CVS 2024 Flashcards
What are the bulbar muscles?
a group of muscles in the head and neck. These muscles are involved in speaking, swallowing, chewing, and holding the jaw in place. The nerves that control these muscles are located in a part of the brainstem called the bulbar region.
What is a normal heart weight for an autopsy?
Diagnostic criteria used for the classification of a morphologically normal heart are a heart weight of below 500 g in males, and below 400 g in females.
Chagas disease
Trypanosoma cruzi is transmitted to animals and people by insect vectors (Various species of triatomine bugs) and is found only in the Americas (mainly, in rural areas of Latin America where poverty is widespread). Chagas disease (T. cruzi infection) is also referred to as American trypanosomiasis.
Carney complex
Seen in 5% of patients with cardiac myxomas
PRKAR1A germline mutation.
Manifestations of Carney complex[6]
Lentiginosis 60-70%
Cardiac myxoma 30-60%
Primary pigmented nodular
adrenocortical disease (PPNAD) 25-60%
Ovarian cyst 20-67%
Skin myxoma 20-63%
Testicular tumor 33-56%
Ductal carcinoma of the breast 25%
Thyroid tumor 10-25%
Melanotic schwannoma 8-18%
Acromegaly 10%
Osteochondrotic myxoma <10%
Multiple blue nevi
Gorlin syndrome
Gorlin syndrome (also known as nevoid basal cell carcinoma syndrome) is associated with cardiac fibromas.
22q11.2 deletion syndrome
DiGeorge syndrome may be first spotted when an affected newborn has heart defects or convulsions from hypocalcemia due to malfunctioning parathyroid glands and low levels of parathyroid hormone (parathormone).Infections are common in children due to problems with the immune system’s T cell-mediated response that in some patients is due to an absent or hypoplastic thymus.
DiGeorge syndrome
Salient features can be summarized using the mnemonic CATCH-22 to describe 22q11.2DS, with the 22 signifying the chromosomal abnormality is found on the 22nd chromosome, as below:[17]
Cardiac abnormality (commonly interrupted aortic arch, truncus arteriosus and tetralogy of Fallot)
Abnormal facies
Thymic aplasia or hypoplasia
Cleft palate
Hypocalcemia/hypoparathyroidism early in life
Velopharyngeal insufficiency
a disorder of structure that causes a failure of the velum (soft palate) to close against the posterior pharyngeal wall (back wall of the throat) during speech in order to close off the nose (nasal cavity) during oral speech production. T
Truncus arteriosus
Truncus arteriosus is a rare type of heart disease that is present at birth (congenital) in which there is a single main blood vessel, rather than the normal two, carrying blood away from the heart.
velocardiofacial syndrome
DiGeorge syndrome, also known as 22q11.2 deletion syndrome, is a syndrome caused by a microdeletion on the long arm of chromosome 22.[7] While the symptoms can vary, they often include congenital heart problems, specific facial features, frequent infections, developmental delay, intellectual disability and cleft palate.[7] Associated conditions include kidney problems, schizophrenia, hearing loss and autoimmune disorders such as rheumatoid arthritis or Graves’ disease.[7][8]
DiGeorge syndrome is typically due to the deletion of 30 to 40 genes in the middle of chromosome 22 at a location known as 22q11.2.[3] About 90% of cases occur due to a new mutation during early development, while 10% are inherited.[7] It is autosomal dominant,
What is the remnant of the umbilical vein?
Within a week of birth, the neonate’s umbilical vein is obliterated and is replaced by a fibrous cord called the round ligament of the liver (also called ligamentum teres hepatis). It extends from the umbilicus to the transverse fissure, where it joins with the falciform ligament of the liver to separate segment 4 from segments 2 and 3 of the left hepatic lobe.
What is the remnant of the umbilical arteries?
The umbilical artery regresses after birth. A portion obliterates to become the medial umbilical ligament (not to be confused with the median umbilical ligament, a different structure that represents the remnant of the embryonic urachus). A portion remains open as a branch of the anterior division of the internal iliac artery. The umbilical artery is found in the pelvis, and gives rise to the superior vesical arteries. In males, it may also give rise to the artery to the ductus deferens which can be supplied by the inferior vesical artery in some individuals.
urachus
The urachus is a fibrous remnant of the allantois, a canal that drains the urinary bladder of the fetus that joins and runs within the umbilical cord.[1] The fibrous remnant lies in the space of Retzius, between the transverse fascia anteriorly and the peritoneum posteriorly
vitelline duct
Generally, the duct fully obliterates (narrows and disappears) during the 5–6th week of fertilization age (9th week of gestational age), but a failure of the duct to close is termed a vitelline fistula. This results in discharge of meconium from the navel (umbilicus).[2] About two percent of fetuses exhibit a type of vitelline fistula characterized by persistence of the proximal part of the vitelline duct as a diverticulum protruding from the small intestine, Meckel’s diverticulum, which is typically situated within two feet of the ileocecal junction and may be attached by a fibrous cord to the abdominal wall at the umbilicus.
The human allantois
The human allantois is a caudal out-pouching of the yolk sac, which becomes surrounded by the mesodermal connecting stalk known as the body-stalk. The vasculature of the body-stalk develops into umbilical arteries that carry deoxygenated blood to the placenta.[5] It is externally continuous with the proctodeum and internally continuous with the cloaca. The embryonic allantois becomes the fetal urachus, which connects the fetal bladder (developed from cloaca) to the yolk sac. The urachus removes nitrogenous waste from the fetal bladder.[6] After birth the urachus becomes obliterated as the median umbilical ligament.
sinus venosus atrial septal defect
represent 5% of atrial septal defects.
Can be present in the superior vena cava (most common) or inferior vena cava connecting to the left atrium.
Most common congenital heart defect in newborns?
Ventricular septal defect. Most close after birth.
Membranous type (most common). The pansystolic murmur of VSD can be confused with the pansystolic murmur of mitral regurgitation. The VSD becomes louder towards the sternum, and the murmur of mitral regurgitation gets louder away from the sternum
Patients with a small-sized VSD without evidence of pulmonary arterial hypertension (PAH) or Eisenmenger syndrome can participate in competitive sports.
Patients with small, asymptomatic, VSDs with the absence of PAH have an excellent prognosis without any intervention and SBE prophylaxis is currently not indicated based on the 2017 AHA guidelines.
Ventricular septal defect size classification.
While VSDs are classified according to location, they can also be classified into size. The size is described in comparison to the diameter of the aortic annulus. They are considered small if they measure less or equal to 25% of the aortic annulus diameter, medium if they measure more than 25% but less than 75%, and large if they are greater than 75% of the aortic annulus diameter.
Four types of ventricular septal defects
Type 1: (infundibular, outlet) This VSD is located below the semilunar valves (aortic and pulmonary) in the outlet septum of the right ventricle above the crista supraventricularis, which is why it is sometimes also referred to as supracristal.
Type 2: (membranous) This VSD is, by far the most common type, accounting for 80% of all defects. It is located in the membranous septum inferior to the crista supraventricularis. It often involves the muscular septum when it is commonly known as perimembranous. The septal leaflet of the tricuspid valve sometimes forms a “pouch” that reduces the shunt and can result in spontaneous closure.
Type 3: (inlet or atrioventricular canal) This VSD is located just inferior to the inlet valves (tricuspid and mitral) within the inlet part of the right ventricular septum. It only represents 8% of all defects. It is seen in patients with Down syndrome.
Type 4: (muscular, trabecular) This VSD is located in the muscular septum, bordered by muscle usually in the apical, central, and outlet parts of the interventricular septum. They can be multiple, assuming a “Swiss cheese” appearance. They represent up to 20% of VSDs in infants. However, the incidence is lower in adults due to the tendency of spontaneous closure.
