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Open in new tab Download slide OBJECTIVES Effects of aortopulmonary collaterals (APCs) on outcomes after the total cavopulmonary connection (TCPC) are unclear. This study evaluated the incidence of APCs before and after TCPC and analysed the impacts of APCs on adverse outcomes. METHODS A total of 585 patients, who underwent TCPC from 1994 to 2020 and whose preoperative angiographies were available, were included. Pre-TCPC angiograms in all patients were used for the detection of APCs, and post-TCPC angiograms were evaluated in selected patients. Late adverse events included late death, protein-losing enteropathy (PLE) and plastic bronchitis (PB). RESULTS The median age at TCPC was 2.3 (1.8–3.4) years with a body weight of 12 (11–14) kg. APCs were found in 210 patients (36%) before TCPC and in 81 (14%) after TCPC. The closure of APCs was performed in 59 patients (10%) before TCPC, in 25 (4.2%) at TCPC and in 59 (10%) after TCPC. The occurrences of APCs before and after TCPC were not associated with short-term or mid-term mortality. The APCs before TCPC were associated with chylothorax (P  = 0.025), prolonged chest tube duration (P  = 0.021) and PB (P  = 0.008). The APCs after TCPC were associated with PLE (P  < 0.001) and PB (P  < 0.001). With APCs following TCPC, freedom from PLE and PB was lower than without (P  < 0.001, P  < 0.001). CONCLUSIONS APCs before TCPC were associated with chylothorax, prolonged chest tube duration and PB. APCs after TCPC were associated with both PLE and PB. The presence of APCs might affect the lymph drainage system and increase the incidence of chylothorax, PLE and PB. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). METHODS In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. RESULTS The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8–3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P  = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P  = 0.007, 20 years: P  = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P  < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P  = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P  < 0.001). CONCLUSIONS There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Our aim was to evaluate outcomes following a total cavopulmonary connection (TCPC) in patients with preoperatively impaired ventricular function (VF). METHODS: Of 483 consecutive TCPC patients, 44 (9.1%) had impaired VF (ejection fraction <50%, Group A), and 439 patients had normal VF (ejection fraction >_50%, Group B). We compared the clinical outcomes between the groups. RESULTS: The median age at TCPC was 2.8 (interquartile range 1.9-8.3) years in Group A and 2.3 (1.8-3.5) years in Group B (P = 0.025). An atrioventricular valve (AVV) operation prior to (38.6 vs 27.1%, P < 0.001) and concomitant with (31.8 vs 12.1%, P < 0.001) the TCPC was performed more frequently in Group A. The median intensive care unit stay (7.0 vs 7.0 days, P = 0.737) and 30-day survival (97.7 vs 98.4%, P = 0.737) were not significantly different between groups. Freedom from death, transplantation (P = 0.115) and catheter intervention (P = 0.603) showed no difference between groups. However, freedom from cardiac reoperation was significantly lower in Group A (P < 0.001). VF was resolved in 22 of the 39 (56.4%) survivors in Group A. The recovered patients had a lower incidence of AVV reoperation (0 vs 6, P = 0.002) and pacemaker rhythm (0 vs 5, P = 0.006). CONCLUSIONS: In patients planned for TCPC, impaired VF is often associated with AVV regurgitation. TCPC can be performed with low risk and comparable clinical results except for cardiac reoperation in patients with impaired VF when compared to patients with normal VF. Following TCPC, VF recovers in half of the survivors. A competent AVV and sinus rhythm are prerequisites for recovery. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. METHODS: Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC <18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. RESULTS: Median age at TCPC was 1.4 (interquartile ranges: 1.3–1.5) years in group A and 2.5 (1.9–4.5) years in group B. Duration of intensive care unit stay (6 vs 7 days), hospital stay (20 vs 20 days), and 30-day survival (100 vs 98%) was not significantly different between groups. Estimated survival (95.3 vs 92.1%), freedom from reoperation (93.7 vs 86.3%), freedom from catheter intervention (60.1 vs 77.0%), and freedom from protein losing enteropathy (97.6 vs 93.8%) at 10 years was not significantly different between groups. At last follow-up, no patient in group A but 13 patients in group B exhibited reduced ventricular function (P = 0.035). Exercise-capacity testing showed that both peak oxygen uptake (36.4 vs 28.6 ml/kg/min; P = 0.026) and its percentage of predicted value (82.9 vs 70.0%; P = 0.004) were significantly higher in group A (n = 6, mean postoperative period: 8.9 years) than in group B (n = 119, mean postoperative period: 8.7 years). CONCLUSIONS: Fontan completion for TCPC can be performed at an early age with a low perioperative risk and good intermediate results. Earlier unloading of the systemic ventricle and earlier elimination of chronic cyanosis by staged cavopulmonary connection might be advantageous for preservation of long-term ventricular function. [ABSTRACT FROM AUTHOR]

OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis. CONCLUSIONS: Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern. [ABSTRACT FROM AUTHOR]

Aims: Patients with congenital heart disease usually show diminished exercise capacity and quality of life. However, there is only little information about daily activity, a marker for lifestyle, exercise capacity, and the prevention of arteriosclerosis. This study investigated exercise capacity, quality of life, daily activity, and their interaction with univentricular heart physiology after total cavopulmonary connection (TCPC). [ABSTRACT FROM PUBLISHER]