Rate and Outcomes of Pulmonary Stenosis and Functional Pulmonary Atresia in Recipient Twins with Twin-Twin Transfusion Syndrome

Fetal Diagn Ther. 2017;41(3):191-196. doi: 10.1159/000448075. Epub 2016 Jul 26.

Abstract

Objective: To evaluate the rate of pulmonary stenosis and functional pulmonary atresia (PS/PA) in recipient twins prior to fetal surgery for twin-twin transfusion syndrome (TTTS) and their pre- and postnatal outcomes.

Methods: We carried out a prospective study including 260 cases of TTTS. Echocardiography was performed before laser surgery to detect the presence of PS/PA. The outcomes of recipients with and without PS/PA were compared. The need of postnatal cardiac interventions and the survival rate at 6 months of age were also evaluated.

Results: PS was observed in 16/260 (6.2%) of recipient twins and PA in 12/260 (4.6%). After fetal surgery, 10/28 (35.7%) recipients died, 9/28 (32.1%) showed in utero regression, and 9/28 (32.1%) had persistence of PS/PA. Postnatally, seven recipients underwent percutaneous balloon pulmonary valvuloplasty, one required surgical valvotomy and one palliative surgery. Pregnancies with recipient twins with PS/PA had lower survival of at least one twin (67.9 vs. 83.6%, p = 0.045) and lower overall survival (57.1 vs. 72.8%, p = 0.015) at 6 months of age.

Conclusion: PS and PA were observed in 10.8% of recipients. Among these, about one third showed persistence of pulmonary valve pathology after delivery, which stresses the need for strict follow-up.

Keywords: Laser surgery; Pulmonary atresia; Pulmonary stenosis; Recipient twin; Twin-twin transfusion syndrome.

MeSH terms

  • Adult
  • Cohort Studies
  • Female
  • Fetofetal Transfusion / diagnostic imaging*
  • Fetofetal Transfusion / mortality*
  • Follow-Up Studies
  • Humans
  • Pregnancy
  • Prospective Studies
  • Pulmonary Atresia / diagnostic imaging*
  • Pulmonary Atresia / mortality*
  • Pulmonary Valve Stenosis / diagnostic imaging*
  • Pulmonary Valve Stenosis / mortality*
  • Survival Rate / trends
  • Treatment Outcome