Blood flow through the heart THERAPEUTIC MANAGEMENT

✓ Depends on VSD size

o Small – close spontaneously
o Moderate – cardiac catheterization
o Large (>3mm) – open heart surgery
▪ Sched: before 2 years old to prevent
pulmonary artery hypertension =
cardiac failure
▪ Heart can become infected due to
recirculating blood flow
▪ Uses ECMO machine
✓ Prophylactic antibiotics for 6 months
o Prevents bacterial endocarditis

Atrial septal defect

- An opening present at septum between atria
- Same results as VSD
CLASSIFICATION:
1. ↑ pulmonary blood flow TYPES:
2. ↓ pulmonary blood flow 1. Ostium primum (ASD 1)
3. Obstruction to blood flow leaving the heart o opening at lower end of septum
4. Mixed blood flow – oxy and deoxy blood mix in 2. Ostium secundum (ASD 2)
the heart or great blood vessels o opening near center of septum
o may be asymptomatic until infection starts
Disorders with
ASSESSMENT
↑ Pulmonary Blood Flow (LEFT SIDE)
✓ Harsh systolic murmur over left 2nd or 3rd intercostal
Blood flows from the left side to the right side (greater
space
to lesser pressure) through some abnormal opening or
✓ 2nd heart sound will be auscultated
connection between the two systems or the great
- As the volume of blood crossing, it causes the
arteries.
pulmonic valve to close consistently later
than the aortic valve.
Ventricular septal defect
✓ ECG with color flow doppler
- Most common birth defects ✓ Cardiac catheterization
- An opening present at septum between ventricles - Reveals separation in atrial septum and
- Oxygenated blood is shunted back into pulmonary increased O2 saturation in the right atrium
circulation
- RESULTS: Right ventricular hypertrophy and THERAPEUTIC MANAGEMENT
increased pulmonary artery pressure
✓ Surgery
ASSESSMENT o 1 – 3 years old
o Muse be repaired in girls to prevent emboli
✓ 4 – 8 weeks age in pregnancy
✓ Easy fatigue o Large defect – Silastic or Dacron patch is
✓ Loud, harsh, pansystolic murmur along left sternal sutured to occlude space
border @ 3rd or 4th intercostal space o Postoperatively, observe arrhythmia
✓ ECG with color flow doppler or MRI reveals ▪ Edema can interfere SA node function
- right ventricular hypertrophy (RVH) and
pulmonary artery dilation
Atrioventricular canal defect / THERAPEUTIC MANAGEMENT

✓ IV Indomethacin or Ibuprofen
Endocardial cushion defect
✓ Prostaglandin inhibitors
- incomplete fusion of the endocardial cushion o Lowers PGE1 level and encourages PDA
(septum of the heart) at the junction of the atria closure
and the ventricles. ✓ Insertion of Dacron-coated stainless-steel coils by
- Blood may flow between all 4 chambers of heart cardiac catheterization when child is 6 months – 1
- Same symptoms as ASDs year old
- ECG reveals 1st degree heart block ✓ Surgery
o Due to impulse condition is halted o Ductal ligation
before AV node o If no surgery, child is at risk for heart failure
The SA (sinoatrial) node generates an electrical signal that causes and infectious endocarditis from the
the upper heart chambers (atria) to contract. The signal then recirculating blood
passes through the AV (atrioventricular) node to the lower heart
chambers (ventricles), causing them to contract, or pump. The SA
node is considered the pacemaker of the heart.

THERAPEUTIC MANAGEMENT

✓ Surgery
o Valve repair
o Septal repair
o Postoperatively, observe for jaundice
▪ RBC destruction by newly constructed
valves
✓ Prophylactic anticoagulation and antibiotic therapy
post-op.

Patent ductus arteriosus

- Accessory fetal structure that connects the
pulmonary artery to the aorta
Disorders with
- Usually closes 7 – 14 days
- If fails to close, oxy blood will shunt aorta to ↓ Pulmonary Blood Flow (RIGHT SIDE)
pulmonary artery with deoxy blood because of
• Involves obstruction to pulmonary blood flow
increased pressure in the aorta
• Increased pressure on right side of heart
- Shunted blood returns to left atrium then left
• Deoxygenated blood circulates
ventricle, out to the aorta and shunts back to the
pulmonary artery
- RESULTS: Right ventricle hypertrophy and Tricuspid Atresia
ineffective heart action - complete lack of formation of the tricuspid valve
with absence of direct connection between the
ASSESSMENT right atrium and right ventricle
- As long as the foramen ovale and ductus arteriosus
✓ Wide pulse pressure remain open, the child can obtain adequate
- Low diastolic pressure because of shunt or oxygenation
runoff of blood which reduces resistance o At the point they close, infant will develop
✓ Continuous machinery murmur heard at upper left ▪ Extreme cyanosis
sternal border or under left clavicle in older children ▪ Tachycardia
✓ ECG ▪ Dyspnea
 ASSESSMENT

✓ ECG
✓ Chest X-ray
✓ Echocardiography
- Ultrasound that makes pictures of heart to
show direction and amount of blood flow
through its various parts and blood vessels

THERAPEUTIC MANAGEMENT

- Secondary defects: ✓ Prostaglandin Eq

o Atrial Septal Defect (ASD) o < 1-2 weeks old
o Ventricular Septal Defect (VSA) o Reopens ductus arteriosus between
▪ ASD & VSD allow deoxy and oxy pulmonary artery and aorta to improve
blood to mix, providing at least some blood flow to the lungs or body
oxygen supply to circulate. ✓ Blalock-Taussig (BT) Shunt
o Patent ductus arteriosus (PDA) o establishes a connection between the
▪ Allows blood to pass from aorta to arteries to the body and the arteries to the
pulmonary artery lungs
▪ Allows oxy blood to circulate to the o involves the placement of a small Goretex
lungs to pick up oxygen tube between the artery to the arm
▪ Strains heart and increases blood (subclavian artery) and the arteries to the
pressure in lung arteries lungs (pulmonary artery).
o Pulmonary valve stenosis ✓ Place band around the pulmonary artery
▪ Narrowing or blockage of the - If there is too much pulmonary blood flow
passageway of lungs due to large VSD from tricuspid atresia
o Transposition of the Great Arteries (TGA) - blood flow to the lungs will need to be limited
▪ Reversed positions of aorta and to protect the lungs from becoming damaged
pulmonary artery by too much blood flow
▪ Occurs when VSD is very large ✓ Modified BT shunt
- For inadequate blood flow to the aorta due to
tricuspid atresia with VSD and TGA
- blood from the normal size left ventricle will
need to be routed to the aorta, and the aorta
will usually need to be reconstructed

Pathophysiology:
a) Absence of the tricuspid valve and no continuity
between the right atrium and right ventricle
b) Blood crosses through the patent foramen ovale
into the L atrium bypassing the lungs.
c) Blood reaches the lungs for oxygenation by being
shunted back through PDA.
3 Stages of Hypoplastic Left Heart Syndrome ▪ allows a more normal color in the skin and
Surgery: lips as well as a more normal oxygen
saturation in the blood.
(1) Norwood Procedure ▪ Complications:
▪ within the first 2 weeks of an infant’s life i. Lung problems
▪ atrial septectomy and transection and ii. Heart rhythm problems
ligation of the distal main pulmonary artery.

(2) Bi-directional Glenn Tetralogy of Fallot

▪ Done @ 3-6 months - four abnormalities that results in insufficiently
▪ The superior vena cava is detached from the oxygenated blood pumped to the body.
heart and connected directly to the - POVR
pulmonary artery and the BT shunt is 1. P ulmonary stenosis
removed. 2. O verriding Aorta – positioned over VSD
▪ allows blood from the upper body to flow 3. V SD (usually large)
directly to the lungs to pick up oxygen
4. R ventricle Hypertrophy
without having to be pumped by the heart.
- classified as a cyanotic heart defect
▪ prevents blood that already has oxygen from
returning to the lungs, and thereby keeps the
heart from doing unnecessary work.
▪ some levels of cyanosis will persist

(3) Fontan Procedure

▪ 2 – 5 years old
▪ third operation required to optimize their
circulation
▪ Involves connection of the inferior vena cava
directly to the pulmonary artery, thereby
forcing all blood returning from the body to
pass through the lungs and pick up oxygen
before being pumped to the body.
ASSESSMENT Pulmonary Stenosis
✓ ECG, Cardiac catheterization, Echocardiography - Narrowing of the pulmonary valve or the
✓ Cyanosis, Difficulty feeding, Weight gain failure pulmonary artery just distal to the valve.
✓ Retarded growth and physical development - Inability of the right ventricle to evacuate blood by
✓ Dyspnea on exertion, Clubbing of fingers and toes way of the pulmonary artery because of the
✓ Polycythemia obstruction leads to right ventricular hypertrophy.
✓ Tet spells (hypoxic episodes)
- A sudden, marked increase in cyanosis
followed by syncope.
- Older children will often squat during a tet
spell, which increases systemic vascular
resistance and allows for a temporary
reversal of the shunt.

THERAPEUTIC MANAGEMENT
✓ Prostaglandin infusion
o Keeps ductus arteriosus open which
provides additional pulmonary blood flow
ASSESSMENT
and increases the child’s O2 level
✓ O2 administration ✓ Asymptomatic or have mild right- sided heart
✓ Knee-chest position failure symptoms
o Gives physiologic relief to an overstressed ✓ For severe narrowing:
heart by trapping blood in the lower - Cyanosis
extremities. - R – L shunting across the foramen
✓ Propranolol (beta-blocker) ovale due to of increased right-sided
o Aids in vessel dilation heart pressure.
✓ Surgery - Systolic ejection murmur usually loudest at
o Brock procedure the upper left sternal border
▪ relieves the pulmonary stenosis, VSD, - heavy or rapid breathing
and overriding aorta. - shortness of breath
▪ BPW - fatigue
- rapid heart rate
MNEMONIC: BPW ✓ Chest X-ray
A. Blalock Tausig Shunt (BT shunt) ✓ ECG
- Subclavian artery to pulmonary artery ✓ Echocardiogram
B. Pott’s Shunt ✓ Cardiac catheterization
- descending aorta to L pulmonary artery
C. Waterson’s Shunt THERAPEUTIC MANAGEMENT (SEVERE STENOSIS)
- Ascending aorta to R pulmonary artery
✓ Balloon dilation or Valvuloplasty
o interventional cardiac catheterization
procedure is the most common treatment
choice.
✓ Valvotomy
o surgical release of scar tissue within the
pulmonary valve leaflets that is preventing
the valve leaflets from opening properly.
✓ Valvectomy w/ or w/o transannular patch
o surgical removal of the valve and the
widening of the outflow patch to improve
 blood flow from the right ventricle into the Disorders with
pulmonary artery. Obstruction of blood flow leaving the heart
✓ Patch enlargement
o Patches are used to enlarge the narrowed • Congenital anomalies cause the blood flow leaving
areas. the heart to be obstructed because a vessel or a
✓ Pulmonary valve replacement valve is narrower than usual.
o a surgical procedure that is often • Pressure from blood flow increases prior to the
recommended in adulthood. narrowing and decreases after the narrowing.

Aortic Stenosis
• IV catheters - prevents blood from passing freely from the L
• Arterial line ventricle of the heart into the aorta.
- arterial catheter is a piece of long plastic - Increased pressure
tubing or line that is placed directly into an and hypertrophy of
artery or a blood vessel, when a patient's the left ventricle
blood pressure or blood-gas measurements occur.
need to be monitored, - If L ventricular
- There are four (4)main arteries in which an pressure becomes
arterial catheter can be inserted. acute, pressure in
▪ dorsalis pedis artery in the foot the L atrium also
▪ femoral artery is in the groin area. increases, resulting
▪ radial artery in the wrist in back-pressure in
▪ brachial artery located in the elbow pulmonary veins,
• NGT and possibly
• Urinary catheter pulmonary edema.
- After surgery, the heart will be a little
weaker than it was before, and, therefore, ASSESSMENT
the body may start to hold onto fluid,
✓ ECG
causing swelling and puffiness.
✓ Murmur
- Diuretics may be given to help the kidneys
- Systolic sound heard loudest in the 2nd right
remove excess fluid from the body.
interspace.
• Chest tube
- May be transmitted to the right shoulder,
- a drainage tube may be inserted to keep the
clavicle, and up the vessels of the neck
chest free of blood that would otherwise
If severe:
accumulate after the incision is closed.
✓ Faint pulses
• Cardiac Monitor
✓ Hypotension
- a machine that constantly displays a picture
✓ Tachycardia
of your child's heart rhythm, and monitors
✓ Inability to suck for long periods
heart rate, arterial blood pressure, and
✓ When child is active, chest pain occurs because the
other values.
coronary arteries receive inadequate blood.

THERAPEUTIC MANAGEMENT (SEVERE STENOSIS)

✓ Beta-blocker or Calcium Channel Blocker

o Reduces hypertrophy
✓ Balloon Valvuloplasty
✓ Artificial Valve Replacement
o Children generally continue to receive
anticoagulation or antiplatelet therapy.
o Antibiotic prophylaxis against endocarditis
 Disorders with
Coarction of the aorta
Mixed Blood Flow
- Narrowing of the lumen of aorta
- Difficult for blood to pass • Deoxy and oxy blood mix in the heart and great
- Blood pressure increases proximal to the coaction vessels
and decreases distal to it
- Increased blood pressure in the heart and upper
portions of the body result as pressure in the
Transposition of great vessels
subclavian artery increases. - the aorta and the pulmonary artery are switched

ASSESSMENT

✓ ECG FACTORS IN MOTHER THAT INCREASE RISK:

✓ Absence of femoral pulses. 1. Age over 40
✓ Absent brachial pulses 2. Alcoholism
- Children who have an obstruction proximal to 3. Diabetes
the left subclavian artery. 4. Poor nutrition during pregnancy
✓ Bp in the arms will be at least 20 mm Hg higher than 5. Rubella or other viral illness during pregnancy
in the legs.
✓ ECG, Echocardiography, MRI, Chest X-ray ASSESSMENT

THERAPEUTIC MANAGEMENT (SEVERE STENOSIS) ✓ ECG, Echo, Chest x-ray, cardiac catheterization,
Pulse oximetry
✓ Interventional Angiography (balloon catheter) ✓ Cyanosis
✓ Surgery ✓ Clubbing of fingers and toes
o narrowed portion of the aorta is removed ✓ Poor feeding
and the new ends of the aorta are ✓ Shortness of breath
anastomosed
o scheduled by 2 yrs. Of age THERAPEUTIC MANAGEMENT
✓ Digoxin and Diuretics before surgery
o Reduces severity of CHF from hypertension ✓ IV prostaglandin
o keep the ductus arteriosus open, allowing
some mixing of the two blood circulations.
✓ Balloon atrial septostomy
o create a large hole in the atrial septum to
allow blood to mix.
✓ Jatene / Arterial Switch Procedure
o permanently correct the problem within the
baby's first week of life.
 o switches the great arteries back to the Nursing diagnoses and related interventions
normal position and keeps the coronary
Preoperative Phase
arteries attached to the aorta
o requires cardiopulmonary bypass Deficient knowledge related to cardiac surgery and its
✓ Nursing Care before Surgery: outcome
o ensure oxygen saturation remains normal
for this defect, which is 75% to 85% INTERVENTIONS
o monitoring for signs of increased pulmonary (1) Parents visit ICU before surgery and meet the
blood flow staff who will care for the child
o support the family as they wait for their (2) Prepare parents for amount of equipment that
newborn to undergo heart surgery will surround child after surgery
(3) Because the child will need to cough, breathe
Cardiac Surgery deeply, and use incentive spirometry to help the
lungs expand after surgery, introduce these
exercises and the idea of chest tubes and
supplemental oxygen preoperatively so a child
(1) A child must be healthy before cardiac surgery.
can learn that these will be expected.
(2) Vital signs (blood pressure, temperature, pulse,
respiration, and oxygen saturation) will be noted to
Outcome Evaluation:
establish baseline values.
» Parents and child accurately state the reason for
(3) An accurate height and weight are also recorded
surgery and expected outcome.
(4) Laboratory values must be checked, specifically
chemistry, hematology, and coagulation values.
(5) Blood products are typed and cross-matched.
Postoperative Phase
(6) It is also important to ensure that consents for Risk for ineffective cardiopulmonary tissue perfusion
surgery and anesthesia have been signed by the related to depressed myocardial function after surgery
family and the appropriate provider.
INTERVENTIONS
(1) Taking accurate vital signs is essential in the
immediate postoperative period.
(1) Initial management occurs in ICU until
(2) Continuous cardiac monitoring
hemodynamically stable and lines are removed
(3) Assisted ventilation with endotracheal intubation
(2) Require Cardiopulmonary Bypass (CBP), which
is frequently necessary.
allows the surgeon to manipulate the heart in a
(4) Blood pressure will be monitored:
bloodless field while maintaining perfusion and
• directly via intra-arterial catheter
oxygenation to the body
• indirectly via automated blood pressure
▪ Child is cooled to decrease metabolic
recording device.
demand and oxygen consumption, thereby
(5) Hemodynamic monitoring through a central
protecting the brain and other organs during
venous catheter monitors the central venous
the low circulatory flow that occurs with CPB.
pressure (CVP) from immediately above the right
▪ the body responds to the CBP by releasing
atrium as an indicator of preload.
mediators, which cause inflammatory
(6) Pulse oximetry and Near-infrared spectrometry
responses, capillary leaks, and hematologic
(NIRS) monitoring
abnormalities.
(7) An indwelling urinary catheter is inserted at the
▪ Acute complications
time of surgery so urine output can be carefully
i. fluid shifting out of the IV space to tissue
measured and recorded postoperatively.
ii. poor urine output
• Preferred urine output after surgery is
iii. edema
approximately 0.5 to 1 ml/kg/hr for infants
iv. poor circulating volume
and will be weight-based for older children.
v. Anemia
(8) Laboratory tests such as arterial blood gases,
vi. Altered coagulation
hemoglobin, hematocrit, clotting time, and
▪ These effects will be greater if the bypass run
time was long.
 electrolytes must also be monitored closely to Impaired gas exchange related to atelectasis and/or
assess postoperative hemodynamic status. collection of fluid in the chest cavity (pleural effusions)
(9) IV inotropic agents such as milrinone (Primacor) or
INTERVENTIONS
dopamine may be added to enhance cardiac
output and decrease afterload. (1) Measures to Prevent Impaired Gas Exchange.
(10) Sternal closure can be delayed after cardiac • Endotracheal suctioning is necessary while a
surgery, usually in small infants with complex child is receiving ventilatory assistance to
cardiac surgical repairs. prevent secretion accumulation in the lungs
• Allowing the sternum to remain open, until or obstruction of the endotracheal tube.
tissue swelling resolves, prevents • Assist with chest physiotherapy (percussion
compression of the heart that would occur and vibration) as needed to help keep lung
with the closure of the chest, which would secretions mobile.
impact cardiac contractility. • As soon as the endotracheal tube and
• A sterile occlusive dressing is applied to help ventilator are removed, encourage the child
shield the open incision from potential to cough and breathe deeply or use an
infection incentive spirometer at hourly intervals as a
• After swelling and edema have decreased, way to mobilize secretions.
the sternum will be closed (usually about 24 • To minimize pain, administer prescribed
to 72 hours after surgery) analgesia or alert the child to use the
patient-controlled analgesia (PCA) pump 10
Outcome Evaluation: to 15 minutes before it is time to breathe
» Vital signs remain within normal limits deeply.
» Physical examination demonstrates adequate • Teach children how to use a pillow as a
cardiac output. splint while coughing and deep breathing.
• Be certain parents understand that games
Risk for excess or deficient fluid volume related to fluid such as blowing cotton balls or bubbles, or
shifts accompanying cardiac surgery using the incentive spirometer, are
INTERVENTIONS important exercises to help achieve lung
(1) Child may develop relative hypovolemia after expansion.
cardiac surgery because: • Chest tube drainage systems are used after
• Of increased production of aldosterone by complex cardiac surgeries to collect air from
the adrenal glands and an increase in the pleural space, and fluid, typically blood,
antidiuretic hormone secretion by the from the mediastinum, but may be used in
pituitary gland in response to stress. other situations such as isolated pleural
effusions, chylothorax, or pneumothorax.
• cardiopulmonary bypass causes fluids to
(2) Nursing concerns when working with a child who
shift from the intravascular system to the
interstitial spaces because of an has a chest drainage system include:
inflammatory response that causes leaky • Keeping the system closed and below chest
capillaries. level
i. Ensuring suction control chambers are
Outcome Evaluation: filled or set for the ordered amount of
» Child maintains weight suction. If a fluid-filled system, ensure
» Skin turgor is good bubbles are in the chamber.
» Urine output is normal • Making sure the water seal chamber is filled
to the manufacturer’s recommended level
• Monitoring for bubbles in the water seal
chamber, which can indicate either a leak in
the drainage system or an air leak from the
patient’s lungs
 • Assessing and documenting the amount,
color, and consistency in the drainage
system routinely and as ordered.
• Report any drainage that changes in
volume, color, or bloodiness.
• NOT aggressively milking or stripping chest
tubes, as this can cause very high levels of
negative pressure.
• But it is important to keep the tubes freely
draining, which can be difficult if the
drainage is frank blood, which may have
clots in it.
• it is important for the child to get out of bed,
ambulate, cough, or perform pulmonary
toilet maneuvers.
• It is important to obtain a level of pain
control that allows the child to be awake
and interactive.

Outcome Evaluation:
» Child’s respiratory rate remains within age-
appropriate parameters; there are no rales,
crackles, or other adventitious breath sounds;
chest tubes function effectively.