Congenital CV Abnormalities - Severson Flashcards
What is ectopia cordis, and what is believed to cause this condition?
- congenital malformation in which the heart is abnormally located either partially or totally outside of the thorax
- due to failure in folding of the lateral body wall and its meeting with the cephalic fold
How does the dextrocardia develop?
- abnormal folding of the embryonic heart
What developmental structures and events are responsible for an Atrial Septal Defect?
- Excessive resorption of 1° septum
- Incompetent foramen ovale
- due to hypoplastic growth of 2° septum
- Inadequate development of the primary septum
- produces basal opening
- Sinus venosus development defect
How do Atrial Septal Defects (ASDs) manifest after birth?
- Left → Right shunting
- increased pulmonary flow
- only large ones lead to pulmonary hypertension with cyanosis tardive and cardiac failure
- Right ventricular failure
- Paradoxical embolism
What developmental abnormalities occur in ventricular septal defects (VSD)? How are they clinically manifested?
- 90% due to malformation of membranous portion of ventricular septum
- Muscular septum involvement may exhibit multiple sites
- swiss-cheese septum
How are Ventricular Septal Defects clinically manifested?
- Size dependent
- Pulmonary stenosis
- Murmur
- Cyanosis tardive in long-standing cases
What is the role of the bulbar/truncal ridges (aorticopulmonary septum) in the development of Persistent Truncus Arteriosus?
- complete developmental failure of the bulbar & truncal ridges
- aorticopulmonary septum never forms
What is the role of the bulbar/truncal ridges (aorticopulmonary septum) in the development of Transposition of the Great Arteries?
- failure of the normal spiraling of the aorticopulmonary spetum
What is the role of the bulbar/truncal ridges (aorticopulmonary septum) in the development of Tetralogy of Fallot?
- anterior malalignment of the aorticopulmonary septum
- causes tetrad of abnormalities
- VSD
- pulmonary stenosis (tiny pulmonary trunk)
- overriding (large) aorta
- right ventricular hypertrophy develops progressively from resistance to blood flow through the right ventricular outflow tract.
What are the two types of Coarctation of the Aorta that can occur?
- Postductal (adult form)
- 95%
- 2xMales vs. females
- Preductal (infantile form)
What are the clinical manifestations of a Postductal Coarctation of the Aorta?
- Increased BP in arms
- Decreased BP in legs
- Notched ribs
- enlarged intercostal/interthoracic arteries
- Weak pulses in lower limbs
- Complications:
- CHF
- CVA
- rupture
- infection
What are the clinical manifestations of a Preductal Coarctation of the Aorta?
- Patent ductus arteriosus
- Early CHF due to hypertrophy of R. ventricle
- Cyanosis in lower half of body
What clinical situations may prevent closure of the Ductus Arteriosus?
- Transposition of the Great Arteries
- Pulmonary stenosis & Atresia (orifice closed)
- Coarctation of Aorta
- Aortic stenosis, & Atersia
- Preterm birth (lungs not developed)
- Congenital Rubella Syndrome
- Down Syndrome
How might a Patent Ductus Arteriosus be treated?
- Premies/Newborns:
- Indomethacin up to 1 week
- 1+ week Postnatal:
- Surgery with catheter-placed occluder
What are the clinical features of Patent Ductus Arteriosus?
- “Machine-like” murmur
- Maternal rubella (german measles) during early weeks of pregnancy
- Infectious endocarditis
- Pulmonary hypertension
- Slightly enlarged liver