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76 Cards in this Set

  • Front
  • Back
what happens when the abdominal left SVC opens into the right atrium through the coronary sinus?
double superior vena cava
what occurs when the left anterior cardianl vein persists, forming a superior vena cava on the left side?
left superior vena cava
what is caused by abnormal neural crest cell migration resulting in partial development of the AP septum?
persistent truncus arteriosus
what results in one large vessel leaving and receiving blood from both the right and left ventricles?
persistent truncus arteriorsus
what usually presents with a membraneous ventricular septum defect?
persistent truncus arteriorsus
what is when a newborn is severly cyanotic (R to L shunting of blood)??
persistent truncus arteriorsus
what is caused by abnormal neural crest cell migration, where the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle so the systemic and pulmonary circulation are separated from each other?
d-transportation of the great arteries (complete)
in what case would a fetus be incompatible with life unless a shunt exists like a VSD, patent foramen ovale, or patent ductus arteriosus?
d-transportation of the great arteries (complete)
what is pulmonary stenosis, VSD, destraposition of the aorta, right ventricular hypertrophy caused by abnormal neural crest cell migration resulting in skewed development of the AP septum?
tetralogy of fallot
what results in pulmonary trunk with a small diameter, whereas the aorta has a large diameter?
tetralogy of fallot
what is associated clinically with marked cyanosis?
tetralogy of fallot
what is the most common clinically significant Atrial Septum Defects?
forament secundum defect
what is caused by excessive resorption of septum primum, secundum or both?
forament secundum defect
what results in an opening between the right and left atria?
forament secundum defect
what is the condition where there is only formation of only one atrium?
common atrium (con triloculare biventrulare)
what is closure of foramen ovale during prenatal?
premature closure of foramen ovale
what results in hypertrophy of the right side of the heart and under development of the left side of the heart?
premature closure of foramen ovale
what is caused by failure of fusion of the AV cushions?
persistent common AV canal
what causes a large hole that can be found in the center of the heart?
persistent common AV canal
what defect occurs when the tricuspid and bicuspid valves are represented by one valve common to both sides of the heart?
persistent common AV canal
what is caused by failure of the posterior and septal leaflets of the tricuspid valve to attach normally to the annulus fibrosus?
ebstein's anomaly
hat is when the right ventricle is divided into large, upper, "atrialized" portion and a small, lower, functional portion?
ebstein's anomaly
what is complete agenesis of the tricuspid calve so that there is no communication between the right atrium and right ventricle?
tricuspid atresia (hypoplastic right heart)
what causes cyanosis always associated with patent foramen ovale, IV septum defect, overdeveloped left ventricle and underdeveloped right ventricle?
tricuspid atresia
what is the most common type of VSD?
membranous ventricular septum defect
what is caused by faulty fusion of the right bulbar ridge, left bulbar ridge, and AV cushions?
membranous ventricular septum defect
what occurs when large VSD presents with R to L shunting of blood, increased pulmonary blood for, and pulmonary hypertension?
membranous ventricular septum defect
what is caused by a single or multiple perforations in the muscular IV septum?
muscular ventricular septum defect
what is caused by failure of the membranous and muscular IV septa to form?
common ventricle (cor triloculare biatratum)
what is characterized by aortic constriction?
coarctation of the aorta
what is found inferior to the ductus arteriosus and clinically associated with increased blood pressure in the upper extremities, lack of pulse in the femoral artery?
postductal coarctation
what is a constriction located superior to the ductus arteriosus?
preductal coarctation
what is characterized by a vascular ring around the trachea and esophagus?
double pharyngeal arch artery
what results from failure of the distal part of the right aorta to disappear, so right and left arches form?
the vascular ring characteristic of double pharyngeal arch artery
what is a connection between the left pulmonary artery and aorta due to failure of closure?
patent ductus arteriosus
what is associated with maternal rubella infection during early pregnancy?
patent ductus arteriosus
what is present in infants born at high altitude, hypoxia and immaturity?
patent ductus arteriosus
what causes a L to R shunting of oxygen rich blood from the aorta back into the pulmonary circulation?
patent ductus arteriosus
what is treated with prostaglandin synthesis inhibitors (indomethacin), which promote closure?
patent ductus arteriosus
what is a benign tumor of schwann cells of neural crest origin?
schwannoma
what causes well circumscribed, encapsulated masses that may or may not be attached to the nerve?
schwannoma
what has clinical signs of tinnitus and hearing loss?
schannoma
what is von recklinghausen disease?
neurofibromatosis type I
what is the most common autosomal dominant disorder due to a mutation of the NF1 gene that codes for the protein neurofibromin?
neurofibromatosis type I - von reckinghausen disease
what causes multiple neural tumors (neurofibromas) over the body with proliferation of elements of peripheral nerve including neuritis, fibroblasts and schwann cells of the neural crest origin?
neurofibromatosis type I - von recklinghausen disease
what causes pigmented lesions and pigmented iris harmatomas?
neurofibromatosis type I - von recklinghausen disease
what is CHARGE association?
Coloboma of the retina, lens or choroid
Heart defects (tetralogy of fallot, VSD, PDA)
Atresia Choanae
Retardation of Growth
Genital abnormalities in males (cryptochidism)
Ear abnormalities or deafness
what is an autosomal dominant disorder due to the mutation int he PAX3 gene or MITF gene?
waardenburg syndrome
what is the lateral displacement of lacrimal puncta, broad nasa root, heterochromia of the iris, congenital deafness, and piebaldism including a white forelock and triangular area of hypopigmentation??
waardenburg syndrome
what causes posterior skin dimple in the median plane of the sacral region of the back?
spinal dermal sinus
what does the dimple in spinal dermal sinus indicate?
the region of the closure of the caudal neuropore at the end of week 4
what is a defect of the vertebral arch that results of failure of the embryonic halves of the arch to grow normally and fuse in the median plane?
spina bifida occulta
what appears as a small dimple with a tuft of hair in the median plane?
spina bifida occulta
what is a severe neural tube defect in which the neural tissue and/or meninges protrude through a defect in the vertebral arches to form a cyst-like sac?
spina bifida cystica
what is a severe neural tube defect where only fluid filled meninges protrude through the defect?
spina bifida meningocele
what is a severe neural tube defect where neural tissue is included in the sac?
spina bifida meningomyeocele
what is the most severe type of spina bifida causing paralysis from the level of the defect caudally?
spina bifida rachischisis
what is failure of closure of the posterior neuropore during week 4 of development?
spina bifida rachischisis
what results from failure of the posterior neuropore to close during week 4; usually occurring in the lumbosacral region?
lower NTD
what causes anencephaly?
upper NTD
what results from the failure of the anterior neuropre to close during week 4 of development?
upper NTD
what occurs when the bony skull fails to form resulting in a skull defect usually in the occipital region?
cranium bifida
what results when meninges and part of the brain herniate?
cranium meningoencephalocele
what results when the protruding brain contains part of the ventricular system?
cranium meningohydroencephalocele
what is a severe anomaly of the brain that results from failure of the rostral neuropore to close during week 4?
anencephaly and meroencephaly
what is when the brain is abnormal and calvaria is defective?
anencephaly and meroencephaly
what causes nervous tissue degeneration, and a rudimentary brainstem and functioning neural tissue in living infants?
anencephaly and meroencephaly
what causes caudal vermis and tonsils of the cerebellum and medually oblongata herniate through the forament magnum?
arnold-chiari malformation
what has clinical symptoms of compression of CN IX, CN X and CN XII and include difficulty swallowing, laryngeal stridor, diminished gag reflex, apnea, and vocal cord paralysis?
arnold-chiari malformation
what demonstrates aqueductal stenosis?
arnold-chiari malformation
what results from dilations of the ventricles due to an excess of CSF that may results from blockage of CSF circulation or overproduction of CSF due to a choroid plexus papilloma?
hydrocephalus
what are the two categories of hydrocephalus?
communicating (non obstructive) hydrocephalus
noncommunicating (obstructive) hydrocephalus
what is a free communication between the ventricles and the subarachnoid space?
communicating (non obstructive) hydrocephalus
what is congenital aqueductal stenosis?
non-communicating (obstructive) hydrocephalus - dandy-walker syndrome
what is the presence of a huge, fluid-filled cystic cavity that completely replaces the cerebral hemispheres?
hydranencephaly
what results from the cystic cavity that forms as a result of occlusion of the internal carotid arteries in utero causing widespread destruction of the cerebral?
hydranencephaly