Topic 4: Heart Defects (In-class notes)

congenital heart disease

interference in the development of the heart structures during fetal development; septal walls/valves may fail to develop completely, BVs/valve may be stenotic, narrowed, or transposed, fetal circulations structures may not close after birth

• increased pulmonary blood flow

• obstruction of blood flow from ventricles

what can cause acyanotic defects

• decreased pulmonary blood flow

• mixed blood flow 

what can cause cyanotic defects

• atrial septal defect (ASD)

• ventricular septal defect (VSD)

• patent ductus arteriosus (PDA)

• AV canal

what are the types of acyanotic defects that cause increased pulmonary blood flow

• coarctation of the aorta

• aortic stenosis

• pulmonic stenosis

what are the types of acyanotic defects that cause obstruction of blood flow from the ventricles

• tetralogy of fallot (TOF)

• tricuspid atresia

what are the types of cyanotic defects that cause decreased pulmonary blood flow

• transposition of the great vessels

• hypoplastic left heart syndrome

what are the types of cyanotic defects that cause mixed blood flow

• heart rate and rhythm

• EKG

• murmur

• S2 splitting

• S3

• pulses: central vs peripheral, upper vs lower extremities

• BP: upper vs lower extremities

• skin temp

• cap refill

• pallor/mottled?

what does circulation assessment for children with heart disease include

• birth history

• history of syndrome/congenital defects

  • especially down syndrome

• CXR to look at the size of the heart and congestion of the lungs

• EKG to look for dysrhythmias

• hemodynamic monitores to get pressures in the heart chambers

• holter monitor

• echo

• doppler

• cardiac cath/angiogram

• stress tests

• electrolytes: K, Mg, Ca

• BUN/Cr

• LFTs

• SpO2 and ABGs

• CRP and ESER

• D dimer

• Lactic acid

what information (history, diagnostics, labs) may be relevant to have for a child with a heart defect

• evaluate CO

• determine structures and blood flow patterns

• corrective/palliative interventional procedures

• measure O2 saturation and pressure in chambers

what are rationales for cardiac catheterization

• bleeding/hemorrhage

• arrhythmias (most deadly complication that requires call to the HCP for orders)

• exposure to radiation/dye

• clotting

• hematoma

• valve damage

• stroke

what are common complications of a cardiac cath

• ensure pressure dressing remains dry and intact

  • may need a sandbag to occlude the artery

• patient must lay flay for 6 hours

  • may have to sedate younger children who can’t sit still

• observe the puncture site for redness, swelling, drainage, and bleeding

• rest and quiet activities for the first 3 days and avoid strenuous exercise

• acetaminophen for pain

• keep follow up appointments

what is nursing care after a cardiac cath

left to right because the left side is stronger, so there is more blood in the RV which increases the amount of blood flowing to the lungs

what is the direction of blood in defects that increase pulmonary blood flow (PDA, ASD, VSD, and AV canal)

• tachypnea and tachycardia as a compensatory mechanism

• heart murmur

  • smaller holes create a louder murmur

• diaphoresis

• poor weight gain due to increased metabolic demand

• frequent respiratory infections due to pulmonary congestion

• HF

what are the general S/S for defects that increase pulmonary blood flow (PDA, ASD, VSD, and AV canal)

The small hole between the aorta and pulmonary artery (ductus arteriosis) did not close after birth, so blood enters the pulmonary artery via the aorta and increases the workload of the left side of the heart; a small amount of blood is lost back into the lungs

what is the issue with patent ductus arteriosus

• indomethacin (Indocin): NSAID/prostaglandin inhibitor causes inflammatory response and spasm which closes the hole

• cardiac cath or ligation via thoracotomy

• coil

what is the treatment for patent ductus arteriosus (PDA)

a passageway or hole in the septum that divides the RA from the LA due to the foramen ovale staying open so it doesn’t go to the LV

what is the issue with an atrial septal defect (ASD)

• if small, many will spontaneously close within the first 18 months of life

• surgically placed mesh patch if not closed by 3 yrs

what is treatment for atrial septal defect (ASD)

an abnormal opening between the right and left ventricles

what is the issue with ventricular septal defect

• dacron patch for large hole

  • requires cardiopulmonary bypass surgery (full open chest procedure)

• HF if not repaired

what is the treatment for a ventricular septal defect (VSD)

• CVICU

• external pacemaker

• chest tube drains

• continuous EKG monitoring

• risk for cardiac and pulmonary emboli and infection

• keep air moving in lungs with positive pressure devices or intubation

what is post op nursing care after cardiopulmonary bypass surgery (full open chest procedure)

instead of separate valves on the right and left, there’s one large valve between the upper and lower chambers and the abnormal valve leaks blood into the ventricles so the heart is forced to work harder and becomes larger

what is the problem with an AV canal defect

• bad APGAR

• blue or purple tint to lips, skin, and nails

• difficulty breathing, very wet/crackly lungs

• poor weight gain and growth

• heart sounds abnormal, can’t even hear a “lub dub” so not a true murmur

what are the S/S of an AV canal defect

pulmonary HTN and HF

what happens if an AV canal defect is not repaired

• diuretics

• inotropes to improve CO and contractility

• ACE inhibitors

• nutrition (feed and grow before surgery)

• surgery around 6-18 months old in multiple stages with several patches and reconstruction needed

what is used to correct an AV canal defect

• have remaining heart defects after surgery

• received an artificial heart valve

• received artificial (prosthetic) material during heart repair

• will receive a heart valve prosthetic in the next 6 months

when should a child take preventive antibiotics before certain dental/surgical procedures after surgical correction of an AV canal defect

• CVICU

• continuous cardiorespiratory monitor

• Swan-Ganz pressure lines

  • pressure should be decreased post op; if increased the repair was a failure

• prevent infection

• external pacemaker

• hydration, fluids, electrolytes, nutrition

• monitor for dysrhythmias

• pain management (morphine)

• early mobilization depending on age and developmental level

what is nursing care for after a child get an AV canal defect repaired

right to left, and deoxygenated and oxygenated blood mix with blood low in O2 getting pumped to the body tissues

what is the flow of blood for disorders with decreased pulmonary blood flow (TOF and tricuspid atresia)

• pulmonary stenosis

• ventricular septal defect (VSD)

• overriding aorta

• right ventricular hypertrophy

what are the four heart defects seen with tetralogy of Fallot (TOF)

pulmonary stenosis

what defect in tetralogy of Fallot (TOF) causes decreased blood flow

• heart murmur

• polycythemia (high RBCs)

• tire easily, especially with exertion

• difficulty with feeding and gaining weight

• mild/cyanotic at birth

• hypercyanotic episodes (blue Tet spells)

  • increased cyanosis, hypoxemia, dyspnea, agitation, comes on with crying

what are the S/S of tetralogy of Fallot (TOF)

• place the infant in the knee-to-chest position to increase systemic volume and preload, helping the heart fill, pump, and increase CO

• use a calm and comforting approach to reduce the metabolic demand

• give morphine SQ/IV to decrease metabolic demand, cause vasodilation, and help them calm down

• IV fluid replacement and volume expansion if needed

• repeat morphine admin IV

• blow by O2

• surgically placed patch over the ventricular septal defect with cardiopulmonary bypass (open heart surgery), repair/replacement of the pulmonary valve, aorta correction over the LV

what is the nursing management of tetralogy of Fallot (TOF)

• CVICU

• continuous cardio-respiratory monitoring

• Swan-Ganz

  • Pressure should be increased post op

• Prevent infection

• External pacemaker

• Hydration, fluids, electrolytes, nutrition

• monitor for dysrhythmias 

what are the post op considerations after surgical repair of tetralogy of Fallot (TOF)

valve between the RA and RV fails to develop so blood comes into the RA and has nowhere to go, also an opening between the right and left ventricle

what is the issue with tricuspid atresia

foramen ovale and PDA

what must remain open to maintain minimally adequate oxygenation for those with tricuspid atresia

• give birth prematurely so that the child has an ASD, which provides a route for some circulation

• keep the foramen ovale and PDA open

• Fontane procedure: 3 stage surgical procedure to bypass the RV altogether by taking the inferior/superior vena cava into the RA and pumps all the blood into the pulmonary artery 

what is the management for tricuspid atresia

coarctation of the aorta

narrowing of the aorta in which blood flow is impeded, causing pressure to increase proximal to the defect and decrease distally; BP increases in the heart and upper portion of the body and decreases in the lower portions of the body; complications include aortic rupture, aortic aneurysm, and CVA

• infants:

  • labored or rapid breathing

  • heavy sweating

  • poor grwoth

  • high BP and bounding pulses in upper extremities

  • cool lower extremities with low BP and weak pulses

  • risk for acidosis due to lactate being released

  • HTN due to activation of the RAAS system

  • pale or gray appearance

• older children:

  • dizziness

  • HAs

  • fainting

  • bloody nose

what are the S/S of coarctation of the aorta

• end-to-end anastomosis

• graft enlargement

• balloon angioplasty

what is management for the coarctation of the aorta

obstruction below the aortic valve, of the valve itself, or above the valve, results in decreased CO, LV hypertrophies, low BP everywhere, and high risk for HF

what is the issue with aortic stenosis

• typically is asymptomatic

• poor feeding

• lethargy

• chest pain when active

• dizziness with prolonged standing

what are the S/S of aortic stenosis

involve a mixing of well oxygenated blood with poorly oxygenated blood that results in systemic blood that contains lower levels of oxygen than what the child need, CO is decreased and HF occurs 

what are mixed congenital heart defects

severe tetragens and exposure to other things in utero 

what are mixed defects usually related to

The pulmonary artery and aorta are transposed from their normal position and there is a hole between the right and left ventricles

what is the issue with the mixed defect of transposition of the great vessels (arteries)

• before surgery keep PDA open to give blood a way to reach the lungs

  • IV prostaglandin E1

• corrective surgery at 4-7 days old

  • balloon septostomy (temporary procedure)

  • arterial switch (Jatene procedure): flips the artiers and closes VSD/PDA if open

what is the management for transposition of the great vessels

• severe cyanosis

• rapid breathing

• increased HR (270s)

• difficulty feeding, poor appetite, poor weight gain

what are the S/S of transposition of the great vessels

all structures on the left side of the heart are severely underdeveloped, the LV is non functioning, and the mitral/aortic valves are completely closed/very small

what is the issue with hypoplastic left heart syndrome (HLHS)

• palliative care

• heart transplant within first few weeks

• three stage palliative reconstruction surgery

what are the only options of care for a child with hypoplastic left heart syndrome (HLHS)

• cyanotic

• unresponsive

• difficulty breathing and feeding

what are the S/S of hypoplastic left heart syndrome (HLHS)

• structural abnormalities

• cardiomyopathy

• dysrhythmias

• electrolyte imbalances

• sepsis

• severe anemia

what are common causes of HF in infants and children

• tachypnea

• tachycardia

• pale, cool extremities

• abnormal weight GAIN

• ascites

• activity intolerance, fatigue

• diaphoresis

• cough, wheezing, grunting

• cyanosis

• poor feeding, anorexia

• splenomegaly, hepatomegaly

what are the S/S of HF in chldren

• provide a neutral thermal environment to prevent fever and cold stress

• treat existing infections

• reduce the effort of breathing 

• use meds to sedate irritable child (morphine)

• provide for rest

• decrease environmental stimuli (limit visitors to just parents)

• cool humidified oxygen via oxygen hood, NC, or face tent

what does management for HF in children include

abnormal immune response to group A strep usually 2-6 weeks after URI

what causes rheumatic fever

• Carditis leads to murmur and chest pain

  • pericarditis: friction rub and pain

  • endocarditis: murmurs and pain

  • myocarditis: decreased CO, HF S/S

• polyarthritis: swollen, hot, painful joints

• SQ nodules over bony prominences

• erythema marginatum: pink rash, pale center

• arthralgia, fever, prolonged PR interval, increased ESR

what are the S/S rheumatic fever

• diagnose and promptly treat strep infection with high-dose penicillin

• anti-inflammatories: salicylates, aspirin

• bedrest

• for carditis: O2, diuretics, fluid and salt restriction, digoxin, ACE inhibitors 

what does management for rheumatic fever include

unknown cause, through to result from an infectious cause

what is the cause of Kawasaki disease

acute febrile illness that causes widespread systemic vasculitis (inflammation and pain of small/medium sized BVs)

what is Kawasaki disease

• very high fever

• B/L conjunctival redness

• oropharyngeal S/S: strawberry tongue

• erythematosus body rash

• induration and edema of extremities

  • redness of palms and soles

  • swelling of fingers and toes

• cervical lymphadenopathy 

what are the S/S of Kawasaki disease

• IV gamma globulin (blood product)

• aspirin, clopidogrel, enoxaparin, warfarin to prevent systemic clots

• take VS frequently

• lower fever

• bleeding precautions

• prevent contractures of swollen/painful joints

• pain managment

what is the treatment for Kawasaki disease

aortic aneurysm

what is the most common cardiac complication of Kawasaki disease