PEDIATRIC CARDIAC DISORDERS

It’s a worthwhile area to study!

Cardiac Disorders in Peds

Two major groups of disorders: Congenital

Aka “born with” Most structural defects

Acquired Develop later in life Bacterial endocarditis Rheumatic fever Kawasaki disease Systemic HTN

Incidence & Causes

5 to 8 in 1000 live births Cause unknown

Multiple factors Genetics/family history Environment

Toxins Viruses

Maternal chronic illness (diabetes, seizure meds) Chromosomal abnormalities

Down syndrome DiGeorge syndrome Noonan or William syndrome Trisomy 13 or 18

Older Classifications of CHD

Acyanotic “pink” NO unoxygenated blood goes to the

periphery Cyanotic

“blue” Unoxygenated blood is shunted to the

periphery May be pink

Newer Classifications of CHD Hemodynamic characteristics

Increased pulmonary blood flowToo much to lungs; “pink”; pulmonary

edema Decreased pulmonary blood flow

Too little to lungs; “blue”; cyanotic Obstruction of blood flow out of the heart

Can’t get to lungs or body Mixed blood flow

Most common

Comparison of CHD Classification Systems

Background info/Hemodynamics Review fetal to neonatal circulation (also pp. 1342-1343

Hockenberry, 9th ed.)

Blood flows from area of high pressure to one of low pressure (Fig. 34-7 p. 1351 Hockenberry, 9th ed.)

The greater the pressure gradient, the greater the rate of flow.

The greater the resistance, the lower the rate of flow

In the NORMAL HEART, pressures on the R side are less than the L side, and the resistance in the pulmonary circulation is less than that in the systemic circulation.

Fetal circulation

Fetal to Neonatal Circulation

Normal Heart Pressures(Fig. 34-7 p.1351)

The proposed pulse-oximetry monitoring protocol based on results from the right hand (RH) and either foot (F).

Kemper A R et al. Pediatrics 2011;128:e1259-e1267

©2011 by American Academy of Pediatrics

Tests of cardiac function

Prenatal ultrasound Chest x-ray Electrocardiogram

(ECG) Echocardiogram Cardiac

catheterization Stress test

(dobutamine or exercise)

Cardiac MRI

Cardiac Catheterization

Invasive routine diagnostic procedure Benefits

Better visualization Actual pressures, sats, hemodynamic

values Risks:

Hemorrhage Fever N/V loss of a pulse transient dysrhythmias

Nursing interventions for Cardiac

Catheterization (p.1348,9 9th ed. Hockenberry) Pre-procedure:

Complete a thorough hx & physical exam Check for allergies to iodine and shellfish age appropriate teaching & preparation

Don’t forget the parents NPO 4-6 hrs before procedure; sedation~ IV or po Monitor VS, SaO2, Hgb, Hct, coags, BMP Mark pedal pulses—before procedure to ensure correct

palpation afterwards. Determination the amount of sedation based on the

child’s age, condition & type of procedure

Cardiac Catheterization

Post-procedure: √ for bleeding at site of insertion of catheter in groin √ pulses esp. distal to site of insertion, temp & color of

extremities, VS q 15 Remember the 5 P’s (pain, pallor, pulse, paresthesia, paralysis)

OR CMTS—circulation, mobility, temperature, sensation √ heart rate for one full minute, for signs of dysrhythmias or

bradycardia Prevent bleeding by keeping leg immobilized for 4-8 hrs I & O, especially O. Fluids may be offered po starting with clear

liquids. √ Labs; infants are at risk for hypoglycemia—monitor blood

glucose as child may need IV with dextrose Encourage the child to void to promote excretion of contrast

medium.

Cardiac Catheterization (cont’d) Potential cardiac catheterization

complications: Nausea &/or vomiting Low-grade fever Loss of pulse in catheterized extremity Transient dysrhythmias Acute hemorrhage from entry site

apply direct continuous pressure at 2.5cm above the catherter entry site to localize pressure over the location of the vessel puncture.

Keep child flat and notify the physician Prepare for possible administration of additional

fluids prn

Cath lab

Congestive heart failure (Fig. 34-8 p. 1353)

Symptoms of CHF

Increased work of breathing

Tachycardia Decreased pulses Decreased urinary

output Poor weight gain Diaphoresis with

activity

Hepatomegaly Cold, cool

extremities, especially with stress or activity

JVD? Decreased BP is LATE

sign

Defects with Increased Pulmonary Blood Flow

Abnormal connection between two sides of heart leads to Increased blood

volume on right side of heart

Increased pulmonary blood flow

Decreased systemic blood flow

PDA, ASD, VSD Symptoms

Increased work of breathing

Rales/rhonchi and/or wheezing

Failure to thrive

Patent Ductus Arteriosus

Ductus doesn’t close Common in preemies “machinery” murmur

audio Treatment

Indomethacin Cath lab Ligation

Atrial Septal Defect

Hole between two atria of heart

Usually asymptomatic

If not treated, increased risk of atrial dysrhythmia or stroke

Usually close on own

Ventricular Septal Defect

Hole between two ventricles of heart

Symptoms related to size & location of VSD and amount of pulmonary blood flow

Fix by patching with Goretex

Atrioventricular Canal

ASD, VSD, and affected mitral & tricuspid valves

Associated with Down syndrome

Symptoms related to size of holes, degree of valvular involvement, & size of ventricles

Often accompanied with pulmonary hypertension

Nursing Management

AVOID OXYGEN!!!!!!!! Especially pre-op

Diuretics Monitor VS, I & 0, daily

wt. Encourage rest periods

to conserve energy Monitor labs: Hgb, Hct,

electrolytes Closely monitor

feedings May need higher calorie

feeds

Obstructive Defects

Coarctation of the aorta, aortic stenosis, pulmonic stenosis

Symptoms dependent upon area of obstruction

Coarctation of Aorta

Narrowed aorta leads to decreased systemic blood flow

May not present until early childhood

Bounding upper extremity pulses, weak to absent lower extremity pulses

HYPERTENSION!!!!!!!

Post-op Coarctation Care

Neuro checks Urine output Blood pressure

PAIN!!!!!!!

Aortic stenosis

Obstructs blood flow to body

Leads to left ventricle hypertrophy

Asymptomatic often Chest pain with

exercise Sometimes see

sudden death Repair with

ballooning, repair, or replacement of valve

Pulmonary Stenosis and Catheter Placement

Leads to right ventricular hypertrophy which may lead to reopening of the foramen ovale. If severe, my lead to congestive heart failure.

Defects with Decreased Pulmonary Blood Flow and Mixed Defects

May or may not be cyanotic (usually are) Tetralogy of Fallot Transposition of Great Arteries Truncus Arteriosus Hypoplastic Left Heart Syndrome (HLHS) LOTS of other defects that are

uncommon, book discusses them

Effects of Hypoxemia

Main clinical manifestations: Cyanosis Polycythemia

Thicker blood Clubbing Clotting abnormalities Delayed growth and development – can be

associated with any heart defect

Hypoxemia Management

Prostaglandin E1 given if cyanosis shown as newborn

Assess for and treat tet spells Surgery

Corrective or palliative—often staged

Prevent dehydration AVOID OXYGEN!!!!!

Tetralogy Of Fallot

Hypercyanotic “tet spells”

Acutely cyanotic ↓ pulm. blood flow & ↑ right to left

shunting Prompt tx to prevent brain damage &/or

death Calm infant/child Place in knee chest position Toddler will get in “squatting” position to

compensate for hypoxia Give oxygen Morphine/fentanyl/versed given

Knee-Chest Position

Tet Repair

Complicated Dependent on how big

RV is, how stenotic pulmonic valve is, and how big the VSD is

Either fly or die Palliative shunt:

modified Blalock-Taussig shunt (p.1364, Table 34-4, 9th ed.)

Complete repair—operative mortality <3%!

Transposition of the Great Arteries

NOT GOOD! Cath lab initially Prostaglandins Surgery at 6-7 days old—

arterial switch of pulmonary artery and aorta, but also coronary arteries are switched and re-anastomosed.

Long term prognosis very good

Hypoplastic Left Heart Syndrome

VERY VERY VERY BAD!! Mortality rates range from 10-30% (2002-04)

Can not correct easily 3 staged surgeries: Norwood, Mod Blalock Taussig, & Glenn

procedure vs. transplant Long-term data not in yet, will probably need transplant

Management of Children with Mixed Defects

Medications Digoxin—KNOW!! pp.1354-

1358—good info on meds Improves contractility of heart Review dig toxicity—pulse

rates in infants & children Diuretics—furosemide

Watch for what ?? Ace-inhibitors (angiotensin

converting inhibitors—the PRIL’s) Reduce afterload on the heart

make heart pump more efficiently.

Beta-blockers—cause decreased heart rate, BP * vasodilatation

Decrease cardiac workload Meds-as stated Decrease stimulation Cluster care Maintain neutral thermal

environment Sedation for irritable child

Remove accumulated fluid & sodium Closely monitor I&O Restrict fluid in acute

phase Weigh daily if stable

Continued management of CHF Nutrition

Smaller, more frequent feeds

High calorie formula Decrease respiratory

effort Rest Avoid colds, RSV Position with HOB Avoid crying and distress

Family support/education Keep them present,

holding, rocking, AMAP

Improve tissue oxygenation Meds assist with this

by increasing efficiency of the heart

Oxygen may be added with appropriate order, especially if there is pulmonary edema, or lower respiratory infection.

Post-operative Care

PAIN!!!!!!!!!!!!!!!!!!! Cardiac monitoring

Heart rate Blood pressure Intracardiac pressures

Chest tube care Quantity & quality of

output Urine output

Minimum 1 ml/kg/hour

Neurological checks Move all extremities Back to baseline

Respiratory care Deep breathing IS

Rest & activity Up next day Ambulate

GI distress Avoid vomiting

Care of the Family and Child with Congenital Heart Disease

Help family adjust to the disorder May be grieving loss of normal child

Educate family Help family cope with effects of the disorder Prepare child and family for surgery

Remember developmental level of child Pain, scars, IS, activity

Refer to support group with families who have already been through the experience

TOUCH is the IL Assoc. This link opens a broad site, then click on IL.

Support group with lots of links for families and persons with CHD

Website: From Cincinnati Children’s Hospital

Kawasaki Disease

Multisystem disorder involving vasculitis & may progress to coronary arteries causing aneurysm formation

Leading cause of acquired heart dz in US Etiology still unknown 3 phases:

acute subacute convalescent

Criteria for KD (must meet 5 out of 6)

(Box 34-10, p. 1388, Hockenberry (9th ed) fever > 5 days conjunctival infection without exudate oral changes: erythema, “strawberry tongue,

fissured lips extremities changes: peripheral edema,

erythema of palms and soles, peeling of hands & feet

erythematous rash cervical lymphadenopathy

Other manifestations

Symptoms of inflammation C reactive protein level ESR

Cardiac symptoms L ventricular function as seen on

Echocardiogram Children do NOT generally have sx of CHF

Other lab changes Anemia Leukocytosis with ‘L shift’

Kawasaki continued

Tx best within first 7- 10 days. : ASA 80-100mg/kg/day initially.

This is one dx that requires use of high doses of aspirin even in children. Dose is decreased to 3-5 mg/kg/day once afebrile 48-72 hrs.

IVIG 2 g/kg over 8-12 hr Here is a website with some good

information on the diagnosis and management of this disease: http://www.kdfoundation.org/

Newburger, J. W. et al. Circulation 2004;110:2747-2771

From the American Heart Association

Education of parents

Teach parents common signs of Aspirin toxicity while on high doses of ASA Tinnitus Headache Dizziness Confusion

Teach parents to report recurrence of fever

Teach parents CPR Inform parents that final cardiac sequelae

may not be known for some time.

QUESTIONS