Single ventricle and pregnancy: yesterday, today and tomorrow
S.R. Mravyan^1, V.A. Petruhin^1, A.P. Melnikov^1, I.V. Barinova^1, V.P. Pronina^2, A.A. Golovin^1, D.A. Andreev2

¹ Moscow Regional Research Institute of Obstetrics and Gynecology
² Moscow regional research clinical institute named after M.F. Vladimirskiy

Summary
The paper analyzes management of pregnancy and delivery tactics in patients with single ventricle after Fontan procedure. Despite palliative nature of procedure patients have significantly better quality and duration of life and possibility of pregnancy. Published data points that these patients are characterized by thrombosis development (including placental vessels), arrhythmia, cardiac and placental insufficiency, enteropathy. Palliative surgery significantly improves maternal and perinatal outcomes. The clinical case presents a pregnant woman with single ventricle after Fontan procedure with detected hereditary thrombophilia, determined previous cases of miscarriage. This combination is very rare and unfavourable. Prescription and optimization of heparin dosages contributed to child bearing and thrombosis and embolism prevention. The most important in clinical practice are poor perinatal outcomes of pregnancy in women with single ventricle. Timely surgical correction and medical treatment of cervical incompetence allow making pregnancy as long as possible.
Anesthesia is of great importance during delivery. Choice of anesthesia is often determined by the urgency of surgery. Authors have analyzed own experience and literature data on anesthesia. It can be optimized by intraoperative fluid therapy with colloidal solution, correction of acidosis and hypokalemia. Placenta's examination revealed structural changes as hypoplasia, premature ripening of the chorionic villi., thickening of stem arteries, as well as numerous syncytial knots, indicating chronic uteroplacental ischemia.

Keywords: pregnancy; single ventricle; Fontan procedure.
For citation: Mravyan S.R., Petruhin V.A., Melnikov A.P. et al. Single ventricle and pregnancy: yesterday, today and tomorrow // RMJ. 2017. № 2. P. 140–144.