Cardiovascular Dysfunction in the Child N425

(ppt adapted from Norene Rouse, RN, MSN, CNS)

Presented by: Barbara Welton, RN, MSN CNS

Cardiac Disease in Children: Statistics

Thousands of infants born each year have congenital cardiovascular defects. Of those who have these defects:
410% have atrioventricular septal defect. 811% have coarctation of the aorta. 914% have Tetrology of Fallot. 1011% have transposition of the great arteries. 1416% have ventricular septal defects. 48% percent have hypoplastic left heart syndrome
Source: 2011 American Heart Association

Etiology of Congenital Heart Disease Most causes are unknown Main structures develop between 6-12 weeks gestation Valve, myocardium, and ductus arteriosis problems can occur later in the pregnancy Some relate to a disease in the mother, such as rubella, viral infection, diabetes, or lupus

Other Etiology
Genetics Environment Medications Illegal drugs Alcohol Chromosomal syndromes Higher incidence if parent, (especially mother) had a defect. Multi-factoral

The Heart
Muscular, four-chambered organ Primary purpose is to pump blood to the body by:
electrical stimulation rhythmic contraction of heart muscle blood flows from an area of high pressure to area of low pressure valves prevent back flow

Normal blood flow through the heart and lungs.

Flow of Blood

1. Blood enters into RA from body. 2. Blood flows from RA to RV. 3. RV blood goes to the lungs through the PA. 4. Blood enters lungs for oxygenation and returns via the PV into the LA. 5. Blood flows from LA to LV. 6. Blood moves from LV to the body via the aorta.

Assessment Priorities
Abnormal heart sounds Color Rales or crackles in lungs Enlarged Liver (firm abdomen) Pulses and refill time Edema FTT

Stroke Volume the amount of blood ejected from the ventricles with
each contraction

Is influenced by: Preload, Contractility, Afterload and HR

Electrical Conduction of the Heart: SA node (pacemaker), AV node, bundle of His (Left and Right bundle branches), and the purkinje fibers.

Frank-Starling Law
In cardiac physiology, the rule stating that cardiac output increases in proportion to the diastolic stretch of heart muscle fibers.

Preload Stroke volume Heart rate Myocardial contractility Afterload

Cardiac output

Frank-Starling Law
The preload in the intact heart is related to the resting length of the muscle fibers which in turn is dependent upon the amount of filling of the chamber. Up to a point, an increase in preload lengthens the myocyte and stimulates a more vigorous contraction (Frank Starling Law) by increasing the number of activated myosin-actin bridges and the amount of calcium released from the sarcoplamic reticulum.

Diagnostic Testing
Blood work ( Hct?) Echocardiogram EKG/Holter monitor Chest X-ray Heart Catheterization Electrophysiology (EP) Stress/Exercise Testing MRI or CT scan

Common Testing for CHD

Echocardiography
Can give information relating to pressures, diameters, ventricular function

Hearth Catheterization
Pros and Cons

Echocardiography:
Transthoracic -M-Mode -2-D -Doppler TEE Fetal Blue = away; Red = towards

4-D flow vector mapping

3D

MRI

Cardiac Catheterization
Study various functions of the heart. Observe vessels and flow when dye injected. Measure Oxygen concentration across the valves and walls (septa) of the heart. Measure pressures within each chamber of the heart and across the valves. Procedure can even be performed in small, newborn infants. Perform procedures once done in surgery.

The Child Going to Heart Cath

Answer questions Keep things simple Validate anxiety Walk through the area Prepare for procedure - Pre-op meds, monitoring - Insertion site prep - Equipment - Diversion activities - Time frame for procedure - Using pictures

Post Heart Catheterization

Focus on child after procedure - bedside report, check site - control/prevent bleeding at site - keeping flat - monitoring of VS - check leg pulses, perfusion, color - light diet initially - comfort measures Ask parents what they know Reinforce/provide appropriate information after MD has discussed with family Home care instructions

"Eisenmengers Syndrome"
When VSD defects go unrepaired into the teen or young adult years, increased pulmonary blood flow can cause changes in the pulmonary vasculature. Eisenmenger Syndrome occurs when the normal left to right shunting that occurs with septal defects switches to a right to left shunt due to the development of increased pulmonary vascular damage leading to resistance. This pressure in the lungs can equal or exceed systemic vascular resistance which causes the reversal of blood flow. This is a cyanotic condition.

Children born with Eisenmenger syndrome are born with a hole between the two chambers -- the left and right ventricles -- of the heart (ventricular septal defect). The hole allows blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body. The increased blood flow and high pressure damages the small blood vessels in the lungs.

Heart Disease in Children

The two types of heart disease in children are "congenital" and "acquired." Congenital heart disease is present at birth. Acquired heart disease develops sometime during childhood or adulthood.

Congenital Heart Disease

These are structural problems About 40,000 children are born with heart defects each year At least 8 per 1000 are born with a heart defect per year About 1 million Americans with cardiovascular defects are alive today. Many adults are being diagnosed now with heart defects

Congenital Heart Disease

Acyanotic
Cyanotic

Systemic arterial Systemic arterial saturation is low. saturation is Pulmonary blood normal. flow can be Pulmonary blood increased or flow is normal or decreased. increased. Mixing of blood Oxygenation is with and without taking place oxygen

Acyanotic Heart Defects

PDA ASD VSD AV canal Coarctation of the Aorta Aortic Stenosis Pulmonary Stenosis

Cyanotic Heart Defects

Tetralogy of Fallot (TOF) (has VSD) Pulmonary Atresia with IVS (no VSD) Transposition of the Great Vessels Total Anomalous Pulmonary Venous Return (TAPVR) Truncus Arteriosus Tricuspid Atresia Hypoplastic Left Heart Syndrome

Atrial Septal Defect (ASD)

Abnormal hole between the two atria. Excess blood moves from L to R through hole causing increased flow to RV and then to lungs. This causes the heart to work harder

Signs & Symptoms of ASD

Usually asymptomatic in infancy and early childhood, may wait to see if close on own. May show s/s of CHF Shortness of Breath Difficulty feeding Poor weight gain Enlarged right heart Pulmonary hypertension can occur in time Atrial dysrhythmias

Treatment of ASD

Stitch repair

Amplatzer

Patch repair
Closure recommended before 5 years of age.

Placing the Amplatzer during Heart Catheterization

Ventricular Septal Defect (VSD)

Most common cardiac lesion Many close spontaneously A hole or holes occurs between the Left and Right ventricles Higher pressure on left causes blood to leak back into the RV Can cause enlargement of RV and then weakening of the muscle Repair to control CHF and maintain adequate growth

Ventricular Septal Defect

(VSD)
Sites

Signs & Symptoms of a VSD

Enlargement of the right heart from increased volume of blood. Possible lung congestion/CHF (moderate sized defects) Arrhythmias Inflammation

Treatment:
Stitches/Patches Closure device-not recommended-too problematic

Patent Ductus Arteriosus

Connects aorta & pulmonary arteries

Increased pulmonary blood flow. Ductus arteriosus fails to close after birth. Normal lungs release bradykinin causing construction of smooth muscle in ductus Hypoxia problems prevent the release of bradykinin so remains open Oxygenated blood flows from aorta back into the lungs.

Signs & Symptoms of PDA

May show no problems initially In time will show s/s of CHF Failure to thrive SOB on exertion Cold sweat Respiratory infections Bounding pulses

Treatment:
Indomethacin or ibuprofen for premature infants Surgery to tie off or divide if large Coil implantation

Patent Ductus Arteriosus (PDA)

Patent Ductus Arteriosus (PDA)

Size of the vessel determines procedure used.

Coarctation of the Aorta (COA)

Pinching or narrowing of part of the aorta that sends oxygenated blood from the heart to the rest of the body. Could extend to a section of the aorta. Increased risk of stroke. May have hypoplastic aortic arch

Signs & Symptoms of Coarc

May not have any symptoms until older or need emergency surgery at birth. Higher BP to upper body so high risk of stroke. Check BP in all 4 extremities. Decreased or absent pulses to lower body. Blood flow to lower body also reduced including the kidneys and gut.

Signs & Symptoms of Coarc cont Increased pressure above the narrow area causing LV to work harder causing ventricle to enlarge then become weakened. Blood then backs up into the lungs and CHF develops. May present as a shelf-like obstruction May present as a tubular obstruction

Repair of Coarctation of the Aorta

Repair approach dependent on proximity of other structures, severity of side effects, and age of child at diagnosis

Subclavian Flap Repair

Coarctation Repair
Uses lateral thoracotomy entry Closed heart procedure End to end or end to side of anastomosis preferred repair Re-coarctation after neonatal repair not uncommon End to End Anastomosis:

Balloon Angioplasty for Coarctation Repair

Effective for reducing discrete coarctation and/or add stent to keep open. Can develop tearing or aneurysm formation in the aorta

Heart Cath procedure:

Coarctation of the Aorta

Post op issues: Incision issues-picking child up High blood pressure dam effect, will come back from surgery with meds to help and go home with meds. Body has adjusted to needing high pressure to make blood flow. Lower part of body may have adjusted to the lesser volume of blood and have smaller vessels. Development of collaterals

Cyanotic Heart Defects

Increased pulmonary blood flow Transposition of the great arteries (mixed defect) Truncus arteriosus TAPVR Decreased pulmonary blood flow Pulmonary atresia Tricuspid atresia **Tetrology of Fallot (TOF)** **Know for exam

Tetrology of Fallot (TOF)

1. PS - severity of
condition depends on degree of obstruction.

2. VSD 3. Overriding aorta 4. Hypertrophic RV

Signs & Symptoms of TOF..

Cyanosis at birth Pink vs. Blue Tet Murmur Crying leads to cyanosis Leads to irritability and then can become unresponsive (Tet spell) Pulmonary artery may be hypoplastic, nonconfluent, and supplied by aortopulmonary collaterals. Size dictates surgical repair

Managing a Tet Spell

A sudden change in blood flow from L R to R L Prolonged spell syncope, seizure, arrest Knees to chest/squatting position to decrease venous return from legs and raise systemic vascular resistance thus decreasing R to L flow. Oxygen Morphine Inderal

Repair of TOF
Palliative repair with Blalock-Taussig Shunt getting blood to the lungs. Open and patch Pulmonary Artery VSD patch

Surgery for CHD (Pre-op)

Knowledge deficit: child/family Promote nutrition (feeding tubes) Altered cardiac output - fluid volume excess, pulmonary hypertension, increased blood flow to lungs, CHF, frequent respiratory infections Activity intolerance fatigue, weakness, disease process, circulatory compromise Risk for infection debilitated physical status Altered growth and development decreased oxygenation

The Post-Op Heart

Decreased Cardiac Output - surgical
complications, bleeding, fluid shifting Altered Comfort incision site, treatments and procedures Risk for infection from surgery, and IV lines Compromised Family Coping from childs surgery, childs hospitalization, lack of support systems, financial considerations Activity Intolerance post op status Altered Growth and Development from underlying disease process

Caring for the Child and Family...

Coping keeping family updated at all times with the plan of care. Educate regarding meds, therapies, and procedures Allow family to help with care when appropriate. ATC visiting. Encourage caring for self. Allow personal items at bedside.

The Discharge
Discuss with Discharge Coordinator Wound care Medications Monitoring progress Nutrition needs Activities Equipment needs Educational needs

Congestive Heart Failure

CHF is a group of signs and symptoms that reflect the hearts inability to effectively pump blood to meet the metabolic requirements of the body. The body wants equal amounts of blood going to the body and the lungs to maintain a fluid balance. When one exceeds the other heart failure occurs.

Etiology of CHF in Children Heart rate- Arrhythmias: SVT, VT,

Bradycardia

Preload- Shunt lesions: VSD, AV canal,

Single Ventricle; Anemia and Volume overload

Afterload- Left heart obstructive

lesions: Coarctation, Aortic Stenosis, HTN

Contractility- Myocardial disease:

Myocarditis, Cardiomyopathy

Clinical Signs of CHF

Tachycardia Pale color Feeding difficulties Diaphoresis especially with feeding Tachypnea with increased WOB Retracting and Grunting and wet lungs Decreased distal pulses and cool extremities Facial edema Distended abdomen, enlarged spleen and liver with weight gain, but FTT

Treatment of CHF
Diuretics:
- Furosemide (Lasix) 0.5 to 2 mg/kg/dose IV - Hydrochlorothiazide (HydroDiuril) 2-4 mg/kg/day - Chlorothiazide (Diuril) 20-40mg/kg/day - Ethacrynic acid (Edecrin) 1mg/kg/dose - Spironolactone (Aldactone) 1-3 mg/kg/day Potassium sparing? Electrolyte changes?

Treatment of CHF
Digitalis Digoxin
- Total Digitalizing Dose 0.04 mg/kg - Give dose, then dose in 4-8 hours
and the last dose in 4-8 hours

- Toxicity: vomiting & arrhythmias

- Rarely causes bradycardia (HR < 90) - More concern regarding heart block - Teach parents to take HR? - What should you teach?

- Maintenance dose: 10 mcg/kg/day divided

Treatment of CHF
Reducing Afterload (ACE Inhibitors)
- Enalapril: 0.1 mg/kg (may increase to 0.2-0.3 mg/kg/day) - Captopril: Neonates: 0.1-0.4 mg/kg/dose every 6-24 hr Infants: 0.5-0.6 mg/kg/day divided every 6-24 hr Children: 25 mg/day divided every 12 hrs Adolescents: begin 25 mg TID, Max 450 mg Works by causing vasodilation of peripheral vessels.

Nursing Care Plan for Child with CHF

Increased caloric requirements Infection control issues-handwashing Energy conservation (frequent naps) Balance fluids and electrolytes (labs) Do they have IV fluids running on top of their feedings? Look at total volume intake. Medication administration Monitor results of above

Acquired Heart Disease

Rheumatic Fever Bacterial Endocarditis Kawasakis Disease

Rheumatic Fever
Autoimmune reaction to Group A Beta-hemolytic streptococcal (GABHS) pharyngitis. It involves joints, skin, brain, heart valves, blood vessels Major cardiac findings of RF is inflammation of the heart in: endocardium, pericardium, and myocardium Less frequent in US due to quick diagnosis and antibiotic treatment of strep throat or scarlet fever.

S/S Rheumatic Fever

Joint inflammation - swelling, tenderness, and redness over multiple joints. Usually the larger joints in the knees or ankles. The inflammation "moves" from one joint to another over several days. Small nodules or hard, round bumps under the skin Change in the child's neuromuscular movements: chorea Rash - a pink rash with odd edges that is usually seen on the trunk of the body or arms and legs. Fever, weight loss, fatigue, stomach pains

Jones Criteria
Major Manifestations*
Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules Aschoff bodies

Minor Manifestations
Clinical features:
Arthralgia and Fever

Laboratory features:
1. Previous infection (ASO) 2. Elevated Sed Rate (ESR) 3. Positive C-reactive protein 4. Prolonged PR interval

Supporting Evidence of Previous Group A Strep Infection: 1. Positive throat culture or rapid streptococcal antigen test
2. Elevated or increasing streptococcal antibody titer

presence of two major or of one major and two minor manifestations indicates a high probability of acute rheumatic fever if supported by evidence of previous group A streptococcal infection.

*The

Jones Criteria Terminology

C-Reactive Protein marker for acute inflammation Antistreptolysin-O titer (ASO) streptococcal antibody tests for previous infection Erythrocyte sedimentation rate (ESR) used to monitor inflammatory or malignant disease Subcutaneous nodules (Aschoff bodies)
Histological findings

Management of RF
Administration of Penicillin Prevent permanent heart damage Treat other symptoms Prevent recurrences of RF through prophylactic use of antibiotics

Nursing management of RF
Encourage compliance with meds especially for long-term therapy. May need monthly IM injections if non-compliant Assist with recovery from s/s-chorea movements are particularly frustrating Emotional support Prevent recurrence

Bacterial Endocarditis
Bacteria in the bloodstream lodge on abnormal heart valves or other damaged heart tissue. Certain normal bacteria that live on parts of your body can enter the system through surgical and dental procedures which cause a brief bacteremia. Although bacteremia is common after many invasive procedures, only certain bacteria commonly cause endocarditis.

Risks of Bacterial Endocarditis

Microorganisms grow on the endocardium that is subjected to turbulence within the heart. An artificial (prosthetic) heart valve Heart valves damaged (scarred) by conditions such as rheumatic fever Congenital heart or heart valve defects (narrowing) Mitral valve prolapse with a murmur

Risks of Bacterial Endocarditis.

Hypertrophic cardiomyopathy Patients who already have some kind of heart problem have a greater risk
Children with congenital, rheumatic, or degenerative heart diseases have a 60-80% risk

Drug abusers have a much higher risk of getting endocarditis.

S/S of BE
Low-grade intermittent fever New heart murmur Anorexia, myalgias, HA Enlarged spleen Petechiae oral mucosa Osler nodes: painful nodules-sign of vasculitis Janeway spots/nodules: painless hemorrhagic areas on palms and soles Roths spots: retinal hemorrhages

A C are Osler Nodes and were painful D is a Janeway Lesion and was non-tender

Bacterial Endocarditis
Treatment: Penicillin IV

for 4-6 weeks

70% streptococcal species 20% staph species 10% other: H flu, fungal, gram negative bacteria May need prophylaxis for procedures but this is changing May need surgical intervention

Kawasakis Disease
A.K.A. mucocutaneous lymph node syndrome This condition was described by Dr Kawasaki in Japan in the 1960s and is an inflammation of the arteries of the body probably triggered by a virus infection. The danger in the disease is that the inflammation can cause damage to the coronary arteries and affect the heart muscle.

Kawasakis Disease
80% are under 5 years of age Peak incidence in toddlers Boys > Girls Without treatment, 20-25% can develop damage to blood vessels that supply the heartesp. Coronary dilation Greatest risk in <1 yr of age Will form aneurysms

Kawasakis Disease
Specific cause is unknown
(possibly a virus)

Progressive inflammation of small and medium sized vessels (vasculitis). Walls can become damaged to the point of developing coronary artery aneurysms in some which can lead to blood clot formation.

Phases of Kawasakis
Acute phase: abrupt onset of high fever that doesnt respond to antibiotics or antipyretics. Symptoms then progress Subacute phase: resolution of fever, high risk of developing coronary artery aneurysms. Peeling begins Convalescent phase: all clinical signs have resolved. Lab values altered. Resolved at the end of 6-8 weeks after onset.

S/S of Kawasakis Disease

Requires 5 of 6 principle criteria Fever ranging between 38-41 C Discrete conjunctival injection without exudate Changes in the mouth consisting of: --Erythema of lips --Strawberry tongue --Diffuse oropharyngeal erythema

S/S of Kawasakis Disease

Polymorphous erythematous rash Changes in the hands and feet consisting of: --Firm indurative peripheral edema --Diffuse erythema of palms and soles --Convalescent desquamation of digits, palms, and soles Unilateral cervical lymphadenopathy

Treatment for Kawasakis

Reduce fever, rash, joint inflammation, pain and to help prevent blood clots from forming. --Aspirin 80-100 mg/kg/day, every 6 hours (once fever resolved reduce aspirin to 3-5 mg/kg/day, give daily) Decrease the risk of developing coronary artery abnormalities (when given early) --IV Immune Globulin (2g/kg) give over 10-12 hours (special blood product)

Treatment for Kawasakis

For patients with coronary artery aneurysms, blood clots or high risk: Warfarin (Coumadin), Lovenox (injection) High dose aspirin Once the fever has resolved aspirin dose is reduced. Once stable child can go home. Aspirin is usually continued for weeks or indefinitely. F/U monitoring