作者: Peter J. Bartz , David J. Driscoll , Joseph A. Dearani , Francisco J. Puga , Gordon K. Danielson
DOI: 10.1016/J.JACC.2006.07.053
关键词:
摘要: OBJECTIVES We sought to evaluate the early and late results of modified Fontan operation for patients with heterotaxy syndrome, assess variables traditionally known correlate poor outcome, current health status survivors after procedure. BACKGROUND The natural history unoperated cardiac lesions in is be poor. Therefore, has been proposed improve survival. METHODS Patients were identified from Mayo Clinic database. Hospital outpatient records abstracted preoperative, operative, postoperative clinical hemodynamic data. All not deceased sent questionnaires. RESULTS A total 142 syndrome had a operation. Asplenia was present 76 (54%). Median age at 9 years (range 2 35 years). follow-up 4 0 23 There 61 deaths (43%), 32 (23%) within 30 days or before hospital discharge. 5-, 10-, 15-year survival 64%, 57%, 53%, respectively. In modern era (1995 2004) mortality 10%. Of 81 survivors, questionnaires available 41 (51%). Eighty percent reported having no mild symptoms. However, 19 (46%) arrhythmias, 5 (12%) thromboembolic event, 1 (2%) developed protein-losing enteropathy. CONCLUSIONS Early improved operation; however, morbidity remain substantial. Better strategies long-term treatment this high-risk group need identified.