Complications following the Fontan procedure include prolonged pleural drainage and readmission for effusions. To address these complications, a post-Fontan management pathway was implemented with primary goals of reducing chest tube duration/reinsertion rates and decreasing hospital length of stay and readmissions.

Fontan patients were identified by retrospective chart review (2017–2019) to obtain baseline data for chest tube duration/reinsertion rates, hospital length of stay, and readmission rates for effusion. A post-Fontan management pathway was implemented (2020–2021) utilising post-operative vasopressin, nasal cannula oxygen until chest tube removal, and discharge regimen of three times daily diuretics, sildenafil, and afterload reducing medications. Patients were followed to evaluate primary outcomes.

The pre- and post-pathway groups were similar in single ventricle morphology, demographics, and pre-operative haemodynamics. Forty-three and 36 patients were included in the pre- and post-pathway cohorts, respectively. There were statistically significant reductions in chest tube duration (8 vs. 5 days, p ≤ 0.001), chest tube output on post-operative day 4 (20.4 vs. 9.9 mL/kg/day, p = 0.003), and hospital readmission rates for effusion (13[30%] vs. 3[8%], p = 0.02) compared to baseline. There was an absolute reduction in hospital length of stay (11 vs. 9.5 days, p = 0.052). When combining average cost savings for the Fontan hospitalisations, readmissions for effusion, and cardiac catheterisations within 6 months of Fontan completion, there was a $325,144 total cost savings for 36 patients following pathway implementation.

Implementation of a post-Fontan management pathway resulted in significant reductions in chest tube duration and output, and readmission rates for effusion in the perioperative period.

The bi-directional cavopulmonary anastomosis forms an essential staging procedure for univentricular hearts. This review aims to identify risk factors for morbidity, mortality, and suitability for Fontan completion.

A total of 114 patients undergoing cavopulmonary anastomosis between 1992 and 2012 were reviewed to assess primary – mortality and survival to Fontan completion – and secondary outcome endpoints – re-intubation, new drain, and ICU stay. Median age and weight were 8 months and 6.9 kg, respectively. In 83% of patients, 1–3 interventions had preceded. Norwood-type procedures became more prevalent over time.

Extubation occurred after a median of 4 hours, median ICU stay was 2 days; 10 patients (8.8%) needed re-intubation and 18 received a new drain. Higher central venous pressure and transpulmonary gradient were risk factors for new drain insertion (p<0.01). Higher pre-operative pulmonary pressure correlated with increased inotropic support and prolonged intubation (p=0.01). Need for re-intubation was significantly affected by younger age at operation (p=0.01). Hospital and pre-Fontan mortality were 11.4 and 5.3%, respectively. Operative mortality was independently affected by younger age (p=0.013), lower weight (p=0.02), longer bypass time (p=0.04), and re-intubation (p=0.004). Interstage mortality was mainly influenced by moderate ventricular function (p=0.03); 82% of survivors underwent or are candidates for Fontan completion.

The cavopulmonary anastomosis remains associated with adverse outcomes. Age at operation decreases with rising prevalence of complex univentricular hearts. Considering the important impact of re-intubation on hospital mortality, peri-operative management should focus on optimising cardio-respiratory status. Once this selection step is taken, successful Fontan completion can be expected, provided that ventricular function is maintained.

The management of patients with Fontan physiology who undergo scoliosis surgery is difficult. The purpose of this article was to describe our experience in the management of patients with Fontan circulation undergoing spinal surgery for correction of scoliosis.

This was a retrospective study including patients with Fontan physiology who underwent spinal orthopaedic surgery. Anaesthetic management, post-operative complications, paediatric intensive care unit and total hospital stay, and the need for blood transfusions were analysed.

We identified eight children with Fontan physiology who had undergone spinal surgery from 2000 to 2010. All patients were receiving cardiac medications at the time of spinal surgery. The mean age at surgery was 14.8 years (range 12–21). In all, three patients needed inotropic support with dopamine (3, 5, and 8 μg/kg/min), which was started during surgery. During the immediate post-operative period, one patient died because of hypovolaemic shock caused by massive bleeding and dysrythmia. Mean blood loss during the post-operative period was 22.2 cc/kg (7.8–44.6). Surgical drainages were maintained for a mean time of 3 days (range 1–7). The mean hospital stay was 9.2 days (range 6–19). Pleural effusions developed in two patients. On follow-up, one patient presented with thoracic pseudarthrosis and another with a serohaematoma of the surgical wound.

Spinal surgery in patients with Fontan circulation is a high-risk operation. These patients must be managed by a specialised team.