Download Pregnancy in the Patient Post Fontan operation: Results of a Registry

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