Tyson A Fricke1, Yves d'Udekem2, Christian P Brizard2, Gavin Wheaton3, Robert G Weintraub2, Igor E Konstantinov4. 1. Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia. 2. Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Melbourne, Victoria, Australia. 3. Women's and Children's Hospital, Adelaide, South Australia, Australia. 4. Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Melbourne, Victoria, Australia. Electronic address: igor.konstantinov@rch.org.au.
Abstract
BACKGROUND: Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution. METHODS: Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13 years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11 patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]). RESULTS: A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4 (24%) late deaths, occurring at 6 weeks, 3.5 years, 4 years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6 of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15 years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years. CONCLUSIONS: Surgical repair of SVAS in children Williams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up.
BACKGROUND:Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution. METHODS: Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13 years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11 patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]). RESULTS: A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4 (24%) late deaths, occurring at 6 weeks, 3.5 years, 4 years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6 of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15 years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years. CONCLUSIONS: Surgical repair of SVAS in childrenWilliams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up.
Authors: Annalisa Angelini; Cira di Gioia; Helen Doran; Marny Fedrigo; Rosa Henriques de Gouveia; Siew Yen Ho; Ornella Leone; Mary N Sheppard; Gaetano Thiene; Konstantinos Dimopoulos; Barbara Mulder; Massimo Padalino; Allard C van der Wal Journal: Virchows Arch Date: 2020-04-07 Impact factor: 4.064
Authors: Rosa Roemers; Jolanda Kluin; Frederiek de Heer; Sara Arrigoni; Regina Bökenkamp; Joost van Melle; Tjark Ebels; Mark Hazekamp Journal: World J Pediatr Congenit Heart Surg Date: 2018-03