Pediatric Cardiovascular Assessment

Lynn Boyle RN, MSN, CCRN

Statistics

♥ In the US, a child is born with a CHD every 15 minutes.

♥ Nearly one of every 100 babies is born with a CHD.

♥ Congenital heart defects are the #1 cause of birth defect related deaths.

♥ Congenital heart defects are the leading cause of all infant deaths in the United States.

Statistics

♥ Each year approximately 40,000 babies are born in the United States with a congenital heart defect.

♥ Thousands of them will not reach their first birthday and thousands more die before they reach adulthood.

♥ However, with recent changes in both medical and surgical management outcomes have improved dramatically.

Think Congenital Heart Defect When…..

♥ Murmur and cyanosis♥ Cyanosis without respiratory

distress♥ Crying worsens cyanosis♥ Abnormal weight loss, poor

feeding with tachypnea♥ Discordant upper/lower pulses♥ Infants appear septic♥ Diagnosed with a syndrome

When thinking about the defects, look carefully at the name...

ATRESIA = missing, not developed STENOSIS = narrowed, stiff, not flexible HYPOPLASTIC = small, not developed SEPTAL = either atrial or ventricular wall which

divides the chambers right & left ANOMALOUS = wrong connection or direction TETRALOGY = four

THE TERMINOLOGY GIVES YOU CLUES...

And more terminology…

♥ Vasoactive → raise or lower HR and BP♥ Vasopressors → typically act to raise BP♥ Inotropic → work through and receptors

to vasodilate, vasoconstrict and / or enhance contractility

♥ Chronotropic → change HR by affecting nerves controlling the heart OR

by changing the rhythm from the SA node

Cardiac Output

♥ Cardiac Output (CO) = HR x SV

volume of blood ejected from the LV / min

♥ Good CO = good tissue perfusion

♥Examples of good cardiac output??

Cardiac Output

♥ Stroke Volume

♥ Heart Rate

♥Influenced by:♥ Preload ♥ Afterload♥ Compliance♥ Contractility

Stroke Volume

♥ Volume of blood ejected from the LV with each contraction / ventricular beat

♥60-75% of ventricular volume

♥Stroke volume = Preload + Contractility + Compliance + Afterload

Summary of Clinical Manifestations

♥ Left Heart Failure- dyspnea

- tachycardia

- S3 sound

- crackles, frothy sputum,

- oliguria

- decreased SaO2, PaO2

- increased PAWP, SVR

- decreased CO

♥ Right Heart Failure- jugular venous distension

- hepatojugular reflux

- dependant pitting edema

- hepatomegaly

- anorexia, nausea

- weight gain

- increased CVP

- increased PVR

Treatment of Heart Failure

Goals of treatment include:♥ Maximize cardiac performance♥ Decrease myocardial oxygen demand♥ Improve cardiac contractility ♥ Optimize preload♥ Decrease afterload

It all starts here……

Fetal Heart Development

Fetal Circulation

♥ Heart development @ 4-7 weeks

♥ Fetus tolerates sats due to cardiac output

♥ PVR high in fetus, and starts to fall after the 1st week of life

(www.chop.edu/consumer/your_child/condition_section_index.jsp?id=-9306&printable=1)

Fetal Circulation: Shunts Foramen Ovale

Opening in the mid-atrial wall

Fetus: right to left shunt

Neonate: left to right shunt

Functional closure versus anatomic closure

www.clevelandclinic.org

Fetal Circulation: Ductus Arteriosus

http://www.nhlbi.nih.gov/health/dci/Diseases/pda/pda_what.html

A word about shunting...

© Left to right♥ Oxygenated blood

from systemic side flows to pulmonic side

♥ body receives oxygenated blood

♥ RedBlue=Pink

♥ Acyanotic

Right to left♥ Deoxygenated

blood from the pulmonic side flows to the systemic side

♥ body receives deoxygenated blood

♥ BlueRed=Blue

♥Cyanotic

Another word about shunting…vascular resistance to flow

♥ PVR: Pulmonary Vascular Resistance♥ Increased= vasoconstriction/decreased flow♥ Decreased = vasodilation/increased flow

♥ SVR: Systemic Vascular Resistance♥ Increased = vasoconstriction/decreased flow♥ Decreased = vasodilation/increased flow

Why is this important?

Why is this important?

♥ In addition to the adjusting newborn’s transitional circulation….♥ PVR falls and SVR increases, closing the functional

shunts…..

♥ What we, as NURSES, do at the bedside, both to and for the patient, will affect the pattern of both systemic and pulmonary blood flow.

Why is this important?Nursing Considerations

What do we do at the bedside that affects SVR? Medications-vasoactive infusions Pain control Temperature Regulation

♥ What do we do at the bedside that affects PVR?♥ Suction♥ Hyperventilate/hyperoxygenate♥ Pain control♥ Acidosis

Why is this important?♥ Oxygen is a vasodilator♥ CO2 is a potent pulmonary vasoconstrictor

These are key points to remember when attempting to control where the blood will “want” to go.

Always keep the PATH of LEAST

RESISTANCE in mind.

How we care for our patients WILL

dramatically affect their hemodynamics.

Classification of CHD

♥ Increased Pulmonary Blood Flow♥ Decreased Systemic Blood Flow♥ Decreased Pulmonary Blood Flow♥ Mixed Lesions

Classification of CHD

Obstruction to systemic blood flow Coarctation of the Aorta Aortic stenosis Interrupted Aortic Arch

Acyanotic

Obstruction to pulmonary blood flow Tetralogy of Fallot/TOF

Pulmonary stenosis/PS Pulmonary Atresia/PA

Cyanotic

Classification of CHD

Increased pulmonary blood flow VSD ASD PDA CAVC

Acyanotic

♥ Variable

pulmonary blood

flow (Mixed

Lesions)

♥ HLHS

♥ Truncus

Arteriousus

♥ TGA

Cyanotic

Decreased Pulmonary Blood Flow♥ Cyanotic heart disease

♥ Obstruction of blood flow to the lungs♥ Embryonic developmental failure: stenosis or atresia of

pulmonary valve and/or artery

♥ Degree of cyanosis♥ Dependent upon the degree of shunting or obstruction

♥ Development of long term problems:♥ Polycythemia, clotting abnormalities, pulmonary

vascular disease♥ High risk for CVA/stroke

♥ Supportive therapy: PGE1 (Prostaglandins)

♥ Surgical interventions: Palliation or correction

Prostaglandins♥ PGE1, Prostin VR, Alprostadil

♥ Maintains the patency of the ductus arteriosus♥ Infusions: 0.01 - 0.4 mcg/kg/min♥ Reduce rate when desired effect achieved♥ Side Effects:

• flushing• bradycardia • tachycardia• hypotension • fever

• APNEA-greatest risk in the first four hours

Decreased PBF: TOF• 1. PULMONARY

STENOSIS/RIGHT VENTRICULAR OUTFLOW TRACT (RVOT) OBSTRUCTION (RVOTO)

• 2. RIGHT VENTRICULAR HYPERTROPHY (RVH)

• 3. AORTA “OVERRIDES” or “sits over” the VSD (ventricular septal defect)

• 4. VENTRICULAR SEPTAL DEFECT (VSD)

(http://cincinnatichildrens.org)

Decreased PBF: TOFWhen pulmonary vascular resistance(PVR) is

high: right to left shunt across the VSD

When systemic vascular resistance (SVR) is high: left to right shunt across the VSD

Mild to severe cyanosis depends upon the degree of infundibular stenosis (the area under the pulmonary valve)

Boot shaped heart on CXR

“Tet Spell” “Hypercyanotic Spell” “Hypoxic Spell”

Treatments Knee-chest position

( SVR)♥ MSO4

( agitation)♥ NaHCO3

(correct acidosis)♥ Oxygen

( pulmonary vasodilator)

♥Phenylephrine ( SVR)

(http://www.nlm.nih.gov/medlineplus/ency/imagepages/18134.html)

Palliative B-T (Blalock-Taussig) Shunt

♥ Right or left subclavian artery to the pulmonary artery

♥Modified= use of

artificial material for a graft/conduit

http://www.americanheart.org/images/ImagePicker/13032-inter-full.jpg

Surgical Repair: TOF(www.cincinnatichildrens.org)

5. The muscular right ventricular outflow tract obstruction

is cut away (resected). 6. The VSD has been closed with a

patch. 7. A look from the outside of the patch repair to the pulmonary artery to enlarge the outflow tract.

What about these patients?♥Bambi, 5 year old, arrives to the ED with a

complaint of constant headaches, mild facial edema. BP of the upper extremities are 30mmHg higher than the lower and femoral pulses are hard to palpate…

♥ Harry is a 12 year old who collapses suddenly when playing soccer…

Obstruction to Systemic Blood Flow

Signs and symptoms of low cardiac output:• decreased perfusion to the gut (may lead to future

complication of necrotizing enterocolitis)• shock (critical aortic stenosis)• myocardial dysfunction

Elevated LA, RA pressures; CHF, pulmonary edema

Left ventricle failure overtime due to stress of pumping against high resistance

Defect may be ductal dependent

Obstruction to blood flow: Coarctation of the Aorta

Narrowing of Aorta Pressure to head,

pressure to body Bounding upper pulses,

lower pulses Systolic difference

between UE and LE ~ 15-30mm Hg

Hypotension and acidosis May need PGE1 pre-op Post-op considerations

http://pediatriccardiology.uchicago.edu.

Obstruction to blood flow: Aortic Stenosis

♥ Narrowing: above, at, or below aortic valve

♥ May be CRITICAL:– cardiac output-LVOT

obstructed♥ LVH

– Increased LAP – Reflects increased

pulmonary vascular congestion

– coronary artery perfusion myocardial infarction

Here is another….

♥ A 3 year old child presents to the ED with a history of increased respiratory infections, increasing fatigue with play with a tendency to sweat “buckets”, difficulty gaining weight despite frequent meals.

♥ The child is tachycardic and has a respiratory rate of 50. A CXR shows increased vascular markings and a large cardiac silhouette.

Increased Pulmonary Blood Flow

Left to right blood flow pattern (shunting)What color will the child be?

May lead to congestive heart failure: alteration in oxygenation tachypnea, tachycardia diaphoretic, periorbital edema

Clinical picture: small thin infant/child with history of feeding issues and pneumonias

Increased Pulmonary Blood Flow

♥ Remember that oxygen INCREASES pulmonary vasodilation and blood flow through the lungs.

♥ What will giving oxygen to your patient do? Think with a NURSING Focus!!

♥ Risk for developing pulmonary hypertension.

Increased PBF: PDA♥ Ductus does not close♥ Preterm infant, acidemia,

hypoxia♥ Blood flows from Ao PA

(left to right)♥ PVR, SVR determine

hemodynamics♥ Increased pulmonary blood

flow and RVH♥ Female:male 2:1

http://www.childrenshospital.org/az/Site507/Images/ei_0350.gif

Increased PBF: ASD♥ L>R shunt♥ blood to RA /RV♥May/may not be

symptomatic♥ Risk:

♥atrial dysrhythmias

♥ stroke/emboli♥pulmonary

HTN, Eisenmenger’s

http://www.childrenshospital.org/az/Site477/Images/normal_large.gif

Increased PBF: VSD

♥ L>R shunting (acyanotic)♥ blood to RV ♥ Over time: can lead to

PHTN and RVH♥ CHF is common, occurs

typically at 2-4 weeks 2o

decreasing PVR♥ Spontaneous closure can

occur if small (muscular type)

http://www.childrenshospital.org/az/Site500/Images/ei_0435.gif

Increased PBF: CAVC/AVC♥ Endocardial cushion defect

• Low ASD• High VSD • Clefts of mitral and

tricuspid valves

♥ Flow determined by:• PVR and SVR,

chamber compliance, ventricular pressures, symmetry of the ventricles

♥ Highly associated w/Down Syndrome-40-60%

What do you think of this child?

♥ Within 24 hours after birth, the baby looked “shocky” and deeply cyanotic.

♥ Heidi is a 20 day old infant brought to the ED in respiratory distress, grunting, flaring, hypotensive, bradycardic.

Variable PBF: TGA

www.driscollchildrens.org

Transposition of the Great Arteries (TGA)

♥Blood flows in parallel circulation

♥The Aorta arises from the Right Ventricle

♥The Pulmonary Artery arises from the Left Ventricle

♥Child presents with profound cyanosis

TGA

♥Without one/both of the fetal structures (PDA and PFO), the child will die within minutes of birth

♥Emergency procedure in the cath lab or even at the bedside to restore mixing of blood

Jantene ProcedureArterial Switch Procedure

♥The vessels are surgically moved and re-anastomosed to the correct ventricle as early as possible.♥ If not done early, ventricles

will fail to pump correctly

♥Linked to mothers w/ IDDM

Variable PBF: HLHS

♥ Descending Ao PDA dependent

♥ Flow to systemic and pulmonary circulation are dependent on PVR and SVR

http://www.childrenshospital.org/az/Site502/Images/ei_0248.jpg

Variable PBF: HLHS

♥ Norwood Procedure (Stage I):• anastomosis of

the main pulmonary artery to the aorta to create a “neo-aorta”

• BT shunt• ASD

http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/hlhs.htm

Stage 2: Hemi-Fontan

Bi-directional Glen or Hemi-Fontan

Goal is to maintain the flow of deoxygenated blood to the lungs by attaching the superior vena cava directly onto the pulmonary artery

http://www.chc-pa.org/PrinterFriendly.cfm?PID=1078

Fontan Procedure

♥ Attach the IVC to the RA and PA.

♥ All returning deoxygenated blood directed straight to the lungs.

♥ Will cause an increase in pressure in the lung vessels

♥ Small fenestration or hole will be created in the baffle in the atrium

♥ Fenestration acts as “pop off” valve

http://www.chc-pa.org/Patient_Info.cfm?ID=1079

A word about single ventricle physiology after the Fontan...

♥ Remember that one functional ventricle pumps to the systemic circulation.

♥ The ventricle may become overloaded.♥ Pulmonary blood flow is passive, non-pulsatile

directly from the SVC and IVC to the lungs.♥ If the PA pressure is normal, the systemic venous

pressure should allow for forward flow by exceeding pulmonary artery resistance.

Goal is to find the balance between adequate oxygenation & systemic perfusion

Post-operative Care/Management

Post-operative Care/Management

♥ Bleeding♥ Arrhythmia/Pacing♥ Heart failure/poor

function♥ Hypoxemia♥ Pleural Effusions♥ Pneumonia♥ Temperature instability

♥ Acid-base balance♥ Fluid/electrolyte

management♥ Renal Complications♥ Neurologic

Complications♥ Infection♥ Pharmacologic Support

Post-operative Care/Management♥ Bleeding♥ Arrhythmia♥ Heart failure/poor

function♥ Hypoxemia♥ Fluid management♥ Neurologic

Complications♥ Pharmacologic Support

♥ Diuretics, heparin♥ WHY??

♥ Why are these considerations so important??

Why are these considerations so important??

♥ Cyanotic CHD♥ Increased Hgb/Hct (Polycythemia) due to chronic

hypoxemia♥ Increased blood viscosity♥ Diuretics—>decrease amount of extra

fluidfurther increase viscosity.♥ High risk of CVA/Stoke♥ Must monitor hydration status carefully!!

♥ Cannot be allowed to become too dehydrated

♥ Similar considerations for CHF patients

Genetics, Syndromes, and CHD

♥ Down Syndrome♥ Trisomy 18♥ Trisomy 13♥ Marfan’s Syndrome♥ Turner’s Syndrome♥ Noonan’s Syndrome

♥ CHARGE Syndrome♥ 22q Deletion Syndrome♥ Duchenne muscular

dystrophy♥ Velo-Cardio-Facial

Syndrome (VCFS)

Acquired Heart Disease

♥ Kawasaki Disease♥ Rheumatic Heart Disease♥ Myocarditis♥ Cardiomyopathy

Kawasaki Disease

♥ A leading causes of acquired heart disease in children in the US

♥ Etiology unknown ♥ Incidence: 80% are children ages 1-5yrs♥ Pathophysiology involves acute, diffuse, vasculitis of

the medium size arteries and small vessels of the body → particularly the coronary arteries; potential for coronary artery aneurysm

Diagnosis

♥ No definitive laboratory or diagnostic test♥ Diagnosis is based on CDC criteria

♥ Fever >5 days, unresponsive to antibiotics♥ Bilateral conjunctivitis♥ Oral mucosal changes (strawberry tongue♥ Peripheral extremity changes (erythema)♥ Rash♥ Cervical lymphadenopathy

Kawasaki Disease

http://circ.ahajournals.org

Clinical Manifestations and Complications

♥ Aseptic meningitis♥ Increased ESR♥ Leukocytosis♥ Elevated liver enzymes♥ Thrombocytosis

♥ Anemia♥ ECG and

Echocardiography changes

♥ Aneurysm♥ Infarction

Kawasaki Disease

http://circ.ahajournals.org

Management and Treatment

♥ IVIG-IV Gamma Globulin♥Anti-inflammatory agent: reduce inflammation

& decrease incidence of coronary abnormalities♥ Within the first 10 days of symptoms♥ High dose: 2 gm/kg, q 8-12 hours♥ Decreases the acute phase of the illness♥ Hemodynamic monitoring for CHF ♥ Pre-med with Benadryl

Management and Treatment

♥ Aspirin therapy♥ High dose (80-100mg/kg/day) for 2-4 days for

inflammation, fever reduction♥ Anti-platelet doses after fever has been reduced♥ Low dose aspirin during the convalescent phase

♥ Antithrombotic therapy♥ Surgical intervention → if aneurysm is

present

Rheumatic Fever

♥ Rheumatic fever is an inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever).

♥ The disease can affect the heart, joints, skin, and brain.

Rheumatic Fever

♥ Rheumatic fever primarily affects children ages 6 -15, and occurs approximately 20 days after strep throat or scarlet fever.

♥ Can affect valves within the heart, most often mitral and or aortic valve.

♥ Valves often become stenotic, requiring replacement.

Rheumatic Fever

♥ Dysfunctional valves and often lead to left ventricular dilation and congestive heart failure, sometimes decades later.

♥ Vegetation may develop on damaged valves and become infected leading to endocarditis.

♥ Myocarditis is present but is not the direct cause of heart failure.

Rheumatic Fever

♥ Rheumatic fever is likely to come back in people who don't take low-dose antibiotics continually, especially during the first 3 -5 years after the first episode of the disease.

♥ Cardiac complications may be severe, particularly if the heart valves are involved.

Rheumatic FeverDiagnosis

♥ Polyarthritis♥ Myocarditis♥ Subcutaneous skin

nodules♥ Rapid, jerky

movements (Sydenham chorea)

♥ Skin rash♥ Fever♥ Increased

sedimentation rate♥ Abnormal CBC

Myocarditis

♥ Myocarditis♥ Inflammation of the myocardium♥ Can be viral or bacterial

♥ Attempt to shorten the inflammatory/acute phase in order to prevent permanent damage to muscle

Myocarditis

♥ Variable presentation♥ Fatigue♥ Arrhythmias♥ Fever♥ Dyspnea♥ Exercise Intolerance♥ Decreased urine output♥ Edema

♥Legs, face

Cardiomyopathy

♥ Failure of the heart muscle♥ Several types

♥ Dilated, hypertrophic, restrictive♥ Dilated is most common

♥ dilation and impaired contraction of one or both ventricles

♥ Myocarditis often progresses into Dilated Cardiomyopathy (DCM)

Cardiomyopathy

♥ Variable presentation♥ Fatigue♥ Arrhythmias/Cardiac Arrest/Sudden death♥ Fever♥ Dyspnea♥ Exercise Intolerance♥ Decreased urine output♥ Edema

♥Legs & face

Myocarditis/Cardiomyopathy

♥ Nursing Considerations♥ Promote rest♥ Careful I/O monitoring♥ Monitor of arrhythmias♥ Medication administration/teaching ♥ Assist devices and Transplantation are often the

result of these processes.♥ This requires a tremendous amount of care, education

and support for both the child and the family.

http://www.chop.edu

What is theclinical significancepre or post-op?

A few words about arrhythmias….♥ Assess the patient FIRST

♥ Is the child’s clinical condition compromised by the arrhythmia?

♥ Too Fast/Too Slow♥ Treat the underlying cause, if possible

♥ Hypothermia, hypovolemia, electrolyte imbalances

♥ Cardioversion vs. Defibrillation♥ What’s the difference?♥ When to use and with what rhythm?

♥ Pacemakers♥ Temporary and permanent

Cardiac Catheterization

♥ Diagnostic♥ Provide pressures/data♥ Drug Studies-Pulmonary HTN

♥ Interventional♥ Closure of ASD/VSD♥ Coil embolization of collateral vessels♥ Ablation of aberrant conduction pathways

Cardiac Catheterization

♥ Pre-cath Care-Nursing Considerations♥Allergies-shellfish, dyes, food, medications?♥Teaching

♥Patient and family♥ What to expect (warm, flushed feeling w/dye, flat

time)

♥NPO time?♥Assessments/Documentation

♥ Diaper rash??

♥ Pre-medication/Sedation/Anesthesia♥Conscious sedation vs. intubation

Cardiac Catheterization♥ Post-cath Care

♥ Site check♥ Groin, neck, brachial♥ Bleeding, hematoma♥ Distal circulation

♥ Flat time-4-6 hours♥ How to do this in a child?? ♥ Nursing Considerations

♥ Parental assistance, distraction, medical play, environmental modifications, medications.

Nursing considerations

♥ Patient education/support issues♥ Age appropriate answers to questions♥ Medical play ♥ Prepare child for what they will experience♥ Do not lie or “sugar coat”♥ Offer reasonable choices

♥ “whens” and “wheres” ♥ helps the child to feel in control

Nursing considerations

♥ Parental Support♥ Stressful-may not have comparable coping

strategies♥ Can “make” or “break” relationships

♥ Marriage/relationship issues♥ High divorce rates

♥ Bonding issues♥ include parents in child’s care-ADLs, diapering♥ Family-centered care, family rounds

♥ Social work, chaplain services

Psychosocial/Family Considerations

♥ Stress♥ Chronically ill child♥ “Will my child live?”♥ “Will my child ever be normal?”

♥ High prevalence of learning disabilities, behavior issues

♥ Siblings♥ Long periods of separation from parents/sibling♥ Death and dying questions

♥ Work/financial considerations

Psychosocial/Family Considerations

♥ Nurses play a pivotal role in supporting families.

♥ Social work, child life therapists, PT/OT, educators, physicians, external support groups play a tremendous role in helping these families not only cope but thrive and live full, happy, healthy lives.

A mother’s point of view….

• http://www.youtube.com/watch?v=zB8ySBjX2-U

THANK YOU & GOOD LUCK!