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216 Cards in this Set

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Cytomegalovirus
cyto-, "cell", and -megalo-, "large") is a viral genus of the viral family known as Herpesviridae or herpesviruses. It is typically abbreviated as CMV.
How CMV infects
Infected through direct contact, exposures to secretions, blood transfusions
How CMS infects fetus
Fetal infection via placenta
Early exposure increases fetal risk
30-40% risk of transmission to fetus
20-25% risk post natal
CMV causes
-The Most Common infectious cause of mental retardation, deafness, visual impairment
-Detectable abnormalities in fetus associated with poor neurodevelopmental outcome
-Mortality 30-60% within 2 years
Are infants symptomatic from CMV?
90% of congenitally infected infants are asymptomatic at birth
Neurologic issues found in 30% of these infants within 1st year of life
Assumed fetal infection in documented maternal infection IF...
Progressive IUGR
Microcephaly
Hepatomegaly/splenomegaly
Cerebral & visceral calcifications
Hydrops
CMV Ultrasound findings
Brain
Ventriculomegaly
Periventricular echogenicity
Calcifications – often non shadowing
Microcephaly
Cerebellar/CM abnormalities


Periventricular cystic changes
Diagnosis of fetal infection
via amnio
Performed at least 7 weeks after the assumed infection
Evaluate Baby for
growth (IUGR), anemia (MCA), fetal hydrops
Parvovirus
20-30% of women infected transmit to fetus
Risk of fetal death highest (20%) when transmitted <20 weeks
Parvovirus causes
Normal developmental outcome in most children who survive infection
Neurodevelopmental delays reported in severe infection
Ultrasound for Parvovirus
hydrops, anemia
Transfusions when anemic (shown via MCA)
Parvovirus Ultrasound
Ascites
-Can progress to hydrops  cardiac failure from severe anemia
-Placentomegaly
-Polyhydramnios
-Echogenic bowel
Varicella
Chicken Pox virus
Maternal infection <20 weeks 6% fetal transmission
1/3 of infected fetuses have clinical manifestations, usually cutaneous
1-2% of fetuses have severe clinical findings
Children can be neurologically asymptomatic
Neurologic impairment depends on location and extend of lesions
Varicella Ultrasound
-Intrahepatic and intracranial calcifications
-Polyhydramnios from neurologic impairment that hinders swallowing
-Limb hypoplasia, contractures
Varicella Ultrasound
Amniotic Band Syndrome
Entrapment of fetal parts by disrupted amnion
Current theory is rupture of amnion
Chorionic side of membrane is sticky > entrapment of fetal parts > vascular constriction >deformity/amputation
Occurs between 6-18 weeks
Treatment
possible in utero lysis of bands
ABS imaging
Asymmetric disruption of defects is hallmark of syndrome

Craniofacial deformities often severe – often asymmetric 

Abdominal wall defects

Edema of distal extremities secondary to constriction
May progress to limb amputations

Amnioti...
Asymmetric disruption of defects is hallmark of syndrome

Craniofacial deformities often severe – often asymmetric

Abdominal wall defects

Edema of distal extremities secondary to constriction
May progress to limb amputations

Amniotic band in contact with deformity
ABS ultrasound
http://www.sonoworld.com/TheFetus/page.aspx?id=1658
http://www.sonoworld.com/TheFetus/page.aspx?id=1658
Beckwith-Wiedemann Syndrome
Multigenetic disorder
Beckwith-Wiedemann Syndrome Classic Triad
Macrosomia
Omphalocele (usually small)
Macroglossia
Macrosomia
Omphalocele (usually small)
Macroglossia
Beckwith-Wiedemann Syndrome Other Findings
Nephromegaly – but normal echogenicity
Hepatomegaly
Hemihyperplasia
Ear groove
Polyhydramnios
BWS Risks
Increased maternal risk Preeclampsia
Increased risk for PTD
Increased risk of Wilms Tumor
Hypoglycemia (infant)

http://www.sonoworld.com/TheFetus/page.aspx?id=2527
BWS ultrasound
Meckel-Gruber Syndrome
Autosomal recessive
Autosomal recessive
Meckel-Gruber Syndrome classic triad
Classic triad:
Renal Cystic Dysplasia
Cephalocele
Polydactyly

Should have 2/3 classic features
Classic triad:
Renal Cystic Dysplasia
Cephalocele
Polydactyly

Should have 2/3 classic features
Ability to properly evaluate abnormalities hindered by severe
oligohydramnios to anyhydramnios
Meckel Gruber Syndrome Genitourinary Tract
Renal cystic Dysplasia most consistent finding

Grossly enlarged, echogenic
10-20x normal size
+/- large (visual) cysts

Large AC
Bladder/stomach small/absent
Usually anhydramnios
MGS CNS
Occipital Encephalocele – 60-80%
Dandy Walker abnormalities
Microcephaly
Agenesis CC
Ventriculomegaly
Holo
MGB Extremities
Postaxial Polydactyly
The most difficult to see secondary to anhydramnios
Clubbed feet
Short limbs
Bowing long bones
MGS Facial Malformations
Cleft lip/palate
Micrognathia
Micropthalmia
Micrognathia
Ear malformations
Sloping forehead
Sirenomelia AKA mermaid syndrome
Rare, usually lethal malformation characterized by varying degrees of lower extremity fusion and associated skeletal, GI, and genitourinary abnormalities
possible causes of Sirenomelia
Possible a vascular accident 
Blood diverted away from lower extremities in early embryology
Possibly caused by abnormality in blastogenesis
Very early defect due to disruption of caudal mesoderm in 3rd week of gestation
Possible a vascular accident
Blood diverted away from lower extremities in early embryology
Possibly caused by abnormality in blastogenesis
Very early defect due to disruption of caudal mesoderm in 3rd week of gestation
Sirenomelia imaging
Single or fused lower extremities
Absence of normally tapered lumbosacral spine
Mid trimester anhydramnios due to bilateral renal agenesis or other renal abnormalities
2VC
Sirenomelia seen when?
Findings can be seen in 1st trimester
Ultrasound image of sirenomeila
Tuberous Sclerosis
Genetic tumor disorder
Tuberous Sclerosis Genetics
Autosomal dominant
>50% new mutations
Variable expressivity
Tuberous Sclerosis causes
Rhabdomyomas
Brain lesions
Kidney lesions
Cardiac Rhabdomyomas
Multiple rhabdo’s = 100% TS
Single rhabdo = 50% TS
Tumor involves ventricles or IVS
As early as 22 weeks
Arrhythmias
Multiple rhabdo’s = 100% TS
Single rhabdo = 50% TS
Tumor involves ventricles or IVS
As early as 22 weeks
Arrhythmias
Outflow or inflow tract obstruction
> congerstive heart failure > hydrops > death
Renal lesions
Cysts
Angiomyolipomas
Appreciated after birth
Skeletal Dysplasia
Defined as abnormal growth & density
Dwarfism occurs secondary to?
Skeletal Dysplasia
Lethal forms of SD are ?
severe in their presentation
Nonlethal SD are less ?
severe in appearance
Assess limb shortening
measure all long bones (R/U/T/F/H/Fm)
Dysplasia if
2 standard deviations below mean
Assess bone contoure
thickness, abnormal bowing, fractures, ribbon-like appearance
Degree of Ossification?
: hypomineralization
Thoracic Circumference & Shape looks like?
Narrow chest, Bell shaped
SD Hand & Foot Anomalies
Talipses, polydactyly
Talipses, polydactyly
SD Face & Profile
Clefts, frontal bossing, micrognathia, hypertelorism
Clefts, frontal bossing, micrognathia, hypertelorism
Other SD anomalies
Hydrocephalus
Heart defects
Hydrops?
Rhizomelia
Shortening of proximal bone (humerus, femur)
Shortening of proximal bone (humerus, femur)
Mesomelia
Shortening of middle segments (R/U/T/F)
Shortening of middle segments (R/U/T/F)
Micromelia
Shortening of entire extremity
Shortening of entire extremity
Thanatophoric Dysplasia
severe skeletal disorder characterized by a disproportionately small ribcage, extremely short limbs and folds of extra skin on the arms and legs.
severe skeletal disorder characterized by a disproportionately small ribcage, extremely short limbs and folds of extra skin on the arms and legs.
most common form of lethal SD
Thanatophoric Dysplasia
Thanatophoric Dysplasia
Thanatophoric Dysplasia Type 1:
Curved, short femurs; flat vertebral bodies
Curved, short femurs; flat vertebral bodies
Thanatophoric Dysplasia Type 2:
Straight, short femurs; flat vertebral bodies; cloverleaf skull
Straight, short femurs; flat vertebral bodies; cloverleaf skull
Thanatophoric Dysplasia US Findings
Severe micromelia
Cloverleaf Skull
Narrow Thorax w/ shortened ribs
Protuberant Abdomen
Frontal Bossing
Hypertelorism
Flat Vertebral Bodies
Polyhydramnios
Hydrocephalus
Hydrops
Narrow Thorax
Shortened Ribs
Achondroplasia
Results from decreased endochondrial bone formation

Usually spontaneous mutation; can be autosomal recessive
The most common nonlethal SD
Achondroplasia
Advanced paternal age increases risk of?
Achondroplasia
Achondroplasia Prognosis
depends on type
Heterozygous Achondroplasia:
good survival rate, normal intelligence
May require orthopedic or neurologic surgical intervention
Homozygous Achondroplasia
Lethal
US findings more severe, including narrow thorax
US Findings: *MAY NOT BE EVIDENT UNTIL AFTER ?
22 WEEKS!!!*
Achondroplasia US Findings
Rhizomelia
Macrocephaly
Trident Hands (short proximal/middle fingers)
Depressed nasal bridge
Frontal Bossing
Mild Ventriculomegaly
Rhizomelia
Macrocephaly
Trident Hands (short proximal/middle fingers)
Depressed nasal bridge
Frontal Bossing
Mild Ventriculomegaly
Achondrogenesis
is a number of disorders that are the most severe form of congenital chondrodysplasia (malformation of bones and cartilage). These conditions are characterized by a small body, short limbs, and other skeletal abnormalities.
Rare, lethal SD
Achondrogenesis
Achondrogenesis cause
Caused by cartilage abnormalities that result in abnormal bone formation and hypomineralization
Achondrogenesis type 1
: More severe, autosomal recessive
: More severe, autosomal recessive
Achondrogenesis type 2
Less severe, spontaneous mutation
Less severe, spontaneous mutation
Achondrogenesis Ultrasound
US Findings:
Severe micromelia
Decreased or absent ossification of spine
Macrocephaly
Short trunk
Short thorax & Short Ribs
Micrognathia
Polyhydramnios
Hydrops
US Findings:
Severe micromelia
Decreased or absent ossification of spine
Macrocephaly
Short trunk
Short thorax & Short Ribs
Micrognathia
Polyhydramnios
Hydrops
Achondrogenesis Ultrasound
Achondrogenesis Ultrasound Legs
Achondrogenesis Ultrasound arm
Osteogenesis Imperfecta
Rare disorder of collagen production leading to brittle bones
Osteogenesis Imperfecta Manifests in ?
teeth, skin, ligaments, blue sclera
Osteogenesis Imperfecta has how many types?
4 types
4 types
Osteogenesis Imperfecta Type 1:
Autosomal Dominant, mild presentation. Not likely diagnosed prenatally
Autosomal Dominant, mild presentation. Not likely diagnosed prenatally
Osteogenesis Imperfecta Type 2
Most severe; lethal. May be dominant, recessive, spontaneous mutation
Most severe; lethal. May be dominant, recessive, spontaneous mutation
Osteogenesis Imperfecta type 3
Severe. May be dominant or recessive
Severe. May be dominant or recessive
Osteogenesis Imperfecta type 4
Autosomal Dominant, also mild presentation. Similar to type 1; not likely diagnosed prentally
Autosomal Dominant, also mild presentation. Similar to type 1; not likely diagnosed prentally
Prognosis of Osteogenesis Imperfecta
Type 1 & 4: multiple fractures, short stature

Type 1 children may also suffer from kyphoscoliosis & deafness

Type 2: Infants die shortly after birth secondary to respiratory complications

Type 3: produces significant handicaps with progressive deformities of long bones and spine
Osteogenesis Imperfecta Type 2 US Findings
Generalized hypomineralization, especially calvarium (compressible) 
Multiple fractures: long bones, ribs, spine
Narrow thorax
Micromelia
Polyhydramnios
Generalized hypomineralization, especially calvarium (compressible)
Multiple fractures: long bones, ribs, spine
Narrow thorax
Micromelia
Polyhydramnios
Osteogenesis Imperfecta Type 3 US Findings
Similar to type 2 but less severe
Similar to type 2 but less severe
Short-Rib Polydactyly Syndrome
Lethal SD
Short ribs, short limbs, polydactyly
Autosomal Recessive
Lethal SD
Short ribs, short limbs, polydactyly
Autosomal Recessive
US Findings
Short-Rib Polydactyly Syndrome
Narrow thorax, short ribs
Polydactyly
Micromelia
Midline Facial Cleft
Narrow thorax, short ribs
Polydactyly
Micromelia
Midline Facial Cleft
Other Short-Rib Polydactyly Syndrome findings can include:
CNS anomalies
Cardiovascular system
Genitourinary tract
Polyhydramnios
Postural Anomalies
The abnormal movements can also lead to abnormal contractures & postural deformities
Normal development requires
normal movement
Decreased fetal movement caused by:
Oligohydramnios
Multiple gestations
Congenital uterine anomalies
Fetal Nerves
Connective Tissue
Musculature
Arthrogryposis Multiplex Congenita
Severe contractures of extremities b/c of abnormal innervation, muscles, & connective tissue
Severe contractures of extremities b/c of abnormal innervation, muscles, & connective tissue
Arthrogryposis Multiplex Congenita caused by?
May be sporadic or inherited
Arthrogryposis Multiplex Congenita Prognosis
Varies
US Findings Arthrogryposis Multiplex Congenita
Rigid Extremities
Flexed Arms
Hyperextension of knees
Clinched Hands
Talipses
Poly/oligo
+/- CNS, renal, facial Anomalies
Rigid Extremities
Flexed Arms
Hyperextension of knees
Clinched Hands
Talipses
Poly/oligo
+/- CNS, renal, facial Anomalies
US Findings Arthrogryposis Multiplex Congenita
Lethal Multiple Pterygium Syndrome Characterized by ?
webbing Across joints & multiple contractures
webbing Across joints & multiple contractures
Lethal Multiple Pterygium Syndrome is autosomal ________?
Autosomal Recessive
Lethal Multiple Pterygium Syndrome US Findings
Limb Contractures
Webbing Across Joints
Cystic Hygroma
Limb Contractures
Webbing Across Joints
Cystic Hygroma
Misc Limb Abnormalities Hand Anomalies
Syndactyly: fused fingers
Syndactyly: fused fingers
Ectrodactyly
AKA Split Hand orLobster Claw Deformity
AKA Split Hand orLobster Claw Deformity
Ectrodactyly ultrasound
Missing digits
Hypodactyly
Hypodactyly
Polydactyly:
too many digits
too many digits
Clinched Hands
Talipes
club foot
club foot
Talipes Findings and causes
Half are unilateral
Male predominance
Usually idiopathic & isolated
Can be associated with chromosomal Abnormalities, NTD’s, syndromes, musculoskeletal disorders
Oligohydramnios, multiple gestations
Rocker Bottom Foot
Prominent Heel and a convex sole
Rocker Bottom Foot Associated with ?
multiple syndromes, chromosomal abnormalities (T18!!)
multiple syndromes, chromosomal abnormalities (T18!!)
Environmental teratogens increase risk by?
7%
Most SENSITIVE period for cardiac development?
3 ½ - 6 ½ weeks
Cardiovascular system 1st organ system to reach _______ _______.
functional state
At end of which week blood is circulating
3rd
Which week heart starts to beat?
5th
Fetal circulation
Foramen Ovale:
Opening between two atrias, blood flows from right to left
Opening between two atrias, blood flows from right to left
Ductus Arteriosus:
Communication b/t pulmonary artery & descending aorta
Closes after birth
Communication b/t pulmonary artery & descending aorta
Closes after birth
Ductus Arteriosus ultrasound
DA Ultrasound
Fetal Risk Factors:
IUGR
Cardiac Arrhythmias
Abnormal Amnio (trisomy)
Abnormal Heart Rate
Other anomalies
Renal anomalies
GI Anomalies
When more than 1 abnormality present, the risk for cardiac defects increases further
Maternal Risk Factors
Fam Hx cardiac defect
DM (VSD, Transposition GA’s, ToF)
Lupus
Teratogen (lithium, Alcohol)
Familial Risk Factors:
Genetic Syndromes, heart defect in previous child
Parent with congenital heart defect
Normal Heart Images
Cardiac Axis
Situs
4C View
LVOT
RVOT
Aortic Arch
M-Mode
cardiac normal axis
45 degree axis from spine
Heart normal size
Cardiac circumference is ½ chest circumference
Small chest or large heart?
Four-Chamber view
Apex pointed to left
Right side slightly larger than left
Foramen Ovale flap visualized bowing into left atrium
Moderator band identified in right ventricle
Mitral and tricuspid valves assessed
Tricuspid valve sits slightly inferior to mitral...
Apex pointed to left
Right side slightly larger than left
Foramen Ovale flap visualized bowing into left atrium
Moderator band identified in right ventricle
Mitral and tricuspid valves assessed
Tricuspid valve sits slightly inferior to mitral valve

Pulmonary veins seen entering Left Atrium
Right lower vein not imaged
SVC & IVC enters right atrium
Interventricular Septum
4 chamber view
normal 4chamber view clip
http://www.youtube.com/watch?v=8lnuJvlDSE0
Examples of normal AND abnormal
http://www.centrus.com.br/DiplomaFMF/SeriesFMF/doppler/capitulos-html/chapter_12.htm
Things to consider with out flow track
Size/width of OFT’s
Position
LVOT Standard view:
Long Axis
RVOT Standard View
Short axis
Sometimes Long axis
LVOT US
http://www.youtube.com/watch?v=hfJOZ7FJMqU
http://www.youtube.com/watch?v=hfJOZ7FJMqU
RVOT
http://www.medison.ru/uzi/eho393.htm
Right Ventricular Outflow Tract
http://www.medison.ru/uzi/eho393.htm
Right Ventricular Outflow Tract
OFT’s Crossing
http://www.youtube.com/watch?v=AfDTNciZl9g
AO arch
Levocaria:
normal position
Levoposition
Heart displaced further left than normal
Dextrocardia
Heart in right chest, apex points right
Dextroposition:
Heart is in right chest, apex points medially or left
Mesocardia
Apex points midline
Ventricular Septal Defect - VSD
Defect (hole) in the septum of the heart
20% of all congenital heart disease
Ventricular Septal Defect - VSD
Ventricular Septal Defect - VSD
Associated cardiac abnormalities 50%
Ventricular Septal Defect - VSD
Ventricular Septal Defect - VSD
Ventricular Septal Defect - VSD Membranous
More common – 75%
high up in ventricle (near crux) in outflow tract of left ventricle, immediately below aortic valve
VSD Muscular:
10-15%
Defect in muscular portion of of septum, anywhere from apex to to base
Large, small, single, many
% of VSD’s close within 2 yrs of life
40
% close by 5 yrs
60
VSD US
Atrioventricular Septal Defect - AVSD
AKA endocardial cushion defect

deficiency of the atrioventricular septum of the heart. It is caused by an abnormal or inadequate fusion of the superior and inferior endocardial cushions with the mid portion of the atrial septum and the muscular...
AKA endocardial cushion defect

deficiency of the atrioventricular septum of the heart. It is caused by an abnormal or inadequate fusion of the superior and inferior endocardial cushions with the mid portion of the atrial septum and the muscular portion of the ventricular septum.
Atrioventricular Septal Defect - with T21
40% of cases
ASVD prognosis with surgery
Excellent
AVSD Imaging Best diagnostic clue
= missing crux of heart
AVSD imaging Color doppler:
Confirm defects
Regurgitation

http://www.youtube.com/watch?v=921fShockXI
US normal vs. AVSD
AVSD US
Ebstein Anomaly
Apical displacement of septal and posterior tricuspid valve leaflets
Valve leaflet lowered into right ventricle
Results in atrialization of RV

- congenital heart defect in which the septal leaflet of the tricuspid valve is displaced towards t...
Apical displacement of septal and posterior tricuspid valve leaflets
Valve leaflet lowered into right ventricle
Results in atrialization of RV

- congenital heart defect in which the septal leaflet of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.
In utero mortality rate of Ebstein Anomaly
45%
Absence of antegrade flow across pulmonary valve is
lethal
Ebstein Anomaly Best Imaging Clue
Right Atrial Enlargement***
Apical Displacement of tricuspid valve***
sits lower in heart than normal
Normal = 1-2mm lower than mitral valve
Right Atrial Enlargement***
Apical Displacement of tricuspid valve***
sits lower in heart than normal
Normal = 1-2mm lower than mitral valve
Ebstein Anomaly - Imaging
Cardiomegaly
Right atria large while right ventricle small from atrialization – only a small amount of functional right ventricle left
Pulmonary artery often from lack of flow
Color Doppler to eval regurg through tricuspid valve
Cardiomegaly
Right atria large while right ventricle small from atrialization – only a small amount of functional right ventricle left
Pulmonary artery often from lack of flow
Color Doppler to eval regurg through tricuspid valve
Ebstein Anomaly - Imaging
http://www.sonoworld.com/TheFetus/page.aspx?id=3002
Hypoplastic Left Heart
Hypoplastic Left Heart
Hypoplastic Left Heart
Underdeveloped left side of the heart (LV + LA)
Mitral stenosis/atresia
Aortic stenosis/atresia
Hypoplastic ascending Ao and coarctation
Hypoplastic Left Heart Lethal if
-untreated

20% IUFD
Improving surgical techniques = 80 to near-100% mortality
Long term survival unknown
Hypoplastic Left Heart Aneuploidy:
13% turners
T13/18
The amount of hypoplasia depends
on when the left sided atresia developed
Hypoplastic Left Heart, Right vent supplies both
pulmonic and systemic blood flow
Hypoplastic Left Heart Pulmonary veins return blood to
right atrium
Hypoplastic Left Heart:nOverload of right vent may lead to
CHF >Pericardial effusions > Hydrops
Hypoplastic Left Heart - Imaging
Abnormal 4-chamber view
Most cases detected at anatomy scan
Hypoplastic Left Heart - Imaging Left ventricle:
Small or nonexistent 
Hypocontractile and hypertrophic
Small or nonexistent
Hypocontractile and hypertrophic
Hypoplastic Left Heart Right ventricle
Dilated and often wraps under left ventricle apex
Hypertrophied with good function
Dilated and often wraps under left ventricle apex
Hypertrophied with good function
Hypoplastic Left Heart Atria
Interatrial septum bowed left to right
Only outlet for flow from left atrium
Left atrium is hypertrophic, right atrium dilated
Hypoplastic Left Heart Pulmonary artery
dilated
Hypoplastic Left Heart Ductuc arteriosus (DA)
dilated
Hypoplastic Left Heart Ascending aorta
small
Associated with coarctation
Hypoplastic Left Heart case
Case Report
http://www.sonoworld.com/TheFetus/page.aspx?id=2683
http://www.sonoworld.com/TheFetus/page.aspx?id=2525
Congenital heart disease with 4 components
Tetralogy of Fallot
Tetralogy of Fallot
Tetralogy of Fallot
RVOT obstruction/stenosis
VSD
Overriding Ao
Right ventricular hypertrophy (see after birth)
RVOT obstruction/stenosis
VSD
Overriding Ao
Right ventricular hypertrophy (see after birth)
4 Chamber view normal in >95% of cases
Tetralogy of Fallot
Tetralogy of Fallot OFT imaging key in diagnosis
Ascending Ao overrides perimembranous VSD
Pulmonary artery narrow
Ascending Ao overrides perimembranous VSD
Pulmonary artery narrow
Most Common Cyanotic congenital heart disease
Tetralogy of Fallot
Tetralogy of Fallot
Tetralogy of Fallot Prognosis dependent on aneuploidy
Chromosomal abnormality in 45% of cases

Isolated = excellent long term prognosis with repair
>94% survival
Prognosis worse if there is absence of the pulmonary valve
32% mortality at 4 years
Transposition of Great Arteries
Ao and PA are switched
Ao comes from right ventricle
PA comes from left ventricle
Ao and PA are switched
Ao comes from right ventricle
PA comes from left ventricle
OFT’s do not criss cross – instead they run parallel to each other
Transposition of Great Arteries
Transposition of Great Arteries
DA and Foramen Ovale = the only communication between right and left side of fetal circulation
These close at birth = BLUE BABY
Surgery required within 1st week of life
Transposition of Great Arteries
Transposition of Great Arteries
Truncus Arteriosis
Single vessel (truncus) arising from the heart
Gives rise to Ao and PA
Single tuncal valve  with 1-6 cusps
May cause stenosis +/- regurg
Single vessel (truncus) arising from the heart
Gives rise to Ao and PA
Single tuncal valve with 1-6 cusps
May cause stenosis +/- regurg
40% of liveborn infants with truncus 22q11 deletion
Prognosis dependent on associated abnormalities
Outcome worse with interruption of Ao Arch
Truncus Arteriosis
Truncus Arteriosis
Hypertrophic Cardiomyopathy
Primary disorder of cardiac muscle
Thickened but non dilated left ventricle
Primary disorder of cardiac muscle
Thickened but non dilated left ventricle
causes of Hypertrophic Cardiomyopathy
Familial
50% have a genetic mutation in chromosome 1, 14, or 15
Non-familial: Noonan Syndrome, other chromosomal abnormalities
Other causes:
Diabetes Mellitus
50% type 1
25% type 2
Rare in gestational DM
Metabolic Causes
Fetal renal disease
TTTS
Hypertrophic Cardiomyopathy mortality rate
High mortality rate – 52%
Hypertrophy of myocardium
Asymmetric in distribution
Septum most often involved
May be confined to apex or free wall
May be symmetric (concentric hypertrophy)
+/- cardiomegaly
Hypertrophy of myocardium
Asymmetric in distribution
Septum most often involved
May be confined to apex or free wall
May be symmetric (concentric hypertrophy)
+/- cardiomegaly
Hypertrophic Cardiomyopathy
Myocardial disease not usually associated with structural or pericardial malformations
Dilated Heart with decreased systolic function
Final common pathway for diverse disease process that leads to heart failure
Dilated Cardiomyopathy
Dilated Cardiomyopathy
Dilated Cardiomyopathy Overall mortality
rate 82%
57% IUFD, remainder in neonatal period
Hydrops poor prognostic sign
Post natal options are few
Survivors may require transplant
Dilated Cardiomyopathy Imaging
Cardiomegaly
Poor myocardial contractility
Myocardium thin
+/- hydrops
Pulsed Doppler:
Reserved flow in:
IVC
DV
Pulsatile UV
Atrioventricular regurg color and pulsed
Ectopic or extra beats which can arise anywhere (atria or ventricle) within myocardium to produce an irregular rhythm
Irregular Rhythm

There can be one, many or blocked beats
Irregular Rhythm
1-2% pregnancies will have an arrhythmia
PAC/PVC account for 90%
Usually requires no treatment
Most resolve by by delivery – very rare to cause problems in neonate
Frequent PAC’s 2-5% risk developing SVT
Reduction in maternal caffeine, alcohol, nicotine suggested
Periodic monitoring for tachycardia
Irregular Rhythm Imaging
M-Mode
Curser needs to be placed over atria + ventricle
PAC:
Early atrial contraction
Compensatory pause of left ventricle
Normal rhythm resumes
Tachyarrhythmia
Supraventricular tachycardia (SVT): any tachycardia with an origin above ventricles
Rates typically > 200
with sustained tachyarrhythmia
Hydrops develops 50-75%
Harder to treat
Tachyarrhythmia Imaging
Sustained heart rate > 200
M-mode through atrium + ventricle
Atrioventricular Block
Transmission of electrical impulses from atria to vents is blocked
Atrioventricular Block May be caused by
immature conducting system
Absense of connection to the AV node
Abnormal anatomic position of AV node
Maternal Lupus increased risk
Atrioventricular Block diagnosis
Diagnosed with M-Mode through Atria and vent
2nd degree heart block most commonly seen
2:1 (2 atrial beats to every 1 ventricular beat)
Diagnosed with M-Mode through Atria and vent
2nd degree heart block most commonly seen
2:1 (2 atrial beats to every 1 ventricular beat)