The purpose of this study was to describe pregnancy in female heart recipients whose indication for transplantation was due to congenital heart defect. Data were collected by the National Transplantation Pregnancy Registry (NTPR) via questionnaires, phone interviews, and medical records. Total, there are 83 heart recipients with 141 pregnancies reported to the NTPR to date. Of these, 17 recipients (28 pregnancies) had an initial diagnosis of a congenital heart defect (hypoplastic left heart syndrome 7, transposition of the great vessels 3, tricuspid atresia 1, septal defect 1, fibroelastosis 1, and other 4). Mean age at first transplant was 12.4±10.7 yrs (range 0.05-29 yrs). The transplant to conception interval was 11.5±6.1 yrs and 52% of the pregnancies were reported as being planned. Conceptions occurred between June 1990 and August 2014. Immunosuppression during all the pregnancies was calcineurin-inhibitor based with mycophenolic acid product (MPA) exposure in 7. Outcomes included: 7 miscarriages (all MPA exposure) and 22 live births (one set of twins) with a mean gestational age of 35.4±4.1 wks and a mean birthweight of 2403±777 g. Four children had birth defects: 1 duodenal atresia, Tetralogy of Fallot and atrioventricular canal defect (MPA exposure), 1 facial defects (MPA exposure), 1 pectus excavatum, 1 Dandy Walker syndrome with vermian hypoplasia of cerebellum, and a 5th with long QT syndrome diagnosed later in childhood. Comorbid conditions during pregnancy: hypertension 13 (46%) and preeclampsia 6 (27%). One recipient had mild rejection during pregnancy treated with an increase in oral prednisone. One recipient was retransplanted 12 yrs post-delivery but subsequently died, another died 1.7 yrs after pregnancy, one reported reduced function, and 14 recipients reported adequate graft function.

Conclusions: Female heart transplant recipients born with congenital heart defects have reported successful pregnancies with a majority reporting adequate graft function postpartum. MPA exposure in this group confers increases in miscarriages and birth defects. The higher frequency of birth defects in the offspring not exposed to MPA in utero in this group warrants further study. All transplant centers are encouraged to refer pregnancies to the NTPR.

CITATION INFORMATION: Coscia L, Armenti D, Constantinescu S, Moritz M. Pregnancy Outcomes in 17 Female Heart Transplant Recipients with the Initial Diagnosis of Congenital Heart Defect. Am J Transplant. 2016;16 (suppl 3).

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