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
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-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%
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
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
THANKSSilvia Placidi
UOC di Aritmologia Pediatrica e Sincope Unit
Ospedale Pediatrico Bambino Gesù Palidoro