Fetal and Neonatal Circulatory Disorders in Twin to Twin Transfusion Syndrome (The Secondary Publication)

J Nippon Med Sch. 2019;86(4):192-200. doi: 10.1272/jnms.JNMS.2019_86-301.


Twin to twin transfusion syndrome (TTTS) is a major complication of monochorionic diamniotic (MD) twins, and its onset is known to be associated with placental vascular anastomoses and blood flow imbalance. In a typical case of TTTS, the recipient develops polyhydramnios, weight gain, cardiomegaly and hydrops fetalis in the uterus. In contrast, the donor develops oligohydramnios and intrauterine growth restriction. Recently, the significance of the renin-angiotensin-aldosterone system (RAAS) that transfers from the donor to the recipient has attracted interest in the fetal circulation of TTTS. The donor has decreased renal blood flow due to decreased circulating blood volume. For this reason, the secretion of RAAS hormones is augmented in the fetal kidneys of the donor. In TTTS, these RAAS hormones from the donor transfer to the recipient through the anastomosed vessels. In addition to excess preload, the recipient heart is exposed to excess afterload due to systemic vasoconstriction through RAAS hormones. Commonly occurring complications in the recipient include myocardial hypertrophy, atrioventricular valve regurgitation, and pulmonary valve stenosis or pulmonary atresia. Fetoscopic laser photocoagulation (FLP) has been introduced recently because neither mortality nor neurological morbidity have been satisfactorily improved with conventional treatment. FLP is a curative method that may improve the prognosis of TTTS. In Japan, this procedure has been performed frequently, and positive neurological outcomes have been achieved.

Keywords: cardiac anomaly; cardiomyopathy; fetoscopic laser photocoagulation (FLP); recipient; twin to twin transfusion syndrome (TTTS).

Publication types

  • Review

MeSH terms

  • Blood Volume
  • Cardiomegaly / embryology
  • Cardiomegaly / etiology
  • Female
  • Fetal Diseases / etiology
  • Fetal Diseases / physiopathology
  • Fetal Growth Retardation / etiology
  • Fetofetal Transfusion* / diagnostic imaging
  • Fetofetal Transfusion* / etiology
  • Fetofetal Transfusion* / pathology
  • Fetofetal Transfusion* / therapy
  • Fetoscopy
  • Fetus / blood supply*
  • Humans
  • Low-Level Light Therapy
  • Polyhydramnios / etiology
  • Pregnancy
  • Prognosis
  • Pulmonary Valve Stenosis / embryology
  • Pulmonary Valve Stenosis / etiology
  • Renal Circulation
  • Renin-Angiotensin System / physiology