RM

UTILITA’ e LIMITI

Lucia Manganaro

Dipartimento di Scienze Radiologiche

Oncologiche e antamopatologiche

AGGI ORNAMENTI DI

ECOCARDI OGRAFI A

FETALE

I I a edizione

I NFORMAZI ONI GENERALI

COMI TATO SCI ENTI FI CO

Prof.ssa Flavia Ventriglia: U.O.C. di Cardiologia Pediatrica - Azienda Policlinico Umberto I Roma- Sapienza Università di Roma; email: flavia.ventriglia@uniroma1.it Prof. Bruno Marino: U.O.C. di Cardiologia Pediatrica Azienda Policlinico Umberto I Roma- Sapienza Università di Roma; email: bruno.marino@uniroma1.it ECM Il corso è in fase di accreditamento per 15 ECM Discipline principali di riferimento: Cardiologia, Ginecologia, Pediatria, Neonatologia, Genetica medica

SEDE DEL CORSO: Aula Magna della Clinica Pediatrica Policlinico Umberto I, Viale Regina Elena n°324— 00161 Roma Il corso è aperto ad un massimo di 70 partecipanti + 30 riservati a studenti, specializzandi, masterizzandi delle aree interessate

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00 Comprende: Materiale congressuale + coffee breaks + work lunches—Attestato di partecipazione, crediti ECM. Gratuito  per  tutti  i  professionisti  dipendenti  dell’Azienda  Sanitaria  Policlinico Umberto I e per gli studenti, specializzandi e masterizzandi delle aree di interesse con documentazione allegata

I PARTECIPANTI POTRANNO PRESENTARE DEI CASI CLINICI DA DISCUTERE IN SEDE CONGRESSUALE IL 19/4 ORE 16

S

Dipart iment o di Pediat ria

Policlinico Umbert o I

Università “Sapienza”

Roma

18/ 19 Aprile 2015

Con la collaborazione:

SEGRETERI A ORGANI ZZATI VA Ufficio Formazione – Azienda Policlinico Umberto I mail: formazione.ecm@policlinicoumberto1.it tel. 06/49974962 Fax 06/49974961 Sito web: policlinicoinformazione.blogspot.it

Con il Pat rocinio

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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

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

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

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

b27-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.

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

3. Cardiac masses

?

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.

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

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

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.

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

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)

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)

5.Abnormalities of great artery

position and connection 5

Azygos

continuation

Double aortic arch

5.Abnormalities of great artery position and

connection 6

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

DIV

PULMONARYATRESIA

DIV27 Week

AP?

SVC

AO

AO

SVC

AO

PULMONARY ATRESIA

32° WEEK

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.

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

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)

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 extracardiacpathologies *

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

ROLE OF FETAL MRI

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 extracardiacpathologies *

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

ROLE OF FETAL MRI

Lungs

Thymus

Other malformations (BRAIN)

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

Cardiac masses

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)

ROLE OF FETAL MRI

Postmortem cardiac imaging in fetuses

3-D cardiac postmortem MRI

MARIAS (magnetic resonance imaging autopsy study) compared the

diagnostic accuracy of 3-D cardiac postmortem MRI with conventional

autopsy and histopathology assessment in fetuses and children

Complete atrioventricular

septal defect (dotted line)

on axial T2-weighted

postmortem MRI in a 22-

week gestation fetus with

trisomy 21. Note air in the

right ventricle (RV)

secondary to fetocide

injection and a trace of

pericardial effusion. LV left

ventricle

Fetal cardiac postmortem

MRI in a 29-week gestation fetus

shows a large cardiac teratoma (T)

following unsuccessful in utero

laser ablation. The large teratoma

displaces the heart posteriorly and

laterally on (a) axial and (b)

oblique coronal T2-weighted

images. LV left ventricle, Rvrig

ventricle

Fetal cardiac postmortem MRI shows

hypoplastic left heart syndrome

on sagittal T2-weighted image in a 22-

week gestation fetus. LV left

ventricle, RV right ventricle.

Three-dimensional cardiac postmortem MRI can provide

equivalent structural information to that of conventional autopsy

in the majority of larger fetuses, newborns and children.

This technique may have a major role in developing lessinvasive

autopsy methods. Moreover, routine use of cardiac

postmortem MRI as an adjuvant to conventional autopsy may

increase the yield from conventional autopsy. Further study of

high-field postmortem MRI, postmortem CT and micro-CT

will continue to optimize the best methods for this form of

less-invasive postmortem assessment.

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

- cardiac triggering

LIMITS OF CARDIOVASCULAR MRI

Inability to study : - valvular disease (indirect signs)

- rhythm disorders

FETAL CARDIAC MRI

FETAL CARDIAC MRI

3D MRI SEQUENCES

Higher SNR

Ability to reformat images in muliple planes

Fast free –breathing in vivo fetal imaging using time-resolved 3D-MRI

technique: preliminary results

Liu J et al . Quant Imaging Med Surg 2014

FETAL CARDIAC MRI

More sensitive to fetal motion

FETAL MRI

Although advances in magnetic resonance technology have expanded the clinical role of MRI

for pediatric patients with CHD, the application of MRI to the fetal heart has been limited

because of the small size of fetal cardiac structures, random fetal motion, and the challenge

of gating the rapidly beating fetal heart in the absence of a fetal electrocardiogram.

Furthermore, in contrast to conventional ultrasound technology, MRI requires expensive,

large, less portable equipment, as well as specialized expertise to perform and interpret.

Nevertheless, MRI offers several advantages over obstetric ultrasound. Fetal position, rib

calcification, maternal obesity, and oligohydramnios, particularly during the third trimester,

interfere more with ultrasound imaging than with MRI. If the challenges relating to motion and

cardiac gating can be overcome, MRI has the potential to provide high-resolution imaging of

the fetal heart in multiple planes and to generate volume data sets with greater resolution

than those obtained with ultrasound, offering the potential to provide robust quantitative

evaluation of cardiac function and chamber volumes and to provide unique perspectives on

venous and arterial anatomy, visceroatrial situs, and thoracic extracardiac malformations

affecting fetal cardiovascular structure/function.

Lucia Manganaro

Department of Radiological Sciences

Policlinico Umberto I Hospital, “Sapienza” University of Rome

lucia.manganaro@uniroma1.it