The twin-twin transfusion syndrome: spectrum of cardiovascular abnormality and development of a cardiovascular score to assess severity of disease.

Abstract

Current means of grading twin-twin transfusion syndrome does not characterize cardiovascular aspects adequately. We sought to develop a score that describes the magnitude of cardiovascular severity in twin-twin transfusion syndrome. Fetal echocardiograms of 150 monochorionic/diamniotic twins were reviewed. Blinded to Quintero stage, we applied a cardiovascular score to each twin set and compared it to the Quintero grade. The score is a composite of variables that include ventricular hypertrophy, dilation, function, valve regurgitation, great artery size, and diastolic properties in the recipient and umbilical artery flow in the donor. Doppler indices of vascular and ventricular function were measured. Mean age was 21 +/- 3 weeks. Discrepancy was noted in degree of severity between Quintero and cardiovascular stages. The score correlated well with myocardial performance index of the recipient right ventricle (r2 = .65). We describe the spectrum of cardiovascular abnormalities that are seen in twin-twin transfusion syndrome and propose a scoring system for assessment of severity.