Enrico Chiappa pdf

(1)

Nuove possibilità diagnostiche e terapeutiche delle cardiopatie congenite

Les nouvelles chances diagnostiques et thérapeutiques

des cardiopathies congénitales

PERCORSI DECISIONALI PER LA GESTIONE DEL BAMBINO E DELL'ADOLESCENTE 25 - 30 Luglio 2009, Tabarka Tunisia

Enrico Chiappa

Azienda Ospedaliero – Universitaria MEYER

Firenze

(2)

Azienda Ospedaliero-Universitaria Meyer, Firenze E. Chiappa

Prevalenza

delle Cardiopatie Congenite

Diagnosi Prenatale

Trattamento Radicale Precoce

Terapia Intensiva Cardiologica Terapia

Intensiva Neonatale

Diagnosi Neonatale Precoce Tecniche

Chirurgiche o Transcatetere

(3)

La Diagnosi Prenatale

(4)

Valutazione accurata e precoce del cuore fetale

(5)

Valutazione accurata e precoce del cuore fetale

(6)

The Impact of Prenatal Diagnosis on Cardiology and Cardiac Surgery Today Valutazione accurata e precoce del cuore fetale

(7)

Ecografia Fetale 3D

(8)

Ecografia 3D nella valutazione delle malformazioni fetali

• Faccia

• Colonna

• Estremità

• Apparato GE

• Apparato GU

• Cuore

N° malformazioni Vantaggio Indifferente Svantaggio

103 53 (51%) 46 (45%) 4 (4%)

(9)

Ecocardiografia fetale 3D-4D (STIC)

(10)

Multiplanar Orthogonal

Ecocardiografia fetale 3D-4D (STIC)

(11)

Multiplanar Tomography

Ecocardiografia fetale 3D-4D (STIC)

(12)

Multiplanar tomography

Ecocardiografia fetale 3D-4D (STIC)

(13)

Surface-Mode Rendering

Ecocardiografia fetale 3D-4D (STIC)

(14)

Inversion-Mode Rendering

Ecocardiografia fetale 3D-4D (STIC)

(15)

F Vinalis, L Mandujano°, G. Vargas and A giuliano

*Concepción, Punta Arenas°, La Serena°°, Cile Ultrasound Obstet Gynecol 2005; 25:25-31

Prenatal diagnosis of congenital heart disease using 4D STIC telemedicine via Internet link:

a pilot study

(16)

F Vinalis, 2005

Prenatal diagnosis of congenital heart disease using 4D STIC telemedicine

(17)

Lo Screening Prenatale delle Cardiopatie

La Diagnosi Prenatale

(18)

• Quali gravidanze?

• Quando?

• Come?

• Quali operatori?

Lo Screening Prenatale delle Cardiopatie

(19)

• Positive family history 1-4%

• Arrhythmias 5%

• Maternal diseases 14%

• Extracardiac malformations 25-45%

• Chromosomal anomalies 15-90%

• Abnomal heart appearance 35-40%

Risk Factors and Detection Rate of CHD

L. Fermont et al, 1991

(20)

Simplified examination

The four-chamber view

(21)

Simplified examination

The four-chamber view

(22)

Extended examination The ventricular outflows

PA RV

LV

AO

(23)

Extended examination The 5 basic transverse views

1 2

3 4

5 A

P

L

R

(24)

Factors affecting detection rate of CHD by fetal echo

Highest Lowest

Population high risk low risk

Ultrasound system high resolution, cardiac dedicated

different resolution level, general obstetric

Examiners

(number, skill)

few,

experienced

many,

differently experienced Type of cardiac

assessment

4-chamber + outflows,

complete 4-chamber

Examination ( time, n° sessions)

unlimited, multiple

limited,

at specified time

(25)

Position and dimensions of the heart

Morphology and dimensions of chambers The venoatrial connection

The atrial septum and foramen ovale The atrioventricular connection

The atrioventricular valves The ventricular septum

The thickness of ventricular walls The ventricular function

The cardiac rhythm

The four-chamber view checklist

(26)

• 91 ecografisti ostetrici

• 29 Centri di diagnosi prenatale

• 2 corsi teorici (16 ore ciascuno)

• Una giornata di esercitazioni pratiche per ogni centro (115 ore totali di insegnamento)

Improved Prenatal Detection of CHD

Through a Training Program in the Piedmont Region (2003)

E Chiappa e coll

(27)

46

80 0 20 40 60 80 100

2000-02 2003-04

Mean number of CHD/year prenatally detected

+73%

E. Chiappa e coll. 2004

Improved Prenatal Detection of CHD Through a Training Program in the Piedmont Region

(28)

18%

44%

0 10 20 30 40 50

2000-02 2003-04

% Hospital admissions* of CHD with prenatal diagnosis

E. Chiappa e coll. 2004

* < 1 year of age

Improved Prenatal Detection of CHD Through a Training Program in the Piedmont Region

(29)

Impatto sull’incidenza delle CC alla nascita

La Diagnosi Prenatale

(30)

4 799

Affected Pregnancies

557 (50%) Continuation

53 (6%)

Intrauterine Death

4 165 (85%) Born Alive 1 124 (23.4%)

Prenatal Diagnosis

567 (50%) Termination

C. Bull & BPCA, The Lancet 1999

Impact of fetal diagnosis on spectrum of CHD in UK

(17 pediatric centres UK 1993-95)

(31)

Fetal diagnosis

Undiagnosed Total CHD

% of Total Total Born

Alve

In Utero

Death TOP Prenatal

Diagnosis TOP

HLH 228 57 8 163 177 348 66 47

AVSD 218 91 13 114 452 579 38 20

UVH 145 45 3 97 109 209 69 46

PAIVS 59 19 4 36 68 108 55 33

TAPVD 2 2 0 0 25 25 8 0

TOF

Truncus 73 43 8 22 606 636 11 3

TGA 12 10 1 1 391 393 3 0.3

C. Bull & BPCA, The Lancet 1999

Impact of fetal diagnosis on spectrum of CHD in UK

(32)

C. Bull & BPCA, The Lancet 1999 Cases of serious CHD

Diagnosis Rate

%

Born alive with prenatal

diagnosis Liveborn Total Prenatal

Diagnosis England and

Wales 3684 4304 1089 25.3 12.7

Scotland 344 357 34 9.6 6.1

Northern

Ireland 137 138 1 0.7 0

Total 4165 4799 1124 23.4 11.7

Impact of fetal diagnosis on spectrum of CHD in UK

(33)

Potential effect of national diagnostic rate approximating that of 15 areas with higher detection rate

C. Bull & BPCA, The Lancet 1999

National

Fetal Diagnostic Rate

National

Decrease of Interventions

54 % 218 (204 - 275)

40% 138 ( 127 -183)

30% 58 (50 -92)

* Presumed termination rate of 50%

(34)

…about half of the complex forms of CHD

which the pediatric cardiologist and surgeons would expect to see and treat postnatally may only be seen once in prenatal life.

L.Allan, Heart 2000; 83: 367-370

(35)

Impatto sulla sopravvivenza delle CC

La Diagnosi Prenatale

(36)

Specific CHD in fetal and neonatal series

0 2 4 6 8 10 12 14 16 18

%

VSD TGA TOF Coarc HLHS PAIS

PS

AVSD MA

TA

AS TVD

Eb

fetal

neonatal

Allan, Hornberg, Sharland 2000

(37)

CHD commonly overlooked in prenatal diagnosis

• Ventricular septal defect

• Atrial septal defect

• Patent ductus arteriosus

• Coarctation of the aorta

• Mild-modertate PS or AS

(38)

Detection of TGA in Fetuses Reduces Neonatal Morbidity and Mortality

D Bonnet, A Coltri, G Butera, L Fermont, J Le Bidois, J Kachaner, D Sidi

Circulation 1999; 99; 916-918

(39)

The Impact of Prenatal Diagnosis on Cardiology and Cardiac Surgery Today

Postnatal Prenatal P

N° cases 250 68

age at admission (h) 3  210 2.2  2.8 < 0.01

mech. ventilation 38% 17.6% < 0.01

metabolic acidosis 56% 8% < 0.05

preop. mortality 6% 0% < 0.05

postop. mortality 8.5% 0% < 0.01

hospital stay(d) 30  17 24  11 < 0.01

Bonnet et al, Circulation 1999; 99; 916-918

Detection of TGA in Fetuses Reduces Neonatal Morbidity and Mortality

(40)

Centri di Riferimento e Bacini d’Utenza

La Diagnosi Prenatale

(41)

Substandard Centre

High-standard Centre

A

B

(42)

• Reduced cost of postnatal transport

• Competition among centers

• Free access to data of each centre, reviewed by an indipendent control agency

• Centre with global care to mother, fetus, and child

Prenatal diagnosis and in utero transportation of CHD

(43)

Aspetti Psicologici e Medico-legali

La Diagnosi Prenatale

(44)

• Emotionally better-prepared

• Seek for second opinions

• More time to investigate all options

• Delivery planning at the best

• Reduced risk of litigation

Psychological and legal aspects in prenatal diagnosis

(45)

Aspetti Economici

(46)

Prenatal Diagnosis

Only Postnatal

Diagnosis p

Length of stay

(days) 25.5 ± 4.7 6.5 ± 11.7 < 0.006 Cost

(US $) 81 639 ± 16 935 33 726 ± 7 645 < 0.002 Survival

(%) 64 44 0.22

JA Copel et al, Ultrasound Obstet Gynecol 1997

Outcome in infants requiring univentricular repair

(47)

Prenatal Diagnosis

Only Postnatal

Diagnosis p

Length of stay

(days) 10.0 ± 6.0 13.0 ± 2.4 0.58

Cost

(US $) 30 277 ± 16 869 64 616 ± 9 441 < 0.07 Survival

(%) 96 76 0.05

JA Copel et al, Ultrasound Obstet Gynecol 1997

Outcome in infants amenable to biventricular repair

(48)

Progressi in Ecocardiografia

(49)

• Acquisizione

• Compressione “intelligente”

• Archiviazione, esportazione

• Rielaborazione e valutazione

• Teleconsulto, didattica

L’ecocardiografia digitale

(50)

La miniaturizzazione delle attrezzature

(51)

Ingressi

Computer

Connessioni

• Banda larga > 384 Kbps

• ISDN (1=128 Kbps)

• ADSL

• Connessione satellitare

• Ecocardiografo

• Telecamera

• Microfono

• Scanner

Teleconsulto ecocardiogafico “Real-time”

(52)

Nuove Tecniche Diagnostiche

Delle Cardiopatie Congenite

(53)

• Definizione anatomica e funzionale delle CC

• Calcolo della portata

• Caratterizzazione tissutale

• Vitalità miocardica

RMN cardiaca nelle Cardiopatie Congenite

Applicazioni

(54)

RMN Cardiaca

(55)

AP sin AP dx

RMN Cardiaca

(56)

Multislice CT

(57)

Multislice CT

(58)

Multislice CT

(59)

Multislice CT

Doppio Arco Aortico

(60)

Multislice CT

Coartazione Aortica

(61)

Fistola AV Polmonare

Multislice CT

(62)

Atresia Polmonare con DIV e Collaterali Aortico-Polmonari

Multislice CT

(63)

Multislice CT

Atresia Polmonare con DIV, Shunt BT Dx e Sin

(64)

Multislice CT

Origine Anomala della Coronaria Sin dall’Arteria Polmonare

(65)

Il Cateterismo Cardiaco Interventistico

(66)

Il Laboratorio di Emodinamica

(67)

Il Cateterismo Cardiaco Interventistico

(68)

CardioSEAL STARFlex

Atrial Septal Defect Occluders

(69)

Das Angel Wings ELEX

Atrial Septal Defect Occluders

(70)

AMPLATZER Occluder

Atrial Septal Defect Occluders

Amplatz

Mullins

(71)

Chiusura DIA con Amplatzer Device

G.F. 6 aa

(72)

G.F. 6 aa

Chiusura DIA con Amplatzer Device

(73)

BioSTAR

Jux et al, ASD Closure Using a Totally Resorbable Matrix, 2007

STARFlex

Bioresorbable Atrial Septal Defect Occluders

(74)

BioSTAR

Bioresorbable Atrial Septal Defect Occluders

Jux et al, ASD Closure Using a Totally Resorbable Matrix, 2007

(75)

Bioresorbable Atrial Septal Defect Occluders

(76)

BioSTAR STARFlex

30 gg

90 gg

180 gg

(77)

Amplatzer Muscular VSD Occluder

(78)

Amplatzer Muscular VSD occluder

(79)

Amplatzer Membranous VSD occluder

(80)

D.C. 20 mesi

Angioplastica con Catetere a Palloncino

(81)

D.C. 20 mesi

Angioplastica con Catetere a Palloncino

(82)

D.C. 20 mesi

Angioplastica con Catetere a Palloncino

(83)

Cutting Ballon

(84)

Cutting Ballon

(85)

Stents

(86)

Agioplastica Aortica con Impianto di Stent

Prima Dopo

(87)

Impianto Transcutaneo di Valvole

(88)

Impianto Transcatetere di Valvola Polmonare

MELODY Valve

(89)

Impianto Transcatetere di Valvola Polmonare

MELODY Valve

(90)

Impianto Transcatetere di Valvola Aortica

(91)

Procedure Ibride

(92)

Trattamento Ibrido del Cuore Sinistro Ipoplasico

(93)

ECMO

(94)

ECMO

Basic ECMO

Circuits

(95)

Progressi nella Diagnosi e Trattamento

delle Aritmie

(96)

Monitoraggio Holter

(97)

Loop Recorder esterno

(98)

Reveal, Medtronic, Minneapolis, USA

Loop Recorder Impiantabile

(99)

Defibrillatore Impiantabile (ICD)

(100)

Implantable Cardioverter Defibrillator

(101)

Multicenter Pediatric ICD Registry

Anatomic Diagnoses of Pediatric and Congenital ICD Recipients

(102)

Multicenter Pediatric ICD Registry

Diagnoses of ICD Recipients With Structurally Normal Hearts

(103)

QT Lungo S. di Brugada

(104)

Lo Studio Elettrofisiologico

(105)

Lo Studio Elettrofisiologico

Sistema Carto

(106)

Sistema EnSite

Lo Studio Elettrofisiologico

(107)

Sistema EnSite

Lo Studio Elettrofisiologico

(108)

L’Ablazione Transcatetere

(109)

La Crioablazione Transcatetere

(110)

Enrico Chiappa pdf

Nuove possibilità diagnostiche e terapeutiche delle cardiopatie congenite

Les nouvelles chances diagnostiques et thérapeutiques

des cardiopathies congénitales

PERCORSI DECISIONALI PER LA GESTIONE DEL BAMBINO E DELL'ADOLESCENTE 25 - 30 Luglio 2009, Tabarka Tunisia

Enrico Chiappa

Azienda Ospedaliero – Universitaria MEYER

Firenze

Prevalenza

delle Cardiopatie Congenite

Diagnosi Prenatale

Trattamento Radicale Precoce

Terapia Intensiva Cardiologica Terapia

Intensiva Neonatale

Diagnosi Neonatale Precoce Tecniche

Chirurgiche o Transcatetere

La Diagnosi Prenatale

Valutazione accurata e precoce del cuore fetale

Valutazione accurata e precoce del cuore fetale

The Impact of Prenatal Diagnosis on Cardiology and Cardiac Surgery Today Valutazione accurata e precoce del cuore fetale

Ecografia Fetale 3D

Ecografia 3D nella valutazione delle malformazioni fetali

• Faccia

• Colonna

• Estremità

• Apparato GE

• Apparato GU

• Cuore

N° malformazioni Vantaggio Indifferente Svantaggio

103 53 (51%) 46 (45%) 4 (4%)

Ecocardiografia fetale 3D-4D (STIC)

Multiplanar Orthogonal

Ecocardiografia fetale 3D-4D (STIC)

Multiplanar Tomography

Ecocardiografia fetale 3D-4D (STIC)

Multiplanar tomography

Ecocardiografia fetale 3D-4D (STIC)

Surface-Mode Rendering

Ecocardiografia fetale 3D-4D (STIC)

Inversion-Mode Rendering

Ecocardiografia fetale 3D-4D (STIC)

F Vinalis*, L Mandujano°, G. Vargas and A giuliano*

*Concepción, Punta Arenas°, La Serena°°, Cile Ultrasound Obstet Gynecol 2005; 25:25-31

Prenatal diagnosis of congenital heart disease using 4D STIC telemedicine via Internet link:

a pilot study

F Vinalis, 2005

Prenatal diagnosis of congenital heart disease using 4D STIC telemedicine

Lo Screening Prenatale delle Cardiopatie

La Diagnosi Prenatale

• Quali gravidanze?

• Quando?

• Come?

• Quali operatori?

Lo Screening Prenatale delle Cardiopatie

• Positive family history 1-4%

• Arrhythmias 5%

• Maternal diseases 14%

• Extracardiac malformations 25-45%

• Chromosomal anomalies 15-90%

• Abnomal heart appearance 35-40%

Risk Factors and Detection Rate of CHD

L. Fermont et al, 1991

Simplified examination

The four-chamber view

Simplified examination

The four-chamber view

Extended examination The ventricular outflows

PA RV

LV

AO

Extended examination The 5 basic transverse views

1 2

3 4

5

A

P

L

R

Factors affecting detection rate of CHD by fetal echo

Highest Lowest

F Vinalis, L Mandujano°, G. Vargas and A giuliano