AIM: The twin-twin transfusion syndrome (TTTS) complicates 10-30% of monochorionic pregnancies. The incidence of pulmonary stenosis and endocardial fibroelastosis is especially high in the recipient twin. We report a novel finding of four cases of coarctation of the aorta and hypoplastic aortic arch in the donor to raise awareness of cardiac lesions in twins affected by TTTS. METHOD: Retrospective review of both neonatal database and mortality data from 2002 to 2007 with cross-validation from the local tertiary cardiology unit data (1998-2006) to identify children presenting with coarctation who were also twins. RESULTS: We identified four monochorionic twin pairs affected by the TTTS, delivered between 25 weeks and 36 weeks' gestation, where the donor was found to have coarctation of the aorta or a hypoplastic aortic arch. In addition, two of the four recipients also had cardiac abnormalities. There was a high mortality rate of 30% for both twins, and a high morbidity rate, especially for neurological sequelae. CONCLUSION: We believe that the types of abnormalities seen may be explained by the altered fetal blood flow and haemodynamics in TTTS. Given the increased prevalence of congenital heart disease in TTTS, with an increased risk of coarctation in the donor twin and pulmonary stenosis in the recipient, intra-uterine surveillance and a post-natal comprehensive cardiac assessment for both twins is warranted.
AIM: The twin-twin transfusion syndrome (TTTS) complicates 10-30% of monochorionic pregnancies. The incidence of pulmonary stenosis and endocardial fibroelastosis is especially high in the recipient twin. We report a novel finding of four cases of coarctation of the aorta and hypoplastic aortic arch in the donor to raise awareness of cardiac lesions in twins affected by TTTS. METHOD: Retrospective review of both neonatal database and mortality data from 2002 to 2007 with cross-validation from the local tertiary cardiology unit data (1998-2006) to identify children presenting with coarctation who were also twins. RESULTS: We identified four monochorionic twin pairs affected by the TTTS, delivered between 25 weeks and 36 weeks' gestation, where the donor was found to have coarctation of the aorta or a hypoplastic aortic arch. In addition, two of the four recipients also had cardiac abnormalities. There was a high mortality rate of 30% for both twins, and a high morbidity rate, especially for neurological sequelae. CONCLUSION: We believe that the types of abnormalities seen may be explained by the altered fetal blood flow and haemodynamics in TTTS. Given the increased prevalence of congenital heart disease in TTTS, with an increased risk of coarctation in the donor twin and pulmonary stenosis in the recipient, intra-uterine surveillance and a post-natal comprehensive cardiac assessment for both twins is warranted.
Authors: Tim Van Mieghem; Liesbeth Lewi; Léonardo Gucciardo; Philip Dekoninck; Dominique Van Schoubroeck; Roland Devlieger; Jan Deprest Journal: Int J Pediatr Date: 2010-08-08