Chapter 13 Flashcards
____ is a condition where the heart lies on the right side of the thorax instead of the left and it occurs when the heart loops to the left instead of the right
Dextrocardia
Occurs with____, a complete reversal of asymmetry in all organs, or may be associated with laterality sequences [heterotaw] in which only some organ positions are reversed
situs inversus
Occurs with situs inversus, a complete reversal of asymmetry in all organs, or may be associated with ____ in which only some organ positions are reversed
laterality sequences [heterotaw]
Pulmonary vein was thought to originate as an outgrowth of the left atrium, and this concept made it difficult to determine the etiology of a rare birth defect where the pulmonary veins drain into other vessels or directly into the right atrium, a condition called ____
total anomalous pulmonary venous return [TAPVR]
Classic examples of cardiovascular teratogens include ___ and ____. Others include ___, ___, and many other compounds. Maternal diseases, such as insulin-dependent diabetes, have also been linked to cardiac defects
rubella virus - thalidomide
RA [Accutane] - alcohol
Mutations in the ___ gene result in Holt-Dram syndrome, characterized by preaxial [radial] limb abnormalities and ASDs
TBX5
Mutations in the TBX5 gene result in___, characterized by preaxial [radial] limb abnormalities and ASDs
Holt-Dram syndrome
Holt-Oram syndrome is one of a group of ___ illustrating that the same genes may participate in multiple developmental processes. For example, TBX5 regulates forelimb development and plays a role in septation of the heart. Holt-Oram syndrome is inherited as an autosomal dominant trait
heart-hand syndromes
Mutations in a number of genes regulating production of sarcomere proteins cause ____ that may result in sudden death in athletes and the general population
hypertrophic cardiomyopathy
___ is a defect in which the morphologic left ventricle is on the right and connects to the right atrium through a mitral valve
Ventricular inversion
The result is cardiac hypertrophy due to disruption in the organization of cardiac muscle cells [___], which may adversely affect cardiac output and/or conduction
myocardial disarray
The defect is sometimes called____ because the pulmonary artery exits the morphologic left ventricle and the aorta exits the morphologic right ventricle
L-transposition of the great arteries
___ and ___ are rare defects that cause an under development of the right or left sides of the heart, respectively
Hypoplastic right heart syndrome [HRHS] and hypoplastic left heart syndrome [HLHS]
___ is a congenital heart abnormality with an incidence of 6.4/10,000 births and with a 2:1 prevalence in female to male infants
ASD
One of the most significant defects is the __ defect, characterized by a large opening between the left and right atria
ostium secundum
This condition, known as common atrium or ___, is always associated with serious defects elsewhere in the heart
cor triloculare biventriculare
___-, leads to massive hypertrophy of the right atrium and ventricle and underdevelopment of the left side of the heart.
Premature closure of the oval foramen
Whenever the atrioventricular cushions fail to fuse, the result is a ___, combined with a defect in the cardiac septum
persistent atrioventricular canal
____ of the atrioventricular canal not only divide this canal into a right and left orifice but also participate in formation of the membranous portion of the interventricular septum and in closure of the ostium primum
Endocardial cushions
This defect, the ____, is usually combined with a cleft in the anterior leaflet of the tricuspid valve
ostium primum defect
____, which involves obliteration of the right atrioventricular orifice, is characterized by the absence or fusion of the tricuspid valves
Tricuspid atresia
___ is a condition where the tricuspid valve is displaced toward the apex of the right ventricle, and as a result, there is an expanded right atrium and a small right ventricle
Ebstein anomaly
___ involving the membranous or muscular portion of the septum are the most common congenital cardiac malformation, occurring as an isolated condition in 12/10,000 births
VSDs
____, the most frequently occurring abnormality of the conotruncal region, is due to an unequal division of the conus resulting from anterior displacement of the conotruncal septum
Tetralogy of Fallot
Tetralogy of Fallot, the most frequently occurring abnormality of the ___ region, is due to an unequal division of the conus resulting from anterior displacement of the conotruncal septum
conotruncal
a narrow right ventricular out flow region, ___
pulmonary infundibular stenosis
Tetralogy of Fallot occurs in 9.6/10,000 births but occurs as a common feature in individuals with ___
Alagille syndrome
90% of cases, there is a mutation in___, the ligand for ___ signaling that regulates neural crest cells forming the conotruncal [outflow tract] septum
jAG1 - NOTCH
____ results when the conotruncal ridges fail to form such that no division of the outflow tract occurs
Persistent [common] truncus arteriosus
___ is an example of the 22q11 deletion syndrome characterized by a pattern of malformations that have their origin in abnormal neural crest development
DiGeorge sequence
____ occurs when the conotruncal septum fails to follow its normal spiral course and runs straight down
Transposition of the great vessels
___ of the pulmonary artery or aorta occurs when the semilunar valves are fused for a variable distance
Valvular stenosis
____, fusion of the thickened valves may be so complete that only a pinhole opening remains
Aortic valvular stenosis
___ is a rare anomaly in which the heart lies on the surface of the chest. It is caused by failure of the embryo to close the ventral body wall
Ectopia cordis
Under normal conditions, the ____ is functionally closed through contraction of its muscular wall shortly after birth to form the ligamentum arteriosum
ductus arteriosus
Under normal conditions, the ductus arteriosus is functionally closed through contraction of its muscular wall shortly after birth to form the ___
ligamentum arteriosum
A ____, one of the most frequently occurring abnormalities of the great vessels [8/I0,000 births], especially in premature infants, either may be an isolated abnormality or may accompany other heart defects
patent ductus arteriosus [PDA]
In___, which occurs in 3.2/10,000 births, the aortic lumen below the origin of the left subclavian artery is significantly narrowed
coarctation of the aorta
Because the constriction may be above or below the entrance of the ductus arteriosus, two types [____ and ___] may be distinguished
preductal and postductal
___ occurs when the artery is formed by the distal portion of the right dorsal aorta and the seventh intersegmental artery
Abnormal origin of the right subclavian artery
A ___ surrounds the trachea and esophagus and commonly compresses these structures, causing difficulties in breathing and swallowing
vascular ring
With a___ , the right dorsal aorta persists between the origin of the seventh intersegmental artery and its junction with the left dorsal aorta
double aortic arch
In a ___, the left fourth arch and left dorsal aorta are obliterated and replaced by the corresponding vessels on the right side
right aortic arch
An ____ is a very rare defect [3/l,000,000 live births] caused by abnormal regression patterns in the right and left fourth aortic arches
interrupted aortic arch [IAA]
A ____ occurs when the left sacrocardinal vein fails to lose its connection with the left subcardinal vein
double inferior vena cava
___ is caused by persistence of the left anterior cardinal vein and obliteration of the common cardinal and proximal part of the anterior cardinal veins on the right
Left superior vena cava
____ arises when the right subcardinal vein fails to make its connection with the liver and shunts its blood directly into the right supracardinal vein
Absence of the inferior vena cava
A ___ is characterized by the persistence of the left anterior cardinal vein and failure of the left brachiocephalic vein to form]
double superior vena cava
The persistent left anterior cardinal vein, the____ drains into the right atrium by way of the coronary sinus
left superior vena cava,
