Acute 2 - Unit 1: Congenital Heart Disease (CHD)

Coarctation of the aorta S&S

*Blood pressure that is higher in the arms than in the legs* Weak pulse or no pulse in the groin area Cold legs or feet Nosebleeds Dizziness Fainting Leg cramps with exercise

Exemplar #1 Ventricular Septal Defect (VSD)

*Increased pulmonary blood flow* - L to R shunt (high pressure side to low pressure side) Septum between ventricles. Can be pin prick size, or very large. Larger it is, bigger impact on flow. Murmur heard. Classified as an *acyanotic* heart defect because no unoxygenated blood to body: -It does not cause cyanosis. -There is no mixing of unoxygenated blood into systemic circulation.

Exemplar #2 Coarctation of the Aorta

*Obstruction to blood flow out of the heart* *Acyanotic* Causes high blood pressure before the stricture (wherever it may be) Often occurs with other CHDs

Coarctation of the aorta can lead to CHF because it causes what?

*Pressure overload* which increases afterload. Meaning, what the heart is having to push against, the pressure that's in the system, is really increased because the lumen it's pushing through is narrowed. So it's purely a physical problem. Any SNS activity that leads to vasoconstriction you have even greater pressure that it has to pump against.

Caring for adult patient with Hx: CHD-> complications

- Arrhythmias - Clots (MI,PE, DVT, stroke) - Endocarditis - HTN - Impaired lung function - Fluid overload - Drug side effects - Psych-Social issues (because of multiple hospital visits, or meds that have caused them issues etc.)

Acyanotic CHD

-Increased pulmonary blood flow (L to R shunt) (exemplar = VSD) -Obstruction to blood flow out of the heart (exemplar = Coarctation of the Aorta)

Drug manipulation of PDA

-Indomethicin (and NSAIDs) used to close PDA, but the -Prostaglandin E can be used to keep it open (in the instance of TGA)

Treatment of VSD

-May close naturally during the first 7 years of life (it needs to be monitored) -Large may need surgery (Balloon septostomy- catheter through groin that inserts patch)

Transposition of the Great Arteries Treatment

-Medication: IV Prostaglandin E-->can due in utero (keep PDA open - mix some O2 blood into body) -Surgical: Arterial balloon septostomy (at birth to create ASD or VSD - can happen in utero) Arterial Switch (can do with first week of life)

What can cause TET spells and what can help prevent it?

-constipation->give stool softeners to prevent -too much activity (SOB)-->knee to chest sitting -tx for spasm: give O2, morphine, fluid bolus, possible beta blocker (increased pulm blood flood to reduce impact of spasms).

Pulse Ox Screen for CHD

-goal is >95% on right hand and right foot pulse ox (or <3% difference between them)=negative for CHD (negative is good) -If reading is 90-95% do reading total of three different times with an hour break between each time. If positive for CHD (not greater than 95%) get referral. -If <90% the first time automatic positive and need referral

Services offered by ACHD (adult w/ congenital heart defect) programs

-routine or advanced imaging -cardiac anesthesia guidance (will there be problems with anesthesia when they come in later on for a surgery - there will be a consult) -heart failure management and transplantation evaluation (HF is big concern for CHD, so it needs to be watched closely) -support for young patients transitioning from pediatric to adult care -contraception and family planning (females can have big problems with the increased blood volume that comes with normal pregnancy, they may need meds or tx) -family and patient support and guidance (insurance, appts, support groups) -career and financial guidance (jobs with good insurance, school accommodations

What are the four exemplars of CHD we are supposed to focus on?

1) Ventricular Septal Defect (VSD) 2) Coarctation of the aorta 3) Tetrology of Fallot 4) Transposition of the Great Arteries (TGA)

Name two changes in the flow of blood through the heart that are initiated with the first few breaths of a new born

1. Patent Ductus Arteriosus - chemoreceptors respond to the increased oxygenation after birth closing the PDA 2. Foramen ovale - baroreceptors respond to increased Left sided heart pressure after birth, closing the foramen ovale.

Examples of acyanotic CHD that cause increase pulmonary blood flow

1. Ventircular Septal Defect *exemplar* 2. Atrial Septal Defect 3. Patent Ductus Arteriosis 4. Atrioventricular canal

Amount of test questions

3

Acyanotic vs. Cyanotic

Acyanotic: Only oxygenated blood flowing to the body (and oxygenated blood to lungs). Cyanotic: Un-oxygenated blood flow to the body

What are factors in the mother that may increase the risk for CHD?

Age over 40 Alcoholism Diabetes Poor nutrition during pregnancy (prenatal nutrition) Rubella or other viral illness during pregnancy Some genetic links

Tetrology of Fallot: Overriding Aorta

Aorta is located between R and L ventrical, directly over VSD - that's how O2 poor blood from R ventricle goes directly into aorta, instead of pulmonary artery.

Exemplar #4 Transposition of the Great Arteries (TGA)

Can be classified as a "cyanotic" heart defect because (mixed blood flow): -Aorta is transposed and pulm. artery is transposed - into the wrong places into ventricle. -Blood goes from lungs, picks up O2, returns to the heart, goes back to the lungs and NEVER goes to the body. -Other side of the heart, blood goes from body returns to heart and goes back to the body; it never picks up O2. -2 totally seperate blood flows that never mix at all, and there's no oxygenated blood going to the body. -*This defect in it's pure form is not compatible with life*

What is CHD?

Can involve any area of the heart. Change flow of blood in heart (hemodynamic flow problem). There are more than 35 kinds of defects, and patients can have more than one kind of defect.

What should the nurse do if she/he suspects coarctation of the aorta?

Check blood pressure in all four extremities

Tetralogy of Fallot S&S

Cyanosis--> lips, hands, feet first *"TET" spells*->extreme cyanosis Heart murmur -turbulence of blood flow -very common Poor feeding - Get tired during eating, causes less intake Poor growth - because of poor food intake, and lack of O2 to the body. Clubbing of fingers in older kids - with chronic hypoxemia Low activity

Planning Nursing care goals in patients with CHD

Decrease work of the heart = diuretic Decrease systemic vascular resistance = ACEI Increase effectiveness of contractility = Digoxin Increase circulation to kidneys Increase nutrition (added protein to formula) Tailor activities - very important - tailoring eating position during bottle feeding= each suck gives more volume and keeps them from getting tired out

Cyanotic defects

Decreased pulmonary blood flow Mixed

Exemplar #3 Tetrology of Fallot

Decreased pulmonary blood flow - cyanotic defect. Most complex. Outcome: Not enough blood is able to reach the lungs to be able to be oxygenated and the O2 poor blood flows into the body.

Acyanotic defect: Obstruction of blood flow out of the heart

Due to stenosis: 1. Coarctation of aorta *exemplar* 2. Aortic stenosis 3. Pulmonic stenosis Physically constrict blood flow out of the heart

How to classify CHD

Effect of defect on blood flow: -Increased pulmonary blood flow (left to right shunt) -Obstruction to blood flow out of the heart -Decreased pulmonary blood flow -Mixed blood flow Effect of defect on oxygenation of blood flowing to the body: -Acyanotic No un-oxygenated blood flowing to the body -Cyanotic Un-oxygenated blood flowing to body

Children with acyanotic heart defects rarely, if ever, have episodes of cyanosis while children with cyanotic heart defects are frequently cyanotic: True or false?

False. Any child with CHD can have cyanotic periods. And just because a child has a cyanotic defect doesn't mean they are cyanotic. It's only saying that there is unoxygenated blood flowing out to the body (small amounts don't necessarily lead to cyanosis).

Tetralogy of Fallot treatment: complete

Going in and widening pulmonary blood vessels and widening pulmonary valve (or replace valve), repair VSD (which keeps O2 rich and O2 poor blood from mixing). 6 week healing time. Stay in hospital.

Tetrology of Fallot: Right ventricular hypertrophy

Heart is having to work so hard to pump that extra blood and against the pressure of that stenosed valve. Muscle gets very thick and that then decreases the amount of blood that can actually fill the ventricle.

PDA S&S

Heart murmur Fast Breathing - SOB Poor feeding Poor weight gain Tiring easily Sweating with exertion, such as while feeding

Patent Ductus Ateriosus (PDA) has what effect on blood flow to the lungs?

It increases blood flow to the lungs

Cerebral vascular accident can be a complication of cyanotic heart defects. What physiological compensatory mechanism causes this to occur?

Kids with sever hypoxemia (sensed by chemoreceptors) from these defects develop polycythemia (increased viscosity of blood due to abnormally increased concentration of hemoglobin in the blood). Vasoconstriction from SNS stimulation results in narrower vessels. Thick sticky blood + narrowed vessels = increased chances of thrombosis (usually causing stroke).

Which side of the heart is considered the high pressure side and why?

Left side because of SVR (systemic vascular resistance)

PDA treatment

Medicines to encourage closure of PDA: NSAIDS, Indomethicin If they don't close with meds they will be tx with surgery: Surgery->Catheter/coil->usually wait until symptomatic

Patent Ductus Arteriosus

Present at birth. May be noticeable but may not. Usually have murmur at birth. Usually they will wait a couple of days to see if it will close on its own. If wet lungs occur, or trouble feeding, it is symptomatic and the doctors will want to close it. While it's essential for fetal circulation (or it's helpful in other disorders [like TGA]), it can't remain open if there are issues.

Exemplar #3 four defects involved with Tetrology of Fallot

Pulmonary valve Stenosis Right Ventricular Hypertrophy Overriding Aorta Ventricular Septal Defect (large)

What kind of congenital heart screening does the nurse do 24 hours after an infant is born? How many defects does it screen for?

Pulse Ox Screening, 7

What are the normal pressures of the heart?

Right Atria: 3 Right Ventricle: 25/0-5 Left Atria: 5-10 Left Ventricle: 120/0-10

Transposition of the Great Arteries S&S

Seen soon after birth: Blueness of the skin Clubbing of the fingers or toes Poor feeding Shortness of breath

Ventral Septal Defect S&S

Shortness of breath Pale skin Increased Resp rate Increased HR Frequent respiratory infections Slow growth b/c cant eat. Blood flow is higher in the lungs which makes it harder to swallow/breath. This can damage lungs over time.

Acyanotic defect: Increased pulmonary blood blow (L to R shunt)

Shunt of blood from high pressure side of heart through a defect to low pressure side of heart *(ie: atrial septal defect [ASD], ventricular septal defect [VSD], or patent ductus arteriosis [PDA])*. Increases amount of blood going to lungs and can cause "wet lungs" (increased pressure in lungs and get less O2 exchange over time). Acyanotic because there is only oxygenated blood going to body.

Coarctation of the aorta treatment

Surgery to remove the narrowed part of the aorta and graft it together in some fashion Stenting (angioplasty)

Tetralogy of Fallot treatment

Surgery: Complete (can be by 6 months)-open heart procedure. Better because then kid can have normal growth and development, and then they don't have to come back later for surgery. Temporary or Palliative-->until baby can handle surgery (neonatal)

Cyanotic defect: Decreased pulmonary blood flow

Tetrology of Fallot Tricuspid atresia

Common anomalies (defect) with TGA

To promote life: Patent Ductus Arteriosis Hole or defect in ventricular septum Hole or defect in atrial septum

Examples of Cyanotic CHD d/t mixed blood flow

Transposition of great arteries *exemplar* Total anomalous pulmonary venous return Truncus arteriosis Hypoplastic left heart

Cyanotic defect: Mixed

Transposition of the Great Arteries (TGA) Total anomalous pulmonary venous return Truncus arteriosus Hypoplastic left heart

Tet Spells

Very cynotic period due to stress: Have a hard time breathing Become very tired and limp Not respond to a parent's voice or touch Become very fussy Pass out Blue during times of feeding or crying

Assessment findings indicating CHD

Vital signs changes Heart Murmurs Cyanosis Activity intolerance Growth/Skill Retardation History of frequent respiratory infections

Children with Tetrology of Fallot

Will self -limit activity by squatting. How does this knee-chest/squat position relieve shortness of breath? a. ↓ preload b. ↓ workload You'll see this in kids who have had the temporary repair or no repair at all. (Most kids are completely repaired at infancy)

CHD effect cardiac output through:

a. Volume overload b. Pressure overload c. Decreased contractility d. High cardiac output demands These effects can lead to heart failure.

*Nursing concerns and care for the patient with a congenital heart defect are based on:* Answer?

a. alteration in __________________ of blood b. changes in ___________________ of blood

In CHF the body compensates to increase CO. One method is SNS stimulation. What does the SNS do to compensate?

increase Catacholamines = increase HR +Systemic vascular resistance, decrease Blood flow to kidneys Volume overload Pressure overload Decreased contractility Decreased electrolytes Increased cardiac demands

Transposition of the great arteries is called a cyanotic CHD because

it always causes cyanosis and is not compatible with life UNLESS there is a ASD, VSD etc so some oxygenated blood can circulate

Children with tetralogy of fallot will self-limit activity by squatting. How does this knee chest/squat relieve SOB?

it decreased preload d/t trapping venous blood in the LE via compression

Why are children with VSD prone to developing upper respiratory infections?

overloading of fluid (blood) to the lungs