Congenital Heart Defects Flashcards
Tetralogy of Fallot
cyanotic
When affected babies cry or have a bowel movement, they may develop a “tet spell” where they turn very blue, have difficulty breathing, become limp, and occasionally lose consciousness. Other symptoms may include a heart murmur, finger clubbing, and easy tiring upon breastfeeding.
Squatting!
Tetralogy of Fallot: Risk Factors
cyanotic
A mother who uses alcohol, has diabetes, is over the age of 40, or gets rubella during pregnancy. It may also be associated with Down syndrome.
Tetralogy of Fallot
cyanotic
4 defects:
pulmonary stenosis, narrowing of the exit from the right ventricle
VSD
RVH
an overriding aorta, which allows blood from both ventricles to enter the aorta
Tetralogy of Fallot: Treatment
surgically in 1st yr:
increasing the size of the pulmonary valve and pulmonary arteries and repairing VSD
Tetralogy of Fallot: Tet Spell
squatting: increases systemic vascular resistance and allows for a temporary reversal of the shunt. It increases pressure on the left side of the heart, decreasing the right to left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation
Hypoplastic Left Heart Syndrome (HLHS)
cyanotic
left side of the heart is severely underdeveloped. It may affect the left ventricle, aorta, aortic valve, or mitral valve.
ECG: RAD, RVH, but not indicative of HLHS
HLHS: Treatment
cyanotic
staged reconstructive surgery (Norwood or Sano procedure within a few days of birth, Glenn or Hemi-Fontan procedure at 3 to 6 months of age, and the Fontan procedure at 1 1/2 to 5 years of age) or cardiac transplantation.
remember: RV does the work
Transposition of the Great Arteries
cyanotic
Pulmonary artery + aorta are swapped
two separate “parallel” circulatory systems are created
ECG: RVH, RAD, not specific
Transposition of the Great Arteries
cyanotic
Symptoms:
Fast breathing (tachypnea)
Difficulty breathing (dyspnea)
tachycardia
Poor feeding
VSD, ASD, patent ductus arteriosus may also be present
Truncus Arteriosus
cyanotic
truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. This results in one arterial trunk arising from the heart and providing mixed blood to the coronary arteries, pulmonary arteries, and systemic circulation
Truncus Arteriosus
cyanotic
single artery arising from the two ventricles which gives rise to both the aortic and pulmonary vessels
abnormal truncal valve
right sided aortic arch in about 30% of cases (not shown)
large ventricular septal defect
pulmonary hypertension
complete mixing occurring at level of the great vessel
right-to-left shunting of blood
Truncus Arteriosus
cyanotic
Cyanosis presents at birth Heart failure may occur within weeks Systolic ejection murmur is heard at the left sternal border Widened pulse pressure Bounding arterial pulses Loud second heart sound Biventricular hypertrophy Cardiomegaly Increased pulmonary vascularity Hypocalcemia (if associated with DiGeorge syndrome)
Tricuspid Atresia
cyanotic
complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection.[2] This leads to a hypoplastic or absent RV.
must have ASD, VSD present
Coarctation of the Aorta (acyanotic)
the aorta is narrow, usually in the area where the ductus arteriosus (ligamentum arteriosum after regression) inserts
Coarctations are most common in the aortic arch.
Other defects may be present, typically on left side of heart
Eisenmenger’s Syndrome
the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a VSD, ASD, or less commonly, patent ductus arteriosus) causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt.
