Simone R. F. Fontes Pedra, MD Si R F F P d MD Sao Paulo Brazil UNIDADE FETAL
FROM CONCEPTION TO MATURITY: BUILDING A FETAL THERAPY PROGRAM Simone R F Fontes Simone R. F. Fontes Simone R. F. FontesPedra Pedra, MD Pedra, MD MD Carlos A. C. Pedra Carlos A. C. Pedra, MD, MD Hospital do Coração Hospital do Coração São Paulo São Paulo Brazil UNIDADE FETAL This program was partially supported by a grant provided by the Brazilian Ministry of Health
Disclosure Information From Conception to Maturity: Building a Therapy Fetal Program Simone RFF Pedra, Carlos AC Pedra, Simone RFF Pedra, Carlos AC Pedra*, As a faculty member for this program, I disclose the following relationships with industry: (GRS): Grant/Research Support (C): Consultant (SB): Speaker s Bureau (MSH): Major Stock Holder (AB): Advisory Board (E): Employment (O) Oth Fi i l or Material M t i l Support S t (O):Other Financial * AGA Medical Company: C; SB * WL Gore and Associates: C * Atrium: GRS * Bioassist (AGA and Numed rep in Brazil): C * Neomex (Cardia rep in Brazil): C Bi di l (Atrium (At i il) C * Biomedical rep iin B Brazil): * TecMedic (Occlutech rep in Brazil): C
Fetal program x fetal x fetal interventional program interventional program Fetal echocardiography Fetal interventional therapy py Fetal cardiac program F Fetal medical t l di l therapy Delivery & neonatal therapy py UNIDADE FETAL
Fetal program x fetal interventional program Fetal cardiac program program Pediatric Cardiology Center? Obstetric Center General Hospital General Hospital p UNIDADE FETAL
What is ideal? Cardiac diagnostic d ag ost c facilities Fetal cardiac program Cardiac ICU + Fetal medicine program + Interventional i l cardiology di Cardiac surgery P E D I A T R I C
HC Hospital do Coração HCor H it l d C ã Main acti Main activity: Cardiology it Cardiolog Adult Pediatric Other medical fields (Adults): Vascular Surgery, Gastroenterology, Pneumology Gastroenterology, Pneumology, Oncology,, Oncology, O Orthopedics, Urology th di U l No Obstetrics until we started! UNIDADE FETAL
HCor Hospital do Coração Pediatric ICU Pediatric cardiologists 24/7 Pediatrician and neonatologist Si Since November N b 2007! Pediatric Cardiology Private (50%) P i t ( %) Philanthropy Philanthropy projects approved by the Ministry of Health, including fetal cardiology (50%)
H t i How to insert a fetal unit in a pediatric cardiology center? t f t l it i di t i di l t? People Hospital administration support Hospital staff support Obstetric team: Physicians Nurses Neonatologist delivery room Neonatologist delivery room UNIDADE FETAL
H t i How to insert a fetal unit in a pediatric cardiology center? t f t l it i di t i di l t? People Fetal medicine team Diagnosis / Di Diagnosis / kariotyping i / / k k i kariotyping i Surgery/Interventions S Surgery/Interventions /I t ti key person kkey person Multidisciplinary team Multidisciplinary team UNIDADE FETAL
How to insert a fetal unit in a pediatric cardiology center? Equipment and facilities Delivery room (regular OR) Delivery room (regular OR) Prepared for neonate resuscitation! Doppler and Doppler and cardiotocography cardiotocography for fetal monitoring Milk bank Milk bank UNIDADE FETAL
Results 2/2009 to 6 2/2009 to 6/2012: /2012: 5 in 5 in hospital medical hospital medical treatment for fetal treatment for fetal tachiarrhythmias tachiarrhythmias 19 19 fetal cardiac procedures (7 were performed before the fetal unit fetal cardiac procedures (7 were performed before the fetal unit was established at HCor was established at HCor)) > 100 > 100 deliveries of babies with severe CHD deliveries of babies with severe CHD planned C section planned C section (38 (38 39 weeks GA) (2,5 2,5 deliveries / month) deliveries / month) d li i / th) DELIVERY AT HCor ONLY FOR BABIES WHO NEEDED INTERVENTION IN THE FIRST DAYS OF LIFE
Results Deliveries HEART DISEASE Nº HLHS 32 TGA IVS 16 PA IVS & PA complex CHD 16 OTHER 32* TOTAL 96 * LVOTO, CoAo, IAo Arch, Tumors,Critical PS, Critical AS,etc.
B fi f f l i i Benefits of a fetal unit inserted in Pediatric Cardiology center d i P di i C di l No transportation of unstable babies N t t ti f t bl b bi Mother and baby in the same hospital (no separation trauma) Cardiac team prepared to receive the sick neonate immediately after birth Definitive treatment performed earlier D fi iti t t t f d li High rates of breast High rates of breast feeding (even in HLHS) feeding (even in HLHS) UNIDADE FETAL
Benefits of a fetal unit inserted in Pediatric Cardiology center Antegrade aortic flow Ductal flow O2 sats = 95% O2 sats = 55% 2nd hour of life
Benefits of fetal unit inserted in Pediatric Cardiology center 32% * 68% SURVIVAL DEATH *Including RAI + obstructed veins, LAI + CHB, HLHS IAS, PA IVS + left coronary fistulas etc (some died before intervention) UNIDADE FETAL
What e are getting no! What we are getting now! Pediatric cardiology program + Fetal cardiac program Complete fetal medicine program* + Infertility center *Fetal transfusion; Laser for TT-TS; Tracheal occlusion for diaphragmatic hernia; Vesical/PleuralDrainage
F t l i t Fetal interventions ti initial experience i iti l i
F t l i t Fetal interventions ti Period: 07/2007 to 12/2012 Pts: 18 Procedures: 19 Atrial septostomy: 4 Pulmonary valvuloplasty: 3 Aortic valvuloplasty: 12 Gestational age: 23 35 (29 2,5 weeks) From April to Dec/2011 6 new cases From April to Dec/2011
F t l i t Fetal interventions ti Methods: Multidisciplinary team (fetal cardiologist, fetal medicine specialist interventional cardiologist) medicine specialist, interventional cardiologist) Operating room p g Tocolysis prophylaxis: Ca++ Blockers Maternal spinal anesthesia + midazolan
Fetal positioning external version Fetal anesthesia: Intramuscular or chordal Fentanil + Pancuronium + Atropine
F t l i t Fetal interventions ti 17 & 18 G 17 & 18 G 15 cm long Chiba needles Pre mounted balloon Marked system
Fetal interventions li i atrial septostomy i l Indications HLHS Intact IAS / restrictive ASD T b l fl Turbulent flow across IAS IAS Dilated LA Bidirectional flow PV
F t l i t Fetal interventions ti atrial septostomy t i l t t 4 cases All technically successful procedures Created ASDs: 2,5 mm 3mm Complications 1 fetal demise 12 hs after 1 thrombus formation LV RA LA RA LA
F t l i t Fetal interventions ti atrial septostomy t i l t t Case II Case IV (+fetal aortic valvuloplasty)) valvuloplasty IAS stenting 2 weeks of life Hybrid I Balloon septostomy h f lif 2nd hour of life Norwood/Glenn (6 months age) Death h Long NICU stay Case I
F t l i t Fetal interventions ti pulmonary valvuloplasty l l l l t Case 1 Case 1 PA IVS Case 2 critical PS Case 2 Mild to moderate RV hypoplasia Mild to moderate RV hypoplasia TV z scores = 2,5 TV z scores = TV z scores = 1 TV z scores = PV z scores PV z scores = PV z scores = 1 PV 8 PV z scores = 0,8 Case 3 critical PS Case 3 Moderate RV hypoplasia TV z scores = 2 TV z scores = PV z scores = PV z scores = 1 Case 1 Case 2 Case 2
F t l i t Fetal interventions ti pulmonary valvuloplasty l l l l t 26 semanas pré intervenção 34 semanas
F t l i t Fetal interventions ti Pulmonary valvuloplasty Case 1: unsuccessful Postnatal: RF perforation + PDA stent l f i Case 2: successful C f l Antegrade ductal flow @ 37 weeks Postnatal balloon valvuloplasty + PDA stent Case 3: successful Antegrade ductal flow @ 38 weeks y Now 10 days old: NICU on PGs
Procedure balloon 37 weeks gestation Forward ductal flow Immediately after Forward flow PV + PI 2nd day of life Following day Ductal spasm / retrograde flow PV + ductal stent
After fetal and neonatal pulmonary p y valvuloplasty (+PDA stent) 5 months of age
F t l i t Fetal interventions ti Aortic valvuloplasty A ti l l l t Indications Predictors of success CAS/AoA with impending HLHS CAS/AoA Retrograde flow transverse arch L R shunt atrial level Monophasic LV inflow LV long axis Z score > 2 LV long axis Z score > MV annulus Z score > 3 MV annulus Z score > Gradient across AoV Gradient across AoV 10 mmhg Gradient LV LA (MR) 15 mmhg Gradient LV McElhinney et al Circulation et al Circulation. 2009. 2009
F t l i t Fetal interventions ti Aortic valvuloplasty 12 cases Severe hydrops: 3 Severe MR: 8 EFE: 12 Hypoplastic LV: 3 ( (1 needed atrial septostomy) d d t i l t t ) LV
Aortic valvuloplasties outcomes 12 cases (3 hypoplastic (3 hypoplastic LV) Premie viable 1 died after BAV biventricular Term viable 8 Term viable 8 Biventricular 2 Borderline LV 2 Neonatal valvuloplasty 2 Hybrid 2 Hybrid = 2 (1 had NBAV) Survival = 4 Technically successful = 11 Univentricular 4 Hybrid = 2 H b id Survival = 1 Premie severe hydrops 2 Died after birth Supportive care = 1 Norwood = 1 2o stage death Death = 1
Pre procedure Procedure@ 30 wks 35 weeks MR 35 weeks 2nd day of life
All fetal interventions Complications Maternal: 0 Fetal demise: 1 B d Bradycardia di : 8 (needed epinephrine) 8 ( d d i hi ) Tamponade : 7 (needed drainage) Thrombus formation: 1
F t l i t Fetal interventions ti Lessons learned from Boston experience Ideal GA for procedures: Valvuloplasties: 22 to 30 weeks S Septostomy: 28 to 33 weeks 8 k Unfortunately most of the cases are referred too late!
F t l i t Fetal interventions ti Lessons learned with time for valvuloplasty Give intracardiac epinephrine before inflating the balloon At least 3 balloon inflations (for valvuloplasties) 3 ( p ) Valve insufficiency after the procedure is a marker of success and improve with time
F t l i t Fetal interventions ti Other potential interventions TGA with severely restrictive PFO Tricuspid valve anomalies and restrictive PFO D i l i f TV i Device occlusion of TV in severe regurgitant i lesions
F t l i t Fetal interventions ti Other potential interventions Aortic valvuloplasty in HLHS CNS and coronary blood flow (better neurological outcomes and RV fxn?) + maternal hyperoxigenation* pulmonary venous return antegrade aortic flow (increase LH size?) *According to Kohl Pediatric Cardiology 2010
27 + weeks immediately before the procedure 30 + weeks 2 weeks after the procedure
A ti l l l t in hypoplastic Aortic valvuloplasty i h l ti LV Maternal hyperoxygenation Started in the 33rd week gestation Five day in hospital course (~8 hrs per day) 2 courses (@ 33rdd and 35 d th week) k Daily fetal echo/ultrasound control Elective delivery at our center 38th week Elective delivery at our center
3 months of age h f Aortic valvuloplasty in hypoplastic LV + maternal hyper oxigenation @ 5 months of age after hybrid procedure 24 hs after Hybrid procedure 24 hs AV z score pre = 2,9 AV z score post= 1 MV z score pre = 3,3 MV z score post= 1,5 LVLA z score pre = 4 LVLA z score post= 2,5 AscAo z score pre = 0,7 Asc Ao z score post= +2
F t l i t Fetal interventions ti Conclusions Fetal interventions are feasible in experienced hands (skilled fetal specialist and pediatric interventionalist) Prenatal treatment may change the natural history of severe CHD With increasing experience, it is likely that the range of indications will be broadened. Aortic valvuloplasty in hypoplastic LV may be considered in association with maternal hyperoxygenation in selected patients