THE CHILD WITH A CARDIOVASCULAR DISORDER: CONGENITAL DEFECTS

OBJECTIVES

• Contrast anatomic and physiologic differences in the cardiovascular systems of infants and children vs. adults.

• Discuss assessments, medications, diagnostic tests, and interventions for pediatric cardiovascular disorders.

• Differentiate various congenital heart defects:

  • Tetralogy of Fallot

  • Patent Ductus Arteriosus

  • Atrial Septal Defect

  • Ventricular Septal Defect

  • Coarctation of the Aorta

  • Transposition of the Great Vessels

  • Hypoplastic Left Heart Syndrome

CARDIOVASCULAR DISORDERS IN CHILDREN

Congenital Heart Disease

• Structural anomalies present at birth.

• Affects about 36,000 babies annually.

• Half of all cases diagnosed within the first week of life.

• 28% associated with syndromes or chromosomal defects.

Acquired Heart Disease

• Disorders occurring after birth, often as complications of congenital heart defects.

• Often related to infections or autoimmune disease

• Can occur as complications from congenital heart disease treatments/interventions.

FETAL HEART DEVELOPMENT

• Heartbeat begins at 2-3 weeks gestation; development is completed by 8-10 weeks.

• Risk factors for congenital heart disease include:

  • Maternal substance use (alcohol, drugs).

  • Exposure to infections during pregnancy.

  • Family history of congenital heart disease (genetic predisposition).

  • Most causes remain unknown.

FETAL CIRCULATION

• Changes that happen when the cord is cut:

  • First breath - Lungs open up

  • Pulmonary vascular resistance is decreased. Blood flows into lungs.

  • Right atrial pressure decreases

  • Foramen ovale closes

  • Ductus arteriosus closes

• Three key structures:

  1. Ductus Arteriosus: shunts blood from pulmonary artery to aorta.

  2. Foramen Ovale: shunts blood from right to left atrium.

  3. Ductus Venosus: bypasses the hepatic system to vena cava.

PERSISTENT FETAL CIRCULATION

• Patent Ductus Arteriosus (PDA): shunts blood from pulmonary artery to aorta.

  • When it remains open after birth: Oxygenated blood returns to lungs from aorta, even thought it is not needed.

• Patent Foramen Ovale (PFO): shunts blood from left to right atrium.

  • Treated like a atrial-septal defect where the pressure changes cause chaos from mixing blood.

VARIATIONS IN HEART STRUCTURE AND FUNCTION

• Heart Size: the size of a child's fist.

• Children < 7: heart lies horizontally, apex at the 4th intercostal space.

• Children > 7: apex at the 5th intercostal space.

• Higher heart rates in infants (average 120 beats/min) compared to teens (average 60s).

• Heart muscle compliance and contractility are less in infants and young children.

• The heart of the newborn, it is more stiff and the only way to increase output it by increasing heart rate.

HEALTH HISTORY OF CARDIAC PATIENTS

• Present Illness: Symptoms include activity intolerance, failure to thrive, tachypnea, color changes, and delayed growth. They might be sweating around their head.

  • Tachypnea is a very early, classic, sign of heart failure in kids.

• Pregnancy History: Maternal infections, medications, and illnesses.

• Family History: Congenital heart disease and history of early sudden death at an early age.

  • Sudden cardiac death at a young age is a BIG red flag

PHYSICAL EXAM

• Inspection: Assess general appearance, color, sweating, dysmorphic features, and edema.

• Palpation: Evaluate pulse quality and amplitude, check for hyperactive precordium, and assess for hepatomegaly.

• Auscultation:

  • Listen to apical pulse (rate and rhythm).

  • Identify murmurs: location, quality, and intensity (grade 1-6).

  • Measure blood pressure in upper and lower extremities

  • 50% of newborns may have an innocent murmur.

LAB AND DIAGNOSTIC TESTING

• Tests may include CBC, blood gas, ECG, chest x-ray, echocardiography, and cardiac catheterization.

• Might see polycythemia when hypoxic

• Critical Congenital Heart Disease (CCHD) screening is essential. Pulse ox on upper and lower extremity to see difference.

CARDIAC CATHETERIZATION

• A definitive diagnostic procedure for children with cardiac diseases.

• 3 Types:

  1. Diagnostic: looking at vessels and exploring. Most benign.

  2. Interventional: They did something to fix or help.

  3. Electrophysiologic: Laser away that electric system piece.

• Procedure: Catheter insertion via femoral arteries/veins into the heart.

  • More risk for artery usage because of bleeding.

• Complications: Risk of bleeding, infection, and arrhythmias.

  • Pressure dressing to see if bleeding

  • Clear and constant inspection of the site

  • Monitor vitals for infection and arrythmias

  • Ask if there were any arrythmias during cath.

• Pre cath:

  • History and physical exam

  • Allergies to shellfish or iodine

  • Mark pedal pulses

  • Education

• Post cath:

  • Vitals

  • Dressing checks of extremity q 15 mins for one hour, then q 30 min checks for one hour, until stable.

  • Neurovascular assessment of pulses of the affected extremity for a baseline

  • Monitor for complications

  • Bed red with legs straight for 4-6 hours

  • Pain management

  • Education

CONGENITAL HEART DISEASE TYPES

1. Disorders with increased pulmonary blood flow (Acyanotic: Dont turn blue)

   • Atrial Septal Defect (ASD)

   • Ventricular Septal Defect (VSD)

   • Patent Ductal Arteriosis (PDA)

2. Disorders with decreased pulmonary blood flow

3. Obstructive disorders

4. Mixed defects

INCREASED PULMONARY BLOOD FLOW/ACYANOTIC

• Key conditions include:

  • Atrial Septal Defect (ASD)/Patent Foramen Ovale (PFO)

  • Ventricular Septal Defect (VSD) (More complex and longterm effects)

  • Patent Ductus Arteriosus (PDA)

• Symptoms:

  • Heart murmurs

  • Signs of congestive HF from constant lung involvement:

    • Tachypnea

    • Poor growth

    • Frequent Respiratory infections

Ventricular Septal Defect (VSD)

• Hole in bottom part of heart where the electrical system is.

• Left-to-right shunt (Oxygenated blood pumped out and recirculated through the lungs)

  • Left has higher pressure than the right side of heart because it needs to pump to the whole body.

• Right side of the heart needs to be pumping higher volume of flow overtime

• High risk for conduction issues

  • May need a pacemaker at some point

• Symptoms:

  • Heart murmurs

  • Signs of congestive HF from constant lung involvement:

    • Tachypnea

    • Poor growth

    • Frequent Respiratory infections

• Treatment:

  • Cardiac Catheterization

  • VSD mainly gets open heart surgery to fully repair the septal wall

Patent Ductus Arteriosis (PDA)

• Symptoms:

  • Heart murmurs

  • Signs of congestive HF from constant lung involvement:

    • Tachypnea

    • Poor growth

    • Frequent Respiratory infections

• Left-to-right shunt

• Widened pulse pressure

• Treatment:

  • Ibuprofen (by inhibiting prostaglandin) (Found to close PDA)

  • Tylenol

  • Catheterization with a coil embolization to close it

  • Surgical removal of the PDA

DECREASED PULMONARY BLOOD FLOW/CYANOTIC (will turn blue)

• Tetralogy of Fallot (TOF): Involves 4 defects including pulmonary stenosis, ventricular septal defect, overriding aorta, and right ventricular hypertrophy.

• Pulmonary stenosis: Narrowing that is not allowing oxygen to get to the lungs.

• Right-to-left shunt (Un-oxygenated blood mixes with oxygenated blood)

• Symptoms: Cyanosis (due to right-to-left shunting), polycythemia, and hypoxic spells.

• Care during hyper-cyanotic spells: calm approach, knee-chest positioning, O2 supplementation, and medications.

• Medications:

  • Morphine sulfate (relaxes pulmonary artery, decreases respiratory demands)

  • IV fluids

  • IV propranolol (Only in severe cases)

• Knee-Chest position:

  • Increases systemic vascular resistance

  • Improves pulmonary blood flow

OBSTRUCTIVE DISORDERS

• Coarctation of the aorta (COA)

• Involve stenosis or narrowing of vessels. Example: Coarctation of the Aorta (COA), showcasing:

  • Increased blood pressure and bounding pulses in upper extremities.

  • Epistaxis and headaches in older children.

• Must check femoral pulses in infants

• Repair: End-to-end anastamosis

• Could try ballooning with cardiac cath, but if too severe, requires anastamosis repair

MIXED DEFECTS

• Transposition of the Great Vessels (TGV) and Hypoplastic Left Heart Syndrome (HLHS).

• Usually associated with another defect or there is a patent duct that stays open.

Transposition of the great vessels (TGV)

• Severe cyanosis without murmur

• Diagnosed early

• Repair: Arterial switch operation (Cut vessels and switch them back)

• Patients decomp. quickly, give them prostaglandin to hold them over until we find out what is going on.

Hypoplastic Left Heart Syndrome (HLHS)

• Increasing cyanosis

• Circulatory collapse

• Right side has to do the pumping for the whole body

• Heart transplant is in their future

• Treatment:

  • Comfort care

  • Transplant

  • 3-surgery palliative repair as a bridge to transplant by age of 5

  • Prostaglandin given to keep the PDA open for survival

THE CHILD WITH A CARDIOVASCULAR DISORDER - ACQUIRED HEART DISEASE

OBJECTIVES

• Compare symptoms and management of acquired pediatric heart disorders including heart failure, acute rheumatic fever, Kawasaki disease, hypertension, and hyperlipidemia.

HEART FAILURE

• Occurs most often in children with congenital heart defects

• Signs: Sweating, blue color around mouth/eyes, breathing issues, fast heart rate, poor feeding, swelling around eyes and hands, and growth issues.

• Nursing Management: Support cardiac function with medications like Digoxin and Furosemide. Promote rest and nutrition. Daily weights, promote oxygenation

• Increase calories and small frequent feedings

Decreased cardiac output

• Monitor VS

• Assess cardiac status (pulses and heart rate)

• Administer medications:

  • Digoxin (decreases heart rate and increases force of contraction)

  • Furosemide (inhibits reabsorption of sodium)

    • Increased risk for hypokalemia

DIGOXIN

• Increases cardiac contractility and slows heart rate.

• Educate on dosing, signs of toxicity, and monitoring of heart rates and electrolytes.

• 2x day at 12-hour intervals, one hour before food or 2 hours after

• NOT FLEXIBLE and needed to be measured ACCURATELY

• Missed doses: 4 hour rule, wait until next dose; if 2 doses missed, notify doctor

• Signs of Dig toxicity:

  • Nausea

  • Vomiting

  • Decreased appetite

  • Bradycardia

  • Drowsiness

  • Lethargy

• Nursing considerations:

  • Monitor electrolytes - Hypokalemia increases effect

  • Withhold if heart rate:

    • <60 in adolescents/older children

    • <70 in young children

    • <90 in infants

INEFFECTIVE TISSUE PERFUSION

• Positions to maximize chest expansion (e.g., Fowler's position).

• Monitor vitals (pulse ox) and respiratory status (lung sounds, WOB).

• Oxygen PRN

IMBALANCED NUTRITION

• High-protein, high-calorie diet recommended for children.

• Infants: High-calorie formular or breast milk fortifier; may use 24-30 cal/oz oil or carbs added.

• For infants, means frequent small feedings or gavage if needed.

  • 20 mins every 3 hours

• Gavage reminder

• Special nipple

• Monitor growth

  • 1 oz weight gain/day for infants

  • 120-150 cals/kg/day

INTERRUPTED FAMILY PROCESSES

• Support families and involve them in care.

• Provide anticipatory guidance and encourage family involvement.

RISK FOR INFECTION

• Stress on hygiene practices, immunizations, and antibiotics prior to procedures.

• Maintain strict hand hygiene

• Assess VS - Temp

• Up-to-date vax and avoid ill people

• Prophylactic antibiotics to prevent infective endocarditis

  • Treatment: IV antibiotics 4-6 weeks

ACQUIRED HEART DISEASE

• Key conditions include:

  • Acute rheumatic fever

  • Hypertension

  • Hyperlipidemia

  • Kawasaki disease

ACUTE RHEUMATIC FEVER

• Criteria based on Jones criteria (2 major or 1 major and 2 minor). Treatment includes antibiotic therapy, corticosteroids, and rest.

• From recent strep infection

• J<3nes criteria:

  • Joints

  • Carditis

  • Nodules

  • Erythema

  • Sydenham’s chorea (jerking movements around extremities)

• Minor criteria

  • Fever

  • ESR (inflamm. marker)

  • Arthralgia (joint pain)

  • Long PR interval

• Treatment:

  • Antibiotic therapy: Full 10 day course of Penicillin

  • Corticosteroids

  • Rest - STRICT

  • Pain management

  • Cardiac complication treatment (Aortic stenosis, etc.)

  • Education

HYPERTENSION

• Screen starting at age 3; norms vary by age, gender, and height.

• Differentiate between primary and secondary hypertension types.

• 3 Separate readings and rule out secondary HTN

• Primary: Associated with BMI and waist circumference

• Secondary: Frequently related to renal or cardiac disease

• Treatment:

  • Monitor BP and symptoms

  • Education

  • Antihypertensives PRN

HYPERLIPIDEMIA

• Regular screening guidelines based on age; dietary management and lifestyle changes are crucial.

• Risk assessment screening at 2 years old based on family history

• Universal screening at 9-11 years and again between 18-20 years

• Lipid screening (Fasting) elevated:

  • Total Cholesterol ≥ 200

  • LDL ≥130

• Treatment:

  • Management and education

  • Medications PRN

KAWASAKI DISEASE

• Heart muscle inflammation with no known cause

• Acute systemic vasculitis most common in young children, peak age 1-2 years.

• Diagnosed via clinical criteria including fever duration and symptoms.

  • Fever of atleast 5 days duration AND at least 4 of the following:

    • Bilateral conjunctival injection (red inflammed sclera)

    • Changes of lips/oral mucosa (dry mucosa, strawberry tongue)

    • Changes of peripheral extremities (erythema/edema to hands and feet)

    • Polymorphous rash (Red rash all over body)

    • Cervical lymphadenopathy (lymph node swelling)

• Treatment involves IVIG, aspirin, and supportive measures (comfort and IV fluids).

• Monitor for complications, including coronary artery aneurysms.

• Avoid live vax for 11 months after IVIG

• Echo at diagnosis, 3wks and 8wks