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Pregnancy in the Patient Post Fontan operation: Results of a Registry Mary M. Canobbio, RN, MN, FAAN Lecturer, School of Nursing Clinical Nurse Specialist , Ahmanson-UCLA Adult Congenital Heart Disease Center Pregnancy in the Patient Post Fontan operation The Fontan procedure and its modifications is the definitive operation for a number of complex congenital heart defects (CHD). Cardiac complications after Fontan surgery are common and survival is limited The number of females of childbearing age with Fontan operations continues to increase. It is important to determine longterm outcomes after pregnancy. Fontan Registry Multi-centered collaboration Lead Investigators Mary Canobbio, Jamil AboulHosn. UCLA ACHD Center, School of Nursing, LA Calif. Frank Cetta, Carole Warnes, Mayo Clinic, Rochester, MN,* Candice Silversides, Jack Colman, * Mount Sinai Hospital, University of Toronto,ON Contributors Children's Hospital of Boston, Boston, MA* Le Bonheur Children's Medical Center, Memphis, TN* Medical College of Georgia, Augusta, GA* University of Arkansas, Little Rock, AR* University of Southern California,Los Angeles,CA Yale School of Medicine * Reported in original cohort . Canobbio, Mair, JACC 1996. Purpose The purpose of this multi-centered study was to determine the short and long-term outcomes of pregnancy in women following Fontan operation. Pregnancy Fontan operation Methods Retrospective chart reviews were performed of women at 10 North American centers who reported a pregnancy after Fontan operation Pregnant women with Fontan operation followed during and after pregnancy were included in this study. Pregnancy outcomes in 14 women were previously reported.* Clinical data pertaining to pregnancy outcomes and late outcomes were obtained by review of the medical records *Reported in original cohort . Canobbio, Mair, JACC 1996. Total Number of Pregnancies: N= 93 93 Fetal demise 3 (3%) Abortions 27 (29%) Live births = 63(68%) Miscarriage 22 (24%) Elective AB 5 (5.5.%) Characteristics of Mothers who had a live birth : N=60 Age at last Fontan surgery: 15.2 yrs (2-38) Primary Defect T.atresia 22 (35%) DILV 17 (6%) DIRV 2 (3%) DOLV 2 (3%) DORV 8 (13%) Univentricular 12 (20%) Type of Fontan Connection (58) RA-PA connection 21 10 21 RA-RV connection Lateral tunnel Cavo-pulmonary (with/without Glenn) Extra-cardiac conduit Cardiac history prior to 2 4 pregnancy SVT/Atrial flutter Ventricular dysfunction PLE Thrombosis/PE 20 3 3 4 Characteristics of Mothers who had a live birth : N= 63 Clinical status at time of pregnancy Pacemaker = 3 Ventricular function Prior to pregnancy (EF Mean): LV: 47 % RV: 51% At time of pregnancy LV:47% RV: 46% Cardiac Medications Antiarrhythmic Beta blockers ASA Diuretics Anticoagulation Digoxin N= 2 N= 5 N= 9 N=5 N= 5 N =17 Pregnancy Outcomes Live births: N=63 Median age at pregnancy 25 yrs (range17-36) Number of women with Multiple births One live birth Two live births Three live births 56 6 1 Obstetrical History Gestational age: 35 wks (25-40) Mode of delivery (N=60/63): Vaginal Cesarean section: Indication for C-section Cardiac : Obstetrical Anesthesia:(42/63) : Epidural = 32 General = 5 26 34 8 24 Spinal Unknown =5 = 21 Obstetrical History Adverse ma ternal cardiac events 24/56 (42%) Deaths Heart failure Arrhythmias 0 7 14 (Atrial =12, Ventricular =2) Desaturation 1 Pulmonary Embolus 1 Cardiac arrest during delivery 1 Timing of Cardiac Events Antepartum Labor and Delivery Early post partum period Eight month post-delivery Obstetrical complications 15 1 9 1 Pre-eclampsia Vaginal bleeding 4 4 2 3 1 Placental abruption PROM IUGR Ventral hernia(CS) Admission to ICU: 1 17/63 Monitoring Post arrest Incarcerated hernia A. fib post delivery V. dysfunction 13 1 1 1 1 Fetal Outcomes Birth weight: Median 2169 gm Neonatal death = (760-3572) 1 (premature) Congenital anomalies: Cardiac lesions (ASD/VSD) Cerebal palsy : Pul. Hyperplasia/club foot Sickle cell trait =2 =1 =1 =1 Follow-up Women 47 (82% of original cohort) Mean follow-up after last pregnancy 6.65yrs (range 1-22) Mean follow-up from 1st pregnancy 9.75 yrs Late Outcomes NYHA Functional Class (N=26/47) I =15 II =7 III=3 IV=1 Late deaths : 5 Cause of death: thromboembolic 2, ventricular failure 2, unknown 1 Long-term Survival 44 mothers at time 0. At 5 years there are 25 at risk. At 10 years there are 12 at risk. At 20 years there is only 1 at risk. CONCLUSION Pregnancy in women with Fontan circulation is associated with risk for the mother and baby. Cardiac and obstetric complication rates are high. There were no maternal deaths during pregnancy in this series. However, it is unclear from this data if long-term maternal mortality is related or impacted by pregnancy Detailed counseling continues to be warranted in this population…. To ensure successful outcomes