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Outcomes After Surgery Historically, surgical repair of common arterial trunk has been associated with high mortality Thus the Pediatric Cardiac Care Consortium reported a mortality rate of 44% for patients who underwent surgery between 1985 and 1993.54 Although short-term outcomes have since improved, more recent audits continue to report significant mortality in this group of patients A recent update from the Society of Thoracic Surgeons reported an operative mortality of 9.4%.55 Brown and coworkers reported an early mortality rate of 17% for patients undergoing surgery between the years 1978 and 2000 The median age at the time of surgery was 76 days This report highlighted the contribution of associated cardiac anomalies, such as interruption of the aortic arch, or stenosis of the truncal valve, to perioperative mortality These anomalies were associated with a perioperative mortality of 29%, compared with 9% for patients without these complicating factors.56 Patients with the 22q11 deletion syndrome have been shown to require a more prolonged stay in the intensive care unit and in the hospital after surgery, as well as more reinterventions in the early postoperative period.57 A recent retrospective review of patients who underwent surgery in Melbourne, Australia, between 1979 and 2014, demonstrated an early mortality rate of 11.7% The requirement for postoperative extracorporeal membrane oxygenation, the weight of the patients, prior surgical intervention, and an anomaly of a coronary artery were identified as risk factors for early mortality.58 In this series, interruption of the aortic arch or the requirement for repair of the truncal valve was not a risk factor for early mortality Early transcatheter or surgical reintervention to the pulmonary arteries or right ventricular outflow tract will be required in up to one-in-three patients within the first year after surgery In a series of patients who underwent surgery in Boston, the use of a smaller conduit was an independent risk factor.59 In the medium term, the functional developmental outcome is satisfactory for most patients.60 However, after repair, many patients demonstrate ongoing and significant comorbidities, commonly requiring surgical and catheter-based interventions, with abnormalities in exercise performance, functional status, and health-related quality of life.61 The main problem in the medium term is related to the conduit placed between the right ventricle and pulmonary arteries In a recent series from Boston, in which the majority of patients received a homograft conduit, the freedom from either reintervention or surgery at 14 years was only 4%.62 Although replacement of the conduit between the right ventricle and the pulmonary arteries has become the definitive method to relieve obstruction, transcatheter angioplasty has been reported in a number of series, with variable outcomes (Fig 40.21) However, there are a number of reports that suggest that percutaneous implantation of stents may result in significant immediate hemodynamic improvement and, in some patients, may prolong the life span of the conduit by several years Transcatheter replacement of the pulmonary valve, using essentially a valved stent, is likely to revolutionize the care of these patients In an early multicenter study of the Melody valve, of 404 patients enrolled in the study, 46 had undergone repair of common arterial trunk.63 For smaller patients, a periventricular approach may be used.64 As newer valves are developed, the opportunities for percutaneous approaches in smaller patients will be expanded FIG 40.21 Transcatheter dilation of conduit stenosis (A) Right ventriculogram demonstrates a significant stenosis of the conduit (B) Inflation of the balloon revealed a waist at the site of the stenosis was relieved by further inflation (C) At the end of the procedure, a repeat ventriculogram demonstrated partial relief of the stenosis With improvements in perioperative survival, there is increasing information emerging about outcomes in the longer term In the Melbourne series of patients operated on between 1979 and 2014, the overall survival rate was 73.6% at 30 years.58 The presence of DiGeorge syndrome was a risk factor for late mortality Freedom from reoperation to the right ventricular outflow tract was 4.6% at 20 years In this series, the requirement for repair of an interrupted aortic arch or of the truncal valve were not risk factors for later mortality In a smaller series of patients who underwent surgery in Tokyo between 1974 and 2002, actuarial survival was 97% at 10 years and 93% at 40 years.65 However, freedom from reoperation to the pulmonary outflow tract was only 3% and from replacement of the truncal valve was 70% at 20 years after the initial surgery
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