Prevalence and progression of recipient-twin cardiomyopathy in early-stage twin-twin transfusion syndrome

Abstract

Objective: The management of twin-twin transfusion syndrome (TTTS) in its early stages (Quintero Stages I and II) is controversial. We describe the prevalence, severity, incidence and rate of progression of recipient-twin cardiomyopathy in Stages I and II TTTS.

Methods: Among 451 cases of TTTS evaluated between 2004 and 2009, 123 (27.3%) cases of Stages I and II were reviewed. Echocardiography was used to 'upstage' cases based on the presence or absence of mild (IIIA), moderate (IIIB), or severe (IIIC) recipient cardiomyopathy. Progression was defined by worsening in the degree of recipient-twin cardiomyopathy from initial presentation or failure to respond to amnioreduction. Outcome data included progression of recipient-twin cardiomyopathy, treatment and survival to birth. Data were compared by the chi-square, Fisher's exact test or t-test as appropriate.

Results: Seventy-seven of 123 (62.6%) cases were Quintero Stage I and 46/123 (37.4%) Quintero Stage II. Eighty (65.0%) were upstaged to Cincinnati Stage IIIA (n = 25), IIIB (n = 23) or IIIC (n = 32). Management included observation in 11 (8.9%), amnioreduction in 26 (21.1%), amnioreduction followed by selective fetoscopic laser photocoagulation (SFLP) in 43 (35.0%) and primary SFLP in 43 (35.0%). Of 80 cases managed by observation or amnioreduction initially, 43 (53.8%) progressed within a mean duration of 1.4 ± 1.5 weeks. The incidence of progression increased significantly as degree of recipient-twin cardiomyopathy at presentation worsened: Stage I, 9/27 (33.3%); Stage II, 8/15 (53.3%); Stage IIIA, 8/16 (50.0%); Stage IIIB, 10/10 (100%); and Stage IIIC, 8/12 (66.7%) (χ(2) = 14, P < 0.01). Overall fetal survival was 205 out of 244 (84.0%). Fetal survival with observation only was 81.8% (18/22), with amnioreduction only it was 92.3% (48/52), with initial observation or amnioreduction followed by SFLP it was 86.9% (73/84) and with primary SFLP it was 76.7% (66/86).

Conclusion: Echocardiography demonstrates a high incidence of recipient-twin cardiomyopathy in early-stage TTTS. The more advanced the recipient-twin cardiomyopathy is, the more likely is progression to occur during observation or following amnioreduction.