How Does Imaging Inform Fetal Cardiovascular Treatment?

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1 How Does Imaging Inform Fetal Cardiovascular Treatment? Edgar Jaeggi, MD Head, Fetal Cardiac Program Labatt Family Heart Center Department of Pediatrics The Hospital for Sick Children University of Toronto Toronto, Canada

2 Rationale of Prenatal Treatment The rationale for prenatal therapy is to improve survival for conditions with a high risk for perinatal death. If death is not imminent, the outcome should be sufficiently modified to justify the potential risks of the intervention.

3 Pharmacotherapy Prenatal Treatment Tachyarrhythmias Antiarrhythmic therapy Cardiac NLE Steroids, IVIG, β-mimetics Thyrotoxicosis PTU + β-blocker Heart failure: - EA/TVD + circular shunt Indomethacin - Anemia Transfusion - Cardiomyopathy Digoxin Left heart hypoplasia Oxygen Invasive treatment Twin-twin transfusion Critical AS and PAIVS RAS + obstructive lesion Laser Balloon valvuloplasty Atrial septoplasty, septal stenting

4 SUPRAVENTRICULAR TACHYARRHYTHMIAS

5 Machin GA. Am J Med Genet 1989;34: 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%

6 Assessment of Fetal Tachyarrhythmias Structural Heart Disease Hemodynamic Consequences: cardiac function; effusions; AV regurgitation; fetal activity

7 Arrhythmia characteristics Duration: Rhythm: Assessment of Fetal Tachyarrhythmias brief; intermittent; incessant; permanent regular; irregular Mechanism: - V-V Rate how fast - A-A Rate normal (VT); <300 (SVT); >300 (AF) - A-V Ratio 1:1 with normal AV interval 1:1 with abnormal AV interval > 1:1 (A > V) < 1:1 (V > A) AV dissociation

8 AV Reentrant Tachycardia (AVRT) 60% of fetal SVT cases Diagnosis 29 (19-38) weeks Heart rate 250 ( ) bpm Incessant 60% Hydrops 39% Jaeggi E et al. Circulation 2011

9 AV Reentrant Tachycardia (AVRT) V V A A Short VA V A V V A

10 Atrial Flutter (AF) 30% of fetal SVT cases Diagnosis 33 (25-39) weeks A-V relationship mainly 2:1 (1:1-4:1) A rate 440 ( ) bpm V rate 220 ( ) bpm Incessant 84% Hydrops 13% Jaeggi E et al. Circulation 2011

11 Atrial Flutter (AF) A A A A A A A A A V V V V

12 Atrial Ectopic Tachycardia: 9% of fetal cases A Long VA intervals

13 When to treat? Brief, well tolerated runs of SVT or AF Close fetal monitoring, no treatment More persistent or incessant SVT or AF - near term Delivery, postnatal cardioversion Prolonged or persistent SVT or AF - earlier Prenatal therapy

14 P e rc e n t o f F e tu s e s Freedom from prenatal conversion to sinus rhythm SVA Mechanism AF n=36 P = SVT n= d a y s Jaeggi E et al. Circulation 2011

15 Freedom from prenatal conversion to sinus rhythm Arrhythmia Pattern Fetal Hydrops Percent of Fetuses P < n=85 n=26 Intermittent Incessant days Percent of Fetuses P = 0.04 Hydrops Non-Hydrops n= n= days AF and SVT SVT

16 Freedom from prenatal conversion to sinus rhythm Percent of Fetuses SVT n= days 1 st Line Drug Therapy n=26 D vs. S: P = 0.01 F vs. S: P = 0.02 D vs. F: P = 0.41 Digoxin Flecainide Sotalol n=35 Percent of Fetuses MV Analyses AF Digoxin Flecainide Sotalol days S vs. F: P = 0.03 S vs. D: P = 0.05 D vs. F: P = 0.71 n=9 n=10 n=17 Jaeggi E et al. Circulation 2011

17 In-utero Catheter Interventions Structural heart disease Critical aortic stenosis Outflow obstruction with restrictive/intact atrial septum Pulmonary atresia with intact ventricular septum

18 Severe Aortic Stenosis Early: often dilated, hypertrophied LV +/- dysfunction Later: often progression to HLHS

19 Treatment of HLHS: 3 Surgeries or Heart Transplant Norwood Modified Glenn Fontan Neonatal Period 4 6 months 2 4 years

20 Outcome of HLHS Norwood Procedure and Shunt Type Fetal Diagnosis and Type of Surgery N=12 N=38 N=14 Ohye R et al. NEJM 2010 Unpublished SickKids data

21 Advancement of the needle across the womb into the fetal chest

22 Findings associated with Progression to HLHS Retrograde flow in transverse aortic arch Severe LV dysfunction Monophasic short MV inflow L-R shunt across foramen ovale Nii M et al. J Am Coll Cardiol 2004 Makikallio K et al. Circ 2006

23 McElhinney DB et al. Circ 2010;121:

24 HLHS with open atrial septum with restricted atrial septum LA+PV pressure

25 Consequences of Severe Restrictive Atrial Septum Severe hypoxemia and acidosis at birth emergency atrial septostomy (EAS) +/- ECMO Pulmonary vascular anomalies and lymphangiectasia

26 Left Heart Obstruction, Atrial Septum and PV Doppler From Jaeggi et al. Butera, Tulzer, Pedra (eds). Springer 2016;

27 Without prenatal intervention (n=6) IUD = 2 P < n=44 EAS = 4 n=18 n=4 Chaturvedi, Ryan, Seed, van Arsdell, Jaeggi, Int J Cardiol 2013

28 Outcome after Fetal Intervention of CHD 31 Cases 40 Procedures /2017 Technical Success N=3 (3/3; 100%) N=28 (32/37; 87%) 12 IAS 10 CAS 6 PATR Pregnancy Outcome IUD N=3 IUD N=4 LB N=24 Prenatal Survival 0% 86% Death - CAS 19 w (bradycardia) - CAS: 21 w (bradycardia) - PA/IVS: 24 w (bradycardia) - Severe AS + PA/IVS (3 rd procedure) - CAS 21 w LV puncture) - HLHS/IAS/MR: 36 w (perforation) - HLHS 36 w (stent occlusion)

29 Take Home Message Prenatal ultrasound is an essential component in the detection and evaluation of fetal cardiac abnormalities that may benefit from in-utero treatment. Urgent referral to a center with expertise in the prenatal management of such conditions is critical. Prenatal treatment is available to prevent or treat fetal heart failure (arrhythmias), to establish atrial shunting (e.g. HLHS/IAS) or to improve the chances of a biventricular outcome (severe AS/PS).

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