Total cavopulmonary connection procedure in adults: A review of the medical literature

Abstract

Thanks to advances in surgical techniques, the number of patients with complex congenital heart disease reaching adulthood is increasing [1], [2]. 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 [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17]. 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 7 years (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 30 days 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 15 years 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 > 30 years, male sex, preoperative mean pulmonary arterial pressure > 15 mmHg, postoperative right atrial pressure > 20 mmHg, 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.