393: Prevalence and progression of recipient twin cardiomyopathy in early stage twin-twin transfusion syndrome (TTTS)


      Management of TTTS in early stages (I, II) is controversial. We describe the prevalence, severity, and incidence of progression of recipient twin (RT) cardiomyopathy (CM) in early Quintero Stage I and II TTTS.

      Study Design

      Among 451 TTTS evaluated between 2004-2009, 124 (27%) cases of stages I and II were reviewed. The cases were “upstaged” to Cincinnati IIIA, IIIB, or IIIC if mild, moderate, or severe recipient cardiomyopathy (RTCM) was detected on echocardiography (ECHO), respectively. Progression of RTCM was defined by worsening of cardiomyopathy in subsequent ECHO or failure to respond to amnioreduction (AR). Outcome data included progression of RTCM, treatment, and survival at birth. Data were compared by Chi-square, Fisher's exact, or T- test as appropriate.


      There were 77/124 (62%) stage I and 47/124 (38%) stage II cases. 65% (81/124) were upstaged to Cincinnati IIIA (26/81), IIIB (23/81) and IIIC (32/81). Treatment included observation in 11 (9%), AR in 26 (20%), AR followed by laser procedure (SFLP) in 43 (35%), SFLP in 43 (35%), and cord coagulation (RFA) in 1. 43/80 (54%) cases treated by observation or AR initially progressed at a mean of 1.4±1.5 weeks. The incidence of progression increased significantly if RTCM was more advanced initially: 9/27 (33%) Stage I, 8/15 (53%) Stage II, 8/16 (50%) IIIA, 10/10 (100%) IIIB, and 8/12 (67%) IIIC (p < 0.01). Overall fetal survival was 82% (174/212) in 106 cases with outcome data.


      Echocardiography demonstrates a high incidence of RTCM in early stage TTTS. Progression is more likely for RT with more advanced CM.