DIAGNOSI E TERAPIA DELLE BRADIARITMIE FETALI

A g g ior na m ent i D i Ecoca rd iograf ia Feta le I I Ed i z ione 19 A pr i le 2015

Dipa r t im ento D i Ped iat r ia Po l i c l in i co Um ber to I Un ivers i tà « Sa p ienza » Rom a

Silvia Placidi

UOC di Aritmologia Pediatrica e Sincope Unit

Ospedale Pediatrico Bambino Gesù Palidoro

FETAL ARRHYTHMIASINCIDENCE 1-3%

CLINICAL IMPACT: POTENTIONAL CAUSE FOR HYDROPS, HEART FAILURE, IU DEATH.

TACHYCARDIA (HR>180/MIN)

BRADYCARDIA (HR<100/MIN) (40%)

FETAL BRADYCARDIASBLOCKED ATRIAL BIGEMINY (secondary, most common)

SINUS BRADYCARDIA FETAL DISTRESS

CHD

LONG QT SYNDROME

ATRIOVENTRICULAR BLOCK (70%) ASSOCIATED WITH CHD (40-50%)

ISOLATED (70-90% IMMUNO-MEDIATED)

FUNCTIONAL 2:1 AVB IN LQTS

FETAL BRADYCARDIASBLOCKED ATRIAL BIGEMINY

SINUS BRADYCARDIA FETAL DISTRESS (maternal therapy, maternal hypotension, reflex

bradycardia due to compression)

CHD (left atrial isomerism)

LONG QT SYNDROME

ATRIOVENTRICULAR BLOCK (70%) ASSOCIATED WITH CHD (40-50%)

ISOLATED (70-90% IMMUNO-MEDIATED)

FUNCTIONAL 2:1 AVB IN LQTS

FETAL BRADYCARDIASBLOCKED ATRIAL BIGEMINY

SINUS BRADYCARDIA FETAL DISTRESS

CHD

LONG QT SYNDROME

ATRIOVENTRICULAR BLOCK (70%) ASSOCIATED WITH CHD (40-50%)

ISOLATED (70-90% IMMUNO-MEDIATED)

FUNCTIONAL 2:1 AVB IN LQTS

FETAL PRESENTATION OFLONG QT SYNDROMEBRADYCARDIA

VT

II DEGREE AV BLOCK

Ishikawa et al. Fetal Diagn Ther 2013

FETAL PRESENTATION OFLONG QT SYNDROME

Ishikawa et al. Fetal Diagn Ther 2013

21 FETUSES

TIME OF PRESENTATION 16-38 WEEKS OF GESTATION

IN UTERO CLINICAL SIGNS OF LQTS 76% BRADYCARDIA (19% MILD

BRADYCARDIA: 100-110 BPM)

19% VT

1 CASE PLEURAL EFFUSION

AVB CONFIRMED PRE OR POST NATALLY IN 52%

FETAL PRESENTATION OFLONG QT SYNDROME

Ishikawa et al. Fetal Diagn Ther 2013

AT LEAST 20-30% OF PATIENTS WITH LQTS

EXHIBIT INITIAL SIGNS SUGGESTIVE OF CARDIAC DISEASE IN UTERO

FETAL PRESENTATION OFLONG QT SYNDROME

Ishikawa et al. Fetal Diagn Ther 2013

PROPORTION OF FETUSES WITH LQTS AMONG FETUSES WHO UNDERWENT ECHOCARDIOGRAPHY FOR VARIOUS REASONS

NEONATAL PRESENTATION OFLONG QT SYNDROME

AVB 3:1FV 75/minWIDE QRS COMPLEXQT 520 msecQTc 604 msec

FETAL BRADYCARDIASBLOCKED ATRIAL BIGEMINY

SINUS BRADYCARDIA FETAL DISTRESS

LONG QT SYNDROME

CHD

ATRIOVENTRICULAR BLOCK (70%) ASSOCIATED WITH CHD (40-50%)

ISOLATED (70-90% IMMUNO-MEDIATED)

FUNCTIONAL 2:1 AVB IN LQTS

C-AVB DIAGNOSIS

Nil et al. Heart 2006

C-AVB DIAGNOSIS

Carvalho. Ultrasound Obstet Gynecol 2014. Heart 2006

C-AVB MANAGEMENT

ASSOCIATED WITH CHD

ISOLATED C-AVB ANTIBODY MEDIATED

HEMODYNAMIC SIGNIFICANCE

C-AVBMANAGEMENT

Lopes et al. Circulation 2008

116 C-AVB 57 ISOLATED 59 ASSOCIATED WITH CHD

FETAL DEATH 10% 40%

NEONATAL DEATH 14% 57%

SURVIVORS 77% 26%

PM IMPLANTATION 70% 63%

C-AVBPROGNOSIS

Jaeggi et al. Ultrasound ObstetGynecol 2005

Lopes et al. Circulation 2008

AB POSITIVE C-AVB 2% OF PREGNANCIES WILL DEVELOP ANTIBODIES MEDIATED CCAVB

MATERNAL ANTI RO/SSA AND ANTI LA ANTIBODIES

19% RECURRENCE WHEN A PRIOR FETUS HAS BEEN AFFECTED

RISK OF CARDIAC MANIFESTATION IF ANTI RO ANTIBODIES ARE >50 U/ML (JAEGGI, JACC 2011)

MATERNAL ANTIBODIES INITIATE INFLAMMATION OF THE AV NODE AND THE MYOCARDIUM IN THE SUSCEPTIBLE FETUS

REPLACEMENT WITH FIBROSIS: HEART BLOCK TYPICALLY BETWEEN 20-24 WEEKS

OTHER AB MEDIATED CARDIAC MANIFESTATIONS

CARDIOMYOPATHY

ENDOCARDIAL FIBROELASTOSIS

SINUS NODE DISEASE

QT PROLONGATION

CONGENITAL HEART DEFECTS (ASD, DUCTUS)

Chockalingam et al. J of Rheum 2011

AB POSITIVE C-AVB PROGNOSISMETA ANALYSIS OF REPORTED SERIES

TOTAL 234 FETUSES

TOP/IUD 13%

NEONATAL DEATH 8%

SURVIVAL AFTER MONTH 80%

RISK FACTORS: EFE, POOR VENTRICULAR FUNCTION, HEART RATE<55/MIN, HYDROPS

PM IMPLANTATION 60-70% PTS <1YR

AB POSITIVE C-AVB THERAPYBETAMIMETICS: TO INCREAS HR

DEXAMETHASON (CONTROVERSIAL USE): WHEN? NEVER (IRREVERSIBLE C-AVB, MANY SIDES EFFECTS)

ALWAYS (AS PREVENTION OF CARDIOMYOPATHY)

ONLY WHEN MAJOR RISK FACTORS ARE PRESENT

ONLY FOR 2° DEGREE AVB (PREVENTION C-AVB)

IVIG +/- STEROIDS WITH ENDOCARDIAL FIBROELASTOSIS (TRUCCO ET AL, JACC 2011)

AB POSITIVE C-AVB THERAPY

Jaeggi et al. Circulation 2004

1990-2003

37 FETUSES (92% AB+)

MEAN AGE AT DIAGNOSIS 25+/-5 GESTATIONAL AGE

22 TREATED FETUSES 21DEXA

9 DEXA+ BETA MIMETICS

AB POSITIVE C-AVB THERAPY

Jaeggi et al. Circulation 2004

AB POSITIVE C-AVB THERAPY

175 fetuses with AVB (80% AB+)

38% treated (dexa) for 10 weeks (1-21)

91% born alive

No difference in outcome in steroids vs non steroids group

Risk factors for death: <20 weeks

HR<50 BPM

Hydrops

Poor LV function

> 1 factor 10 fold increased fetal mortality, 6 fold in the neonatal periodindependently of treatment

66% PMK before 1 year

8 children developed cardiomiopathy (4,5%) Eliasson et al. Circulation 2011

C- AVB: INDICATIONS TO PM IMPLANTATIONC- AVB WITH CHD AND HEART FAILURE (HYDROPE AND HR<60)

C-AVB AND HR< 55 BPM +/- PAUSES>3’’

C-AVB AND LQTS

VENTRICULAR DYSFUNCTION AND/OR HEART FAILURE AND/OR GROWTH ARREST

FUTURE ?

THANKSSilvia Placidi

UOC di Aritmologia Pediatrica e Sincope Unit

Ospedale Pediatrico Bambino Gesù Palidoro

AB POSITIVE C-AVB THERAPY