Comprehensive Cardiac Assessment and Assisted Reproductive Technology Considerations in a Long-Lost Patient With Fontan Circulation.

BACKGROUND

Women with Fontan physiology (modified World Health Organization class III-IV) increasingly pursue pregnancy while assisted reproductive technology in this physiology adds additional hemodynamic and thrombotic risks.

CASE SUMMARY: A 29-year-old woman with tricuspid atresia palliated by a 22-mm extracardiac Fontan, lost to cardiology follow-up since age 15, was referred by her reproductive endocrinology and infertility specialist before assisted reproductive technology. Right heart catheterization, cardiac magnetic resonance, ambulatory monitoring, stress echocardiography, and laboratory testing showed a well-compensated Fontan without high-risk features. Prophylactic enoxaparin was started with ovarian stimulation and held briefly for oocyte retrieval; a comprehensive pregnancy heart team managed her care and followed her closely.

DISCUSSION: The case illustrates failed pediatric-to-adult transition, Fontan-specific preconception evaluation before assisted reproduction, and the importance of a multidisciplinary pregnancy team.

TAKE-HOME MESSAGE: Women with Fontan physiology considering pregnancy, with and without assisted reproductive technology, warrant preconception pregnancy heart team evaluation and individualized thromboprophylaxis; lapse in transition is a modifiable driver of late presentation.