ABC | Volume 112, Nº2, February 2019

Original Article Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation Gabriel Carmona Fernandes, Guilherme Viotto Rodrigues da Silva, Luiz Fernando Caneo, Carla Tanamati, Aida Luiza Ribeiro Turquetto, Marcelo Biscegli Jatene Instituto do Coração (InCor) – Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP – Brazil Mailing Address: Gabriel Carmona Fernandes • Rua Pintassilgo, 516, apt. 98. Postal Code 04514-032, Vila Uberabinha, São Paulo, SP – Brazil E-mail: gcfcarmona@gmail.com , gumasmam@hotmail.com Manuscript received March 20, 2018, revised manuscript July 23, 2018, accepted July 23, 2018 DOI: 10.5935/abc.20180256 Abstract Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. Objective: To evaluate the results of conversion from FK to TCPC. Methods: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. Results: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. Conclusions: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia. (Arq Bras Cardiol. 2019; 112(2):130-135) Keywords: Heart Defects Congenital/surgery; Arrihythmias, Cardiac/surgery; Fontan Procedure; Mortality; Fontan‑Kreutzer Prodedure. Introduction The Fontan operation (FO) is an important landmark in the history of congenital heart diseases because it increased the life expectancy of children with single-ventricle hearts. 1,2 After the development of the superior cavopulmonary connection (Glenn operation), the survival rate in univentricular hearts increased leading to the development of FO. The first description by Fontan and Baudet, 3 was depicted as a right‑heart bypass in patients with tricuspid atresia to improve the basal saturation and consequently improve their quality of life and life expectancy while avoiding the complications of chronic hypoxia. These and other techniques that use atrial as a conduit are called atrium-pulmonary connections. Many other techniques and strategies for Fontan operation have been developed since it´s description. A few years after the first description, in 1973, this technique was modified by Kreutzer, 4 where the right atrial appendage was connected directly to the trunk of the pulmonary artery with a shorter surgical time than Fontan's previous description. The Fontan-Kreutzer technique (FK) was widely performed and diffused at the beginning, but complications were observed in the long range, such as enlarged atrium, atrial arrhythmias, stasis intracavitary thrombosis and compression of pulmonary veins. 5-9 These complications are difficult to treat leading to worsening functional class by New York Heart Association (NYHA) and often evolving to ventricular dysfunction and failure of the univentricular circulation. The next technique, described by de Leval in 1988, 10 , was the cavopulmonary connection using intra-atrial lateral tunnel. In 1990, Marcelletti et al. 11 described the total cavopulmonary connection (TCPC) using extra-cardiac tube. In subsequent studies it was observed that the TCPC presented better results than the previous techniques. 2,12-16 Nowadays the TCPC is themost used, however, many patients in whom the old techniques, such as FK, were performed survived and it was possible to observe long-term complications. A treatment option for these patients was to performa conversion of the FK to TCPC. The removal of the atrium from the pulmonary circulationwoulddecrease the volumetric overload reducing atrial dimensions and consequently lessening secondary outcomes. 17-26 130