Cardiac disease accounts for approximately 1% of all pregnancies. Tinman syndrome, though rare, is a disorder encompassing physical and/or electrophysiological cardiac mutations that may present at any time in life.  Historically, women with cardiac anomalies were discouraged from pregnancy. While medical advancements have increased the possibility of pregnancy in this population, they may also be minimizing the perception of risk and severity of complications for mom and baby. This presentation will cover the challenges of dealing with the multidisciplinary care of a pregnant woman with an ICD/pacemaker and neonate with tetrology of fallot.

Case:

41 year old G7P3 with a rare genetic disorder causing cardiac anomalies ~NKX2.5 (otherwise known as the “Tin man” gene) begins prenatal care at 16 weeks for an unplanned pregnancy with unknown LMP. Maternal complications of pregnancy included severe itching due to cholestasis, vaginal bleeding and suspected chronic abruption.  A fetal echo at 24 weeks confirmed tetrology of fallot with pericardial effusions.  She was hospitalized for vaginal bleeding at 32 weeks and again at 33-36 weeks for vaginal bleeding, suspicious for chronic abruption.  The patient’s experience (“gut”) was challenged by several test findings and she struggled to trust herself as well as care givers.  The strong family history of varied cardiac anomalies throughout her family, herself and her children increased her anxiety. Several social factors, marital discord, family dynamics, financial stress, and spiritual distress presented unique challenges far exceeding the difficulty of her physical management. The need for a holistic approach to care for this patient and her family became evident as well as the educational needs of the staff caring for this family.

Conclusion:

Regardless of specialty, nurses must be aware of developing trends in all fields as multifactorial complications present themselves in these complex patients. Both maternal and neonatal complications may be compounded by the physiological alterations and risks innate to pregnancy.  As a trusted resource and advocate, RNs must have a holistic approach in their care by identifying and providing the necessary resources. The collaboration of varied services and medical specialties was necessary to provide consistent care, comfort and reassurance for this patient and her family.  The teamwork inspired staff to brainstorm and debrief, resulting in a building of trust among caregivers and the patient while providing the basis for safe, compassionate care.

Keywords: Tin man, ICD/pacemaker, Tetrology of Fallot, abruption, pregnancy and cardiac anomalies