Basic Description of Congenital Abnormalities Flashcards
Supravalvular Mitral Stenosis Ring
Rare
characterized by an abnormal ridge of connective tissue on the atrial side of the MV which can lead to MS
(frequently part of Shone Complex)
Shone Complex
Multiple levels of left heart obstruction are present
Includes Aortic Arch Hypoplasia or Coarctation, AV Stenosis, or Subaortic Stenosis
Eisenmengers
With significant L to R shunting exaggerated volumes over time to lunges cause irreversible damage
R pressure exceeds L pressures and shunt changes direction R to L
Results in decreased oxygen saturation and cyanosis
Situs Inversus
Organs of the chest and abdomen are arranged in a perfect mirror image reversal of normal positions
Aortic Coarctation
Narrowing of the proximal descending thoracic aorta just upstream from the entry of the ductus arteriosus
Marfans Syndrome
Gene defect in the coding of fibrillin a part of elastic fibers
Sinus of Valsalva Aneurysm
Aneurysm of one of the sinus; appears as a thin dilated area that projects into adjacent cardiac structures often within a fistula
Kawasaki Disease
A febrile illness that can dilate the proximal coronary arteries and cause LV dysfunction in children
Myxomatous MV Disease
Thick and redundant leaflets and apparatus, often presents as MVP
Patent Foramen Ovale (PFO)
Failure of foramen ovale to close (25% incidence) results in PFO
Partial Anomalous Pulmonary Venous Return (PAPVR)
One or more PV drain into RA or SVC or IVC
Total Anomalous Pulmonary Venous Return (TAPVR)
Diagnosed in children
Patent Ductus Arteriosus
Ductus (fetal communication between descending Ao and PA) remains open after birth
Ventricular Inversion
AKA CCTGA
CCTGA: Congenitally Corrected Transposition of the Great Arteries
AKA L- Transposition
Ventricles Switch Positions
Anatomic RV (with TV) = systemic ventricle
Anatomic LV (with MV) = venous ventricle
Transposition of the Great Arteries
AKA D- Transposition
Ao and PA have switched places and a shunt sustains life (Ao from RV, PA from LV)
