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PEDIATRIC CARDIOLOGY

Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome

Jane Lougheed, MD^*,1, Brian G. Sinclair, MD, Karen Fung Kee Fung, MD, Jean-Luc Bigras, MD^*,2, Greg Ryan, MD^b, Jeffrey F. Smallhorn, MBBS^* and Lisa K. Hornberger, MD^*,*

^* Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, Toronto, Canada
^b the Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Canada
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Eastern Ontario, Ottawa, Canada
Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Ottawa General Hospital, University of Ottawa, Ottawa, Canada

Manuscript received March 5, 2001; revised manuscript received July 10, 2001, accepted July 26, 2001.

^* Reprint requests and correspondence: Dr. Lisa Hornberger, Division of Cardiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 2A1
hornberg{at}sickkids.on.ca

OBJECTIVES

The goal of this study was to determine the prevalence and evolution of acquired right ventricular outflow tract obstruction (RVOTO) in the recipient twin in twin-twin transfusion syndrome (TTTS).

BACKGROUND

Twin-twin transfusion syndrome complicates 4% to 26% of diamniotic monochorionic twin gestations and is associated with high fetal morbidity and mortality. Cardiac dysfunction and biventricular hypertrophy may develop in the recipient twin with the potential for RVOTO.

METHODS

This was a retrospective review of a two-center experience of TTTS to describe the prevalence and evolution of acquired RVOTO in the recipient twin. Right ventricular outflow tract obstruction was diagnosed or excluded by fetal or postnatal echocardiography or clinical assessment.

RESULTS

Of 73 twin pregnancies with TTTS identified between 1994 to 1998, a total of seven (9.6%) were complicated by RVOTO in the recipient twin: two subvalvar/muscular, four valvar and one combined. Of 44 pregnancies with fetal echo, six had in utero RVOTO with antegrade flow diagnosed at gestational ages ranging from 19 to 27 weeks. In utero progression occurred in four cases over a period of four to eight weeks, with the development of RVOT atresia by delivery. Postnatal progression of RVOTO occurred in two cases, one of which required pulmonary balloon valvuloplasty at age two years. Postnatal regression of subvalvar RVOTO occurred in two cases in early infancy. Death related directly or indirectly to the RVOTO occurred in all four patients who developed complete RVOT obliteration.

CONCLUSIONS

Right ventricular outflow tract obstruction may occur in the recipient twin of at least 9% of pregnancies complicated by TTTS. Right ventricular outflow tract obstruction progression is common in utero and may worsen neonatal outcome.

Abbreviations and Acronyms   BVH = biventricular hypertrophy   PS = pulmonary stenosis   PV = pulmonary valve   RVOT = right ventricular outflow tract   RVOTO = right ventricular outflow tract obstruction   TR = tricuspid regurgitation   TTTS = twin-twin transfusion syndrome

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