Thanks to advances in surgical techniques, the number of patients with complex congenital heart disease reaching adulthood is increasing [1Marelli A.J., Ionescu-Ittu R., Mackie A.S., Guo L., Dendukuri N., Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010 Circulation 2014 ;  130 (9) : 749-75610.1161/CIRCULATIONAHA.113.008396 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 2Stuart A.G. Changing lesion demographics of the adult with congenital heart disease: an emerging population with complex needs Future Cardiol 2012 ;  8 (2) : 305-31310.2217/fca.12.8 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti]. Among them, patients with not palliated or not fully palliated univentricular hearts remain difficult to manage. The benefit–risk balance for total cavo pulmonary connection (TCPC) is very difficult to assess in these patients. This review aims to describe the characteristics of TCPC surgery in adults.

We reviewed publications between 1980 and 2020 and studied the characteristics of TCPC surgery in adults: the preoperative patient's characteristics, the indications for surgery, the surgical techniques, the short- and long-term results. We found 15 articles [3Humes R.A., Mair D.D., Porter C.B., Puga F.J., Schaff H.V., Danielson G.K. Results of the modified Fontan operation in adults Am J Cardiol 1988 ;  61 (8) : 602-60410.1016/0002-9149(88)90772-2 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 4Warnes C.A., Somerville J. Tricuspid atresia in adolescents and adults: current state and late complications Br Heart J 1986 ;  56 (6) : 535-54310.1136/hrt.56.6.535 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 5Gates R.N., Laks H., Drinkwater D.C., e al. The Fontan procedure in adults Ann Thorac Surg 1997 ;  63 (4) : 1085-109010.1016/s0003-4975(96)01256-8 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 6Veldtman G.R., Nishimoto A., Siu S., e al. The Fontan procedure in adults Heart 2001 ;  86 (3) : 330-33510.1136/heart.86.3.330 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 7Burkhart H.M., Dearani J.A., Mair D.D., e al. The modified Fontan procedure: early and late results in 132 adult patients J Thorac Cardiovasc Surg 2003 ;  125 (6) : 1252-125910.1016/s0022-5223(03)00117-x

Cliccare qui per andare alla sezione Riferimenti, 8Ovroutski S., Alexi-Meskishvili V., Ewert P., Nürnberg J.-H., Hetzer R., Lange P.E. Early and medium-term results after modified Fontan operation in adults Eur J Cardiothorac Surg 2003 ;  23 (3) : 311-31610.1016/s1010-7940(02)00829-1 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 9Mott A.R., Feltes T.F., McKenzie E.D., e al. Improved early results with the Fontan operation in adults with functional single ventricle Ann Thorac Surg 2004 ;  77 (4) : 1334-134010.1016/j.athoracsur.2003.10.011 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 10Fujii Y., Sano S., Kotani Y., e al. Midterm to long-term outcome of total cavopulmonary connection in high-risk adult candidates Ann Thorac Surg 2009 ;  87 (2) : 562-57010.1016/j.athoracsur.2008.10.040[discussion 570].  [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 11Valente A.M., Lewis M., Vaziri S.M., e al. Outcomes of adolescents and adults undergoing primary Fontan procedure Am J Cardiol 2013 ;  112 (12) : 1938-194210.1016/j.amjcard.2013.08.021 [inter-ref]

Cliccare qui per andare alla sezione Riferimenti, 12Podzolkov V.P., Zelenikin M.M., Yurlov I.A., e al. Immediate results of bidirectional cavopulmonary anastomosis and Fontan operations in adults Interact Cardiovasc Thorac Surg 2011 ;  12 (2) : 141-14510.1510/icvts.2010.253484

Cliccare qui per andare alla sezione Riferimenti, 13Roubertie F., Peltan J., Henaine R., e al. Early to midterm results of total cavopulmonary connection in adult patients Ann Thorac Surg 2013 ;  95 (3) : 941-94710.1016/j.athoracsur.2012.10.004 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 14Ly M., Roubertie F., Kasdi R., e al. The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients Ann Thorac Surg 2014 ;  98 (6) : 2181-218610.1016/j.athoracsur.2014.07.043 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti, 15Fuchigami T., Nagashima M., Hiramatsu T., e al. Long-term follow-up of Fontan completion in adults and adolescents J Card Surg 2017 ;  32 (7) : 436-44210.1111/jocs.13157</DOI>

Cliccare qui per andare alla sezione Riferimenti, 16Ono M., Beran E., Burri M., e al. Long-term outcome of preadolescents, adolescents, and adult patients undergoing total cavopulmonary connection J Thorac Cardiovasc Surg 2018 ;  156 (3) : 10.1016/j.jtcvs.2018.03.155[1166–1176.e4].

Cliccare qui per andare alla sezione Riferimenti, 17Yang J., He B.-C., Chen J.-M., e al. Results of surgery on adults with functional single ventricle without prior cardiac surgery in childhood J Card Surg 2019 ;  34 (12) : 1556-156210.1111/jocs.14306 [cross-ref]

Cliccare qui per andare alla sezione Riferimenti].

This surgery was most often performed in the second decade of the patients’ lives. The adult survivors with univentricular heart disease are more likely to have a left ventricle. The median time from palliation to TCPC is 6 to 7years (Table 1). The decision to perform a TCPC was based overwhelmingly on cyanosis. Atriopulmonary anastomosis gradually gave way to intracardiac TCPC and now to TCPC with the insertion of an extracardiac tube (Fig. 1). The postoperative mortality rate (less than 30days after surgery) is highly variable in the series ranging from 0 to 20%. Hemodynamic failure with low cardiac output of the systemic ventricle accounts for almost 50% of the causes of death in most series (Fig. 2). Mortality at 5, 10 and 15years reported in four series is 76%–95%, 71%–81% and 66%–68%, respectively (Table 2). Risk factors for mortality at a distance from the operation include age>30years, male sex, preoperative mean pulmonary arterial pressure>15mmHg, postoperative right atrial pressure>20mmHg, postoperative recovery for haemorrhage.

In adult patients who have not undergone surgery, the literature data show that the intervention is feasible with acceptable short- and long-term results.