title: Insights into the pathophysiology of twin-twin transfusion syndrome.
abstract: Twin-twin transfusion syndrome (TTTS) is attributed to trans-anastomotic transfusion between twins. Anastomoses are ubiquitous in monochorionic (MC) placentae, yet TTTS develops in only 15%. Although ex vivo and in vivo studies fail to identify a unique anastomotic signature, TTTS placentae are typically associated with an imbalance in unidirectional arteriovenous anastomoses with absent bidirectional anastomoses. Doppler detection of an artery-artery anastomosis reduces the chance of TTTS, whereas, in those that develop the disease, it improves stage-independent survival. Selective laser is often curative, but an increasingly recognized risk of persistent or reverse TTTS may be attributable to atypical arteriovenous anastomoses not identifiable from the chorionic plate. Simple dysvolaemia fails to explain several phenotypic features, including haematological concordancy, recipient hypertension, and reversibly absent end diastolic flow in the donor. The renin-angiotensin system is upregulated in the donor and downregulated in the recipient's kidneys, while paradoxically raised renin levels in the recipient may contribute to raised afterload along with endothelin. Although research is limited in humans by therapy and the lack of a suitable experimental model, further studies of placental and vascular pathophysiology may not only refine current treatment modalities but may also, in addition, suggest further avenues for downstream management such as genetic predisposition testing or pharmacological intervention.
authors.affiliation: Experimental Fetal Medicine Group, Institute of Reproductive and Developmental Biology, Imperial College London, UK. p.galea@imperial.ac.uk
authors.forename: Paula
authors.initials: P
authors.lastname: Galea
authors.forename: Venu
authors.initials: V
authors.lastname: Jain
authors.forename: Nicholas M
authors.initials: NM
authors.lastname: Fisk
mesh_terms: D002823:Chorion / Q000000981:diagnostic imaging; D005260:Female; D005330:Fetofetal Transfusion / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D021041:Placental Circulation; D011247:Pregnancy; D012084:Renin-Angiotensin System; D014427:Twins; D016216:Ultrasonography, Prenatal