Anatomic stenosis – causes obstruction to blood flow
•
Pressure load on the ventricle
•
Decreased cardiac output
Clinical manifestations – associated with low cardiac output
TX:
Aortic stenosis and coarcation – treated the same as defects that increase
pulmonary blood flow
Hypoplastic left heart syndrome – treated the same as defects that decrease
pulmonary blood flow and mixed defects
Defects that ↓ Pulmonary Blood Flow
(
Pulmonary Stenosis, Tetralogy of Fallot,
Pulmonary Atresia)
Common causes
•
Obstruction of pulmonary blood flow to lungs
•
No connection of the right-sided blood flow to lungs
Common manifestations
•
Cyanosis
•
Hypercyanotic spells
•
Poor weight gain
•
Polycythemia
Treatment - depends upon defect
Cyanosis:
Hgb reduced by 5 g/100ml in veins
Anemia: O2 sat very low before cyanosis is observed
Polycythemia: cyanosis is obvious at a higher O2 sat
Crying: cyanosis worse. Prolonged
crying should be avoided
Cyanotic spells require immediate treatment.
Managing Hypercyanotic Episodes:
Goal
-to decrease pulmonary resistance
•
Calm the child
•
Oxygen
•
Morphine and propranolol intravenously
•
PRBC
•
Goal – to increase systemic vascular resistance
•
knee-chest position
•
IV fluids to exand circulatory volume
•
Dopamine (sympathomimetic agent)
•
Immediate palliative or corrective surgery
Mixed Defects:
Complex defects
•
Transposition of the great arteries
•
Truncus arteriosus
Cyanosis and congestive heart failure
•
If the pulmonary blood flow is increased –– then
what symptoms????
•
If pulmonary blood flow is decreased – then what symptoms??
TX:
Early intervention to prevent secondary damage
Surgical repair -
See Table 26-7
Palliative surgery initially
Antibiotic prophylaxis for infective endocarditis
Prostoglandin E
1
(PGE
1
) to reopen the ductus arteriosus
Aggressive treatment of hypercyanotic episodes
Cardiomyopathy:
Etiology – primary or no known cause
Symptoms: CHF
Approx. 40% die within 2 years or receive a heart transplant
These diseases enlarge your heart muscle or make it thicker and more rigid
than normal. In rare cases, scar tissue replaces the muscle tissue.
Some
people live long, healthy lives with cardiomyopathy. Some people don't even
realize they have it. In others, however, it can make the heart less able to
pump blood through the body. This can cause serious complications,
including
Heart failure
Abnormal heart rhythms
Fluid buildup
in your lungs or legs
Endocarditis
, an inflammation of the heart lining
Heart attacks
,
high blood pressure
or infections can all cause
cardiomyopathy.
Etiology – primary or no known cause, Viruses-myocarditis
Dilated (and hypertrophic) cardiomyopathy presents with signs and
symptoms of
congestive heart failure (CHF)
. Infants with this disease may
demonstrate fast and heavy breathing with feedings, sweating with feedings,
lethargy or inactivity, or poor weight gain. Older children may have difficulty
