Common cardiac dysmorphologies Flashcards
Cardiovascular dysmorphologies are what? what a the two categories?
1.cardiovascular dysmorphologiesare defects due to abnormal heart and great vessels formation during the embryonic and/or fetal period 2. They can be classified broadly into two categories: Cyanotic heart disease or Acyanotic heart disease
When you hear cyanotic think what? Acyanotic?
- When you hear cyanotic think right‐to left shunts (where deoxygenated blood is placed into circulation 4. When you hear acyanotic think left‐to‐right shunts (which means that good oxygenated blood keeps getting placed back into pulmonary circulation)
What are some right to left shunts?
explain persistent ductus arteriosus?
Cyanotic
Right‐to‐Left
Failure of truncus arteriosus
Does not separate into aorta and pulmonary artery
Results in single great artery that receives blood from both ventricles and feeds the systemic, pulmonary, and coronary circulations
Explain transposition of the great vessels? common in?
Cyanotic
Right‐to‐Left
Aorta and pulmonary trunk are switched. [failure of the aorticopulmonary septum to form a spiral]
Causes two completely separate circulations where the right ventricle is pumping
deoxygenated blood to the body via the aorta and the left
ventricle is pumping oxygenated blood to the lungs via the pulmonary trunk.
For the infant to survive this they require another defect! A shunt that will connect the two circulations: a patent ductus arteriosus or a patent foramen
ovale
Drugs like prostaglandin E can be administered to
the fetus to make sure the ductus arterious stays open
Common in population of diabetic mothers
explain tricuspid atresia?
Cyanotic
Right‐to‐Left
Tricuspid atresia is a congenital defect in which one the tricuspid valve fails to form –so no connection between the right atrium and right ventricle. Therefore babies with tricuspid atresia tire easily, are often short of breath and have blue‐tinged skin.
Because the right ventricle is not receiving systemic blood to pump it becomes hypoplastic.
For the infant to survive this they require two additional defects! A shunt to connect the two atria (patent foramen ovale) and a ventricular septal defect.
what is tetrology of fallot?
Cyanotic Right‐to‐Left
Tetralogy of Fallot a group of 4 congenital heart defects that occur simultaneously that usually results from issues with the spiral septum that normally divides the truncus arteriosus into the pulmonary trunk and aorta. • VSD ventricular septum defect
- pulmonary stenosis (@one or more of: infundibulum, pulmonary valve, pulmonary trunk)
- Overriding aorta / dextropositionof aorta (ascending aorta straddles a ventricular septal defect and simultaneously receives blood from both left ventricle and right ventricle)
- RVH (radiograph may display “boot shaped heart”); right ventricle hypertrophy is a consequence of high resistance of blood out the right ventricle due to stenosis
how does squatting help an individual with Tetralogy of fallot?
Explain total anomalous pulmonary venous return
Cyanotic Right‐to‐Left
Total anomalous pulmonary venous return (TAPVR)
“In a baby with TAPVR, oxygen‐rich blood does not return from the lungs to the left atrium. Instead, the oxygen‐rich blood returns to the right side of the heart. Here, oxygen‐rich blood mixes with oxygen‐poor blood. This causes the baby to get less oxygen than is needed to the body. To survive with this defect, babies with TAPVR usually have a hole between the right atrium and the left atrium that allows the mixed blood to get to the left side of the heart and pumped out to the rest of the body. Some children can have other heart defects along with TAPVR, aside from the atrial septal defect. Because a baby with this defect may need surgery or other procedures soon after birth, TAPVR is considered a critical congenital heart defect.”
what are the left to right shunts?
Explain atrial septal defects?
Acyanotic Left‐to‐Right
Failure of the Septum Secundummost common (90%) • Incomplete or fenestrated oval fossa
- Usually near center of septum
- Usually not associated with other defects
Murmur (pulmonary valve) Usually asymptomatic until 30’s
Explain a patent foramen ovale?
Acyanotic Left‐to‐Right
Persistent patent foramen ovale persistent patent foramen ovaleis a common form of atrial septal defect
a defective septum primum formation is the most common cause due to
incomplete adhesion between the valve of the foramen ovale and the septum secundum postnatally
They are usually acyanotic unless other congenital defects are present which increase right atrium pressure relative to left atrium pressure
up to 25% of all adults have a persistent “probe‐patent foramen ovale” (i.e. big enough to transmit a surgical probe) in the superior part of the floor of the fossa ovalis. However, this defect is small and usually not clinically significant.
if large ,repair of these defects can be achieved surgically by using a transcatheter approach through the IVC and into the atria, where a septal occluderis deployed and secured.
Explain ventricular septal defects?
Acyanotic Left‐to‐Right
ventricular septal defects
- 42% of all survivablecardiac dysmorphologies; occurs more frequently in males than females
- usually found in the membranous interventricular septum due to its incomplete development and lack of fusion with muscular interventricular septum
- higher pressure left ventricle blood is shunted into right ventricle bypassing systemic circuit (i.e. left to right shunt) so ventricular septal defects are usually acyanotic
Incomplete closure of septum Most common congenital cardiac anomaly Only 20‐30% are isolated; usually associated with other anomalies
explain Patent ductus arteriosus
Acyanotic Left‐to‐Right
persistent patent ductus arteriosus
Represents a small portion of survivable cardiac dysmorphologies
higher pressure aortic blood is shunted into pulmonary trunk bypassing systemic circuit (i.e. L to R shunt); generally acyanotic
if large, it must be corrected pharmacologically or surgically(surgical treatment is by direct ligation or a less invasive, catheter‐based device that is threaded through the vasculature and positioned to occlude the patent ductus ateriosus.)
often, children with a patent ductus arteriosus may be fine until they become more active and then experience trouble breathing when exercising and demonstrate a failure to thrive.
a continuous murmur usually is evident over the left sternal border to just below the clavicle
if not treated, pulmonary hypertension or congestive heart failure may result
explain atrioventricular septal defects?
Acyanotic Left‐to‐Right
Embryologic failure of superior and inferior endocardial cushions of AV canal to fuse Incomplete closure of septum Malformation of AV valves
what two additional defects must be present with tricuspid atresia in order for a new born to be viable?
