Glossary of fetal heart and thorax

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us appearance: relies on the demonstration of narrowing of the vessel and enlarged right ventricular (RVOT); prenatal diagnosis is extremely difficult
coarctation of aorta
a group of cardiac malformations characterized by a small or absent lv; lethal; result of abnormalities that limit flow of blood thru lt side of the heart; ao atesia or stenosis is the resulting factor; assoc w/ auto rec. condition; idiopathic
hypoplastic of the left ventricle
may be unilatera or bilateral; may result from immune or nonimmune hydrops or from CHF; also may occur in fetuses with chromsomal abnormalies or in the fetus with a cardiac mass
pleural effusion
opening in the muscular or membranous part of interventricular septum; defect may be small and clinically insignificant and close after birth, or large, causing significant shunting of blood across the defect; best prognosis if not assoc w/ other defect
ventricular septal defect
the normal fetal heart rate is __ to __ bpm; in the 1st trimester, the heart rate begins around 90 bpm and inc to 170 bpm before returning to a normal rate and sinus rhythm.

a rare anomaly in which the heart is located partially or completely outside the fetal thorax, protruding through a defect in the anterior chest wall. in many cases the heart is structurally abnormal in addition to being abnormally located;
ectopic cordis
us appearance: heart seen within the amniotic cavity connected to the rest of the fetus through a defect in the ant thoracic wall
ectopic cordis
involves a disease of the myocardial tissue in the heart; this disease process is caused by several problems, including exposure to a virus or bacteria that leads to an infection or metabolism problem; thickenening of muscles w/i heart
rare, benign cardiac tumor arising in the myocardium and tend to grow during gestation;multiple or singular; most common cardiac tumor identified in the fetus
sono, a round oval echogenic mass is seen arising from the myocardium; pericardial fluid may also be seen
heart lesion in which only one great art arises from the base of the heart; one single vessel instead of 2 from ventricles, bifurcates after it comes off heart; from this great art arises the pulmonary trunk, systemic art, and coronary arts
truncus arteriosus
sono cannot be diagnosed via the 4-chamber view demonstrating a single large ventricle seen arising from the heart; absent of the ventricular outflow tract; will see one large vessel coming off in ventricular outflow tract
truncus arteriosus
poor prognosis; presence of a single cardiac ventricle communicating with two atria; due to an absent of the ventricular system; have both mitral and tricuspid valves
univentricular heart
sono dx relies ont he demonstration of a single ventricle; also a rudimentary ventricular may also be seen; four chamber view is best way to dx; because will only see 3 chambers
univentricular heart
us appearance: can be diagnosed by the 4-chamber view by demonstrating a smal left ventricle; mitral valve dysplasia or absence of the valve; narrowed or stenosis of the aortic valve; LVOT (pulmonary artery and aorta)
hypoplastic of the left ventricle
reversed ventricular outflow tracts, with pulmonary art arising from the lv and the ao arising from the rv; occurs due to an abn completion of the loops in embryonic development; usually accomp by vsd; good prognosis with surgery
transposition of the great vessels
us appearance: normal 4-chamber view is seen; long axis view is the best techniques to dx; VSD may be seen
transposition of the great vessels
complex cardiac anomaly comprising four cardiac abnormalities in the neonate; most common form of a cyanotic heart disease (blue baby); severity of the disease varies according to the pulmonary stenosis present, the more stenosis the greater the cyanosis
tetralogy of fallot
what are the four signs of tetralogy of fallot?
pulmonary stenosis, VSD, overriding aorta (goes more into LV than normal), ventricular hypertrophy (occurs after birth because of pulmonary stenosis)
us appearance: may have a normal 4-chamber view, best diagnosed via LVOT; widened aorta extending over the VSD; narrowing of the pulmonic valve; good prognosis w/ corrective surgery
tetralogy of fallot
stricture anywhere along the lumen of the aortic arch frequently assoc w/ tubular hypoplasia of the arch; two types: juxaductal and preductal (arteriosus); narrowing at desc ao; seen just inferior to L subclavian art or where ductus arteriosus joins
coarctation of the aorta
almost always associated with other cardiac anomalies such as: aortic stenosis, aortic insufficiency, VSD, ASK, transposition of the great vessels, and truncus arteriosus
coarctation of the aorta
us appearance: small RV and large RA; 4-chamber heart is abnormal with enlargement of the heart with a l=dilated right atrium; tricuspid is displaced toward the apex of the RV; regurgitation can be seen on spectral or color doppler
ebstein's anomaly
small or absent right cardiac ventricle; results from pulmonary stenosis or atresia with an intact ventricular septum or from stenosis or atresia of the tricuspid valve; may cause fetal cardiac failure and hydrops b/c of overworked heart
hypoplatic of the right ventricle
space b/w both atria and vents; severe cardiac anomaly characterized by large defect in central part of heart;divided into complete, incomplete, and partial forms; assoc w/ other structural anomalies or aneuploidy (tri 21); poor prognosis
atrioventricular septal defect
A-V septal defect:
this is the failure of the endocaridal cushion to form causing abnormal tricusp valve;
this involves both av valves and atrium and ventricular septa; problem with mitral, tricusp, and septa b/w atria and vents

*atrioventricluar septal defect

us appearance: can be seen on a 4-chamber view, as a large defect in the middle of the heart, with absence of portions of the atrial and ventricular septa; search for other anomalies
atrioventricular septal defect
defect in wall separating the two atria; hard to dx b/c already have opening (foramen of ovale); not always recognized during fetal life unless part of the intraatrial septum is missing; may be caused by the failure of the foramen to close;postnatal dx
atrial septal defect
sonographic diagnosis relies on the demonstration fo the defect in the atrial septum; defect must be large to identify sonographically
atrial septal defect
us appearance: diagnosed on a 4-chamber view, by visualizing a gap in the septum between the right and left ventricle; also associated with other caridac problems
ventricular septal defect
the 3 most common types of congenital heart diseases in order.
*ventricular septal defect, atrial septal defect, pulmonary stenosis

cardiac anomalies detected prenatally tend to be more severe than those missed on ultrasound. thus, the survival rate of only __% for cardiac defects detected prenatally is much worse than the survival rate of approx. __% anomalies undetected on us

specific risk factors indicate that the fetus is at a higher than normal risk for congenital heart disease and warrant a fetal echocardiogram. htese may be divided into the following three categories. what are the three risk factors
fetal risk factors, maternal risk factors, familial risk factors
IUGR, cardiac arrhythmias, abnormal amniocentesis (indicating a trisomy), abnormal amniotic fluid collections,abnormal heart rate, and other anomalies as detected by the sonogram, such as hydrops fetalis (erythroblastosis fetalis)
fetal risk factors indicating fetal echocardiography
previous occurence of congenital heart disease is siblings or parents; a maternal disease known to affect the fetus, such as diabetes mellitus; maternal infections; and maternal use of drugs such as lithium or alcohol
maternal risk factors indicating fetal echocardiography
genetic syndromes of the presence of congenital heart disease in a previous sibling; familial history of congenital heart disease
familial risk factors indicating fetal echocardiography
embryology of the fetal heart: the paired heart endocardial heart tube gradually fuse along the embryonic midline to forma a single tubular heart. it fused __ to __. the heart starts to beat at day __, but the circulation does not start until days __ to _
*caudal to cranial *22 *27-29 *sinus *pulmonary vein
as it elongates, it bends on itself forming a u-shaped ___loop. the bulboventricular loop will bend ventrally, caudally and slightly to the right. as the cardiac looping is developing the paired atria form a common chamber and move into the ___sac.

the endocardial cushion develop in the ___region of the heart. the septum primum grows from the dorsal wall of the primitive __and fuses with the endocardial cushion (develops into septa).
septum secundum overlaps foramen secundum, the opening of the septum primum. there is also an opening b/w the free edge of the septum secundum and the dorsal wall of the atrium- the __.
*foramen ovale

human teratogens produce or raise the incidence of congenital malformations; __% are caused by environmental agents or teratogens.
the primitive heart is a tubular structure that forms like a large blood vessel from the mesenchymal cells int he cardiogenic area of the embryo. paired endocardial heart tubes develop before the end of the __ week and begin to fuse to form the __
*primitive heart
blood circulation starts by the end of 5th wk as a tubular heart begins to beat. the vasc system begins during 5th wk in the wall of the ys, connecting stalk, and _. blood vessels begin to develop _ days later

lung masses are separate from the __and are located above the level of the diaphragm.
echocardiography is beneficial in excluding cardiac involvement, and eval of an intact diaphragm is necessary to exclude __ .abnormalities in cardiac __ and fetal hydrops may be present in fetuses w/ lung masses b/c of compression of venous return and _
*diaphragmatic hernia
*cardiac failure

the lungs do not grow properly when there is a: small ___cavity,or the fetus is unable to practice breathing movement or mass w/i chest

reduction in lung volume (lung cells) resulting in small, inadequately developed lungs; most commonly occurs from prolonged oligohydramnios or secondary to a small thoracic cavity as a result of a structural or chromosomal abnormality.
pulmonary hypoplasia
most common lung cyst detected prenatally; occur as a result of abnormal budding of foregut and lack any communication w/ the trachea or bronchial tree; typically occur w/i the mediastinum or lung; infrequently they are inferior to the diaphragm
bronchogenic cyst
sono appearance it's small circumscribed masses without evidence of a mediastinal shift or heart failure; amniotic fluid volume is within the normal range; usually go away after birth
bronchogenic cyst
accumulations of fluid within the pleural cavity that may appear as isolated lesions or secondary to multiple fetal anomalies; the most common reason is chylothorax (milky fluid with emulsified fat within) occurring as a right-sided unilateral collection
pleural effusion
sono appearance it appears as echo-free peripheral masses on one or both sides of the fetal heart. the effusions confom to the thoracic cavity and often compress lung tissue; eval for signs of hydrops by looking at ab and head
pleural effusion
extra pulmonary tissue is present within the pleural ung sac (intralobar) or is connected to the inferior border of the lung within its own pleural sac (extralobar).
pulmonary sequestration
two types of pulmonary sequestration? which one has better prognosis?
*intra- and extraloba. *intralobar
sono: echodense solid mass resembling lung tissue usually in the lower lobe of the lung; a little more hyperechoic than the rest of the lung; lesions may resemble a ccaml; doppler may help. may also see hypoplastic lung, hydrops, and other anomalies
pulmonary sequestration
pulmonary sequestration.

this type are spherical whereas this type occur on the left side the majority of the time and rarely below the diaphragm and are cone/triangular masses

intralobar, extralobar
an abnormality in the formation of the bronchial tree with secondary overgrowth of the mesenchymal tissue from arrested bronchial development; cysts grow in area of thoracic cavity
congenital cystic adenomatoid malformation (CAML or CCAML)
what are the three types of CAML
macrocystic (type I), medium size cyst
one or several large cysts replace normal lung tissue (single or multiple cysts measuring more than 2 cm up to 10 cm)best prognosis
macrocystic CAML
lesions consist of multiple small cysts
type II CAML
are characterized as bulky, large, non-cystic lesions appearing as echo-dense masses of the entire lobe; cyst so small it looks like one big echogenic mass; worst prognosis
type III CAML "microcystic"
lung compressing may occur and hydrops may develop and thus pulm hypo; may have hydramnios secondary to esophageal compression, preventing swallowing; sono, a cystic,complex, or solid lesion may be seen; hydramnios and hydrops may be seen
cystic adenomatoid malformation (CAML)
herniation of the ab viscera into the chest resulting from a congenital defect int he diaphragm; this defect probably develops between the 6th and 10th week of gestation when the gut is returning from the yolk sac and the diaphragm is developing
congenital diaphragmatic hernia
the most common type of diaphragmatic defect occurs posteriorly and laterally in the diaphragm formamen of ___.
two types of diaphragmatic hernias
formaen of bochdalek (left) and foramen of morgaghi (right)
usually are on lt side of diaphragm, and lt-sided organs (sto, spl, and portions of liv) enter the chest thru opening; these organs shift the heart and mediastinal structures to the rt side of chest
foramen of bochdalek diaphragmatic hernia
hernias on the rt side that are ant and med; may communicate w/ pericardial sac allowing rt sided ab viscera (liv, gb, intestines) to enter chest; the hrt may be normally positioned and surrouned by pleural fluid and the sto may be in normal position
foramen of morgagni diaphragmatic hernia
sono it appears as ab contents w/i the chest cavity; hydrops, cardiac anomalies may be seen; look for liver, gb, and/or intestines in chest cavity
diaphragmatic hernia
due to failure of chest to form around and enclose heart; usually isolated but may be assoc with pentalogy of cantrell of amniotic band syndrome; dismal prognosis
ectopic cordis
heart floating around in amniotic fluid partiall or completely
ectopic cordis
A-V septal defect: this type the fetus has some of the other types of findings, usually an atrial septum and cleft mitral valve
partial AV septal defect
the tricuspid is dysplastic and incompetent, leading to regurgitation and enlargement of RA; may adhere to ventricular endocardium or closely attached to vent wall
ebstein's anomaly
an abnormal displacement of the septal eaflet of the tricuspid valve toward the apex of the RV; valve displaced into the RV so that a portion of the RV is contiguous with the RA
ebstein's anomaly
; this malfomation may involve one or more lobes of the lung or an entire lung, or in rare cases bilateral; prognosis depends on type; 3 types
while in the u form a dividing wall bisects the lower heart area called the _, into two ventricles and the pulmonary artery and ao
approx _ of infants born w/ congenital heart disease have other anomalies as well. we can detect about _ % of abnormalities by seeing 4-chamber heart. others require lvot and rvot
can be congenital or sporadic anomaly; permits the ab organs to enter chest; b/c of herniation the lungs are compressed and can become hypoplastic
diaphragmatic hernia
prognosis is poor if detected prior to birth, if stomach is in chest or dilated, pulmonary hypoplasia is main cause of death
diaphragmatic hernia
usually the stomach is in the chest near the heart instead of below the diaphragm. dx by seeing rt sided heart and stomach in chest on lt. look for peristalsis or displacement of liver
foramen of bochdalek diaphragmatic hernia
this probably develops from separate outpouching of the foregut by separation of a segment of the developing lung from the tracheobronchial tree
pulmonary sequestration
the extra lung tissue is nonfunctional and receives its blood supply from the system circulation(thoracic ao with venous drainage into ivc) doesn't function but still being fed
pulmonary sequestration
sono dx relies on demonstration of cardiomegaly specific wall abnormalities, and poor wall contractility; hypertrophy of muscles
cardiomyopathy (hypertrophic cardiomyopathy)
cardiac axis may be evaluated in a four-chamber heart view by estimating the angle at which the intraventricular septum cross-sects the __ at the ant chest wall
the normal cardiac axis ranges from _ to _ degrees with average of 45 degrees. deviation may suggest intrathoracic __
measurements of thoracic circumference may aid in estimating the size of the thoracic cavity and may predit an abnormally small chest and secondary __
pulmonary hypoplasia
mortality rate approaches 50%, prognosis poorer w/ hydrops; some reasos are lymphangiectasia, CCAM, sequestration, dia hernia, hamartoma, pulmonary vein atresia, other unknown causes; may cause pulm hypoplasia
pleural effusion
often assoc with tuberous sclerosis (tumors within kidney, spleen, liver, retina. cardiac failure and hydrops may result from obstruction of blood flow by tumor or from poor cardiac contractility; poor prognosis if hydrops develops
pulmary hypoplasia may be uni or bilat and often assoc w/ other fetal anomalies. prognosis is grave. _% die after birth. later the onset the better the prognosis
about wk 5 blood vessels from the embryo join those o the ys, stalk, and chorion to form __ cv system/ blood formation begins at the __ week
the sup part of the septum primum breaks down creating an opening called the __. as this foramen develops, another membraneous fold, the __ grows into the atrium to the right of the septum primum
*foramen secundum
*septum secundum
after the foramen secundum and the septum secundum form, the interventricular septum begins as a ridge in the floor of the __ ventricle
when evaluating the fetus for a lung __, the sonographer should check for normal heart position and axis and measure the thoracic circumference. (don't do this on basic scan unless you suspect abnormality).

the interventricular septum slosly grows toward the endocardial cushion. division of the vents results in development and fusion of the truncal ridges forming the __
aorticopulmonary septum
this separates ao from pulmonary vessels
truncal ridges
bradycardia is < _ bpm. tachycardia is > _ bpm
most common congenital lesion of the heart
ventricular septal defect
congenital heart disease is most common severe congenital abnormality. development of congenital heart disease is multifactorial. enviroment and chromosome abnormalities are assoc. tri 21 has _% incidence this disease, usually AVS defect

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