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DIAGNOSI E TERAPIA DELLE
BRADIARITMIE FETALI
Aggiornamenti Di Ecocardiografia Fetale II Edizione 19 Aprile 2015
Dipartimen...
FETAL ARRHYTHMIAS
INCIDENCE 1-3%
CLINICAL IMPACT: POTENTIONAL CAUSE FOR HYDROPS,
HEART FAILURE, IU DEATH.
TACHYCARDIA (...
FETAL BRADYCARDIAS
BLOCKED ATRIAL BIGEMINY (secondary, most common)
SINUS BRADYCARDIA
 FETAL DISTRESS
 CHD
 LONG QT S...
FETAL BRADYCARDIAS
BLOCKED ATRIAL BIGEMINY
SINUS BRADYCARDIA
 FETAL DISTRESS (maternal therapy, maternal hypotension, r...
FETAL BRADYCARDIAS
BLOCKED ATRIAL BIGEMINY
SINUS BRADYCARDIA
 FETAL DISTRESS
 CHD
 LONG QT SYNDROME
ATRIOVENTRICULAR...
FETAL PRESENTATION OF
LONG QT SYNDROME
BRADYCARDIA
VT
II DEGREE AV BLOCK
Ishikawa et al. Fetal Diagn Ther 2013
FETAL PRESENTATION OF
LONG QT SYNDROME
Ishikawa et al. Fetal Diagn Ther 2013
21 FETUSES
TIME OF PRESENTATION 16-38 WEEKS...
FETAL PRESENTATION OF
LONG QT SYNDROME
Ishikawa et al. Fetal Diagn Ther 2013
AT LEAST 20-30% OF PATIENTS WITH LQTS
EXHIBIT...
FETAL PRESENTATION OF
LONG QT SYNDROME
Ishikawa et al. Fetal Diagn Ther 2013
PROPORTION OF FETUSES WITH LQTS AMONG FETUSES...
NEONATAL PRESENTATION OF
LONG QT SYNDROME
AVB 3:1
FV 75/min
WIDE QRS COMPLEX
QT 520 msec
QTc 604 msec
FETAL BRADYCARDIAS
BLOCKED ATRIAL BIGEMINY
SINUS BRADYCARDIA
 FETAL DISTRESS
 LONG QT SYNDROME
 CHD
ATRIOVENTRICULAR...
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-AVB
MANAGEMENT
Lopes et al. Circulation 2008
116 C-AVB 57 ISOLATED 59 ASSOCIATED
WITH CHD
FETAL DEATH 10% 40%
NEONATAL D...
C-AVB
PROGNOSIS
Jaeggi et al. Ultrasound Obstet
Gynecol 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
...
OTHER AB MEDIATED CARDIAC
MANIFESTATIONS
CARDIOMYOPATHY
ENDOCARDIAL FIBROELASTOSIS
SINUS NODE DISEASE
QT PROLONGATION
CONG...
AB POSITIVE C-AVB
PROGNOSIS
META ANALYSIS OF REPORTED SERIES
TOTAL 234 FETUSES
TOP/IUD 13%
NEONATAL DEATH 8%
SURVIVAL AFTE...
AB POSITIVE C-AVB THERAPY
BETAMIMETICS: TO INCREAS HR
DEXAMETHASON (CONTROVERSIAL USE): WHEN?
 NEVER (IRREVERSIBLE C-AV...
AB POSITIVE C-AVB THERAPY
Jaeggi et al. Circulation 2004
1990-2003
37 FETUSES (92% AB+)
MEAN AGE AT DIAGNOSIS
25+/-5 GE...
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 diff...
C- AVB: INDICATIONS TO PM
IMPLANTATION
C- AVB WITH CHD AND HEART FAILURE (HYDROPE AND
HR<60)
C-AVB AND HR< 55 BPM +/- PA...
FUTURE ?
THANKS
Silvia Placidi
UOC di Aritmologia Pediatrica e Sincope Unit
Ospedale Pediatrico Bambino Gesù Palidoro
AB POSITIVE C-AVB THERAPY
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21 s placid diagnosi e terapia delle bradiaritmie fetali

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21 s placid diagnosi e terapia delle bradiaritmie fetali

  1. 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. 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. 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. 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. 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. 6. FETAL PRESENTATION OF LONG QT SYNDROME BRADYCARDIA VT II DEGREE AV BLOCK Ishikawa et al. Fetal Diagn Ther 2013
  7. 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. 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. 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. 10. NEONATAL PRESENTATION OF LONG QT SYNDROME AVB 3:1 FV 75/min WIDE QRS COMPLEX QT 520 msec QTc 604 msec
  11. 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. 12. C-AVB DIAGNOSIS Nil et al. Heart 2006
  13. 13. C-AVB DIAGNOSIS Carvalho. Ultrasound Obstet Gynecol 2014. Heart 2006
  14. 14. C-AVB MANAGEMENT ASSOCIATED WITH CHD ISOLATED C-AVB ANTIBODY MEDIATED HEMODYNAMIC SIGNIFICANCE
  15. 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. 16. C-AVB PROGNOSIS Jaeggi et al. Ultrasound Obstet Gynecol 2005 Lopes et al. Circulation 2008
  17. 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. 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. 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. 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. 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. 22. AB POSITIVE C-AVB THERAPY Jaeggi et al. Circulation 2004
  23. 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. 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. 25. FUTURE ?
  26. 26. THANKS Silvia Placidi UOC di Aritmologia Pediatrica e Sincope Unit Ospedale Pediatrico Bambino Gesù Palidoro
  27. 27. AB POSITIVE C-AVB THERAPY

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