Pediatric Cardiovascular Disease

• Health Promotion, Restoration and Maintenance of the Family: Care of The Child with Cardiovascular Dysfunction is presented by Professor Meghan McCrillis from Quinsigamond Community College NUR201.
• Course content is the intellectual property of QCC faculty
• Sharing handouts or postings is prohibited.

Heart Structure Abbreviations

• AO = aorta
• FO = foramen ovale
• PA = pulmonary artery
• PV = pulmonary vein
• RA = right atrium
• LA = left atrium
• RV = right ventricle
• LV = left ventricle
• SVC = superior vena cava
• IVC = inferior vena cava

Heart Valves

• MV = mitral valve
• AV = aortic valve
• TV = tricuspid valve
• PV = pulmonic valve

Pediatric Cardiovascular Disease

• Congenital Heart Disease/Defect (CHD) occurs in 32,000 births yearly.
• Acquired Heart Disease can be due to a complication or long-term effects of CHD.
• Acquired conditions include heart failure, infective endocarditis, and hypertension.

Fetal Circulation

• Blood flows from high to low pressure/resistance.
• Fetal lungs are deflated during fetal circulation.
• The fetal heart receives oxygenated blood from the umbilicus.
• Oxygenated blood travels via the foramen ovale from the right atrium (high pressure) to the left atrium (low pressure).

Post-Natal Circulation

• After the first breath, pressure in the heart reverses.
• Oxygenated blood goes from the left atrium (high pressure) to the right atrium (low pressure, deoxygenated blood).

Congenital Heart Defects

• Four classifications: increase pulmonary blood flow, obstructive blood flow, decrease pulmonary blood flow, and mixed blood flow

Disorders with Increased Pulmonary Blood Flow

• These are acyanotic congenital defects with a left to right shunt
• Ex: patent ductus arteriosus (PDA), atrial septal defect (ASD), ventricular septal defect (VSD)
• Symptoms: fatigue, murmur, risk of endocarditis, CHF, growth retardation

Increased Pulmonary Blood Flow Defects

• Blood flows from the higher pressure left side of the heart to the lower pressure right side.
• Left to right shunt indicates a cyanotic heart defect.
• Disorders are caused by abnormal connections between the two sides of the heart.
• Risks include: heart failure, poor weight gain, impaired growth/development, CHF, pulmonary infections and hypertension.

Atrial Septal Defect (ASD) Pathophysiology

• Communication between the right and left atria occurs
• Higher pressure of oxygenated blood from the left side of the heart goes to the lower pressure right side (to R shunt)

Atrial Septal Defect (ASD) Clinical Signs

• Most children are asymptomatic.
• Some children may have pulmonary hypertension, heart failure & atrial arrhythmias, and stroke.

Atrial Septal Defect (ASD)

• 80% close spontaneously within 18 months of life.
• Large or persistent defects can be surgically corrected by age 3.
• Treatments: cardiac catheterization and surgical repair

Ventricular Septal Defect (VSD) Pathophysiology

• Left to right shunt
• Most common CHD
• Frequently associated with coarctation of the aorta (COA), PDA, transposition of great vessels & pulmonary stenosis
• Small Defects have 50% spontaneous closure by age 2

Ventricular Septal Defect (VSD) Clinical Signs

• Small openings may be asymptomatic.
• CHF is common with large defects.
• Murmur present
• Risk for infective endocarditis

Ventricular Septal Defect (VSD) Treatment

• MRI or Echocardiogram confirms diagnosis.
• Treatments: surgical repair; repair via cardiac catheterization is under clinical trial

Patent Ductus Arteriosus (PDA) Pathophysiology

• Failure of the ductus arteriosus to close within the first few weeks of life
• Vascular communication between the pulmonary artery and aorta
• Left to right shunt with high pressure in the aorta, which is the 2nd most common defect

Patent Ductus Arteriosus (PDA) Clinical Signs

• CHF, murmur, R ventricular hypertrophy, widened pulse pressure, bounding peripheral pulses
• Risk for endocarditis

Patent Ductus Arteriosus (PDA) Treatment

• Echocardiogram confirms diagnosis.
• Administer IV indomethacin (Indocin).
• Prostaglandin is a hormone that causes vasodilation and smooth muscle relaxation.
• Indocin inhibits prostaglandin synthesis and leads to patent ductus closure.
• Treatments: cardiac catheterization and surgical interventions to close the duct

Obstructive Disorders

• Coarctation of the aorta
• Pulmonary stenosis

Coarctation of the Aorta (COA) Clinical Manifestations

• Narrowing of the aorta
• May not be picked up until later during BP screen; patients may present with epistaxis, headache, dizziness due to hypertension (HTN), and/or CHF
• Can lead to ruptured aorta, aortic aneurysm & CVA
• Upper extremities: increased BP & bounding pulses in arms
• Lower extremities: weak or absent femoral pulses, cool lower extremities with lower BP

Coarctation of the Aorta Evaluation and Treatment

• Echocardiogram determines the extent of narrowing.
• CXR indicates cardiomegaly.
• Balloon angioplasty
• Stents
• Surgical end to end anastomosis via thoracotomy incision
• Pre-op IV alprostadil (Prostaglandin, PGE 1) to vasodilates the ductus arteriosus muscle

Pulmonary Stenosis Pathophysiology

• Narrowing between the R ventricle & the pulmonary arteries

Pulmonary Stenosis Clinical Signs

• Depend on the severity of stenosis, may be asymptomatic
• CHF
• Murmur
• Cyanosis, hypercyanotic spells with severe stenosis; pressure can force the foramen ovale to reopen
• Right ventricular hypertrophy occurs due to resistance to blood flow, causing increased workload for the right ventricle. At risk for endocarditis

Pulmonary Stenosis: Treatment

• R ventricular hypertrophy evident on CXR
• Echocardiogram determines extent of obstruction.
• Treatment is dependent on the severity of stenosis.
• Balloon dilation via cardiac catheter to dilate the valve
• Dilation with stent
• Surgical intervention.

Disorders with Decreased Pulmonary Blood Flow and Mixed Defects

• Tetralogy of Fallot
• Truncus Arteriosus
• Transposition of The Great Vessels
• Tricuspid Atresia

Tetralogy of Fallot (decreased pulmonary blood flow)

• Combination of 4 defects
• Right to left shunt results in cyanotic heart
• Cyanosis with pulmonic stenosis worsens during the 1st year of life
• Murmur, hypercyanotic spells
• Palliative or complete repair for surgical repair

Hypercyanotic Tet Spells

• Related to increased O₂ needs with limited pulmonary blood flow.
• Rarely seen before 2 months of age, most frequent in 1st year of life.
• Spell may occur spontaneously or with feeding, bathing, IV insertion, stressful procedures etc.
• Persistent cyanosis with CHD places a child at high risk for neurological compromise.
• Cerebral hypoxia can lead to CVA, developmental delays, and death.

STAT Interventions for TET Spells

• Calm baby
• Knee chest position improves pulmonary blood flow
• 100% oxygen
• Morphine IM, SC or IV
• IV fluids to keep well hydrated to prevent CVA's
• Beta-adrenergic blocking: propranolol (Inderal), atenolol (Tenormin), metoprolol (Lopressor) decreases heart rate and force of contraction to increase pulmonary blood flow

Hypoxemia: Manifestations

• Cyanosis: blue discoloration in the mucous membranes, skin & nail beds
• Hypoxemia: decrease in tissue oxygenation, resulting in impaired cellular processes
• SpO2 is usually apparent with sats below 80-85% in room light

Etiology of Hypoxemia

• Cardiac: cyanosis increases with crying, doesn't improve with oxygen, and tachypnea
• Respiratory: cyanosis decreases with crying, improves with oxygen, and respiratory distress

Consequences of Long Term/Chronic Hypoxemia

• Polycythemia: increased RBC's increases viscosity of blood
• Clubbing: thickening and flattening of the tips of fingers & toes
• Fatigue with feeding and poor weight gain
• Tachypnea and dyspnea
• Hypercyanotic spells (tet spells)
• Neurological Complications: stroke, brain abscess, and developmental delay.

Mixed Defects

• Transposition of great vessels (TGV)
• Total anomalous pulmonary venous connection
• Truncus Arteriosus
• Hypoplastic left heart syndrome

Transposition of the Great Vessels/Arteries

• Pulmonary artery arises from the left ventricle and the aorta arises from the right ventricle; vessels switch places
• No communication between systemic & pulmonary circulation
• Increased chance of CHF with other factors (PDA, PFO, VSD)

Transposition of the Great Vessels: Dx and Clinical Signs

• Diagnosed with echocardiogram
• Significant cyanosis without a murmur in newborns is a red flag.
• Size and type of associated defects will determine signs
• Large septal defects, PDA or VSD (murmurs heard) correlate with less severe cyanosis
• Severely cyanotic with minimal communication

Transposition of the Great Vessels: Treatment

• IV alprostadil (Prostaglandin, PGE 1) administered to keep ductus arteriosus open while awaiting surgical repair
• Increases blood mixing and acts as a vasodilator; use caution for platelet aggregation in pts prescribed anticoagulants and antihypertensives
• Balloon atrial septostomy increases mixing by opening the atrial septum
• Surgical repair within 1 week

Total Anomalous Pulmonary Venous Connection

- Rare defect in which the pulmonary veins drain to the right side of the heart ,or into one or more of the systemic veins.
- shunting of blood Right (high pressure) to Left is allowed by associated ASD & PFO which then results in a mix of blood
- Diagnosed with an echocardiogram and cardiac cath

• Pulmonary vein is anastomosed to the L atrium
• Aortic Septal Defect is closed
  • Patients might be cyanotic

Truncus Arteriosus

• Rare CHD where : - the aortic valve & pulmonary artery share the same valve, with 2-5 leaflets positioned over both ventricles
  • One artery supplies the pulmonary & systemic circulation/A VSD may also be present.
  • Systemic bloodflow declines Manifestations: cyanosis, tires easily, difficulty feeding, poor growth & restlesness

Treatment: echocardiogram pre-op: IV alprostadil surgery to maintain ductus arteriosus.

Hypoplastic Left Heart Syndrome: Pathophysiology

• Underdevelopment of left heart in which: Requires ASD, and a patent foramen Ovale (PFO) which allows the mixing of blood.
• Prenatal diagnostics includes to fetal echocardiogram
• Prenatal and postnatal ultrasound Clinical signs with cyanosis with CHF, deterioration once PDA closes; Decrease cardiac output (CO) cardiovascular collapse follows

Heart SyndromeTreatment:

Hypoplastic Left

IV alprostadil (Prostaglandin, PGE 1) to maintain ductal patency for survival In absence of ASD Surgical correction done in several stages leading to a heart transplant

• Prognosis

   -Fetal within the first month of life
  
    -30% mortality post op
  

ACQUIRED CONGENITAL DISORDERS

-Heart Failure

       -Infective Endocarditis

        -Rheumatic Fever

          -Kawasaki Diseas

Congestive Heart Failure (CHF):Definition

• In-ability to pump an (adequate)amount of blood to the (systemic) circulation to meet demands of the body Etiology

Most frequently in children with heart defects.Or secondary to cardiomyopathy, myocarditis, fluid overload, hypertension, severe electrolyte imbalance, sepsis or severe anemia.

Diagnostic Evaluation: Tachypnea,Retractions, Tachycardia at rest/ clinical symptoms,dyspnea ,activity intolerance,weight gain,ECG & CXR, (Echocardiogram)

CHF: THERAPEUTIC MANAGEMENT:

Decrease Cardiac Demands

• Semi-fowler (to elevate HOB to a 45 degree angle).
• Decrease Physical activity
• Control infections with medication
• Plan rest in cycles.

Remove Accumulated Fluid and Sodium.

 -Low sodium Diet
  -Diuretics & FluidRestriction

Improve (Tissue) Oxygenation

• Monitor the respiration in order to note: signs & symptoms
• Use Cool Humidifies 02

Maintain Nutritional Status

• Monitor feeding-Usually every 3 hour.
• May use gavage supplements in breastfed babies
• Greater Caloric Needs

- Monitor Cardiac Function

 -(Decreased Urinary output) &
 -Decrease output

- Inotropes (Cardiac Glycosides)

Digoxin (lanoxin):

• Increases output
• decreased urinary overload.
• decreased (Venous) pressure
• adverse vomiting ,diarrhea ,digitalis

toxicity

• Serum of theophylline Range-( 0.8-2.0-ng.ml) Inotropes (cardiac glycosides)

Nursing Interventions Check apical pulse for one minute prior to administration. Hold med if apical pulse is: < 90/min. in infants < 70/min. in older child < 60/min. in adolescence and adults Obtain rhythm strip-assess for prolonged P-R interval and dysrhythmias.

Compare dose calculation with another nurse and validate correct amount when drawn up in syringe. Do not round. Labs -Sodiums, digoxin

Angiotensin-converting enzyme(ACE): Inhibitors

Adverse ( Vasotec) Lisinopril (Previval) / Enalapril Maleate (capoten):

Effect: Cough ,Hypertension & Hyper-kalemia

• Nursing intervention: Before and after BP / Assess side effects

( teaching):

PointsSide effect/ what meds( to consult).

• Adjuvant -Drugs: Treat CHF*

• Loop Diuretics-

Furosemide (Diuretics)-decrease: lasix and Bumex Intervention: (vs.) Daily. weight asses in the lytes causes increase give food with-Potassium/ hyperglycemia.Diet( Low sodium)

-Thiazide Diuretics:

• Hydrochlorothiazide causes decrease ( preload)-increase.

• ( Nursing )Intervention: With Med’s give the food BP.

( Aldosterone) antagonist:

Spironolactone( Adalatone) eplenore (Inspra) Diuretics : Potassium - usually need loop if you’re also

- Nursing interventions:

Daily weigh the the med prior and Potassium.

Infective Endocarditis:

Sub acute: bacterial endocarditis infective =the heart.

Infection of Valves and Linings in the heart bacteria fungal infections and -Staphylococcus

Infective Endocarditis:

Signs: - low Fever fatigue ,Anorexia,weight loss, Heart Failure

• Extracardiac: emboli ( hemorrhages): Skin on

Janeways lesions(palms and fingers).

Treatment Prophylaxis 1hr b4,IV administration.

Labs /Diagnoses=visualize the ECHO

Acute-Rheumatic- Fever.

Causes/Occurs after (Pharyngitis) strep throat and the ( Valves (skin, heart,),subcutaneous, Tissue & Joint

• Jones

ECG

- ARF

Major

• Cardio (itis): inflammation

• Poly Arthritis (inflammation)

• minor Fever

• Elevated SED rate and Prolong Pr

• Arthraglia

• (KD) -Kawasaki Disease

A (Acute)systemic vasculitis or cause (of Inflammation),( unknown ),but or May

Cause/be infection/inflamation ( Arterioles)or capillaries

• Progress and can cause coronary artery aneurysms -Leading cause in and young children.

P-FINDINGS exam :

103F,( unresponsive)-Fever,

red in - eyes -(without) fluid

M- Mucus= Tongue will become dry and Strawberries .red

L- lymphnode

B/ RASHES = Palms, Bumpy/red Feet rash + the soils

Jaundice is

Therapeutic Management:

IV for 4 days/ Reduce Thrombosis and inflammation ,Aspirin Monitor adverse

- Nursing management

Labs

and -A (complete Blood count),

• monitor heart ,vs/ weight, output

• Comfort the (patients).and family
• Immunization 11 m04
• Teachings is( desquamation

and(peeling )

.AHA

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