Diagnosis and Management of Major Fetal Arrhythmias

Transcription

1 Diagnosis and Management of Major Fetal Arrhythmias Edgar T Jaeggi, MD, FRCPC Head, Fetal Cardiac Program Associate Professor (Pediatrics) The Hospital for Sick Children University of Toronto Toronto, Canada

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3 Wall Motion: M-mode

4 Flow: SVC/Aorta and MV/Aorta Doppler A E A Ao SVC

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6 Normal Rhythm and Conduction

7 A A A A A A V V V V V V

8 AV VA

9 Definition of Fetal Arrhythmia Normal fetal rhythm: regular, bpm Arrhythmia: slower, faster and/or irregular Incidence: > 1% of fetuses > 90%: Premature atrial contractions Transient sinus bradycardia Transient sinus tachycardia < 10%: Major arrhythmia 43%: persistently slow HR 57%: persistently fast HR

10 Cardiac Output and Heart Rate RCO Flow Reversal (%) in IVC Rudolph AM. Am J Obstet Gynecol 1976;124 Reed et al. Circulation 1990;81:

11 Cardiac Causes of Fetal Hydrops Meta-analysis of 47 major, non-selected series (N=804 Cases) 8% 3% 3% 13% 41% Cardiac Malformation Arrhythmias High-output Failure Cardiomyopathy Cardiac Tumor Other 32% Machin GA. Am J Med Genet 1989;34:

12 FETAL BRADYARRHYTHMIAS

13 Mechanisms of Sustained Fetal Bradycardia 12% 2% Complete AV block 18% 2:1 AV block Atrial/junctional bigeminy 60% Sinus bradycardia 8% Atrial standstill : N = 51

14 SINUS BRADYCARDIA A V V V V A A A A A Regular slow (70-90/min) A and V rate + normal AV relation

15 NON-CONDUCTED ATRIAL BIGEMINY A A2 A V V V V V A A A A A A A2 V A2 Regular irregular A rhythm + regular slow (60-90/min) V rate

16 COMPLETE ATRIO-VENTRICULAR BLOCK A A A A A Normal A rate + slow (35-80/min) V rate + AV dissociation

17 Case 1: 27 year old healthy; G5, A3, P1 Regular slow heart rate at 25 weeks

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19 Intermittent irregular heart rate

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23 What is happening?

24 A A A A A A A A V V V V

25 A A A A A A A A V V PVC V V

26 A A A A A A A A V V PVC V V

27 What is your diagnosis? Regular atrial rhythm with AVdissociation and structurally normal heart = Isolated Complete AV block

28 What is your diagnosis? Complete AV block Premature ventricular beats

29 What is your diagnosis? Complete AV block Premature ventricular beats Intermittent irregular fast heart rate = VT or JET

30 What is your diagnosis? Complete AV block Premature ventricular beats Intermittent VT or JET Pericardial effusion

31 What is your diagnosis? Complete AV block Premature ventricular beats Intermittent VT or JET Pericardial effusion Maternal antibody-test (ELISA): anti-ro >100 U/ml anti-la > 100 U/ml

32 CAVB AV-block without CHD (60%): High level of maternal anti-ro antibodies (> 90%) in 1-2% of exposed fetuses: 1) damage of AV node heart block; 2) carditis function; effusion; ventricular arrhythmia and/or 3) tissue damage endocardial fibrolelastosis; dilated CMP AV-block with CHD (40%): Anatomical disruption of the electrical conduction system Left atrial isomerism (LAI) Congenitally corrected transposition (cc-tga)

33 Risk Factors of Adverse Outcomes Mortality Untreated isolated CAVB 25% 43% CAVB with CHD 82% 86% RF of poor outcome Fetal hydrops 87% 100% Endocardial fibroelastosis 100% Delivery < 32 weeks gestation 67% Ventricular rate < 55 bpm (ICAVB) 50% 86% Ventricular rate < 65 bpm (CAVB with CHD) 100% Rapid drop in fetal heart rate > 5 bpm 55%

34 Fetal Treatment Options To increase the fetal heart rate and contractility β-sympathomimetics (salbutamol; terbutaline) delivery with immediate pacing To mitigate the inflammatory fetal cardiac insult fluorinated steroids (dexamethasone; betamethasone) immune globulin

35 Treatment Protocol of Antibody-related AV block Modified from JAEGGI et al. Circulation 2004

36 Impact of Transplacental Treatment on Survival Era Treatment Survival (%) n = 19 p < 0.01 n = Survival (%) n = 18 n = 21 n = 13 p < 0.02 Tx Protocol Steroid No Steroid follow-up (years) follow-up (years) JAEGGI et al. Circulation 2004

37 2 weeks on dexamethasone

38 Summary: Slow Heart Rate Persistent bradycardia: CAVB + CHD > atrial bigeminy > sinus bradycardia Atrial bigeminy: Benign, no treatment required Isolated immune-mediated CAVB: Dexamethasone + β-stimulation at HR <50-55 bpm + elective delivery in tertiary care center improved 1-year survival to > 90%

39 FETAL TACHYCARDIA

40 Causes of Major Fetal Arrhythmia at SickKids 5% 38% 40% SVT Atrial flutter Sinus tachycardia Complete AV block Sinus bradycardia 6% 11%

41 SHORT VA TACHYCARDIA V APW A Orthodromic AV reentry V A V 240 beats/min Short VA tachycardia of /min

42 ATRIAL FLUTTER (2:1 AV CONDUCTION) atrial freewall ventricular free wall A A A A A A A A A A V V V V /min A rate /min V rate + mainly 2:1 AV ratio

43 Case 2: 27 weeks, short VA SVT HR bpm

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45 DV flow reversal DV

46 1 st Choice Management of SVT/AF Non-sustained SVT without hydrops Observation; Treatment if frequent runs Sustained SVT without hydrops Sotalol 80 mg bid.; delivery if > 37 weeks Sustained SVT with hydrops Sotalol 160 mg bid. (-tid.) or Flecainide 100 mg tid. (-qid.); Direct UA injection of amiodarone / adenosine Atrial flutter Sotalol; delivery if > 37 weeks

47 Day 15: S 480 mg/d + F 400 mg/d SVT 175 bpm - HR: from /min - Hydrops: - Fetal movements: - Cardiac function:

48 SVT 175 bpm Plan: direct injection next day

49 Day 16: S 480 mg/d + F 400 mg/d - Converted to SR - Fetal movements: normal - Systolic function: +/-normal - Hydrops: resolved after 5 wks SVT 175 bpm +1 day SR 130 bpm

50 Emily at 6 months: no postnatal medication required

51 Summary: Fast Heart Rate HR >180 bpm: short VA SVT > atrial flutter > long VA tachycardia Antiarrhythmic drug treatment for SVT/AF: In the absence of hydrops, low tachycardia-related mortality and morbidity despite low acute success rates (+/- 50%) with Sotalol, Flecainide and Digoxin Reentrant SVT + hydrops: 16% mortality

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53 Isolated CAVB: HSC Experience Freedom (%) from Death Freedom (%) from Pacing P = 0.09 N=12 N=18 years Deaths: 1 IUD, 1 NND, 1 PAHT, 1 PM related 1 PM related N=10 P < DXMT + B-S DXMT only N=18 years

54 Comparison of 1 st line transplacental antiarrhythmic therapies in fetal tachyarrhythmias De Groot E.E.C.(1), Blom N.A.(1), Clur S.A.(1), Rammeloo L.(1), Jaeggi E.(2) Center of Congenital Heart Disease Amsterdam-Leiden, The Netherlands, (1) The Hospital for Sick Children, Toronto, Canada (2) AEPC 2007

55 Diagnosis at presentation 95 Patients 69 AVRT (73%) 21 AF (22%) 5 AET (5%) 24 hydrops 45 no hydrops 5 cardiac anomalies no hydrops no cardiac anomalies 0 hydrops 2 cardiac anomalies De Groot E, Blom NA, Clur SA, Rammeloo L, Jaeggi E. AEPC 2007

56 1 st choice treatment groups (N=65) Group I: Flecainide +/- Digoxin (F +/- D), 15 pts (300 mg; mg) Group II: Sotalol +/- Digoxin (S +/- D), 31 pts (160 mg; mg) Group III: Digoxin (D), 19 pts (loading: 1mg; maintenance: 0.5 mg ( mg)) Hydrops: F (n=7) and S (n=10) vs. group III (n=1) (p=0.05) In 15 cases (16%) other drugs used after 1 st line failure

57 Acute success rate of transplacental therapy Outcome No Pts Success Partial Success Failure P (SR < 5d) (SVT <10%) F +/- D 15 4 (27%) 5 (33%) 6 (40%) S +/- D (48%) 1 (4%) 14 (48%) D 17 4 (24%) 4 (24%) 9 (53%) NS Total 61* 22 (36%) 10 (16%) 29 (48%) Hydrops versus no hydrops: Tx failure in 59% vs 43 % (NS) AVRT versus AF: Tx failure in 42% vs 60 % (NS) De Groot E, Blom NA, Clur SA, Rammeloo L, Jaeggi E. AEPC 2007

58 Mortality and Morbidity Total mortality: 9/95 (9%): In 4/95: arrhythmia-related (all AVRT + hydrops: 16%) In 5/95: postnatal and related to cardiac tumors (N=3), glutaric acidemia type II (N=1), severe asphyxia (N=1) Neurological complications 2/95 (2%): intraventricular hemorrhage with good outcome De Groot E, Blom NA, Clur SA, Rammeloo L, Jaeggi E. AEPC 2007