CYANOTIC HEART DISEASE

2. MIXED BLOOD FLOW

 Cardiac anomalies that involve mixing of blood from the pulmonary and systemic circulation
in the heart chambers
 The mixing results in a relative deoxygenation of systemic blood flow, although a cyanosis
is most always visible.

a. TRANSPOSITION OF GREAT ARTERIES

 The aorta arises from the right ventricle instead of the left and the pulmonary artery
arises from the left ventricle instead of the right.
 Blood enters the heart from the vena cava to the right atrium right ventricle
out to the aorta to the body completely deoxygenated
returns to the vena cava.
 A secondary source of blood enters the heart from the pulmonary veins left
atrium left ventricle pulmonary artery lungs to be oxygenated
returns to left atrium
 Atrial and ventricular septal defects occur in connection with transposition
 Occur in large newborns ( 9-10lbs)
 Occur most often in boys

Assessment:
 Cyanotic from birth
 Enlarged heart
 Heart changes
 Low oxygen saturation

Diagnosis:
 Echocardiography – reveals enlarge heart
 ECG – reveals heart changes
 Cardiac catheterization – reveals low oxygen saturation

Therapeutic Management:
 PGE1/prostaglandin – IF NO septal defect
 To keep the ductus arterisus patent
 Balloon atrial septal pull-through operation
 A deflated balloon catheter is passed from the right atrium through the foramen ovale into the
left atrium
 The balloon is then inflated and the catheter is drawn back into the right atrium---enlarging the
opening of the foramen ovale and creates an artificial ASD.
 Done at week to 3 months of age
 Involves an arterial switch procedure in which the major vessels are switched in position.
 Survival rate is 95%
 b. TOTAL ANOMALOUS PULMONARY VENOUS RETURN
 The pulmonary veins return to the right atrium or the superior vena cava instead to the
left atrium
 For blood to reach the systemic circulation, it must shunt across a patent foramen ovale
or a PDA.

Assessment:
 Absent spleen
 Mildly cyanotic
 Easily gets tired

Therapeutic Management:
 Surgery – reimplanting the pulmonary veins into the left atrium.
 Balloon atrial septal pull-through- to enlarge a foramen ovale
 Maintain on a continuous IV infusion of PGE1 to help keep the ductus arteriosus

c. HYPOPLASTIC LEFT HEART SYNDROME

 The left ventricle is nonfunctional.
 Lacks adequate strength to pump blood into the systemic circulation
 Causes the right ventricle to nypertrophy as it tries to maintain the entire heart action.
 There may be accompanying mitral or aortic valve atresia.

Assessment:
 Mild to moderate cyanosis
 Deoxygenated blood is shunted across the foramen ovale because of the greater pressure on
the right

Therapeutic Management:
 Ultrasound – prenatally
 Echocardiography
 Prostaglandin therapy – to maintain a PDA---to increase blood supply to the aorta
 Heart transplantation – to prolonged the child’s life