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OBGYN II Lecture 6- Congenital Anomalies of the Thorax
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Gravity
Terms in this set (50)
shape of thoracic cavity
bell shaped with ribs, clavicles & diaphragm as borders
fetal lungs
-homogeneous
-increasing echogenicity as lung development progresses
diaphragm in fetus
smooth, hypoechoic arched line separating chest & abdominal contents
Fetal cardiothoracic (C/T) circumference ratio
-assessment of fetal cardiac & thoracic/chest wall anomalies
-normally < 0.5 throughout gestation
apex of the heart
points left and touches anterior chest wall
heart chamber seen to right of midline
ONLY posterior aspect of right atrium
heart chamber closest to spine
left atrium
levoposition of heart
heart is displaced further than normal into the left chest
dextroposition of heart
1. heart is located in right side of chest
2. cardiac apex points medially
**
heart maintains normal axis
**
dextrocardia
heart in the right chest with apex pointing to the right
mesocardia
heart is central with apex pointed anteriorly
abnormal cardiac axis associations
associated with 50% mortality & abnormal cardiac position with 81% mortality
development of the diaphragm
1. develops at about 9 weeks, visualized as early as 10 weeks
2. divides the 2 cavities
situs inversus
total or partial reversal of abdominal organs
ectopia cordis
heart develops outside chest cavity
pericardial effusions
abnormal fluid collection surrounding epicardial layer of the heart (> 2mm)
hydrothorax (pleural effusion)
anechoic pleural fluid displacing lungs away from chest wall & compresses lungs
pulmonary hypoplasia
underdeveloped lungs
causes of pulmonary hypoplasia
1. masses compressing lungs
2. skeletal malformations restricting lung growth
3. severe prolonged oligohydraminos
4. majority associated with major structural or chromosomal abnormalities***
pleural effusion
accumulations of fluid within pleural cavity
most common cause of pleural effusion
chylothorax
chylothorax
right-sided, unilateral collection of fluid from malformed thoracic duct leaking lymphatic fluid
associated anomalies with pleural effusion
1. small percent associated with chromosomal abnormalities
2. in fetus with cardiac mass
3. poor perinatal outcome associated with hydrops, underlying structural abnormalities, pulmonary hypoplasia & early gestational age at diagnosis
Cystic adenomatoid malformation
intrapulmonary mass of variable echogenicity can shift mediastinum creating hydrops
Congenital Cystic Adenomatoid Malformation (CCAM)
1. failure of bronchial maturation & lack of normal alveoli
2. abnormal formation of bronchial tree occurs before 7 weeks during embryologic development
diagnosis of Congenital Cystic Adenomatoid Malformation (CCAM)
1. diagnosed as early as 16 weeks
2. usually unilateral
4. prognosis varies on type of lesion: type I lesion > type II & III
100% mortality rate of CCAM
if hydrops develops and is untreated as a result of CCAM
CCAM- type I
1. most common type
2. single or multiple cysts greater than 2 cm & up to 10 cm replace normal lung tissue
CCAM- Type II
multiple small cysts less than 1 cm
CCAM- Type III
large, echo-dense masses of entire lung lobe
Anomalies associated with CCAM
1. anomalies are more frequent if CCAM is bilateral
2. Chromosomal anomalies = rare
Bronchopulmonary Sequestration
congenital anomaly where during development a mass of lung tissue becomes isolated from rest of lung
Extralobar Bronchopulmonary Sequestration
NON-FUNCTIONING supernumerary lobe of the lung that has its own blood supply
intralobar Bronchopulmonary Sequestration
abnormal lung tissue that shares systemic blood supply and venous drainage
extralobar & intralobar similar characteristics
Both are a spherical or triangular echogenic mass in the inferior portion of the thorax or upper abdomen
Bronchogenic cyst
1. most common lung cyst detected prenatally
2. small, simple cyst near mediastinum or centrally in lung
3. normal AFI
Diaphragmatic hernia
1. complex mass, usually on left side, creates mediastinal shift
2. associated with substantial morbidity/mortality
indications of a diaphragmatic hernia diagnosis
1. Peristalsis of bowel in thorax
2. Displaced stomach
ventricular septal defect
1. most common congenital lesion of the heart
2. septum divided into 2 segments- the membranous & muscular areas
membranous defects of ventricular septal defects
1. common type
2. found just below aortic leaflets sometimes its sucked into defect
Complete Atrioventricular Septal Defect
1. endocardial cushion defect
2. a single undivided free floating leaflet stretching across both ventricles
general prognosis for fetus with cardiomyopathy
very poor- severely decreased cardiac function leads to congestive heart failure with pericardial effusion, bradycardia, & death
Tetralogy of Fallot
*
most common form of cyanotic heart disease
*
1. High, membranous ventricular septal defect
2. Aorta displaced over ventricular septal defect
3. Pulmonary valve stenosis
4. Right ventricular hypertrophy (thickening of wall)
Hypoplastic left heart syndrome
1. small, hypoplastic left ventricle
2. with aortic and/or mitral dysplasia or atresia
3. autosomal-recessive condition
Ebstein's Anomaly
1. posterior & septal leaflets of tricuspid valve are displaced downward into right ventricle
2. large right atrium with pathologically small right ventricle
Ebstein's Anomaly in utero
frequently causes cardiomegaly, hydrops & arrhythmias
Transposition of the Great Arteries
1. the aorta is connected to the right ventricle
2. the pulmonary artery is connected to the left ventricle
problems that occur in the neonatal period with Transposition of the Great Arteries
happens when there is inadequate mixing of oxygenated & unoxygenated blood
Coarctation of the aorta
1. narrowing of aortic arch at the site of the ductal insertion near the left subclavian artery
2. diagnosed after delivery when the ductus closes & narrowed portion becomes evident
The most common cardiac tumors in fetuses
rhabdomyomas tending to be multiple & involve the septum
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