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STUDIO DEL CUORE CON RM FETALE Lucia Manganaro Dipartimento di Scienze Radiologiche Oncologiche e antamopatologiche

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Page 1: Studio del cuore con rm fetale Manganaro

STUDIO DEL

CUORE CON RM

FETALE

Lucia Manganaro

Dipartimento di Scienze Radiologiche

Oncologiche e antamopatologiche

Page 2: Studio del cuore con rm fetale Manganaro

BACKGROUND

Congenital heart disease is one of the most frequent prenatal malformations (incidence of 5/1000 live births),it represents the primary cause of death in the first year of life

Considering the wide range of severity, a good prenatal examination acquires a great importance in order to formulate an early diagnosis and improve the pregnancy management

Nowadays investigation of CHD is performed with echocardiography considered the standard reference for diagnosis

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Various studies demonstrated the potential role of fetal magnetic resonance imaging as an adjunctive imaging technique in the prenatal evaluation of CHD.

MRI may add other clinical information regarding associated extracardiac pathologies *

MRI could be advisable from the second trimester of pregnancy, when a preliminary ultrasound examination proves inadequate or diagnostically inconclusive *

MRI could offer a better imaging compared to US in an advanced gestational stage because of the progressive reduction of amniotic fluid and the ribs ossification

* Manganaro L; Savelli S; Di Maurizio M et al. Assessment of congenital heart disease (CHD): Is there a role for fetal magnetic resonance imaging

(MRI)?. European Journal of Radiology 72 (2009) 172-180.

Role of fetal MRI

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KEY POINTS

Techniques

Anatomical considerations

Classification

Discussion

Conclusions

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STANDARD PROTOCOL OF STUDY

Localization sequence : T2-weighted half-Fourier single-shot turbo spin-echo sequence (HASTE)(TR 1,000, TE 118/151, matrix 256×134, slice thickness 6 mm, FOV 27×20 cm) acquired in the coronal plane to evaluate fetal lie.

Anatomical study: T2 weighted HASTE sequences with a slice thickness of 3-4 mm, performed on a multiplanar orientation.

Static phase of study: ultrafast gradient-echo (GRE) sequences with SSFP, known as fast imaging with steady-state free precession (True-FISP)( TR/TE 3.5/1.5, matrix 256×144, slice thickness 3–4 mm, FOV 40×30 cm), oriented in the three planes of the space

Dynamic phase of study : real-time cine MRI True-FISP 2D sequences, known as TF2D on Magnetom Avanto (TR/TE 248.5/1.1, matrix 192×99, slice thickness 3–4 mm, FOV 38×26 cm, FA 77°, temporal resolution two slices/s), repeated dynamically (40 times)

MRI examination takes between 20 and 45 minutes depending on the movement of the fetus

1.5-T MR unit system, phased body coil (Siemens Magnetom Avanto, Erlangen,Germany).

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suboptimal anatomical visualization of all the fetus body,

in particular of static fluid fast acquisition time

low ratio rumors-signal enables the avoidance of holding

mother’s breath highly sensitive to flow, heart and blood-filled vessels are

visualised as a lack of signal

T2 HASTE sequences

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True-FISP (fast imaging with steady-state

free precession) sequences

Are not influenced by movement

highlight structures wirh fluid in motion showing high signal intensity

both static and motion fluid are visualised with elevated signal

The short echo time allows a good evaluation of the myocardial thickness and interventricular septum thanks to the higher contrast resolution blood-tissue on endocardium *

* Gorincour G, Bourliè Re-Najean B, Bonello B et al. Feasibility of fetal cardiac magnetic resonance imaging: preliminary

experience. Ultrasound Obstet Gynecol 2007; 29: 105–110

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suboptimal visualization of the cardiac

movement

effective evidentation of the morphological

features

impossibility of fetal cardiac triggering

Real-time cine MRI True-FISP 2D

sequences

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NEW PROSPECTIVES

Gradient Echo T1 w 3D

Angiography like

sequence:

Technical parameters:

TR: 25

TE: 8.4

Flip angle: 10

FOV: 17.7 cm x 29.9 cm

Matrice: 256 x 192

Slice thickness: 4.00

Distance factor: 50%

TA: 40s

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TRANSVERSAL VIEWS: - Four chambers

- Five chambers

- Three vessels

SAGITTAL VIEWS: -

Short axis left ventricle

- Tricuspid-aortic cut

- Long axis of the ductus

arteriosus

- Long axis of the aortic arch

ANGULATED VIEWS: -

Long axis of the left ventricle

- Aortic arch and ductus

arteriosus

Projection

Page 11: Studio del cuore con rm fetale Manganaro

T2 HASTE sequences evaluate fetal lie (longitudinal, transverse,

oblique), presentation (cephalic, breech, shoulder), and anatomical

details

True-FISP sequences allow the highlighting of the cardial apex

orientation and the cardial situs

ANATOMICAL CONSIDERATIONS

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ANATOMICAL CONSIDERATIONS

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ANATOMICAL CONSIDERATIONS

Page 14: Studio del cuore con rm fetale Manganaro

Four chamber view

- heart size and orientation

- cardiac apex position

- ventricular septum angulation

- dimension of cardiac chambers

- integrity of ventricular and atrial

septum

Anatomy 1

RV

RA

LA

LV

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Five chamber view

- aorta arising from the left ventricle in the center of the heart

Anatomy 2

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Three vessels view:

- size and position of

pulmonary artery (PA),

aorta (Ao) and of

superior vena cava

(SVC)

Aortic arch view:

- aortic arch and SVC

Anatomy 2

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Short axis of the left

ventricle:

- right ventricle shape

- left ventricle shape

- position of both ventricles

Tricuspid-Aortic view:

- right chambers position

- IVC and SVC outflow

- aortic outflow

Anatomy 4

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Long axis of the ductus arteriosus :

- origin of pulmonary artery that connects to the Ao via the ductus, forming

a ductal arch

Anatomy 5

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Long axis of the left ventricle:

- origin of the Aorta from left ventricle in the center of the heart

Anatomy 6

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Anatomy 7

Aortic arch and ductus arteriosus view

- Simultaneous view of aortic arch and ducrus

- Same size of two vessels

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CINE-MR videos

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Fetal MRI study is based on the analisis of both direct and

indirect signs of pathology

DIRECT SIGNS INDIRECT SIGNS

cardial situs

cardiothoracic index

anomalies of the volume and

conformation of chambers and

myocardial

malrotation of the cardiac axis

septal defects

vessel’s pathologies

anomalies of the origin, size and

course of the great arteries

absence of anatomical structures

increased size of vessel before a

stenotic region

hypertrophy of the papillary

muscles

presence of cardiomegaly

pericardial effusion

Detection of CHD

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To simplify the understanding of CHD we identify

7 categories:

1. cardial situs anomalies

2. right and left ventricular hypoplasia

3. cardiac masses

4. great vessel abnormalities

5. abnormalities of transposition and connection

6. defects of inflow and outflow

7. septal defects

Characterization of CHD

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a

c

d

Situs inversus is easy to recognize after a first valuation of the position of the fetus compared to the mother in order to define the left and right sides. Moreover fetal MRI allows assessment of the visceroatrial situs in relation to the bronchi. The fluid-filled bronchial tree appears as high-signal-intensity structures on SSFP images *.

*Brugger PC, Stuhr F, Linder C, Prayer D. Methods of fetal MR: beyond T2-weighted imaging. Eur J Radiol2006

1.Cardial situs anomalies

b 27-week gestation fetus with complete situs inversus. Fetus position is transversal with head on the right side of

the mother as indicated by the position of the liver( white arrow) and stomach (red arrow)

Figure a) represent the posterior plan of the coronal view acquired on the mother, it shows the liver (arrow)

instead of the stomach, which is endeed shown on an anterior plane of the coronal view ( figure b).

c)Liver is located on the left side of the fetus. d) heart is located on the right side.

Page 25: Studio del cuore con rm fetale Manganaro

Right ventricular

hypoplasia:

reduction of the right

ventricular cavity with thick

walls (three vessels view)

Left ventricle

hypoplasia:

mitral and aortic atresia, both

left chambers appear reduced

with virtual cavity,the apex is

composed of the right

ventricle

2.Hypoplasia

32-week gestational age fetus with hypoplastic left heart syndrome. a) hypertrophic right

ventricle ,right ventricle occupies the aepex of the heart

a b

26-week gestational age fetus with hypoplastic lright heart syndrome. a)

hypertrophic left ventricle

Page 26: Studio del cuore con rm fetale Manganaro
Page 27: Studio del cuore con rm fetale Manganaro

Rhabdomyomas :

• most frequent prenatal tumors,

• usually associated with tuberous sclerosis

• frequently distributed in the left ventricle (move simultaneously with ventricles)

• appear hypointense in T2 weighted sequences and hyperintense in T1 weighted

sequences

• it’s required the study of the brain

3.Cardiac masses 1

33-week gestational age fetus affected by tuberous sclerosis,a) nodular hypointense lesion (arrow) located in the right

ventricle. b-c) subependymal rhabdomyoma located next to the lateral ventricle (arrows)

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3. Cardiac masses

?

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Teratomas:

• inhomogeneous in T2 weighted

sequences for the combination of

solid and fluid components

• differential diagnosis with thorax

pathologies (ex: CCAM , BPS)

3.Cardiac masses 2

25 week gestation fetus affected by perycardial teratoma.

Multilobulated lesion with inhomogeneous signal in T2w

sequences (arrow), located in the perycardium.

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Aortic coartaction (CoA): reduction of the left ventricle; There is considerable overlap in the relative size of

the aortic arch, therefore a diagnosis of coartaction is generally a provisional diagnosis even in

echocardioghraphy. preliminary experiences demonstrated how measurement, on the three vessel view, of

the main mediastinal pulmonary artery to ascending Ao diameter ratio can be a helpful tool in distinguishing

true CoA[-]

Slodki M, Rychik J, Moszura et Al.Measurement of the great vessels in the mediastinum could help distinguish true from false-positive

coarctation of the aorta in the third trimester.J Ultrasound Med. 2009 Oct;28(10):1313-7.

.

4.Great vessels abnormalities

32 week gestation fetus affected by aortic coartaction (arrows). a-b) Gradient Echo T1 weighted 3D sequences

a b c

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4.Great vessels abnormalities2

Aortic corctation 2:

28-week gestation

fetus.

a)vessel view (arrow)

b)hypoplastic left

ventricle (arrow)

c)aortic coartaction

(arrowhead)

Aortic corctation 3:

27-week gestation fetus

with DiGeorge

syndrome. Both axial

scans illustrate the

aortic coartaction (long

arrow) and the thymus

absence (short arrow) .

a b c

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Transposition:

aorta arises form the right ventricle and the pulmonary artery from the left

ventricle, commonly associated to concordant atrio ventricular connection.

5.Abnormalities of great artery position and

connection 1

Ao Ao

RA

AP

LA

Ao

RV

RA

IVC

SVC

33-week gestational age fetus with complete transposition of the great vessels. Aorta (Ao) arising from the right

ventricle (RV), in a heart with concordant atrioventricular connections.

Page 33: Studio del cuore con rm fetale Manganaro

Double outlet right ventricle:

both great arteries arise mainly from the right ventricle. Signs of the

pathology are the disproportion of ventricles, the arteries position and

origins. Aorta and pulmonary artery show a parallel orientation

5.Abnormalities of great artery position and

connection 2

Both great arteries Ao (*) and PA (arrow) arise from right ventricle, and show parallel orentation

Page 34: Studio del cuore con rm fetale Manganaro

Fallot tetralogy:

aorta exiting the heart overriding ventricles, myocardial hyperthrophy,

interventricular septal defects caused by wrong alignment

5.Abnormalities of great artery position and

connection 3

38-week gestational age fetus with Fallot tetralogy. Aorta exiting the heart overriding

ventricles (arrows)

Page 35: Studio del cuore con rm fetale Manganaro

Common arterial trunk:

only one artery arising from the heart, which gives rise to aorta and

pulmonary artery; right position of the aorta, the possible interruption or

agenesis of the aorta, the absence or stenosis of the arterial duct

5.Abnormalities of great artery position and

connection 4

33-week gesttional fetus with common arterial trunk, unique artery (arrows) which gives rises to aorta and pulmonary artery

(arrow-head)

Page 36: Studio del cuore con rm fetale Manganaro

5.Abnormalities of great artery

position and connection 5

Azygos

continuation

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Outflow defects:

obstructive left outflow:

disproportion of the left and right

chambers with the possibility of a

wider right ventricle

obstructive right outflow:

enlargement of the right ventricle,

associated to a right atrial

enlargement and myocardial

thickness.

Inflow defects:

mitral stenosis or valves

deficiency

6. Outflow and Inflow defects

30° week gestation fetus affected by obstructive right outflow (tricuspidal

atresia). Severe reduction of the right ventricle with virtual lumen (arrow) and

right atrium dilatation (*). B) absente visualization of pulmonary outflow

Page 38: Studio del cuore con rm fetale Manganaro

DIV

PULMONARY ATRESIA

DIV 27 Week

AP?

SVC

AO

AO

SVC

AO

Page 39: Studio del cuore con rm fetale Manganaro

PULMONARY ATRESIA

32° WEEK

Page 40: Studio del cuore con rm fetale Manganaro

Septal atrial defects:

secundum atrial septal defect,

difficult to diagnose because of

the physiological persistence of

the foramen ovale. In wide defects

indirect signs such as an

enlargement of the right atrium

can be associated.

Septal ventricular defects:

well studied in the four chamber

views, often associated with other

pathologies such as a Fallot

syndrome.

7.Septal defects 1

32-week gestational age fetus with septal ventricle defect. Lack of

continuity in the lower septal part (arrow)

27-weekgestational fetu swith wide septal artial defect, absence of the atrial

sepum (arrow) in a four chamber view.

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Common atrioventricular

septal defect:

associated with a deficiency in

the central septal , a unique

central valve and a defect of the

ventricular septum which

appear to unevenly divide the

heart (unbalanced ventricles)

More difficult is the diagnosis

of partial atrioventricular septal

defect characterized by only the

atrial defect.

7.Septal defects 2

a) Wide septal atrial and ventricular defect . b) malrotation of the

cardiac axis

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29-weeks fetus - VENTRICULAR SEPTAL DEFECT

(VSD) with possible association of coartaction of the aorta excluded by angio-MR sequences

T1 3D SPOILED

GE angio-MR

sequences to

assess the aorta

(MIP)

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Lungs

Thymus

Other malformations (BRAIN)

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Page 45: Studio del cuore con rm fetale Manganaro

Studies with heterogenus and small population

Absence of standardized measurement and protocols

Technical and Anatomical Limitations :

Severe heart malrotation

Small heart size

Low evaluation of motion fluid

Absence of real time resolution due to:

- Fast fetal heart rate

- Low time resolution

- Impossibility of cardiac triggering

LIMITS OF CARDIOVASCULAR MRI

Inability to study : - valvular disease (indirect signs)

- rhythm disorders

Page 46: Studio del cuore con rm fetale Manganaro
Page 47: Studio del cuore con rm fetale Manganaro

FETAL MRI ALLOWS:

Anatomical characterization of the fetal heart:

TrueFISP sequences

Suboptimal dynamic evaluation of cardiac function:

Real-time Cine-RM sequences

According to the preliminary experiences, other studies with

significantly large population may offer a standardization of the MRI

examination.

CONCLUSIONS 1

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Nowadays few studies indicate that indications to fetal MRI

include inconclusive US examination* or coexistence of

extracardial associated pathologies

However in literature it is demonstrated that MRI may be used

as an adjunctive imaging technique associated to

echocardiography in order to confirm and better define the

diagnosis

CONCLUSIONS 2

* Saleem SN. Feasibility of MRI of the Fetal Heart with Balanced Steady-State Free Precession Sequence Along Fetal Body and

Cardiac Planes. AJR 2008; 191: 1208-1215

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FETAL MRI

The development of new dynamic sequences may contribute to

expand the role of fetal MRI; moreover the development of

angiography-sequences may be helpful to visualize fetal

vascular in 2D or 3D * making it an indispensable tool in the

study of the fetal heart, in order to obtain a precise prenatal

diagnosis and a better delivery management.

*Baker E.. What’s new in magnetic resonance imaging? CardiolYoung 2001.11: 445–452

CONCLUSIONS 3

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- Dott.ssa V. Vinci

- Dott.ssa A. Tomei

- Dott.ssa F. Fierro

- Dott. P. Sollazzo

- Dott.ssa D. Irimia

- Dott. ssa E. Sergi

--Dott.ssa S. Bernardo

Lucia Manganaro

Department of Radiological Sciences

Policlinico Umberto I Hospital, “Sapienza” University of Rome

[email protected]