness, tinnitus
What is the nursing considerations for medications for Kawaski disease?
Avoid other NSAIDs and avoid measles and varicella vaccinations for 11 months due to IVIG
What is normal urine output?
1-2 mL/kg/hr
What HR should be digoxin be held?
<60 for adolescent, <70 for children, <90 for infants
What are signs of digoxin toxicity?
N/V, bradycardia, dysrhythmias, yellow halos
What is the nursing management for digoxin?
Monitor K+, give 1 hour before or 2 hours after feeding to allow better absorption
What interventions help drecrease cardiac workload?
Cluster care, allow rest periods, maintain neutral thermal environment, prevent fever, and support nutriton
Why do infants with CHDs have increased nutritional support?
Hearts and lungs work harder, and an increase in workload causes fatigue, making feeding difficult
What is the nursing management for infant feeding?
Give small amounts q3-4 hours, limit duration of feeds, use high calorie formula or supplements during breast feeding
What should be assessed before a cardiac catherization?
Signs of infection, height/weight, and allergies to contrast or meds
Why might a cardiac catherization be postponed if a child has a fever, cough, or skin infection?
Increases risk for endocarditis
What is the nursing management pre catherization?
NPO 4-6 hours, hold digoxin, and mark pulses
What assessments are important after a cardiac catherization?
Vitals, low extremity neurovascular checks, and pressure dressings