Chapter 13

Question 1

____ 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

Answer 1

Dextrocardia

Question 2

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

Answer 2

situs inversus

Question 3

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

Answer 3

laterality sequences [heterotaw]

Question 4

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 ____

Answer 4

total anomalous pulmonary venous return [TAPVR]

Question 5

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

Answer 5

rubella virus - thalidomide
RA [Accutane] - alcohol

Question 6

Mutations in the ___ gene result in Holt-Dram syndrome, characterized by preaxial [radial] limb abnormalities and ASDs

Answer 6

TBX5

Question 7

Mutations in the TBX5 gene result in___, characterized by preaxial [radial] limb abnormalities and ASDs

Answer 7

Holt-Dram syndrome

Question 8

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

Answer 8

heart-hand syndromes

Question 9

Mutations in a number of genes regulating production of sarcomere proteins cause ____ that may result in sudden death in athletes and the general population

Answer 9

hypertrophic cardiomyopathy

Question 10

___ is a defect in which the morphologic left ventricle is on the right and connects to the right atrium through a mitral valve

Answer 10

Ventricular inversion

Question 10

The result is cardiac hypertrophy due to disruption in the organization of cardiac muscle cells [___], which may adversely affect cardiac output and/or conduction

Answer 10

myocardial disarray

Question 11

The defect is sometimes called____ because the pulmonary artery exits the morphologic left ventricle and the aorta exits the morphologic right ventricle

Answer 11

L-transposition of the great arteries

Question 12

___ and ___ are rare defects that cause an under development of the right or left sides of the heart, respectively

Answer 12

Hypoplastic right heart syndrome [HRHS] and hypoplastic left heart syndrome [HLHS]

Question 13

___ 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

Answer 13

ASD

Question 14

One of the most significant defects is the __ defect, characterized by a large opening between the left and right atria

Answer 14

ostium secundum

Question 15

This condition, known as common atrium or ___, is always associated with serious defects elsewhere in the heart

Answer 15

cor triloculare biventriculare

Question 16

___-, leads to massive hypertrophy of the right atrium and ventricle and underdevelopment of the left side of the heart.

Answer 16

Premature closure of the oval foramen

Question 17

Whenever the atrioventricular cushions fail to fuse, the result is a ___, combined with a defect in the cardiac septum

Answer 17

persistent atrioventricular canal

Question 17

____ 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

Answer 17

Endocardial cushions

Question 18

This defect, the ____, is usually combined with a cleft in the anterior leaflet of the tricuspid valve

Answer 18

ostium primum defect

Question 19

____, which involves obliteration of the right atrioventricular orifice, is characterized by the absence or fusion of the tricuspid valves

Answer 19

Tricuspid atresia

Question 20

___ 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

Answer 20

Ebstein anomaly

Question 21

___ 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

Answer 21

VSDs

Question 22

____, 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

Answer 22

Tetralogy of Fallot

Question 23

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

Answer 23

conotruncal

Question 24

a narrow right ventricular out flow region, ___

Answer 24

pulmonary infundibular stenosis

Question 25

Tetralogy of Fallot occurs in 9.6/10,000 births but occurs as a common feature in individuals with ___

Answer 25

Alagille syndrome

Question 26

90% of cases, there is a mutation in___, the ligand for ___ signaling that regulates neural crest cells forming the conotruncal [outflow tract] septum

Answer 26

jAG1 - NOTCH

Question 27

____ results when the conotruncal ridges fail to form such that no division of the outflow tract occurs

Answer 27

Persistent [common] truncus arteriosus

Question 28

___ is an example of the 22q11 deletion syndrome characterized by a pattern of malformations that have their origin in abnormal neural crest development

Answer 28

DiGeorge sequence

Question 29

____ occurs when the conotruncal septum fails to follow its normal spiral course and runs straight down

Answer 29

Transposition of the great vessels

Question 30

___ of the pulmonary artery or aorta occurs when the semilunar valves are fused for a variable distance

Answer 30

Valvular stenosis

Question 31

____, fusion of the thickened valves may be so complete that only a pinhole opening remains

Answer 31

Aortic valvular stenosis

Question 32

___ 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

Answer 32

Ectopia cordis

Question 33

Under normal conditions, the ____ is functionally closed through contraction of its muscular wall shortly after birth to form the ligamentum arteriosum

Answer 33

ductus arteriosus

Question 34

Under normal conditions, the ductus arteriosus is functionally closed through contraction of its muscular wall shortly after birth to form the ___

Answer 34

ligamentum arteriosum

Question 35

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

Answer 35

patent ductus arteriosus [PDA]

Question 36

In___, which occurs in 3.2/10,000 births, the aortic lumen below the origin of the left subclavian artery is significantly narrowed

Answer 36

coarctation of the aorta

Question 37

Because the constriction may be above or below the entrance of the ductus arteriosus, two types [____ and ___] may be distinguished

Answer 37

preductal and postductal

Question 38

___ occurs when the artery is formed by the distal portion of the right dorsal aorta and the seventh intersegmental artery

Answer 38

Abnormal origin of the right subclavian artery

Question 39

A ___ surrounds the trachea and esophagus and commonly compresses these structures, causing difficulties in breathing and swallowing

Answer 39

vascular ring

Question 39

With a___ , the right dorsal aorta persists between the origin of the seventh intersegmental artery and its junction with the left dorsal aorta

Answer 39

double aortic arch

Question 40

In a ___, the left fourth arch and left dorsal aorta are obliterated and replaced by the corresponding vessels on the right side

Answer 40

right aortic arch

Question 41

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

Answer 41

interrupted aortic arch [IAA]

Question 42

A ____ occurs when the left sacrocardinal vein fails to lose its connection with the left subcardinal vein

Answer 42

double inferior vena cava

Question 43

___ 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

Answer 43

Left superior vena cava

Question 44

____ arises when the right subcardinal vein fails to make its connection with the liver and shunts its blood directly into the right supracardinal vein

Answer 44

Absence of the inferior vena cava

Question 45

A ___ is characterized by the persistence of the left anterior cardinal vein and failure of the left brachiocephalic vein to form]

Answer 45

double superior vena cava

Question 46

The persistent left anterior cardinal vein, the____ drains into the right atrium by way of the coronary sinus

Answer 46

left superior vena cava,