1. DIAGNOSI E TERAPIA DELLE
BRADIARITMIE FETALI
Aggiornamenti Di Ecocardiografia Fetale II Edizione 19 Aprile 2015
Dipartimento Di Pediatria Policlinico Umberto I Università «Sapienza» Roma
Silvia Placidi
UOC di Aritmologia Pediatrica e Sincope Unit
Ospedale Pediatrico Bambino Gesù Palidoro

2. FETAL ARRHYTHMIAS
INCIDENCE 1-3%
CLINICAL IMPACT: POTENTIONAL CAUSE FOR HYDROPS,
HEART FAILURE, IU DEATH.
TACHYCARDIA (HR>180/MIN)
BRADYCARDIA (HR<100/MIN) (40%)

3. FETAL BRADYCARDIAS
BLOCKED 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

4. FETAL BRADYCARDIAS
BLOCKED 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

5. FETAL BRADYCARDIAS
BLOCKED 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

6. FETAL PRESENTATION OF
LONG QT SYNDROME
BRADYCARDIA
VT
II DEGREE AV BLOCK
Ishikawa et al. Fetal Diagn Ther 2013

7. FETAL PRESENTATION OF
LONG 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%

8. FETAL PRESENTATION OF
LONG 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

9. FETAL PRESENTATION OF
LONG QT SYNDROME
Ishikawa et al. Fetal Diagn Ther 2013
PROPORTION OF FETUSES WITH LQTS AMONG FETUSES WHO
UNDERWENT ECHOCARDIOGRAPHY FOR VARIOUS REASONS

10. NEONATAL PRESENTATION OF
LONG QT SYNDROME
AVB 3:1
FV 75/min
WIDE QRS COMPLEX
QT 520 msec
QTc 604 msec

11. FETAL BRADYCARDIAS
BLOCKED 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

12. C-AVB
DIAGNOSIS
Nil et al. Heart 2006

13. C-AVB
DIAGNOSIS
Carvalho. Ultrasound Obstet Gynecol 2014. Heart 2006

14. C-AVB
MANAGEMENT
ASSOCIATED
WITH CHD
ISOLATED C-AVB
ANTIBODY
MEDIATED
HEMODYNAMIC
SIGNIFICANCE

15. C-AVB
MANAGEMENT
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%

16. C-AVB
PROGNOSIS
Jaeggi et al. Ultrasound Obstet
Gynecol 2005
Lopes et al. Circulation 2008

17. 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

18. 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

19. AB POSITIVE C-AVB
PROGNOSIS
META 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

20. AB POSITIVE C-AVB THERAPY
BETAMIMETICS: 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)

21. 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

22. AB POSITIVE C-AVB THERAPY
Jaeggi et al. Circulation 2004

23. 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 period
independently of treatment
66% PMK before 1 year
8 children developed cardiomiopathy (4,5%) Eliasson et al. Circulation 2011

24. C- AVB: INDICATIONS TO PM
IMPLANTATION
C- 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

25. FUTURE ?

26. THANKS
Silvia Placidi
UOC di Aritmologia Pediatrica e Sincope Unit
Ospedale Pediatrico Bambino Gesù Palidoro

27. AB POSITIVE C-AVB THERAPY