Cardiac Round 2
Defects with decreased pulmonary blood flow, cyanotic disorders:
Pulmonary Stenosis
Tetralogy of Fallot
Transposition of the Great Arteries
Tricuspid Atresia

Pathology:
The amount of blood to the pulmonary artery is decreased
Shunting of unoxygenated blood from the right side to the left
Mixing of oxygenated and unoxygenated
Infants are hypoxic and cyanotic
Polycythemia (^ hematocrit)
Erythrocytosis (red cell production increases)
Increased blood viscosity
Increases ventricular work

Pulmonary stenosis:
Pulmonary_Stenosis_heartdefectsforeveryone.blogspot.jpg
Narrowing of the pulmonary value
This obstructs blood flow from the right ventricle to the lungs
Incidence - 8-10%

Manifestations:
Mild to moderate can be asymptomatic
Severe – dyspnea with exertion, fatigue, cyanosis
Murmur

Treatment:
Valvuloplasty during cardiac cath
Surgery valvotomy

Tetralogy of Fallot
4 components
VSD
Pulmonary stenosis
Right ventricular hypertrophy
An overriding aorta
9-14% of cases of heart disease, most common cyanotic heart defect

Right ventricle hypertrophied because of pumping against resistance at the stenosis
Of the pulmonary artery

Manifestations:
Vary depending on degree of malformation
Profoundly cyanotic or not
Tet spells result during exertion such as feeding, crying, defecating
Result of decrease in pulmonary blood flow

Diagnosis:
Echo

Treatment:
Surgical intervention

Nursing Interventions:
Knee-chest position for tet spells, squatting
Supplemental oxygen
Decrease blood viscosity
Surgical repair at 3-12 months


Transposition of the Great Arteries:
Great vessels reversed
Incidence 7-8%
Parallel circulation with no oxygenated blood getting to the system
Only chance for survival is the PDA, ASD, PFO
Prostaglandin therapy

Manifestations:
Cyanosis not responsive to oxygen
Appear otherwise healthy

Diagnosis:
ECHO
Sometimes cardiac cath.

Treatment:
Stabilization
Prostaglandin therapy
Atrial septostomy


Defects that obstruct left ventricular outflow
Coarctation of the Aorta
Aortic Stenosis
Hypoplastic left heart syndrome

Manifestations
Decreased cardiac output – shock, acidosis, death
Older child asymptomatic, upper extremity hypertension, diminished pulses in the lower extremity

Diagnosis:
Echo
MRI

Treatment
Surgical
Balloon dilatation
Control hypertension

Coarctation of the Aorta
coarctationoftheAorta_by_mykentuckyheart.com.jpg
Narrowing of the aorta most commonly across from the PDA
Incidence 8-11% CHD

Pathology:
  • Narrowing of the aorta, which sends blood to the body.
  • Like a kink in a garden hose pressure builds up on the other side, the aortic ‘kink’ causes left ventricular hypertrophy (enlargement) as it pushes against the pressure

Clinical manifestations:
*Blood pressures in the upper extremities are higher than the lower extremities (should be approximately the same)*

Diagnosis:
Examination
Echo
MRI possibly for more details

Treatment:
Relief of obstruction by balloon dilatation or surgery (which cuts out the kink and reattaches the ends)
Surgery at 3-5 years of age so it will not reoccur
Medications utilized are medications for hypertension



Aortic Stenosis –
Narrowing of the aortic valve
Incidence 3-6%

Pathology:
Increased ventricular work, enlargement (hypertrophy)

Manifestations:
Diagnosis usually not made until the child is older unless severe
Murmur
Exertional dyspnea
Angina



Hypoplastic Left Heart Syndrome:
Lack of development of the left ventricle (pump) which circulates oxygenated blood to the body through the aorta.

hypoplastic-heart-syndrome.jpg

Manifestations:
Cyanotic
Cardiovascular collapse, hypotension, tachycardia, cyanosis, tachypnea

Diagnosis:
Echo

Treatment:
Used to be inoperable, comfort measures until death
Died after the PDA closed
Presently 3 options
  • No treatment
  • Cardiac transplantation
  • Palliative surgery, prostaglandins, O2 <21%, or CO2 causes vasoconstriction of pulmonary
  • O2 sat levels ~ 75%




Survivors:Hypoplastic Heart Survivors




References:

O'Brien, P., & Baker, A. L. (2009). Chapter 25: The child with cardiovascular dysfunction. In M. J. Hockberry & D. Wilson (Eds.), Wong's essentials of pediatric nursing (8th ed., pp. 861-910). St. Louis, MO: Mosby Elsevier.

Pictures/Videos:
Stenosis pictures located at heartdefectsforeveryone.blogspot.com

Tetralogy of fallot video located at

Transposition of the Great Vessels videos located at:

Coarctation of the aorta image located at mykentuckyheart.com

Hypoplastic left heart syndrome image located at
http://www.pediatrics.wisc.edu/research/research-groups/hokanson/congenital-heart-diseases.html

WYFF4 Left Hypoplastic Heart Interview Emersion Rose Smith
http://youtu.be/haRrrq9gVdI