Embryology
In an embryo, the foramen ovale is used to shunt blood from the _____ to the _____
The remnant structure is known as the _____
Right atrium to left atrium
Fossa ovalis
Name the structures (and at which vertebral levels) that pass through the diaphragm
IVC @ T8
Esophagus @ T10
Aorta @ T12
What are the four manifestations of Tetralogy of Fallot?
ToF is an anterior deviation of the infundibular septum that leads to 1. pulmonary stenosis, 2. VSD, 3. overriding aorta, and 4. right ventricular hypertrophy
Which type of cardiomyopathy has a genetic cause in 50% of cases?
Hypertrophic Cardiomyopathy
10% of dilated cardiomyopathies are inherited
Which four cell types do not have mitochondria?
Erythrocytes, white skeletal muscle, retina/sclera, chondrocytes
What does the bulbus cordis become?
Smooth walled part of the R. Ventricle (infundibulum) and L. Ventricle (aortic vestibule)
The internal thoracic artery bifurcates into
VSD, ASD, ToF, and PDA
Mutations in desmosomal genes lead to
Arrythmogenic Right Ventricular Cardiomyopathy
What is the most common cause of congenital lactic acidosis?
Deficiency in pyruvate dehydrogenase complex
When the aorticopulmonary septum fails to form, _____ occurs.
When the aorticopulmonary septum fails to twist, _____ occurs.
Persistant truncus arteriosis, trasnposition of the great vessels
What originates from T1-T4 to innervate the heart?
Turner syndrome is associated with which congenital heart defects?
Bicuspid aortic valve, coarctation of the aorta
Long QT Syndrome is associated with which genes? Are these loss of function or gain of function mutations?
SCN5A = Sodium channel, gain of function LTQ3
KCNQ1 = K+ channel, loss of function LTQ1
KCNQ2 = K+ channel, loss of function LTQ2
Phenylalanine can be converted into Tyrosine by
Phenylalanine hydroxylase (PAH)
Name the structure that becomes the ligamentum arteriosum, it's embryological function, and the nerve that runs under the remnant structure in an adult heart.
Ductus arteriosis, shunt within the liver between umbilical vein and IVC, left recurrent laryngeal nerve
Sinusoidal capillaries are fenestrated to _____ and are found in the _____, _____, and _____
Allow larger molecules (even cells) to move between blood and tissues
Liver, spleen, bone marrow
DiGeorge syndrome is associated with which congenital heart defects?
Truncus arteriosis, ToF
An uncommon form of Long QT that can also present with hearing loss
Jervell and Lange-Nielson Syndrome
What bioactive products are synthesized from tyrosine?
What arteries arise from pharyngeal arches 3, 4, and 6?
3: Common carotid
4: aortic arch, R. subclavian
6: pulmonary arteries (R & L), ductus arteriosis
Preganglionic fibers enter the sympathetic chain via _____, and postganglionic fibers leave the sympathetic chain via _____
Gray rami communicans
Prenatal lithium exposure leads to which congenital heart defect?
Ebstein anomaly
A change in the TTR gene manifests as
Hereditary Transthyretin Amyoidosis
What are the two roles of Vitamin K (and Gla) in hemostasis?
1. Localization of the cascade at the site of injury
Factors 2, 7, 9, and 10 can be bound to membrane phospholipids at the site of inury via Gla residues and Ca2+ bridges
2. Synthesis and release of coagulation factors from the liver