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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

View larger version (39K): [in a new window]   Figure 1 (A) A two-dimensional image that demonstrates significant biventricular hypertrophy with reduced chamber size in the recipient of a twin-twin transfusion syndrome at 22 weeks of gestation. There is also a pericardial effusion and skin edema, as evidence of fetal hydrops and polyhydramnios. (B) Severe tricuspid insufficiency is also often seen as shown in this image. (C) In this same hydropic recipient twin, there was evidence of diastolic dysfunction including a prolonged isovolumic relaxation time, umbilical venous pulsations and, as shown here, significant A wave reversal in the inferior vena cava (IVC). LV = left ventricle; RA = right atrium; RV = right ventricle.

View larger version (42K): [in a new window]   Figure 2 (A) Echocardiographic image demonstrating pulmonary valve stenosis observed in one of the recipient fetuses at 27 weeks of gestation. The pulmonary valve was thickened and doming. By color flow mapping (B) and pulsed Doppler (C), there was flow disturbance below the valve suggesting the presence of subvalve obstruction as well. LV = left ventricle; PA = pulmonary artery; PV = pulmonary vein; RV = right ventricle; RVOT = right ventricular outflow tract.

View larger version (64K): [in a new window]   Figure 3 The cardiac pathology observed at postmortem in case 3 confirmed the presence of severe left (A) and right (B) ventricular hypertrophy. (C) There was muscular narrowing of the subpulmonary area, and the pulmonary valve was thickened and dysplastic.