OBGYN II Lecture 6- Congenital Anomalies of the Thorax

Terms in this set (50)

shape of thoracic cavity

bell shaped with ribs, clavicles & diaphragm as borders

-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

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**

heart in the right chest with apex pointing to the right

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

total or partial reversal of abdominal organs

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

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

1. most common type
2. single or multiple cysts greater than 2 cm & up to 10 cm replace normal lung tissue

multiple small cysts less than 1 cm

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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