Pediatric Cardiac Disorders

Factors Influencing Cardiovascualar Status

Genetic:

• Chromosomal alterations

Environmental:

• Pollutants

Maternal:

• Toxins, infection, chronic illness, alcohol

• Certain medications used to treat chronic conditions, such as antiarrhythmic drugs, anticonvulsants, and antidepressants (e.g., lithium and possibly SSRIs).

Multifactorial:

• Most common cause (mixture of all three)

• Primary cause of CHD; influenced by genetics and environment.

Types of Developmental and Biologic Variances in Cardiovascular Status

Embryonic:

• Occurs during embryonic development.

Childhood:

• Occurs during childhood development.

Changes are triggered by the first breath:

• Pulmonary Artery Drops:

  • Closure of the ductus arteriosus

• Right Atrium Pressure Drops and Pressure in Left Atrium Increases:

  • Closure of the foramen ovale

• Vasoconstriction:

  • Closure of the ductus venosus

Cardiac Nursing Assessment: Health History

Patient’s History:

• Infections

• Chromosomal abnormalities

• Prematurity

• Autoimmune disease

• Medications

Maternal & Fetal History:

• Birth history

• Maternal use of medications

• Radiation exposure

• Maternal illness (e.g., coxsackievirus, cytomegalovirus, influenza, mumps, or rubella)

Postnatal History

• What happened once that baby was born?

Family History:

• Heart disease

• Hyperlipidemia

Cardiac: Physical Examination

“Across the Room Assessment”:

• Cyanosis

• Shortness of breath (SOB)

• Difficulty breathing (DIB)

• Tachypnea

• Clubbing

• Eating difficulties

• Failure to thrive (FTT)

• Activity and general appearance

• Edema or jaundice (portal HTN → CHF)

**If a caregiver reports that their infant starts sweating during feeds, either breastfeeding or bottle-feeding, this is a red flag! Think cardiac!**

Palpation:

• Apical impulse (AI)

• Peripheral pulses (all of them!) and capillary refill time (CRT)—will be prolonged with cardiac issue.

• Liver borders—an enlarged liver may indicate right heart failure

Auscultation:

• Heart sounds

• Rate and rhythm

Compare upper and lower extremity blood pressure (BP).

Cardiac: Making a Diagnosis

Depends on the Symptoms:

• Holter monitor—(worn for day, weeks, or months)

• Chest X-ray

• Electrocardiogram (ECG)

• Echocardiogram (ultrasound of the heart)

• Arteriogram

• Prenatal ultrasound (US)—a fetal cardiologist will evaluate any cardiac concerns during pregnancy

• Cardiac catheterization

Cardiac Catherization

Pre-Catheterization Checklist:

• Thorough health history and physical exam:

  • Establish a baseline for post-catheterization.

• Obtain baseline vital signs

• Note fever or other signs and symptoms of infection

• Note allergies

• Review medications

• Note the NPO status

• Review labs

  • CBC, CMP, PT/PTT/INR

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For a right-sided catheterization, the catheter is threaded to the right atrium via a major vein such as the femoral vein.

For left-sided catheterization, the catheter is threaded to the aorta and heart via an artery.

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Post-Catheterization Care:

• Bedrest (4 to 8 hours) with a straight leg (as much as possible).

• Record vital signs frequently.

  • Pay attention to subtle differences (changes in HR)

• Monitor for hypotension and bleeding.

• Check insertion site.

• Monitor and compare catheterized extremities.

• Assess the child’s neurovascular status and level of consciousness.

• Monitor for arrhythmias, hypotension, or infection.

• Monitor site for hematoma and/or bleeding.

• If hematoma or bleeding, position flat and apply direct pressure 2.5 cm (1 in) above the catheter site.

  • Don’t leave the patient/room → phone a friend → PCP!

Types of Cardiac Disease

Congenital:

• Tetralogy of Fallot

• Tricuspid Atresia

• Atrial Septal Defect (ASD)

• Ventricular Septal Defect (VSD)

• Atrioventricular (AV) Canal Defect

• Patent Ductus Arteriosus (PDA)

Acquired:

• Heart Failure

• Endocarditis

• Rheumatic Fever

• Cardiomyopathy

• Hypertension (HTN)

• Kawasaki Disease

• Hyperlipidemia

• Transplant

Congenital Heart Disease

Decreased Pulmonary Blood Flow:

• Tetralogy of Fallot

• Tricuspid Atresia

  **Cyanosis

Increased Pulmonary Blood Flow:

• Atrial Septal Defect (ASD)

• Ventricular Septal Defect (VSD)

• AV Canal

• Patent Ductus Arteriosus (PDA)

  **Heart failure, ventricular hypertrophy, fluid retention, pulmonary hypertension.

  **CHF cluster care to reduce time of patient stress

Obstructive:

• Coarctation of the Aorta

• Aortic Stenosis

• Pulmonary Stenosis

  **Patient has a condition that “obstructs” or limits blood flow.

Mixed:

• Transportation of the great vessels

• Total Anomalous Pulmonary Vein Connection

• Truncus Arteriosus

• Hypoplastic Left Heart Syndrome

Tetralogy of Fallot

Decreased Pulmonary Blood Flow

• 4 Defects:

  • Pulmonary Stenosis (narrowing)

  • Right Ventricular Hypertrophy—(obstructed blood flow to the lung causes increased right-sided pressure).

  • Overriding Aorta (goes over the septum)

  • Ventricular Septal Defect (VSD)

  **PROVe = Pulm Stenosis, R Vent Hypertrophy, Overriding Aorta, VSD**

• Treatment: Hypercyanotic Spells or “Tet Spells”

  • Use a calm, comforting approach.

  • Place in a knee-to-chest position (#1)—it increases peripheral vascular resistance, which reduces right-to-left shunt at the VSD = increased pulmonary blood flow.

    • Educate parents!

  • In the Hospital:

    • Provide supplemental oxygen.

    • Administer Morphine (IV, IM, or SQ)—helps the child calm, reduces tachypnea, and decreases vascular resistance.

    • Supply IV Fluids

Tricuspid Atresia

Decreased Pulmonary Blood Flow

• Tricuspid valve does not develop.

• Blood does not go directly into the right ventricle.

• Deoxygenated blood passes through a patent foramen ovale (PFO) in the atrial septum.

• Blood mixing at the pulmonary artery and aorta.

  ________________________________________________________

• Cyanosis at birth or a few days later.

• Rapid respiration and poor feeding.

• May have coolness and clamminess to extremities.

• **Prostaglandins will be administered to keep the PDA open (connection between the pulmonary artery and the aorta open).

Atrial Septal Defect

Increased Pulmonary Blood Flow

• Hole in the wall dividing the left and right atria.

• Often asymptomatic.

  • Unless it is extreme, in which they will develop signs and symptoms of heart failure.

• Increased blood flow results in:

  • Shortness of Breath (SOB)

  • Fatigue

  • Failure to Thrive (FTT) over time.

• Repairs are usually performed around 2 to 3 years of age.

Ventricular Septal Defect

Increased Pulmonary Blood Flow

• The most common congenital heart defect!

• Hole in the wall between the left and right ventricles.

• Asymptomatic if small.

• Left to right shunt.

• Loud, harsh holosystolic murmur—rapid heartbeat. (can hear without stethoscope)

• Increased flow to lungs leading to pulmonary hypertension.

• Heart failure if not repaired.

• Failure to thrive (FTT).

Atrioventricular Canal (AV Canal)

Increased Pulmonary Blood Flow

• Failure of endocardial cushions to fuse.

• Tricuspid and mitral valves do not get separated.

• ASD and VSD are present.

• Left to right shunting.

• Pulmonary edema.

• Often associated with Trisomy 21.

• Difficulty breathing, poor weight gain and growth, cyanosis, heart murmur.

Patent Ductus Arteriosus

Increased Pulmonary Blood Flow

• Second most common congenital heart defect!

• Persistent connection between the aorta and pulmonary artery.

• More common in premature infants and those born in high-altitude areas.

• Can occur to accommodate right to left shunting diseases.

• If small, may be asymptomatic.

• If larger, may exhibit signs of heart failure.

Coarctation of the Aorta (Coarct)

Obstructive Disorder

• Narrowing of the aortic lumen.

• BP in all 4 extremities:

  • Upper extremities will be higher than lower.

• Heart Failure Symptoms

• Care:

  • Cluster care to minimize stress.

  • Digoxin administration to help increase cardiac output and perfusion.

  • Diuretics to reduce edema.

  • Higher calorie feeds.

Aortic Stenosis

Obstructive Disorder

• Narrowing; Restricted blood flow from the left ventricle to the aorta.

• Typically Asymptomatic:

  • Failure to Thrive (FTT)

  • Faint pulses

  • Easy fatigue

  • Chest pain

Pulmonary Stenosis

Obstructive Disorder

• Narrowing; Restricted blood flow from the right ventricle to the pulmonary artery.

• Right ventrical hypertrophy—similar to tetralogy of fallot.

• Typically asymptomatic:

  • Failure to Thrive (FTT)

  • Faint pulses

  • Easy fatigue

  • Chest pain

Transposition of the Great Vessels

Mixed Defect

• Pulmonary artery and aorta are switched.

• May also have ASD or VSD.

• Treatment:

  • Surgical switch*

  • Balloon atrial septostomy

  • Prostaglandins (PGE)

****Just to need to know that the great vessels need to be surgically switched****

Total Anomalous Pulmonary Vein Connection

Mixed Defect

• Pulmonary veins do not connect to the left atrium.

• Pulmonary veins connect to the right atrium or superior vena cava.

• PFO or ASD is usually present.

• Symptoms:

  • Cyanosis

  • Fatigue

  • Poor feeding

Truncus Arteriosus

Mixed Defect

• Single large vessel.

• Decreased systemic blood flow.

• Requires surgical intervention.

  • Not compatible with life for long (weeks or months).

Hypoplastic Left Heart Syndrome

Mixed Defect

• Very small left ventricle and a very large right ventricle to compensate.

• Often diagnosed prenatally.

• Requires several surgeries.

• Often leads to transplant.

Congenital Heart Defect: Nursing Management

• Medication as prescribed:

  • Diuretics often prescribed.

• Improve Oxygenation:

  • Frequent assessments.

  • Semi-Fowlers (child), 45-degree angle (infant).

  • Use oxygen sparingly (it is a vasodilator—it can decrease BP).

• Weigh daily.

  • Try using the same scale and take it about the same time of day.

• Strict I & Os.

• Allow for periods of activity and rest.

  • Cluster care

• Adequate Nutrition:

  • Increased nutritional needs.

  • Oral with supplements enterally (NGT) as needed.

  • High-calorie feedings.

  • Cautious breastfeeding and bottle feeding.

• Family Coping/Education.

• Infection Prevention.

Acquired Heart Disease: Heart Failure

• Most commonly seen in Congenital Heart Disease

• Most cases occur by 6 months of age.

• Cannot Pump Blood Effectively:

  • Reduced cardiac output

  • Hypertrophy

• Signs & Symptoms:

  • Sweating during feeds**

  • Poor feeding

  • Increased work of breathing (WOB)/respiratory distress**

  • Decreased urine output

• Poor Cardiac Output:

  • Low BP

  • Tachycardia

  • Gallop heart rhythm

  • Cool, pale skin**

• Fluid Overload:

  • Edema**

  • Crackles in lungs**

Nursing Care

• Monitor:

  • Vital signs (V/S), ECG, and cardiac status

  • Intake & output (I & O), daily weights

• Promote Rest:

  • Cluster care to minimize stress

• Provide Adequate Nutrition:

  • Infant Nutrition:

    • Feed every 3 hours when rested; hold in a semi-upright position

    • Allow rest during feedings; gavage feed if unable to consume milk

    • Increase caloric density of feeds

    • Encourage breastfeeding mothers to alternate with high-density formula or fortified breast milk

Infective Endocarditis

• At-Risk Patients:

  • Those with CHD, prosthetic valves, and central lines.

• Definition:

  • A microbial infection of the endothelial surfaces of the heart’s chambers, septum, or most commonly, the valves

• Causes:

  • Bacteria or fungi gain access to the endothelium

  • Infection can spread to other parts of the body

• Symptoms:

  • Vague flu-like symptoms (low-grade fever, pale, etc.)

  • Fatigue

  • Anorexia or weight loss

• Monitor:

  • Full Cardiac—ECG leads, HR, RR, Pulse ox, & BP.

• Treatment:

  • IV Antibiotics or antifungals for approximately 4 to 6 weeks.

Acute Rheumatic Fever

Cause:

• Delayed sequela of group A streptococcal pharyngitis (strep throat).

• Develops 2 to 4 weeks after the initial infection.

• Affects joints, CNS, skin, and subcutaneous tissue.

• Causes chronic, progressive damage to the heart and valves.

Diagnosis & Lab Tests:

• Modified Jones Criteria**

• Throat Culture

  • Detects group A streptococcal pharyngitis (recommended for all school-aged children with sore throats)

• ASO Titer (Antistreptolysin O Titer)***

  • Elevated or rising titer; the most reliable diagnostic test.

  • Checks for old strep throat

• CRP (C-Reactive Protein)

  • Elevated in response to inflammatory reaction

• ESR (Erythrocyte Sedimentation Rate)

  • Elevated in response to inflammatory reaction

Treatment:

• Antibiotics—long time (usually until they are 21 years old)

• NSAIDs

• Corticosteroids

Acute Rheumatic Fever: Modified Jones Criteria

The Diagnosis of Acute Rheumatic Fever requires the presence of either two major criteria or one major plus two minor criteria.

• Major Criteria:

  • Carditis

    • inflammation of the heart.

  • Migratory polyarthritis***

    • multiple joints hurting moving around (not always the same one).

  • Erythema marginatum**

    • subcutaneous nodules under the skin (as shown in picture)

  • Sydenham chorea

    • CNS features; muscle weakness, falling, trouble speaking, etc.

• Minor Criteria:

  • Polyarthralgia**

    • multiple areas that hurt (doesn’t necessarily moves around)

  • Elevated ESR or CRP

  • Prolonged PR interval (unless carditis is a major criterion)

“Joint pain, chest pain, and a ‘funny rash’ with reported sore throat a month ago…”

Cardiomyopathy

• Myocardium cannot contract properly.

• Most cases are idiopathic.

• May result in heart failure.

• Often requires a heart transplant.

Nursing Management:

• Monitor for clots.

• Administer vasoactive medications (Beta-blockers, Calcium Channel Blockers, ACE inhibitors).

• Provide diuretics.

• Administer anticoagulants. (rx for bleeding—medical alert bracelet)

• Allow some activity but promote rest.

Hypertension

Increased Prevalence—Primary Hypertension:

• Overweight or obesity

• ≥ 95th percentile for gender, age, and weight

Assessment:

• Growth delay

• Obesity

• Symptoms:

  • Fatigue

  • Blurred vision

  • Headache

  • Behavioral or vision changes

Treatment:

• Weight reduction

• Dietary changes

• Increased physical activity

• Pharmacological treatment

Step-Wise Approach:

• Initial Evaluation: If a child has one elevated BP reading and is overweight or obese, start with a urine test to assess renal function. Avoid unnecessary invasive testing initially.

• First Intervention: Education on activity and dietary modifications. Schedule a follow-up in 4 weeks to reassess BP.

• If No Improvement in 4 Weeks: Perform blood work to evaluate for underlying causes. Consider referral to a nutritionist or lifestyle intervention programs (e.g., weight management camps). Reassess BP after lifestyle modifications.

• If BP Remains Elevated Despite Lifestyle Changes: Initiate pharmacological treatment. Continue monitoring and follow-up for treatment efficacy.

[CARDIAC LECTURE] Kawasaki Disease

• Acute systemic vasculitis

• Unknown etiology

• More common in boys than girls

• 80–90% of cases occur in children <5 years old

• Higher prevalence in children of Asian ancestry

• Widespread inflammation of medium-sized muscular arteries

Symptoms:

• Fever for 5 or more days along with:

  • Strawberry tongue

  • Cracked, red lips

  • Cervical lymphadenopathy (usually on one side)

  • Redness of palms or soles

  • Edema of hands or feet

  • Generalized rash

  • Bilateral bulbar conjunctival injection (without exudate)—pink eye w/ no drainage

Course & Complications:

• Self-limiting, typically resolves in <8 weeks

• Potential complications:

  • Coronary artery abnormalities

  • Long-term risks: heart failure, myocardial infarction, arrhythmias

  • Aneurysms

Diagnosis:

• Patient must have at least 4 out of 5 criteria from different symptom categories.

[CARDIAC LECTURE] Kawasaki Disease: Nursing Care

Administer IVIG:

• Most effective when given within the first 7 to 10 days of illness.

• Single infusion over 8 to 12 hours.

Administer Aspirin:

• High-dose aspirin until afebrile for 72 hours

• Low-dose aspirin for 6 weeks.

  **On aspirin because we are worried about aneurysms**

Clinical Monitoring:

• **Initial Echocardiogram and then rechecked a couple of times**

• Signs & Symptoms to monitor:

  • Tachycardia

  • Gallop rhythm

  • Muffled heart sounds

  • Arrhythmias (continuous cardiac monitoring recommended)

Comfort Measures:

• Control fevers with appropriate medications.

• Moisturize lips to prevent dryness.

• Hydration: Offer cool liquids and popsicles for comfort.

Caregiver Education:

• Monitor fevers at home.

• Follow up with PCP and cardiology for ongoing care.

• Avoid live vaccines for at least 11 months post-IVIG treatment.

Treatment Options for Cardiac Disease in Children

• Interventional catheterization

• Cardiac surgery

• Pacemakers

• Cardiac transplantation

• Medical Management Interventions:

  • Pharmaceutical

  • Dietary

  • Activity

• Supportive care/community care

Heart Transplant

• Over 500 children receive heart transplants per year.

• Evaluation of candidacy involves thorough assessment of the child’s overall health and suitability for transplant.

• Recovery varies and depends on individual factors, but it typically involves a period of intensive monitoring and care.

• Lifelong treatment is necessary, including antirejection medications to prevent organ rejection and ensure the success of the transplant.

Heart Transplant: Post-Operative Interventions

• Maintain cardiac output.

• Prevent Cardiac Tamponade:

  • MEDICAL EMERGENCY:

    • Hypotension

    • Muffled heart sounds (beating through water)

    • Decreased systemic perfusion

    • Sudden cessation of chest tube drainage (just stops)

    • Narrowing pulse pressures

• Manage temporary pacing wires.

• Maintain fluid and electrolyte balance.

• Promote respiratory function.

• Prevent hemorrhage and arrhythmias.

• Monitor neurological functioning.

• Prevent infection (incision care).

• Manage sedation and pain.

• Manage nutrition.

• Provide psychosocial support.

Pharmacologic Interventions for Heart Failure

• Diuretics: decrease preload

  • Furosemide (Lasix) and Spironolactone (Aldactone)

    • Daily weights

    • Monitor labs and I & Os—low potassium

    • Encourage the child to eat foods high in potassium (bran cereals, bananas, legumes, leafy vegetables, oranges)

• Positive inotropic agents: contractility

  • Digoxin (Lanoxin)

    • Hold digoxin for HR < 90 (infants) and < 60 (adolescents)**

    • Administer at the back of the mouth and give water following (prevents tooth decay).

    • Do NOT give an extra dose if missed, or readminister if the child vomits.

    • Observe for digoxin toxicity (decreased HR, appetite, N/V)**

  • Dopamine, Dobutamine, Epinephrine

• Vasodilators:

  • Nitroglycerin, nitroprusside (Nipride)

  • Captopril (Capoten), Enalapril (ACE inhibitors)

  • Monitor BP before and after administration

  • Monitor for hyperkalemia